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Cleft Lip and Palate Anomaly in India: Clinical Profile, Risk Factors and

Current Status of Treatment: A Hospital Based Study

Indian Council of Medical Research

Task Force Project Report

Indian Council of Medical Research (ICMR), New Delhi

and Centre for Dental Education and Research

All India Institute of Medical Sciences (AIIMS), New Delhi

Cleft Lip and Palate Anomaly in India: Clinical Profile, Risk Factors and

Current Status of Treatment: A Hospital Based Study

Indian Council of Medical Research

Task Force Project Report

Coordinating Centre

Centre for Dental Education and Research All India Institute of Medical Sciences, New Delhi

Contributing Centres


Safdarjung Hospital, New Delhi

Medanta- The MEDICITY Hospital, Gurgaon

Pre-pilot study

Duration- 2 years

March 2010 to March 2011

extended upto March 2012

Pilot study

Duration- 3 years

April 2012 to March 2014

extended upto June, 2015

Published By

Indian Council of Medical Research, New Delhi Division of Non Communicable Diseases

Dr. Bela Shah Former Head (Upto 11th Feb 2013 and

12th Feb 2016 – 31st May 2016) Dr. D. K. Shukla Former Head (12th Feb 2013 – 11th Feb 2016) Dr. R. S. Dhaliwal Head (1st June 2016 – till date) Dr. Ashoo Grover Scientist ‘E’, Programme Officer


Professor O. P. Kharbanda CHIEF - Centre for Dental Education and Research Head, Division of Orthodontics and Dentofacial Deformities

Published in 2016

Cleft lip and palate anomaly in India: Clinical profile risk factors and

current status of treatment: A hospital based study

PRINCIPAL INVESTIGATOR AND COORDINATOR

Prof. O. P. Kharbanda


CONTRIBUTORS

Centre for Dental Education and Research, AIIMS, New Delhi

Chief Investigator

Co-investogators

Prof. O. P. Kharbanda

Dr. S. C. Sharma

Dr. Madhulika Kabra

Dr. Sushma Sagar

Dr. Maneesh Singhal

Dr. Neerja Gupta

Dr. Manju Mehta

Safdarjung Hospital, New Delhi

Chief Investigator

Co-investogators

Dr. Karoon Agrawal

Dr. N. N. Mathur

Medanta-The MEDICITY Hospital, Gurgaon

Chief Investigator

Co-investogators

Dr. Rakesh Khazanchi

Dr. K. K. Handa

CONTRIBUTORS

Panel of experts

Dr. Anil Kohli, Delhi Dr. S.G. Damle, Mullana, Haryana

Dr. Ashok Utreja, Chandigarh Dr. K. Sreedharan, Chennai

Dr. T. Samraj, Salem Dr. I. C. Verma, Delhi

Dr. G. S. Meena, Delhi

Research staff engaged in the project

Institute Names Designation

ICMR HQs None

CDER, AIIMS, New Delhi 1. Dr. Neeraj Wadhwan Senior Research Officer

2. Ms. Parul J. Rathod Senior Research Fellow

3. Ms. B. Aarthi Data Entry Operator

4. Mr. Netra Pal Dental Technician

5. Ms. Nisha Bansal Computer Programmer

6. Ms. Neha Takhi Computer Programmer

7. Ms. Pooja Maurya Computer Programmer

Safdarjung Hospital, Delhi Dr Parul Narang Research Assistant

Medanta- The MEDICITY

Hospital Gurgaon Dr Upaasna Vinayak Research Assistant

ICMR

Dr. Bela Shah

Dr. D. K. Shukla Dr. R. S. Dhaliwal

Dr. Ashoo Grover

Dr. Ravinder Singh

NIC

Dr. Savita Dawar

Executive Summary

Cleft treatment requires a multidisciplinary approach extended from the birth until adulthood.

Many of these children are born in rural areas where resources for treatment and awareness

on cleft care are limited. Consequently, many patients may receive limited or suboptimal care due to various reasons. This study was aimed at evaluating and identifying the patterns of

the congenital defects of face, cleft lip and palate among patients visiting three major hospitals across Delhi and the National Capital Region (NCR). The objectives included

establishing baseline data on a spectrum of clinical profile of cleft patients, treatment protocols, quality of treatment and their residual treatment needs. The experience gained

from a study of these three target centres would then be used to lay a framework to conduct

a nationwide multicentre study in terms of logistics, feasibility and difficulties. The study titled Cleft Lip and Palate anomaly in India: Clinical profile, Risk factors and Current status of treatment: a Hospital-based study was started in 2010 as a task force project of ICMR. The pilot phase, which started in 2012, encompassed three cleft centres across Delhi and NCR,

namely, AIIMS, Safdarjung Hospital and Medanta-The MEDICITY. The relevant data for 126

subjects exhibiting non-syndromic cleft lip and palate was recorded on a specially designed performa. Each case was evaluated by a team of specialists comprising of a Plastic Surgeon,

an Orthodontist, an ENT Surgeon, a Dental Surgeon, a Speech therapist and an Audiologist. Clinical records included the subject’s profile and intraoral photos, dental study models,

audiometric and speech evaluation data. The current report highlights that among the samples of the cleft patients assessed in the project, the treatment needs were significantly

high. There was a wide variation in age at primary lip and palate surgery with a significant

percentage of cases requiring lip and nose revision surgeries. Fifty five percent cases had post-surgical oro-nasal fistula and a large proportion of operated UCLP cases had complex

orthodontic treatment needs.

There seems to be an urgent need to devise strategies to improve the delivery of

quality care to the afflicted subjects, with the joint efforts of all the experts and health care

providers. It must be mentioned here that the data is not representative of the outcome of the three centres. It also tries to highlight that larger multicentre studies are needed in the

Indian setup so that the patients not only receive treatment but also the quality of the treatment is monitored for better outcomes. The results indicate a lack of uniform protocol

followed in providing care to cleft patients. A great variation was found in the quality of

treatment received by many of the patients.

Forward

A comprehensive management of cleft patients requires a multidisciplinary approach

extended from birth until adulthood. Many of these children are born in rural areas where resources for treatment and awareness on cleft care are limited. Consequently, many patients

may receive limited or suboptimal care due to multitudes of reasons. This study was aimed to evaluate and identify pattern of the congenital birth defects of face, cleft lip and palate

among patients visiting three major hospitals across Delhi and National Capital Region (NCR). The study titled “Cleft Lip and Palate anomaly in India: Clinical profile, Risk factors and

Current status of treatment: a Hospital based study” was started in 2010 as a Task Force

project of ICMR. Pre-Pilot phase of the study was conducted between 2010 to 2012 in the Department of Orthodontics and Dentofacial Deformities and ENT, AIIMS, New Delhi. The

Pilot phase, which started in 2012, encompassed three cleft centres across Delhi and NCR, namely, AIIMS, Safdarjung Hospital and Medanta-The MEDICITY. Current report highlights

that larger multicentre studies are needed in the Indian setup so that the patients do not only

receive treatment but also the quality of the treatment is monitored for better treatment outcomes. The results indicate a lack of uniform protocol followed in providing care to cleft

patients. A great variation was found in the quality of treatment received by many of the

patients.

Report of three centres of Delhi and NCR presents the situation of the status of cleft

care in India. The profilometric analysis of cleft care has provided a glimpse on the ground realities related to the treatment of cleft patient in different parts of India. It is hoped that

this Report would be useful for researchers and planners in their endeavor to work towards strengthening the management of cleft lip and palate anomaly in the country and work out

guidelines /protocols for proper management of CLP in the Indian social milieu that is ailing

with limited health care resources.

Dr. Soumya Swaminathan

Director General, ICMR

Preface

Cleft lip and palate is the most common congenital deformity of the craniofacial region with

an average worldwide incidence of 1 in 700. Its incidence in Asian population is reported to be around 2.0 per 1000 live births or higher. In India, though national epidemiological data is

not available, many studies from different parts have reported a variation in the incidence of cleft anomaly. Based on rough estimates, it has been suggested that approximately 35,000

newborn cleft patients are added every year to the Indian population. With many patients having less than optimum care in a not-so-organized setup, the cumulative burden of persons

affected with this birth defect is huge. Although India has a large and extended network of

medical facilities, interdisciplinary cleft care is provided in only a few hospitals. Day-to-day interactions with these patients exhibit significant variation in treatment provided and the

quality of outcome, with some having had excellent treatment outcomes while many patients, unfortunately, received suboptimum, limited or no treatment at all. The reasons for this are

many and varied. The awareness in the society and amongst the health professionals on the

critical aspects of interdisciplinary care of this anomaly may be lacking. Affordability and availability of experts may also contribute to the quality of treatment. There is a lack of

interdisciplinary approach in majority of the centres, and hence, there is a need for better interaction amongst the specialists. This lack of interdisciplinary approach and the need for it

in the Indian setup has been stressed previously also. This ongoing Task Force Project was initiated by the Indian Council of Medical Research to evaluate the current status of treatment

and treatment needs of cleft patients. The ultimate aim is to work out a national registry and

guidelines for cleft care in India.

The long term objectives are to initiate a National Registry for the patients with

congenital birth defects of the face and jaws and also to establish strategies that will address a multitude of challenges associated with the prevention and treatment of this deformity. The

aim is to improve the quality of life (QOL) of children suffering from Cleft lip palate and such

deformities so as to offer them a hope for a normal living.

Professor O.P. Kharbanda

CHIEF - Centre for Dental Education & Research Head - Division of Orthodontics and Dentofacial Deformities

All India Institute of Medical Sciences, New Delhi PRINCIPAL INVESTIGATOR/ COORDINATOR- Task Force Project DIRECTOR- WHO Collaborating Centre for Oral Health Promotion

Acknowledgement

We gratefully acknowledge the valuable contribution of the Chairperson and the Members of the Task Force Group for providing continuous guidance and support in implementing the

pre-pilot and pilot phase of the study. We also acknowledge the investigators engaged in

undertaking the ICMR funded Task Force Study on “CLEFT LIP AND PALATE ANOMALY IN INDIA: CLINICAL PROFILE, RISK FACTORS AND CURRENT STATUS OF TREATMENT: A

HOSPITAL BASED STUDY (2012-2014)” and providing meaningful outcome in the form of ‘IndiCleft Tool’. The tool is ready to be taken up further in the multicentric nationwide study

which will be useful in achieving aims and objectives during the main phase. The members

enthusiastically participated in discussions and provided immensely useful inputs drawn from their vast experience in the subject. We also thank the Reviewers for their suggestions and

timely advice.

We are grateful to Director General, ICMR for envisioning the Task Force Study in the

Indian context and encouraging us to take the initiative. We would like to thank Scientist – ‘F’ from National Informatics Centre for her valuable efforts. We also thank the Head, Division of

Non-Communicable Diseases (NCD) for being a constant guide and support.

Our special thanks to the administrative staff of Division of Non-Communicable Diseases and the financial staff of ICMR headquarters for smooth implementation and timely

release of grants to make the project a success.

Oct., 2015 Dr. Ashoo Grover

Dr. R. S. Dhaliwal

Abbreviations followed uniformly in text

UCLP : Unilateral Cleft Lip and Palate

BCLP : Bilateral Cleft lip and Palate

CP : Cleft Palate

CL : Cleft Lip

CLA : Cleft Lip and Alveolus

List of tables Table 1-Distribution of sample according to age

Table 2- Distribution of sample according to the type of cleft & sex

Table 3-Classification of cleft (Nagpur classification)

Table 4- Distribution of sample according to Nagpur classification

Table 5- Patients with positive familial history of Cleft

Table 6-History of medical problems in mother during 1st trimester of affected pregnancy

Table 7- History of Drug usage in mother during affected pregnancy

Table 8- History of radiation exposure to mother during 1st trimester of a affected

pregnancy

Table 9- Use of intoxicants by mother during 1st trimester of a affected pregnancy

Table 10- History of exposure to smoke during 1st trimester

Table 11- Effect of cleft deformity on the social acceptability of the patient

Table 12- Post natal counseling of parents with regards to feeding of child with cleft and

his treatment possibilities related to cleft

Table 13- Distribution of the patients who received correct advice for at least one of the

evaluated variables

Table 14- Age wise distribution of lip repair

Table 15- Age at palatal repair excluding alveolus

Table 16- Previous history of pre-surgical orthopaedic treatment

Table 17- Previous history of dental treatment

Table 18- Positive history of post surgical orthodontic treatment

Table 19- Supernumerary teeth

Table 20- Presence of anterior crossbite

Table 21- Presence of posterior crossbite in the sample of 55 cases

Table 22 - Overjet in the sample

Table 23- Overbite in the sample

Table 24- Goslon Yardstick scores and their interpretation

Table 25- Distribution of subjects according to the Goslon Yardstick

Table 26- Widest gap in the cleft, palate + alveolus

Table 27- Length of the palate

Table 28- Length of scar in unilateral clefts

Table 29- Angulation of scar in unilateral cleft

Table 30- Length of scar in bilateral cleft

Table 31- Angulation of scar in bilateral cleft

Table 32- Assessment of lip seal

Table 33- Evaluation of lip symmetry

Table 34- Overall appearance of lip

Table 35- Overall appearance of nose

Table 36- Evaluation of nasal septum in unilateral clefts

Table 37- Evaluation of nasal septum - Bilateral clefts

Table 38- Evaluation of nostril floor width

Table 39- Evaluation of the length of the palate in the sample

Table 40- Evaluation of post surgical scarring of the palate in the sample

Table 41- Mobility of the palate in the sample

Table 42- Status of uvula in operated cases of cleft palate

Table 43- Presence of fistula in the sample

Table 44- Assessment whether the fistula is symptomatic or not

Table 45- Evaluation of the size of the oronasal fistula in the sample

Table 46- Speech abnormality due to presence of the fistula

Table 47- Evaluation whether the fistula has been operated previously or not in the

sample

Table 48- Status of Tonsils in the sample according to type of cleft

Table 49- Incidence of Ear Discharge

Table 50- Status of tympanic membrane in the sample in unilateral cleft

Table 51- Status of tympanic membrane-Bilateral cleft

Table 52- Prevalence of hearing abnormalities in the sample as a function of type of cleft

Table 53- Degree of hearing loss in the sample

Table 54- Relation of hearing loss with different types of cleft – Unilateral Cleft

Table 55- Relation of hearing loss with different types of cleft – Bilateral Cleft

Table 56- Impedance Audiometry and their inference

Table 57- Status of middle ear based on Impedance

Table 58- Status of middle ear based on Impedance- Bilateral cleft

Table 59- Distribution of the sample according to nasality of speech

Table 60- Speech Articulation in the sample

Table 61- Status of Affected articulation in the sample

Table 62- Description of speech sample

Table 63- Overall speech intelligibility in various cleft types

List of figures

Figure 1- Cleft patient assessment tool

Figure 2- Intraoral photographs

Figure 3- Supplemental intraoral photographs

Figure 4- Dental study model

Figure 5- Complete organizational setup of the project

Figure 6- “The Indicleft Team”

Figure 7- Schedule and timing protocol of cleft care

Figure 8 (1-12)- Pedigree Charts

Figure 9- Distribution of proposed centres in multicentre study

List of graphs

Graph 1- Distribution of sample according to age

Graph 2- Distribution of sample according to the type of cleft & sex

Graph 3- Distribution of sample according to Nagpur classification

Graph 4- Patients with positive familial history of Cleft

Graph 5- History of medical problems in mother during 1st trimester of affected pregnancy

Graph 6- History of Drug usage in mother during affected pregnancy

Graph 7- History of radiation exposure to mother during 1st trimester of a affected pregnancy

Graph 8- History of exposure to smoke during first trimester

Graph 9- Distribution of the patients who received correct advice for at least one of the evaluated variables

Graph 10- Age wise distribution of lip repair

Graph 11- Age at palatal repair excluding alveolus

Graph 12- Presence of anterior crossbite

Graph 13- Goslon score

Graph 14- Evaluation of lip symmetry

Graph 15- Overall appearance of lip

Graph 16- Overall appearance of nose

Graph 17- Evaluation of nasal septum in unilateral clefts

Graph 18- Evaluation of nasal septum - Bilateral clefts

Graph 19- Evaluation of nostril floor width

Graph 20- Evaluation of the length of the palate in the sample

Graph 21- Evaluation of post surgical scarring of the palate in the sample

Graph 22- Presence of fistula in the sample

Graph 23- Evaluation of the size of the oronasal fistula in the sample

Graph 24- Status of Tonsils in the sample according to type of cleft

Graph 25- Prevalence of hearing abnormalities in the sample as a function of type of cleft

Graph 26- Degree of hearing loss in the sample

Graph 27- Distribution of the sample according to nasality of speech

Graph 28- Speech Articulation in the sample

Graph 29- Overall speech intelligibility in various cleft types

Contents

1 Introduction …………………………………………………………………………... 1

2. Aims and objectives ………………………………………………………………… 2

3. Subjects and methods

➢ Pre Pilot Phase 2012-1012 …………………………………………….

➢ Pilot Phase 2012 -2014

• Sample and methodology ……………………………….………

• Structuring of an expert team: “The Indicleft Team” ...

• Patient evaluation ………………………….……………………….

• Assessment of etiology of cleft …….………………………….

• Dental history and examination ……………………………….

• Orthodontic Treatment History ………………………………..

• Evaluation of primary cleft deformity …………..…………..

• Evaluation of secondary cleft deformity …………………..

• Evaluation of lip ……………………………………………………..

• Evaluation of nose ………………………………….………………

• Evaluation of secondary palate ………………………………..

• Assessment of post-surgical palatal fistula ………………..

• ENT evaluation ………………………………………………………

• Speech assessment ………………………………………………..

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4. Analysis and results

• Distribution of sample according to age and sex ……………..

• Distribution and types of cleft ……………………………..………..

• Classification of cleft …………………………….………………………

• Etiology of cleft: genetic and environmental risk factors ….

• Effect of cleft deformity on the social acceptability of the patient ………………………………………………………………………..

• Post natal counseling of parents with regards to feeding and treatment possibilities related to cleft ………………………

• Age wise distribution of primary lip and palate repair ………

• Previous history of dental and orthodontic treatment ………

• Dental examination ………………………………………………………

• GOSLON Yardstick ……………………………………………………….

• Examination of primary cleft ………………………………………….

• Examination of Secondary Cleft Deformity ………………………

• Examination of the Nose ……………………………………………….

• ENT Examination ………………………………………………………….

• Hearing evaluation ……………………………………………………….

• Speech assessment ………………………………………………………

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5. Conclusions ……….…………………………………………………………………… 70

6. Future directions …………………………………………………………………….. 71

7. S & T benefits occurred

• Lists of research publications with complete details ……..…. 73

8. Procurement and Usage of equipments …….………………………………. 74

1 Indian Council of Medical Research Task Force Project

1 Introduction

Cleft of the lip and /or the palate (CL±P) is a

congenital birth defect which is characterized

by complete or partial cleft of the lip and/ or

the palate. The severity of the cleft may vary

from the trace of notching of the upper lip to a

complete non- fusion of the lip, the primary

palate and the secondary palate. Cleft lip and

palate anomaly constitutes nearly one-third of

all congenital malformations of the

craniofacial region with an average worldwide

incidence of 1 in 700. Its incidence in Asian

population is reported to be around 2.0 per

1000 live births or higher. In India, even

though a national epidemiological data is not

available, many studies from different parts of

the country have reported a variation in the

incidence of cleft anomaly. Sidhu and

Deshmukh reported the incidence of Cleft Lip

and Palate (CL+P) at AIIMS, New Delhi to be

1.4 per 1000 live births. Mossey and Little

estimated from various multicentric studies

across India that the incidence of CLP in India

ranges from around 0.93-1.3 for cleft lip and

palate.

Based on rough estimates, it is

suggested that approximately 35,000 newborn

cleft patients are added every year to the

Indian population. With many patients having

less than optimum care in a not so organized

setup, the cumulative burden of persons

affected with this birth defect is huge.

Although India has a large and extended

network of medical facilities, interdisciplinary

cleft care is provided in only a few hospitals.

Day-to-day interactions with these patients

exhibit significant variations in treatment

provided and the quality of outcome with

some having excellent outcomes while many

patients received sub-optimum, limited or no

treatment. The reasons are many and varied.

The awareness in the society and amongst the

health professionals on critical aspects of

interdisciplinary care of this anomaly may be

lacking. Affordability and availability of

experts may also contribute to the quality of

treatment. Gopalakrishna and Agrawal (2010),

following a national survey on trends in the

management of patients with CLP in India,

concluded in their study, “Management of

Cleft Lip and Palate (CLP) differs in India.

Primary surgical practices are almost similar

to other studies. There is a lack of

interdisciplinary approach in majority of the

centres and hence, there is a need for better

interaction amongst the specialists.” This lack

of interdisciplinary approach and the need for

it in the Indian setup has been stressed

previously also. This taskforce project was

initiated by the Indian Council of Medical

Research, to evaluate the current status of

treatment vis-à-vis CLP and the medical needs

of cleft patients. The aim is to work out a

national registry and guidelines for cleft care

in India.


2 Aims and Objectives

To assess the multidisciplinary, multicentre

project titled Cleft Lip and Palate anomaly in

India: Clinical profile Risk factors and current

status of treatment: A hospital based study was

initiated under the aegis of Indian Council of

Medical Research (ICMR), New Delhi in 2010

as a pre-pilot study aimed to assess the

feasibility of a larger pilot study. The pre-pilot

phase was successfully completed in the

Department of Orthodontics & Dentofacial

Deformities and ENT, AIIMS, New Delhi

(2010-12).

The pilot phase of the study (2012-14) was

commissioned by the ICMR encompassing

three cleft-care centres with high case load

across Delhi and National Capital Region

(NCR). The study is essentially aimed to

evaluate the socio-economic, demographic

details of patients suffering from cleft lip and

palate (visiting the 3 enrolled hospitals), their

treatment profile and the residual treatment

needs of the same subjects. The objectives of

the pilot phase were:

i. To utilize the study tools developed in the

pre-pilot phase for the evaluation of the

clinical profile of CLP anomaly. The

experience gained would result in its

suitable modifications for its further use in

a nationwide multicentre study.

ii. To establish a methodology for a

nationwide collection of data for the

clinical profile for Cleft lip and palate

anomalies and its major treatment needs

including the logistics, feasibility and

difficulties expected in the execution of the

multicentre project.

The report of the Pre-pilot and Pilot

phase would be used to formulate a

multicentre study which will be aimed to:

1. Identify patterns of the congenital birth

defects of the face, cleft lip and palate in

India.

2. Establish the baseline data of a spectrum of

problems of cleft patients, discuss the

protocols of treatment given to these

children and their actual treatment needs.

3. Ascertain the risk factors associated with

congenital defects of the face: nutritional,

environmental and genetic.

The long term objectives are to initiate a

‘National Registry’ for the patients with

congenital birth defects of the face and jaws

and also to establish strategies that will

address the multitude of challenges associated

with the prevention and treatment of this

deformity. These will include:

a. Identify risk factors associated with the

development of cleft lip and palate

b. Develop strategies to minimize risk factors,

thereby reducing the incidence of Cleft Lip

and Palate (CLP) anomaly

c. Develop protocols for interdisciplinary care

which is feasible and affordable in India

with minimum burden on patients, parents

and service providers

d. Improve treatment outcomes

The ultimate aim is to improve the quality of

life (QOL) of children suffering from Cleft lip

palate and such deformities so that these

children can have a better living.


3 Subjects and Methods

A. Pre pilot phase 2010-2012

This phase of study was conducted at the

Department of Orthodontics and

Dentofacial Deformities, Centre for Dental

Education and Research (CDER) and ENT,

at the All India Institute of Medical

Sciences (AIIMS), New Delhi, from April

2010 to March 2012. The key highlights

were:

1. On 2nd June 2007, Prof. O.P. Kharbanda

wrote to Dr Jagdish Kaur (of DGHS,

MOHFW) to request for the inclusion of

cleft lip and palate, along with speech

and hearing defects, in the national

health surveys.

2. Core group experts of oral health met at

the ICMR headquarters and

recommended to Prof. Kharbanda to

initiate a pre-pilot study for 1 year to

focus on the spectrum of cleft patients to

develop a pilot proposal for 3 to 4

centres.

3. On 23rd July 2009, an application was

sent to ICMR for the initiation of the

task force project.

4. On 28th January 2010, an ethical

clearance was received.

5. On 23rd March 2010, the project was

initiated.

6. On 31st March 2012, the project was

completed.

7. An exhaustive study tool was developed

which had seven sections (Figure-1).

The multidisciplinary clinical

examination was to be performed by the

following specialists. Surgeon/Plastic

Surgeon, Orthodontist, Dental Surgeon,

ENT specialist and Speech therapist.

8. Standard operating procedure (SOP) for

recording the extraoral and intraoral

clinical photographs (Figure- 2 and 3),

dental study models (Figure- 4) and

investigations on hearing defect and

evaluation of speech were developed.

9. Planning was carried out to extend the

project onto the other hospitals across

Delhi.

Figure 1- Cleft patient assessment tool

Cleft Lip and Palate Anomaly in India: Clinical Profile, Risk Factors and Current Status of Treatment: A Hospital Based Study


Figure 2- Intraoral photographs

Figure 3- Supplemental intraoral photographs

Subjects and Methods


Figure 4- Dental study model

Figure 5-Complete organizational setup of the project



B. Pilot phase 2012 -2014

1. Sample and methodology

This study was conducted with the active

collaboration of All India Institute of

Medical Sciences, New Delhi, Safdarjung

Hospital, New Delhi, Medanta- The

MEDICITY Hospital Gurgaon, Haryana,

INDIA. A total number of 164 cases with cleft

lip and palate anomaly were recorded from

these three hospitals involved in the project

(55 from AIIMS, 54 from Safdarjung, 55 from

Medanta- The MEDICITY). The sample

consisted of 99 males and 65 females with a

wide age distribution (18 months to 516

months; a mean of 147.4 months) (Table 2).

The majority of the subjects belonged to the

age group of 5 (>6yrs -<=12yrs) (53 subjects)

(Table 1).

Data was recorded from three high

volume cleft care centres in Delhi and

National Capital Region (NCR) which

involved two public funded and one private

hospital.

The main highlights of the centres were:

• AIIMS: located in South Delhi; is an

autonomous institute which is a tertiary

care centre too. The combined cleft clinic

was established in the orthodontic unit in

the 1970’s.

• Safdarjung hospital; also located in South

Delhi; is a high volume centre supported by

the central government. It has a renowned

plastic and burns unit where cleft patients

are treated for primary and secondary

surgeries.

• Medanta- The MEDICITY; is also a

renowned hospital with an advanced plastic

surgery unit.

At each of the centres, the Departments of

Plastic surgery, Orthodontics and ENT were

the major input holders for support and

coordination of the study. The collection of the

data was carried out by the specifically

designated “Indicleft Team”. The complete

organizational setup of the project is indicated

in Figure 5.

2. Structuring of an expert team: “The Indicleft Team”

The “Indicleft team” included experts from

various medical specialties (Figure-6). It was

divided into a supervisory team (called

investigators) and a mobile team of research

staff. The members of the supervisory team

were based in three locations: AIIMS,

Safdarjung and Medanta- The MEDICITY

hospitals. The mobile team included a doctor,

an audiologist & a speech therapist and a

dental lab technician. Apart from the mobile

team, research staff was also deputed at the

three centres to work under the guidance of

supervisory staff. The research staff at AIIMS,

Safdarjung and Medanta- The MEDICITY

hospital performed the local data collection in

the form of taking down the medical history,

making impressions and other project-related

work at the respective centres. The research

staff at the centres also was responsible to

coordinate with the technical unit of CDER

and for the follow-up of the cleft patients of

their own centres. The mobile research team

would go to the various centres for

coordinating with the staff at the centres.

As previously mentioned, the team

comprised experts from various fields. This

was very important because cleft patients need

diverse types of treatment which can only be

provided by experts of the fields (Figure-7).

The individual role of each expert in the team

can be summarized below:

• The role of plastic surgeon: The plastic

surgeon is one of the most important

members of the team. He is responsible

for the primary and the secondary

surgeries of the defect. The surgeries are

particularly important because a good

primary surgery has a major effect on the

final outcome of the treatment and for

post-surgical maxillary growth. Poor

repair of the soft palate leads to

velopharyngeal incompetence and nasal

intonation along with nasal regurgitation.

The fistula many a times results from

poor surgical technique which can be best

understood by a plastic surgeon. The

fistula may also lead to nasal

regurgitation and nasal intonation. To

evaluate all these defects, the services of a

surgeon, preferably a plastic surgeon

would be required for proper assessment

of the gravity of the situation. Thus the

role of the plastic surgeon cannot be

under estimated in the project.



• The role of orthodontist: The role of an

orthodontist cannot be overemphasized

when dental management of the cleft

child is concerned. Since nearly all of the

cleft children have dental deformities

secondary to cleft lip and palate (barring

perhaps just a few cases with isolated CP

and submucous clefts), all cleft children

need extensive orthodontic support for

management of their dentition right from

the start of the eruption of the primary

teeth to the time of growth completion at

18 years or till the final surgical

correction of the cleft defect. Thereafter,

the retentive aspects of orthodontic

correction take over and the patient is

followed in an adult life for over viewing

the alignment of teeth achieved earlier. In

many centres, the orthodontist has been

chosen as a team leader in the

multidisciplinary cleft team.

• The role of ENT specialist: Since many

of the patients with cleft have a defective

Eustachian tube function secondary to the

altered muscle attachments on the tube

and due to persistent nasal regurgitation,

many suffer from hearing defects

including CSOM (Chronic Suppurative

Otitis Media), tympanic membrane

defects and sometimes advanced hearing

loss resulting from adhesions of the ear

bones. It is of utmost importance to have

an ENT expert to examine the cases for

necessary interventions and referrals.

• The role of speech pathologist: As

mentioned earlier, due to velopharyngeal

incompetence, palatal fistulas as well as

poor repair of the lip, many patients have

distorted speech sounds, misarticulation

and most even have hypernasality. It is

indeed a necessity to evaluate the defects

in speech by an expert who can diagnose

the speech alteration, counsel the patient

and provide necessary speech therapy.

• The role of medical/dental graduate:

Overall assessment of the medical history

condition and /or dental condition

requires dedicated manpower support so

that the experts can focus on their specific

fields of interest; this leads to increased

efficiency of the experts and better quality

of recording of the data. Hence, support

by a medical/dental graduate to the

constituted team of experts cannot be

discounted.

Figure 6- The ‘Indicleft’ team



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3. Patient evaluation

The record keeping for the cases involved

prior consent and approval. Each patient

inducted in the study was subjected to the

following investigations, all of which were

non-invasive type.

1. Evaluation of the patient using the

specially-designed performa developed

during the pre-pilot phase of the project.

The said performa evaluated the

following aspects of cleft care (Figure-4):

a. General details: the personal details

like the patient’s name and address,

contact details

b. Socio-demographic profile

c. Evaluation of the risk factors

associated with etiology of cleft.

d. Evaluation of dental status of cleft

patients and their orthodontic

treatment profile

e. Evaluation of primary and secondary

cleft deformity

f. Assessment of hearing and speech

evaluation

2. The patients’ extraoral and intraoral

standard clinical photographs were

recorded.

3. Study models of each patient above 5

years of age were prepared for the project

using alginate impression material and

poured in orthodontic grade white dental

stone.

4. Assessment of etiology of cleft

The assessment was done by direct interview

of the parents/guardians of the subject. The

interview consisted of various parameters

known to be associated with genesis of the

cleft. Broadly, they could be divided as genetic

or environmental.

a. Genetic: Evaluation of genetic factors

consisted of pedigree analysis. The

pedigree was formed as per the interview

with the subjects’ parents and it was

evaluated whether the subject had any

predisposition to familial occurrence of

the cleft.

b. Environmental factors: The

environmental factors were also evaluated

by interview with the subjects’ parents.

The time of the 1st trimester of pregnancy

was particularly evaluated for the

presence of any risk factors associated

with the genesis of cleft. The interview

consisted of the following evaluations:

I. Exposure to radiation

II. Exposure to medication

III. History of sickness in the 1st trimester

IV. Use of intoxicants/ smoking

V. Exposure to smoke by either use of

chulha or passive smoking.

VI. History of recurrent abortions

5. Dental history and examination

The dental history and examination consisted

of verbal evaluation as well as clinical

examination. The subjects were evaluated for

any history of previous dental treatment by

way of direct interview as well as clinical

examination of dentition for presence of any

fillings or root canal treatment. Further, the

maxillary arch was evaluated for the presence

of supernumerary teeth and their position was

noted in relation to cleft.

6. Orthodontic treatment history

This aspect of taking history involved

evaluation of whether the subject had any

previous orthodontic treatment in his lifetime.

This included evaluation for pre-surgical

orthopedics, orthodontic treatment with

removable appliances or fixed appliances.

a. History of pre-surgical orthopedic

treatment: This was evaluated by

interview with subject’s parents. Parents

were asked whether any orthopedic

procedures like nasoalveolar moulding

were carried out on the child before the

primary surgery was done. The response

was noted as yes or no.

b. Previous history of orthodontic

treatment: This was evaluated by an

interview with the subject’s parents.

Parents were asked whether any

orthodontic procedures like arch

expansion, treatment with removable

plates, fixed orthodontic treatment were

carried out at any time during the

subject’s lifetime. The response was

noted as yes or no.

c. Examination of Overjet and Overbite

along with Goslon Index: Overjet in

the dentition of the subjects was



evaluated on the dental cast articulated

in the centric relation position. The

overjet was measured in mm between

the most proclined tooth in the upper

arch and the most retroclined tooth in

the lower arch. The overjet could be

positive or negative depending upon the

relation of the dentitions. Similarly, the

overbite was measured in millimeters

(mm) as the vertical distance of overlap

between the upper and the lower

anterior teeth.

The orthodontic treatment

need was assessed using GOSLON

Yardstick according to the criteria

defined by Mars et al and grouped into

categories 1-5. The inclusion criteria for

GOSLON assessment included:

i. All cases with UCLP anomaly

ii. Age more than 6 years

iii. All cases operated for primary clefts

iv. No history of previous orthodontic

treatment

v. Non syndromic

d. Presence of cross bites: Cross bites can

be of two types: anterior and posterior.

The anterior dental segment comprises

from canine to canine tooth and

comprises 6 teeth. The posterior

segment consists of teeth posterior to

the canine tooth i.e. the premolars and

the molars. In our analysis, we studied

the presence of cross bite as single

tooth, 1-3 teeth, or more than 3 teeth.

However, since single tooth cross bites

are usually not indicative of underlying

growth disturbance, these types of cross

bites were grouped with the category of

“cross bite absent” when assessing the

posterior segment relations. Cross bites

of more than 1 tooth were taken to be

indicators of growth restriction/collapse

of segment and were grouped together

for ease of analysis and data

presentation.

7. Evaluation of the primary cleft deformity

The Cleft deformity was evaluated by a Plastic

surgeon. Cases with primary and secondary

cleft deformities were evaluated on separate

parameters. Cases with primary cleft were

evaluated for the following parameters:

a. Maximum Width of the cleft in the

palatal region (including alveolus): It was

evaluated objectively by measuring the

maximum width of cleft in the palate. The

width was noted in mm and classified into

three categories: <2mm, ≥2- <5mm and

≥5mm.

b. Length of the palate: The length of the

soft palate was judged subjectively by

asking the patient to open the mouth wide

and say ‘aaaahhhh’ repetitively while

noting the elevation of the soft palate

during this maneuver. The soft palates were

classified as short or inadequate in length.

8. Evaluation of secondary cleft deformity

This was done for all cases which were

operated for cleft lip and/or palate. As

previously, the lip and palate were evaluated

separately.

9. Evaluation of the lip

a. Evaluation of lip scar: It was done for

both unilateral and bilateral cases of cleft

which involved the lip. It consisted of

evaluation of width of scar and was

measured in mm directly during clinical

examination. For unilateral cases, only the

affected side was evaluated while for

bilateral cleft both the sides were

evaluated separately. It was classified as

below:

<=0.5mm >0.5-<=1mm >1mm

b. Angulation of the scar: It was done by

direct clinical examination for both

unilateral and bilateral cases of cleft

which involved the lip. The evaluation

was done by direct clinical examination.

The cases were classified as vertical and

oblique cleft of lip.

c. Quality of lip scar repair: This was also

done by direct visual examination for all

cases involving the lip. The rating was

subjective and based on the width of the

scar, the quality of scar tissue, presence of

cross hatches across the scar line,

amongst other criteria. For the bilateral

cases, however, the right and the left sides

were not evaluated separately. Instead the

upper lip was examined in entirety and



the lips were classified as: poor, fair,

good, very good and excellent.

d. Evaluation of lip seal: Lip seal was

evaluated by asking the patient to relax

his lips in normal posture and observing

whether the lips meet at rest or not

completely. Further, the patient was asked

to blow his cheeks with the lips sealed

with each other and it was observed

whether the patient could maintain

effective lip seal or not during the task.

The lip seal was classified as present or

absent.

e. Evaluation of lip symmetry: Lip

symmetry was subjectively evaluated

clinically by comparing the left and the

right side of the upper lip and noting

whether they are symmetrical or not.

f. Overall appearance of lip: It was done

for both unilateral and bilateral cases of

cleft which involved the lip. Overall lip

appearance was judged subjectively on

the basis of the lip symmetry, thickness of

the vermilion border, presence of

crosshatches across the scar line and the

width (length of the scar). The lips were

classified as poor repair, fair, good, very

good and excellent.

10. Evaluation of nose

a. Overall appearance of nose: The

appearance of the nose depended upon the

nasal symmetry, deviation of the tip of

nose and nasal septum, length of the

columella, width and symmetry of the alar

bases, amongst other factors. The nose

repair was classified as poor, fair, good,

very good and excellent.

b. Evaluation of nasal septum: The nasal

septum was evaluated clinically for any

deviation to either side of the midline. It

was classified as deviated or not deviated.

c. Evaluation of nostril floor width: This

evaluation was carried out for all cases of

cleft involving the lip and palate. The

nostril floor width was evaluated

subjectively by comparing the affected

side with the normal side. The nostril

floor width was classified as equal or

unequal.

11. Evaluation of the secondary palate

a. Length of the palate: The length of the

soft palate was also judged subjectively

by asking the patient to open the mouth

wide and say ‘aaaahhhh’ repetitively a

few times and noting the elevation of the

soft palate during this procedure. The soft

palates were classified as short or

adequate in length.

b. Post surgical scarring of the palate:

This evaluation was subjective and was

carried out during clinical examination by

observing the palatal contour, amount of

scarring and fibrosis. It was classified as

little, acceptable or too much.

c. Mobility: Criteria of evaluation were

same as in point 3a. The mobility was

classified as satisfactory and

unsatisfactory.

d. Status of uvula: Uvula was examined by

direct intraoral examination. The shape

and size of uvula was noted subjectively

and classified as well formed, not well

formed and bifid.

e. 12. Assessment of post surgical palatal fistula

Fistula assessment was done by direct clinical

examination and the presence or absence of

fistula was noted. In cases with palatal fistula,

the following additional parameters were also

evaluated:

a. Size of fistula: It was noted by measuring

the longest diameter of the fistula using

blunt-ended calipers. The assessment was

routinely done during the clinical intraoral

examination. However, in cases where the

fistula was located in an inaccessible area

or where caliper cannot reach safely like

the soft palate, the size of fistula was

noted on the dental cast using a suitable

caliper. The size was noted in mm.

b. Whether the fistula is symptomatic or

not: This assessment was subjective and

based on patient interview. The patient

was asked whether there is a nasal

regurgitation of fluids during daily

activity. In the cases with positive history

of regurgitation the fistula was regarded

as symptomatic. In cases with non-

specific response water holding test was



performed where by the patient was asked

to hold water in mouth for around 10-15

seconds. During this period the nasal

regurgitation of fluid was noted and the

fistula was categorized accordingly.

c. Speech abnormality due to fistula: The

examination consisted of clinical

evaluation wherein the speech of the

subject was evaluated without obliterating

the fistula. In the 2nd phase the fistula was

obliterated with moist gauze or an

orthodontic relief wax and the speech was

again evaluated. Both the speech samples

were noted for any change in nasalance or

articulation. Consequently, the speech

abnormality due to fistula was categorized

as yes or no.

13. ENT evaluation

ENT evaluation was done by an

otolaryngologist. The evaluation is consisted

of:

a. Assessment of tonsils: The tonsils were

evaluated clinically and were classified

into 4 grades, based on Neiminen study,

2002 titled ‘Snoring and Obstructive

Sleep Apnea in young children’ Grade I

tonsils within tonsillar fossa, Grade II

tonsils not reaching the mid line between

anterior faucial pillar and uvula, Grade III

tonsils medially from the midline and

Grade IV tonsils with in maximally 4

millimeters in between.

b. Status of tympanic membrane: Its

evaluation was done by an ENT surgeon

using an Otoscope. In each patient, both

ears were examined irrespective of the

side or type of cleft. The tympanic

membranes were classified as normal,

retracted or perforated.

c. Assessment of hearing ability: This

evaluation was only done in ears with an

intact tympanic membrane. The

assessment was done in both ears using a

combined pure tone and impedance

audiometry unit (Interaccoustics, USA).

The cases were classified according to the

presence or absence of hearing loss, and

the type of hearing loss i.e. conductive,

sensorineural and mixed type.

14. Speech assessment

Speech assessment was done by a speech

therapist and each subject was assessed for

hypernasality, articulation defects and overall

speech intelligibility.

a. Assessment of hypernasality: This was

subjective and hypernasality was

classified as present or absent.

b. Articulation defects: The speech was

assessed for presence of articulation

defects using a predefined articulation test

which is standardized on the Hindi

language. The type of misarticulation was

judged using the following categories:

substitution, omission, deletion and

addition.

c. Speech intelligibility: Overall speech

intelligibility was judged on the basis of a

predefined criterion which is standardized

on Hindi language.


4 Analysis and Results

This three centre pilot phase of ICMR-funded

Task Force project was undertaken to assess

the feasibility and difficulties encountered in

undertaking such a study across India and to

establish a protocol for the same. This report is

focused to highlight the current treatment

profile and the residual treatment needs of the

patients with cleft anomaly visiting the three

prominent cleft care hospitals across Delhi and

NCR. The data analysis of cases pooled from

the three centres exhibited significant variation

in the timings and outcome of surgery,

complexity of orthodontic treatment and

speech and hearing defects. It is pertinent to

mention that the cases recorded at each of the

centres were a mix of those who had their

treatment at their respective centre and those

cases which were treated elsewhere but were

referred/sought further treatment. Hence, the

results of this pooled data do not necessarily

reflect the treatment outcome of the three

centres alone. They only reflect the quality of

care which many of the cleft patients in our

society end up receiving.

A. Distribution of sample according to age and sex

In our study, out of the 164 cases, the 42 cases

(25.6%) belonged to age group less than 6

years, 51 cases (31.1%) were between 6-12

years of age, 32 cases (19.5%) were between

12-18 years of age and 39 cases (23.8%) were

more than 18 years of age (Table 1, Graph 1).

Among the 164 cases, 99 cases (60.4%) were

male and 65 (39.6%) were female.

B. Distribution and type of cleft

When the sample was analyzed according to

the type of cleft, UCLP was found to be the

biggest category with 78 cases followed by

BCLP (38 cases) and CP (26 cases). When the

type of cleft was analyzed as a function of

cleft; amongst the males, the majority of the

cases belonged to the UCLP (52 cases)

category followed by BCLP (26 cases) and CP

(13 cases). Amongst the females, 26 cases had

UCLP, 12 had BCLP while 13 cases had cleft

palate (CP) (Table 2, Graph 2)

C. Classification of cleft

In our study, the Nagpur classification system

was used to classify the types of clefts (Table

3), both operated and unoperated. It was found

that majority of the cases (116 cases) were

found to be belonging to Group-III of Nagpur

Classification (unilateral and bilateral cleft lip

and palate) (Table 4, Graph 3).

Table 1-Distribution of sample according to age

Centre

>18mo -<=6yrs

(4)

>6yrs -<=12yrs

(5)

>12yrs - <=18yrs

(6)

>18 yrs

(7) Total

AIIMS 9 24 12 10 55

Safdarjung 14 12 10 18 54

Medanta-

The MEDICITY 19 15 10 11 55

Total 42

(25.6%)

51

(31.1%)

32

(19.5%)

39

(23.8%)

164

(100%)

n=164; Data represents no. of patients in each category



Table 2- Distribution of sample according to the type of cleft & sex

CL CL B/L CLA CLA B/L UCLP BCLP CP Total

Male 3 1 2 2 52 26 13 99

(60.4%)

Female 9 0 5 0 26 12 13 65

(39.6%)

Total 12

(7.3%)

1

(0.6%)

7

(4.3%)

2

(1.2%)

78

(47.6%)

38

(23.1%)

26

(15.9%)

164

(100%)


Analysis and Results


Table 3-Classification of cleft (Nagpur classification)

Group I Cleft of lip

Group I (A) Cleft of lip with cleft of alveolus

Group II Cleft of palate alone

Group II(S) Submucous cleft of palate

Group III Cleft of lip and palate

Table 4- Distribution of sample according to Nagpur classification

No. of cases Group I Group I

(A) Group II

Group II

(S) Group III Total

AIIMS 1 2 3 1 48 55

Safdarjung 5 3 9 1 36 54

Medanta- The MEDICITY

7 4 11 1 32 55

Total 13

(7.9%)

9

(5.5%)

23

(14.1%)

3

(1.8%)

116

(70.7%)

164

(100%)




D. Etiology of cleft: genetic and environmental risk factors

D1. Genetic influence: Cleft lip and

palate is known to have a multifactorial

inheritance and the role of genetic influences

is well documented although the

environmental influences may have a bigger

role in the etio-pathogenesis. In our data, out

of the sample of 164 cases, 24 cases had

positive familial history with regards to cleft

lip and palate while the remaining 140 cases

did not reveal any positive familial history.

This implies that 14.6% cases had a familial

association of cleft [Table 5, Graph 4, Figures

8.1-8.12].

D2. Environmental influences: The

environment has been ascribed a dominant role

in the etiology of cleft, especially in the first

trimester of pregnancy when the cleft develops

or rather, the components of the maxillary and

the palatal processes fail to fuse together in the

developing fetus thus leading to cleft. The

environmental influences can be many,

ranging from known risk factors like maternal



smoking and alcohol consumption, exposure to

smoke leading to foetal hypoxia, nutritional

deficiencies, medical illnesses, use of oral

contraceptives, certain medications, amongst

other risk factors.

In our study, we evaluated a few of the

known risk factors including maternal

smoking and alcohol consumption, intake of

drugs during the first trimester of pregnancy

and exposure to smoke during the same time

by the use of chulha at home or due to passive

smoking. The results of our study are

mentioned below.

D2.1. History of medical problems in mother during 1st trimester of affected pregnancy

In the sample of 164 subjects, 64 cases (39%)

were found where the mother had a positive

history of illness during 1st trimester of the

affected pregnancy. When the type of disease

was enquired, a variable pattern was found

with responses ranging from non-specific

fever to viral fever, tuberculosis, thyroid,

vomiting, etc. (Table 6, Graph 5).

D2.2. History of drug intake during the 1st trimester of affected pregnancy

Out of the 164 cases, 44 (26.8%) had a

positive history of maternal use of drugs

during the first trimester of affected

pregnancy, but were unaware about its dosage

and duration of use. (Table 7, Graph 6)

D2.3. History of maternal radiation exposure and use of intoxicants during the 1st trimester of affected pregnancy

Out of 164 subjects, only 4 cases gave a

positive but incomplete history of radiation

exposure of mother during 1st trimester of

affected pregnancy (Table 8, Graph 7) while 1

case revealed use of some intoxicants during

1st trimester of affected pregnancy. (Table 9)

D2.4. History of exposure to smoke and mode of cooking during 1st trimester

In the recent past, the role of maternal

exposure to smoke in the first trimester of

pregnancy has been implicated in the etiology

of cleft palate possibly due to fetal hypoxia

leading to interference in palatal shelf fusion.

In the Indian scenario, this is especially valid

for the rural setups where it has been

postulated that exposure to smoke emanating

from the use of chulha for cooking might be

related to increased incidence of cleft in the

progeny. In our sample, out of 164 cases, 89

cases (54.26%) gave positive history of

exposure to smoke during 1st trimester of

pregnancy (Table 10, Graph 8). This sample

included mothers exposed to smoke from

cigarette smoking, passive smoking and/or use

of chulha during the 1st trimester of the

affected pregnancy.



Figure 8.1 Pedigree Chart




Table 5- Patients with positive familial history of Cleft

Familial history positive

(1)

Familial history negative

(2)

Total

24

(14.6%)

140

(85.4%)

164

(100%)


Table 7- History of Drug usage in mother during 1st trimester of

affected pregnancy

Centre Yes No Total

AIIMS 11 44 55

Safdarjung 21 33 54

Medanta- The MEDICITY 12 43 55

Total 44

(26.8%)

120

(73.2%)

164

(100%)


Table 6-History of medical problems in mother during 1st trimester of affected pregnancy

Centre Yes No Total

AIIMS 12 43 55

Safdarjung 13 41 54

Medanta- The MEDICITY 39 16 55

Total 64

(39%)

100

(61%)

164

(100%)




Table 8- History of radiation exposure to mother during 1st trimester of affected pregnancy

Centre Yes No

AIIMS 1 54

Safdarjung 3 51

Medanta- The MEDICITY 0 55

Total 4

(2.5%)

160

(97.5%)


Table 9- Use of intoxicants by mother during 1st trimester of affected pregnancy

Centre Yes (Code 1) No (Code 2)

AIIMS 0 55

Safdarjung 1 53


Total 1

(0.6%)

163

(99.4%)


Table 10- History of exposure to smoke during 1st trimester

Centre Yes (Code 1) No (Code 2)

AIIMS 27 28

Safdarjung 40 14


Total 89

(54.3 %)

75

(45.7%)




E. Effect of cleft deformity on the social acceptability of the patient

In the modern society, the value of facial

aesthetics and a beautiful face cannot be

underemphasized. In other words, in the

current scenario, facial beauty has more

importance than ever before. People with

pleasing smiles are more likely to be

professionally successful and have higher self-

esteem compared to people with compromised

facial esthetics. The problem of facial beauty

has even more relevance in patients with cleft

anomaly as they not only have to deal with the

issues of facial scar but also problems affected

due to articulation of speech, higher nasalance

which hampers communication and therefore,

lead to lowered self-esteem. Hence, this aspect

was given its due importance in our study as

the primary objective of the study is to

document the treatment needs of patients with

cleft. The problem becomes even more severe

in the Indian setup where many patients

receive less than optimal primary surgeries and

many receive only partial treatments.

In our sample, only the adult (18 years

and above) patients were selected for

evaluation in this category where 39 cases

were found. Of the 39 cases, only 32 cases

could be evaluated (7 cases from Safdarjung

not applicable) and the results were quite on

the expected lines. When the sample of 32

subjects were enquired whether the cleft had

affected their professional life, 29 of the

responded gave a positive reply (Table 11).

F. Post natal counselling of parents with regards to feeding and treatment possibilities related to cleft

One of the significant aspects of cleft care is

the post natal counselling of the parents

regarding the anomaly, the effects of the

anomaly and the treatment possibilities. Since,

many of the parents have never seen such an

anomaly before in life, they are many times

not aware that the defect is correctable and that

the treatment must follow a certain course to

give expected results. Also, the feeding of the

cleft child is difficult, challenging and has to

be done in a specific way. Many of the parents

are not well versed with feeding such a child

and consequently the child suffers from

various problems like malnutrition, inadequate

weight gain, poor health, respiratory and nasal

diseases and even possibility of death due to

aspiration while feeding. The problem is more

acute in rural areas where access to medical

facilities and online resource is limited.

In our study we evaluated whether the

parents of the child with cleft received post

natal counselling or not. We specifically

attempted to know whether they received

correct advice in the following parameters: a.

Feeding of the cleft new born, b. timeline for

surgical interventions, c. possible speech

defects that may occur and its correction, d.

dental and orthodontic interventions.

In our evaluation, we found that out of

the 164 cases, parents of 91 cases received

correct post natal counselling for one or more

of the parameters we evaluated (mentioned

above) while 73 cases did not receive any post

natal advice regarding the management of the

deformity (Table 12). Amongst these 73 cases,

many received advice later from various

sources like family elders, other patients with

cleft or from hospitals they went for treatment.

A few of the cases even reported that they

were told by the dais, which assisted in

delivery that the child would not survive.

Out of the 91 cases who received post-

delivery advice, only 9 received correct advice

for all the parameters evaluated, namely

feeding, surgical correction of deformity,

dental and hearing and speech abnormalities

(Table 13, Graph 9). The remaining 82 cases

received incorrect advice for at least one of the

parameters. Interestingly, hearing and speech,

and dental and orthodontic treatment were the

criteria which were paid the least attention to

amongst the defined criteria when the post

natal parental counselling was done.

G. Age wise distribution of primary lip and palate repair

The primary lip and palatal surgeries of the

cleft are extremely important in deciding the

outcome of the treatment as it is one of the

most important parameters dictating the post-

surgical growth of the maxilla in all the 3

planes. Growth restriction following primary

surgery of palate is common in many patients

leading to sagittal maxillo-mandibular

discrepancy causing development of Class III

malocclusion. Under normal circumstances,

the primary lip repair should be carried out

between 3-6 months of age.



1) Primary Lip Repair

When the age at primary lip surgery was

analyzed, of the 164 cases, 26 cases of CP and

2 unoperated cases were excluded. For the

remaining 136 cases, the age of primary lip

surgery varied considerably ranging from 2

months to 180 months. Majority of cases were

operated within 6 months of birth (75 cases;

45.7%) while 61 cases (37.19%) were operated

beyond 6 months of birth (Table 14, Graph

10).

2) Age at primary palatal surgery

When the age of primary palatal surgery was

analyzed, out of the 164 cases, 142 cases had a

cleft of the palate. Out of these, 20 cases

remained un-operated for the palate at the time

of presentation. The remaining 122 cases,

when analyzed, showed significant variation in

timing of the palatal surgery varying from 3

months to 228 months. 72 out of 122 cases

were operated within 18 months of birth (59%)

while the remaining received surgery after 50

months (41%) cases (Table 15, Graph 11).

Pre-surgical orthopaedics is indicated

in few cases for correction of severe cleft

defects so that the surgeon can easily

approximate the cleft segments without risking

the stretching of the tissue. It is indicated only

in the first few months after birth and although

recently its efficacy has been questioned, it

remains a useful technique in selected cases. In

our sample of 164 cases, when the history for

pre-surgical orthopaedic treatment was

recorded, it was found that not even a single

case received the said treatment (Table 16).

H. Previous history of dental and orthodontic treatment

Cleft patients usually have a multitude of

dental problems including missing teeth,

supernumerary teeth, abnormal tooth positions

and inclinations, increased incidence of

impacted teeth, amongst other problems. In

many cases increased incidence of caries,

gingival and periodontal disease is seen.

Hence any cleft child should be put under the

supervision of a dentist after the eruption of

the first primary tooth. i.e. age of 1 year

onwards. Orthodontic intervention is usually

indicated after the age of 6 years, when the

first permanent molars have completely

erupted. The treatment usually involves

expansion of the upper arch so that by the age

of 9-10 years the child can undergo alveolar

bone grafting (ABG). Full fixed orthodontic

treatment is usually indicated after the eruption

of complete permanent dentition i.e. 12-13

years.

Out of 164 cases, 58 cases (35.4%)

reported that they sought some sort of dental

treatment during their lifetime while a majority

of the cases, as many as 106 (64.6%), did not

report any history of dental treatment (Table

17). The dental treatment included any sort of

filling, scaling, root canal treatment, extraction

of teeth, etc.

Out of 164 cases, 42 cases fell in age

group 18 months-6 years. Since this age group

has little relevance in orthodontic treatment,

they were excluded from analysis. Of the

remaining 122 cases above 6 years, 28 cases

(23%) reported that they sought some sort of

orthodontic treatment during their lifetime

while a majority of 94 cases did not reported

any history of orthodontic treatment. Out of

the 12 cases who sought orthodontic treatment,

the age range for seeking treatment varied

widely between 4 years to 22 years (Table 18).



Table 11- Effect of cleft deformity on the social acceptability of the patient

Centre Yes

(Code 1)

No

(Code 2)

Sample considered/ total sample

AIIMS 10 0 10/55

Safdarjung 9 2 11/54

Medanta-

The MEDICITY 10 1 11/55

Total 29

(90.6%)

3

(9.4%)

32/164

*Safdarjung 43cases NA


Table 12- Post natal counseling of parents with regards to feeding of child with cleft

and his treatment possibilities related to cleft

Centre

Patients who received

correct advice for at least 1 parameter

% Patient who

did not receive correct advice

%

AIIMS 37 67.3 18 33.7

Safdarjung 26 48.1 28 52.9

Medanta- The MEDICITY 28 50.9 27 49.1

Total 91 55.4 73 44.6


Table 13- Distribution of the patients who received correct advice for at least one of the evaluated variables

Centre All the

parameters

Feeding Surgical

correction Dental

Hearing and

speech

More than one

parameter

AIIMS 3 13 12 4 3 2

Safdarjung 0 1 7 0 0 18

Medanta-

The MEDICITY 6 3 2 0 0 17

Total 9 17 21 4 3 37




Table 14- Age wise distribution of lip repair

Centre ≤6 months >6 to ≤12

months >12 - ≤18

months >18 months -

≤6 years >6 years

AIIMS 26 16 2 6 0


Medanta-


Total 75

(55.2%)

31

(22.8%)

9

(6.6%)

17

(12.5%)

4

(2.9%)


Table 15- Age at palatal repair excluding alveolus

Centre ≤6 months >6 to ≤12

months >12 - ≤18

months >18 months - ≤6 years

>6 years

AIIMS 2 14 11 18 3


Medanta-


Total 6

(4.9%)

42

(34.4%)

24

(19.7%)

43

(35.2%)

7

(5.7%)

AIIMS- 52 with Cleft palate-4 unoperated; Safdarjung-46 with Cleft palate-10 unoperated; Medanta- The MEDICITY-44 with Cleft palate-6 unoperated


Table 16- Previous history of pre-surgical orthopaedic

treatment

Centre Yes No

AIIMS 0 55 (100%)

Safdarjung 0 54 (100%)

Medanta- The MEDICITY 0 55 (100%)

Total 0 164 (100%)




Table 17- Previous history of dental treatment

Centre Yes

(1)

No

(2)

AIIMS 19 36

Safdarjung 13 41

Medanta-The MEDICITY 26 29

Total 58 (35.4%) 106 (64.6%)


Table 18- Positive history of post surgical orthodontic treatment

Centre Yes No

AIIMS 12 43

Safdarjung 5 49


Total 28 (17.1%) 136 (82.9%)




I. Dental examination

1) Presence of supernumerary teeth

Supernumerary teeth are common in cleft

patients because the surgical intervention

frequently leads to aberrations in tooth

formation leading to various developmental

anomalies like missing teeth, supernumerary

teeth, malformed teeth, dilacerations of roots,

impacted teeth, etc.

In the sample of 164 cases, 12 cases had a

completely unoperated cleft. None of these

had any supernumerary teeth. Of the

remaining 152 cases with operated cleft, 132

cases (86.8%) did not have supernumerary

teeth while 20 cases had supernumerary teeth

(13.2%) (Table 19). Of the 20 subjects with

supernumerary teeth, 19 teeth were in relation

to cleft while 1 teeth was unrelated to cleft.

2) Presence of dental cross bite in the sample

Patients with cleft frequently suffer from

maxillo-mandibular growth anomalies leading

to restriction of maxillary growth in all three

planes while the mandible may grow normally.

This causes development of Angle Class III

malocclusion in the sagittal plane, cross bites

in both the anterior and posterior segments of

the dentition and loss of vertical maxillary

height in many cases.

The anterior dental segment comprises

of 6 teeth from canine to canine. The posterior

segment consists of teeth posterior to the

canine tooth i.e. the premolars and the molars.

In our analysis we studied the presence of

cross bite as single tooth, 1-3 teeth, or more

than 3 teeth. However, since single tooth cross

bites are usually not indicative of an

underlying growth disturbance; these types of

cross bites were grouped with the category of

“cross bite absent” when assessing the

posterior segment relations. Cross bites of

more than 1 tooth were taken to be indicator of

growth restriction/collapse of segment and

were grouped together for ease of analysis and

data presentation.

3) Growth restriction in the anterior segment of maxilla (as a function of anterior cross bite)

In the sample of 164 cases, 21 cases of cleft

were unoperated, 6 did not cooperate while 6

could not be assessed and therefore, excluded

from further analysis. Of the remaining 131

cases, 48 cases (32.9%) did not have an

anterior dental cross bite while the remaining

98 cases (67.1%) had cross bite ranging from

single tooth to complete anterior segment

(Table 20, Graph 12). Most of the cases with

cross bite of more than single tooth belonged

to UCLP (52%) and BCLP (30.6%). If we

consider presence of cross bite in more than 3

teeth as an indicator of sagittal growth

restriction of maxilla, 43 cases (43.9%) had

significant restriction of growth of maxilla in

the sagittal plane, all belonging to UCLP and

BCLP categories.

4) Growth restriction in the posterior segment of maxilla (as a function of posterior cross bite)

In the sample of 164 cases, 21 cases of cleft

were unoperated, 6 did not cooperate and 6

could not be assessed and therefore excluded

from further analysis. Of the remaining 131

cases, 73 cases (55.7%) did not have posterior

cross bite while 58 cases (44.3%) had

significant posterior cross bite (more than 1

tooth). Amongst the 58 cases with cross bite,

39 cases (67.24%) had bilateral cross bite

while 19 cases (32.75%) had unilateral cross

bite.

When the distribution of posterior cross

bite was seen across the various cleft types,

cases with UCLP and BCLP had the maximum

proportion of cases with cross bites more than

1 tooth. In UCLP cases 34 out of 62 cases

(54.8%) had posterior cross bite while 28 did

not have posterior cross bite. In BCLP, out of

34 cases, 22 cases (64.7%) had posterior cross

bite. Interestingly, out of these 22 cases, 20

had a bilateral posterior cross bite (Table 21).

5) Overjet and Overbite in the sample

Overjet is the representation of the relation of

anterior teeth in the sagittal plane. In normal

circumstances, overjet is positive with the

upper anterior teeth in front of lower anterior

teeth. Overbite represents the vertical overlap

of anterior teeth. In ideal circumstances both

overjet and overbite remains between 1-2 mm.

In cleft patients however the maxillary growth

is retarded and this may lead to alteration in

the ideal overjet and overbite relations. With

growth restriction of maxilla, the overjet may

reduce and even become negative which

implies a greater treatment challenge.



In our sample, out of 164 cases, 19 cases

could not be evaluated. Of the remaining 145

cases, 74 cases (51.4%) showed a positive

favourable overjet of more than 1 mm while

25 cases (17.24%) had an overjet of -3mm or

lesser (Table 22). These cases pose a

significant challenge for orthodontic treatment

and many of these would ultimately require

combine orthodontic and orthognathic surgery.

When the overbite in the sample was assessed

it was seen that 10 cases out of 145 (6.8%) had

an anterior open bite while 93 cases (64.13%)

had 0-2mm of overbite. 42 cases (29%) had

more than 2mm of overbite (Table 23). It must

be stressed here that a positive overbite is

indicative of better maxillary growth and thus

cases with deep bite (more than 2 mm) are

considered more favourable during treatment

while cases with 0-1 mm overbite may

progress to anterior open bite if the future

growth of maxilla is unfavourable.

J. GOSLON YARDSTICK

The outcome of the primary cleft surgeries on

the maxillo-mandibular growth can be

evaluated using the GOSLON yardstick. The

Goslon yardstick essentially evaluates the

sagittal maxillo-mandibular dental arch

relations and reflects, in part, the effect of cleft

surgeries on the sagittal growth restriction of

maxilla. It also reveals the complexity of

orthodontic treatment anticipated. Goslon

yardstick has been devised to be selectively

used in cases with UCLP anomaly only.

The dental arch relations in our study

were assessed using Goslon Yardstick. All

cases with operated UCLP anomaly above 7

years of age were selected for the evaluation.

Of the 164 cases, only 40 cases fit the

inclusion criteria used for applying the Goslon

Yardstick. Dental study models of all these

cases were evaluated for the anteroposterior

dental arch relations and grouped into

categories 1-5 (Table 24).

The findings revealed that 20 (50%) out

of 40 cases fell in Goslon category 3, 10 in

Goslon category 4 (25% cases), and 9 cases in

Goslon category 2 (22.5%) (Table 25, Graph

13). Only 1 case was found in category 5.

Thus, it was seen that 75% of the cases with

operated UCLP had complex orthodontic

treatment needs while 28% cases required

combined orthognathic surgery and

orthodontic treatment for optimal outcome.

Only 25% cases had minor orthodontic

treatment needs.

K. Examination of primary cleft

Although in today’s social setup, many cleft

cases receive surgical intervention for the

primary clefts, there are still patients who end

up receiving partial or no surgeries at all

especially if we move to semi urban and rural

setups. Such cases remain unoperated for

periods significantly beyond the expected date

of lip and/or palate surgery. This segment of

the report covers the clinical profile of such

cases. In our sample, 12 cases were unoperated

and 9 cases were partially operated.

1) Widest gap in the cleft palate (including alveolus)

Out of the sample of 21 cases, only 10 could

be evaluated. Of the 10 cases, the widest gap

in the cleft palate (including alveolus) was also

more than 1 cm in 8 cases (80%) (Table 26).

2) Length of the palate

Out of the 21 unoperated/partially-operated

cases, 15 could be assessed. Amongst the 15

cases, 6 cases (40%) had adequate palatal

length while 9 (60%) had inadequate palatal

length (Table 27).



Table 19- Supernumerary teeth

Centre

Present

Absent Related to cleft

Not related to cleft

Both

AIIMS 9 0 0 43

Safdarjung 8 1 1 40

Medanta- The MEDICITY 1 0 0 49

Total 18 (11.8%) 1(0.7%) 1 (0.7%) 132(86.8%)

* AIIMS- 3 cases completely unoperated, Safdarjung- 4 cases completely unoperated & Medanta- The MEDICITY 5 cases completely unoperated not included


Table 20- Presence of anterior cross bite

Type of cleft Absent N Single tooth >1-<=3 Teeth >3 teeth

CL 11 0 0 0

CLA 5 2 1 0

UCLP 11 8 16 26

BCLP 6 3 11 16

CP 9 2 3 0

Total 42

(32.3%)

15

(11.6%)

31

(23.8%)

42

(32.3%)

*AIIMS-4 cases unoperated; Safdarjung-10 cases unoperated and 6 could not be assessed; Medanta- The MEDICITY 7 cases unoperated and 6 cases non cooperative.




Table 21- Presence of posterior cross bite in the sample of 55 cases

Type of cleft

Present

Absent Unilateral Bilateral

CL 0 0 10

CL B/L 0 0 1

CL A 0 0 6

CLA B/L 1 0 1

UCLP 16 18 28

BCLP 2 20 12

CP 0 1 15

Total 19

(14.5%)

39

(29.8%)

73

(55.7%)

*AIIMS-4 cases unoperated; Safdarjung-10 cases unoperated and 6 could not be assessed; Medanta- The MEDICITY 7 cases unoperated and 6 cases non cooperative.


Table 22 - Overjet in the sample

CL CLA UCLP BCLP CP Total

Less than equal to -3mm

0 0 13 12 0 25

-2mm to 0 0 2 29 11 4 46

1mm and above 12 6 27 12 17 74

Total 12

(8.3%)

8

(5.5%)

69

(47.6%)

35

(24.1%)

21

(14.5%)

145

(100%)

n= 145 *9 cases in Medanta- The MEDICITY and 10 cases in SJ NA



Table 23- Overbite in the sample

CL CLA UCLP BCLP CP

Total GIVE IN %

Less than 0 0 0 8 2 0 10

0-2mm 9 3 42 22 17 93

More than 2mm 3 5 19 11 4 42

Total 12

(8.3%)

8

(5.5%)

69

(47.6%)

35

(24.1%)

21

(14.5%)

145

(100%)

n= 145 *9 cases in Medanta- The MEDICITY and 10 cases in SJ NA

Table 24- Goslon Yardstick scores and their interpretation

Goslon category

Usual Dental

relation Orthodontic Treatment need

Treatment outcome

1 Positive overjet

Mild malocclusion; minimal or no orthodontic treatment required

Excellent

2 Positive overjet

Mild malocclusion; minimal orthodontic treatment required

Good

3 Edge to edge bite

Complex orthodontic treatment to correct the Class III

malocclusion but a good result can be anticipated

Fair

4

Negative

overjet

1-3 mm

Complex orthodontic treatment

needs; future orthognathic surgery may be required

Poor

5

Negative

overjet >3 mm

Definite requirement of orthognathic surgery

Very poor



Table 25- Distribution of subjects according to the Goslon Yardstick

Goslon score Number of cases

1 0

2 9 (22.5%)

3 20 (50%)

4 10 (25%)

5 1 (2.5%)

Total 40


Table 26- Widest gap in the cleft , palate + alveolus

Type of cleft ≤0.5cm >0.5 - ≤1cm

> 1cm Total

CLA B/L 0 0 0 0

UCLP 1 1 5 7

BCLP 0 0 0 0

CP 0 0 3 3

Total 1 1 8 10

* 3 cases of AIIMS excluded (Submucus CP), 1 case of Safdarjung excluded (Submucus CP) & Medanta- The MEDICITY- 4 cases of submucus CP, 1 case of CL and 2 cases did not cooperated are excluded. n=10; Data represents no. of patients in each category

Table 27- Length of the palate

Type of cleft Adequate Inadequate

UCLP 4 3

BCLP 0 0

CP 2 6

Total 6 9

n=15 * 5cases could not be assessed and 1case of CL excluded.



L. Examination of secondary cleft deformity

A lot of cases in today’s setup get operated for

their primary cleft, at various ages. That,

however, does not mean the end of treatment.

A host of issues crop up after the primary

surgeries, some related to surgery, some

unrelated to surgery. The outcome is gravely

dependent on the quality of surgical repair as

that dictates the long term growth

characteristics of the maxilla. Apart from that,

the Eustachian tube function, altered dental

development, residual scars, palatal function

and altered speech are few of the aspects

which merit consideration. This section of the

report aims to put some light on these issues.

i) Evaluation of lip (and scar)

Surgical repair of cleft lip result in scar

formation which may vary from a fine

imperceptible line to thick, ugly and fibrous

scars. The amount of scar tissue depends upon

many variables including the severity of the

cleft, the quality of surgery, timing of the

repair, etc. Lip scars can create both esthetic

and functional problems. A heavy lip scar can

make for an unsightly facial appearance

leading to psychosocial issues while functional

problems can result due to altered speech,

improper lip seal, altered orofacial muscular

balance and malocclusion due to pressure from

fibrosed tissue.

The upper lip, in our study, was evaluated

for bilateral symmetry, the amount and quality

of scar tissue, the size (length) of scar and its

angulation. The overall quality of repair was

evaluated on the basis of these parameters and

subjective evaluation to rate the outcome from

poor through very good.

ii) Evaluation of the lip scar

When the width (length) of the lip scar was

evaluated in patients with unilateral cleft

(UCL, UCLA and UCLP), 97 cases were

detected; out of which 2 case were unoperated.

Among the remaining 95 operated cases, 14

cases (15%) had a scar less than (equal to) 0.5

mm while 46 cases (48.4%) had a scar varying

between 0.5-1 mm (Table 28). As many as 35

cases had a scar width more than 1 mm.

When the angulations of the lip scar

were evaluated, vertical scars (57 cases (60%)

were more common than oblique scars (38

cases; 40%) (Table 29). The quality of scar

repair, when evaluated subjectively, depending

upon multiple factors, it was found that the

quality of scar repair varied between poor to

good while the categories of ‘very good’ and

‘excellent’ remained blank.

Amongst the cases with bilateral lip

involvement (BCL, BCLA and BCLP), the

right and the left side were evaluated

separately. Amongst the 41 cases, on the right

side, 16 patients (39%) had a scar more than 1

mm in length which is obvious to create

esthetic problems. Only 10 cases (24.4%) had

a scar less than 0.5mm (Table 30) while 15

cases had a scar length between 0.5-1mm

(36.6%). The predominant angulation of the

scar was vertical (75.6%) (Table 31).On the

left side, 20 out of 41 scars (48.8%) had scar

width more than 1 mm while only 8 cases

(19.5%) had an aesthetic scar less than 0.5 mm

wide. In angulation, 29 (70.7%) out of 41 scars

had vertical angulation.

iii) Evaluation of lip seal: at rest and while blowing

Achieving lip seal in cleft patients can be

quite a challenge in patients with cleft

anomaly especially if the quality of repair is

not satisfactory, or if the scar is overwhelming

and restricts mobility of the lip or if the

premaxilla is protruded significantly. Lip seal

while blowing indicates if the perioral muscles

are able to make tight seal at lips to prevent air

escape when lips are stressed. In cases with

cleft lip proper approximation of muscle fibers

while surgical correction of defect is necessary

for proper lip function; in cases with improper

lip function, lip seal while blowing may be

absent. Additionally, presence of excessive

scar tissue, reduced height of upper lip as well

as protruding premaxilla may also lead to

absence of lip seal while blowing.

In our sample, although the quality of

scar repair was not good in majority of the

sample but lip seal was present in most of the

subjects. Of the sample of 136 cases, 2 cases

were unoperated for lip and were excluded. Of

the remaining 134 cases assessed for lip seal at

rest, 14 cases (10.4%) were found to have

deficient lip seal at rest while only 5 cases out

of 124 cases lacked proper seal while blowing

(Table 32). Note that the sample for lip seal at

rest and while blowing is not same as 9 cases



were partially unoperated cleft were excluded

while 1 did not cooperate for blowing.

iv) Evaluation of lip symmetry

Lip symmetry was analyzed to judge the

overall symmetry of the upper lip, irrespective

of the side of involvement. In unilateral cases,

it involved comparing the affected side to the

normal side. In bilateral cases, it involved

comparing not only both the sides but also

both the sides to a normal unaffected lip. In

our sample of 164 cases, 26 had CP while 2

cases were unoperated and thus were

excluded. Of the remaining 134 cases, only 38

cases (28.4%) had good lip symmetry while 49

cases (36.6%) had fair lip symmetry. 30 cases

(22.4%) had poor lip symmetry while only 4

cases had excellent lip symmetry (Table 33,

Graph 14).

v) Overall appearance of lip

Overall appearance of the lip was

considered a combined result of lip

symmetry, width of the scar, cross hatches

across scar line and thickness of the

vermilion border. The lips were

subjectively evaluated by a single expert

for each of the 3 institutions. In our sample

of 164 cases, 26 cases of CP and 2

unoperated cases for lip were excluded. Of

the remaining 134 cases, 33 cases (24.6%)

cases had a poor overall lip appearance, 54

(40.3%) had fair appearance while 43

(32.1%) cases had good or very good lip

appearance (Table 34, Graph 15).

Table 28- Length of scar in unilateral clefts

Type of cleft ≤0.5mm >0.5 - ≤1mm >1mm

CL U/L 1 6 4

CL A U/L 1 4 2

UCLP 12 36 29

Total 14 46 35

*AIIMS- 22 cases did not have cleft lip and 1 case unoperated, Medanta- The MEDICITY- 27 cases did not have cleft lip and 1 case unoperated, Safdarjung- 18 cases did not have cleft lip n=95; Data represents no. of patients in each category

Table 29- Angulation of scar in unilateral cleft

Type of cleft Vertical Oblique

CL U/L 9 2

CL A U/ L 6 1

UCLP 42 35

Total 57

(60%)

38

(40%)




Table 30- Length of scar in bilateral cleft

Type of cleft

Right Left

≤0.5mm >0.5 - ≤1mm

>1mm ≤0.5mm >0.5 - ≤1mm

>1mm

CL B/L 0 1 0 0 1 0

CL A B/L 0 1 1 1 0 1

BCLP 10 13 15 7 12 19

Total 10 15 16 8 13 20


Table 31- Angulation of scar in bilateral cleft

Type of cleft

Right Left

Vertical Oblique Vertical Oblique

CL B/L 1 0 1 0

CL A B/ L 1 1 1 1

BCLP 29 9 27 11

Total 31 10 29 12




Table 32- Assessment of lip seal

Type of cleft

Lip seal at rest Lip seal while blowing

Present Absent Present Absent

CL 9 1 11 0

CL B/L 1 0 1 0

CLA 5 2 7 0

CL A B/L 1 1 1 1

UCLP 71 5 68 1

BCLP 33 5 32 3

Total 120

(89.6%)

14

(10.4%)

119

(96%)

5

(4%)

1 case could not assessed and 2 cases unoperated

12 cases could not be assessed

6 cases of CP excluded



Table 33- Evaluation of lip symmetry

Poor Fair Good Very good Excellent Total

CL 0 3 5 2 0 10

CL B/L 0 0 0 0 1 1

CL A 1 3 3 0 0 7

CLA B/L 0 2 0 0 0 2

UCLP 18 24 22 9 3 76

BCLP 11 17 8 2 0 38

Total 30

(22.4%)

49

(36.6%)

38

(28.4%)

13

(9.7%)

4

(2.9%) 134

26 cases of CP excluded and 2 cases unoperated for lip


Table 34- Overall appearance of lip

Type of cleft Poor Fair Good Very good Excellent Total

CL 0 6 2 2 0 10

CL B/L 0 0 0 0 1 1

CL A 1 5 1 0 0 7

CLA B/L 1 1 0 0 0 2

UCLP 14 31 19 9 3 76

BCLP 17 11 9 1 0 38

Total 33

(24.6%)

54

(40.3%)

31

(23.1%)

12

(9%)

4

(3%) 134





M. Evaluation of the nose

i) Overall appearance of nose

Like the previous parameter this also evaluates

the overall aesthetic outcome of nose

following surgeries for cleft lip and palate. The

examination was subjectively done by a single

expert. Various parameters were taken into

account while doing the analysis, including

deviation of nasal septum, length of columella,

alar base width, bilateral symmetry etc.

In our sample of 164 cases, 26 cases had

cleft palate only and 2 cases were unoperated

for lip. These were excluded from further

analysis. Analysis of the remaining 134 cases

revealed that 10 cases (7.5%) had a very good

result while 25 cases (18.7%) had a good

result. As many as 47 cases (35%) had a poor

outcome for appearance of nose (Table 35,

Graph 16).

ii) Evaluation of nasal septum

Patients with cleft lip and palate anomaly

frequently show deviation of nasal septum. In

unoperated cases this happens due to altered

anatomical relationships which causes

deviation in growth direction, while in

operated cases it happens predominantly due

to the stretch created by scar tissue.

In our sample of 164 cases, 12 cases

could not be assessed (Table 36, Graph 17)

and were excluded from further analysis.

Analysis of the remaining 152 cases was

separately done for unilateral cleft cases and

other type of clefts. The rest of the cases were

grouped together. For the unilateral cleft cases,

out of 97 cases, 24 cases (24.7%) had a non-

deviated nasal septum, while 49 cases (50.6%)

had deviation of nasal septum to the opposite

side to that of the cleft.

For the rest of the cases which included

cases of bilateral cleft and CP (Table 37,

Graph 18), out of the 55 cases, 17 had a non-

deviated septum (31% cases) while 19 cases

(34.5%) cases showed deviation of nasal

septum to either side.

iii) Evaluation of width of the nostril floor

Distortion of the alar base and dome

frequently occur following surgical correction

of cleft deformity. This may lead to a variety

of alar malformations like widening, stretching

and depression of the alar dome. In our

analysis we categorized the width of the alar

base as equal and unequal. Of the 164 subjects,

26 cases with CP and 2 unoperated cases of lip

were excluded. Of the remaining 136 cases, 95

cases (69.9%) had an equal nostril floor width

while 41 cases (30.1%) had an unequal width

(Table 38, Graph 19).

Palatal evaluation in secondary cleft deformity

a. Evaluation of the length of soft palate

Adequate length of soft palate is essential for

achieving velopharyngeal competence. In

velopharyngeal incompetence, the posterior

aspect of soft palate does not connect to the

Passavant’s ridge of the posterior pharyngeal

wall. This causes nasal intonation of speech

and nasal escape of liquids. Velophryngeal

incompetence is frequently seen in patients

with cleft lip and palate, especially in operated

cases where the scar tissue and the restriction

of growth due to effects of surgery lead to a

short soft palate and/or inadequate movement

of palate during function.

In our sample out of 164 cases, only

120 could be assessed (20 cases were

unoperated and 15 cases had cleft lip only

while 3 cases could not be evaluated due to

young age, 6 cases of CLA excluded). Of the

remaining 120 cases, 49 cases (40.8%) had a

short palatal length while 71cases (59.2%) had

an adequate length (Table 39, Graph 20). If we

consider the two largest categories UCLP and

BCLP, the percentage of cases with short

palatal length within these categories is even

higher (60% in UCLP; 66% in BCLP).

b. Evaluation of scarring and mobility of the soft palate

In our sample, out of 164 cases, only 121

could be assessed (20 cases were unoperated

and 15 cases had cleft lip only while 2 cases

could not be evaluated due to young age, 6

cases of CLA excluded). Of the remaining 121

cases, only 31 cases (25.6%) had little scaring

of palate. Majority of cases (72 cases; 59.5%)

had an acceptable scaring of palate while 18

cases (14.9%) cases had significant scaring

(Table 40, Graph 21).

The mobility of the soft palate governs the

functional efficacy of the same, as effective lift

of the palate is essential for proper



velopharyngeal closure. In our sample, out of

164 cases, only 118 could be assessed (20

cases were unoperated and 15 cases had cleft

lip only while 4 cases could not be evaluated

due to young age, 6 cases of CLA excluded.

Of the remaining 118 cases, 17 cases (14.4%)

were deemed to have unsatisfactory mobility

while 101 (83.6%) had satisfactory mobility

(Table 41).

When the uvula was assessed in the

sample, in the 121 cases assessed, the majority

of cases showed a uvula which was not well

formed or bifid (74 cases; 61%) (Table 42).

c. Presence of post-surgical palatal fistula

Palatal fistulas may result in cleft surgeries

due to various factors, both related to surgery

as well as patient-related factors. The residual

fistula is associated with recurrent nasal

infections, nasal regurgitation of liquids and

also may affect the quality of voice.

In our sample, out of 164 cases, 20

cases were unoperated, 1 could not be assessed

due to the age factor. These cases were thus

excluded. Of the remaining 143 cases, only 65

cases (45%) did not have a fistula while 78

cases (55%) had a residual palatal fistula

(Table 43, Graph 22). Amongst the 78 cases

with fistula, majority of fistulas were located

in the peri-alveolar region (65 cases; 83%). Of

the 78 cases with fistula 57 cases (73%) gave

positive history of nasal regurgitation (Table

44).

The maximum diameter/length of the

fistula was noted in each case to evaluate the

size of the fistula. For the purpose of

categorization and representation, the size of

the fistula was classified as follows: a. Less

than 2mm in maximum length, b. 2-5 mm, c.

More than 5 mm. In our sample, out of 78

cases with palatal fistula, 28 cases each

(35.9%) had fistula in category a and b. Fistula

of more than 5mm in length was seen in 22

cases (28.2%). (Table 45, Graph 23).

The incidence of speech abnormality

due to fistula was found to be quite low in the

sample. Only 76 of the 78 cases could be

assessed (Table 46). Of the 76 cases, 35 cases

(46%) with fistula had significant speech

changes when the fistula was blocked

temporarily during speech assessment.

Closure of palatal fistula is frequently

undertaken due to a variety of functional and

psychological reasons like speech abnormality,

recurrent nasal regurgitation of fluids,

recurrent infections and psychological

concerns amongst parents and patients. Of the

77 cases assessed in the category, 26 cases

(33%) had been operated previously for fistula

closure, while 51 did not receive any surgery

for the same (67%). (Table 47)

Table 35- Overall appearance of nose

Type of cleft Poor Fair Good Very good

Excellent Total

CL 0 4 5 1 0 10

CL B/L 0 0 0 1 0 1

CL A 2 4 1 0 0 7

CLA B/L 1 0 1 0 0 2

UCLP 29 27 12 8 0 76

BCLP 15 17 6 0 0 38

Total 47

(35%)

52

(38.8%)

25

(18.7%)

10

(7.5%) 0 134





Table 36- Evaluation of nasal septum – Unilateral clefts

Non-Deviated Cleft side Non cleft side

CL 3 2 7

CL A 1 3 3

UCLP 20 19 39

Total 24

(24.7%)

24

(24.7%)

49

(50.6%)


Table 37: Evaluation of nasal septum - Bilateral clefts

Type of cleft

Non-Deviated

(0)

Left

(1)

Right

(2) Total

CL B/L 1 0 0 1

CLA B/L 1 0 1 2

BCLP 6 16 16 38

CP 9 3 2 14

Total 17

(31%)

19

(34.5%)

19

(34.5%) 55

Cases excluded Medanta- The MEDICITY-12 cases of CP NA


Table 38- Evaluation of nostril floor width

Type of cleft Unequal Equal Total

CL 4 7 11

CL B/L 1 0 1

CL A 1 6 7

CL A B/L 1 1 2

UCLP 21 56 77

BCLP 13 25 38

Total 41

(30.1%)

95

(69.9%) 136

26 cases of CP excluded and 2 cases unoperated




Table 39- Evaluation of the length of the palate in the sample

Type of cleft Short Adequate Total

UCLP 26 43 69

BCLP 17 18 35

CP 6 10 16

Total 49

(40.8%)

71

(59.2%) 120

AIIMS-3 cases unoperated, 1 case of CL, 2 cases could not be assessed due to younger age and 2 cases of CLA Safdarjung- 10 cases of unoperated for the palate and 8 cases of CL Medanta- The MEDICITY- 7 cases unoperated, 6 cases of CL, 1 case could not be assessed and 4 cases of CLA


Table 40- Evaluation of post surgical scarring of the palate in the sample

Little Acceptable Too much Total

UCLP 15 47 7 69

BCLP 10 19 8 37

CP 6 6 3 15

Total 31

(25.6%)

72

(59.5%)

18

(14.9%) 121

AIIMS-3 cases unoperated, 1 case of CL, 2 cases could not be assessed due to younger age and 2 cases of CLA Safdarjung- 10 cases of unoperated for the palate and 8 cases of CL Medanta- The MEDICITY- 7 cases unoperated, 6 cases of CL and 4 cases of CLA


Table 41- Mobility of the palate in the sample

Type of cleft Satisfactory Unsatisfactor

y Total

UCLP 61 7 68

BCLP 27 7 34

CP 13 3 16

Total 101

(85.6%)

17

(14.4%) 118

AIIMS-3 cases unoperated, 1 case of CL, 2 cases could not be assessed due to younger age and 2 cases of CLA Safdarjung- 10 cases of unoperated for the palate, 8 cases of CL and 1 case could not be assessed Medanta- The MEDICITY- 7 cases unoperated, 6 cases of CL, 4 cases of CLA and 2 cases could not be assessed




Table 42- Status of uvula in operated cases of cleft palate

Type of cleft Well

formed Not well formed

Bifid Total

UCLP 26 40 3 69

BCLP 8 27 2 37

CP 5 7 3 15

Total 39

(32.3%)

74

(61%)

8

(6.7%) 121

AIIMS-3 cases unoperated, 1 case of CL, 2 cases could not be assessed due to younger age and 2 cases of CLA; Safdarjung- 10 cases of unoperated for the palate and 8 cases of CL; Medanta- The MEDICITY- 7 cases unoperated, 6 cases of CL and 4 cases of CLA


Table 43- Presence of fistula in the sample

Type of cleft Absent Peri-

alveolar Hard

Palate

Junction of Hard and Soft

Palate

Soft Palate

Multiple fistula

Total

CL U/L 11 0 0 0 0 0 11

CL B/L 1 0 0 0 0 0 1

CL A U/L 6 1 0 0 0 0 7

CLA B/L 0 2 0 0 0 0 2

UCLP 31 32 3 0 2 1 69

BCLP 7 27 2 0 0 1 37

CP 9 3 1 0 3 0 16

Total 65

(45.5%)

65

(45.5%)

6

(4.2%) 0

5

(3.5%)

2

(1.3%) 143

* AIIMS- 3 cases unoperated and 1 can’t assess , Safdarjung- 10cases unoperated/partially unoperated & Medanta- The MEDICITY- 7 cases unoperated/partially operated




Table 44- Assessment whether the fistula is symptomatic or not

Type of cleft Symptomati

c Asymptomatic Total

CL A 0 1 1

CLA B/L 2 0 2

UCLP 25 13 38

BCLP 26 4 30

CP 4 3 7

Total 57

(73 %)

21

(27%) 78


Table 45- Evaluation of the size of the oronasal fistula in the sample

Type of cleft <=2mm

(1)

2-5mm

(2)

>5mm

(3) Total

CL A 1 0 0 1

CLA B/L 0 2 0 2

UCLP 16 12 10 38

BCLP 8 12 10 30

CP 3 2 2 7

Total 28

(35.9%)

28

(35.9%)

22

(28.2%) 78




Table 46- Speech abnormality due to presence of the fistula

Type of cleft Yes

(1)

No

(2) Total

CL A 0 1 1

CLA B/L 0 2 2

UCLP 15 23 38

BCLP 15 14 29

CP 5 1 6

Total 35 (46%) 41(54%) 76

2 cases of Safdarjung could not be assessed


Table 47- Evaluation whether the fistula has been operated previously or not in the sample

Type of cleft Yes No

CL A 0 1

CLA B/L 0 12

UCLP 10 17

BCLP 13 17

CP 3 4

Total 26

(33%)

51

(67%)

Patients with fistula: AIIMS-34; 1 case from AIIMS did not remember; Safadarjung-20 cases; Medanta- The MEDICITY- 24 cases n=77; Data represents no. of patients in each category



N. ENT examination

Pharyngeal tonsil also can be enlarged in

children with recurrent throat infections and

children with cleft palate anomaly. For our

purpose we evaluated the tonsils between

Grades I through IV. Grade I represents

normal tonsils while Grade IV represents

enlarged, infected tonsils. In our sample, 160

cases were analyzed while 4 cases were

excluded due to young age (non-cooperative).

Out of the 160 cases, 101 cases (63.1%) had

Grade I tonsils and 51 (31.9%) had Grade II

tonsils which is considered to be a less severe

form of tonsillitis (Table 48, Graph 24).

i. Incidence of Ear Discharge

Ear discharge is a representation of middle ear

infection which occurs commonly in cleft

palate patients due to a dysfunction of the

Eustachian tube. Out of the sample of 164

cases, 5 cases were young and could not be

tested. The remaining 159 cases showed

presence of ear discharge in 11 cases (7%),

while the 148 cases (93%) showed no

evidence of clinically evident ear discharge

(Table 49). These 11 cases were distributed in

the age group of 6 yrs-18 yrs. It has been

shown that children of a relatively younger

age, that is, below 10 years may undergo

spontaneous remission of the disease by 10-12

years. However, the older age groups do

require intervention in the form of surgery or

medical help to correct the discharge;

otherwise middle ear adhesion and consequent

hearing loss may be the consequences. Even in

younger age groups serial follow-ups would be

required to ensure that discharge corrects

spontaneously.

ii. Status of tympanic membrane in the sample

Afflictions of the tympanic membrane can be a

significant factor in the development of

hearing loss, which is usually milder and

treatable in affected patients. In cleft patients

the affliction of tympanic membrane can be

broadly classified as retraction and perforation.

Subsequently, retraction is followed by

eventual perforation, if left untreated. Cleft

patients are known to have middle ear

infections and consequent higher incidence of

tympanic membrane defects compare to

normal population.

Unilateral cleft: For ease of comparison, the

sample data was restructured to represent ears

on the same and opposite sides in unilateral

clefts and right and left sides in bilateral clefts.

Out of the 164 subjects, cases with unilateral

cleft constituted 97 cases. Out of the 97 cases,

4 cases could not be evaluated due to non-

cooperation.

Of the remaining 93 cases, when the ear on the

same side of cleft was evaluated, 57 cases

(61%) had normal tympanic membrane while

38 cases (40%) had a retracted or perforated

tympanic membrane. It should be remembered

here that retraction is a precursor to

perforation and that it may resolve

spontaneously in younger children while it

may progress to perforation in more vulnerable

patients. Hence the cases would require

medical treatment, repeated follow-ups and

surgical repair in cases with perforation.

When the tympanic membrane on the

side opposite to the side of cleft was evaluated

in cases with unilateral cleft patients, it was

found that 25 out of 93 cases (26%) had

affected tympanic membrane with 68 having

no problem with the membrane (73%) (Table

50). This distribution is similar to the figures

obtained on the affected side also.

Bilateral Cleft: In the cases with bilateral

cleft lip and/or palate, the ears on the sides

were evaluated as right and left sides. Out of

the sample of 164 cases, 67 cases had cleft

other than unilateral cleft. Of the 67 cases, 5

were non-cooperative and excluded. Hence,

only 62 cases could be assessed.

On the right side 39 cases (62.9%) had

normal TM, while 23 cases (37%) had an

affected TM (Table 51). On the left side 37

cases (59%) had a normal TM while 25 cases

(40.3%) had affected TM. The maximum

number of affected TM was seen in BCLP

cases.



Table 48- Status of Tonsils in the sample according to type of cleft

Type of cleft Grade I

(1)

Grade II

(2)

Grade III

(3)

Grade IV

(4) Total

CL U/L 11 1 0 0 12

CL B/L 1 0 0 0 1

CLA U/L 6 1 0 0 7

CLA B/L 2 0 0 0 2

UCLP 45 28 3 0 76

BCLP 17 15 4 1 37

CP 19 6 0 0 25

TOTAL 101

(63.1%)

51

(31.9%)

7

(4.4%)

1

(0.6%) 160

4 cases from AIIMS could not be assessed due to younger age


Table 49- Incidence of Ear Discharge

Type of cleft Yes

(1)

No

(2)

CL U/L 0 12

CL B/L 0 1

CLA U/L 0 7

CLA B/L 0 2

UCLP 6 70

BCLP 4 33

CP 1 23

TOTAL 11

(7%)

148

(93%)

4 cases from AIIMS could not be assessed due to younger age and 1 case of Medanta- The MEDICITY could not be assessed due to younger age. n=159; Data represents no. of patients in each category



Table 50- Status of tympanic membrane in the sample in unilateral cleft

Type of cleft

Same side of cleft Opposite side of cleft

Normal

(1)

Perforated

(2)

Retracted

(3)

Normal

(1)

Perforated

(2)

Retracted

(3)

CL U/L 11 0 1 12 0 0

CLA U/L 6 0 1 6 0 1

UCLP U/L 40 7 27 50 4 20

TOTAL 57 7 29 68 4 21

Unilateral cases 97; 2 cases of AIIMS and 2 cases of Medanta- The MEDICITY did not cooperated

Data represents no. of ears in each category

Table 51- Status of tympanic membrane-Bilateral cleft

Type of cleft

Right Left

Normal

(1)

Perforated

(2)

Retracted

(3)

Normal

(1)

Perforated

(2)

Retracted

(3)

CL B/L 1 0 0 1 0 0

CLA B/L 1 0 1 1 0 1

BCLP 18 1 17 18 3 15

CP 19 0 4 17 1 5

TOTAL 39 1 22 37 4 21

Bilateral cases 67; 2 case of AIIMS and 3 cases of Medanta- The MEDICITY did not cooperated

Data represents no. of ears in each category



O. Hearing evaluation

1. Prevalence of Hearing defects in the sample

Out of the sample of 164 cases, 30 cases could

not be assessed. Of the remaining 134 cases

that could be assessed for hearing, 74 of the

134 cases (55.2%) had normal hearing while

60 cases (44.8%) had hearing defects in one or

both the ears. Amongst those affected, 45 of

the 60 cases (75%) had bilateral hearing loss

of certain degree while a few of the cases with

unilateral cleft had a hearing loss of the same

side (Table 52, Graph 25). This is remarkable

because hearing loss of the ear of the same

side of cleft is documented but loss on both the

sides in such a percentage is alarming.

Moreover, the sample had many cases which

came to the orthodontic OPD and did not even

know that they had hearing abnormalities.

However, amongst the sample of 60

cases which had a documented hearing loss, 44

cases (73.3%) had a mild level of hearing loss

while 15 (25%) had a moderate loss of hearing

(Table 53, Graph 26). Severe or profound type

of loss was not found in the sample.

In our study we also tried to document

the type of hearing loss in the group. Broadly

we categorized the hearing loss as conductive,

mixed or sensorineural. The cases for ease of

documentation of hearing loss pattern were

divided into unilateral and bilateral cleft cases.

In the 31 unilateral cleft cases, 3 cases

had normal hearing as assessed on PTA.

Remaining 28 cases showed hearing loss on

the same side of the cleft while 24 showed

hearing loss on the opposite of the cleft (Table

54).

Amongst the bilateral cleft and CP

category, 29 had affected hearing, of which 24

ears on right side and 23 on left side had

conductive hearing loss (Table 55).

2. Status of middle ear function

Impedance audiometry is a versatile objective

technique to test the middle ear function as far

as sound conduction is concerned. The

versatility of this technique is highlighted by

the fact that the technique can be carried out in

infants as young as barely a few months. In

our setup, we used the Interaccoustics®

combined audiometry tympanometry unit to

assess the status of middle ear in the sample.

Standardized protocol for the testing was

followed and the middle ear function was

graded on the basis of the type of

tympanogram (Table 56). This test however,

can be done only in cases where the tympanic

membrane is intact with little or no cermuen

accumulation in the external auditory canal.

For ease of data presentation and

segregation, the data is presented separately

for unilateral and bilateral cleft cases with

special reference to UCLP and BCLP as these

were the largest groups. When the sample of

unilateral cleft cases was tested for the middle

ear function on the same side of cleft, it was

found that out of 97 cases; only 66 cases could

be assessed. Of the 66 cases, 38 cases (57.5%)

had normal middle ear function while 28

(42.4%) had affected middle ear function

(Table 57) on the same side as cleft. Of the

affected cases, 20 out of 28 cases (71.4%) had

Type B tympanogram indicating immediate

intervention by an otolaryngologist to prevent

further loss of hearing. For the opposite side

only 64 ears could be examined. Of the 64

ears, 40 cases (62.5%) had normal middle ear

function while 24 (37.5%) had affected middle

ear function (Table 57,58) on the opposite side

as cleft. Of the affected cases, 16 out of 24

cases (66.67%) had Type B tympanogram

indicating immediate intervention by an

otolaryngologist to prevent further loss of

hearing.

In bilateral cleft cases, 48 ears were

evaluated on each side. On the right side, 20

(41.6%) and 18 (37.5%) on right and left side

respectively had normal function (Table 58).

P. Speech assessment

Speech assessment in the sample was

comprehensive and consisted of assessment of

hypernasality, presence of articulation defects

in those with defective speech and assessment

of speech intelligibility.

Hypernasality of speech in cleft cases

occurs due to velopharyngeal incompetence

occurring in cleft patients because of

incompetency, inadequacy or mislearning of

the soft palate. Assessment of hypernasality

was done by a single observer (speech

pathologist) and the assessment was

subjective. The cases were rated only in 3

categories: hypernasal, normal or hyponasal

speech.



In the sample of 164 cases, 22 cases could

not be assessed due to non-cooperation. Of the

remaining 142 cases, only 27 cases had normal

nasality (19%) while the 114 out of 164

(80.3%) had hypernasality (Table 59, Graph

27). This lends credence to the fact that many

operated cases of cleft lip and palate have

hypernasality due to nasal escape of air during

phonation via either VPI or symptomatic

oronasal fistula. Unfortunately, the assessment

of the nasality was subjective and categorized

as present or absent, which overestimates the

true picture as even those with mild

hypernasality are also categorized as

hypernasal speech. However, the assessment

was done by a single operator and thus

operator bias was excluded from the equation.

1. Defects in speech articulation

Similar results were seen for articulation

defects in the sample. In our study, a detailed

speech analysis was done and articulation

defects in various categories of speech sounds

was evaluated. Of the 164 cases, 24 cases

could not be assessed due to young age.

Amongst the remaining 140 cases, 108

(77.2%) had an articulation defect ranging

from mild to severe problems (Table 60,

Graph 28). The results show that in UCLP,

BCLP and CP the articulation defects span

across all categories of speech sounds. In

UCLP and BCLP the major sounds affected

were palatal, retroflex, dental and alveolar

(Table 61).

2. Speech Intelligibility

Overall speech intelligibility is a subjective

evaluation of speech on the basis of the extent

of comprehensibility of the vocal speech of the

subject by the listener. Speech intelligibility is

a function of both articulation and nasality. In

our settings the assessments was done by a

single operator who was an expert in the field

of speech and language pathology. The

intelligibility ratings were standardised into 7

categories on the basis of pre-defined criteria

(Table 62). In our sample, a total of 139 cases

were evaluated out of 164 cases enrolled for

the report. In these 139 cases, the speech

intelligibility was normal in only 22 cases

(15.8%). However, majority of cases had only

mild speech intelligibility problems. If we

consider patients with I-0 to I-2 to be having

socially acceptable speech with only mild

intelligibility problems, a total of 70 out of 139

patients (50.3%) had clinically acceptable

speech. The remaining 49.7% cases had

clinically significant speech intelligibility

problems which would be detrimental to their

quality of life (Table 63, Graph 29).

Thus, it is evident that patients with

cleft anomaly tend to develop a multitude of

problems including surgical, dental,

orthodontic, hearing and speech, to name a

few. The findings of the pooled data recorded

from the three centres across the National

Capital Region (NCR) highlight the need for

improvement in the quality of cleft care. It is

clear that the outcomes in this sample of

patients are way behind those seen in some of

the good European centres like Copenhagen

and Oslo.

The current pilot project was primarily

aimed at establishing a protocol for a larger

multicentre study and its logistic operative

feasibility. The road ahead includes the

expansion of the project on a pan-India level,

with inclusion of multiple centres representing

the different regions of the nation. It is obvious

that to make this study truly representative of

the population, we would have to make

changes in the study design.



Table 52- Prevalence of hearing abnormalities in the sample as a function of type of cleft

Type of cleft Right ear Left ear Both Normal

CL L 0 1 0 8

CL R 0 0 0 1

CL B/L 0 0 1 0

CLA L 0 0 1 4

CLA R 0 0 0 1

CLA B/L 0 0 1 1

UCLP L 3 4 12 20

UCLP R 2 0 8 13

BCLP 1 2 14 17

CP 1 1 8 9

Total 7

(5.2%)

8

(6 %)

45

(33.6%)

74

(55.2%)

AIIMS-6 CNT; Safdarjung-13 CNT and Medanta- The MEDICITY- 11 CNT


Table 53- Degree of hearing loss in the sample

Type of cleft Mild

(1)

Moderate

(2)

Moderately

Severe

(3)

Severe

(4)

Profound

(5)

CL U/L 1 0 0 0 0

CL B/L 0 1 0 0 0

CLA U/L 0 1 0 0 0

CLA B/L 1 0 0 0 0

UCLP 20 8 1 0 0

BCLP 12 5 0 0 0

CP 10 0 0 0 0

TOTAL 44

(73.3%)

15

(25%)

1

(1.7%) 0 0




Table 54- Relation of hearing loss with different types of cleft – Unilateral Cleft

Type of cleft

Same side Opposite side

Conductive

(1)

Mixed

(2)

Sensorineural

(3)

Conductive

(1)

Mixed

(2)

Sensorineural

(3)

CL U/L 1 0 0 0 0 0

CLA U/L 0 1 0 0 0 1

UCLP U/L 23 0 3 19 1 3

Total 24 1 3 19 1 4

28 ears affected on same side and 24 ears affected on opposite side

Table 55- Relation of hearing loss with different types of cleft – Bilateral Cleft

Type of cleft

Right Left

Conductive

(1)

Mixed

(2)

Sensorineural

(3)

Conductive

(1)

Mixed

(2)

Sensorineural

(3)

CL B/L 0 0 1 0 0 1

CLA B/L 1 0 0 1 0 0

BCLP 15 2 0 14 2 0

CP 8 0 1 8 0 1

Total 24 2 2 23 2 2

* the value represents the ears and not the cases hence, the sample on right and left may not necessarily be same

Table 56- Impedence Audiometry and their inference

Type of Curve Inference Treatment

need

Type-A Normal middle ear function No

Type-B Negative Middle ear pressure with low compliance

High

Type-C Normal compliance but Negative

Middle ear pressure High

Type-Ad High compliance; normal middle ear pressure

Low

Type-As Normal pressure; low compliance Mild



Table 57- Status of middle ear based on Impedance

TYPE OF CLEFT

Same side Opposite side

Type-A Type-B Type-C Type-

Ad Type-

As Type-A Type-B Type-C

Type-Ad

Type-As

CL U/L 7 1 0 0 1 8 0 0 0 0

CLA U/L 4 0 0 0 0 4 0 0 0 0

UCLP 27 19 5 0 2 28 16 6 0 2

TOTAL 38 20 5 0 3 40 16 6 0 2

Table 58- Status of middle ear based on Impedance- Bilateral cleft

TYPE OF CLEFT

Right Left

Type-A Type-B Type-C Type-

Ad Type-

As Type-A Type-B Type-C

Type-Ad

Type-As

CL B/L 0 0 0 0 0 0 0 0 0 0

CLA B/L 2 0 0 0 0 1 0 0 0 1

BCLP 12 16 1 1 0 10 15 2 3 1

CP 6 7 1 0 2 7 6 1 0 1

TOTAL 20 23 2 1 2 18 21 3 3 3

Table 59- Distribution of the sample according to nasality of speech

Type of cleft Normal Hyper

nasality Hypo nasality

CL U/L 10 1 0

CL B/L 1 0 0

CLA U/L 7 0 0

CLA B/L 1 1 0

UCLP 7 59 1

BCLP 1 34 0

CP 0 19 0

TOTAL 27

(19%)

114

(80.3%)

1

(0.7%)

CNT-AIIMS-5 cases; Safdarjung-11 cases and Medanta- The MEDICITY-6 cases




Table 60- Speech Articulation in the sample

Type of cleft Affected Normal

CL U/L 2 9

CL B/L 0 1

CLA U/L 2 5

CLA B/L 1 1

UCLP 52 13

BCLP 33 2

CP 18 1

TOTAL 108 (77.2%) 32 (22.8%)

CNT-AIIMS-6 cases; Safdarjung-11 cases and Medanta- The MEDICITY-7 cases


Table 61- Status of affected articulation in the sample

Typ

e o

f

cle

ft

Pa

lata

l

Re

tro

fle

x

De

nta

l

La

bio

-

de

nta

l

Bil

ab

ial

Alv

eo

lar

Glo

tta

l

Ve

lar

Vo

we

ls

CL U/L 1 0 1 0 0 1 0 0 1

CL B/L 0 0 0 0 0 0 0 0 0

CLA U/L 0 0 0 0 1 1 0 0 0

CLA B/L 1 1 1 1 1 1 1 1 1

UCLP 46 34 35 7 25 45 6 18 20

BCLP 33 23 26 10 18 28 8 16 17

CP 14 16 13 3 11 16 4 8 15



Table 62- Description of speech sample

Point scale

0 Normal

1 Can understand without difficulty; however, feel speech is not normal

2 Can understand with little effort occasionally need to ask for repetition

3 Can understand with concentration and effort

specially by sympathetic listener

4 Can understand with difficulty and concentration by family but not others

5 Can understand with effort if content is known

6 Cannot understand at all even content is known

Table 63- Overall speech intelligibility in various cleft types

Type of cleft I-0 I-1 I-2 I-3 I-4 I-5 I-6

CL U/L 9 1 1 0 0 0 0

CL B/L 1 0 0 0 0 0 0

CLA U/L 6 1 0 0 0 0 0

CLA B/L 1 0 0 0 0 1 0

UCLP 5 21 11 12 3 10 3

BCLP 0 8 5 5 3 11 2

CP 0 2 3 4 4 4 2

TOTAL 22

(15.8%)

33

(23.8 %)

20

(14.4%)

21

(15.1%)

10

(7.2%)

26

(18.7%)

7

(5%)

CNT-AIIMS-7 cases; Safdarjung-11 cases and Medanta- The MEDICITY-7 cases n=139; Data represents no. of patients in each category


5 Conclusions

A total of 164 cases with cleft lip and palate

anomaly were recorded from three hospitals

involved in the project (55 from AIIMS, 54

from Safdarjung, 55 from Medanta- The

MEDICITY). At each of the centres, the

Departments of Plastic surgery, Orthodontics

and ENT were the major input holders for

support and coordination of the study. The

collection of the data was carried out by the

specifically designated Indicleft Team. The

“Indicleft team” included experts from various

medical specialties. It was divided into a

supervisory team (called investigators) and a

mobile team of research staff. The members of

the supervisory team were based in three

locations: AIIMS, Safdarjung and Medanta-

The MEDICITY hospitals. The Key

observations of the study included:

• Wide variation in age at primary lip (range

2 to 180 months) and palatal surgery (3 to

228 months) were noted.

• A significant percentage of cases required

lip and nose revision surgeries (36% and

35% respectively)

• Fifty five percent cases had a post-surgical

oronasal fistula

• A large proportion (77.5%) of the operated

UCLP cases had complex orthodontic

treatment needs.

• A high proportion of patients had hearing

defects (44.7%) and many of these also had

concomitant tympanic membrane

afflictions also (nearly 40 % cases), in one

or both the ears.

• Around fifty percent (49.7%) cases had

clinically relevant speech intelligibility

problems.

Thus, it can be concluded that in the sample of

cleft patients assessed in the project, the

treatment needs were significantly high. There

seems to be an urgent need to devise strategies

to improve the delivery of quality care with

joint efforts of all experts and health care

providers. It must be mentioned here that the

data is not representative of the outcomes of

the three centres.


6 Future Directions

The knowledge and experience gained

from the ongoing task force project of

ICMR to evaluate the treatment needs of

cleft patients will be served as the

foundation to conduct a nationwide

multicentric study consisting of at least

one centre in each representative region

of the country (Figure-9) with the

ultimate aim to enable the cleft-ridden

children to live anormal life by

initiating a National Registry for the

patients with congenital defects of the

face and jaws, and to establish strategies

that will address the multitude of

challenges associated with the

prevention and treatment of this

deformity. The study will lead to the

formulation of national-level guidelines

for the treatment of these birth defects.

The outcome will lead us the way for:

• Quantifying issues and problems in the

delivery of cleft care in India

• Planning strategies for the prevention

and reduction of this anomaly

• Planning and implementing

multispeciality quality care suited to

the Indian scenario

For the better understanding of etiology

of CLP, inclusion of a genome/exome-

wide scans using next-generation

sequencing in future multicentric study

will offer an immense opportunity to

potentially identify novel causative

variants associated with genetic diseases.

Understanding of the genetic basis CLP

will be helpful in taking effective

preventive measures.


Figure 9- Distribution of proposed centres in multicentre study


7 S&T benefits occurred

List of research publications with complete details

1. Kharbanda OP, Agrawal K, Khazanchi R,

Sharma SC et al. Clinical profile and

Treatment Status of subjects with Cleft

Lip and Palate anomaly in India: Report

of a three centre study. Journal of Cleft

Lip Palate and Craniofacial Anomalies

2014; 1:26-33.

2. Abstract of the Scientific poster titled

“Multicentric Collaborative

Interdisciplinary Research in Cleft Lip

and Palate: Experience from a Pilot

Study” at the 12th International Congress

on Cleft Lip and Palate and related

craniofacial anomalies, 5-10 May,

Florida, USA, 2014.

3. OP Kharbanda, 13th Annual Conference

of Indian Society of Cleft Lip, Palate and

Craniofacial Anomalies

(INDOCLEFTCON 2014)” Lucknow,

India from 14th to 16th February 2014.

4. Parul Rathod, Speech outcome among

Indicleft children 13th Annual Conference

of Indian Society of Cleft Lip, Palate and

Craniofacial Anomalies

(INDOCLEFTCON 2014)” Lucknow,

India from 14th to 16th February 2014.

5. Abstract of scientific paper titled

“Multicentric Collaborative

Interdisciplinary Research in Cleft Lip

and Palate: Experience from a Pilot

Study” at the International

Comprehensive Cleft Care Conference in

Guwahati-Assam, India, 9-10th Nov 2013.

Abstract

6. Abstract of scientific paper titled “Timing

of surgery and dental arch relationships in

patients with UCLP anomaly: preliminary

results from a multicentric study in India”

at the Indian Society for Dental Research,

26th Annual conference, 3rd – 6th

October 2013, New Delhi.

7. Abstract Parul Rathod – Approach to

speech in cleft palate patients GOLDEN

JUBILEE CELEBRATION: SafPlastCon,

Department of Burns, Plastic &

Maxillofacial Surgery, Safdarjung

Hospital and VMMC, New Delhi. March,

2013.

8. Abstract of the Scientific presentation

titled “Timing of surgery and dental arch

relationships in patients with UCLP

anomaly: preliminary results from a

multicentric study in India” at the

Indocleftcon 2013 (12th annual

conference of ISCLPCA) at Nagpur,

Maharashtra, 17-20 Jan 2013.


Procurement/usage of equipment S.

No. Name of Equipment Make/Model

Cost

FEE/Rs

Date of Installation

Utilization rate %

1. Impedance audiometry unit

Interaccoustics, USA 4,41,000 07-08-2013 100%

2. Computer desktop HP Compaq 8200 Elite Small Form Factor

43, 877

19-06-2012

100%

3

Canon DSLR camera EOS 700D (18-55mm) 54,900 09-12-2013 100%

4

Canon Macro Ring

Lite

MR-14Ex

33,495

09-12-2013

100%

5.

Canon DSLR camera

With macro ring flash

LP-E8 & Remote

EOS 700D (18-55) IS STM

(MR – 14EX)

95,629 06-11-2013 100%

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