DEVELOPMENTAL DISORDERS

•Cerebral Palsy•Autism•Cleft Palate/Lip

Presented by,Forum. Thakker

Nisha . Salian

CEREBRAL PALSYCerebral palsy (CP) is an umbrella term for a

group of disorders affecting body movement, balance, and posture.

Cerebral refers to the brain

Palsy refers to a physical disorderInfants are usually slow to reach

developmental milestones such as rolling over, sitting, crawling, and walking.

BRAIN STRUCTURE

Types of Cerebral PalsyClassification of CP is related to the type of

movement disorder and/or by the number of limbs involved

The location of the brain injury will determine how movement is affected.

Causes of Cerebral PalsyDamage to certain parts of

the developing brain. Injury during child birth, if

the baby got stuck in the birth canal with no oxygen supply.

In premature infants it can be caused by bleeding in the brain, brain infections such as encephalitis, meningitis, and herpes simplex.

Head injuries.Sever jaundice.Medical negligenceMany times it is unknown

SymptomsDelayed milestonesAbnormal muscle

toneAbnormal

movementsSkeletal deformitiesJoint contracturesMental retardationSeizures

Speech problemsSwallowing

problemsHearing lossVision problemsDental problemsBowel and/or

bladder control problems

TerminologiesScoliosis— Scoliosis is an abnormal curving of the spine. The spine might look

like the letter “C” or “S.”

Malrotation—Intestinal malrotation is twisting of the intestines (or bowel) caused by

abnormal development while a fetus is in utero, and can cause obstruction.

Intestinal Volvulus--Intestinal volvulus is defined as a complete twisting of a loop of

intestine around its mesenteric attachment site.

Sialorrhea—An excessive flow of saliva.

Other ComplicationsGI issuesThe underlying neurologic impairment in

cerebral palsy can affect the gastrointestinal system, most notably oral-motor function and motility (especially colonic, which typically results in constipation).

THE BRAIN-GUT AXIS

Dysphagia/Oral Motor DysfunctionDue to history of feeding difficulties, extended feeding

times, malnutrition, and/or a history of aspiration pneumonia

Medications used to reduce muscle tone can also increase dysphagia risk

DysmobilityPresent in several forms: Dysphagia, Gastroesophageal Reflux Disease (GERD), Delayed Gastric Emptying and Dumping SyndromeChildren with severe gastroesophageal reflux that is

unresponsive to medical therapy may require a surgical antireflux procedure (ARP)

ConstipationDysmotility, hypotonia, medications, and

nonambulation contribute to constipation.Ensuring adequate fluid intake prior to increasing

fiber intake can help prevent additional problems with constipation.

Pulmonary aspirationThe result of swallowing dysfunction with aspiration

of saliva and/or aspiration of gastric contents.

Spinal AbnormalitiesScoliosis progression can result in diminished gastric

capacity, GERD, difficulty in positioning for feedings, and changes in motility that can cause early satiety, nausea, and vomiting.

Bone HealthOsteopenia and osteoporosis are frequently

encountered due to Reduced ambulation and weight-bearing

activity,Malnutrition, Limited sun exposure and The use of anticonvulsant medication, which

alters vitamin D metabolismOsteoporosis should be prevented with an

adequate intake of Dietary Calcium, Phosphorus and Vitamin D.

Nutritional Assessment of Children with Cerebral Palsy

Medical issues, such as wounds, illness, or surgery, are typically the primary focus in the acute care setting.

The ultimate goal in nutritional intervention should be to optimize health, fitness, growth, and function in individuals with cerebral palsy

In clinical practice, the Subjective Global Assessment technique is not a good assessment tool for most individuals with disabilities, as the body habitués is not considered in this assessment.

A physical assessment is important to identify signs and symptoms of dehydration and malnutrition.

Anthropometric MeasurementsPhysical examination: The physical examination should detect signs of

malnutrition Triceps skinfold thickness and mid-arm circumference Head circumference lower leg length or upper arm lengthScoliosis, increased or decreased muscle tone should

be assessedAuscultation of the lungs may reveal signs of chronic

aspiration. Abdominal examination and, if needed, a rectal

examination, may reveal constipation.

Stature Measurement:Changes due to scoliosis, spasticity,

contractures, and/or limb differences may even make the individual appear to “shrink”

Knee height, Upper arm length, Recumbent length, or Tibial lengths are techniques used to estimate stature

Weight:Weight does not reflect the typical

distribution of body fat and muscle.Fat stores are typically depleted and muscle

stores are lowAlterations in body composition should be

considered when estimating energy needs

Clinical AssessmentNutritional history: • The type (purees, liquids and solid food) and the

amount of food.• If the child is able to self-feed, the amount of spilling

should be assessed.• Signs of oromotor dysfunction

Medical history: An assessment of gastroesophageal reflux symptoms

such as emesis, regurgitation, pain or food refusalChronic respiratory problems, recurrent pneumonia and

respiratory symptoms suggestive of chronic aspirationsRecurrent infections, decubitus ulcers and constipationA review of the child’s medication

Growth history: Birth weight, length and head circumference

and all previous weight, length and head circumference measurements

It helps determine whether there is a decrease in growth velocity.

Investigation:Extensive blood work is usually not necessary.A complete blood count may help detect iron

deficiency. Serum albumin may reflect nutritional status,

but is not very reliable in this population. Electrolytes are usually normal. Phosphorus, calcium, alkaline phosphatase and

vitamin D levels may be measured in patients with suspected osteoporosis and may be combined with a bone density scan.

A gastric emptying scan is useful in diagnosing delayed gastric emptying and possibly pulmonary aspiration of gastric content.

Nutritional ManagementEnergy

Krick method Kcal/day = (BMR × muscle tone factor × activity factor) + growth factorMuscle tone factor: 0.9 if decreased, 1.0 if normal, 1.1 if increasedActivity factor: 1.15 if bedridden, 1.2 if dependant, 1.25 if crawling, 1.3 if

ambulatoryGrowth factor: 5 kcal/g of desired weight gain  Height-based method14.7 cal/cm in children without motor dysfunction13.9 cal/cm in ambulatory patients with motor dysfunction11.1 cal/cm in nonambulatory patients

Resting energy expenditure-based method 1.1× measured resting energy expenditure

The effect of medications on energy expenditure is important to consider

ProteinProtein needs are estimated using the RDA and

actual weight or appropriate weight for height .

There are no guidelines for estimating protein needs of individuals with disabilities under stress such as surgery.

Protein intake has been increased up to 1.5–2 g/kg/day in clinical practice for presurgical/postsurgical planning and wound healing with normal renal status.

Fluid Requirements

Fluid loss through Sialorrhea or sweating.

Actual body weight is used to estimate fluid needs using the Holliday-Segar equation

Weight Calculation

1-10 Kg 100 ml/kg

10-20 Kg 1000 ml + 50 ml/kg for each kg

>20 Kg 1500 ml + 20 ml/kg for each kg

Vitamin D

Shinchuk and Holick recommend supplementation if 25(OH)-D levels are less than 30 ng/mL

The recent Institute of Medicine (IOM) report recommends supplementation only when 25(OH)-D levels are less than 20 ng/ml.

25(OH)-D levels should be checked every three months until levels are within normal range

Feeding and Feeding RegimenImprove oral intakeAdequate positioning of the child during mealsFood consistency may be adjustedFood caloric density may be increasedOral intake can be maintained as long as there is no risk of

aspirationEnteral nutritionBefore 12 months of age, an infant formula should be usedPediatric 1 kcal/ml formula is preferred. A 1.5 kcal/ml formula

may be used with careful monitoring of hydration status.Feeding regimen: The choice of feeding regimen will be based on The child’s enteral access Activities, Caloric needs and Tolerance to feeds.

ConclusionMalnutrition should not be considered

normal in CP children.

Nutritional intervention should be provided by a multidisciplinary team of professionals to ensure adequate growth, improve quality of life and optimize functional status.

Early nutritional intervention, appropriate support and continuing follow-up are necessary to ensure success.

Autism Spectrum Disorder (ASD)The term autism spectrum disorders (ASD)

has been used to describe their variable presentation.

Prevalence studies indicating that they are present in 6 per 1000 children.

These disorders are characterized by three core deficits:

Impaired communication, Impaired reciprocal social interaction and

restricted,Repetitive and stereotyped patterns of

behaviors or interests.

Pervasive Developmental Disorders (PDD) is a group of neuro-developmental disorders characterized by impairments in communication, reciprocal social interaction and restricted repetitive behaviors or interests.

The term autism spectrum disorders (ASD) has been used to describe their variable presentation.

Listed in the Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition (DSM-IV) and the International Classification of Diseases, Tenth Edition (ICD-10); ASD is used to describe 3 of the following 5 PDD--

Autistic disorder, Asperger disorder, Pervasive Developmental Disorder-Not

Otherwise Specified (PDD-NOS),Rett Syndrome andChildhood Disintegrative Disorder.

Autistic DisorderQualitative abnormalities in social

interactions, Markedly aberrant communication skills, restricted repetitive and stereotyped

behaviors, and Moderate mental retardation with

intelligence quotients (IQs) of approximately 35-50.

Three fourths of autistic children function in the mentally retarded range.

Asperger’s SyndromePersistent impairment in social interactions

and by repetitive behavior patterns and restricted interests.

No significant aberrations or delays occur in language development or in cognitive development.

Generally evident in children older than age 3 years and occurs most often in males.

Severe social impairment is associated with this condition.

Pervasive Developmental Disorder- Not Otherwise Specified (PDD-NOS)

Describes a child aged 9 years with poor peer interactions, normal verbal abilities, and mild nonverbal disabilitiesThe mild nonverbal disabilities make it

difficult for the child to follow subtle social cues that most children easily interpret as anxiety, anger, or sadness.

Rett SyndromeOccurs almost exclusively in females.

The specific mutation on the gene related to Rett syndrome (methyl-CpG binding protein-2 [MECP2]) was identified late in 1999.

Initially have seemingly healthy development with low tone and subtle slowing of development.

Early clinical feature is deceleration of head growth that begins when the individual is aged 2-4 months.

Individuals who are less severely affected may tolerate or even prefer interpersonal contact, show affection to others, and suffer from learning disabilities and speech fragmentation related to breathing irregularity.

Childhood Disintegrative DisorderAlso known as Heller syndrome.Characterized by a loss of previously

acquired language and social skills and results in a persistent delay in these areas.

Children develop normally through age 3 or 4.

Social and emotional development also regresses, resulting in an impaired ability to relate with others.

No single causative factor for childhood disintegrative disorder has been

identified.

AUTISMAutism is a complex developmental disorder

that has the following three defining core features:

Problems with social interactionsImpaired verbal and nonverbal

communicationA pattern of repetitive behavior with narrow,

restricted interests The diagnosis of autism may not be made

until a child reaches preschool or school age.The behavioral characteristics of autism are

almost always evident by the time the child is aged 3 years

EPIDEMIOLOGYASD occurs more often in boys than girls,

with a 4:1 male-to-female ratio.

The reported prevalence rates of autism and its related disorders have been increasing worldwide from approximately 4 per 10 000 to 6 per 1000 children.

The possibility that the increase in the reported cases is a result of unidentified risk factor(s) cannot be ruled out

ETIOLOGYThe exact cause of autism and the other

ASDs is still not known. The etiologic theories have changed over the

years.

Obstetric complications Mothers who experienced diabetes,

hypertension, or obesity during pregnancy are more likely to have children with autism spectrum disorders and other neuro-developmental disabilities.

An infectious basis The large number of children with autistic disorder

born to women who were infected in the rubella epidemic.

Also Cytomegalovirus infection is said to be associated with development of autism.

Genetic factorsAutism is both familial and heritable.1. The recurrence rate in siblings of an autistic child

is 2% to 8%2. Monozygotic twins have a higher concordance rate

than Dizygotic twins—90% and 10%, respectively.3. Fragile X syndrome is identified to be associated

with Autism.

Environmental factors1. Prenatal infections with rubella and

cytomegalovirus.2. The role of heavy metals in the etiology of

autism is controversial.3. Exposures to toxins, chemicals, poisons, and

other substances have been hypothesized to cause autism.

HypothesisThe potential adverse effect of the measles,

mumps, rubella (MMR) vaccine andThimerosal, a mercury-based preservative used

in some vaccines.

DiagnosisThe severity of these impairments varies

significantly among children with ASD.The impairments can be subtle and may not

be detected before school age.Some of the widely used instruments for

screening these high-risk children.1. The Checklist for Autism in Toddlers

(CHAT), 2. Modified-Checklist for Autism in Toddlers

(M-CHAT), 3. Childhood Autism Rating Scale (CARS)

Specific tools commonly used in the assessment include the

Autism Diagnostic Interview-Revised (ADI-R) and

The Autism Diagnostic Observational Schedule (ADOS).

Currently, there are no laboratory or radiologic tests to diagnose ASD.

FEEDING ABNORMALITIESRestricted range of foodsUnusual eating behaviors such as food

cravings and pica.Higher instances of food selectivity by typeSelectivity by type was defined as “eating a

narrow range of food that was nutritionally inappropriate eating only a few different foods and often [refusing] to eat entire food groups

Two types of physiological issues that can directly or indirectly impact feeding skills and/or behavior:

1) Sensory processing issues

2) Gastrointestinal (GI) issuesSensory modulation:•Sensory modulation allows an individual to appropriately filter the multitude of sensory information that constantly bombards the nervous system.• Dysfunction-hyperresponsivity, hyporesponsivity, and/or fluctuating responsivity resulting in atypical responses such as sensory seeking or sensory avoidance behaviors.

Sensory processing difficulties that both directly and indirectly impact eating processes :-• abnormal responses to taste and smell• heightened sensitivity to tactile input • auditory filtering problems

•Gastrointestinal alterations:• Ileal lymphoid nodular hyperplasia (LNH) ,•Mucosal abnormality •Low cho enzymes•Increased exocrine secretions of pancreas•Intestinal mucosa is abnormally permeable•Increase in immunoglobins levels

Pathways:

•Antigens in the diet•Thimerosal-increased ionic conductance and permeability•Elevated levels of VIP-impair gut motility, development and secretion•Reduced secretin -the reduced blood levels of secretin could allow gastric HCl secretion to increase abnormally, elevate intestinal permeability

G.I. issues: •GI disorders include gastroesophageal reflux disease (GERD) and constipation, diarrhea, or other symptoms resulting from food allergies.•Difficulty expressing their discomfort and/or correctly identifying its source. •Difficulty with self-monitoring during the meal may also affect the child’s ability to complete the meal •This would be more likely if the child with ASD does not connect the internal feeling of hunger with the consumption of food.•Children with ASD appear to eat based on external stimuli such as the time on a clock or the presence of food rather than on feelings of hunger

If a child’s appetite regulation is impaired or is unconnected to food consumption, it could force the child to use other methods to monitor food intake (e.g., visual appearance of the amount of food left on the plate; amount of time spent at the table, etc.); this, in turn, could lead not only to difficulty judging when mealtime is finished, but also to over- or undereating.

ALTERATION IN METABOLISM:

•Decreased sulphate levels( Sulfation is important for many reactions including detoxification, inactivation of catecholamines, synthesis of brain tissue, sulfation of mucin proteins which line the gastrointestinal tract.•Increased oxidative stress-indicated by high levels ofGSSG•Decreased NADPH, ATP(may be due to mitochondrial dysfunction) •Decreased SAM (impaired methylation)• Primary amino acids: elevated glutamate- Overstimulation leads to excitotoxicity, creating oxidative stress, mitochondrial damage and may play a role in neurodegeneration

•Decreased tryptophan may be due to decreased protein intake, and/or impaired digestion of protein into amino acid. Tryptophan is a precursor to the synthesis of serotonin, so decreased tryptophan is likely to impair serotonin synthesis (neurogenesis and neurotransmission)

REMOVING CASEIN AND GLUTEN FROM DIETImplementation of a strict casein- and gluten-free

(CFGF) diet leads to symptomatic improvement individual, improvement in social cognitive and communication skills.

Mechanism: During digestion, pre-opioid type compounds from casein and gluten in the diet are activated because of an incomplete breakdown of proteins.

These exorphins (i.e.caseomorphins and gluteomorphins) are then absorbed into the circulation where they exert an opioid- type action on the brain.

•Transfer of peptides across the lumen of the gut is thought to occur due to the ‘leaky’ nature of the gastrointestinal tract .• Caseomorphins and gliadomorphins are potent psychosis inducing factors.•Elimination is a two step process:•The first phase is removal of casein via removal of milk and other dairy products.•Benefits seen in 2-3 days in children and 10-14 days in adults.•Symptoms linked to casein intake include projectile vomiting; eczema, particularly behind the knees and in the crook of the elbow; white bumps under the skin; ear discharges and infections; constipation, cramps, and/or diarrhea; and respiratory disorders resembling asthma.

•Gluten exclusion requires the removal of several common cereals from the diet, wheat, barley, rye, and oats.

• Elimination process usually takes a minimum of 3-4 weeks, and a trial period of three months is appropriate.

•Once the main sources of food intolerance – casein, gluten, and gliadin – are removed from the diet, other foods may emerge as sources of symptoms.

• Parents can keep food diaries, to associate the child’s consumption of a particular food with deterioration in behavior, sleep patterns, or performance.

VITAMINS:Vitamin B6 and Magnesium:Vitamin B6:•Metabolic pathways of neurotransmitters, including serotonin, gammaamino- butyric acid (GABA), dopamine, epinephrine, and norepinephrine. •Study: Each child received vitamin B6 at a dose of 2.5-25.1 mg/kg body weight/day (75-800 mg per day). •Benefits seen from this trial, included better eye contact, less self-stimulatory behavior, more interest in surroundings, fewer tantrums, and better speech.•Magnesium:Required for a wide range of enzyme-catalyzed metabolic pathways.

•An intake threshold for achieving benefit may be approximately 200 mg vitamin B6 (as pyridoxine) and 100 mg magnesium per day for the 70 kg individual.

Folic Acid:•Folic acid is essential to numerous metabolic pathways.

•Favourable results on several non-fragile X autistic children by giving relatively large doses of folic acid (0.5- 0.7 mg/kg/day).

Vitamin C:•Vitamin C involved in a plethora of metabolic, antioxidant, and bio-synthetic pathways, and as a cofactor for certain enzymes necessary for neurotransmitter synthesis.

•In a double-blind trial for 30 weeks, vitamin C (8 g/70 kg body weight/day) improved total symptom severity and sensory motor scores .

Vitamin A:•Vitamin A is especially important for cell growth and differentiation, especially in epithelial tissues of the gut, brain•Core autism symptoms, such as language, eye contact, ability to socialize, and sleep patterns, were consistently improved .

Zinc:•Zinc is needed for the development and maintenance of the brain, adrenal glands, GI tract, and immune system.

•Serotonin synthesis relies on zinc-activated enzymes and for antioxidant enzyme activity and other proteins important for growth and homeostasis, cognition.

Lithium: low levels are seen,supplementation improved mood stabilization.

ESSENTIAL FATTY ACIDS:

•Essential fatty acids (EFAs) function as homeostatic constituents of cell membranes, helping to relay signal information from outside the cell to the cell interior and are precursors to eicosanoids that influence other cells, similar to hormones.

•Essential fatty acids, particularly the omega-3s, are deficient in other neurodevelopmental disorders.

CARNITINE: is an amino acid indispensable for energy generation.• Valproate, a drug prescribed for seizures, is known to deplete carnitine.• Constipation and self-abuse decreased while mood improved.BIOPTERIN:•Biopterin, in its reduced form (5,6,7,8- tetrahydrobiopterin, R-BH4), is a limiting factor for the biosyntheses of dopamine, epinephrine, and serotonin. •Autistic children, particularly those six years or younger, can have relatively low R-BH4 in their cerebrospinal fluid (CSF) and abnormally high urine R-BH4, indicating increased loss from the body. •Also, the enzyme (dihydropteridine reductase) that recycles biopterin into its biologically active reduced form, R-BH4, is lower in autism.

INOSITOL: is a precursor for the synthesis of phosphatidylinositol (PI), a phospholipid that is part of a complex cellular transmission pathway that facilitates serotonin receptor function.

CLEFT LIP /PALATECLEFT LIP Craniofacial malformation An opening in the upper lip between the

mouth and the nose... it can range from a slight notch in the coloured portion of the lip to complete separation in one or both sides of the lip extending up and into the nose.

CLEFT PALATE:“the roof of the mouth is not joined completely ranging from just an opening at the back of the soft palate to a nearly complete separation of the roof of the mouth (soft and hard palate). CLEFT LIP PALTE:Infants are born with a cleft lip and palate (i.e. cleft lip + palate). This type of cleft extends from the lip to the hard or soft palate

• CLEFT LIP occurs when an epithelial bridge fails, Due to lack of mesodermal delivery and proliferation. CL usually occurs at the junction between the central and lateral parts of the upper lip on either side. The cleft may affect only the upper lip, or it may extend more deeply into the maxilla and the primary palate. (Cleft of the primary palate includes CL and cleft of the alveolus.)

• If the fusion of palatal shelves is impaired also, the CL is accompanied by CP, forming the CLP abnormality.

• In general, patients with clefts have a deficiency of tissue and not merely a displacement of normal tissue.

ETIOLOGY:•Family History: Cleft lip more likely to be inherited than cleft palate•Race: More common in Native American, Hispanic & Asian patients•Sex: Males 2x as likely to have cleft lip; Females 2x as likely to have cleft palate•Environmental factors: exposure of fetus to alcohol, cigarette smoke, or drugs•Maternal Nutrition Deficiencies:especially lack of folate.

DIAGNOSIS: USG, Genetic testing, physical examination

SUCKING PERFORMANCE AND FEEDING PROBLEMSCleft of lip :Without adequate closure around

the nipple, the infant may have problems producing a suck powerful enough to extract milk from the breast or bottle nipple

Cleft of the lip and palate will usually result in an inability to form a complete seal, and negative air pressure cannot be generated .

EXTENSIVE FEEDING PROBLEMSNasopharyngeal reflux, choking, prolonged feeding

time, and slow or little weight gain, inadequate nutritional intake.

Feeding is an immediate concern due to the delay in growth of children born with clefts. This can be a major concern for infants who will be undergoing surgery to correct their cleft

A primary feeding concern associated with cleft palate is the formation of negative air pressure, necessary for adequate swallowing Without negative air pressure, a swallow cannot be properly triggered and aspiration or choking may occur.

•Recurrent aspiration will lead to respiratory infections including pneumonia and even death.•Signs and symptoms -inefficient or ineffective suck, and excessive air intake. This excessive air intake may result in choking and/or gagging.• Infants witth clefting of the hard palate may limit the normal use of the tongue to compress the nipple.• If the soft palate is not intact when it is elevated it does not create a barrier in which food and liquid cannot pass through the nasopharynx.• Signs and symptoms associated with the inability to seal off the nasal cavity include: nasal reflux, and inefficient or ineffective suck

•The impact of a cleft is not necessarily restricted to the oral cavity. There may be airway deficits due to a cleft palate

• Clinical signs or symptoms associated with an upper airway obstruction are: Inspiratory stridor Glossoptosis Micrognathia• Clinical signs or symptoms related to neurological impairments coinciding with a cleft include: Incoordination of suck Swallow and respiratory sequenceHypotonicity and hypertonicity

DIFFERENT FEEDING TECHNIQUESARTIFICIAL NIPPLES:Infants with isolated cleft palate may benefit

most from being fed using crosscut nipples. Artificial nipples with large holes are

recommended so that infants may passively receive a bolus of milk where a cleft palate prevents them from extracting it independently from the bottle

•Advantage: Faster feeding times, less vomiting, satisfactory weight gain, and parental acceptance for infants.

•Disadvantage:The rapid flow may imperil the infant’s ability to synchronize sucking, swallowing, and breathing if milk is delivered directly to the pharynx.

COMPRESSIBLE BOTTLES: •Compressible bottles allow the feeder to deliver milk to the infant who is unable to generate suction and extract fluid independently. Gentle squeezing of the plastic bottle forces formula from the tip of the nipple avoiding necessity of negative pressure created by sucking.• Advantage:easier to use for the feeding of babies born with cleft conditionthe feeder is able to control the amount of milk expelled into the infant’s mouth.•Disadvantage:No effect on child’s weight and growth

HABERMAN FEEDER:The Haberman feeder is specifically designed for cleft lip +/- palate use. Its elongated nipple can be compressed if the infant has difficulty in applying adequate negative pressure. Advantage:1) it has flow lines on the nipple that assist in helping the infant achieve optimal flow from the nipple.2) flow can be monitored without the necessity of squeezing the bottle. 3) Valve that prevents back flow, which reduces the excessive air buildup. The excessive airbuild up can cause uncomfortable gas and stomach problems as well as burping.

BREAST FEEDING AND PROSTHESES:

•Feeding obturators are passive devices designed to provide a normal contour to the cleft alveolus and hard palate. They separate the oral and nasal cavities and in doing so provide a surface to appose the nipple during suckling.

• Feeding device is inserted over the infant's hard palate, which allows him or her to compress the nipple easier because it provides a contact point and helps the infant to express milk.

•Advantage:Reduced choking, nasal discharge, and bottle feed duration and improved parental confidence in their sample of infants with cleft lip and palate who were prescribed feeding obturators. . It facilitates feeding, reduces nasal regurgitation and shortens the length of time required for feeding

•Disadvantage:A feeding appliance can be costly and needs to be replaced as the child grows to fit his or her mouth. Oral hygiene is also a concern because it is a plastic appliance, which can cause irritation to the palate.

•However,Strong (Level I) evidence show that obturators do not facilitate feeding or weight gain in breastfed babies with a CLP, and that they do not improve the infant’s rate of bottle feeding. •There was moderate evidence (Level II-2)obturators do not facilitate suction during bottle feeding.This is because obturators do not facilitate complete closure of the soft palate against the walls of the throat during feeding.

Position ,pacing and other care•Infants should be in an upright position with good head neck and trunk support.

•Feeding times should be limited so that infants do not experience hunger and unsatisfactory feeding.

•Burping the infants after feeding

•Oral care after feeding- one can use clean water, or water with hydrogen peroxide

TRANSITION TO SOLID FOODSSpoon feeding for infants with a cleft lip +/-

palate should begin at approximately six months of age.

Strained, thin pureed, foods should not be a problem for infants with clefts

Avoid thickened foods to ensure that these consistencies do not get lodged in the cleft area .

PREOPERATIVE CARE:•Assess fluid and calorie intake daily.•Assess weight daily (same scale,same time, with infant completely undressed).• Observe for any respiratory impairment.• Provide 100–150 cal/kg/day and 100–130 mL/kg/day of feedings and fluid. • Facilitate breastfeeding.•Hold the infant in a semisitting position.•Give the mother information on breastfeeding the infant with a cleft lip and/or palate such as plugging the cleft lip and eliciting a let-down reflex before nursing.

BENEFITS OF BREAST MILK IN INFANTS WITH CL/CLP

Feeding with breastmilk protects against otitis media in infants with a CP

Babies are more prone to otitis media than the general population due to the abnormal soft palate musculature.

Moderate to weak evidence that breastmilk can promote intellectual development and school outcomes in babies with clefts.

Antibacterial agents in breastmilk promote postsurgical healing and reduce irritation of mucosa.

Breastfeeding facilitates the development of oral facial musculature speech,bonding, and pacifying infants postsurgery

POST-OPERATIVE CARE:•CL repair (cheiloplasty) is generally carried out within a few months of birth and CP repair (palatoplasty) takes place between 6 and 12 months of age.•Maintain intravenous infusion as ordered.•Begin with clear liquids, then give half-strength formula or breast milk as ordered.•Give high-calorie soft foods after cleft palate repair•(Levels I and II-2) It is safe to commence/recommence breastfeeding immediately following CL repair and moderate evidence (Level II-2) for initiating breastfeeding 1 day after CP repair. •There is strong evidence (Level I) that breastfeeding immediately following surgery is more effective for weight gain, with lower hospital costs, than spoon feeding.

BIBLIOGRAPHY: Adams.J. Nutritional and metabolic status of children with autism vs.

neurotypical children, and the association with autism severity. Nutrition & Metabolism 2011, 8:34

Agarwal.A. A feeding appliance for a newborn baby with cleft lip and palate.Natl J Maxillofac Surg.2010;Jan-Jun(1):91-93.

Goplakrishna.A. A status report on management of cleft lip and palate in India.Indian J Plastic Surgery 2010;43(1):66-75

Hooper B. Feeding interventions for growth and development in infants with cleft lip, cleft palate or cleft lip and palate (Review). Cochrane Database of Systematic Reviews 2011, Issue 2.

Julie Reid.A review of feeding intervention in infants with cleft palate.Craniofacial J.2004;41:268-277

Justine Ashby.Feeding therapy and technique for children with cleft lip/palate.Southern Illinois University,2009

Kidd P. Autism, An Extreme Challenge to Integrative Medicine. Part II: Medical Management. Alternative Medicine Review .2002;7(6):472-499.

Reilly S. Guidelines for Breastfeeding Infants with Cleft Lip, Cleft Palate,or Cleft Lip and Palate.Breastfeeding medicine.2007;2:243-250

Twatchmann J. Addressing Feeding Disorders in Children on the Autism Spectrum in School-Based Settings: Physiological and Behavioral Issues. LANGUAGE, SPEECH, AND HEARING SERVICES IN SCHOOLS .2008;39:261–272

White J Intestinal Pathophysiology in Autism.Experimental Biology and Medicine 2003, 228:639-649.