clinical aspects of cleft lip repair

Ahmed Atef, Msc, MRCSSpecialist of plastic surgeryMataria Teaching Hospital

Clinical Aspects of Cleft Lip Repair

Objective

• Epidemiology • Embryology• Surgical Anatomy • Classification• Management• Future

Epidemiology

Epidemiology

Cleft lip / Cleft Palate is the the most common craniofacial malformation

Second most common congenital defect

The overall incidence is 1 in 1,000 live births.

White : 1 in 750 live births.

Asian : 1 in 500 live births.

African : 1 in 2,000 live births.

Epidemiology

Male-to-female ratio of 2:1.

The ratio of left (L) to right (R) to bilateral (B) clefts (L:R:B): 6:3:1.

The ratio of CLP to CL is 2:1.

Three percent are syndromic.

Epidemiology

Assosiated Syndromes:a. Van der Woude’s syndrome

b. Trisomy 13 (Patau syndrome)

c. Trisomy 21 (Down syndrome)

Epidemiology

Embryology

Embryology

Development of the face begins in the fourth week in utero, when neural cells migrate and fuse with mesodermal elements to form the facial primordium.

It results from the fusion– Two mandibular

processes– One frontonasal process– Two maxillary processes Ends in 8th week

Embryology

Embryology

• The critical developmental period of the lip and primary palate occurs during weeks 4 to 6 of gestation.

• Cleft lip is caused by failure of union between medial nasal process and maxillary prominence

Embryology

Surgical Anatomy

Anatomic subunits

Surgical Anatomy

Surgical Anatomy

Normal measurements.

a. Vertical length (height) of the upper lip.

(1) Newborn: 10 mm.

(2) Age 3 months: 13 mm.

(3) Adult: 17 mm.

b. The distance between the peaks of Cupid's bow: Approximately 3 mm at 3 months.

Surgical Anatomy

a. Orbicularis oris.

(1) Fibers cross (decussate) in the midline

and create the opposite philtral columns.

(2) Functions as a sphincter (deep fibers)

and for speech (superficial fibers).

b. Levator labii superioris.

(2) Inserts into the dermis at the vermilion

and the lower edge of the philtral columns.

(2) Elevates the upper lip.

Surgical Anatomy

Arterial blood supply: The labial

artery, bilaterally.

Motor innervation: The facial nerve,

CN VII, zygomatic and buccal

branches.

Surgical Anatomy

• Non-cleft side Shortened philtral

height Short columella2/3 cupids bow, 1

philtral column, 1 dimple preserved

Muscle b/w midline & cleft atrophic

Morbid Anatomy

• Cleft side Premaxilla outwardly rotated Unilat shortness of height

columella Philtrum short Orbicularis disrupted and ends

in margin of cleft

Morbid Anatomy

• Nasal deformity Lower lat cartilage attenuated,

medial crus lower in columella, dome lower than normal side

Lateral segment flat, spread across cleft at obtuse angle

Alar base rotated outward in flare

Alar rim distorted by skin without cartilage support.

Vestibular lining deficient

Morbid Anatomy

Alterations in the orbicularis oris and levator labii.

result in disruption of continuity, orientation, and quality of the muscles.

a. Fibers are disoriented and run parallel to the cleft margin.

b. Fibers insert into the alar base on the cleft (lateral) segment and into the columella in the

noncleft (medial) segment, as well as intradermally.

c. Incomplete clefts.

(1) Simonart's band consists of a skin bridge across the nasal sill. It does not usually contain any

significant muscle mass.

(2) Some fibers may cross the cleft, if the cleft is less than two-thirds of lip height.

d. Bilateral complete clefts: No muscle tissue is present in the prolabium.

Morbid Anatomy

Classification

Classification

Striped Y by Kernahan 1971 Millard modification

Complete cleft lip

a. Complete disruption of the

soft tissues to the nasal floor.

b. Tends to be wide, with

greater nasal deformities.

Incomplete cleft lip

Microform cleft lipThree characteristic elements:

- Vermilion notch - Band of fibrous tissue from edge

of red lip to nostril floor- Deformity of ala on notch side

• Bilateral Complete cleft lip

• Bilateral incomplete cleft lip

Managment

Goals of repair

The basic goal of primary cleft lip repair is to reconstitute oral competence and a dynamic muscular sphincter with the orbicularis oris muscle. Equally important is the achievement of cosmetic reconstruction of the lip appearance. The focus is on 1)correct alignment of Cupid’s bow.2)symmetric reconstruction of the vermilion.3)and accurate construction of the philtral column.

Additionally, the goals of primary cleft rhinoplasty performed at the time of initial lip repair are important to achieve.

1)Nasal function is optimized by closing the nasal floor and nostril sill. Establishing a correct position for the alar base.

2)Improve the position and contour of the lower lateral cartilages. This enhances nasal aesthetics in the short term

Initial evaluation

Evaluate for associated anomalies.

Consultations

a. Genetics, for evaluation and possible counseling

b. Feeding/nutrition

(1) The child may need special bottles.

(2) Monitor for appropriate weight gain.

c. Otolaryngology: Children with cleft lip and palate have a high incidence of eustachian tube

dysfunction, and otitis media, requiring close follow-up. The child may need myringotomy tubes.

Preoperative molding

Used to bring wide cleft segments together to minimize tensionduring repair1. Taping Applied across both segments of the lip

2. Lip adhesion: Suturing cleft margins togethera. Incisions should be made in region that will be discarded at

subsequent operationb. Goal: Turn a complete CL into an incomplete CLc. Definitive lip repair performed several weeks to months later

Preoperative molding

Presurgical orthodontics

• Nasoalveorlar Molding treatment (NAM): repositions the neonatal alveolar segments– brings the lip elements into

close approximation– stretches the deficient

nasal components– narrow width of cleft

– After NAM, the definitive single-stage cleft lip and nose repair is performed at 3 to 6 months of age • With this initial operation, the

lip/cleft deformity & nasal reconstruction

Presurgical orthodontics

Rule of Tens

Rule of tens: For increased anesthetic safety, an infant

should

• Be 10 weeks old.

• Weight 10 pounds.

• Hemoglobin level of at least 10 mg/dL.

History of Cleft Lip Repair

• Chinese physicians were the first to describe the technique of repairing cleft lip. The early techniques involved simply excising the cleft margins and suturing the segments together.

History of Cleft Lip Repair

• The evolution of surgical techniques during the mid-17th century resulted in the use of local flaps for cleft lip repair. These early descriptions of local flaps for the treatment of cleft lip form the foundation of surgical principles used today.

Types of repair

A. Straight-line repair 1. Historically, the first cleft lip repairs relied

on freshening the edges of the cleft and

suturing them together. These have been

largely

2. Rose-Thompson repair

a. Modified straight-line repair that can be used

for minor clefts with lip length nearly equal

on both sides of cleft (e.g., forme fruste).

b. Fusiform excision with straight-line closure.

B. Quadrangular flap 1. Proposed by LeMesurier

and Hagedorn. 2. Cupid's bow is derived

from the lateral lip. 3. 90-degree Z-plasty. 4. Violates Cupid's bow and

philtral dimple. 5. Has a tendency to produce

a long lip.

C. Triangular flap

The triangular flap repair was initially described in 1952 by Tennison, In 1959, Randall described a mathematical approach to the triangular flap that was on the basis of precise measurements.

This repair technique is conceptually similar to the rotation advancement repair. The primary difference is that the rotation back-cut in the noncleft segment is performed more inferiorly, closer to the vermilion border. Similarly the advancement segment on the cleft side is designed to occur inferiorly near the vermilion cutaneous border.

The main disadvantage of the triangular flap

The philtrum on the cleft side is violated by the triangular flap. Some authors believe this leaves a more noticeable scar.

Another potential disadvantage is the difficulty in modifying the repair or performing secondary revision at a later stage due to the zigzag scars.

2. Skoog repaira. Consists of two Z-

plasties.b. Violates Cupid's bow

and the philtral dimple.

D. Rotation advancement. The rotation advancement repair of the unilateral cleft lip deformity as described by Millard is the most commonly used method of repair at present in the USA.

Millard Rotation-Advancement

Millard DR. Complete Unilateral Clefts of the Lip. Plas Recon Surg 1960 25(6), 595-605.

• Non-cleft side had 2/3 cupids bow, tubercle, white roll , one philtral column & dimple– release this tissue from abnormal high attachment in

columellar base– rotation incision -> drop cupids bow, philtrum & dimple

into normal position– Leaves triangular gap after rotation– Maintain position by obtaining “filler” flap from cleft side –make horizontal subalar relaxing incision to allow

advancement into gap)

Markings/Design

1 to 2 = 1 to 3 = 2-4 mm2 to 6 = 8 to 7 = 20 mm2 to 4 = 8 to 10 = 9 -11mm3 to 5 + x = 8 to 9

Rotation advancement: Technique

• Make markings: from x to 5 to 3 (rotation flap)

• From point 8 to point 9 • Dissect skin off orbicularis on either side

of cleft• Bilateral gingival sulcus incisions made to

cleft margin• Cheek soft tissue elevated off maxilla

above periosteum (caution w/ infraorbital nerve)

• Free orbicularis from attachments to columella/ alar margin

• Incise along alar margin from 9 to 10• Elevate c flap

Rotation advancement: Closure

• Close intraoral mucosa• Close orbicularis serially • Cleft alar base medialized with stitch to

periosteum of nasal spine• C- flap rotated into columellar defect• Close nasal floor• Inset/sew flaps• Nasal correction at same time

The main advantage of this technique is its flexibility and application. The rotation advancement technique relies on a “cut as you go” strategy that allows continuous modifications during the design and execution of the repair. It does not adhere to strict geometrical principles or measurements.

Another advantage is that the suture line approximates a new philtral column. The aesthetic philtral subunit is not violated, and this tends to create a scar that is more camouflaged.

Minimal tissue is discarded during the rotation advancement technique, and this tends to put less tension on the closure.

Furthermore, the rotation advancement technique allows easy access to the alar cartilages for primary rhinoplasty to be performed at the time of lip repair. This early repair of the nasal deformity can be successful in achieving a more symmetric nasal appearance and possibly avoiding the intermediate rhinoplasty step for many of these children.

The primary disadvantage of the rotation advancement technique is that experience is required to achieve optimal results.

Additionally, the vertical scar that occupies the philtral column can be subject to wound contracture. Such contracture can lead to shortening of the lip on the cleft side with resultant vermilion notching and whistle deformity.

Finally the surgeon needs to be cautious when using the rotation advancement technique to avoid excessive narrowing of the nostril sill on the cleft side. This can lead to nasal vestibular stenosis as the wound matures.

Postoperative care

A. Orders 1. Arm restraints for 3 weeks to prevent disruption of repair. 2. Specialized nipple/bottle to decrease sucking effort when bottle-feeding. 3. Breast-feeding is controversial; based on surgeon preference.

B. Leave Steri-Strips in place over the incision for reinforcement.

C. Follow up in 1 week for suture removal if nonabsorbable skin sutures were used.

Complications of cleft lip repair

1) Inadequate reapproximation of the orbicularis oris muscle

with a failure to reconstitute a competent oral sphincter. This

can result in a visible muscle bulge that is readily apparent

under the skin of the repaired lip on dynamic motion.

2) Inaccurate alignment of the vermilion-cutaneous junction

leaving a small step-off deformity that is readily noticeable

even to the untrained eye.

3) vertical scar contracture or inadequate rotation can cause

shortening of the lip segment leading to a notch in the

vermilion and a whistle deformity.4) scar contracture causing a narrow nostril sill with vestibular

stenosis.5) wound healing complications such as dehiscence and scar

widening

Future

• Fetal surgery

• In utero cleft repair

Thank you