Cleft Lip and Cleft Palate

OVERVIEW (Nelson-equivalent content from Schwartz & Bailey & Love)

Definition

Incidence / Epidemiology

• Left-sided cleft lip is twice as common as right and nine times as common as bilateral
• Distribution of cleft types: Cleft lip alone 15%, Cleft lip + palate 45%, Isolated cleft palate 40%
• 29% of CL/P patients have associated anomalies
• Nearly half of isolated CP patients have a diagnosable syndrome

Embryology

• Cleft lip = failure of fusion between the medial nasal prominence and maxillary prominence
• Cleft palate = failure of fusion of the two palatine shelves

Anatomy of Cleft Deformity

• The orbicularis oris muscle continuity is disrupted - it inserts aberrantly into the piriform aperture laterally and the anterior nasal spine medially
• This produces the characteristic cleft lip and nasal deformity
• Key surface landmarks: philtral columns, philtral dimple, white roll (vermilion-cutaneous junction), Cupid's bow

• The levator veli palatini muscles, instead of decussating in the midline to form a functional sling, run parallel to the cleft margin and insert aberrantly into the posterior edge of the hard palate
• This prevents adequate velopharyngeal closure - impairing sucking, speech (causing hypernasal resonance)
• The tensor veli palatini is also disrupted, leading to Eustachian tube dysfunction and recurrent otitis media with effusion (OME)

Classification

• Microform - small notch in vermilion (most minor)
• Incomplete - partial cleft, intact nasal sill
• Complete - entire primary palate involved, extends through nasal sill
• Unilateral vs. Bilateral

• Submucous cleft palate (not in Veau): Triad of bifid uvula + midline translucency ("zona pellucida") + palpable notch of posterior hard palate

Genetics / Etiology

• Multifactorial (most cases)
• Van der Woude syndrome - autosomal dominant, IRF6 gene mutation on chromosome 1; presents with lower lip pits + CL/P
• Stickler syndrome - autosomal dominant (procollagen gene); associated with isolated CP, eye defects, sensorineural hearing loss, joint abnormalities; most common syndrome in Pierre Robin sequence
• Pierre Robin sequence - micrognathia + glossoptosis + respiratory distress; most commonly associated with Stickler syndrome
• Environmental risk factors: maternal smoking, alcohol, folate deficiency, steroids, anticonvulsants (especially phenytoin), retinoids (vitamin A excess)

Associated Problems

1. Feeding difficulties - inability to build negative intraoral pressure (especially cleft palate)
2. Hearing loss - Eustachian tube dysfunction → recurrent OME (children with cleft palate have higher incidence than cleft lip alone)
3. Speech problems:
   • Velopharyngeal incompetence (VPI) - hypernasality, compensatory articulation errors
4. Dental anomalies - hypodontia/hyperdontia, morphological abnormalities, especially in the region of the cleft alveolus (maxillary lateral incisor most commonly affected)
5. Psychosocial issues

SURGICAL MANAGEMENT (Schwartz's Principles of Surgery, 11th ed.)

Multidisciplinary Team (MDT)

Pre-surgical Preparation

• Aims to narrow wide clefts and align dental arches before definitive repair
• Nasoalveolar Molding (NAM): A specialized form of PSIO; additionally elongates the columella and improves nasal tip asymmetry

• An alternative to PSIO for complete clefts
• Surgically converts a complete cleft to an incomplete cleft
• Helps realign dental arches, reapproximate soft tissues, and improve nasal deformity before definitive repair

Timing of Surgery

Cleft Lip Repair

• Pioneer technique that first adequately addressed upper lip length deficiency while preserving philtral anatomy
• A back-cut is designed high on the medial lip element below the columella, enabling downward rotation and leveling of Cupid's bow
• The lateral lip element is advanced into the rotation defect
• Allows anatomic muscular reconstruction (orbicularis oris)
• Most lip repairs today are modifications of Millard's original rotation-advancement principle

• More complex due to bilateral disruption, protrusive premaxilla, and prolabium (prolabial skin lacking muscle)
• Requires elongation of the short columella and reestablishment of bilateral muscular continuity

Cleft Palate Repair

1. Von Langenbeck Repair - Bilateral bipedicled mucoperiosteal flaps elevated from the hard palate and advanced medially. Simple, reliable, but does not lengthen the palate. Best for soft + hard palate clefts.
2. Veau-Wardill-Kilner (V-Y or "push-back") Repair - Retroposition of the mucoperiosteal flaps to lengthen the palate. Improves velopharyngeal function but leaves larger raw areas on the hard palate (risk of growth restriction).
3. Furlow Double-Opposing Z-Plasty - Particularly for soft palate / submucous cleft. Two opposing Z-plasties that simultaneously lengthen the palate and properly reorient the levator muscles. Excellent speech outcomes.
4. Intravelar veloplasty (IVVP) - Focused reconstruction of the levator muscle sling, can be combined with other techniques.

Von Langenbeck bilateral bipedicled mucoperiosteal flap repair - Schwartz's Principles of Surgery

Alveolar Bone Grafting (ABG)

• Performed at 8-10 years (mixed dentition phase), ideally before eruption of the maxillary canine
• Uses autogenous cancellous bone (typically from iliac crest)
• Goals: close oronasal fistula, provide bone support for erupting permanent teeth, stabilize the maxillary arch

Secondary/Revision Surgery

• VPI / Pharyngoplasty: Posterior pharyngeal wall augmentation, pharyngeal flap, or sphincter pharyngoplasty for residual velopharyngeal incompetence
• Cleft lip/nose revision: For misaligned vermilion, lip asymmetry, poor alar base position, or deviated septum - minor adjustments possible before age 14-15; major nasal surgery (open septorhinoplasty) after skeletal maturity
• Orthognathic surgery: Le Fort I osteotomy for midface hypoplasia (a common late sequela), performed after skeletal maturity

Summary of Surgical Timeline

Birth        → Diagnosis, MDT referral, feeding support, NAM/PSIO
~3 months    → Cleft lip repair (Millard rotation-advancement)
9-12 months  → Cleft palate repair (Von Langenbeck / Furlow / V-Y)
8-10 years   → Alveolar bone grafting + orthodontics
4-6 years    → Pharyngoplasty if VPI present (after speech assessment)
12-18 years  → Definitive orthodontics, rhinoplasty, orthognathic surgery

• Schwartz's Principles of Surgery, 11th Edition, Chapter 45 (Plastic and Reconstructive Surgery), pp. 2009-2013
• Bailey and Love's Short Practice of Surgery, 28th Edition, Chapter 50, pp. 762-769