25. Cleft lip and cleft palate

1. Definition

• Cleft lip (cheiloschisis, hare lip, "rabbit mouth") → congenital defect of the upper lip from failed fusion of the medial nasal and maxillary processes
• Cleft palate (palatoschisis, "wolf's mouth") → congenital defect of the palate from failed fusion of the palatal shelves
• Most common congenital anomalies of the maxillofacial area

2. Epidemiology

• Cleft lip ± palate → about 1 in 700 live births; more common in boys, left side
• Isolated cleft palate → 1 in 2000; more common in girls

3. Aetiology and risk factors

• Multifactorial → genetic and environmental
• Family history
• Syndromes → Pierre Robin sequence, Van der Woude, Patau (trisomy 13), 22q11 deletion
• Maternal factors:
  • Smoking, alcohol
  • Drugs → phenytoin, sodium valproate, steroids, retinoids
  • Folic acid deficiency
  • Diabetes, obesity
  • Infections in the first trimester

4. Pathogenesis: embryology

• Weeks 4–7 → upper lip and primary palate form by fusion of the medial nasal process with the maxillary processes
• Primary palate → lip, alveolus and palate in front of the incisive foramen
• Weeks 8–12 → palatal shelves of the maxilla fuse in the midline (secondary palate)
• Secondary palate → hard and soft palate behind the incisive foramen
• Failure of fusion → cleft

5. Classification

Cleft lip

• Unilateral, bilateral
• Incomplete → part of the lip, nasal floor intact
• Complete → whole lip into the nostril, often with alveolar cleft
• Microform (forme fruste) → notch or scar only

Cleft palate

• Submucous → bifid uvula, notch in the hard palate, translucent midline (zona pellucida)
• Soft palate only
• Soft and hard palate
• Complete unilateral or bilateral cleft lip and palate

Kernahan's striped Y

• Diagram classification using the incisive foramen as the reference point
• Primary palate cleft → in front of the incisive foramen
• Secondary palate cleft → behind the incisive foramen

6. Clinical picture

Cleft lip

• Visible defect, flattened nostril, deviation of the columella
• Aesthetic deformity
• Alveolar cleft → dental anomalies (missing or extra teeth)

Cleft palate

• Feeding difficulty → cannot create suction; milk comes out through the nose
• Nasal regurgitation, aspiration
• Speech problems → hypernasal speech, nasal air escape (velopharyngeal insufficiency)
• Otitis media with effusion → tensor veli palatini dysfunction, Eustachian tube blocked; hearing loss
• Dental and orthodontic problems, maxillary hypoplasia
• Psychological and social problems
• Pierre Robin sequence → micrognathia, glossoptosis, airway obstruction, U-shaped cleft palate

7. Diagnosis

• Prenatal ultrasound → cleft lip at 18–20 weeks (anomaly scan); palate often missed
• Examination at birth → palpate and inspect the palate in every newborn
• Hearing tests, genetic assessment

8. Principles of treatment

Multidisciplinary cleft team

• Plastic or maxillofacial surgeon
• Orthodontist, dentist
• Speech and language therapist
• Ear, nose and throat (ENT) surgeon, audiologist
• Feeding nurse, psychologist, geneticist

Conservative

• Feeding → special bottles and teats (squeezable), upright position; breastfeeding possible with cleft lip
• Palatal obturator plate → feeding and alveolar moulding
• Presurgical nasoalveolar moulding
• Airway positioning in Pierre Robin sequence

Surgical timeline

1. Cleft lip repair (cheiloplasty) → about 3 months (rule of 10's: 10 weeks, 10 lb, haemoglobin 10 g/dL (100 g/L))
   • Millard rotation-advancement, Tennison-Randall triangular flap
   • Primary rhinoplasty at the same time
2. Cleft palate repair (palatoplasty) → 6–12 months, before speech develops
   • Veloplasty → repair of levator veli palatini muscle sling
   • Von Langenbeck, Veau-Wardill-Kilner push-back, Furlow double-opposing Z-plasty
3. Grommets (ventilation tubes) → otitis media with effusion
4. Speech surgery → pharyngoplasty for velopharyngeal insufficiency, 3–5 years
5. Alveolar bone graft → 8–11 years, before canine eruption; iliac crest bone
6. Orthodontics, orthognathic surgery (maxillary advancement) → after growth, 16–20 years
7. Rhinoplasty, lip revision → adolescence

9. Complications

• Of the condition → aspiration pneumonia, failure to thrive, hearing loss, speech disorder
• Of surgery → airway obstruction, bleeding, wound dehiscence
• Oronasal fistula after palate repair
• Velopharyngeal insufficiency
• Maxillary growth restriction
• Scar, lip and nasal asymmetry