Cleft Lip and Palate: Multidisciplinary Care from Birth, Surgical Repair and Long-Term Follow-up

Cleft lip and palate (CLP) is a closure defect during embryonic fusion (4-12 weeks gestation) of the maxillary and medial nasal processes. It may involve the upper lip, alveolar arch and/or palate (hard and/or soft).

Classification: isolated cleft lip (CL), isolated cleft palate (CP), combined cleft lip and palate (CLP). May be unilateral (left or right) or bilateral; complete (extending to alveolar arch and nasal floor) or incomplete (microform). Submucous cleft palate — no visible cleft but underlying muscle defect — is hard to detect and often discovered when speech problems appear.

Incidence: 1 in 700-1000 births worldwide. Ethnic and geographic variation: highest in Asian populations (1/500), 1/700-1000 in European, lowest in African (1/2000-2500). Türkiye prevalence approximately 1/800-1000.

Aetiology: multifactorial. Genetic (especially non-syndromic CLP — IRF6, FGFR1, MSX1 etc.), environmental (smoking: 2-3-fold risk, alcohol, anticonvulsant use — phenytoin, valproate; folic acid deficiency), syndromic (CLP appears in over 200 syndromes — van der Woude, 22q11.2 deletion, Pierre Robin sequence, Treacher Collins, Stickler etc.).

Prenatal diagnosis: second-trimester (typically 18-22 weeks) ultrasound visualises the cleft lip; palatal cleft is hard to see. 3D/4D ultrasound gives >90% accuracy for cleft lip. After diagnosis: genetic counselling, syndrome screening, birth planning and family counselling are important. Related overview: our general ENT services.

In the first 24-48 hours: detailed physical examination (cleft type, width, side, associated anomalies), ENT evaluation (ear anatomy, syndromic features), dietitian consultation, parent education and psychosocial support.

Feeding is the main challenge. Most isolated cleft-lip infants can breastfeed or use standard bottles. Cleft-palate infants cannot generate intraoral negative pressure — cannot draw milk from breast or standard nipple. Specialty bottles/nipples (Haberman Feeder, Pigeon Cleft Lip and Palate Bottle, Dr. Brown's Specialty Feeder, MAM) use gravity and controlled compression to deliver milk.

Expressing breast milk and feeding via specialty bottle is the common approach. Breast milk is especially valuable for immune protection — CLP infants have high otitis media risk. Caloric support and adequate weight gain monitored; regular dietitian follow-up is critical.

Suspected Pierre Robin sequence (micrognathia + glossoptosis + cleft palate) requires immediate airway assessment — supine position may cause obstructive apnoea; prone positioning, nasopharyngeal airway, or surgery (mandibular distraction osteogenesis, tongue-lip adhesion) may be needed.

Pre-surgical orthopaedic intervention: in some centres, nasoalveolar moulding (NAM) — a custom acrylic intraoral appliance started weeks after birth to approximate maxillary segments and shape the nose. Facilitates surgery and improves cosmetic results.

Cleft lip repair (cheiloplasty) — at 3-6 months. The "rule of tens" guides timing: 10 weeks old, 10 lb weight, haemoglobin 10 g/dL — optimising anaesthetic safety and tissue healing.

Techniques: Millard rotation-advancement (most common), Tennison-Randall (triangular incision), Fisher anatomic subunit (modern — less scar, more natural Cupid's bow). Standard in unilateral clefts; modified Mulliken in bilateral. Nasal deformity (alar collapse, short columella) is partially corrected in primary surgery — "primary rhinoplasty" concept.

Operation 1.5-3 hours. Hospital stay 1-2 days. Postoperative: incision tape for 2 weeks, careful feeding (cup/syringe — direct sucking forbidden 2 weeks), arm restraints (no-no's) so the infant cannot reach the wound. Healing 2-4 weeks; final scar matures over months.

Palate repair (palatoplasty) — at 9-18 months. Early (6-9 months) repair supports speech but risks growth inhibition; late (>18 months) preserves growth but risks speech problems. Optimum timing debated; modern trend 9-12 months (especially with minimally traumatic techniques like Sommerlad's).

Palatoplasty techniques: von Langenbeck, Veau-Wardill-Kilner (V-Y push-back), Furlow double-opposing Z-plasty (soft palate lengthening + levator repositioning), Sommerlad (intravelar veloplasty). Goals: anatomical closure, correct positioning of the levator veli palatini muscle (preventing velopharyngeal insufficiency), no oronasal fistula.

Complications: early (bleeding, dehiscence, infection); late (oronasal fistula 5-15%, velopharyngeal insufficiency 10-25%, maxillary growth disturbance, speech problems). Step-by-step details: tonsillitis page.

More than 90% of cleft-palate children develop eustachian tube dysfunction — abnormal anatomy of soft-palate muscles (especially tensor veli palatini) cannot open the tube. Result: chronic otitis media with effusion, sometimes hearing loss.

Approach: ENT and audiometric assessment every 3-6 months in early years. Persistent effusion with hearing loss prompts ventilation tube placement (often done at the same time as palate repair). Tubes may be replaced several times in childhood. Long-term cholesteatoma risk is elevated — long-term ENT follow-up essential.

Speech development: cleft palate children need early speech therapy. Speech therapist assessment from 12-18 months. Velopharyngeal insufficiency (VPI) — failure of soft palate-pharyngeal wall closure — causes hypernasal speech, nasal air emission and compensatory articulation errors. VPI is clinically assessed by 4-5 years; if persistent, additional surgery (pharyngeal flap, sphincter pharyngoplasty, dynamic palatoplasty) may be needed.

Multidisciplinary cleft team: surgical and orthodontic follow-up, nutrition, speech, hearing, and psychological support are coordinated. Annual or biannual team meetings review progress and adjust the plan.

Education and social integration: by school age, lip scar revision, speech therapy, additional lip/nose surgery may be performed. Schools are informed about potential social stigma; the child's self-esteem is supported by counselling and peer-group programmes.

Alveolar bone graft: 7-11 years, before permanent canine eruption. Corticospongious bone harvested from iliac crest placed into the alveolar cleft; supports tooth eruption, orthodontic movement, and maxillary stability.

Velopharyngeal insufficiency surgery (if needed): 4-6 years, when speech therapy proves insufficient. Pharyngeal flap (flap from posterior pharyngeal wall to soft palate), sphincter pharyngoplasty (palatopharyngeus muscles relocated to mid-upper), Furlow palatoplasty revision are options.

Oronasal fistula repair: postoperative fistula 5-15%. Small asymptomatic ones — observe; symptomatic (fluid leakage, speech problems) — surgical repair (local mucosal flap, tongue base flap, mucoperiosteal flap).

Adolescent surgery: maxillary hypoplasia (class III malocclusion) often develops — orthognathic surgery (Le Fort I advancement or distraction osteogenesis) at 16-18 years after growth completion. Definitive cleft rhinoplasty — final correction of nasal cleft deformity (alar drop, short columella, dorsal hump, septal deviation) at 16-18 years.

Dental care and aesthetics: congenitally absent teeth (especially lateral incisor), malocclusion and crowding are typical. Prosthetics, implants, crossbite correction and aesthetic dentistry in adolescence and young adulthood.

Psychosocial support: for self-esteem, social interaction and academic performance, ongoing psychological support and peer groups through childhood and adolescence are beneficial. Family preparation against teasing and the child's ability to discuss the condition matter.

Long-term prognosis: with early diagnosis, correctly timed surgery and comprehensive multidisciplinary follow-up, the vast majority of children attend normal school, live normal lives, and become productive adults. Aesthetic outcomes show high patient satisfaction. Speech can be normalised; 90%+ achieve socially normal speech. Related reading: our Istanbul ENT services.