FCPS TOACS Mock Test · Bilateral Cleft Lip Deformity

❓ Q1. Describe the findings in the image. What is the most likely diagnosis? How would you classify this cleft?

✅ Model Answer:
• Findings: Bilateral complete clefts of the upper lip extending into the nasal floor. Protruding premaxilla, flattened nasal tip, short columella.
• Diagnosis: Bilateral complete cleft lip (with or without cleft alveolus).
• Classification: Veau Group III (bilateral complete). LAHSHAL system: L A H S H A L (capital letters indicate complete cleft of lip and alveolus on both sides).

❓ Q2. What is the embryological basis of bilateral cleft lip?

✅ Model Answer:
• Upper lip and primary palate develop during weeks 4-7 of gestation.
• Key structures: Paired medial nasal processes (form philtrum, columella, premaxilla) and paired maxillary processes (form lateral upper lip).
• Bilateral cleft lip: Failure of both maxillary processes to fuse with the medial nasal process.
• Results in separate, protruding premaxilla.
• Risk factors: Folic acid deficiency, maternal smoking, diabetes, valproate use, genetic predisposition.

❓ Q3. What is the incidence of cleft lip ± palate? What is the recurrence risk in siblings?

✅ Model Answer:
• Incidence: 1 in 700-1,000 live births (highest in Asians/Native Americans). M:F = 2:1.
• Bilateral cleft lip accounts for ~10-15% of cleft lip cases.
• Recurrence risk (one child affected, no family history): 2-4%.
• One parent affected: 4% per child. Two children affected: 10-15%.
• Prevention: Periconceptional folic acid (4 mg/day for high-risk mothers) reduces risk by 30-50%.

❓ Q4. What syndromes are associated with bilateral cleft lip?

✅ Model Answer:
1. Van der Woude syndrome (most common) – autosomal dominant (IRF6 gene), lower lip pits (pathognomonic), hypodontia.
2. 22q11.2 deletion syndrome (DiGeorge/VCFS) – conotruncal cardiac defects, hypocalcemia, palatal anomalies.
3. Stickler syndrome – cleft palate, myopia, retinal detachment, hearing loss.
4. Pierre Robin sequence – micrognathia, glossoptosis, U-shaped cleft palate.
5. Treacher Collins syndrome – microtia, colobomas, zygomatic hypoplasia.

❓ Q5. What feeding problems occur in an infant with bilateral cleft lip? How do you manage them?

✅ Model Answer:
• Problems: Inability to create suction due to oronasal communication → nasal regurgitation, choking, aspiration, poor weight gain.
• Management: Special cross-cut nipples (Haberman, Pigeon), upright positioning, palatal obturator (dental plate).
• Nasogastric tube if severe feeding difficulty or poor weight gain.
• Monitor growth weekly; feeding therapy involvement.

❓ Q6. What is nasoalveolar molding (NAM)? When is it used in bilateral cleft lip?

✅ Model Answer:
• Definition: Presurgical orthodontic appliance (dental plate with nasal stents) to gradually reposition protruding premaxilla, alveolar segments, and nasal cartilages before lip repair.
• Indication: Complete bilateral cleft lip with severe premaxillary protrusion (reduces tension during lip closure).
• Started within first 2 weeks, adjusted weekly for 3-6 months.
• Benefits: Improves surgical symmetry, reduces need for premaxillary osteotomy or staged repair.

❓ Q7. What is the optimal timing for cleft lip repair in a term infant? Describe the Rule of 10s.

✅ Model Answer:
• Rule of 10s: 10 weeks of age, 10 pounds (4.5 kg), hemoglobin 10 g/dL.
• Typically performed at 3-6 months of age.
• Goals: Symmetric lip and nasal contour, functional oral competence, minimal scar.
• Techniques: Millard rotation-advancement (unilateral); bilateral more challenging (Mulliken repair, Manchester repair).
• Cleft palate repair (if present) delayed to 9-12 months.

❓ Q8. Why are children with cleft palate at risk for hearing loss? How is it managed?

✅ Model Answer:
• Cleft palate causes Eustachian tube dysfunction → impaired middle ear ventilation → persistent otitis media with effusion (OME).
• OME leads to conductive hearing loss (20-40 dB) → delayed speech if untreated.
• Management: Newborn hearing screen (OAE/ABR), myringotomy and ventilation tube insertion (grommets) at time of palate repair, audiology follow-up every 6-12 months.

❓ Q9. What dental and orthodontic problems occur in bilateral cleft lip and palate? What is alveolar bone grafting?

✅ Model Answer:
• Problems: Missing/malformed teeth, hypodontia, class III malocclusion, alveolar cleft (bony gap).
• Alveolar bone grafting: Performed at 6-10 years (mixed dentition) using iliac crest bone graft to bridge the cleft, allow canine eruption, and provide nasal support.
• Orthodontic treatment (braces) often needed.

❓ Q10. What is velopharyngeal insufficiency (VPI) after cleft palate repair? How is it managed?

✅ Model Answer:
• Definition: Inability to close velopharyngeal port during speech → hypernasal speech, nasal air emission. Incidence 10-20% after palatoplasty.
• Management: Speech therapy (first-line), nasopharyngoscopy/videofluoroscopy to assess gap, surgical correction (pharyngeal flap, sphincter pharyngoplasty, Furlow revision).

❓ Q11. What is the long-term prognosis for a child with repaired bilateral cleft lip? What psychosocial support is needed?

✅ Model Answer:
• Excellent prognosis with multidisciplinary care. Most have normal speech, feeding, and appearance.
• May need secondary procedures (rhinoplasty, scar revision) in adolescence.
• Psychosocial support: Parental counseling, peer support groups (Smile Train, Cleft Lip and Palate Association), early intervention for developmental delays, prevent bullying/stigmatization.

❓ Q12. How will you counsel the mother of this newborn with bilateral cleft lip (who did not take folic acid)? Address guilt, treatment plan, and prognosis.

✅ Model Answer:
• “Your baby has a bilateral cleft lip – the lip did not fully join during early pregnancy. This is not your fault. Even with folic acid, clefts can still occur.”
• “Cleft lip is completely correctable with surgery. Your baby will have normal appearance and function.”
• “We will focus on feeding now with special bottles. Lip repair at 3-6 months (Rule of 10s). Palate repair at 9-12 months if needed.”
• “We will check hearing, heart, and genetics. A craniofacial team will support you.”
• “For future pregnancies, take folic acid 4 mg/day starting 1 month before conception – recurrence risk is 2-4%.”

⚕️ FCPS Paediatrics TOACS · Mock Test