FCPS Paediatrics TOACS · Interactive Station

✅ Candidate's structured answer:
• Findings: Bilateral complete clefts of the upper lip extending into the nasal floor. The premaxilla (central portion) is protruding. Nasal tip flattened, columella short.
• Diagnosis: Bilateral complete cleft lip (with or without cleft alveolus/palate – in this case appears isolated cleft lip).
• Classification:
- Veau classification: Group III – bilateral complete cleft lip (unilateral or bilateral).
- LAHSHAL system: Each letter indicates lip (L), alveolus (A), hard palate (H), soft palate (S). Capital letter = complete cleft, lowercase = incomplete. For bilateral complete lip: L A H S H A L (both sides complete lip and alveolus).
- Complete vs incomplete: Complete cleft extends to the nasal floor; incomplete does not.

✅ Candidate's answer:
• The upper lip and primary palate develop between the 4th and 7th weeks of gestation.
• Key structures: Paired medial nasal processes (form the philtrum, columella, premaxilla) and paired maxillary processes (form the lateral upper lip).
• Bilateral cleft lip: Failure of both maxillary processes to fuse with the medial nasal process.
• Consequently, the central premaxilla remains separate and protrudes forward.
• Cleft palate (if present) results from failure of palatal shelf fusion (6th-9th week).
• Risk factors: Folic acid deficiency, maternal smoking, diabetes, valproate use, genetic predisposition.

✅ Candidate's answer:
• Incidence: 1 in 700-1,000 live births (highest among Asians and Native Americans, lowest in Africans).
• Male:Female – cleft lip ± palate more common in males (2:1).
• Bilateral cleft lip accounts for ~10-15% of cleft lip cases.
• Recurrence risk (if one child affected, no family history): 2-4% for another child.
• If one parent affected: 4% risk per child.
• If two children affected: recurrence risk ~10-15%.
• If parent and child affected: 15-20% risk.
• Prevention: Periconceptional folic acid (4 mg/day for high-risk mothers) reduces risk by 30-50%.

✅ Candidate's answer:
• Most cleft lip ± palate are non-syndromic (70%). However, syndromic causes (30% of cleft lip/palate) include:
1️⃣ Van der Woude syndrome – autosomal dominant (IRF6 gene); lower lip pits (pathognomonic), cleft lip ± palate, hypodontia.
2️⃣ Pierre Robin sequence – micrognathia, glossoptosis, U-shaped cleft palate (may have cleft lip less common).
3️⃣ 22q11.2 deletion syndrome (DiGeorge/VCFS) – conotruncal cardiac defects, hypocalcemia, palatal anomalies, typical facies.
4️⃣ Stickler syndrome – cleft palate, myopia, retinal detachment, hearing loss.
5️⃣ Treacher Collins syndrome – microtia, colobomas, zygomatic hypoplasia.
6️⃣ CHARGE syndrome, Apert syndrome, Goldenhar syndrome.
• Evaluation: Dysmorphology exam, ophthalmology, audiology, echocardiogram, genetic testing (microarray, IRF6).

✅ Candidate's answer:
• Problems: Inability to create negative pressure (suction) due to oronasal communication → nasal regurgitation, air swallowing, choking, coughing, slow feeding, aspiration risk, poor weight gain.
• Management:
1️⃣ Special nipples: Large, soft, cross-cut nipple (e.g., Mead-Johnson cleft lip/palate nipple, pigeon nipple, Haberman feeder) – requires less suction.
2️⃣ Palatal obturator (dental plate) – temporary appliance to separate oral and nasal cavities, improves feeding.
3️⃣ Positioning: Upright or semi-upright during feeding; avoid supine.
4️⃣ Nasogastric (NG) tube – if poor weight gain or severe reflux/aspiration.
5️⃣ Breastfeeding: Difficult but possible with nipple shields. Express breast milk and feed with special bottle.
6️⃣ Monitor growth carefully – weekly weight checks.
7️⃣ Speech therapy / feeding team involvement.

✅ Candidate's answer:
• Definition: A presurgical orthodontic technique using a custom-made intraoral appliance (dental plate with nasal stents) to gradually reposition the protruding premaxilla, alveolar segments, and nasal cartilages before lip repair.
• Indication: Used in complete bilateral cleft lip with severe premaxillary protrusion (to reduce tension during lip closure).
• Process: Started within first 2 weeks of life, adjusted weekly for 3-6 months, until premaxilla is aligned and nasal tip improved.
• Benefits: Reduces gap, improves surgical outcome (symmetry), reduces need for premaxillary osteotomy or staged repair.
• Post-NAM, lip repair is performed.

✅ Candidate's answer:
• Timing (Rule of 10s): 10 weeks of age, 10 pounds (4.5 kg), hemoglobin 10 g/dL. Typically at 3-6 months of age.
• Goals: Achieve symmetric lip and nasal contour, functional oral competence, and minimize scar.
• Techniques for unilateral cleft lip: Millard rotation-advancement (most common).
• Bilateral cleft lip repair: More challenging. Requires simultaneous repair of both sides, often staged (one side at a time) or single stage with premaxillary setback if indicated. Techniques: Mulliken repair, Manchester repair.
• Key steps: Release of abnormal muscle attachments, muscle reconstruction, skin closure, nasal tip columella lengthening.
• Cleft palate repair (if present) is delayed to 9-12 months.

✅ Candidate's answer:
• Examination: Inspect the palate visually (depress tongue with a tongue depressor or laryngoscope). Palpate the hard palate for a submucous cleft (bifid uvula, midline notch, translucency).
• Cleft palate can be isolated (does not involve lip) or combined with cleft lip.
• Bilateral cleft lip is associated with cleft palate in ~50-70% of cases.
• Timing of palate repair (palatoplasty): Typically 9-12 months to allow maxillary growth and minimize speech impairment. Earlier repair (6-9 months) may reduce middle ear disease but may impact maxillary growth. Later repair (>12 months) increases risk of speech abnormalities.
• Techniques: Furlow palatoplasty, Veau-Wardill-Kilner pushback.

✅ Candidate's answer:
• Cleft palate causes dysfunction of the Eustachian tube (abnormal insertion of tensor veli palatini muscle) → impaired middle ear ventilation → persistent otitis media with effusion (OME).
• OME leads to conductive hearing loss (20-40 dB) → delayed speech and language development if untreated.
• Management:
- Newborn hearing screen (OAE/ABR) for all infants.
- Myringotomy and ventilation tube insertion (grommets) at time of cleft palate repair (or earlier if recurrent acute otitis media).
- Audiology follow-up every 6-12 months until school age.
- Sensorineural hearing loss may occur in syndromic clefts (Stickler, 22q11.2 deletion).

✅ Candidate's answer:
• Common problems:
- Missing, malformed, or supernumerary teeth (especially lateral incisors).
- Malocclusion (class III, crossbite, open bite).
- Hypodontia (tooth agenesis) or delayed tooth eruption.
- Alveolar cleft (gap in the maxillary bone) – requires bone grafting.
• Management:
- Alveolar bone grafting: 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 in mixed dentition and adolescence.
- Regular dental checkups, fluoride, and oral hygiene emphasized.

✅ Candidate's answer:
• Definition: Inability to close the velopharyngeal port (soft palate against posterior pharyngeal wall) during speech, leading to hypernasal speech (excessive nasal resonance), nasal air emission, and articulation errors.
• Incidence: 10-20% after palatoplasty.
• Risk factors: Wide cleft, syndromic, poor surgical technique, age at repair.
• Management:
- Speech therapy – first-line for mild VPI.
- Nasopharyngoscopy / videofluoroscopy – to assess gap size and pattern.
- Surgical correction: Pharyngeal flap (posterior pharyngeal flap), sphincter pharyngoplasty, or Furlow palatoplasty revision.
- Speech bulb prosthesis – temporary appliance.
- Early diagnosis and treatment are critical for normal speech development.

✅ Candidate's answer:
• Excellent prognosis with multidisciplinary team care (plastic surgery, dentistry, orthodontics, speech therapy, audiology, genetics, psychology).
• Cosmetic outcomes: Modern repair gives good symmetry, but bilateral clefts may have residual nasal deformity and scarring; further revisions (rhinoplasty, scar revision) may be done in adolescence.
• Speech: Most achieve normal intelligible speech; some may have mild VPI requiring secondary surgery.
• Feeding and growth: Usually normal after lip and palate repair.
• School performance: May have higher risk of learning difficulties, especially in syndromic clefts; neurodevelopmental assessment recommended.
• Psychosocial support: Counseling for parents and child, peer support groups (e.g., Smile Train, Cleft Lip and Palate Association), early intervention for any developmental delays. Prevent social stigmatization and bullying.

✅ Candidate's answer:
• Yes, prenatal diagnosis is possible. 2D/3D ultrasound at 18-22 weeks can detect cleft lip (sensitivity >90%), but cleft palate is more difficult.
• Findings: Failure to visualize the normal upper lip contour; "notched" appearance; possible protruding premaxilla.
• Prenatal management:
- Detailed fetal anatomy scan to rule out associated anomalies (cardiac, brain, spine, abdominal).
- Amniocentesis for karyotype/microarray (especially if other anomalies).
- Fetal echocardiogram.
- Referral to craniofacial team and neonatology for postnatal planning.
- Parental counseling: Explain the condition, excellent outcomes, feeding management, surgical plan, and recurrence risk (2-4%).
- Plan delivery at a hospital with NICU and pediatric surgery/craniofacial expertise.

✅ Candidate's structured answer:
• “Your baby has a bilateral cleft lip – the upper lip did not fully join during early pregnancy. This is a very common birth defect, and it is not your fault. Many causes are unknown; even with folic acid, clefts can still occur.”
• “The good news is that cleft lip is completely correctable with surgery. Your baby will have a normal appearance and function. Most children with cleft lip grow up to live completely normal lives.”
• “Right now, we will focus on feeding. Because the lip is open, your baby cannot suck well. We will give you special bottles/nipples, and a feeding specialist will help. We will also check for a cleft in the palate (roof of mouth) – if present, that will be repaired when the baby is older.”
• “Surgery to close the lip is usually done at 3-6 months of age, when the baby is about 10 pounds. The scar will fade over time.”
• “We will also check your baby’s hearing, heart, and genetics because some children with cleft lip have associated conditions.”
• “You will be supported by a team of specialists (craniofacial team). Many children with clefts have successful surgeries and go on to lead healthy, happy lives.”
• “For future pregnancies, you should take 4 mg (4000 mcg) of folic acid daily, starting 1 month before conception and through the first trimester. This reduces the risk of recurrence, which is about 2-4%.”