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Paeds Vivasent-hearing-and-oral-health

Paeds Vivas · ent-hearing-and-oral-health

Cleft lip and palate — branching viva

Branching viva on cleft lip and palate: recognising the cleft at birth and classifying by Veau, the embryology of facial-process and palatal-shelf fusion, the feeding principle of the squeeze bottle, Pierre Robin sequence with airway obstruction, the near-universal otitis media with effusion and grommets, the rule of ten and the staged surgical timeline, and the syndromic associations of Van der Woude, Stickler and 22q11 deletion.

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Target exams

RACP DCEMRCPCH ClinicalRCPSC Pediatrics

Target exams

RACP DCEMRCPCH ClinicalRCPSC Pediatrics
Prompt
Outpatient clinic: a newborn with a complete left-sided cleft of the lip and palate, feeding well with a squeeze bottle, a bilateral refer on the hearing screen, and an anxious mother asking when it can be fixed. The examiner asks: how do you classify this, how did it happen, and what is the pathway — then branches to the airway in Pierre Robin sequence, to the hearing and grommet question, and finally to a second child with a cleft palate, lower-lip pits and a family history.

Branch 1 — The classification, the embryology and the feeding

The candidate should classify the cleft as Veau grade III (unilateral cleft lip and palate) and explain the embryology: the cleft lip arose from failure of fusion of the medial nasal prominence (frontonasal process) with the maxillary process on the left around week six, and the cleft palate arose from failure of the palatal shelves to fuse in the midline between weeks nine and twelve. The lip forms before the palate, which is why both are affected together. [1] [2]

Probe the feeding: the candidate should explain that the cleft palate breaks the oral-nasal seal so the infant cannot generate negative intraoral suction for a standard nipple or the breast, and that the correct technique is an upright position with a squeeze bottle (Haberman or Pigeon teat) delivering milk by compression, with paced feeding and frequent burping. Push on the consequence of a standard bottle — nasal regurgitation, choking, aspiration, prolonged feeding and failure to thrive. [7]

Branch 2 — The airway in Pierre Robin sequence

Introduce a second newborn with a cleft palate, a small recessed chin (micrognathia), noisy breathing and desaturation when supine. The candidate should recognise Pierre Robin sequence (micrognathia, glossoptosis, U-shaped cleft palate) and state that the airway takes priority. The glossoptotic tongue base obstructs the pharynx, especially when supine. [8]

Probe the management: the candidate should name prone or lateral positioning as the first step, a nasopharyngeal airway as the second, and mandibular distraction osteogenesis or tongue-lip adhesion as surgical options for refractory cases. The candidate should explain that the jaw catches up with growth and the airway risk diminishes over the first years. Push on the syndromic associations — Stickler syndrome (collagen, myopia, retinal detachment) and the need for an ophthalmic review. [8]

Branch 3 — The hearing and the grommet question

Ask why the newborn hearing screen referred bilaterally and what it means. The candidate should explain that the otitis media with effusion is near-universal in cleft palate because the cleft disrupts the levator veli palatini and tensor veli palatini muscles that open the Eustachian tube, so the middle ear fills with persistent effusion and produces a conductive hearing loss. [10]

Probe the management: the candidate should state that ventilation tubes (grommets) are typically inserted at the time of palatoplasty to relieve the effusion and protect the speech and language acquisition period, and that hearing is monitored with audiometry and tympanometry through childhood because the effusion can recur. Close on the synthesis: feeding first, airway second, hearing third, then the staged surgical timeline — rule of ten lip repair at three months, palatoplasty with grommets at nine to eighteen months, alveolar bone graft at nine to eleven years — all coordinated by the multidisciplinary cleft team. [10]

References

  1. [1]Vyas T; Gupta P; Kumar S; et al Cleft of lip and palate: A review. J Family Med Prim Care, 2020.PMID 32984097
  2. [2]Worley ML; Patel KG; Kilpatrick LA Cleft Lip and Palate. Clin Perinatol, 2018.PMID 30396411
  3. [7]Penny C; McGuire C; Bezuhly M A Systematic Review of Feeding Interventions for Infants with Cleft Palate. Cleft Palate Craniofac J, 2022.PMID 34714161
  4. [8]Hsieh ST; Woo AS Pierre Robin Sequence. Clin Plast Surg, 2019.PMID 30851756
  5. [10]McGlone M; Solomon D; Bjorling A; et al Otitis Media With Effusion in Patients With Cleft Palate. Clin Pediatr (Phila), 2026.PMID 41450170