Paeds · ent-hearing-and-oral-health
Cleft lip and palate
Also known as Orofacial cleft · Cleft lip · Cleft palate · Cheiloschisis · Palatoschisis · Pierre Robin sequence · Van der Woude syndrome · Craniofacial cleft
Fellowship topic on cleft lip and palate: the embryologic origin in the failure of fusion of the frontonasal, maxillary and mandibular processes and of the palatal shelves; the Veau and Kernahan classifications from an isolated cleft lip through a complete bilateral cleft lip and palate; the newborn priorities of feeding with a squeeze bottle or specialised teat and not allowing aspiration or failure to thrive, the near-universal otitis media with effusion from Eustachian tube dysfunction and the role of grommets at palatoplasty; Pierre Robin sequence with micrognathia, glossoptosis and airway obstruction; the staged surgical timeline of primary lip repair around three months by the rule of ten, palatal repair at nine to eighteen months, secondary alveolar bone grafting at nine to eleven years and orthognathic correction in adolescence; nasoalveolar molding in the first weeks; the syndromic associations of Van der Woude, Stickler and 22q11 deletion; and the multidisciplinary cleft team coordinating surgery, orthodontics, speech, audiology, genetics and psychology across childhood.
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Red flags
- A newborn with a cleft palate and a small recessed chin (micrognathia) has Pierre Robin sequence until proven otherwise and the tongue base can obstruct the airway, especially when supine; prone positioning, a nasopharyngeal airway or in severe cases mandibular distraction may be needed to protect the airway
- An infant with a cleft palate who is choking, coughing or having milk come through the nose during feeding is at risk of aspiration; the feeding technique must be changed immediately to an upright position with a squeeze bottle or specialised cleft teat and paced feeding, and persistent nasal regurgitation or respiratory distress warrants urgent reassessment
- A newborn with a cleft palate and feeding difficulty who is losing weight or not meeting growth milestones needs urgent dietetic and cleft-team input because failure to thrive delays surgical repair and compounds morbidity
- A child with a cleft and a heart murmur, hypocalcaemia or immunodeficiency may have 22q11 deletion syndrome and needs urgent genetics, cardiac and calcium evaluation because the airway and cardiac lesions can be life-threatening
- Bilateral cleft lip and palate with a protruding premaxilla can obstruct the nasal airway and distort feeding, and it needs early nasoalveolar molding and cleft-team review rather than reassurance that the lip will be repaired later
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Clinical exam formats
Board mappings
- General Paediatrics and ENT: recognition of orofacial clefts at birth, the distinction between cleft lip and cleft palate, feeding management, and the newborn hearing screen
- Neonatology: Pierre Robin sequence with airway obstruction, 22q11 deletion with cardiac and calcium risk, and the multidisciplinary referral pathway
- ENT and Craniofacial Surgery: the Veau classification, the rule of ten for lip repair, palatoplasty timing, nasoalveolar molding, and secondary alveolar bone grafting
- Development and disability: otitis media with effusion and conductive hearing loss in cleft palate, the role of grommets, and the speech and language trajectory
- General Paediatrics: recognition of cleft lip and palate, safe feeding advice using a squeeze bottle or specialised teat, and timely referral to the cleft team
- Communication: counselling the family on the staged surgical pathway, the hearing and speech outlook, and the genetic recurrence risk
- Long Case / Structured discussion: a child with cleft lip and palate, the classification, the feeding pathway, the surgical timeline, the hearing management, and the syndromic work-up
- Communication station: explaining the diagnosis, feeding technique, staged repair and multidisciplinary follow-up in plain language
- Level 2 / 3 ENT and Oral Health: orofacial clefts, recognition, feeding, hearing assessment, and the multidisciplinary referral pathway
- Patient safety: recognising Pierre Robin airway obstruction and 22q11 deletion as the must-not-miss priorities in the newborn with a cleft
- Foundation of Practice: the newborn with a cleft lip or palate, the embryology of facial fusion, and the syndromic associations of Van der Woude, Stickler and 22q11 deletion
- Applied Knowledge in Practice: the feeding pathway, the staged surgical timeline, otitis media with effusion, and the multidisciplinary team
- History-taking and management: the child with a cleft, feeding, hearing, speech and the staged reconstruction across childhood
- Communication: explaining the diagnosis, the squeeze-bottle feeding technique, the timing of surgery, and the role of grommets in plain language
- General Pediatrics Content Outline, Eye, Ear, Nose and Throat: orofacial clefts, recognition, feeding, hearing assessment and referral
- Neonatology and Genetics: Pierre Robin sequence, 22q11 deletion, Stickler syndrome and the multidisciplinary work-up
- Patient Care: recognition and stepwise management of orofacial clefts across feeding, airway, hearing and staged surgery, with accurate safety-netting
- Systems-Based Practice: timely and equitable access to multidisciplinary cleft team services for rural, remote and Indigenous families
- Medical Knowledge: facial-process and palatal-shelf embryology, the Veau classification, and the staged surgical timeline
- Medical Expert: cleft classification, the rule of ten, palatoplasty timing, otitis media with effusion and grommets, and the syndromic work-up
- Health Advocate and Collaborator: equitable access to cleft team services for remote and Indigenous families
- Communicator: explaining the feeding pathway, the staged surgical plan, and the hearing and speech outlook
Overview & Definition
Picture a baby born in the delivery suite whose midwife notices a split in the upper lip as she dries the child — perhaps just a small notch, perhaps a gap running up into the nostril, and when the baby cries the palate is open too. The parents are frightened and full of questions the general paediatrician must answer on the spot: will the baby feed, will the baby be deaf, when can it be fixed. This is the everyday face of orofacial clefting, and the skill is not in spotting the gap, which is obvious, but in three judgements made in the first hours of life. Can this baby feed safely without choking or aspirating, and what bottle and technique do you teach the mother today? Is the airway safe, because a cleft palate with a small chin can hide a Pierre Robin sequence that obstructs when supine? And is this an isolated cleft or the face of a syndrome — 22q11 deletion, Stickler or Van der Woude — that changes the whole work-up and the genetic counselling? [2] [3]
An orofacial cleft is a structural gap in the upper lip, the alveolus (the tooth-bearing gum), the hard palate or the soft palate, produced when the embryonic facial processes or the palatal shelves fail to meet and fuse during the first trimester. The gap may be as small as a bifid uvula — the mildest palatal cleft, often found only when a child is investigated for nasal-sounding speech — or as large as a complete bilateral cleft lip and palate in which both sides of the lip, the alveolus and the entire palate are open and the central premaxilla protrudes. Most clefts are visible at birth, but an isolated submucous cleft palate, where the mucosa is intact but the muscles of the soft palate are split in the midline, can be missed until the child presents with hypernasal speech or persistent middle-ear effusion. [1] [3]
The reason cleft lip and palate travel together yet are distinct is embryologic. The lip and the alveolus form first, from the fusion of the medial nasal prominence (a derivative of the frontonasal process) with the maxillary process around the sixth week. The palate forms later, in two stages: the primary palate (the anterior third, behind the upper incisors) fuses with the maxillary processes by about the seventh week, and then the two palatal shelves — tongue-shaped outgrowths of the maxillary processes — reorient from a vertical position on either side of the tongue to a horizontal plane above it and fuse in the midline and with the nasal septum by the ninth to twelfth week. Because the lip forms before the palate, a cleft lip can occur with or without a cleft palate, but an isolated cleft palate has a different embryologic mechanism and a different epidemiology and genetic risk. [3]
References12ShowHide
- [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]Worley ML; Patel KG; Kilpatrick LA Cleft Lip and Palate. Clin Perinatol, 2018.PMID 30396411
- [3]Nasreddine G; El Hajj J; Ghassibe-Sabbagh M Orofacial clefts embryology, classification, epidemiology, and genetics. Mutat Res Rev Mutat Res, 2021.PMID 34083042
- [4]Babai A; Irving M Orofacial Clefts: Genetics of Cleft Lip and Palate. Genes (Basel), 2023.PMID 37628654
- [5]Applebaum SA; Aronson S; Termanini KM; et al Evidence-Based Practices in Cleft Palate Surgery. Plast Reconstr Surg, 2024.PMID 38266141
- [6]Kapadia H; Olson D; Tse R; et al Nasoalveolar Molding for Unilateral and Bilateral Cleft Lip Repair. Oral Maxillofac Surg Clin North Am, 2020.PMID 32165093
- [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
- [8]Hsieh ST; Woo AS Pierre Robin Sequence. Clin Plast Surg, 2019.PMID 30851756
- [9]Haas J; Yuen K; Farrokhyar F; et al Non-operative interventions for Pierre-Robin sequence: A systematic review and meta-analysis. J Craniomaxillofac Surg, 2024.PMID 39256141
- [10]McGlone M; Solomon D; Bjorling A; et al Otitis Media With Effusion in Patients With Cleft Palate. Clin Pediatr (Phila), 2026.PMID 41450170
- [11]Iemura-Kashiwagi M; Okano T; Iwai N; et al Prognosis of otitis media with effusion in pediatric patients with cleft palate during language-acquisition period treated by simultaneous tympanostomy tube placement with palatoplasty. Int J Pediatr Otorhinolaryngol, 2022.PMID 35217270
- [12]Wadde K; Chowdhar A; Venkatakrishnan L; et al Protocols in the management of cleft lip and palate: A systematic review. J Stomatol Oral Maxillofac Surg, 2023.PMID 36410660