Associated Syndromes & Congenital Heart Disease
Up to a third of clefts are syndromic. The key anaesthetic point is that several of these cause a difficult airway — and the difficulty changes with age in different directions.
| Syndrome | Airway-relevant features | Airway with age |
|---|---|---|
| Pierre Robin sequence | Micrognathia, glossoptosis, cleft palate → post-op airway obstruction | Improves (mandibular catch-up growth) |
| Treacher Collins | Mandibulofacial dysostosis, malar/mandibular hypoplasia | Difficult, does not improve |
| Goldenhar (hemifacial microsomia) | Facial asymmetry, ear/eye (eyelid coloboma) & vertebral/scapular anomalies | Worsens with age |
| Velocardiofacial (22q11 / DiGeorge) | Cleft palate, conotruncal cardiac defects, hypocalcaemia, immune deficiency | — |
| Stickler | Pierre Robin phenotype, myopia, arthropathy | — |
| Down syndrome | Macroglossia, atlanto-axial instability, CHD | — |
| Klippel–Feil | Fused cervical vertebrae → restricted neck movement | — |
| Fetal alcohol syndrome | Midface hypoplasia, CHD, developmental delay | — |
🫀 Associated congenital heart disease
- ASD & VSD are the commonest; Tetralogy of Fallot may occur.
- Suggestive history: recurrent URTI, failure to thrive, turning blue on crying (cyanotic disease), a murmur on auscultation.
- Confirm with echocardiography; obtain a cardiology opinion. Consider infective-endocarditis prophylaxis for the at-risk lesion, and plan for the cardiac physiology (e.g. avoid air bubbles with a shunt).
What the Cleft Does to the Child
🍼 Feeding & nutrition
- Cleft lip: cannot form a seal on the breast/teat.
- Cleft palate: cannot generate negative suckling pressure (oro-nasal communication) → nasal regurgitation.
- Fatigue on feeding → malnutrition & anaemia, poor weight gain. Managed with special bottles/teats, spoon & katori, or a feeding obturator.
👂 Recurrent URTI & ear disease
- Nasal regurgitation of feeds & a misplaced tensor veli palatini → Eustachian-tube dysfunction → chronic serous otitis media & recurrent URTI.
- Resulting hearing loss can make the child uncooperative.
🫁 Airway obstruction & sleep
- In severe micro/retrognathia the airway obstructs supine, even awake; the child prefers a lateral/prone posture (tongue falls forward).
- Chronic obstruction/hypoxaemia → OSA and, in older children, pulmonary hypertension.
🩸 Haematology
- Anaemia (nutritional); a small circulating blood volume makes even modest surgical loss significant → group & cross-match.