Long-term surgical and speech outcomes following palatoplasty in patients with treacher-collins syndrome

Abstract

Background: Cleft palate is present in one-third of patients with Treacher-Collins syndrome. The authors present long-term speech and surgical outcomes of palatoplasty in this challenging patient population. Methods: A retrospective review of all patients with Treacher- Collins syndrome and cleft palate was conducted over a 35-year period at a single institution. Demographics, palatal, mandibular, airway, and surgical outcomes were recorded. Speech outcomes were assessed by the same craniofacial speech pathologist. Results: Fifty-eight patients with Treacher-Collins syndrome were identified: 43% (25) had a cleft palate and 16% (9) underwent palatoplasty at our institution. Cleft palate types included 1 Veau I, 5 Veau II, 1 Veau III, and 2 Veau IV. Mean age at the time of palatoplasty was 2.0 years (range, 1.0-6.7 years). Three patients had fistulas (33%) and underwent repairs. Pruzansky classifications included 1 type IIA, 6 type IIB, and 2 type III. Seven patients completed long-term speech evaluations. Mean age at follow-up was 13.9 years (range 2.2-24.3 years). Six patients had articulatory velopharyngeal dysfunction related to Treacher-Collins syndrome. Two patients had structural velopharyngeal dysfunction and required further palatal/pharyngeal surgery. Conclusions: Cleft palate repair in patients with Treacher-Collins syndrome has a high incidence of velopharyngeal dysfunction. However, the majority of patients are articulatory-based in whom further surgery would not provide benefit. Patients with Treacher- Collins syndrome and cleft palate require close evaluation by a speech pathologist as the incidence of articulatory dysfunction is high.