Long-Term Surgical and Speech Outcomes Following Palatoplasty in Patients With Treacher-Collins Syndrome.
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.
*Division of Plastic Surgery, Arkansas Children's Hospital, Little Rock, AR†Hansjorg Wyss Department of Plastic Surgery, New York University Langone Medical Center, New York, NY.
Michael S
MS
Golinko
Etoile M
EM
LeBlanc
Andrew M
AM
Hallett
Michael
M
Alperovich
Roberto L
RL
Flores
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D002972:Cleft Palate / Q000453:epidemiology / Q000601:surgery; D006801:Humans; D007223:Infant; D008342:Mandibulofacial Dysostosis / Q000453:epidemiology / Q000601:surgery; D019647:Oral Surgical Procedures / Q000009:adverse effects / Q000379:methods / Q000706:statistics & numerical data; D012189:Retrospective Studies; D013060:Speech / Q000502:physiology; D016896:Treatment Outcome; D055815:Young Adult