title: A 35-year experience with syndromic cleft palate repair: operative outcomes and long-term speech function.
abstract: BACKGROUND
  Associated comorbidities can put syndromic patients with cleft palate at risk for poor speech outcomes. Reported rates of velopharyngeal insufficiency (VPI) vary from 8% to 64%, and need for secondary VPI surgery from 23% to 64%, with few studies providing long-term follow-up. The purpose of this study was to describe our institutional long-term experience with syndromic patients undergoing cleft palatoplasty.
  
  
  METHODS
  A retrospective review was conducted of all patients with syndromic diagnoses undergoing primary Furlow palatoplasty from 1975 to 2011. Outcomes included postoperative oronasal fistula (ONF) and need for secondary VPI surgery. Speech scores for verbal patients 5 years or older were collected via the Pittsburgh scale for speech assessment. Aggregate scores categorized the velopharyngeal mechanism as competent, borderline, or incompetent. Outcomes were analyzed by patient and operative factors.
  
  
  RESULTS
  One hundred thirty-two patients were included with average age at repair of 20.7 months. Cleft type was 9% submucosal, 16% Veau class I, 50% class II, 12% class III, and 13% class IV. Forty-five syndromes were recorded, most commonly Stickler syndrome (n = 32) and 22q11.2 deletion syndrome [22q11.2DS (n = 19)]. Forty-four patients also had associated Pierre Robin sequence (PRS). The overall ONF rate was 4.5% and was highest in Veau class IV clefts (P = 0.048). Seventy-six patients were included in speech analysis, with an average age at last assessment of 10.4 years. Overall, 60.5% of patients had a competent velopharyngeal mechanism, 23.7% borderline, and 15.8% incompetent mechanism. Fifty percent of 22q11.2DS patients had borderline speech and none had competent speech, compared to 73.3% with Stickler syndrome (P = 0.01) and 71.4% of patients with associated PRS (P = 0.02). Secondary VPI surgery was performed in 11.4% of patients overall. Patients with PRS (13.6%) and with Stickler syndrome (15.6%) had secondary VPI surgery, compared to 31.6% of patients with 22q11.2DS (P = 0.01).
  
  
  CONCLUSIONS
  This study demonstrates low rates of postoperative ONF after modified Furlow palatoplasty in syndromic patients. Speech outcomes were comparable to nonsyndromic patients at our institution, but patients with 22q11.2DS consistently had borderline-incompetent speech and a 3-fold higher incidence of secondary VPI surgery.
authors.affiliation: From the *Division of Plastic Surgery, Perelman School of Medicine at the University of Pennsylvania; †Division of Plastic Surgery, Children's Hospital of Philadelphia, Philadelphia, PA; ‡Division of Plastic Surgery, Johns Hopkins Health System, Baltimore, MD; §Division of Plastic Surgery, Cooper University Hospital, Camden, NJ; ∥Department of Genetics, Children's Hospital of Philadelphia, Philadelphia, PA; ¶The Ohio State University College of Medicine; and #Section of Plastic and Reconstructive Surgery, Nationwide Children's Hospital, Columbus, OH.
authors.forename: Marten N
authors.initials: MN
authors.lastname: Basta
authors.forename: Jason
authors.initials: J
authors.lastname: Silvestre
authors.forename: Carrie
authors.initials: C
authors.lastname: Stransky
authors.forename: Cynthia
authors.initials: C
authors.lastname: Solot
authors.forename: Marilyn
authors.initials: M
authors.lastname: Cohen
authors.forename: Donna
authors.initials: D
authors.lastname: McDonald-McGinn
authors.forename: Elaine
authors.initials: E
authors.lastname: Zackai
authors.forename: Richard
authors.initials: R
authors.lastname: Kirschner
authors.forename: David W
authors.initials: DW
authors.lastname: Low
authors.forename: Peter
authors.initials: P
authors.lastname: Randall
authors.forename: Don
authors.initials: D
authors.lastname: LaRossa
authors.forename: Oksana A
authors.initials: OA
authors.lastname: Jackson
mesh_terms: D002648:Child; D002675:Child, Preschool; D002972:Cleft Palate / Q000150:complications / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D016015:Logistic Models; D008297:Male; D015999:Multivariate Analysis; D010159:Palate / Q000601:surgery*; D011183:Postoperative Complications / Q000175:diagnosis / Q000601:surgery; D019651:Plastic Surgery Procedures / Q000379:methods; D012189:Retrospective Studies; D013064:Speech Disorders / Q000175:diagnosis / Q000209:etiology*; D013577:Syndrome; D016896:Treatment Outcome; D014681:Velopharyngeal Insufficiency / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery