A 35-Year Experience With Syndromic Cleft Palate Repair: Operative Outcomes and Long-term Speech Function.

[22q11.2 deletion syndrome]

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 .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 .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 22 q 11 .2 deletion syndrome [22 q 11 .2 DS (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 22 q 11 .2 DS 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 22 q 11 .2 DS (P = 0 .01 ).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 22 q 11 .2 DS consistently had borderline-incompetent speech and a 3 -fold higher incidence of secondary VPI surgery .

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Diseases presenting "palate at risk" symptom

22q11.2 deletion syndrome

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