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A 35-year experience with syndromic cleft palate repair: operative outcomes and long-term speech results.
Marten N. Basta, BA1, Jason Silvestre, BS1, Cynthia Solot, SLP2, Marilyn Cohen, SLP2, Richard E. Kirschner, MD2, David W. Low, MD2, Don LaRossa, MD2, Oksana A. Jackson, MD1.
1University of Pennsylvania Perelman School of Medicine and Children's Hospital of Philadelphia, Philadelphia, PA, USA, 2Children's Hospital of Philadelphia, Philadelphia, PA, USA.

BACKGROUND
Associated medical comorbidities and developmental delays can put syndromic patients with cleft palate at risk for poor speech outcomes. Reported rates of velopharyngeal insufficiency (VPI) are widely variable, and need for secondary VPI surgery ranges from 23-64% in the literature, with few studies providing long-term follow-up. The purpose of this study was to describe one institution’s long-term experience with a large series of syndromic patients undergoing cleft palate repair.
METHODS
A retrospective review was performed of all patients with syndromic diagnoses who underwent primary palatoplasty at a large pediatric center from 1975-2011. Collected outcome measures included post-operative oronasal fistula and need for secondary surgery for VPI. Speech scores for verbal patients 5 years or older were collected via the Pittsburgh Weighted Values for Speech Symptoms Associated with VPI, and based on the total score, speech outcomes were categorized as competent, borderline, or incompetent. Outcomes were analyzed by syndrome type, association with Pierre Robin Sequence (PRS), Veau cleft type, age at repair, gender, and history of airway management.
RESULTS
132 patients were included, and the average age at palatoplasty was 20.7 months (6-154). Composition of cleft type was 9% submucosal, 16% Veau Class I, 50% class II, 12% class III and 13% class IV. The overall oronasal fistula rate was 4.5%. A total of 48 syndromes were recorded. PRS was diagnosed in 44 patients, of which 32 had Stickler syndrome, and 22q deletions were found in 19 patients. 76 patients (58%) had valid speech records available at a minimum of age 5, and the average age at last assessment was 10.4 years (5-21). Overall, 60.5% of patients had a competent velopharyngeal mechanism, 23.7% borderline, and 15.8% incompetent speech. VPI surgery was performed in 11.4% of patients at an average age of 8.3 years (4.0-15.8). Patients with 22q deletions had relatively poor speech outcomes as no patients demonstrated competent speech mechanisms, compared with 71.4% competent speech in sPRS (p=0.02) and 73.3% in Stickler patients (p=0.01). Pittsburgh speech score for sPRS and Stickler patients were not statistically different from the remaining cohort. 31.6% of 22q patients underwent secondary VPI surgery, significantly higher than the remainder of the cohort (p=0.01). Secondary VPI surgery was undertaken in 13.6% of sPRS patients (p=0.57) and 15.6% of Stickler patients (p=0.53). Other patient characteristics, including Veau Cleft type, gender, and oronasal fistula post-palatoplasty were not associated with poorer speech outcomes. Similarly, history of operative vs. nonoperative airway management in sPRS patients had no effect on speech outcomes, with competent speech in 66.7% and 73.9% of patients, respectively (p=0.71).
CONCLUSIONS
This study demonstrates acceptably low rates of operative complications post-palatoplasty in syndromic patients. While overall incidence of VPI surgery is comparable to non-syndromic patients, those with 22q deletions consistently had borderline or incompetent speech mechanisms and a significantly higher rate of VPI surgery compared to all other groups.
Note: * denotes p value compared to remaining cohort, "a" denotes p value compared to 22q subgroup
GroupN (total)Age at RepairONFVPI SurgeryN (speech)Competent
All13220.7 mo4.5%11.4%7660.5%
sPRS4417.5 mo2.0%13.6 % (p=0.57)*3571.4% (p=0.02)a
Stickler3218.7 mo2.0%15.6 % (p=0.53)*3073.3% (p=0.01)a
22q1928.4 mo5.3%31.6 % (p=0.009)*80.0%


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