Jolibois Marah I, Pekcan Asli, Tanner Aaron, Roohani Idean, Lasky Sasha, Moshal Tayla, Munabi Naikhoba Co, Urata Mark, Magee William P, Hammoudeh Jeffrey A
Division of Plastic and Maxillofacial Surgery, Children's Hospital Los Angeles, Los Angeles, CA.
Albany Medical College, Albany, NY.
J Craniofac Surg. 2025 Jun 25. doi: 10.1097/SCS.0000000000011591.
Many patients with cleft palate require surgical correction of velopharyngeal insufficiency (VPI). Patients with craniofacial syndromes face an increased risk of poor speech outcomes. This study evaluates our institution's experience with syndromic and nonsyndromic patients undergoing palatoplasty and assesses their need for surgical management of VPI.
A retrospective chart review of patients with cleft palate +/- cleft lip who underwent palatoplasty at a tertiary children's hospital from 2004 to 2024 was performed. Demographics, syndromic diagnosis, surgical management of VPI, and speech outcomes were collected. Statistical analyses assessed predictors of VPI surgery.
Of 906 patients, 111 had a diagnosed craniofacial syndrome and 795 were nonsyndromic. The median follow-up time was 10.2 years (IQR: 5.8, 15.9). The most common syndromes included 22q11.2 deletion syndrome (22q11.2DS) (n=26), Stickler (n=22), Van der Woude (n=11), CHARGE (n=10), Treacher Collins (n=8), and Kabuki (n=7) syndromes. Overall, 111 (14.0%) nonsyndromic patients and 28 (25.2%) syndromic patients underwent surgical management of VPI (P=0.002). The 22q11.2DS cohort had the highest VPI surgery rate (57.7%, P<0.001), with 53.8% of these patients experiencing persistent postoperative moderate-to-severe hypernasality. Multivariable logistic regression identified 22q11.2DS and Kabuki syndrome as significant independent predictors of the need for surgical management of VPI.
Patients with syndromic cleft palate underwent surgical management of VPI more frequently than those without craniofacial syndromes. Patients with 22q11.2DS demonstrated greater odds of requiring surgery despite a lack of improvement in resonance and nasal air emission postoperatively. The variability in VPI among syndromic and nonsyndromic necessitates meticulous preoperative planning and individualized management.
