Abstract: Diagnosis, Treatment, and LONG-TERM Speech Outcomes of Occult Submucous Cleft Palate-Associated Velopharyngeal Insufficiency

Abstract

INTRODUCTION: Overt submucous cleft palate (SMCP) is characterized by Calnan’s triad of bifid uvula, hard palate notch and zona pellucida. Because occult SMCP (OSMCP) lacks this triad, diagnosis is challenging and treatment (if indicated by the presence of velopharyngeal insufficiency [VPI]) is delayed. This study discusses diagnosis, treatment, and long-term outcomes of OSMCP. METHODS: A retrospective chart review was performed for all patients presenting to the Cleft-Craniofacial Center of Children’s Hospital of Pittsburgh who were surgically treated for SMCP between September 2004 to September 2015. Patients with any one of Calnan’s triad were excluded from analysis. The primary outcome was the requirement of secondary speech surgery due to persistent VPI. To evaluate long-term speech outcomes, postoperative follow-up was required to be >3 years for study inclusion. Speech was evaluated over time using Pittsburgh Weighted Speech Scores (PWSS). RESULTS: 317 SMCP patients underwent surgical treatment for VPI, 41 patients were diagnosed with OSMCP (mean age at surgery 6.7 years). Furlow palatoplasty was performed for all patients (mean follow-up: 5.5 years). Diagnostic techniques for OSMCP: 1) intraoral physical examination demonstrating vaulted V-shaped palatal elevation with gag or phonation in 87.8% (36/41) of patients; 2) MRI demonstrating sagittally oriented levator palatini muscles in 19.5% (8/41) of patients; and 3) videofluroscopy to evaluate palatal kinetics in 65.8% (27/41) of patients. Secondary speech surgery was performed on 39.0% (16/41) of patients. 55.6% of patients (5/9) with VCFS underwent secondary surgery (p>0.05). Patients undergoing secondary surgery had a significantly lower change in PWSS (postoperative PWSS minus preoperative PWSS) compared to those who did not (4.4 and 10.0; p=0.022). Using videofluroscopy, patients who required secondary speech surgery had lateral wall motion with 53.6% closure compared to 74.3% closure for those who did not (p=0.11). No oronasal fistulae developed. Posterior pharyngeal flap (PPF) was performed in 87.5% of patients as the secondary speech surgery. Two of these patients (14.2%) developed obstructive sleep apnea (OSA) and required PPF takedown. CONCLUSION: Patients with VPI and no overt signs of SMCP often have delayed diagnosis and incorrect clinical management. Accurate diagnosis of OSMCP is imperative to guide appropriate surgical treatment. Furlow palatoplasty is an important procedure for patients with OSMCP-associated VPI. This shows promising outcomes in patients with OSCMP and limits the risk of PPF-related OSA.