Program Director Carle Foundation Hospital Urbana, Illinois
Disclosure: Nothing to disclose.
Abstract: Velopharyngeal insufficiency (VPI) and hypernasality have a high prevalence in patients following cleft palate repair. The presence of hypernasality is even higher in syndromic cleft palate patients. This is a retrospective cohort study of patients who underwent primary palatoplasty at a Carle Foundation Hospital. The purpose is to evaluate the postoperative prevalence of hypernasality and VPI following primary palatoplasties looking at syndromic versus nonsyndromic patients as well as the type of repair performed. The authors expected to see an increased prevalence of VPI in syndromic patients and no difference in hypernasality among different palatoplasty techniques.
The authors evaluated patients who underwent primary palatoplasties from 2008-2018. Data were collected by data engineers using CPT codes and diagnosis codes to formulate a total list of patients. Inclusion criteria included primary palatoplasty from 2008-2018, an adequate follow-up to assess for speech development, hypernasality rating by a speech-language pathologist (SLP), and evaluation for VPI. Patients who did not meet these criteria had a primary palatoplasty at another institution or are under the age of 3 were excluded from this study. Medical records of the included patients were reviewed and the following were noted: type of cleft, age at primary repair, type of repair, syndromic diagnosis, postoperative hypernasality, postoperative VPI, postoperative fistula, postoperative pharyngeal flap. VPI for this study was defined as velar incompetence diagnosed by videofluoroscopy and/or nasoendoscopy. Frequency tables show the numbers of categorical and numerical values within each finding.
The authors evaluated for a statistical correlation of (VPI) in syndromic versus nonsyndromic patients. Additionally, the authors evaluated hypernasality following primary palatoplasties, looking to see if there was an association between the surgical technique and postoperative hypernasality.
A total of 108 patients had primary palatoplasty during 2008-2018. After chart review, 44 patients met inclusion criteria. Four of the 44 patients are nonverbal. Five patients had postoperative VPI requiring a superior-based pharyngeal flap, 4 male and 1 female. Three patients with VPI were syndromic (2 Pierre Robin Syndrome and 1 HOXA2 mutation). Three of the patients with VPI underwent Bardach as the primary palatoplasty and 1 underwent Furlow double opposing Z plasty. Two syndromic patients, 1 with holoprosencephaly and 1 of unknown diagnosis, were unable to be assessed by SLP due to their nonverbal state.
Hypernasality was assessed by a licensed SLP who is a member of the authors' interdisciplinary cleft lip and palate team. Hypernasal resonance was scaled with 1 as normal nasal emission, 2-4 as mild, 5-6 as moderate, and 7 as severe hypernasal resonance. In the 5 patients diagnosed with VPI, the average hypernasal resonance score was 4.5. In the 39 patients who did not have postoperative VPI, hypernasality was present in 13 (33%) of patients with an average score of 3. Two syndromic (Pierre Robin Syndrome: Fragile X) patients were noted to have hypernasality (2/7; 3/7) without VPI. One nonsyndromic patient with persistent hypernasality had a superior-based pharyngeal flap and hypernasality resolved.
The authors recognize that there is a trend in previous literature showing that syndromic patients with cleft palate have an increased risk of VPI following cleft palate repair. VPI has been reported in the literature to occur in syndromic patients with cleft palate repairs up to 33% of the time. The retrospective cohort revealed that 5 (11%) of the 44 accepted patients were noted to have postoperative VPI. Of the 5 patients with VPI, 3 were noted to be syndromic, which does align with the literature.
References: Nam, SM: Surgical treatment of velopharyngeal insufficiency. Arch Craniofac Surg 19.3:163-67, 2018 Sweeney W, Lanier S, Purnell C, and Gosain A: Genetics of cleft palate and velopharyngeal insufficiency. Journal of Pediatric Genetics 04:01:009-016, 2015