Surgical planning for restoring velopharyngeal function in velocardiofacial syndrome Antonio Ysunza *, Ma. C. Pamplona, Fernando Molina, Adriana Herna ´ ndez Cleft Palate Clinic, Hospital Gea Gonzalez, Mexico City, Mexico 1. Introduction Velocardiofacial syndrome (VCFS) was first described by Shprintzen in 1978 [1]. This syndrome is now recognized as the most common syndrome associated with cleft palate and velopharyngeal insufficiency (VPI). Moreover, VCFS constitutes 8% of patients with clefts of the secondary palate [1–4]. In VCFS cases, most clefts are submucous or ‘‘occult’’ submucous clefts. However, VPI is much more frequent in VCFS patients. VPI refers to abnormalities of the velopharyngeal sphincter involving the velum and/or pharyngeal walls [5–7]. Although this disorder is commonly seen in patients with cleft palate and therefore is diagnosed in early evaluations, submucous cleft palate may be overlooked in some individuals with VPI. In this latter population, the diagnosis might be delayed until speech development allows detection by skilled personnel [8,9]. Fre- quency of VPI among individuals with non-syndromic submucous cleft palate is actually quite low, probably under 9% [7,8]. In contrast, VCFS patients show VPI associated with submucous cleft palate in over 70% of the cases [7]. There are several factors contributing to the high frequency of VPI in VCFS. Platybasia, hypotrophy or absence of adenoids, enlarged tonsils and hypotonia and abnormal pharyngeal muscles have been reported [6,7,10,11]. The goal in treating VPI is to restore a functional seal between the nasopharynx and the rest of the vocal tract situated inferiorly, so that normal articulation of speech can occur. Several surgical options have been reported including the Wardill push-back procedure, the Furlow’s palatoplasty, and the minimal incision palatopharyngoplasty [8,9,12,13]. In addition, individualized velopharyngeal surgery is commonly performed when simple palatal repair fails to completely correct velopharyngeal insuffi- ciency [9]. Individualized surgery includes customized pharyngeal flaps and sphincter pharyngoplasties performed according to findings of videonasopharyngoscopy (VNP) and multiview video- fluoroscopy (VF) [7,9]. Ysunza et al. [8], reported that in non- syndromic submucous cleft palate, a minimal incision palatophar- yngoplasty was a safe and reliable procedure for correcting VPI. The use of additional individualized velopharyngeal surgery International Journal of Pediatric Otorhinolaryngology 73 (2009) 1572–1575 ARTICLE INFO Article history: Received 20 July 2009 Received in revised form 5 August 2009 Accepted 7 August 2009 Available online 31 August 2009 Keywords: Cleft palate Velocardiofacial syndrome Surgery Endoscopy ABSTRACT Background: Velocardiofacial syndrome (VCFS) is one of the most common multiple anomaly syndromes in humans. Around 70% of the cases show velopharyngeal insufficiency (VPI), as a consequence of cleft palate. VPI is much more frequent due to special abnormal conditions inherent to VCFS including: platybasia, hypotrophy of adenoid, enlarged tonsils, hypotonia and abnormal pharyngeal muscles. Objective: To evaluate the surgical treatment of VPI in VCFS patients. Materials and methods: In the Hospital Gea Gonzalez at Mexico City, all cases of VCFS from January 2000 to July December 2007 were studied. All patients subjected to velopharyngeal surgery for correcting VPI were selected. Twenty-nine patients underwent velopharyngeal surgery. All operations were planned according to findings of videonasopharyngoscopy (VNP) and multiview video fluoroscopy (MVF). Results: Twenty patients underwent pharyngeal flap operations, and 9 patients were operated on with a sphincter pharyngoplasty. After a pharyngeal flap, 17 cases (85%) improved to normal nasal resonance or mild hypernasality. Three flaps showed moderate hypernasality postoperatively. From the 9 sphincter pharyngoplasties, 6 cases (66%) improved to moderate hypernasality. Four patients (33%) persisted with severe hypernasality postoperatively. There were no complications. Conclusions: Tailor-made pharyngeal flaps seem to be the best option for restoring velopharyngeal function in cases of VPI in VCFS patients. The use of VNP and MVF is useful for planning the operations for VPI, and they are also useful for indicating the removal of tonsils in cases with high risk of obstruction. Moreover, VNP is also useful for preventing damage to the internal carotids which are usually displaced in VCFS patients. ß 2009 Elsevier Ireland Ltd. All rights reserved. * Corresponding author. E-mail address: amysunza@terra.com.mx (A. Ysunza). Contents lists available at ScienceDirect International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl 0165-5876/$ – see front matter ß 2009 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ijporl.2009.08.007