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Multidisciplinary Management of a Patient With Van Der Woude Syndrome: A Case Report Publisher



Tehranchi A1, 2 ; Behnia H3, 4 ; Nadjmi N5, 6 ; Yassaee VR7, 8 ; Ravesh Z7 ; Mina M9
Authors
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Authors Affiliations
  1. 1. Preventive Research Center, Research Institute of Dental Sciences, Shahid Beheshti University of Medical Sciences, Tehran, Iran
  2. 2. Orthodontic Department, Dental School, Tehran, Iran
  3. 3. Dentofacial Deformities Research Center, Research Institute of Dental Sciences, Shahid Beheshti University of Medical Sciences, Tehran, Iran
  4. 4. Dental School, Tehran, Iran
  5. 5. University of Antwerp (UA), Belgium
  6. 6. The Team for Cleft and Craniofacial Anomalies, Oral and Maxillofacial Surgery, University of Antwerp, Prinsstraat 13, Antwerp, 2000, Belgium
  7. 7. Genomic Research Center, Shahid Beheshti University of Medical Sciences, Aarabi St., Yaman Ave., Evin, Velenjak, Tehran, 1966645643, Iran
  8. 8. Dept. of Medical Genetic, Faculty of Medicine, Shahid Beheshti University of Medical Sciences, Tehran, 1966645643, Iran
  9. 9. North Khorasan University of Medical Sciences, Bojnurd, Iran

Source: International Journal of Surgery Case Reports Published:2017


Abstract

Introduction Van der Woude syndrome (VWS) is the most frequent form of syndromic cleft lip and palate (SCLP) accounting for 2% of all patients with CLP. Case presentation We describe the orthodontic treatment of a girl diagnosed with VWS referred by her family dentist for her cosmetic concerns. Discussion Comprehensive orthodontic treatment, secondary bone graft, distraction osteogenesis (for a deficient maxilla), secondary palatoplasty and excision of lower lip pits, as well as orthodontic and prosthetic procedures may provide a satisfactory outcome. Genetic testing showed a known putative splice site mutation (c.174 + 1 G/A) as the prime cause of VWS in our patient and her family. Conclusion SCLP has significant effects on facial aesthetics and the psychosocial status. Parents should be assessed and counseled appropriately. This condition is treatable in the absence of life threatening systemic anomalies. An interdisciplinary team approach is advocated. © 2016 The Author(s)