Mandibular Reconstruction in a 3 Year Old Patient with Marden Walker Syndrome
Roumman Amir *
Al Jalila Children’s Specialty Hospital, Dubai Academic Health Cooperation, Dubai, U.A.E.
Maryam Haitham Salman
Al Jalila Children’s Specialty Hospital, Dubai Academic Health Cooperation, Dubai, U.A.E.
Hana Iqbal
Al Jalila Children’s Specialty Hospital, Dubai Academic Health Cooperation, Dubai, U.A.E.
Masiha Tasneem Banu
Al Jalila Children’s Specialty Hospital, Dubai Academic Health Cooperation, Dubai, U.A.E.
Farheen Khan
Al Jalila Children’s Specialty Hospital, Dubai Academic Health Cooperation, Dubai, U.A.E.
*Author to whom correspondence should be addressed.
Abstract
Introduction: Marden Walker Syndrome is a rare genetic disorder with distinctive craniofacial features, primarily characterized by micrognathia or a small jaw.
Patients' affected by this condition may undergo corrective surgical procedures in order to manage their deformities and improve their overall quality of life.
Case Presentation: A 3-year-old female, known case of Marden Walker Syndrome with severe micrognathia, was admitted for elective mandibular reconstruction surgery. The surgery involved bone grafting with microvascular anastomosis and subperiosteal implantation. Postoperatively, the patient experienced respiratory complications, requiring intubation and subsequent admission to the Pediatric Intensive Care Unit (PICU) for 16 days. She was managed with mechanical ventilation, extubation trials and gradual weaning to room air. Following stabilization, she was successfully extubated and discharged home. The patient is currently receiving follow up with the pediatric plastic surgeons and otolaryngologists.
Conclusion: This case illustrates the challenges faced in managing rare genetic conditions and complex craniofacial anomalies in pediatric patients.
Multidisciplinary care including surgical expertise, respiratory support, and vigilant monitoring, is crucial to achieve successful outcomes in such cases.
Keywords: Marden walker syndrome, craniofacial anomalies, genetic disorder, micrognathia, mandibular reconstruction surgery