Treatment of Condylar Hypoplasia in Alagille Syndrome - A Case Report

Abstract

Abstract Rationale: Alagille syndrome is a rare genetic disorder with dental and facial abnormalities in the head-and-neck area. It is autosomal dominant and occurs in approximately 1 in 100,000 people. No cases of Alagille Syndrome (ALGS) with mandibular hypoplasia and temporomandibular joint ankyloses (TMJa) have been reported to date. Patient Concerns and Diagnosis: A 3-year-old female patient suffering from ALGS came to our hospital affected by unilateral mandibular hypoplasia and TMJa with severe limitation of mouth opening (maximal interincisal distance [MID] of 2 mm). Treatment: A two-phase surgical management approach was undertaken based on computed tomography scans and the patient’s age. The first phase involved mandibular distraction, followed by arthroplasty with amniotic human membrane placement. Outcomes: After immediate post-surgery rehabilitation, the patient showed good mandibular function with no complications after 15 months. MID was 20 mm. Take-away Lessons: This is a rare and interesting case with no previous literature reports. The use of amniotic membranes in surgical management adds further significance.