Treatment of Condylar Hypoplasia in Alagille Syndrome - A Case Report.

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.