Treatment of imperforate submandibular duct: A systematic literature review and case report

Background: Imperforate submandibular ducts are rare with a broad differential including lymphatic malformation, congenital ranula, and thyroglossal duct cyst. Prompt evaluation is important as treatment differs based on the diagnosis. This systematic review aimed to characterize treatments and outcomes for management of imperforate submandibular ducts while describing 2 new cases. Methods: The following systematic review was performed using PubMed and EMBASE: imperforate submandibular duct or congenital atresia submandibular duct. All associated papers were also included. Demographics, birth history, symptoms, treatment, and outcomes were collected. Results: Sixteen articles were included with 22 patients presenting with imperforate submandibular ducts. Most patients were male (67 %), half of them were born at term (50 %), and 15 (62 %) had unilateral imperforate ducts. Eight patients had bilateral presentation. The mean age for initial treatment was 3.5 months. Treatment varied from 11 (48 %) patients undergoing simple incision only, 3 (13 %) underwent incision and ductoplasty, 4 (17 %) underwent marsupialization and ductoplasty, 3 (13 %) underwent marsupialization only, and 2 (9 %) underwent incision and marsupialization. Mean follow-up without recurrence was 20 months. One complication was reported, restenosis after incision only. Two patients presented to our health system a month apart, one male and one female, and both with unilateral cysts. One patient underwent incision and ductoplasty with no recurrence at the 1-week postoperative visit. The second patient is pending incision and ductoplasty. Conclusions: Although an exceedingly rare condition, imperforate submandibular duct cysts more often present in males, unilaterally, and can be managed via multiple methods; however, marsupialization has shown no reported recurrences.