A Rare Case of Cleft Palate Associated With Tongue Hamartoma: A Case Report and Systematic Review

Abstract

Introduction Palate development involves a genetic regulation through a complex molecular mechanism that may be disrupted by environmental factors, resulting in impaired fusion and cleft palate formation. An encounter with a case of cleft palate due to dorsal tongue hamartoma prompted us to perform this systematic review. Objective To review the clinical profile and management approach for a case with cleft palate and tongue hamartoma. Design A systematic literature search was conducted using keywords related to cleft palate and tongue hamartoma in PubMed, Scopus, MEDLINE, and Scielo databases through December 2021, with no time or language restrictions. Patients, Participants Studies reporting patients with cleft palate and tongue hamartoma were included. Main outcome measure(s) Information related to clinical profile, diagnostic tests, histopathology, management, and outcomes were extracted. Fourteen relevant publications were identified with 16 cases reported so far. Among them, thirteen patients were females (81.25%), and 3 were males (18.75%). The age of presentation varied from birth to 19 years. Oral-facial-digital syndrome (type II) was the most commonly associated syndrome. Congenital tongue hamartoma with cleft palate is a rare presentation, which can present as an isolated entity or part of a syndrome. Genetic evaluation is warranted, particularly for multiple hamartomatous lesions. The preferred treatment is immediate excision of hamartoma while following a standard timeline for palatoplasty.