What is Idiopathic Macroglossia? A case series and review of literature

Abstract

<h3>Purpose</h3> Macroglossia is pathological condition which resulted in hypertrophy of tongue muscles. It is classified into congenital and acquired macroglossia. Idiopathic macroglossia (IM) is tongue hypertrophy without systemic or genetic causes. This condition has a significant impact on the quality of life, and often require tracheostomy and percutaneous endoscopic gastrostomy (PEG) to sustain living. Guidelines regarding treatment for this subtype are scant. The purpose of this project to present diagnosis and management of a series of patients with idiopathic macroglossia. <h3>Methods</h3> This was a retrospective case series of a cohort of patients with IM who were treated by Oral and Maxillofacial Surgery (OMS) service at University of Texas Health Science Center in Houston (UTHealth) and Emory University from 2019 to 2021. Inclusion criteria are (1) 18 years or older, (2) diagnosed with macroglossia, (3) managed with surgery, (4) with normal tongue tissue on histopathology results, and (5) with a negative COVID test. Patients were excluded if they are younger than 18 years old and diagnosed with macroglossia due to an underlying etiology such as congenital anomaly, systemic conditions, and intraoral inflammatory changes. Study variables were patient demographics, social history, medical comorbidities, clinical presentation, clinical dimensions, presence of tongue protrusion, difficulty feeding, difficulty in breathing, imaging characteristics/dimensions, pathological findings, management (tracheostomy, PEG, glossectomy), and length of inpatient stay. The outcome variables were normalization of tongue size, return of parenteral nutrition, and able to tolerate tracheostomy decannulation. Data were collected using a standardized collection form. Descriptive statistics were computed. <h3>Results</h3> Five patients (mean age, 45 years) with IM met inclusion criteria. All patients had history of hypertension, cerebral vascular disease, and prolong intubation. All patients presented with anterior tongue enlargement, with mean dimension of 13 × 6 cm, full or partial dentition, altered tongue sensation. They were all managed with tracheostomy to secure the airway, PEG and partial glossectomy. Average length of inpatients stay was 10 days. All IM achieved clinical resolution, 80% of the patients had their tracheostomy decannulated and PEG tube removed. <h3>Conclusion</h3> Management of macroglossia requires multidisciplinary approach. While etiology can often be identified and medical treatment can be initiated for reversible causes, most of the macroglossia cases previously reported required surgical management. Surgical reduction offers the best functional and cosmetic results and minimizes morbidity. In the case idiopathic macroglossia, management should involve tracheostomy and feeding access for the initial stabilization followed by reduction glossectomy for improvement of functional outcomes.