Objectives/hypothesis: To study the presentation, management, and long-term outcome of children presenting with lingual thyroid.
Study design: Institutional review board approved, retrospective study (1993-2004).
Methods: The study was conducted at a tertiary care pediatric medical center. The main outcomes measured were initial presentation, radiographic findings, endocrine evaluation, surgical outcome, pathologic features, complications, need for hormonal replacement.
Results: Four patients presented to the Department of Otolaryngology and Communications Enhancement, Children's Hospital Boston with lingual thyroid between 1993 and 2004. All patients were female, with an age range of 2 to 12 years (x = 6). All patients presented with a mass (1.4-3.5 cm) and most with respiratory or feeding difficulty. Magnetic resonance imaging was obtained in three patients and revealed a mass consistent with lingual thyroid. Thyroid scan confirmed the lingual thyroid as the only functioning thyroid in all four patients. None of the patients responded to hormonal replacement, and all underwent surgical excision of the mass. Surgical approach included midline glossotomy (n = 2) and CO2 laser excision (n = 2). Pathologic evaluation confirmed lingual thyroid in all four patients. No evidence of malignancy was seen in any patient. All four patients require lifelong hormonal replacement.
Conclusions: Lingual thyroid is a rare condition, with an incidence of 1:100,000. This infrequent congenital anomaly is often asymptomatic until a pathologic stress such as systemic disease or physiologic stress such as puberty causes enlargement of the ectopic tissue, leading to dysphagia, dysphonia, and dyspnea. The work-up should include routine blood work including thyroid function tests thyrotropin, thyroxine, and thyroid hormone binding ratio; iodine thyroid scintigraphy; and computerized tomography or magnetic resonance imaging. The majority of patients require surgical excision of the symptomatic mass and, in case of absence of orthotopic thyroid tissue, long-term thyroid hormone replacement.