Surgical treatment of macroglossia caused by systemic primary amyloidosis

Amyloidosis is a heterogeneous group of disorders caused by the extracellular deposition of a fibrillar protein called amyloid. Amyloid involvement of the tongue is almost universally secondary to systemic disease. The clinical manifestations result from the progressive extracellular deposition of amyloid within the suprahyoid muscles. In the late stages, the progressive enlargement of the tongue causes hypo- and oropharyngeal blockage, with obstruction of the upper airways. Conservative excision is a satisfactory treatment for local amyloid masses; the role of surgery in systemic forms is controversial. The authors present a case of systemic primary amyloidosis of the tongue treated surgically by a partial glossectomy via a pull-through approach and discuss the indications and rationale for surgical intervention.