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The authors report a case of amyloidosis-induced macroglossia treated with surgical reduction of the tongue using a keyhole to inverted T method with particular emphasis on the postoperative sequelae. Significant tongue swelling persisted for longer than anticipated requiring tracheostomy to remain in situ for 14 days., (2016 BMJ Publishing Group Ltd.)

Amyloidosis is a disease characterised by the deposition in body tissues of amyloid: abnormal protein in a beta pleated sheet formation. It is a systemic disorder and macroglossia may be seen in all forms. Changes to the normal architecture of the tissues and systemic features of the disease and its underlying cause can complicate the surgical management of the enlarged tongue., (Copyright © 2012 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.)

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., (Copyright © 2012 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.)

A case of macroglossia due to primary amyloidosis is described, followed by a discussion of the various aspects of surgical intervention based on a literature review.

Amyloidosis is a term that describes a heterogeneous group of disorders in which various tissues contain the characteristic insoluble fibrillar protein known as amyloid. A number of different forms of presentation have been described and include senile, familial or hereditary, systemic, localized, secondary and primary forms. We now report our management of a 79-year old woman who suffered from primary A-lambda-light chain amyloidosis. Amyloid changes first presented in the tissues of the tongue and floor of mouth and were seen clinically as a macroglossia. Further investigation led to a primary diagnosis of multiple myeloma IIa, as categorized by Durie and Salmon. Several cases have been reported to date describing secondary development of macroglossia due to long-term known and treated multiple myeloma. However, there has been only one report of macroglossia as a primary finding leading to the diagnosis of multiple myeloma.

A case of extreme macroglossia was observed in a patient with amyloid disease and dysglobulinaemia. A medial diamond-shaped glossectomy was required to reduce the size of the tongue. The details of the surgical and anaesthetic procedure are presented. This type of surgery, greatly appreciated by the patient, can only be undertaken after careful preparation. The reported cases are rare and the indication for surgery is difficult due to the unpredictable nature of the local and general clinical course of the disease.

A lady of 62 years with longstanding macroglossia from primary amyloidosis had the anterior two-thirds of her tongue successfully resected. She had Factor X deficiency. The surgical, anaesthetic and haematological problems encountered are described.