Death Due to Obstruction of Airways by a Hyperplastic Polyp: An Unusual Complication of Treated Desmoid Fibromatosis

Abstract

Desmoid fibromatosis (DF) is a rare, locally aggressive but benign clonal fibroblastic lesion of deep soft tissue. It can occur at any age. Extra-abdominal lesions are more common in the pediatric population, with equal sex predilection. Desmoid fibromatosis of the head and neck, particularly the anterior neck, is more difficult to manage because of the proximity to vital structures and may eventually require definitive airway management with insertion of a tracheostomy tube. Indwelling tracheostomy tubes carry their own set of complications. This is the case of a 21-month-old boy who died suddenly due to tracheal obstruction by a hyperplastic polyp. He was diagnosed with DF of the neck before he was 1 month old and required an indwelling tracheostomy tube as a result. Chronic tracheostomy intubation can be complicated by granulation tissue polyp or, less commonly, fibroepithelial polyp formation that can become large enough to cause airway obstruction. To the authors’ knowledge, this is the first case of hyperplastic polyp causing fatal airway obstruction.