Subglottic and tracheal stenosis associated with multifocal fibrosclerosis

Abstract

This is the first case report in the literature of idiopathic subglottic and tracheal stenosis in a patient diagnosed with multifocal fibrosclerosis (MFS). The patient had unilateral orbital pseudotumour, bilateral pretibal cutaneous manifestations and raised thyroid antibodies, which might indicate subclinical thyroid involvement. Urgent permanent tracheostomy was needed. The patient also underwent tracheal dilatation for her dysphonia. There was no evidence of brain, lung, liver or renal involvement. Although extremely rare, laryngologists should consider the possibility of such a diagnosis in a stridulous patient with multifocal fibrotic manifestations.

Cochleovestibular nerve involvement in multifocal fibrosclerosis

“…1 The diagnosis of multifocal fibrosclerosis is confirmed with tissue biopsy and histopathological examination. Lesions show prominent hyalinising collagen bundles, proliferation of myofibroblastic cells, and infiltration by lymphocytes.…”

“…3 The main presentations of multifocal fibrosclerosis include retroperitoneal and mediastinal fibrosis, sclerosing cholangitis, Riedel thyroiditis and orbital pseudotumours. 1 A number of cases have also been described involving intracranial fibrosis in the form of inflammatory pachymeningitis and suprasellar, parasellar and intrasellar lesions resulting in hypopituitarism or cranial nerve palsies. 1 Depending on the site of involvement, different clinical features and radiological findings may appear.…”

“…1 A number of cases have also been described involving intracranial fibrosis in the form of inflammatory pachymeningitis and suprasellar, parasellar and intrasellar lesions resulting in hypopituitarism or cranial nerve palsies. 1 Depending on the site of involvement, different clinical features and radiological findings may appear. To the best of our knowledge, our patient represents the first documented case of involvement of the cochleovestibular nerve in multifocal fibrosclerosis.…”

“…However, some rare reports have described familial occurrence amongst siblings, suggesting a recessive form of inheritance. 1 Magnetic resonance imaging clearly displays the fibrotic nature of the lesions found in multifocal fibrosclerosis. On T1-weighted sequences, lesions may appear as gadoliniumenhanced, whereas on T2-weighted sequences they are deeply hypodense.…”

“…[1][2][3] It was first reported by Bartholomew et al in 1963. 1 The clinical course of the disease is variable and poorly defined.…”