Gorham-Stout syndrome: the radiologic-pathologic correlation as a diagnostic pathway when bone is vanishing.

Rubens Gabriel Feijó Andrade,Simone Gianella Valduga,Bruno Hochhegger,Osvaldo Andre Serafini,Ana Paula Vieira Fernandes Benites Sperb

doi:10.1590/0100-3984.2017.0145

Abstract

Gorham-Stout syndrome: the radiologic-pathologic correlation as a diagnostic pathway when bone is vanishing.

Highlights

Mounier-Kuhn syndrome should be considered in patients who present with bronchiectasis accompanied by abnormal dilatation of the trachea and the main bronchi
The patient was submitted to surgical biopsy of the right iliac bone, and the histopathology demonstrated spongy bone marrow tissue, the bone marrow having been replaced by fibrovascular tissue with innumerable capillary and cavernous vessels, cortical bone resorption, and reactive immature bone, consistent with massive osteolysis (Figure 1)
And radiologically, the syndrome initially presents as foci of rarefaction in the bone marrow and subcortical bone, with slow, irregular progression that can result in effacement of the diaphysis of the bones, narrowing of the involved ends, and, in some cases, the complete disappearance of the bone

Summary

Introduction

Mounier-Kuhn syndrome should be considered in patients who present with bronchiectasis accompanied by abnormal dilatation of the trachea and the main bronchi. 4. Brito MCB, Ota MK, Leitão Filho FSS, et al Radiologist agreement on the quantification of bronchiectasis by high-resolution computed tomography Radiol Bras. Congenital tracheobronchomegaly (Mounier-Kuhn syndrome): a report of 10 cases and review of the literature.

Results

Conclusion