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Critical Care |

Gorham’s Disease: A Rare Cause of Chylothorax in Children.

Gloriana Malavassi; Arturo Solis-Moya, MD; Adriana Fonseca-Portuguez, MD
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UCIMED, San José, Costa Rica


Chest. 2013;144(4_MeetingAbstracts):342A. doi:10.1378/chest.1700957
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Abstract

SESSION TITLE: Critical Care Student/Resident Case Report Posters III

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM

INTRODUCTION: Gorham’s disease (Massive Osteolysis), described in 1954, is an extremely rare disorder of the musculoskeletal system characterized by replacement of normal bone for hypervascular, fibrous-connective tissue and angiomatosis of blood and lymphatic vessels. The disease usually affects several bones in one anatomic region, instead of a single bone. We present the first case of Gorham’s Disease diagnosed in Costa Rica in a pediatric patient.

CASE PRESENTATION: A twelve year old boy presented to the hospital with a three month history of tiredness, progressive dyspnea on exertion and thoracic pain on his left chest with no previous febrile illness, or any other relevant symptom. He was known to the Urology Department for having a scrotal lymphangiomatosis that had been diagnosed when he was four years old.

DISCUSSION: The child had a massive left pleural effusion, drained by serial thoracentesis, once a day, for three consecutive days which failed to drain completely. The fluid had a milky appearance. Biochemical analysis of the fluid reported 519 mg/dL of triglycerides compatible with chylothorax. Despite the addition of a chest drain there was no clinical improvement. A CT scan of his chest and abdomen was performed, where a giant pelvic lymphangioma, disorganization and reduction of the trabecular density of bones, thinning of several vertebrae, involvement of the pelvic bones and the proximal part of the femurs was documented. Gorham’s Disease was diagnosed based on disseminated lymphangiomatosis associated with bone lymphangiomatosis in the presence of a chylothorax. The integrity of the thoracic duct was reassured with a scintigraphy (Technetium-99m), and consequently surgical approach was excluded. The patient received local radiotherapy on his lower abdomen and left lung, resulting in extensive contralateral pulmonary fibrosis. None of these interventions was successful; the chylothorax didn’t resolve, leading to severe effusion, which finally caused respiratory failure and death, 3 months after completing radiotherapy.

CONCLUSIONS: Gorham’s disease is found rarely in children. Chylothorax is the most feared complication in patients with Gorham's disease and is associated with poor prognosis. It is observed frequently when affection of the thoracic cage exists, due to a direct extension of the lymphatic dysplasia to the pleural space or the thoracic duct. This child was given every intervention reported by other authors but failed to respond to any treatment. This is also the first case to describe Gorham’s disease involving systemic lymphangiomatosis, pleura and multiple bones.

Reference #1: Tie ML, Poland GA, Rosenow S. Chylothorax in Gorham’s syndrome: a common complication of a rare disease. Chest 1994; 105; 208-213.

Reference #2: Soto-Martinez M, Massie J. Chylothorax: diagnosis and management in children. Paediatrics Respiratory Reviews 2009; 10; 199-207.

Reference #3: Dipak P. Gorham’s disease or massive osteolysis. Clinical Medicine & Research 2005; 3; 65-74.

DISCLOSURE: The following authors have nothing to disclose: Gloriana Malavassi, Arturo Solis-Moya, Adriana Fonseca-Portuguez

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