A case of Gorham's disease complicated by bilateral chylothorax.
Gorham's disease is a very rare disorder of uncertain aetiology, characterised by non-neoplastic proliferation of vascular or lymphatic channels within bone leading to resorption and replacement of osseous matrix with angiomatous tissue. Fewer than 200 cases have been reported in the literature. The complication of chylothorax is associated with a high morbidity and mortality. We present the case of a 17-year-old male with Gorham's disease of the sternum, clavicle and rib, complicated by bilateral chylothorax. Despite thoracic duct ligation and radiotherapy, the patient progressively deteriorated and died 36 weeks following initial presentation.
Boyle
Matthew J
MJ
Department of Cardiothoracic Surgery, Auckland City Hospital, Auckland, New Zealand.
Alison
Peter
P
Taylor
Graeme
G
Lightbourne
Bernadette A
BA
D000293:Adolescent; D002916:Chylothorax / Q000150:complications* / Q000175:diagnosis* / Q000601:surgery; D003131:Combined Modality Therapy; D018450:Disease Progression; D017809:Fatal Outcome; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D010015:Osteolysis, Essential / Q000150:complications* / Q000175:diagnosis* / Q000601:surgery; D013902:Radiography, Thoracic; D035583:Rare Diseases; D018570:Risk Assessment; D012720:Severity of Illness Index; D013908:Thoracotomy / Q000379:methods; D014057:Tomography, X-Ray Computed