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Age / Sex : 14 / M
Chief complaint: mild dyspnea & back pain
pleural fluid analysis: chylothorax
1) What is your impression?
Two weeks later, you can see the final diagnosis with a brief discussion of this case.
(Quiz는 quiz일 뿐이오니 답안은 한개만 보내주시기 바라오며, 복수의 답안을 보내주시는 분은 정답이 포함되어 있더라도 부득이 semi-correct answer로 처리토록 하겠습니다.) Courtesy: 이재혁, 경북대병원 * 최다정답자 및 응모자 시상과 관련해서 '전문의' 및 '전공의' 구분이 필요하오니, 변동이 있으신 분들께서는 홈페이지 '개인 정보'의 수정 또는 답안 제출시 정확한 정보 기입을 부탁드립니다.
Diagnosis: Gorham disease
Discussion
Discussion:
• Proliferation
of vascular or lymphatic structures of bone
→ progressive massive osteolysis & soft tisssue extension
• Child or
young adult
• No sex
predominance
• No
inheritance or familial tendency
• Trauma
history : 57%
• Upper arm,
shoulder girdle, mandible : monocentric or polyostotic involvement (no skip)
• New bone
formation (-)
• Malignant
degeneration : 1 case reported
• MR imaging
: various signal change to reflect the degree of neovascular progression and
fibrosis
• Bone scan :
normal or slight uptake
• Chylothorax,
mediastinal lymphangioma
• Gorham
disease with chylothorax
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Osseous involvements: rib, scapula, clavicle,
thoracic vertebra
-
Thoracic duct invasion or communication of
the lymphatic dysplasia with the pleural cavity
-
Hypoproteinemia, malnutrition,
immunosuppression
-
Conservative treatment: mortality rate 64%
-
Surgical ligation of thoracic duct (right
approach)
References:
1.
Duffy et al. A Case of Gorham Disease with Chylothorax
Treated Curratively with Radiation Therapy. Chest 1994;105:208-213
2.
Jannette Collins. Case 92: Gorham Syndrome. Radiology
2006;238:1066-1069
3.
Patel eta l. Gorham’s Disease or Massive Osteolysis. Clinical
medicine & research, 2005;3(2):65-74
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