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Weekly Chest CasesArchive of Old Cases

Case No : 686 Date 2010-12-20

  • Courtesy of Yoo Na Kim, Chin A Yi / Samsung Medical Center, Sungkyunkwan University School of Medicine
  • Age/Sex 52 / M
  • Chief ComplaintStiffness in both shoulders
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4
  • Figure 5

Diagnosis With Brief Discussion

Diagnosis
Madelung disease
Radiologic Findings
Fig 1. Chest PA shows symmetric bulging soft tissue opacities at both shoulder areas.
Fig 2-5. Transverse and coronal contrast-enhanced CT images show symmetric and extensive subcutaneous fat deposition with fine septation in the lower neck, anterior chest wall, and back of torso.
Brief Review
Madelung disease (also known as Launois-Bensaude syndrome, benign symmetric lipomatosis, and multiple symmetric lipomatosis) is a rare disease of lipodystrophy characterized by the presence of multiple, symmetric, enormous, progressive, unencapsulated fatty accumulations diffusely involving the cheeks, the neck, the upper trunk, the shoulder girdle area, and the upper extremities. Madelung disease has been seen most often in men (male-to-female ratio, 15:1) between 30 and 60 years of age. There is an increased prevalence of this disease in the Mediterranean population, and there is a relationship between this condition and excessive alcohol consumption, particularly red wine. Madelung disease has been associated with alcoholism in 60% to 90% of patients, although the cause of this syndrome is unknown. Hyperuricemia, gout, liver disease, polyneuropathy, diabetes mellitus, glucose intolerance, peripheral insulin resistance, renal tubular acidosis, hypertension, hypothyroidism, and hyperlipidemia have occasionally been identified in patients with Madelung disease, some of whom have no history of alcoholism.
The long-term lipomatous deposits are often large and cosmetically deforming, and the upper aerodigestive tract and great veins may be compressed, which can cause dyspnea, dysphagia, and more serious complications, such as superior vena cava obstruction and sudden death in advanced cases. Despite the cosmetic aspect of the patient with a reduced range of motion of the head and neck, Madelung disease is often asymptomatic in most cases.
The diagnosis is usually made on the basis of history, clinical appearances, and imaging examination. Radiologic manifestations of Madelung disease were multiple and symmetric lipomatosis and calcification/ossification within lipomatous masses. Other imaging findings include a relative paucity of fat in the mediastinum, pericardium, abdomen, and pelvis.
Clinical management of Madelung disease involves abstinence from alcohol; however, there is only a slight regression in the magnitude of lipomatous deposits. Surgery is the other component of treatment. It is important to know the extent and distribution of the abnormally proliferated fat, the compression of abnormal fat tissue to major head and neck vessels, trachea and esophagus, and so on before operation.
References
1. Landis MS, Etemad-Rezai R, Shetty K, Goldszmidt M. Case 143: Madelung disease. Radiology 2009; 250:951-954.
2. Zhang XY, Li NY, Xiao WL. Madelung disease: manifestations of CT and MR imaging. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2008; 105:e57-64.
3. Ahuja AT, King AD, Chan ES, et al. Madelung disease: distribution of cervical fat and preoperative findings at sonography, MR, and CT. AJNR Am J Neuroradiol 1998; 19:707-710.
Keywords
Mediastinum, Metabolic and storage lung disesae,

No. of Applicants : 100

▶ Correct Answer : 65/100,  65.0%
  • - Oita University, Faculty of Medicine , Japan Fumito Okada
  • - Chonnam university hospital , Korea (South) Minyoung Jung
  • - Osaka University , Japan Osamu Honda
  • - Department of Radiology, Chonnam National University Hospital , Korea (South) Hyungjun Kim
  • - seoul national university hospital , Korea (South) Ijin Joo
  • - Shinchon Severance Hospital , Korea (South) Ho-Joon Lee
  • - James Paget Hospital , U.K , United Kingdom nabil mahmood
  • - SNUH , Korea (South) Eun LEE
  • - Inha University Hospital , Korea (South) Ju Won Lee
  • - KHOO TECK PUAT HOSPITAL , Singapore SUMER SHIKHARE
  • - Chonnam university hospital , Korea (South) LEE HYUN
  • - CH de la Ce Basque , France Paul ARDILOUZE
  • - Univ. of Miami/Jackson Memorial , United States mantosh rattan
  • - Hospital M de Deus , Brazil Thiago Bento da Silva
  • - Inha University Hospital , Korea (South) Sung Hee Park
  • - Auckland hospital , New Zealand (Aotearoa) Yuranga Weerakkody
  • - Diskapi Yildirim Beyazit Hospital, Ankara , Turkey Meric Tuzun
  • - Yokohama-asahi-chuo-general hospital , Japan Kyoko Nagai
  • - Gifu Chuo Hospital , Japan Yo Kaneko
  • - Doctors Hospital , Bahamas muneesh sharma
  • - kobe city general hospital , Japan rintaro hashimoto
  • - CHRU lille , France manuel toledano
  • - Youngdong Hospital , Korea (South) Sang Min Lee
  • - Toyama University Hospital, Laboratory of Pathology , Japan TOMONORI TANAKA
  • - Ishikawa Matto Central Hospital , Japan Manabu Akimoto
  • - IRSA La Rochelle France , France Denis Chabassiere
  • - Shinchon Severance , Korea (South) Kim Hee Yeong
  • - Chonnam National University , Korea (South) se hee Jung
  • - PSMC, Karamsad , India Viral patel
  • - National Taiwan University Hospital , Taiwan Li-Ta Keng
  • - Kobe City Medical Center General Hospital , Japan Hitomi Nagano
  • - Siirt Devlet Hastanesi , Turkey Armagan Sarac
  • - Assam Medical college , India samudra borah
  • - Bundang CHA hospital , Korea (South) Jung Jin Young
  • - Chonnam national university hosipital , Korea (South) Byung chan Lee
  • - chonnam national university , Korea (South) Byung Hyun Baek
  • - Chonnam national univ. hospital , Korea (South) Choi Seul Gi
  • - korea univ. hospital, radiology department , Korea (South) choo yung
  • - CNUH , Korea (South) Seoin Jeong
  • - Chonnam National University Hospital , Korea (South) Jung Hyun Kim
  • - Chonbuk National University Hospital , Korea (South) Jisoo Song
  • - All India Institute of medical sciences , India Justin Moses
  • - Hotel-Dieu Montreal , Canada nicolas gautier
  • - CNUH , Korea (South) sunkyoung you
  • - Busan Paik hospital, Inje university , Korea (South) Hye Jin Baek
  • - IRCCS Istituto Oncologico - Bari , Italy Carlo Florio
  • - clinique de SAVOIE , France, Metropolitan gay-depassier philippe
  • - IRSA , France jean-luc BIGOT
  • - Radiologie Brabois adultes , France ROPION helene
  • - Seoul Nationial University Bundang Hospital , Korea (South) Hee Seok Choi
  • - chonnam university hospital , Korea (South) shin hyo hyun
  • - Beaulieu clinic Geneva , Switzerland gilles GENIN
  • - jaslok hospital & research centre mumbai , India JAINENDRA JAIN
  • - KUMC ansan , Korea (South) kihwan kim
  • - NDMVP Nashik , India Imran Jindani
  • - jobless , France jean-baptiste Noel
  • - All India Institute of Medical Sciences , India Ashish Gupta
  • - Shree Diagnostics , India amol jagdale
  • - Chonnam national university hospital , Korea (South) SeungJin Lee
  • - Chonnam national university hospital , Korea (South) Hyeong ook Kim
  • - Chonnam national university hospital , Korea (South) Jang-hyeon Song
  • - CNUH , Korea (South) Hyedoo Jung
  • - Chonnam National University Hospital , Korea (South) Daun Lee
  • - Asan Medial center , Korea (South) Chaehun Lim
  • - Kobe City Medical Center General Hospital , Japan Junko Ochi
▶ Correct Answer as Differential Diagnosis : 20/100,  20.0%
  • - Onomichi municipal hospital , Japan Hirofumi Mifune
  • - Montreal , Canada D J
  • - NTUH , Taiwan Kuei-pin Chung
  • - Goa Medical College , India Paresh Desai
  • - KMC,Manipal , India satish maddukuri
  • - Okayama University , Japan Akihiro Tada
  • - radiologist, aditya imaging centre , India vivek patel
  • - Osaka University , Japan Hiromitsu Sumikawa
  • - Virgin Mary Hospital Burgas , Bulgaria VLADISLAV RUSINOV
  • - Hangang sacred heart hospital , Korea (South) You Mie Han
  • - Kangnam Sacred Heart Hospital , Korea (South) Jaewon Kim
  • - Catholic medical center , Korea (South) hyuna kim
  • - Saga University , Japan Ryoko Egashira
  • - Dongnam Institute of Radiological and Medical Sciences , Korea (South) Dae Wook Yeh
  • - CHU nancy Brabois , France Clent Proust
  • - University of British Columbia , Canada Amr Ajlan
  • - chp st martin , France Mariotte benoit
  • - Hospital Sotiria, Athens, Greece , Greece Vasilios Tzilas
  • - NASA SCANS , India RAKESH BHATIA
  • - Yonsei University College of Medicine, Severance Hospital , Korea (South) Hyung Jin Rhee
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