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Weekly Chest CasesArchive of Old Cases

Case No : 1138 Date 2019-08-22

  • Courtesy of Min Kyung Jung, Jeong Min Ko, Hyun Jin Park / St. Vincent’s Hospital, The Catholic University of Korea
  • Age/Sex 50 / F
  • Chief ComplaintRecurrent ascites
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4
  • Figure 5
  • Figure 6

Diagnosis With Brief Discussion

Diagnosis
Thoraco-abdominal lymphangiomatosis
Radiologic Findings
Fig 1-2. Lung window setting of the chest CT scan shows interlobular septal thickening of both lower lobes and right middle lobe.
Fig 3-5. Contrast-enhanced mediastinal window setting of the chest CT scan shows peribronchial hypodense soft tissue attenuation in both lower lobes and small-sized hypodense matted lymph nodes in both paratracheal regions.
Fig 6. 1 month later, the contrast-enhanced mediastinal window setting of the abdomen CT scan shows a small amount of right sided pleural effusion and ascites.

Fig 7-9. Unenhanced mediastinal window setting of chest CT scan obtained after a direct lymphangiography shows iodinated oil scattered in surrounding mediastinum as well as collapsed retroperitoneum, right middle lobe and lower lobes. A moderate to large amount of pleural effusion is also seen in right lung.

She underwent video-assisted thoracic surgery resection of right paracardiac lymph node which revealed mild vascular proliferation with nodular lymphoid aggregates. She also underwent diagnostic laparoscopy which yielded chyloperitoneum.

Fig. 7 After direct lymphangiography

Fig. 8 After direct lymphangiography

Fig. 9 After direct lymphangiography

Brief Review
Lymphangiomatosis is an uncommon disease characterized by the diffuse infiltration of lymphangiomas that can occur in any part of the body containing lymphatics. In particular, diffuse pulmonary lymphangiomatosis is caused by lymphangiomas limited to the thorax. The onset of the disease is insidious, and its clinical presentations are nonspecific (e.g., dyspnea, cough, chest pain, milky sputum, fever, shortness of breath, and wheezing); thus, diffuse pulmonary lymphangiomatosis is often misdiagnosed as other chronic respiratory disease. Though pathologic analysis is the main basis of diagnosis in diffuse pulmonary lymphangiomatosis, it is extremely difficult to obtain satisfactory pathologic results in a clinical setting, which can be attributed to the method and site of biopsy as well as the size of the biopsy specimens.
MDCT after direct lymphangiography is well suited to the diagnosis of diffuse pulmonary lymphangiomatosis because it is capable of clarifying the dilatation and proliferation of lymphatic channels to more clearly depict intrathoracic abnormalities. These abnormalities include abnormal distribution of contrast medium, dilatation of lymphatic channels, mediastinal soft-tissue infiltration, peribronchovascular thickening, interlobular septal thickening, diffuse ground-glass opacities, pleural effusion, extrapleural soft-tissue thickening, pericardial effusion and thickened pericardium, multiple lymphadenopathy in mediastinum, and mediastinal, pericardial, and thoracic aeroceles.
Please refer to
Case 819, Case 833, Case 922, Case 1095,
References
1. Diffuse pulmonary lymphangiomatosis Can Respir J 2013;20(1):52-54
2. Diffuse Pulmonary Lymphangiomatosis: MDCT Findings After Direct Lymphangiography AJR 2017;208:300–305
3. World J Gastroenterol 2014 July 7; 20(25): 8320-8324
Keywords
thorax, abdomen, pulmonary lymphangiomatosis, thoraco-abdominal lymphangiomatosis,

No. of Applicants : 58

▶ Correct Answer : 25/58,  43.1%
  • - Kinki University Faculty of Medicine, , Japan MITSURU MATSUKI
  • - Showa University Fujigaoka Hospital , Japan KYOKO NAGAI
  • - Mayo Clinic , United States AKITOSHI INOUE
  • - King Abdulaziz University Hospital , Saudi Arabia Amr M. Ajlan
  • - , Korea (South) HYEYOUNG CHOI
  • - McGill University Health Center , Canada Alexandre Semionov
  • - Vita Hospital , Brazil DIOGO LAGO PINHEIRO
  • - Other , Korea (South) SEONGSU KANG
  • - Seoul National University Hospital , Korea (South) Ju Gang Nam
  • - Oita University, Faculty of Medicine , Japan Fumito Okada
  • - National Center for Global Health and Medicine , Japan MASATOSHI HOTTA
  • - The University of Tokyo Hospital , Japan TOSHIHIRO FURUTA
  • - University of Tsukuba Hospital , Japan HIROAKI TAKAHASHI
  • - CLINIQUE STE CLOTILDE , Reunion PATRICK MASCAREL
  • - Diskapi Yildirim Beyazit Hospital, Ankara , Turkey MERIC TUZUN
  • - Samsung Medical Center , Korea (South) MIN A LEE
  • - Seoul National University Hospital , Korea (South) SE WOO KIM
  • - Chonbuk National University Hospital , Korea (South) KUM JU CHAE
  • - Toranomon Hospital , Japan KAORU SUMIDA
  • - National Center for Global Health and Medicine , Japan HIROSHI TAKUMIDA
  • - , Japan YUMI MAEHARA
  • - Hamamatsu University Hospital , Japan YUKI HAYASHI
  • - Chonnam National University Hwasun Hospital , Korea (South) JONG EUN LEE
  • - the first affiliatited hospital of nanjing medical univercity , China HAI XU
  • - Jiangsu province hospital , China WANGJIAN ZHA
▶ Correct Answer as Differential Diagnosis : 4/58,  6.9%
  • - Ajou University Hospital , Korea (South) HYERIN KIM
  • - University of Tsukuba Hospital , Japan SODAI HOSHIAI
  • - , Japan SOTA MASUOKA
  • - Ichinomiya Nishi Hospital , Japan Takao Kiguchi
▶ Semi-Correct Answer : 3/58,  5.2%
  • - , Korea (South) JANG SEONG WON
  • - , Taiwan NING CHIEN
  • - Korea University Anam Hospital , Korea (South) KYU-CHONG LEE
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Current Editor : Sung Shine Shim, MD, PhD. Email : sinisim@ewha.ac.kr

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