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Weekly Chest CasesArchive of Old Cases

Case No : 1339 Date 2023-06-19

  • Courtesy of Sunyoung Park / Bundang Seoul National University Hospital
  • Age/Sex 43 / F
  • Chief ComplaintDyspnea for 1 year, SpO2 84%
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4
  • Figure 5

Diagnosis With Brief Discussion

Diagnosis
Hereditary Hemorrhagic Telangiectasia (Osler-Rendu-Weber syndrome)
Radiologic Findings
Fig 1. Chest PA shows ill-defined peripheral opacities in both lower lung zones. Coil embolization is suspected in right lower lung zone.
Fig 2-4. CT scans reveals avidly enhancing nodular lesions which are connected with pulmonary artery
and vein.
Fig 5. Left lung pulmonary angiogram shows multiple pulmonary AVMs with feeding artery, aneurysmal sac, and draining vein.
Brief Review
Pulmonary arteriovenous malformations (PAVMs) are characterized by abnormal connections between the pulmonary arteries and veins. The early and accurate recognition of PAVMs is important not only because of the potential for paradoxical embolization to the brain, but also because treatment with percutaneous endovascular embolization is potentially curative. The overwhelming majority of patients with PAVMs have (autosomal dominant) hereditary hemorrhagic telangiectasia (HHT; known also by the eponymous label, Osler-Rendu-Weber syndrome), which is also associated with the presence of mucocutaneous lesions.
On HRCT, PAVMs are diagnosed by identifying at least one feeding artery connected directly to a draining vein. Some lesions may be more complex characterized by a leash of feeding vessels. Patients with feeding arteries exceeding 3 mm in diameter have traditionally been regarded as candidates for endovascular embolization, although this cutoff is somewhat arbitrary and paradoxical emboli may still occur in the context of smaller PAVMs.
References
Webb, Müller and Naidich's High-Resolution CT of the Lung, Sixth Edition, Chap 22
Keywords

No. of Applicants : 96

▶ Correct Answer : 63/96,  65.6%
  • - Ishikawa Matto Central Hospital , Japan MANABU AKIMOTO
  • - Saitama-Sekishinkai Hosptal , Japan MIHOKO YAMAZAKI
  • - , Japan SUZUNE TSUKAMOTO
  • - , Japan HIROAKI ARAKAWA
  • - The University of Tokyo Hospital , Japan JUN KANZAWA
  • - , Japan KYOKO NAGAI
  • - Shiga University of Medical Science , Japan AKITOSHI INOUE
  • - McGill University Health Center , Canada ALEXANDRE SEMIONOV
  • - Healthy Longevity Medical Center , Japan SHIN-ICHI CHO
  • - Vita Hospital , Brazil DIOGO LAGO PINHEIRO
  • - Other , Korea (South) SEONGSU KANG
  • - , Korea (South) JIN YOUNG LEE
  • - Secomedic Hospital , Japan FUMINORI MIYOSHI
  • - Seoul Medical Center , Korea (South) HYUK GI HONG
  • - The University of Tokyo Hospital , Japan WATARU GONOI
  • - Qatif Central Hospital , Saudi Arabia HANI MAKKY ALSALAM
  • - Osaka University , Japan AKINORI HATA
  • - Shimada General Medical Center , Japan HAYATO NOZAWA
  • - Soonchunhyang University Hospital Bucheon , Korea (South) HYEJOO PARK
  • - OITA UNIVERSITY , Japan FUMIKA ISHITOBI
  • - Hallym University Medical Center , Korea (South) JUNGMIN LIM
  • - Presbyterian Medical Center , Korea (South) SOOYEON JEONG
  • - Gifu University Hospital , Japan Yo Kaneko
  • - Hukuda memorial hospital , Japan SHINTARO KANO
  • - Kyoto University Hospital , Japan YASUHISA KURATA
  • - Ajou University Hospital , Korea (South) YOU NA KIM
  • - Other , Korea (South) CHAEHUN LIM
  • - Osaka Metropolitan University Hospital , Japan SHU MATSUSHITA
  • - Chungbuk National University Hospital , Korea (South) MIHYEON PARK
  • - Jichi Medical University, School of Medicine , Japan MITSURU MATSUKI
  • - University of Tsukuba, Dept of Radiology , Japan MANABU MINAMI
  • - Korea University Guro Hospital , Korea (South) LEE DAKYONG
  • - Osaka metropolitan university Hospital , Japan TATSUSHI OURA
  • - Other , Korea (South) SEONG WON JANG
  • - Dokkyo Medical University , Japan HIROAKI ARAKAWA
  • - Chungbuk National University Hospital , Korea (South) PARK JUN HYEON
  • - Seoul National University Hospital , Korea (South) SUNGHO HONG
  • - Chonnam National University Hospital , Korea (South) GYERYEONG PARK
  • - Chonnam National University Hospital , Korea (South) KIM SANG GYUN
  • - Paras hospital, Panchkula , India SHALEEN RANA
  • - Kyoto University , Japan SHO KOYASU
  • - Hyogo Prefectural Kobe Children , Japan SHUHEI NORIMOTO
  • - Yonsei University,Severance Hospital , Korea (South) SEO BUM CHO
  • - Yonsei University,Yongin Severance Hospital , Korea (South) SEJIN JEONG
  • - Kantou Rousai Hospital , Japan KAORU SUMIDA
  • - Ichinomiya Nishi Hospital , Japan Takao Kiguchi
  • - Nerimahikarigaoka hospital , Japan TAKANA HAYASHI
  • - Matsunami General Hospital , Japan TARO TAKEDA
  • - Chonnam National University Hospital , Korea (South) SEUNG WAN KANG
  • - , Japan YUMI MAEHARA
  • - Shiga University of Medical Science , Japan RYO UEMURA
  • - , Japan NOBUYUKI OTANI
  • - , Korea (South) JIYEONG AN
  • - Toyota Kosei Hospital , Japan YUKI HAYASHI
  • - Diagnose.me (BV) / Royal Perth Hospital , Australia YURANGA WEERAKKODY
  • - , Korea (South) HM PARK
  • - Kyoto university , Japan AKIHIKO SAKATA
  • - the first affiliatited hospital of nanjing medical univercity , China HAI XU
  • - Seoul National University Hospital , Korea (South) YELIM CHOI
  • - Other , Korea (South) YONG HO JANG
  • - , Korea (South) JIN WOO YOON
  • - Hanyang University Hospital , Korea (South) SEUNG JIN YOO
  • - Juntendo University , Japan YUTAKA IKENOUCHI
▶ Correct Answer as Differential Diagnosis : 13/96,  13.5%
  • - Chonnam National University Hwasun Hospital , Korea (South) SEONGWOO CHO
  • - Kyoto City Hospital , Japan YUSUKE UTSUNOMIYA
  • - The University of Tokyo , Japan MOTO NAKAYA
  • - , Korea (South) KANGHWI LEE
  • - , Japan YUKIKO USUI
  • - The University of Tokyo Hospital , Japan TOSHIHIRO FURUTA
  • - Samsung Medical Center , Korea (South) HARIM KIM
  • - , Japan KENTARO KOTANI
  • - , Japan SHUNJIRO NOGUCHI
  • - Korea University Anam Hospital , Korea (South) KYU-CHONG LEE
  • - Kyung-hee University Medical Center , Korea (South) HYE SUN RYU
  • - The University of Tokyo Hospital , Japan HIROSHI TAKUMIDA
  • - Jiangsu province hospital , China WANGJIAN ZHA
▶ Semi-Correct Answer : 13/96,  13.5%
  • - Showa university Northern Yokohama Hospital , Japan KOTA WATANABE
  • - , Korea (South) SEONG MIN AHN
  • - NIMS, HYDERABAD , India BHASKAR K
  • - Seoul National University Hospital , Korea (South) HYUNGIN PARK
  • - Oita University, Faculty of Medicine , Japan FUMITO OKADA
  • - Korea University Guro Hospital , Korea (South) HEEJUN PARK
  • - Myongji Hospital , Korea (South) HYOUNG YEOB KIM
  • - Avrasya Hospital/ISTANBUL , Turkey MURAT ULUSOY
  • - , India PRAVIN RAJ
  • - , China MING LU
  • - Seoul National University Bundang Hospital , Korea (South) YOONAH DO
  • - The Catholic University of Korea Yoeuido St. Mary , Korea (South) CHAWOONG JEON
  • - Shaanxi Provincial Forest Industry Staff Hospital , China GUO JIAN WEI
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