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Weekly Chest CasesArchive of Old Cases

Case No : 1205 Date 2020-11-24

  • Courtesy of Yoon Ki Cha, Jeung Sook Kim / Dongguk University Ilsan Hospital
  • Age/Sex 57 / M
  • Chief ComplaintNo specific chest symptom
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4

Diagnosis With Brief Discussion

Diagnosis
Bronchopulmonary isomerism (heterotaxy)
Radiologic Findings
Figure 1. Chest radiograph shows bulging contour in right paravertebral area in thoracoabdominal junction and bilateral hyparterial bronchi.
Figure 2-4. Mediastinal and lung setting of contrast-enhanced chest CT images show bilateral pulmonary arteries arching over the main bronchi, indicating bilateral hyparterial bronchi, bilobed both lungs showing left isomerism.

Azygos continuation of inferior vena cava, polysplenia and truncated pancreas are also seen in the upper abdomen. Abominal CT image shows small bowels in right side of abdomen and large bowels in left side of abdomen, indicating malrotation.
Brief Review
Thoracoabdominal visceral relationships are described in terms of situs, depending on the position of the cardiac atria and abdominal viscera relative to the midline. Situs solitus, situs inversus, and situs ambiguous (so-called heterotaxy) have been used to describe the normal, mirror image of normal, and non-mirror-image deviation from the normal left-right visceroatrial configuration, respectively. Visceroatrial situs should include a description of the position of the upper lobe bronchi to their ipsilateral pulmonary arteries-the most reliable imaging feature of bronchial situs, and thus atrial morphology. The morphologic right lung is defined when the upper lobe bronchus is positioned above the ipsilateral pulmonary artery in the hilum (eparterial). The morphologic left lung is supplied by a hyparterial bronchus, which courses below the ipsilateral pulmonary artery.
Heterotaxy syndromes are frequently associated with bronchopulmonary isomerism-an identical pattern of branching and lobar configuration in each lung. Bilateral eparterial and hyparterial bronchi characterize right and left bronchial isomerism (bilateral right- and left-sidedness), respectively. Right isomerism (bilateral right-sidedness) typically manifests with bilateral trilobed lungs and eparterial bronchi, bilateral right atria, right aortic arch, and asplenia (or some functional loss). Right isomerism is commonly associated with severe congenital heart disease with high mortality rates. Left isomerism typically manifests with bilateral bilobed lungs and hyparterial bronchi, bilateral left atria, and polysplenia (although this feature is variable). Other commonly associated (although variable) findings include midline liver and intestinal malrotation in both forms of heterotaxy. Left isomerism may also manifest with azygos or hemiazygos continuation of inferior vena cava and a truncated pancreas.
Please refer to
Case 274, Case 311, Case 573, Case 614,
KSTR imaging conference cases 2010 Sprng  Case 3,
References
Christopher Walker, Jonathan Chung, Muller's Imaging of the Chest: Expert Radiology Series, p.151-153, 2nd edition
Keywords
airway, vascular, congenital, isomerism,

No. of Applicants : 67

▶ Correct Answer : 11/67,  16.4%
  • - , Japan HIROAKI ARAKAWA
  • - Showa University Fujigaoka Hospital , Japan KYOKO NAGAI
  • - McGill , Canada ADRIANA SOFIA MORALES
  • - Mayo Clinic , United States AKITOSHI INOUE
  • - CH de la Cote Basque , France PAUL ARDILOUZE
  • - Tiger Gate Hospital , Japan SHIN-ICHI CHO
  • - Gifu University Hospital , Japan Yo Kaneko
  • - The University of Tokyo Hospital , Japan RYO KUROKAWA
  • - Chungbuk National University Hospital , Korea (South) MIHYEON PARK
  • - Wuhan Union Hospital , China QIGUANG CHENG
  • - the first affiliatited hospital of nanjing medical univercity , China HAI XU
▶ Correct Answer as Differential Diagnosis : 4/67,  6.0%
  • - Saitama-Sekishinkai Hosptal , Japan MIHOKO YAMAZAKI
  • - McGill University Health Center , Canada Alexandre Semionov
  • - The University of Tokyo Hospital , Japan TOSHIHIRO FURUTA
  • - , Saudi Arabia RAWAN AHMED ALJEFRI
▶ Semi-Correct Answer : 26/67,  38.8%
  • - Kinki University Faculty of Medicine, , Japan MITSURU MATSUKI
  • - Chungbuk National University Hospital , Korea (South) YEONGTAE PARK
  • - The University of Tokyo Hospital , Japan MOTO NAKAYA
  • - Vita Hospital , Brazil DIOGO LAGO PINHEIRO
  • - , Turkey MEHMET DURAN
  • - , Turkey MURAT TEPE
  • - DELTA CARE HOSPITAL, THANJAVUR, INDIA , India SIVARAJA SUBRAMANIAM
  • - , Korea (South) JIN YOUNG LEE
  • - Seoul Medical Center , Korea (South) HYUK GI HONG
  • - National Center for Global Health and Medicine , Japan MASATOSHI HOTTA
  • - Asan Medical Center , Korea (South) JIEUN KIM
  • - Osaka City General Hospital , Japan SHU MATSUSHITA
  • - Diskapi Yildirim Beyazit Hospital, Ankara , Turkey MERIC TUZUN
  • - Other , Korea (South) MINSU KIM
  • - University of Tsukuba, Dept of Radiology , Japan MANABU MINAMI
  • - Chonbuk National University Hospital , Korea (South) KUM JU CHAE
  • - Asan Medical Center , Korea (South) JIHOON KIM
  • - , Japan HIROAKI ARAKAWA
  • - Other , Korea (South) KYU-CHONG LEE
  • - University of Tsukuba Hospital , Japan SODAI HOSHIAI
  • - Toranomon Hospital , Japan KAORU SUMIDA
  • - Ichinomiya Nishi Hospital , Japan Takao Kiguchi
  • - , Japan KAZUMA TERAUCHI
  • - , Japan YUMI MAEHARA
  • - Chonnam National University Hwasun Hospital , Korea (South) WONGI JEONG
  • - Jiangsu province hospital , China WANGJIAN ZHA
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