대한흉부영상의학회 Korean Society of Thoracic Radiology

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Weekly Chest CasesCases by Disease Category

Case No : 1205 Date 2020-11-24

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