대한흉부영상의학회 Weekly Chest Cases

Case No : 1057 Date 2018-01-30

Courtesy of Min Yeong Kim, MD., Tae Jung Kim, MD. / Department of Radiology, Samsung Medical Center.
Age/Sex 48 / M
Chief ComplaintDyspnea on exertion, one year ago

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Diagnosis With Brief Discussion

Diagnosis: Pulmonary Capillary Hemangiomatosis
Radiologic Findings: Fig 1. Posteroanterior chest radiograph reveals convex pulmonary conus and prominent bilateral central pulmonary arteries.
Fig 2. Main pulmonary artery is engorged comparing to ascending aorta on the axial scans of enhanced chest CT.
Fig 3 & 4. On both axial and coronal images (lung window level), innumerable ground glass nodules are diffusely scattering in both lungs without supleural areas. There is no evidence of interlobular septal thickening.
Fig 5. The right cardiac chambers are enlarged and right ventricle is hypertrophic on a four-chamber view of echocardiographic exam, that means pulmonary arterial hypertension with right ventricular hypertrophy.
Brief Review: Pulmonary capillary hemangiomatosis (PCH) is characterized by abnormal proliferation of capillaries within alveolar septa on pathology. The clinical manifestation includes progressive dyspnea, fatigue and chronic cough with gradual development of right heart dysfunction, which are representing as pulmonary arterial hypertension (PAH). The rarity of PCH frequently insults in misdiagnosis to idiopathic PAH, chronic thromboembolic pulmonary hypertension, pulmonary veno-occlusive disease (PVOD), pulmonary fibrosis, sarcoidosis or pulmonary hemosiderosis. Unlike idiopathic PAH caused by elevated medial hypertrophy of the pulmonary arterioles, PAH is explained by the sustained reflection of pressure elevation in capillary bed as in PCH, or PVOD in the pulmonary veins. PCH may be associated with secondary PVOD, and the distinction of PCH from PVOD may be difficult.
The radiologic appearance typically includes features of both PAH and pulmonary parenchymal abnormalities. The chest radiographic findings are enlarged central pulmonary arteries with right ventricular hypertrophy and occasionally visible diffuse or bibasilar reticulonodular or micronodular GGO. On CT, widespread ill-defined centrilobular GGNs are consistently described in PCH, often mixed with lobular GGOs. Mediastinal lymphadenopathy can be associated. In contrast to PVOD, septal line thickening, pleural effusion or mediastinal lymphadenopathy is unusual but may be present due to secondary PVOD. Pulmonary arteriography usually appears normal. In the setting of unexplained PAH, the clinical and radiologic diagnosis of PVOD and PCH can be very difficult, and histologic examination is regarded as the most reliable means to establish the diagnosis.
Although the only curative therapeutic option of PCH or PVOD is heart-lung transplantation, medical therapies serve as supportive care and a bridge to transplantation. Major pharmacological agents are vasodilators to decreased pulmonary vascular resistance, increase cardiac output and reduce volume overload. In many cases, however, potent vasodilators induce florid and even fatal pulmonary edema in patients with PCH or PVOD. Radiologists play an important role in differentiating PCH or PVOD from idiopathic PAH.
References: 1. Frazier AA1, Franks TJ, Mohammed TL, Ozbudak IH, Galvin JR.From the Archives of the AFIP: pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis. Radiographics. 2007;27(3):867-82.
2. Kothari SS1, Jagia P, Gupta A, Singh N, Ray R. Images in cardiovascular medicine. Pulmonary capillary hemangiomatosis.Circulation. 2009 Jul 28;120(4):352-4.
Keywords

No. of Applicants : 108

▶ Correct Answer : 28/108, 25.9%

- Showa university Northern Yokohama Hospital , Japan KOTA WATANABE
- Kinki University Faculty of Medicine, , Japan MITSURU MATSUKI
- MD , India DEEPANDER SINGH RATHORE
- Other , Korea (South) HAYEON LEE
- Soonchunhyang University Hospital Seoul , Korea (South) SUNG HWAN BAE
- 罹stanbul , Turkey AYHAN YILMAZ
- Onomichi municipal hospital , Japan Hirofumi Mifune
- Ajou University Hospital , Korea (South) Taeyang Ha
- HHS , Canada S LEE
- Samsung Medical Center , Korea (South) MIN YEONG KIM
- The University of Tokyo Hospital , Japan RYO KUROKAWA
- Samsung Medical Center , Korea (South) KYOWON GU
- National Taiwan University Hospital Hsin-Chu Branch , Taiwan LI-TA KENG
- Other , Korea (South) JIN YOUNG KIM
- CLINIQUE STE CLOTILDE , Reunion PATRICK MASCAREL
- Other , Korea (South) SEONGSU KANG
- University of Tsukuba, Dept of Radiology , Japan MANABU MINAMI
- Kyemyung University Dongsan Hospital , Korea (South) NAM YEOKYOUNG
- , Korea (South) HYERIN KIM
- Multimagem Diagn泥sticos , Brazil PEDRO PAULO TEIXEIRA E SILVA TORRES
- Escola Paulista de Medicina , Brazil RENATO MASSON DE ALMEIDA PRADO
- , Korea (South) WUNHA LEE
- Fortis hospital , Mohali , India SHALEEN RANA
- Ajou University Hospital , Korea (South) SUBIN HEO
- University of Utah , United States AKIHIKO SAKATA
- the first affiliatited hospital of nanjing medical univercity , China HAI XU
- Samsung Medical Center , Korea (South) HYUN JUNG YOON
- Ajou University Hospital , Korea (South) YOO YOUNGJIN

▶ Correct Answer as Differential Diagnosis : 15/108, 13.9%

- The University of Tokyo Hospital , Japan Akifumi Hagiwara
- Vita Hospital , Brazil DIOGO LAGO PINHEIRO
- Freelance resident in Reunion Island , Reunion Fabien HO
- Beaulieu clinic Geneva , Switzerland gilles GENIN
- The University of Tokyo Hospital , Japan TOSHIHIRO FURUTA
- Asan Medical Center , Korea (South) JOOAE CHOE
- VDC, HYDERABAD , India KARTHIK RAYASAM
- Diskapi Yildirim Beyazit Hospital, Ankara , Turkey MERIC TUZUN
- Azienda Ospedaliera di Cremona , Italy Pietro Sergio
- Sarawak General Hospital , Malaysia POHSEN TAY
- Wuhan Union Hospital , China QIGUANG CHENG
- The University of Tokyo Hospital , Japan TAKU TAJIMA
- Yujin Yamazaki Hospital , Japan MASASHI TAKAHASHI
- Ichinomiya Nishi Hospital , Japan Takao Kiguchi
- Private sector , Greece VASILIOS TZILAS

대한흉부영상의학회 Korean Society of Thoracic Radiology

Archive of Old Cases

Case No : 1057 Date 2018-01-30

Discussion

No. of Applicants : 108