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Hemangioma – A pointer to Abernethy syndrome?


Department of Cardiology, All India Institute of Medical Sciences, New Delhi, India

Correspondence Address:
Dr. Shyam S Kothari
Department of Cardiology, Cardio-Thoracic Centre, All India Institute of Medical Sciences, Ansari Nagar, New Delhi - 110 029
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/apc.APC_91_20

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Year : 2020  |  Volume : 13  |  Issue : 3  |  Page : 269-271

 

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Diffuse pulmonary arteriovenous malformations or pulmonary arterial hypertension (PAH) may result from congenital portosystemic venous shunts. Hemangioma as a physical sign of congenital portosystemic shunts (like Abernethy syndrome) has not been described. I report two children (one with severe cyanosis from pulmonary arteriovenous malformations and the other with severe PAH) with cutaneous hemangioma and Abernethy syndrome. Hemangioma may be a clinical pointer to portosystemic shunts even in the absence of obvious liver disease.






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Department of Cardiology, All India Institute of Medical Sciences, New Delhi, India

Correspondence Address:
Dr. Shyam S Kothari
Department of Cardiology, Cardio-Thoracic Centre, All India Institute of Medical Sciences, Ansari Nagar, New Delhi - 110 029
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/apc.APC_91_20

Rights and Permissions

Diffuse pulmonary arteriovenous malformations or pulmonary arterial hypertension (PAH) may result from congenital portosystemic venous shunts. Hemangioma as a physical sign of congenital portosystemic shunts (like Abernethy syndrome) has not been described. I report two children (one with severe cyanosis from pulmonary arteriovenous malformations and the other with severe PAH) with cutaneous hemangioma and Abernethy syndrome. Hemangioma may be a clinical pointer to portosystemic shunts even in the absence of obvious liver disease.






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