Splenic Involvement in Hereditary Hemorrhagic Telangiectasia

A 33-year-old man who presented with prolonged epigastric pain was referred to our hospital. He had experienced recurrent epistaxis and had a family history of hereditary hemorrhagic telangiectasia. Computed tomography and magnetic resonance imaging revealed splenomegaly and a 9 cm hypervascular mas...

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Main Authors: Susumu Takamatsu, Kota Sato, Shunsuke Kato, Hiroto Nagano, Shunro Ohtsukasa, Yasuyuki Kawachi
Format: Article
Language:English
Published: Wiley 2016-01-01
Series:Case Reports in Medicine
Online Access:http://dx.doi.org/10.1155/2016/3212947
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author Susumu Takamatsu
Kota Sato
Shunsuke Kato
Hiroto Nagano
Shunro Ohtsukasa
Yasuyuki Kawachi
author_facet Susumu Takamatsu
Kota Sato
Shunsuke Kato
Hiroto Nagano
Shunro Ohtsukasa
Yasuyuki Kawachi
author_sort Susumu Takamatsu
collection DOAJ
description A 33-year-old man who presented with prolonged epigastric pain was referred to our hospital. He had experienced recurrent epistaxis and had a family history of hereditary hemorrhagic telangiectasia. Computed tomography and magnetic resonance imaging revealed splenomegaly and a 9 cm hypervascular mass in his spleen. Computed tomography also showed a pulmonary arteriovenous malformation and heterogeneous enhancement of the liver parenchyma, suggesting the presence of arteriosystemic shunts and telangiectases. Based on these findings, the patient was definitely diagnosed with hereditary hemorrhagic telangiectasia according to Curaçao criteria. He underwent splenectomy, and his symptoms disappeared after surgery. Pathological examination of the resected specimen revealed that the hypervascular lesion of the spleen was not a tumor but was composed of abnormal vessels associated with hereditary hemorrhagic telangiectasia. Symptomatic splenic involvement may be a rare manifestation of hereditary hemorrhagic telangiectasia but can be revealed by imaging modalities.
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institution Kabale University
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spelling doaj-art-2e926cb3d0244c57b4344f5c050c4f1e2025-02-03T01:07:14ZengWileyCase Reports in Medicine1687-96271687-96352016-01-01201610.1155/2016/32129473212947Splenic Involvement in Hereditary Hemorrhagic TelangiectasiaSusumu Takamatsu0Kota Sato1Shunsuke Kato2Hiroto Nagano3Shunro Ohtsukasa4Yasuyuki Kawachi5Department of Surgery, Musashino Red Cross Hospital, 1-26-1 Kyonan-cho, Musashino-shi, Tokyo 180-8610, JapanDepartment of Surgery, Musashino Red Cross Hospital, 1-26-1 Kyonan-cho, Musashino-shi, Tokyo 180-8610, JapanDepartment of Surgery, Musashino Red Cross Hospital, 1-26-1 Kyonan-cho, Musashino-shi, Tokyo 180-8610, JapanDepartment of Surgery, Musashino Red Cross Hospital, 1-26-1 Kyonan-cho, Musashino-shi, Tokyo 180-8610, JapanDepartment of Surgery, Musashino Red Cross Hospital, 1-26-1 Kyonan-cho, Musashino-shi, Tokyo 180-8610, JapanDepartment of Surgery, Musashino Red Cross Hospital, 1-26-1 Kyonan-cho, Musashino-shi, Tokyo 180-8610, JapanA 33-year-old man who presented with prolonged epigastric pain was referred to our hospital. He had experienced recurrent epistaxis and had a family history of hereditary hemorrhagic telangiectasia. Computed tomography and magnetic resonance imaging revealed splenomegaly and a 9 cm hypervascular mass in his spleen. Computed tomography also showed a pulmonary arteriovenous malformation and heterogeneous enhancement of the liver parenchyma, suggesting the presence of arteriosystemic shunts and telangiectases. Based on these findings, the patient was definitely diagnosed with hereditary hemorrhagic telangiectasia according to Curaçao criteria. He underwent splenectomy, and his symptoms disappeared after surgery. Pathological examination of the resected specimen revealed that the hypervascular lesion of the spleen was not a tumor but was composed of abnormal vessels associated with hereditary hemorrhagic telangiectasia. Symptomatic splenic involvement may be a rare manifestation of hereditary hemorrhagic telangiectasia but can be revealed by imaging modalities.http://dx.doi.org/10.1155/2016/3212947
spellingShingle Susumu Takamatsu
Kota Sato
Shunsuke Kato
Hiroto Nagano
Shunro Ohtsukasa
Yasuyuki Kawachi
Splenic Involvement in Hereditary Hemorrhagic Telangiectasia
Case Reports in Medicine
title Splenic Involvement in Hereditary Hemorrhagic Telangiectasia
title_full Splenic Involvement in Hereditary Hemorrhagic Telangiectasia
title_fullStr Splenic Involvement in Hereditary Hemorrhagic Telangiectasia
title_full_unstemmed Splenic Involvement in Hereditary Hemorrhagic Telangiectasia
title_short Splenic Involvement in Hereditary Hemorrhagic Telangiectasia
title_sort splenic involvement in hereditary hemorrhagic telangiectasia
url http://dx.doi.org/10.1155/2016/3212947
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