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A Rare Case of Chylothorax in a Patient with Schimmelpenning Syndrome

Identifieur interne : 007175 ( Ncbi/Merge ); précédent : 007174; suivant : 007176

A Rare Case of Chylothorax in a Patient with Schimmelpenning Syndrome

Auteurs : Bettina Schlolaut ; Hans Heinz Schild ; Joachim Pfannschmidt ; Dirk Kaiser

Source :

RBID : PMC:4360691

Abstract

Chylothorax originating in a patient with Schimmelpenning syndrome is rare and poses a problem in diagnosis and treatment. A 22-year-old male was admitted with dyspnea indicative of a large pleural chylous effusion. Besides conservative dietary treatment measures, the chylous effusion was drained (2,000 mL/day). Computed tomography-lymphography after ligation of the thoracic duct and pleurectomy revealed a small collateral flow of chylous fluid toward the chest wall and entering the thorax. Eventually, local radiation therapy with 36 Gy effectively treated the chylothorax. Five months later, an epitheloid angiosarcoma developing from a preexisting cutaneous lesion was detected and treated by surgical resection.


Url:
DOI: 10.1055/s-0034-1394162
PubMed: 25798367
PubMed Central: 4360691

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PMC:4360691

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<p>Chylothorax originating in a patient with Schimmelpenning syndrome is rare and poses a problem in diagnosis and treatment. A 22-year-old male was admitted with dyspnea indicative of a large pleural chylous effusion. Besides conservative dietary treatment measures, the chylous effusion was drained (2,000 mL/day). Computed tomography-lymphography after ligation of the thoracic duct and pleurectomy revealed a small collateral flow of chylous fluid toward the chest wall and entering the thorax. Eventually, local radiation therapy with 36 Gy effectively treated the chylothorax. Five months later, an epitheloid angiosarcoma developing from a preexisting cutaneous lesion was detected and treated by surgical resection.</p>
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<name>
<surname>Schlolaut</surname>
<given-names>Bettina</given-names>
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<surname>Schild</surname>
<given-names>Hans Heinz</given-names>
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<surname>Pfannschmidt</surname>
<given-names>Joachim</given-names>
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<surname>Kaiser</surname>
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<corresp id="CO140116crt-1">
<bold>Address for correspondence </bold>
Joachim Pfannschmidt, MD
<institution>Department of Thoracic Surgery</institution>
<addr-line>Lung Clinic Heckeshorn at HELIOS Hospital Emil von Behring, Walterhöferstr 11, Berlin 14165</addr-line>
<country>Germany</country>
<email>Joachim.Pfannschmidt@helios-kliniken.de</email>
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<pub-date pub-type="epub">
<day>31</day>
<month>10</month>
<year>2014</year>
</pub-date>
<pub-date pub-type="ppub">
<month>12</month>
<year>2014</year>
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<volume>3</volume>
<issue>1</issue>
<fpage>64</fpage>
<lpage>66</lpage>
<history>
<date date-type="received">
<day>14</day>
<month>7</month>
<year>2014</year>
</date>
<date date-type="accepted">
<day>11</day>
<month>8</month>
<year>2014</year>
</date>
</history>
<permissions>
<copyright-statement>© Thieme Medical Publishers</copyright-statement>
</permissions>
<abstract>
<p>Chylothorax originating in a patient with Schimmelpenning syndrome is rare and poses a problem in diagnosis and treatment. A 22-year-old male was admitted with dyspnea indicative of a large pleural chylous effusion. Besides conservative dietary treatment measures, the chylous effusion was drained (2,000 mL/day). Computed tomography-lymphography after ligation of the thoracic duct and pleurectomy revealed a small collateral flow of chylous fluid toward the chest wall and entering the thorax. Eventually, local radiation therapy with 36 Gy effectively treated the chylothorax. Five months later, an epitheloid angiosarcoma developing from a preexisting cutaneous lesion was detected and treated by surgical resection.</p>
</abstract>
<kwd-group xml:lang="en">
<title>Keywords</title>
<kwd>lung</kwd>
<kwd>rare disorders</kwd>
<kwd>pleural disease</kwd>
<kwd>thoracic duct</kwd>
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