Systemic congenital lymphangiomatosis
Systemic lymphangiomatosis is a rare disease characterized by the exageration of lymphatic channel proliferation, occurring in children and young adults. We describe an extremely rare case of congenital systemic lymphangiomatosis in a newborn who had ascitis and respiratory failure develop immediate...
| Main Authors: | , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Associação Paulista de Medicina
|
| Series: | São Paulo Medical Journal |
| Subjects: | |
| Online Access: | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-31801996000500008&lng=en&tlng=en |
| _version_ | 1818292412904112128 |
|---|---|
| author | Ligia Maria Suppo de Souza Maria Regina Bentlin Eliana Souto de Abreu Carlos Eduardo Bacchi |
| author_facet | Ligia Maria Suppo de Souza Maria Regina Bentlin Eliana Souto de Abreu Carlos Eduardo Bacchi |
| author_sort | Ligia Maria Suppo de Souza |
| collection | DOAJ |
| description | Systemic lymphangiomatosis is a rare disease characterized by the exageration of lymphatic channel proliferation, occurring in children and young adults. We describe an extremely rare case of congenital systemic lymphangiomatosis in a newborn who had ascitis and respiratory failure develop immediately after delivery. Death occurred during the first hour of life. Autopsy findings showed numerous cysts in soft tissues of the cervical area, mediastinum and diaphragm, and several other organs including the liver, spleen, thyroid and kidneys. The severe and diffuse involvement with cysts in both lungs by lymphangiomatosis was associated with poor prognosis and death in our case. |
| first_indexed | 2024-12-13T02:59:33Z |
| format | Article |
| id | doaj.art-8c5372643fd1405f87eaa623aed3e380 |
| institution | Directory Open Access Journal |
| issn | 1806-9460 |
| language | English |
| last_indexed | 2024-12-13T02:59:33Z |
| publisher | Associação Paulista de Medicina |
| record_format | Article |
| series | São Paulo Medical Journal |
| spelling | doaj.art-8c5372643fd1405f87eaa623aed3e3802022-12-22T00:01:53ZengAssociação Paulista de MedicinaSão Paulo Medical Journal1806-946011451278128110.1590/S1516-31801996000500008S1516-31801996000500008Systemic congenital lymphangiomatosisLigia Maria Suppo de Souza0Maria Regina Bentlin1Eliana Souto de Abreu2Carlos Eduardo Bacchi3Universidade Estadual PaulistaUniversidade Estadual PaulistaUniversidade Estadual PaulistaUniversidade Estadual PaulistaSystemic lymphangiomatosis is a rare disease characterized by the exageration of lymphatic channel proliferation, occurring in children and young adults. We describe an extremely rare case of congenital systemic lymphangiomatosis in a newborn who had ascitis and respiratory failure develop immediately after delivery. Death occurred during the first hour of life. Autopsy findings showed numerous cysts in soft tissues of the cervical area, mediastinum and diaphragm, and several other organs including the liver, spleen, thyroid and kidneys. The severe and diffuse involvement with cysts in both lungs by lymphangiomatosis was associated with poor prognosis and death in our case.http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-31801996000500008&lng=en&tlng=enLymphangiomatosisLymphangiomaCongenital lymphangiomatosis |
| spellingShingle | Ligia Maria Suppo de Souza Maria Regina Bentlin Eliana Souto de Abreu Carlos Eduardo Bacchi Systemic congenital lymphangiomatosis São Paulo Medical Journal Lymphangiomatosis Lymphangioma Congenital lymphangiomatosis |
| title | Systemic congenital lymphangiomatosis |
| title_full | Systemic congenital lymphangiomatosis |
| title_fullStr | Systemic congenital lymphangiomatosis |
| title_full_unstemmed | Systemic congenital lymphangiomatosis |
| title_short | Systemic congenital lymphangiomatosis |
| title_sort | systemic congenital lymphangiomatosis |
| topic | Lymphangiomatosis Lymphangioma Congenital lymphangiomatosis |
| url | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-31801996000500008&lng=en&tlng=en |
| work_keys_str_mv | AT ligiamariasuppodesouza systemiccongenitallymphangiomatosis AT mariareginabentlin systemiccongenitallymphangiomatosis AT elianasoutodeabreu systemiccongenitallymphangiomatosis AT carloseduardobacchi systemiccongenitallymphangiomatosis |