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Vol. 3. Issue 5.
Pages 513-521 (September - October 1997)
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Vol. 3. Issue 5.
Pages 513-521 (September - October 1997)
CASO CLÍNICO
DOI: 10.1016/S0873-2159(15)31127-2
Open Access
Linfangioleimiomatose pulmonar - LAM. Um caso de associação de LAM e angiomiolipoma renal
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1983
Carlos Mendonça*, Selma Batalha**, Orlando Vieira***, Cheong Tak Hong****, Lei Wai Seng****
* Assistente Graduado Pneumologia Serviço de Pneumologia, C.H.C. (Director. Dr. Rui Pato) (ex·) Chefe de Serviço Pneumologia. Unidade de Cuidados Intensivos Polivalente. C.H.C.S.J. (Responsável: Dr. Carlos Mendonça) Serviço de Pneumologia, C.H.C.S.J. (Responsável: Dr. Nelson Diogo)
** Assisente Hospitlar, Serviço de Anatomia Patológica, C. H.C.S.J. (Responsável: Dra. Selma Batalha)
*** Assistente Hospitalar, Serviço de Cirurgia Geral, C.H.C.S.J. (Responsável: Dr. Rui Terra)
**** Médico do Internato Complementar de Pneumologia do C.H.C.S.J., C.H.C.S.J. Centro Hospitalar Conde S Januário - MACAU, C.H.C. Centro Hospitalar de Coimbra
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1983
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RESUMO

Relatase o caso clínico de uma jovem mulber, que é submetida a uma nefrectomia radical por volumoso tumor à direita, tendo verificado tratarse dum angiomiolipoma. Até então sem qualquer queixa, sinal ou sintoma do foro respiratório. No decurso do pós-operatório, fez complicação aparatosa no contexto da qual foi identificado um pneumotorax espontâneo bilateral. Após a alta hospitalar, foram observados e registados vários episódios de pneumotorax espontâneo, unilateral. A partir do episodio inicial desenvolve, lenta e progressivamente, um quadro clínico de dispneia. Apresenta-se a evolução clínica e alguns parâmetros caracterizadores das alterações funcionais respiratórias, ao longo de seis anos, bem como a documentação de TAC-HR do tórax e histológica, que permitiram fundamentar o diagnóstico desta associação de entidades raras.

Palavras chave:
Linfangioleirniomatose pulmonar - LAM
angiomiolipoma
pneumotorax espontâneo bilateral
SUMMARY

We present the case report of a young woman submited to a radical nefrectomy of the right kidney due to the presence of a large tumor diagnosed as an angiomyolipoma. Until such time no respiratory signs and symptoms were referred. Complications during post-op led to the identification of a bilateral spontaneous pneumothorax. After being released from the hospital the patient returned several times due to unilateral spontaneous pneumothorax. After the first of these episodes she developed slowly progressive dyspnea. We present the clinical evolution and respiratory function parameters for a six year follow-up as well as thoracic HR-CT scan documentation and histologic studies that permit the diagnosis of these associated and rare entities.

Keywords:
Key -words
Pulmonary Lymphangioleiomyomatosis - LAM
angiomyolipoma
Bilateral spontaneous pneumothoraces
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Copyright © 1997. Sociedade Portuguesa de Pneumologia/SPP
Pulmonology

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