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Boerhaave Syndrome, Pneumothorax, and Chylothorax in a Critically Ill Patient with Tuberous Sclerosis Complex

Tuberous sclerosis complex (TSC) is an autosomal dominant, variably expressed multisystem disease. The predominant pulmonary features of TSC are identical to those of lymphangioleiomyomatosis (LAM). Pneumothorax, multifocal micronodular pneumocyte hyperplasia, and chylothorax are rare complications...

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Autores principales: Ijaz, Mohsin, Rafiq, Arsalan, Venkatram, Sindhaghatta, Diaz-Fuentes, Gilda
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi Publishing Corporation 2015
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4624888/
https://www.ncbi.nlm.nih.gov/pubmed/26550497
http://dx.doi.org/10.1155/2015/509094
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author Ijaz, Mohsin
Rafiq, Arsalan
Venkatram, Sindhaghatta
Diaz-Fuentes, Gilda
author_facet Ijaz, Mohsin
Rafiq, Arsalan
Venkatram, Sindhaghatta
Diaz-Fuentes, Gilda
author_sort Ijaz, Mohsin
collection PubMed
description Tuberous sclerosis complex (TSC) is an autosomal dominant, variably expressed multisystem disease. The predominant pulmonary features of TSC are identical to those of lymphangioleiomyomatosis (LAM). Pneumothorax, multifocal micronodular pneumocyte hyperplasia, and chylothorax are rare complications of TSC. We report a young male with pneumothorax, lung nodules, and chylous effusion who developed empyema thoracis after esophageal rupture. Hospital course was complicated by respiratory failure. Family opted to transfer to hospice care. Chylothorax is a rare complication of TSC with few scattered reports mostly in female patients. Patients with TSC are usually managed by multispecialists and it is important to be aware of the rare pulmonary manifestations of this disease. A male patient with TSC having lung nodules presenting with chylothorax and empyema thoracis from Boerhaave syndrome makes our case unique.
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spelling pubmed-46248882015-11-08 Boerhaave Syndrome, Pneumothorax, and Chylothorax in a Critically Ill Patient with Tuberous Sclerosis Complex Ijaz, Mohsin Rafiq, Arsalan Venkatram, Sindhaghatta Diaz-Fuentes, Gilda Case Rep Crit Care Case Report Tuberous sclerosis complex (TSC) is an autosomal dominant, variably expressed multisystem disease. The predominant pulmonary features of TSC are identical to those of lymphangioleiomyomatosis (LAM). Pneumothorax, multifocal micronodular pneumocyte hyperplasia, and chylothorax are rare complications of TSC. We report a young male with pneumothorax, lung nodules, and chylous effusion who developed empyema thoracis after esophageal rupture. Hospital course was complicated by respiratory failure. Family opted to transfer to hospice care. Chylothorax is a rare complication of TSC with few scattered reports mostly in female patients. Patients with TSC are usually managed by multispecialists and it is important to be aware of the rare pulmonary manifestations of this disease. A male patient with TSC having lung nodules presenting with chylothorax and empyema thoracis from Boerhaave syndrome makes our case unique. Hindawi Publishing Corporation 2015 2015-10-15 /pmc/articles/PMC4624888/ /pubmed/26550497 http://dx.doi.org/10.1155/2015/509094 Text en Copyright © 2015 Mohsin Ijaz et al. https://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Ijaz, Mohsin
Rafiq, Arsalan
Venkatram, Sindhaghatta
Diaz-Fuentes, Gilda
Boerhaave Syndrome, Pneumothorax, and Chylothorax in a Critically Ill Patient with Tuberous Sclerosis Complex
title Boerhaave Syndrome, Pneumothorax, and Chylothorax in a Critically Ill Patient with Tuberous Sclerosis Complex
title_full Boerhaave Syndrome, Pneumothorax, and Chylothorax in a Critically Ill Patient with Tuberous Sclerosis Complex
title_fullStr Boerhaave Syndrome, Pneumothorax, and Chylothorax in a Critically Ill Patient with Tuberous Sclerosis Complex
title_full_unstemmed Boerhaave Syndrome, Pneumothorax, and Chylothorax in a Critically Ill Patient with Tuberous Sclerosis Complex
title_short Boerhaave Syndrome, Pneumothorax, and Chylothorax in a Critically Ill Patient with Tuberous Sclerosis Complex
title_sort boerhaave syndrome, pneumothorax, and chylothorax in a critically ill patient with tuberous sclerosis complex
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4624888/
https://www.ncbi.nlm.nih.gov/pubmed/26550497
http://dx.doi.org/10.1155/2015/509094
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