A Little More Than Just Air and Fluid: An Uncommon Case of Hydropneumothorax

Akash Sengupta; Saikat Dutta

doi:10.5005/jp-journals-11007-0105

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A Little More Than Just Air and Fluid: An Uncommon Case of Hydropneumothorax

Akash Sengupta, Saikat Dutta

Keywords : Case report, Diffuse parenchymal lung disease, Hydropneumothorax, Lymphangioleiomyomatosis, Rare interstitial lung diseases, Tuberous sclerosis, Tuberous sclerosis–lymphangioleiomyomatosis

Citation Information : Sengupta A, Dutta S. A Little More Than Just Air and Fluid: An Uncommon Case of Hydropneumothorax. Indian J Chest Dis Allied Sci 2024; 66 (1):15-19.

DOI: 10.5005/jp-journals-11007-0105

License: CC BY-NC 4.0

Published Online: 03-04-2024

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Abstract

Pulmonary lymphangioleiomyomatosis (PLAM) is among one of the rarest diseases classified under diffuse parenchymal lung diseases. Sporadic LAM is extremely rare, while in patients with diagnosed tuberous sclerosis (TSC) incidence of LAM is quite high with few studies finding it in over 30–40% of TSC patients. Here we describe a case of a young female of 25 years of age presenting at a tertiary hospital in rural West Bengal, for the first time with apparently spontaneous hydropneumothorax and without any neurological features suggestive of TSC. When evaluated for the cause of hydropneumothorax, her thoracic imaging raised the suspicion of lymphangioleiomyomatosis of the lung. Thereafter she was screened for radiological and clinical features of TSC which would explain and establish her affliction with PLAM and she was eventually diagnosed as a case of PLAM associated with TSC. This underscores the importance of giving emphasis on apparently unrelated clinical findings; in this case, a papulonodular rash on her face, that may ultimately prove to be key in deciphering the underlying cause of the current clinical manifestations.

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