A Little More Than Just Air and Fluid: An Uncommon Case of Hydropneumothorax
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Date
2024-03
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Jaypee Brothers Medical Publishers Pvt. Ltd.
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. Keywords: Case report, Diffuse parenchymal lung disease, Hydropneumothorax, Lymphangioleiomyomatosis, Rare interstitial lung diseases, Tuberous sclerosis, Tuberous sclerosis–lymphangioleiomyomatosis
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Keywords
Case report, Diffuse parenchymal lung disease, Hydropneumothorax, Lymphangioleiomyomatosis, Rare interstitial lung diseases, Tuberous sclerosis, Tuberous sclerosis–lymphangioleiomyomatosis.
Citation
Sengupta A, Dutta S. A Little More Than Just Air and Fluid: An Uncommon Case of Hydropneumothorax. The Indian Journal of Chest Diseases and Allied Sciences. 2024 Mar; 66(1): 15–19