A rare pulmonary lymphangioleiomyomatosis disease in a male with tuberous sclerosis complex

Respiratory Medicine Case Reports
Ankita KabiSachin Sogal P

Abstract

Pulmonary Lymphangioleiomyomatosis (LAM) is an uncommon disease and may be associated with tuberous sclerosis complex (TSC). LAM is reported to occur exclusively in females of the premenopausal age group. Here we report a rare entity of lymphangioleiomyomatosis in a male patient of tuberous sclerosis, who developed pneumothorax following mechanical ventilation. A young adult presented to the emergency room with history of recurrent seizures since the 6th month of his age. He was intubated in the emergency room for protection of the airway and was initially maintained on manual ventilation using Bain's circuit. Neuroimaging revealed multiple calcified subcortical nodules and giant cell astrocytoma in left lateral ventricle. On the third day of hospitalization, he developed subcutaneous emphysema on his neck and anterior wall of chest. Contrast-enhanced CT chest revealed presence of subcentimetric thin walled cystic lesions in lungs, pneumomediastinum, right sided pneumothorax, and diffuse subcutaneous emphysema. Right sided pneumothorax was managed by intercostal chest tube drainage. CECT abdomen showed well defined heterogeneously enhancing lesions in right kidney suggestive of angiomyolipoma. A final diagnosis of Lymphangiolei...Continue Reading

References

Jan 1, 1995·Chest·M CastroJ H Ryu
Nov 18, 2000·Thorax·S R Johnson, A E Tattersfield
Jan 11, 2008·The New England Journal of Medicine·John J BisslerDavid N Franz
Feb 7, 2008·Chest·Francis X McCormack
Aug 30, 2008·Lancet·Paolo CuratoloSergiusz Jozwiak
Jul 22, 2018·Human Pathology·Kazuma KobayashiHironobu Sasano

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Citations

Feb 26, 2021·BioMed Research International·Xiaotong DongYunxiang Zhang

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Methods Mentioned

BETA
biopsy

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