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Lymphangioleiomyomatosis (LAM) presenting as recurrent pneumothorax in an infant with tuberous sclerosis: treated successfully with sirolimus
  1. Lalit Takia,
  2. Kana Ram Jat,
  3. Anirban Mandal,
  4. Sushil Kumar Kabra
  1. Pediatrics, All India Institute of Medical Sciences, New Delhi, India
  1. Correspondence to Dr Kana Ram Jat, drkanaram{at}


Lymphangioleiomyomatosis (LAM) either sporadic or a part of tuberous sclerosis complex is rare in paediatric age group. Here, we report a case of LAM with tuberous sclerosis in an infant. She was referred to our institute at the age of 4 months as a case of recurrent bilateral pneumothorax requiring intercostal tube drainage. Detailed history revealed that patient was symptomatic since 1 month of age in the form of seizures. She had respiratory symptoms for last 15 days. General physical examination revealed whitish macular patches. Brain imaging was suggestive of cortical tubers and subependymal nodules. The echocardiography showed right atrial rhabdomyoma. Chest CT revealed multiple cysts suggesting LAM. On the basis of above findings, a diagnosis of tuberous sclerosis complex with LAM was made. The infant was started on sirolimus and there was significant clinical and radiological improvement over a period of 2 and half years without any side effects.

  • paediatrics (drugs and medicines)
  • respiratory medicine
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  • Contributors SKK and KRJ have conception and design of case and revised the case report critically for important intellectual content. LT and AM have acquisition of data and drafted the case report. All authors were involved in the clinical management of the case and approved final version of case report.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent Parental/guardian consent obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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