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Mediastinal lymphangiomyoma in a child: report of a case
R Kataria1, V Bhatnagar, S D Gupta
1Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi.
Insights
Mediastinal lymphangiomyomas are rare, benign lymphatic malformations that can cause life-threatening respiratory failure in children. This case highlights a pediatric patient with stridor and respiratory distress due to an infiltrative mediastinal mass, confirmed as lymphangiomyoma.
Area of Science:
- Pediatric Surgery
- Thoracic Oncology
- Vascular Malformations
Background:
- Lymphangiomas and lymphangiomyomas are benign lymphatic vessel malformations.
- Mediastinal location is uncommon in children but can be critical.
- These hamartomas can present with significant morbidity.
Observation:
- A 23-month-old boy presented with stridor and recurrent respiratory infections.
- He experienced sudden respiratory failure due to a multiloculated cystic mediastinal mass.
- The mass was extensively infiltrative.
Findings:
- Initial diagnosis was suspected lymphangioma.
- Histological examination confirmed lymphangiomyoma due to smooth muscle proliferation.
- This highlights the importance of detailed histopathology.
Implications:
- Early recognition and diagnosis of mediastinal lymphangiomyoma are crucial.
- Management requires careful consideration due to infiltrative nature.
- Further review of terminology and literature is warranted.
Abstract:
Lymphangiomas and lymphangiomyomas are benign abnormal collections of lymphatic vessels which form a cystic mass. The mediastinum is an uncommon location for these hamartomas in children, but may be life-threatening. We report herein the case of a 23-month-old boy who presented with stridor and a history of recurrent respiratory tract infections. Soon after his admission he suddenly developed respiratory failure and was found to have a multiloculated, cystic mediastinal mass which was initially thought to be a lymphangioma. The mass was extensively infiltrative, and histological examination showed prominent smooth muscle proliferation in the walls of the cysts, confirming a diagnosis of lymphangiomyoma. The terminology and relevant literature on the topic are reviewed following the presentation of this case.