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Lymphangiovenous malformation--a rare cause of giant retroperitoneal cyst
M D Ibrarullah1, R Saxena, S S Sikora
1Dept. of Surgical Gastroenterology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India.
Insights
A rare giant retroperitoneal cyst presented as inguino-scrotal swelling in a child. Surgical excision revealed a lymph-angio-venous malformation, highlighting this condition
Area of Science:
- Pediatric Surgery
- Vascular Malformations
- Congenital Abnormalities
Background:
- Retroperitoneal cysts are rare developmental anomalies.
- Congenital inguino-scrotal swelling can indicate underlying retroperitoneal pathology.
- Early diagnosis and management are crucial for pediatric patients.
Observation:
- A five-year-old child presented with rapidly enlarging abdominal swelling.
- Initial manifestation mimicked congenital inguino-scrotal swelling.
- Preoperative ultrasonography identified a multiloculated retroperitoneal cyst.
Findings:
- Emergency surgical exploration was required due to intracystic hemorrhage.
- Histological examination confirmed a lymph-angio-venous malformation.
- Complete excision of the cyst was achieved.
Implications:
- This case underscores the importance of considering retroperitoneal pathology in pediatric inguino-scrotal swelling.
- Lymph-angio-venous malformations, though rare, can present as large retroperitoneal cysts.
- Prompt surgical intervention is vital for managing complications like hemorrhage.
Abstract:
A giant retroperitoneal cyst manifesting as congenital inguino-scrotal swelling to begin with, is reported. The abdominal swelling became clinically obvious at the age of five years because of rapid enlargement over a period of one month. A multiloculated cyst was revealed on preoperative ultrasonography. Intracystic hemorrhage necessitated emergency surgical exploration and excision. Histology of the cyst revealed lymph-angio-venous malformation.