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Operative Management of Intra-abdominal Lymphatic Malformations in Children: A Single Tertiary Center Experience
Hanna Hyvönen1, Johanna Aronniemi2, Päivi Salminen3
1New Children's Hospital, Department of Pediatric Surgery, Helsinki University Hospital and University of Helsinki, P.O. Box 347 (Stenbäckinkatu 9), 00029 HUS, Helsinki, Finland.
Journal of Pediatric Surgery
|June 18, 2024
Summary
Surgical resection is effective for most large, symptomatic intraperitoneal lymphatic malformations (LMs) in children, with low recurrence rates. These malformations often present acutely, posing a risk of intestinal volvulus.
Area of Science:
- Pediatric Surgery
- Congenital Anomalies
- Surgical Oncology
Background:
- Intra-abdominal lymphatic malformations (LMs) are rare congenital anomalies.
- They are classified into intra- and retroperitoneal types.
- This study focuses on intraperitoneal LMs in children.
Purpose of the Study:
- To evaluate surgical resection outcomes for pediatric intraperitoneal LMs.
- To analyze presentation, management, and recurrence.
- To identify associated genetic mutations.
Main Methods:
- Retrospective review of patients ≤16 years with intraperitoneal LMs (2007-2022).
- Exclusion of retroperitoneal LMs.
- Surgical exploration preferred for large (>5 cm) or symptomatic lesions.
Main Results:
- 12 patients (10 male) with mesenteric or omental LMs diagnosed.
- 83% presented with acute symptoms (pain, vomiting).
- 4 patients experienced intestinal volvulus; 91% achieved complete resection; 8% recurrence.
- 75% of LMs showed PIK3CA mutation.
Conclusions:
- Large, pedunculated macrocystic LMs are often resectable.
- Intraperitoneal LMs present acutely with risk of volvulus.
- Low recurrence and intervention rates observed post-surgery.
