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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.
Insights
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.
Background:
Intra-abdominal lymphatic malformations (LMs) are relatively rare congenital anomalies and can be divided into intra- and retroperitoneal subgroups. This study aims to evaluate the outcomes after surgical resection of intraperitoneal LMs in children.
Methods:
The records of all patients ≤16 years of age with intraperitoneal LMs managed at our tertiary center between 2007-2022 were reviewed. Patients with retroperitoneal LMs were excluded. Our preferred approach is surgical exploration for large (>5 cm), symptomatic lesions if they are potentially resectable on imaging.
Results:
A total of 12 patients (10 males) were diagnosed with intraperitoneal LMs located in the small bowel mesentery or omentum at a median age of 6.6 (range, 0.6-14.4) years. Ten (83%) patients presented with acute symptoms, including abdominal pain (n = 8; 67%) and vomiting (n = 3; 25%). Two (17%) intraperitoneal LMs were found on imaging performed for other reasons. Surgical exploration was performed in 11/12 (92%) cases. Intestinal volvulus around a mesenteric macrocyst had occurred in 4 (36%) patients. Complete macroscopic resection was achieved in 10/11 (91%) patients. Most lesions were localized to a short intestinal segment. One mixed type LM with an extensive mesenteric involvement was biopsied as the lesion was deemed to be unresectable in preoperative imaging and the diagnosis was initially unclear. There was one recurrence (8%) during postoperative follow-up on imaging (median 3.6 (range, 1.5-6.9) years) but the patient has remained asymptomatic and not undergone interventions. 6/8 (75%) of screened intraperitoneal LMs were positive for a somatic PIK3CA mutation.
Conclusions:
Most large, pedunculated macrocystic LMs in the small bowel mesentery or omental location were amenable to macroscopic resection. Intraperitoneal LMs tend to present with acute symptoms including a risk for intestinal volvulus based on anatomical location. Our results suggest low recurrence rates or need for further interventions over follow-up.
Level Of Evidence:
III.
