Management, Trends, and Recommendations for Intra-abdominal Lymphatic Malformations: A Single-Center Retrospective Review.

BACKGROUND

Intra-abdominal lymphatic malformations (ILM) are vascular anomalies that arise from the mesentery, omentum, or retroperitoneum. These may present with abdominal symptoms or may be incidentally discovered on imaging. There is not a clear consensus on management or prognosis. The aim of this study is to describe and characterize common presentations, treatments, and outcomes.

METHODS

A retrospective chart review was performed of non-solid organ ILM followed in tertiary vascular anomalies clinic. Descriptive statistics were used to analyze patient demographics, symptoms, treatment, treatment response, and complications.

RESULTS

Fifty-six patients with ILM were identified. Thirty-seven percent had other associated lymphatic or vascular malformations including PIK3CA-related Overgrowth Syndrome (PROS), Generalized Lymphatic Anomaly, and Capillary Venous Lymphatic Malformation. Most patients present with abdominal symptoms (pain = 32 %, obstruction = 9 %, distention = 7 %). Symptomatic patients were treated with sirolimus (n = 22, 39 %), percutaneous drainage (n = 3, 5 %), sclerotherapy (n = 15, 27 %), and or surgery (up front surgery, n = 15, 27 %). Of those who underwent sclerotherapy, 67 % had resolution of symptoms while only 5 required subsequent surgery for persistent symptoms. Surgery was utilized for patients presenting with acute bowel obstruction and when sclerotherapy failed to resolve symptoms. Of those who underwent surgery, 32 % required bowel resection (n = 7) and one patient required an ostomy.

CONCLUSION

Most patients with ILM present with abdominal symptoms and have other associated vascular anomalies. Interventional procedures, such as sclerotherapy, and medical therapy with sirolimus are safe and effective and should be considered first-line therapies. Surgical resection should be reserved for failure of less invasive therapies and for acute bowel obstruction.

TYPE OF STUDY: Treatment Study.

LEVEL OF EVIDENCE: Level III.