Abstract
Background: Intra-abdominal lymphatic malformations are rare
entities without a clear consensus on management or prognosis. These
malformations are medically and surgically complex and often require a
sophisticated, multi-disciplinary approach to treatment.
Methods: A single quaternary institutional, retrospective chart
review between January 2000-December 2019 was performed on all patients
with an initial diagnosis of a non-solid organ intra-abdominal lymphatic
malformation. Demographics, presentation, treatment, and outcome were
reviewed. Results: 24 patients were identified, 62.5% were
male. Median age at time of diagnosis was 2.5 years old, 33% were
present at birth, 50% presented before 2 years old, and 29% presented
≥10 years of age. 75% were lymphatic (majority macrocystic), 20.8%
were mixed lymphatic-venous malformations (LVM). 54% had other
associated lymphatic or vascular malformations including 4 patients with
PIK3CA-Related Overgrowth Spectrum (PROS), 3 with Generalized Lymphatic
Anomaly, and 2 with Capillary Venous Lymphatic malformation (CVLM).
Patients most often presented with abdominal pain (50%). 95.8%
required an intervention. 87.5% were managed initially with
sclerotherapy. Most interventions were performed for abdominal symptoms
either before three years of age or during adolescence. 41.7% underwent
laparoscopic or open surgery. 8.3% underwent surgery as their primary
therapy. Conclusion: Intra-abdominal lymphatic malformations
have a bimodal distribution pattern of presentation; shortly after birth
and again at puberty. Most patients present with abdominal symptoms and
have other associated vascular anomalies. MRI appears to be the most
accurate modality for treatment planning. Interventional procedures such
as sclerotherapy and medical therapy with sirolimus are safe and
effective and should be considered first line therapies. Surgical
resection is often required when the diagnosis is in question or when
more conservative therapies are no longer efficacious.