1. Introduction
Intra-abdominal vascular malformations are rare entities, reported in
the literature to have a 1% incidence, with the true incidence likely
higher given small asymptomatic lesions.1–3 There are
three general types of vascular malformations which are seen in
non-solid organs of the abdomen which include venous, lymphatic,
arteriovenous, and combinations such as lymphatic-venous
malformations.4 Lymphatic malformations (LM) occur
during embryonic development as a result of abnormally formed lymphatic
vessels and represent the most common form of low-flow, non-solid organ,
intra-abdominal vascular malformation.1,4
Intraabdominal lymphatic malformations (ILM) are medically and
surgically complex and require a sophisticated, multi-disciplinary
approach to treatment. There are no consensus guidelines on the
management or prognosis of these lesions. Recent studies have
demonstrated that though LM are rare entities, both inpatient charges
and resource utilization as well as mortality are on the rise for
patients with lymphatic malformations.5 Intraabdominal
lymphatic malformations have a known association with other vascular
malformations, especially those involving the trunk. Patients with
CLOVES syndrome (Congenital lipomatous overgrowth, vascular
malformations, epidermal nevi, and scoliosis/skeletal/spinal anomalies
syndrome), a PIK3CA related overgrowth syndrome (PROS), frequently
manifest with involvement/features on the flanks or
abdomen.4,6 Sirolimus therapy has been used to treat
PROS and has demonstrated safety and efficacy, though it is accepted
that it does not cause shrinkage of the malformations, but rather
stabilizes them and reduce symptoms including
pain.4,7–10 The purpose of this study was to
investigate the natural history and clinical course of this disease
process including required and delivered therapies and treatments.