(199) Constipation-Induced Thrombosis and Compartment Syndrome in an Adolescent With VACTERL Association

Introduction: VACTERL association is a collection of congenital malformations, including vertebral, cardiac, renal, and limb abnormalities, plus anal atresia and tracheoesophageal fistula. Anal atresia is present in 55-90% of patients with VACTERL association, and many develop chronic constipation as a sequela. Chronic constipation can result in fecal impaction, which can compress neighboring neurovascular structures and the gastrointestinal and genitourinary tracts.

Description: A 14-year-old female with VACTERL association and history of chronic constipation on chronic bowel regimen was admitted to the pediatric intensive care unit with progressively worsening abdominal pain for 5 days and altered mental status for 2 days. She had 2 prior abdominal surgeries, cecostomy, and diminished colonic peristalsis. Initial exam revealed a tense, distended abdomen and diminished lower extremity pulses. Abdominal X-ray showed significant stool burden. Abnormal lab findings were lactic acidosis, transaminitis, and acute kidney injury. Electroencephalogram showed diffuse encephalopathy and increased risk of focal-onset seizure. Computed tomography scan of abdomen showed an 8x10x20 cm stool ball in the rectal vault, compromising blood flow to the lower extremities. Doppler ultrasound (U/S) revealed a nonocclusive clot in the left proximal superficial femoral artery and no flow to the right posterior tibial, right dorsalis pedis, left anterior tibial, or left dorsalis pedis arteries. Heparin was initiated and manual dis-impaction was performed in the operating room to relieve stool burden. The patient then developed compartment syndrome in 4 compartments of the bilateral lower extremities. Emergent fasciotomies of both extremities were performed and 2 debridements were subsequently required. Following interventions, doppler U/S showed improved blood flow to affected vessels. She was transitioned to Xarelto for long-term anticoagulation prior to discharge.

Discussion: So far, only two adult cases have been reported of abdominal compartment syndrome and lower limb ischemia resulting from fecal impaction. This is the first reported pediatric patient with VACTERL association who developed lower limb compartment syndrome from fecal impaction, highlighting the importance of appropriate chronic constipation management.