P2.M259: Trust Your Gut: When Intussusception Requires Bowel Resection in Henoch-Schönlein Purpura

Introduction: Henoch-Schönlein purpura (HSP) is an IgA-mediated systemic vasculitis, with multiorgan involvement. In the pediatric population, it is the most common form of systemic vasculitis, with a worldwide incidence of 10-20 cases per 100,000 children/year. It typically affects those 2-10 years old, with 4-7 years old having the highest incidence [1]. Given its systemic nature, many organs can be affected including the skin, kidneys, joints and gastrointestinal tract [2]. With an incidence of 5% in HSP cases, intussusception is an uncommon complication of HSP [3]. We present a case report of intussusception secondary to HSP, that failed surgical reduction and required bowel resection.

Case Description: A 5-year-old female presented from her pediatrician’s office to the Emergency Department (ED) with diffuse non-blanching palpable purpura on bilateral lower extremities and buttocks, with mild periumbilical tenderness to palpation (Figure 1). Three weeks prior, she had been treated for pneumonia. Laboratory workup including urinalysis was significant for hematuria, with no significant proteinuria. She was diagnosed with HSP and discharged home with supportive management and close follow-up with her pediatrician. Three days after this diagnosis, she re-presented to the ED with intermittent abdominal pain, emesis, bloody diarrhea and decreased oral intake. Abdominal x-ray and ultrasound were significant for ileo-ileal intussusception (Figure 2). Non-surgical management with water-soluble contrast enema was unsuccessful. The patient was taken to surgery where manual reduction of the intussusception extracorporeally was unsuccessful, therefore requiring resection of 23.9 cm of incarcerated ischemic bowel and primary anastomosis.

Discussion: Gastrointestinal symptoms are common in HSP, occurring in up to 75% of children, though intussusception is a less frequent phenomenon. Lead points are created by submucosal edema and hemorrhage in the small bowel in HSP [2], unlike ileocolic involvement seen with idiopathic intussusception. Identified risk factors for intussusception development in children with HSP include age less than 6 years old, hematochezia, not receiving glucocorticoid therapy within 72 hours of symptom onset and increased d-dimer levels [3]. Our patient had three of these risk factors.

Non-operative reduction in small bowel intussusception (SBI) is rarely successful. Surgical resection is necessary if manual reduction during operation is not possible, when pathologic lead points are present, or there are bowel complications including ischemia, necrosis or perforation [4-6]. Bowel complications may result from delayed diagnosis and surgery, therefore early surgical referral is recommended in symptomatic SBI [5].

Conclusion: This case report illustrates the importance of considering intussusception as a differential of abdominal pain in children of this age, especially in the setting of known HSP and risk factors. A high suspicion for intussusception is necessary as it is fatal if not diagnosed early, and a delay in surgical intervention of SBI can increase the risk of complications, including need for intestinal resection [4-6].