Case of the Month, April 2018

Submitted by  Paulo Serapio, MD, and Thomas Learch, MD.

Presentation

5 year old female presents to the Emergency Department with new bilateral rashes on her lower extremities as well as subsequent severe intermittent abdominal pain over the past two days.  Pain lasts for approximately 5-15 minutes and has become progressively more frequent.  She also reports two episodes of nonbilious, nonbloody emesis.  She has not had any fevers, but reports having a URI approximately one month ago.  Bowel movements have been unremarkable. 

Vital Signs: 

  • Temp: 97.3
  • HR 115
  • RR 26
  • SpO2 100 on RA
Imaging

Abdominal ultrasound is ordered

Ultrasound demonstrates concentric alternating hyperechoic/hypoechic areas in the LUQ.  This “target sign” appearance is highly suggestive of an intussusception.

A follow-up barium enema is ordered to further delineate the location of this intussusception and for possible therapeutic reduction if identified to be colonic/ileo-colic.

Post-evacuation overhead radiograph after barium-enema demonstrates contrast throughout the length of the colon with reflux into the distal small bowel loops.  No colonic or ileocolic intussusception was seen fluoroscopically during the course of the examination.

Due to persistent symptoms, a CT of the abdomen and pelvis was performed the next day.

Coronal CT of the abdomen and pelvis with oral contrast demonstrates an area of small bowel within small bowel in the right lower quadrant, consistent with ileo-ileal small bowel intussusception.

The patient was taken to the OR for diagnostic laparoscopy and possible intussusception reduction, at which point no frank intussusception was seen.  A long segment of distal small bowel appeared hyperemic and edematous, suggesting intermittent intussusception.  No bowel necrosis was seen.  The patient’s condition improved and she was subsequently discharged the next day.

Four days after discharge, the patient had abrupt recurrence of symptoms and re-presented to the emergency room after several episodes of emesis.  A repeat abdominal ultrasound was performed.

Abdominal ultrasound again identifies a target sign with appearance of bowel within bowel in the right abdomen.  A follow-up CT confirms an ileocolic intussusception

Barium enema reduction was attempted but was unsuccessful.

Diagnosis

Diagnostic laparoscopy confirmed ileocolic intussusception with successful intraoperative reduction. Fullness and bogginess of the cecum was noted with multiple areas of serosal petechiae noted. No bowel necrosis was seen and no bowel was resected.

Constellation of findings with the patient’s characteristic bilateral leg rashes and recurrent intermittent intussusceptions support the diagnosis of Henoch-Schonlein Purpura/IgA Vasculitis.

HSP typically occurs in childhood with a peak incidence between 4-6 years of age. It is associated with intussusceptions which are most commonly ileo-ileal in HSP. These are thought to be secondary to edema/hemorrhage acting as a lead point. Patients with severe GI manifestations of HSP should be assessed for significant complications related to ischemia, perforation or gastrointestinal bleeding. The majority of cases spontaneously resolve with conservative management. Other disease manifestations include arthralgia, palpable purpura and renal disease. In the absence of severe renal disease, many cases are self-limiting and can be treated conservatively.

Reference

Rajalakshmi, P. P., & Srinivasan, K. (2015). Gastrointestinal manifestations of Henoch-Schonlein purpura: A report of two cases. World Journal of Radiology, 7(3), 66. doi:10.4329/wjr.v7.i3.66

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