Intussusception and spontaneous ileal perforation in Henoch–Schönlein purpura

Abstract

Henoch -Schönlein purpura (HSP) is characterized by IgA-dominant deposition of immune complexes in the walls of small vessels, and is the most common cause of non-thrombocytopenic purpura in children. 1 Acute abdominal pain that is often colicky in nature is one of the most frequent symptoms in HSP. Although systemic corticosteroid therapy is often associated with dramatic improvement in relieving abdominal pain, it is inconclusive whether this treatment can prevent such serious intra-abdominal complications as spontaneous bowel perforations. 2 We describe a child with HSP who required repeated emergency laparotomy for intussusception and for ileal perforation that occurred consecutively during the course of corticosteroid therapy. Case reportA previously healthy 5-year-old boy presented with a 3 day history of joint pains and palpable purpura on both legs. He was diagnosed as having HSP based on a typical purpuric rash without thrombocytopenia (platelet count 348 000/mm 3 ; normal, 16 8000 -341 000/mm 3 ) on day 2. He was admitted because of intermittent abdominal pain on day 4. Routine blood and urine tests showed no abnormal fi ndings except slightly decreased activity of coagulation Factor X III (36.0%; normal, 70 -134%) in the plasma. Throat culture yielded Streptococcus pyogenes . He was placed on i.v. prednisolone (2 mg/kg per day) and ampicillin (100 mg/kg per day, for 5 days). Abdominal pain resolved quickly within 24 h after starting therapy. Purpura on the legs and arthralgias fl uctuated over several days despite treatment. On day 9 he complained of abrupt-onset colicky abdominal pain and emesis. The diagnosis of ileocolic intussusception was made on fl uoroscopy with contrast enema as well as on abdominal ultrasonography, which demonstrated the target sign. After failure to treat the condition by hydrostatic methods, an emergency laparotomy was performed. During surgery a segment of ileum measuring 15 cm in length, from the proximal to the ileocecal valve, showed intussusception. The intussusception was manually reduced. The color of the intestine was dark reddish, suggestive of slight ischemic change. There was no evidence of necrosis or perforation of the rest of the bowel. Appendectomy was also performed because the appendix was adherent to the mesentery. On the fi rst postoperative day famotidine and dried human coagulation factor X III concentrate (50 U/kg per day, i.v., for 3 days) were added to the aforementioned regimen.Although the purpuric rash persisted, the postoperative course was uneventful until day 16, when he again developed intermittent abdominal pain. On the following day he became febrile with a temperature of up to 38.4°C, and his abdomen was distended with diffuse tenderness, rebound, and guarding. Abdominal X-ray in a standing position showed subphrenic free air. Ultrasound indicated ascites uprising predominantly in the right lower quadrant and the Douglas pouch. Subsequent CT scan confi rmed the ultrasound fi ndings, and suggested acute panperitonitis. An emergency laparotomy...

Clinical characteristics and associating risk factors of gastrointestinal perforation in children with IgA vasculitis

“…GP is one of the abdominal complications requiring surgical treatment in IgAV children. Other complications include intussusception, massive gastrointestinal bleeding and intestinal necrosis [ 18 ]. The symptoms of children with GP are relatively serious, and delayed treatment may endanger the life of the patients.…”

Clinical Characteristics and Risk Factors of Gastrointestinal Perforation in Children with Henoch-Schönlein Purpura

“…GP is one of the abdominal complications requiring surgical treatment in HSP children. Other complications include intussusception, massive gastrointestinal bleeding and intestinal necrosis [14]. The symptoms of children with GP are relatively serious, and delayed treatment may endanger the life of the patients.…”

Henoch-Schonlein Purpura with hematochezia in an adult patient

“…12 Persistent severe abdominal pain even after initiating corticosteroid therapy is uncommon and should raise the probability of GI complications like bowel ischemia, intussusception and perforation. 13,14 Intravenous immunoglobulin (IVIG) is an alternative for treatment of steroid-resistant cases. 15,16 Other treatment options for refractory GI cases include plasma exchange and immunosuppressive drugs like azathioprine, cyclophosphamide and cyclosporine.…”