Dr Adeena D S. Dr Suma Balan (Pediatric Rheumatology), Dr C Jayakumar
Department Of Pediatrics
Amrita Hospital Kochi
Ten year old girl presented with complaints of pain abdomen which was non radiating , diffuse, increases with food intake and improves with rest, associated with multiple episodes of vomiting which was non-bilious, containing food particles and occasionaly blood tinged. On day 9 of symptoms , palpable purpura was noted over bilateral lower limbs .USG abdomen showed duodenal wall edema with minimal ascites.
CT abdomen showed diffuse symmetrical circumferential wall thickening and enhancement of the distal jejunum and ileal loops, suggesting GI vasculitits.Hence she was referred here for further management.
No history of respiratory symptoms , seizures ,altered sensorium, joint symptoms , melena, hematuria or decreased urine output.
Past history -1 st born of NCM, born term , 2.6 kg Birth wt , with no history of admissions in the past .
O/e Alert
BP 90/60 mm Hg , HR -90/min, all peripheral pulses well felt, NG tube in situ
Pallor +
Palplable purpura over lower limbs
P/a soft , diffuse tendernesss
No organomegaly
Other systems: within normal limits
Labs neutrophilic leucocytosis (17000) with normocytic anemia , high inflammatory markers, elevated amylase and lipase and 2-3 RBC /hpf in urine.A provisional diagnosis of IgA vasculitis with GI involvement was made and started on IV Methylprednisolone at 10 mg/kg .She had persistent severe abdominal pain and upper GI scopy showed bile in bile in stomach, NJ tube was placed and secretions monitored. And once secretions reduced, started on oral liquid feeds. Repeat CT abdomen showed edematous bowel loops with pneumatosis and portal venous gas, indicating intestinal perforation.
Blood cultures grew Klebsiella species. Antibiotics were hiked . A laparotomy was done and 20 cm of small bowel was resected , creating a loop stoma bowel around 40 cm from the DJ and remaining bowel was placed back. She responded well to surgery and was weaned off ventilator and oral intake begun. Steroids were tapered and MMF was added and was discharged after recovery, with stable vitals and advise for regular follow up.
Henoch-Schönlein Purpura (HSP)- A systemic vasculitis characterized deposition of IgA immune complexes.GI findings may occur in 10-40% of patients even before the rashes.HSP associated intestinal perforation is a rarer compliaction with an estimated prevalence of 0.38%.
HSP Clinical Features:
– Palpable purpura (100%)
– Abdominal pain (75%)
– Arthritis (65%)
– Renal involvement (40-50%)
–
Complications:
– Nephritis (40-50%)
– Gastrointestinal bleeding (10-30%)
– Intussusception (5-10%)
– Orchitis (10%)
– Neurological involvement
–
GI vasculitis presents with
– Abdominal pain (90%)
– Vomiting (50-70%)
– Diarrhea (40-60%)
– Gastrointestinal bleeding (30-50%)
Complications:
– Intussusception (10-20%)
– Bowel perforation (5-10%)
– Stricture formation (5%)
– Malabsorption
Management:
– Supportive care
– Corticosteroids (for severe cases)
– Immunosuppressants (for refractory cases)