Life-Threatening Lower Gastrointestinal Hemorrhage in Pediatric Crohn's Disease
A 13 years old boy was admitted to the hospital because of gross hematochezia. There was nothing significant in the patient's past medical history over the past 6 months. He reported suffering from intermittent diarrhea and abdominal pain. Also, he went from 47 kg to 40 kg during the course of 3 months. He was initially believed to have hematochezia and therefore a colonoscopy was performed. After the colonoscopy, he was sent to our hospital with suspicion of inflammatory bowel disease. At his first visit, his blood pressure, pulse rate, respiratory rate, body temperature, height and body weight were 110/65 mm/Hg, 90/min, 20/min, 36.5℃, 164.2 cm (25-50 p) and 40 kg (5-10 p), respectively. He appeared chronically ill and was pale. The physical examination upon admission showed slight pain and tenderness at the peri-umbilical region without muscular guarding. His bowel sounds were normal and the rectal examination revealed an empty ampulla with no other peculiar symptoms regarding peri-anal abscess or fistula. The laboratory investigations during the first exam were as follows: erythrocyte sedimentation rate (ESR) 84 mm/h, C-reactive protein 106 mg/L (normal 0-8 mg/L), albumin 2.8 g/dL, prothrombin time 11.5 sec, partial thromboplastin time 28.1 sec, hemoglobin 10.4 g/dL, leucocytes 9.8×109/L with normal leukocyte differentiation, and thrombocytes 489×109/L. A stool occult blood test was also performed and found to be positive. The colonoscopy showed an inflammatory bowel with multiple linear deep ulcerations, cobble stone appearances and pseudo-polyps (). In addition, in the MRI enterography, a diffuse bowel wall thickening was observed at the distal to terminal ileum, cecum and rectosigmoid colon. Accordingly, he was diagnosed with Crohn's disease. His Pediatric Crohn's disease Activity Index (PCDAI) score was 68. Initially, induction therapy was performed with oral steroids (30 mg/day) and Azathioprine (100 mg/day). He was also given 8weeks of EEN with a polymeric formula (Monowell 2,000 mL daily). After medical therapy, the abdominal pain has declined slightly and the ESR and C-reactive protein (CRP) was reduced but intermittent mild bloody stools were still observed. After the 10th day of hospitalization, approximately 1,000 mL of a fresh bloody stool and dizziness with about a 10-second loss of consciousness had occurred. At this time, his blood pressure had dropped to 80/40 mmHg. In addition, his hemoglobin levels dropped from 10.4 g/dL to 7.6 g/dL. Accordingly, 6 units blood transfusion was performed over a 24 hour time period. After the blood transfusion, his vital signs had stabilized. Based on the patient's past history and the colonoscopy data, we presumed that this gastrointestinal hemorrhage was caused by Crohn's disease. At that point, oral steroid treatment was changed to intravenous steroids and the patient was placed under conservative care. However, even after 10 days, due to over 500 mL of continuous massive hematochezia and repetitive hemoglobin drop, blood transfusion was needed. Thus, a bleeding focus evaluation was performed. From the GI bleeding scintigraphy, the radiotracer was found to be up taken in the mid to left upper quadrant in the abdomen and no abnormal signs were observed in the abdomen CT. A duodenal ulcer was observed in the upper endoscopy but no other acute findings were made. A colonoscopy was then performed and multiple active ulcerations in the distal ileum and inflammatory polyps and cobble stone appearance were found (). Ulcers and bleeding was also expected to be found; however, current active bleeding was not observed and no other bleeding focus was present. For 3 weeks, even with steroid induction and azathioprine therapy, mucosal healing was not successful. The PCDAI score was also 60, which was indicative of continued severe disease activity. Since steroid therapy was ineffective, infliximab infusion (5 mg/kg) was initiated. On the 21st day, a superior mesenteric angiography was performed. In this test, no active bleeding was observed; however, a hypervascular blush, which caused by inflammatory changes, was observed near the proximal ascending colon and ileocecal (IC) valve. To account for this, 2 superior mesenteric arteries were selected using a microcatheter and gel foam arterial embolization was successfully performed ( and ). A hematochezia was found nowhere and the PCDAI score had decreased by 20 points. Starting on the 27th day, mucosal healing and bleeding was thought to be well managed from infliximab infusion and embolization and maintenance therapy was performed using mesalazine (4,000 mg/day). 2 weeks after the first infliximab injection, which corresponded to the 34th day of hospitalization, valve, a second infusion of infliximab (5 mg/kg) was done. However, 650 mL of massive hematochezia was observed on the 35th day of hospitalization. For the first 24 hours, the patient was given 2 units of blood. However, even with active transfusion, the massive rectal hemorrhage continued and his hemoglobin levels dropped from 12.6 gm/dL to 11.6 gm/dL. At this point, we deemed it necessary to perform surgery. To examine the precise operation scale, a second angiography was performed before the operation. The superior mesenteric artery angiography showed a hypervascular blush near the proximal ascending colon and IC valve; however, no active bleeding was observed (). On the 36th day of hospitalization, an ileocecectomy and hand sewn end to side anastomosis were performed. During the operation, wall edema and inflammation was found on the IC valve which was about 30 cm deep into the terminal ileum. An ileostomy was initiated to observe the inside of the lumen and a cobble like appearance, mucosal ulceration and dark blood clot was found. The lumen of the cecum was relatively clean and inflammation was not severe. Although a single bleeding point was not identified, the hemorrhage presumably originated from the diffusely multifocal ulcerated ileal mucosa. Approximately 30 cm of the terminal ileum and cecum together were cut and an ileocecectomy was performed as well as a hand swen end to side anastomosis (). According to the pathologic examination, the ileocecum was erythematous and multiple ulceration, transmural inflammation with perforation and small non-caseating granuloma were observed, a condition that was compatible with Crohn's disease. No colonic diverticula were identified. After the ileocecectomy, the patient's bleeding promptly ceased and he did not require additional blood transfusions. On the 6th postoperative day, a clear liquid diet was started and on the 7th postoperative day, a dark brown colored stool was evident; however, no more hemorrhage episodes occurred and the abdominal pain was managed, which resulted in a steady increase in food consumption. The daily dose of prednisolone was set at 30 mg/day initially and was decreased according to the clinical activity of the disease. On the 12th postoperative day, the PCDAI score was 15 and the wound had cleared; therefore, the patient was discharged. Currently, the patient is in maintenance therapy and receives only infliximab. After the hospitalization, the PCDAI score remained below 10 and disease activity was controlled. The patient has no recurrence of bleeding or evidence of Crohn's disease and is currently in good condition.