Neutropenic Enterocolitis as a Presenting Complication of Acute Lymphoblastic Leukemia: An Unusual Case Marked by Delayed Perforation of the Descending Colon By David B. Wilson, Aarati Rao, Monica Hulbert, Kerry P. Mychaliska, Lori Luchtman-Jones, D. Ashley Hill, and Robert P. Foglia St Louis, Missouri Neutropenic enterocolitis (NE) is a life-threatening complica- tion most commonly seen in patients receiving intensive chemotherapy for acute leukemia. The condition usually af- fects the terminal ileum, cecum, or ascending colon. In rare instances, NE may occur before the initiation of chemother- apy or involve more distal bowel. The authors report the case of a 2-year-old girl who had NE affecting the descending colon as a presenting complication of acute lymphoblastic leukemia. Despite aggressive medical interventions, includ- ing granulocyte infusions, she had a delayed bowel perfora- tion that was managed successfully with surgery. This case highlights the challenges of treating patients who have NE as an initial manifestation of acute leukemia. J Pediatr Surg 39:E25. © 2004 Elsevier Inc. All rights re- served. INDEX WORDS: Acute lymphoblastic leukemia, bowel perfo- ration, enterocolitis, neutropenia, typhlitis. N EUTROPENIC ENTEROCOLITIS (NE) is a seri- ous complication that occurs in patients who have severe neutropenia. 1,2 NE is characterized by transmural inflammation of the bowel wall, which may progress to necrosis and perforation. 1,2 Usually the condition is lo- calized to the terminal ileum, cecum, or ascending colon, although in rare circumstances more distal bowel may be involved. 1 NE occurs most often in patients receiving intensive chemotherapy for acute leukemia, but it has been observed in patients who are neutropenic from other causes. 1-4 We report the case of a child who had NE affecting the descending colon as a presenting compli- cation of acute lymphoblastic leukemia (ALL). Despite aggressive medical management, including granulocyte infusions, she had a delayed bowel perforation necessi- tating surgical intervention. CASE REPORT The patient was a 2-year-old girl admitted to hospital with fever and vomiting. Vital signs included a temperature of 38.9°C, pulse of 160, respiratory rate of 22, and and blood pressure of 115/73. Her examination was notable for pallor, a functional murmur, and molluscum contagiosum. Her abdomen was nontender. Initial laboratory findings included a white blood cell count of 1400/L with 100% lymphocytes, hemoglobin of 6.1 g/dL, and platelet count of 58,000/L. Blood and urine cultures were negative. Bone marrow aspiration found L1 lymphoblasts that expressed TdT, CD19, and CD79a, establishing a diagnosis of precursor-B ALL. Cerebral spinal fluid (CSF) cytology was negative. Bone marrow cytoge- netics showed hyperdiploidy with trisomies of chromosomes 4 and 10, favorable prognostic features associated with a 4-year event free survival of greater than 95% with antimetabolite chemotherapy. 5 Despite empiric antibiotic therapy with ceftazidime, she had abdom- inal pain, distension, and bilious vomiting before the start of chemo- therapy. Ultrasound scan showed ascites and intestinal wall thickening. She was treated with bowel rest and nasogastric drainage, and the antibiotic regimen was broadened to include ceftazidime, vancomycin, clindamycin, gentamicin, and fluconazole. Hours later, she had an acute decompensation marked by tachycardia, hypotension, and increasing respiratory distress. She required mechanical ventilation for 1 week. A catheter was placed in the left chest to drain a sterile pleural effusion. Computed tomography (CT) scan of the abdomen showed marked ascites and diffuse bowel wall thickening that was most pronounced in the colon. Over the next few days, erythema and induration were evident over the left lateral abdominal wall without guarding or rigidity. Serial abdominal films showed scattered air-fluid levels, but no pneumatosis intestinalis or free intraperitoneal air. Doppler ultrasonog- raphy showed a catheter-associated thrombus in the left common femoral vein, which was treated with low-molecular-weight heparin and catheter removal. She started induction chemotherapy with vincristine, methylpred- nisolone, L-asparaginase, and intrathecal methotrexate (Fig 1). Filgras- tim was administered to drive myelopoiesis, and granulocyte infusions were administered daily for 6 days. Her abdominal signs and symptoms improved gradually. Ultrasonography 2 weeks later showed a decrease in ascites and resolution of bowel wall thickening. She had a benign abdominal examination, oral intake was resumed, and she was dis- charged home on the 22nd hospital day with complete neutrophil recovery. She was seen at an outside hospital 2 days later with fever, bilious vomiting, and abdominal distension. Free intraperitoneal air was evi- dent on abdominal radiography. She was transferred to our hospital, where she was noted also to be tachycardic, dehydrated, and had peritonitis. She was fluid resuscitated, started on ampicillin, gentamicin From the Departments of Pediatrics, Molecular Biology & Pharma- cology, Pathology & Immunology, and Surgery, Washington University School of Medicine, St Louis, MO and the St Louis Children’s Hospital, St Louis, MO. Address reprint requests to David B. Wilson, MD, PhD, Department of Pediatrics, Box 8208, Washington University School of Medicine, 660 S. Euclid Ave, St Louis MO 63110. © 2004 Elsevier Inc. All rights reserved. 1531-5037/04/3907-0045$30.00/0 doi:10.1016/j.jpedsurg.2004.03.083 e18 Journal of Pediatric Surgery, Vol 39, No 7 (July), 2004: E25