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By Hana’a Tashkandi Surgical Demonstrator KAAU Typhlitis Definition Epidemiology Pathophysiology Clinical presentation Complications D.D. Investigations Management Prognosis What does it mean? Typhlitis means inflammation of the cecum. It is an acute life-threatening condition characterized by transmural inflammation involving ileum, cecum, or appendix in patients who are severely myelosuppressed and immunosuppressed. What does It Mean? Associated with: Aplastic anemia. Lymphoma. AIDS. Immunosuppression following renal transplantation or during treatment of malignancy. Epidemiology found in 10% of leukemic children who died while undergoing chemotherapy. mortality rate averages 40-50% (cecal perforation, bowel necrosis, or sepsis). Prevalence is equal in males and females. Typhlitis occurs in both children and adults. Pathophysiology The etiology of typhlitis is unknown but pathogenesis is multifactorail. Profound neutropenia, with total neutrophil counts of less than 1000 appears to be a universal predisposing factor. Mucosal injury from cytotoxic drugs plays an important role in the typhlitis observed during chemotherapy. Pathophysiology Cecal distension in typhlitis may impair the blood supply, leading to mucosal ischemia and ulceration. Infection may be involved, especially cytomegalovirus. Bacterial invasion leads to transmural penetration and ultimately perforation. Mucosal and submucosal necrosis can result in intramural hemorrhage. Neoplastic infiltration may be involved in some patients. Clinical Manifestations Watery or bloody diarrhea Fever Nausea Vomiting Abdominal pain (may be localized to right lower quadrant) Possible shock secondary to septicemia or colonic perforation Clinical Findings Abdominal distension Palpation tenderness (usually most marked in RLQ) Occasionally, a palpable mass Diffuse direct and rebound tenderness (suggesting colonic perforation, peritonitis) Hyper-resonant abdomen Absence of bowel sounds D.D. Acute Appendicitis. I.B.D. Enterocolitis. Toxic Megacolon. Small bowel obstruction. Complications Bowel perforation and peritonitis Gastrointestinal bleeding Gastrointestinal obstruction Intra-abdominal abscess Sepsis Death Investigations Complete blood count is used to confirm neutropenia. Stool studies are obtained for the following: Clostridium difficile toxin to rule out pseudomembranous colitis. Culture for enteric pathogens to rule out infectious causes of enterocolitis. Investigations AXR: Plain radiographs are nonspecific but may demonstrate a fluid-filled masslike density in the RLQ, distension of adjacent small bowel loops, and thumbprinting. Free intraperitoneal air and pneumatosis coli rarely are observed. Barium enema and colonoscopy are contraindicated in possible typhlitis because of perforation risk. Investigations CT Abdomen: CT demonstrates circumferential and occasionally eccentric low-attenuation colonic wall thickening and cecal distension. High attenuation within the thickened colonic wall may represent hemorrhage. Inflammatory pericolonic stranding of mesenteric fat is common. CT readily identifies complications, including pneumatosis coli, pneumoperitoneum, pericolonic fluid collections, and abscess. These complications may require urgent surgical management. Management Conservative Surgical Management Conservative management includes the following: Bowel rest and nasogastric suction Close monitoring of patients using serial abdominal examinations in an intensive care setting Intravenous fluids, blood, and platelet transfusions as necessary Management Parenteral broad-spectrum antibiotics: Antibiotics should include agents covering enteric gramnegative and anaerobic organisms, including Clostridium species. Metronidazole also may be considered if pseudomembranous colitis cannot immediately be excluded. Cultures: Obtain blood cultures for fungus and consider antifungal agents if patients do not respond to antibiotics. Management Avoidance of certain medications: Anticholinergic agents, antidiarrheal drugs, and narcotics may worsen the condition or further confuse the clinical picture. Surgical Management indications: Free intra-abdominal perforation Clinical deterioration during conservative medical therapy Differentiation from other acute abdominal conditions for which surgery is indicated Unrelenting intra-abdominal sepsis or abscess formation Continued hemorrhage with a platelet count and coagulation parameters within the reference range Surgical Management Choice of surgical procedures includes the following: 1) Cecostomy and drainage 2) A 2-stage right hemicolectomy or total abdominal colectomy, with or without a primary anastomosis 3) Defunctioning of the colon with a loop ileostomy Prognosis The prognosis generally is poor, with mortality rates varying from 5-100% and averaging about 40-50%. The prognosis depends highly on the rapidity of restoration of the white blood cell count. The potential for recovery may be improved by aggressive and meticulous medical and supportive therapy. Summary Consider the possibility of neutropenic enterocolitis in all patients who are immunosuppressed and have right lower quadrant pain. Early recognition of this condition is paramount to reducing mortality rates and achieving a potentially good outcome. Monitor the patient in an intensive care setting with frequent serial abdominal examinations. Joint management by the medical and surgical teams is essential for optimal management. Thank You Hana’a Tashkandi