P2.249: Unusual Presentation of Acute Typhlitis with Necrotizing Granulomatous Lymphadenitis in a Healthy Teenager

Introduction: Typhlitis is a rare necrotizing inflammation of the cecum generally associated with immunosuppression. We report the unusual case of an otherwise healthy teenager diagnosed with acute typhlitis and found to have necrotizing granulomatous lymphadenitis on multidisciplinary workup.

Case Description: A 17-year-old female was admitted to the pediatric hospital medicine service for 1 month of worsening lower abdominal pain, nausea, vomiting, diarrhea, and weight loss. The patient was recently admitted at an outside hospital for acute typhlitis diagnosed on computerized tomography (CT) which improved with empiric antibiotics. Repeat CT on admission demonstrated marked wall inflammation at the ileocecal valve with bilateral renal abnormalities and right lower quadrant (RLQ) adenopathy, raising concern for lymphoma. Pediatric gastroenterology, hematology-oncology, and surgery were consulted. Colonoscopy demonstrated a hard mass arising circumferentially from the appendiceal orifice into the cecum; snare or resection was deferred due to concern for perforation. Non-specific changes were shown on biopsies of the surrounding intestinal mucosal and surface of the mass. Laparoscopic surgical biopsy of the peri-appendiceal lymph node demonstrated necrotizing granulomas without signs of an oncologic process. Expanded testing for bacterial and fungal etiologies were sent. After extensive discussion with the family and multidisciplinary team, the decision was made to repeat outpatient imaging after discharge to determine a management plan, including possible surgical resection of the ileocecal valve. Follow-up CT demonstrated an improved inflammatory process consistent with typhlitis and no evidence of mass. The patient endorsed near resolution of symptoms. No further intervention or follow-up was planned.

Discussion: The presentation of acute typhlitis without oncologic or infectious involvement in an immunocompetent patient prompted considerable discussion between the primary team and consulting specialists on the best approach to achieve definitive diagnosis and decide appropriate management. Multidisciplinary collaboration led to shared decision-making with the family to delay intervention which ultimately led to avoidance of ileocecal valve resection with potentially long-term complications.

Conclusion: This case is a testament that diametric opinions from consultants and primary team can ultimately lead to favorable outcomes when used to influence decision-making and weighing the best interests of the patient.