Drug reaction with eosinophilia and systemic symptoms in a pediatric patient presenting with seizures, severe enterocolitis, and intestinal stricturing: A case report

Introduction Pediatric Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) is an uncommon hypersensitivity reaction, and multiorgan presentations combining refractory seizures with enterocolitis and intestinal stricturing have rarely been reported in this population. To our knowledge, this represents the first reported case of surgery for a gastrointestinal complication of DRESS in a pediatric patient. Case Presentation We report a case of an 11-year-old male with no significant past medical history presenting with generalized tonic-clonic seizures progressing to status epilepticus, subsequently diagnosed as new onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES) of unknown etiology. Over three months, he developed multiorgan dysfunction attributed to DRESS, including severe enteropathy with hematochezia, hypogammaglobulinemia, and progressive liver dysfunction. Imaging revealed secondary sclerosing cholangitis, post-endoscopic retrograde cholangiopancreatography (ERCP) duodenal hematoma, and worsening small bowel strictures. Ureteral and biliary stenting and gastric decompression provided partial control, but obstructive symptoms persisted despite two and a half months of conservative management. He underwent exploratory laparotomy with resection of the diffusely involved jejunum, primary anastomosis, and multiple stricturoplasties. His post-operative course was uncomplicated, with gradual improvement in intestinal function and eventual tolerance of enteral feeding; he was discharged five weeks post-operatively. Conclusion In pediatric DRESS presenting with severe enterocolitis and intestinal symptoms, surgical resection should be considered to address fibrosis and resulting strictures when obstructive symptoms fail to resolve with conservative management.