**Background**
Eosinophilic esophagitis (EoE) is a chronic, allergen-driven disease characterized by T helper 2-mediated inflammation, leading to smooth muscle hypertrophy and fibrosis of the esophageal epithelium and subepithelium. This remodeling can result in strictures, motility abnormalities, and rare complications such as esophageal intramural dissection (EID) or perforation. EID involves separation of the mucosa/submucosa from deeper muscular layers, creating a false lumen. While most EID cases are idiopathic, they can occur in the setting of chronic inflammation like EoE, especially after endoscopic procedures, ingestion of sharp objects, or emetogenic events. Primary manifestation of EoE with spontaneous EID is extremely rare, particularly in children. This report presents a 14-year-old boy with spontaneous EID as the initial presentation of EoE.
**Methods**
This is a single case report of a 14-year-old male admitted with sudden onset of fever, chest, and epigastric pain that worsened over 7 days. His medical history included food refusal, cough, and runny nose since early childhood, previously evaluated for food allergy and gastroesophageal reflux disease (GERD). He had responded well to ranitidine, lansoprazole (proton pump inhibitor, PPI), and dietary elimination of milk and egg, and was symptom-free until current admission. Physical examination was unremarkable. Laboratory workup showed elevated C-reactive protein but was otherwise normal. Chest X-ray was normal. Computerized tomography (CT) revealed distal intramucosal dissection in the middle and lower esophageal tract without leakage. The patient was started on total parenteral nutrition, pantoprazole, and piperacillin-tazobactam. Upper endoscopy showed crepe-paper-like mucosa, extensive ulceration with a 5 cm mucosal defect starting 28 cm from the incisors ending in a cul-de-sac, and a true lumen followed distally by a pouch measuring approximately 10 cm. Based on these findings, EoE was suspected, and steroid 1 mg/kg/day was started with a three-food elimination diet. Biopsies showed ulceration and rare intraepithelial eosinophils. Further tests revealed high serum immunoglobulin E levels (3427 U) and peripheral blood eosinophilia (450/mm^3). Food allergy tests were unremarkable. The patient was discharged on PPI with oral viscous budesonide (OVB) on day 7. At 4-week follow-up, repeat endoscopy showed almost complete epithelialized laceration with no residual mucosal tears; histopathology was inconclusive for EoE. OVB was continued as 1 mg twice a day with PPI for 3 months, then decreased to 1 mg/day. Due to poor dietary compliance and depressiveness, dietary restriction was tapered. Symptoms of throat clearing and dysphagia recurred after meat and egg introduction. At 6 months, endoscopy showed loss of vascularity and exudative linear furrowing. Pathological examination revealed basal cell hyperplasia, eosinophilic microabscesses, and abundant eosinophil counts (100 eos/hpf), consistent with EoE. Three-food elimination diet was restarted with high dose OVB (2 mg/day) for 3 months, then tapered. He remained asymptomatic on maintenance OVB (0.5 mg/day). Follow-up endoscopy and histopathological examination were normal at 9 months and 1 year.
**Key Results**
The patient presented with spontaneous EID as the first manifestation of EoE after approximately 10 years of undiagnosed symptoms. Initial biopsies were inconclusive for EoE, showing only ulceration and rare intraepithelial eosinophils, likely due to advanced fibrosis. After treatment with PPI, steroids, and elimination diet, the mucosal defect epithelialized within 4 weeks. However, upon reintroduction of meat and egg, symptoms recurred, and at 6 months, histopathology confirmed EoE with 100 eosinophils per high-power field (eos/hpf), basal cell hyperplasia, and eosinophilic microabscesses. The patient achieved clinical and histologic remission with high-dose OVB (2 mg/day) and three-food elimination diet, maintained on OVB 0.5 mg/day with normal endoscopy and histology at 9 months and 1 year.
**Clinical Implications**
This case highlights that EID can be a rare but serious presenting complication of undiagnosed EoE in children. Diagnostic delay of EoE is common, especially in young patients, and is associated with increased risk of fibrostenotic complications such as strictures and EID. Initial biopsies may be inconclusive due to advanced fibrosis, and clinicians should maintain a high index of suspicion for EoE in children with persistent dysphagia or GERD-like symptoms, particularly those with atopic comorbidities. Early endoscopic evaluation is crucial to avoid diagnostic delay and prevent long-term sequelae. Management of EID in EoE is not standardized; conservative treatment with PPI, topical steroids (e.g., OVB), and elimination diet can be effective, but maintenance therapy duration remains unclear. This patient required 1 year of OVB therapy with two normal biopsies before discontinuation. The case underscores the importance of considering EoE in children with unexplained esophageal dissection and the need for long-term follow-up to monitor for recurrence.