An Unusual Cause of Pediatric Dysphagia

Bronchogenic cysts (BCs) are bronchopulmonary fore-gut malformation, mostly located in the middle and superior mediastinum. BCs result from abnormal budding of the primitive tracheobronchial tube. The location of the cyst depends on the embryological stage of abnormal budding. Although periesophageal BCs have been frequently reported in adults, a completely intra-mural cyst is very rare, with about 22 adult cases reported. Approximately 40% of these adults presented with chest pain, cough, or dyspnea, and 14% with dys-phagia. 1 There were no pediatric cases of an intramural esophageal cyst presenting with dysphagia in the English literature on PubMed search from 1960 to 2015. A 12-month-old boy presented with failure to thrive since birth, with a decrease in weight from the 75th to the 5th percentile at time of presentation. Beginning at age 9 months, he began having frequent episodes of vomiting with solid foods. He was started on a histamine H2-receptor antagonist without significant improvement. Past medical history was significant for vesicoureteral reflux treated with nitrofurantoin prophylaxis but was negative for recurrent pneumonia and chronic lung disease. Review of systems was unremarkable except for refusing to accept solid foods, and physical exam revealed no unusual findings. Based on this presentation, further imaging was undertaken to rule out anatomical abnormalities as the cause for his presentation. An upper gastrointestinal series (Figure 1) revealed a fixed defect along the left lateral aspect of the distal esophagus. Computed tomography (Figures 2 and 3) with contrast uncovered an ovoid, fluid attenuated, middle mediastinal structure that was adjacent to the distal esophagus. This was compatible with an esophageal duplication given the proximity to the esophagus, although the differential also included BC. Thoracoscopic resection revealed a cystic mass of the esophagus that was intramural with mucoid material contents. Though the cystic mass appeared intramural, it was completely excised without damaging the native esophagus. Postoperatively the patient did well and tolerated fluids and solids without any vomiting or aversions. Several months later, the family reported that the patient continued to do well with no feeding issues. On histological exam the cyst was lined with ciliated pseudostratified columnar epithelium overlying a layer of discontinuous smooth muscle and an outer wall composed of fibrovascular connective tissue with islands of hyaline cartilage. Mucous glands were noted in this layer (Figures 4 and 5). These findings were consistent with a BC.