Schwannomas are indolent benign lesions arising from schwann cells in the nerve. They are especially rare in the paediatric population. We report an interesting case of a swelling in the upper neck, which highlights an atypical presentation of this tumour, as well as the complex details of its clinical, radiological, and surgical management.
This study reports the case of a male infant born at full term with antenatal suspicion for esophageal atresia (EA). At birth inability to pass a feeding tube and a gasless abdomen on plain film X-ray led to the diagnosis of pure EA according to international guidelines. In the first week of life a stomach could not be identified at surgery for open gastrostomy and a feeding jejunostomy was formed. Subsequent investigations including CT scan did not detect the presence of a stomach. Cervical esophagostomy was performed at 8 weeks old as there was no expectation of achieving an esophageal anastomosis and a deviated septum had complicated the placement of nasal tubes for management of the proximal esophageal pouch. At 6 months of age esophageal replacement with a jejunal pull-up graft was undertaken as staged surgery. At mobilization of a large midline liver to expose the hiatus, a microstomach was revealed, in association with a type 2 pyloric atresia (PA) and a distal tracheoesophageal fistula (TEF). Following ligation of the TEF by lateral thoracotomy, the TEF and microstomach were resected and the jejunal pull-up graft was completed. Discharge home was achieved 1 month post-op. Oral intake was established for solids by 1 year of age. The patient is thriving at 4 years of age with additional overnight jejunal feeds. A unifying genetic diagnosis has not been made to date. Congenital microgastria or PA has been described to co-exist with EA but never previously have all these anomalies been reported occurring together. This unique scenario permitted the distal TEF to be clinically silent and present as a pure atresia. In turn congenital microgastria together with a diagnosis of pure EA obscured the presence of PA, which classically presents with vomiting of feeds and massive gastric distension. Investigations, and in particular cross-sectional imaging, were on the whole unhelpful. This case illustrates that correct diagnosis in this complex case ultimately could only be made at the time of definitive surgery.
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