Differences in the diagnosis and management of pyriform sinus fistula between newborns and children

Abstract

Pyriform sinus fistula (PSF) is a rare congenital entity. We hypothesized that clinical features, diagnosis and treatment may differ between newborns and children. 190 patients diagnosed with PSF were divided into two groups: neonatal (n = 15) and childhood (n = 175). The medical records including demographic and clinical data were retrospectively analyzed. There were 102 boys and 88 girls in this study. Most patients presented as a left-sided neck lesion. A neck mass, with or without infection and respiratory distress, was the common finding in newborn patients. Prenatal diagnosis was made in three cases. However, in childhood group, initial symptoms were neck abscess (78.8%), acute thyroiditis (11.4%), neck mass (6.9%), and thyroid lesion (2.8%). The presence of a cervical mass with air pocket showed on CT image was thought to be the pathognomonic finding of neonatal PSF. The diagnosis was usually established by barium esophagography in older children. Delayed accurate diagnosis was detected in both groups. The median time from onset to diagnosis was 22 months and 1 year respectively. Endoscopic-assisted open surgery was performed successfully in all patients, with good outcomes in majority cases of both groups (93.3% and 95.4%). Recurrence was developed in 5 patients. PSF should be suspected in newborns with cervical mass and in children with recurrent neck infection, especially on the left side. Early diagnosis and treatment might avoid repeated surgical procedures. Complete resection of the cyst and fistula in non-infected state is essential for good outcomes.