Trifid epiglottis in a neonate with choanal atresia and hydrometrocolpos: the third reported case and the first symptomatic neonatal presentation

BACKGROUND: Trifid epiglottis (TE) is an extremely rare congenital laryngeal anomaly. To the best of our knowledge, only two cases have previously been reported in the literature. Both of them have been incidentally detected and associated with polydactyly. CASE PRESENTATION: We report the third known case of TE in a two-day-old female neonate who was admitted to the emergency department due to abdominal distension, oliguria, and poor feeding within the first 24 h of life. Examination revealed stridor during crying, left postaxial polydactyly, right syndactyly, and an anteriorly displaced anus. Imaging findings revealed hydrometrocolpos followed by bilateral hydronephrosis. Her distension and oliguria were successfully treated with surgical drainage. Due to persistent stridor, airway evaluation confirmed right choanal atresia and TE. Other evaluations, including echocardiography, spinal ultrasonography, endocrine studies, and brain magnetic resonance imaging (MRI), were normal. The patient remained stable during one year of follow-up. CONCLUSION: To the best of our knowledge, this appears to be the first reported symptomatic neonatal case of TE associated with choanal atresia and hydrometrocolpos. It highlights the importance of early diagnosis and comprehensive evaluation of rare congenital laryngeal anomalies in neonates. Reporting such cases is crucial to expand the understanding of their clinical spectrum, guide airway management, and perform multidisciplinary care.