Trifid Epiglottis as Part of a Complex Congenital Malformation Spectrum: A Case Report and Literature Review

Congenital anomalies of the epiglottis are exceptionally rare, with only a few cases described in the literature. Among them, bifid epiglottis has been more frequently reported, often in association with syndromic conditions such as Pallister-Hall and Bardet-Biedl syndromes. Trifid epiglottis, however, represents an exceedingly uncommon entity, with only two previously documented cases. Because of its rarity, the clinical significance of trifid epiglottis remains unclear, yet its potential impact on airway protection, phonation, and feeding makes it a finding of considerable importance in neonates with respiratory or swallowing difficulties. We report the case of a ۲-day-old female neonate admitted with abdominal distension, poor feeding, lethargy, and decreased urinary output. On examination, she exhibited polydactyly, cyanosis during crying, and an anteriorly placed anus. Abdominal ultrasonography revealed bilateral dysplastic kidneys with moderate hydronephrosis and hydrometrocolpos. Surgical drainage of hydrometrocolpos was performed, with approximately ۵۰ mL of fluid evacuated, and the urachal tract was ligated. Intraoperatively, the rectum was confirmed to be anteriorly positioned but patent. The patient also had right-sided choanal atresia, which was treated with dilation. Due to persistent respiratory distress and stridor, bronchoscopy was undertaken, revealing a trifid epiglottis, grade ۱ interarytenoid notch, and inflamed bronchi with thick secretions requiring therapeutic aspiration and lavage. The vocal cords and trachea were normal. Cardiac evaluation revealed no associated structural heart defects. Following combined surgical and supportive management, the neonate's condition improved and she was discharged in good general health with close follow-up. This case highlights several important aspects. First, the presence of trifid epiglottis emphasizes the need for meticulous airway evaluation in neonates with unexplained respiratory distress, as such anomalies may be easily overlooked without endoscopic assessment. Second, the coexistence of multiple congenital malformations-including genitourinary, anorectal, and craniofacial anomalies-raises the possibility of an underlying syndromic diagnosis, although definitive genetic testing was not performed in this case. Finally, management of these rare anomalies should be tailored toward stabilization of the airway, relief of obstruction, and prevention of aspiration, while simultaneously addressing life-threatening associated malformations. In conclusion, trifid epiglottis is an exceptionally rare congenital anomaly that may present as part of a complex spectrum of multisystem malformations. Awareness of this entity among neonatologists, pediatric surgeons, and anesthesiologists is crucial, as early recognition and multidisciplinary management can significantly improve outcomes in affected infants