Congenital oropharyngeal teratoma: a case report and systematic literature review

Background: Epignathus is a rare oropharyngeal teratoma (OPT) that arises in the oral cavity and poses a high mortality risk due to airway obstruction. It is frequently associated with midline malformations, most commonly cleft palate. Diagnosis and management are complex, necessitating a multidisciplinary approach. This study presents a case of neonatal OPT and a systematic literature review to assess clinical presentation, diagnostic methods, and treatment strategies. Case report: We report a 33-week gestational-age boy referred to our hospital for a giant intraoral mass causing airway obstruction. Due to difficult intubation, invasive ventilation was achieved via tracheostomy. MRI revealed a large heterogeneous mass with cystic and solid components. Suspecting a vascular anomaly, intralesional bleomycin was attempted but ineffective. Given rapid tumor growth, a multidisciplinary surgical approach was performed, achieving complete excision without intra- or post-operative complications. Histopathology confirmed mature OPT. A systematic literature review was conducted following PRISMA guidelines. A PubMed search using "intraoral teratoma" identified pediatric cases published in the last 20 years. Discussion and Conclusion: OPTs are extremely rare lesions. In the absence of a prenatal diagnosis, emergency transfer of patients to a specialized referral centre is necessary to improve prognosis. Proper surgical planning should involve an experienced multidisciplinary team. Further studies and stronger evidence are needed to establish the best practice for managing this condition.