Frontoethmoidal encephalocele in a Sudanese infant: a rare case report and literature review

Introduction and importance: Frontoethmoidal encephalocele (FEE) is an uncommon congenital abnormality that occurs due to the protrusion of intracranial contents through a hole in the skull bone, at the frontal and ethmoidal bones, with a higher prevalence in Southeast Asia and parts of Africa compared to Western countries. Our case is the first documented case of FEE in Sudan, offering epidemiological data from an underreported region and reviewing similar literature. Case presentation: A 21-day-old Sudanese male infant was presented with a soft, cystic, non-pulsatile swelling over the left nasal bridge, present since birth. It is about 6 × 6 cm. The mother had irregular antenatal care and did not take folic acid supplements. Magnetic resonance imaging (MRI) scan revealed a 0.6 cm defect in the left frontoethmoidal area with herniation of brain tissue and meninges. Our patient underwent successful surgical management and recovered smoothly without complication. At follow-up, cosmetic and functional outcomes were satisfactory. Discussion: FEE poses a technical challenge for reconstruction in a resource-limited setting, and this successful outcome highlights the feasibility of effective surgical treatment with a dedicated multidisciplinary team, which involved excision of the dysplastic tissue and sac, watertight dural closure, and primary skin closure, even without advanced resources. The literature also revealed how FEE is likely caused by multiple factors, including maternal nutritional deficiencies (folic acid and iron). Conclusion: Despite its rarity, FEE remains a significant surgical challenge, particularly in low-resource settings, including Sudan, and can be successfully treated by a multidisciplinary approach, even in the absence of prosthetic materials.