First Case Report of Agnathia-Otocephaly Complex Reported in Niger

Agnathia-otocephaly complex (AOC) is an extremely rare congenital malformation resulting from a disorder of the first branchial arch. In the past, this condition was considered lethal. However, milder variants of the isolated type of AOC have been reported as non-lethal. The condition is characterized by hypoplasia or the absence of the mandible (micrognathia/agnathia), variable displacement of the ears (melotia) with or without auricular fusion (synotia), and a small oral aperture or absence of mouth (microstomia/astomia), with or without tongue hypoplasia or absence (aglossia). AOC can occur as a stand-alone condition or in conjunction with other congenital abnormalities. Antenatal ultrasound is essential for early diagnosis, allowing appropriate counseling and management decisions in severe cases of AOC. However, in developing countries, where pregnancies are not well monitored, this malformation is often not detected until after delivery. We hereby report the first case of AOC in Niger, discovered after delivery in a premature newborn. The objective was to share our department's experience and emphasize the necessity for prenatal diagnosis through effective pregnancy monitoring in our regions.