Agnathia-synotia-microstomia (otocephaly): a case report in an African woman

Pharyngeal arches appear in the 4th and 5th weeks of development of the human embryo. The 1st pharyngeal arch develops into the incus and malleus, premaxilla, maxilla, zygomatic bone; part of the temporal bone, the mandible and it contributes to the formation of bones of the middle ear. The musculature of the 1st pharyngeal arch includes muscles of mastication, anterior belly of the digastric mylohyoid, tensor tympani and tensor palatini. The second pharyngeal arch gives rise to the stapes, styloid process of the temporal bone, stylohyoid ligament, the lesser horn and upper part of the body of the hyoid bone. The stapedius muscle, stylohyoid, posterior belly of the digastric, auricular and muscles of facial expressional all derive from the 2nd pharyngeal arch. Otocephaly has been classified as a defect of blastogenesis, with structural defects primarily involving the first and second branchial arch derivatives. It may also result in dysmorphogenesis of other midline craniofacial field structures, such as the forebrain and axial body structures.