An isolated bilateral pure macrostomia in a 2-year-old girl

Congenital macrostomia is a lateral orofacial cleft between the maxillary and mandibular components of the first branchial arch. Bilateral macrostomia is a rare entity. To date, 17 cases have been reported in the literature, with more than 50% isolated. We present a case of bilateral transverse facial cleft (macrostomia) in a 2-year-old girl. It was not associated with any other anomaly. The cleft involved only the soft tissues of the face. Hence, it was a pure macrostomia. She was the third female child of the family, with 2 elder sisters normal. Nutritional deficiency during intrauterine life and/or advanced maternal age can be causative factors. Transverse facial cleft was closed by Z-plasty incision, with good functional and aesthetic result.