title: Partial craniofacial duplication: a review of the literature and case report.
abstract: Diprosopus (Greek; di-, "two" + prosopon, "face"), or craniofacial duplication, is a rare craniofacial anomaly referring to the complete duplication of facial structures. Partial craniofacial duplication describes a broad spectrum of congenital anomalies, including duplications of the oral cavity. This paper describes a 15 month-old female with a duplicated oral cavity, mandible, and maxilla. A Tessier type 7 cleft, midline meningocele, and duplicated hypophysis were also present. The preoperative evaluation, surgical approach, postoperative results, and a review of the literature are presented. The surgical approach was designed to preserve facial nerve innervation to the reconstructed cheek and mouth. The duplicated mandible and maxilla were excised and the remaining left maxilla was bone grafted. Soft tissue repair included closure of the Tessier type VII cleft. Craniofacial duplication remains a rare entity that is more common in females. The pathophysiology remains incompletely characterized, but is postulated to be due to duplication of the notochord, as well as duplication of mandibular growth centres. While diprosopus is a severe deformity often associated with anencephaly, patients with partial duplication typically benefit from surgical treatment. Managing craniofacial duplication requires a detailed preoperative evaluation as well as a comprehensive, staged treatment plan. Long-term follow up is needed appropriately to address ongoing craniofacial deformity.
authors: Division of Plastic and Maxillofacial Surgery, Children's Hospital Los Angeles (CHLA), Plastic and Reconstructive Surgery, Keck School of Medicine, University of Southern California (USC), Los Angeles, CA, USA.
authors: Melinda A
authors: MA
authors: Costa
authors: Orthodontics, Warwick Dentistry, Warwick Medical School, University of Warwick, Coventry, UK; Formerly, Craniofacial Orthodontics, Division of Dentistry, CHLA, Center for Craniofacial Molecular Biology, USC, Los Angeles, CA, USA.
authors: Ali
authors: A
authors: Borzabadi-Farahani
authors: Department of Genetics, CHLA, Keck School of Medicine, USC, Los Angeles, CA, USA.
authors: Pedro A
authors: PA
authors: Lara-Sanchez
authors: Department of Genetics, CHLA, Keck School of Medicine, USC, Los Angeles, CA, USA.
authors: Daniela
authors: D
authors: Schweitzer
authors: Division of Plastic and Maxillofacial Surgery, Children's Hospital Los Angeles (CHLA), Plastic and Reconstructive Surgery, Keck School of Medicine, University of Southern California (USC), Los Angeles, CA, USA.
authors: Lia
authors: L
authors: Jacobson
authors: Division of Plastic and Maxillofacial Surgery, Children's Hospital Los Angeles (CHLA), Plastic and Reconstructive Surgery, Keck School of Medicine, University of Southern California (USC), Los Angeles, CA, USA.
authors: Noreen
authors: N
authors: Clarke
authors: Division of Plastic and Maxillofacial Surgery, Children's Hospital Los Angeles (CHLA), Plastic and Reconstructive Surgery, Keck School of Medicine, University of Southern California (USC), Los Angeles, CA, USA.
authors: Jeffery
authors: J
authors: Hammoudeh
authors: Division of Plastic and Maxillofacial Surgery, Children's Hospital Los Angeles (CHLA), Plastic and Reconstructive Surgery, Keck School of Medicine, University of Southern California (USC), Los Angeles, CA, USA.
authors: Mark M
authors: MM
authors: Urata
authors: Division of Plastic and Maxillofacial Surgery, Children's Hospital Los Angeles (CHLA), Plastic and Reconstructive Surgery, Keck School of Medicine, University of Southern California (USC), Los Angeles, CA, USA. Electronic address: mageewilliam3@hotmail.com.
authors: William P
authors: WP
authors: Magee
mesh_terms: D002610:Cheek / Q000002:abnormalities / Q000601:surgery; D002972:Cleft Palate / Q000175:diagnosis / Q000601:surgery; D005146:Facial Asymmetry / Q000175:diagnosis / Q000601:surgery; D005260:Female; D006801:Humans; D007223:Infant; D008046:Lip / Q000002:abnormalities / Q000601:surgery; D008334:Mandible / Q000002:abnormalities* / Q000601:surgery; D008437:Maxilla / Q000002:abnormalities* / Q000601:surgery; D008588:Meningocele / Q000175:diagnosis; D009056:Mouth Abnormalities / Q000175:diagnosis* / Q000601:surgery; D009295:Nasal Bone / Q000002:abnormalities / Q000601:surgery; D010902:Pituitary Gland / Q000002:abnormalities
keywords: Accessory mouth; Craniofacial duplication; Diprosopus; Mandibular duplication; Maxillary duplication