Format

Send to

Choose Destination
J Craniomaxillofac Surg. 2014 Jun;42(4):290-6. doi: 10.1016/j.jcms.2013.05.016. Epub 2013 Aug 19.

Partial craniofacial duplication: a review of the literature and case report.

Author information

1
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.
2
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.
3
Department of Genetics, CHLA, Keck School of Medicine, USC, Los Angeles, CA, USA.
4
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.

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.

KEYWORDS:

Accessory mouth; Craniofacial duplication; Diprosopus; Mandibular duplication; Maxillary duplication

PMID:
23969147
DOI:
10.1016/j.jcms.2013.05.016
[Indexed for MEDLINE]

Supplemental Content

Full text links

Icon for Elsevier Science
Loading ...
Support Center