(facial duplication)

Very rare. Girls are twice more often affected than boys. 

Duplication of

-         either the whole face: two faces on a same skull; sometimes associatiated with anencephaly, duplication of the central nervous system or spina bifida. 

-         either a part of the face. For example, two mouths one of which is in the normal position and the other is ectopic (see picture). In these cases, the oral cavities are communicating or not and suction movements are synchronous. Replication can include the mandible, maxilla, a part of the palate.  The cause is probably due to duplication of the 1st branchial arch. There are sometimes associated brain abnormalities including a duplication of the pituitary gland or the hypothalamus. A Tessier cleft (particularly of type 7)  (see this term) can be associated.

Those duplications are often part of a syndrome and, in that case, are associated with Klippel-Feil syndrome, Pierre-Robin triad or cleft lip-cleft palate syndrome.

Anesthetic implications: 

anticipate a difficult intubation/ventilation (application of the face mask); submental intubation may be needed to facilitate the complex surgical approach

References : 

-        Amr SS, Hammouri MF. 
Craniofacial duplication (diprosopus): report of a case with a review of the literature. 
Eur J Obst Gynecol Reprod Biol 1995; 58: 77-80.

-         Suhaili DNP, Somasundaram S, Lau SH, Ajura AJ, Roslan AR, Ramli R.
Duplication of lower lip and mandible: a rare diprosopus. 
Int J Pediatr Otorhinolaryngol 2011; 75: 131-3.

-         Costa MA, Borzabadi-Farahani A, Lara-Sanchez PA, Schweitzer D, Jacobson L, Clarke N, Hammoudeh J, Urata MM, Magee III WP. 
Partial craniofacial duplication: a review of the literature and case report. 
J Craniomaxillofac Surg 2014; 42: 290-6.

-         Ozçelil D, Toplu G, Türkseven A, Senses DA, Yigit B. 
Lateral facial cleft associated with accessory mandible having teeth, absent parotid gland and peripheral facial weakness. 
J Craniomaxillofac Surg 2014; 42: e239-44.

-         Warm A, Cordaro S, Manti S, Barberi I. 
An extra mouth in a newborn: a unique case of facial duplication. 
Arch Dis Child Fetal Neonatal Ed 2014; 99: F342-3. 

-        Hamberis AO, Macias D, Clemmens C, Patel KG.
Duplication of the oral cavity and mandible : a rare congenital craniofacial anomaly.
BMJ Case Reports 2020 ; 13 :e233799

Updated: May 2020