Diprosopus: a review of the aetiology and case report of early surgery in a 7-week-old infant with partial facial duplication
Diprosopus is a rare craniofacial anomaly and is considered a subgroup of conjoined twinning. It encompasses a broad spectrum of duplications from single structure doubling to two completely formed heads on one neck. The aetiology of diprosopus remains a controversial topic, and many hypotheses exist. A multifactorial causal relationship is feasible because of the coexistence of other internal system anomalies of cardiac, gastrointestinal, neurological and respiratory origin. Ovid and PubMed databases were searched, using the key words: “diprosopus”, “craniofacial duplication” and “duplicated mandible”. The identified literature and key referenced articles therein were examined. The purpose was to consolidate the existing body of knowledge on the aetiology and management of diprosopus in order to improve our understanding of this rare condition. Our case report is relatively unique in that it represents a complete duplication of the mandible. Other tissues that were duplicated include the tongue, floor of mouth and the lower lip. It was hypothesised that associated growth abnormalities of the facial skeleton could undergo spontaneous correction if the duplicated mandible and associated tissues were excised early. On review, surgery is the only treatment that can offer functional, aesthetic and psychological improvement. Most patients who were offered surgery (in the reviewed literature) had a partial facial duplication. Surgery is usually deferred until the patient is older when more soft tissue is available to perform the reconstruction and when there would possibly be less growth restriction due to growth centre manipulation. This finding on the timing of surgery was in direct conflict with our hypothesis.
Level of Evidence: Level V, therapeutic study.
KeywordsDiprosopus Duplicated mandible Aetiology Management Craniofacial duplication
Compliance with ethical standards
No financial disclosures.
Conflict of interest
Sieglinde Erica Rabe, Mahendra Daya and Anil Madaree declare that they have no conflict of interest.
Ethical approval was obtained from the Biomedical Research Ethics Committee (BREC) at the University of Kwazulu-Natal, Durban, South Africa.
There was no signed informed consent from the mother of the patient. The patient was from rural Kwazulu-Natal. The Social Worker of Stanger area was contacted and they were unable to locate the patient nor her mother. The ethics committee (BREC) was informed about this, and permission was granted by them to use the information and photos of the patient for my masters in medicine (MMed) degree (which also includes publishing the article). The letter is included in the supplementary forms.
- 13.Okazaki JR, Wilson JL, Holmes SM et al (1987) Diprosopus: diagnosis in utero. Am J Roentgenol 69(2):121–124Google Scholar
- 14.Morabito R, Colonna MR, Mormina E, Stagno d'Alcontres F, Salpietro V, Blandino A, Longo M, Granata F (2014) Accessory oral cavity associated with duplication of the tongue and the mandible in a newborn: a rare case of diprosopus. Multi-row detector computed tomography diagnostic role. J Cranio-Maxillofacial Surg. 42(8):1924–1928CrossRefGoogle Scholar
- 20.Suryawanshi P, Deshpande M, Verma N et al (2013) Craniofacial duplication: a case report. J Clin Diagnostic Res 7(9):2025–2026Google Scholar
- 28.Avery JK, Hayward JR (1969) Case Report: duplication of oral structures with cleft palate. Cleft Palate J 6(4):506–515Google Scholar
- 30.Kaufman MH (2004) The embryology of conjoined twins. Child’s Nerv Syst 20(8–9):508–525Google Scholar
- 35.Stockard CR (1921) Developmental rate and structural expression: an experimental study of twins, “double monsters” and single deformities, and the interaction among embryonic organs during their origin and development. Thirty-two text figures and six plates. Am J Anat 28(2):115–278CrossRefGoogle Scholar
- 45.Chagares DG, McGauran MH (1976) Craniofacial duplication (diprosopus) in a twin. Arch Pathol Lab Med 100(July):392–394Google Scholar
- 55.Fearon JA, Mulliken JB (1987) Midfacial duplication: a rare malformation sequence. Plast Reconstr Surg 79(2):260–264Google Scholar
- 65.Dorf DS, Curtin JW (1982) Early cleft palate repair and speech outcome. Plast Reconstr Surg 70(1):74–81Google Scholar