|Year : 2014 | Volume
| Issue : 2 | Page : 122-123
Diprosopus dirrhinus: A rare case report
Prabir Kumar Jash1, Debarati Chattopadhyay2, Nonavinakere Prabhakera Sunil1, Souradip Gupta1
1 Department of Plastic Surgery, Medical College Kolkata, Kolkata, West Bengal, India
2 Department of Plastic Surgery, Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India
|Date of Web Publication||2-Aug-2014|
Dr. Prabir Kumar Jash
Department of Plastic Surgery, Medical College Kolkata, 88 College Street, Kolkata - 700 073, West Bengal
Source of Support: None, Conflict of Interest: None
Diprosopus or craniofacial duplication is an extremely rare craniofacial anomaly. It is the rarest form of conjoined twins. One of the types of incomplete facial duplication is the duplication of nose known as diprosopus dirrhinus, of which there is only a single case report from India to date. In the present article, the authors describe a 5-year-old boy having diprosopus dirrhinus with severe hypertelorism, right cleft 12 and right upper eyelid coloboma, a combination of anomalies which have not been reported to date.
Keywords: Conjoined twins, craniofacial duplication, diprosopus dirrhinus, eyelid coloboma, hypertelorism
|How to cite this article:|
Jash PK, Chattopadhyay D, Sunil NP, Gupta S. Diprosopus dirrhinus: A rare case report. J Cleft Lip Palate Craniofac Anomal 2014;1:122-3
|How to cite this URL:|
Jash PK, Chattopadhyay D, Sunil NP, Gupta S. Diprosopus dirrhinus: A rare case report. J Cleft Lip Palate Craniofac Anomal [serial online] 2014 [cited 2019 May 26];1:122-3. Available from: http://www.jclpca.org/text.asp?2014/1/2/122/137916
| Introduction|| |
Diprosopus or craniofacial duplication is an extremely rare form of conjoined twins. The term diprosopus refers to a fetus with a single trunk with normal limbs, and varying degrees of facial duplication. It is the rarest form of conjoined twins, with prevalence of 1 in 15,000,000 births.  This is a spectrum of malformation where duplication of specific structures, such as the ears, nose or eyes is possible.  In this article, the authors describe an otherwise healthy child having partial facial duplication in the form of a duplicated nose (diprosopus dirrhinus) together with severe hypertelorism and coloboma of right upper eyelid.
| Case Report|| |
A 5-year-old male child was brought to the outpatient department for his facial deformity. He was born of nonconsanguineous marriage, and the antenatal period was uneventful. He had no difficulty in breathing or speech. Examination of his face demonstrated two noses with four external nares, two of which were hypoplastic. Furthermore, there was severe hypertelorism, with downward displacement of the medial canthus of right eye, coloboma of the medial half of right upper eyelid, and a soft cystic nonpulsatile swelling present superomedial to the right eye near the midline [Figure 1]a-d. Associated was craniofacial cleft 12 on the right side. There was no hypertelorism on the left side. The child had a symmetric mouth opening of normal size, and there was no dental or palatal abnormality. He did not have any other physical anomaly and also there was no evidence of mental retardation. The developmental milestones were normal. Computed tomography (CT) scans with three-dimensional image reconstruction showed duplication of the nasal skeleton, the nasal cavity, and the medial part of the maxillary bones and defect in the right frontal bone corresponding to cleft 12 [Figure 2]a and b. Echocardiography and abdominal ultrasound revealed normal findings, and he had a normal male karyotype.
|Figure 1: Photograph of the child showing duplication of nose, hypertelorism, right upper eyelid coloboma and a cystic swelling near the midline. (a) Frontal view, (b) Worm's eye view, (c) Left lateral oblique view, (d) Right lateral view|
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|Figure 2: (a) Computed tomography scan showing duplication of nasal cavity, (b) Three-dimensional reconstruction showing the bony defect and nasal duplication|
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The child's parents were counseled about the multistage reconstructive procedures required for correction of his craniofacial deformities. However, they were not motivated for surgery in view of the long duration of management and opted not to go for surgical correction. The child is doing well on follow-up.
| Discussion|| |
Craniofacial duplication or diprosopus is an extremely rare congenital anomaly. According to the literature, diprosopus is the rarest form of conjoined twins (0.4%).  This is a spectrum of malformation where duplication of specific structures, such as the ears, nose or eyes is possible.  Most babies with diprosopus are stillborn, and they often have other anomalies like anencephaly, neural tube defects, or cardiac malformations. Partial facial duplication is associated with fewer anomalies, and the prognosis is better.  The postulated pathogenesis of craniofacial duplication involves bifurcation of the notochord rostrally during early fetal development.
In infants with incomplete facial duplication, the mandible and mouth are the most commonly duplicated facial structures.  A less severe instance is where the fetus has a duplicated nose (diprosopus dirrhinus) and the eyes are spaced far apart. Diprosopus can be identified in utero during routine antenatal ultrasound imaging, and therapeutic abortion is an option if the anomaly is detected early enough in the pregnancy.  However, in the present case, antenatal maternal imaging was not done, and the child was born at his home.
The line of management of diprosopus dirrhinus is multistaged. The surgical intervention and osteotomy lines have to be guided by three-dimensional CT. The operative procedure will include resection of the redundant osseous structures and soft tissue of the nostrils, repositioning of the functional nostril and correction of hypertelorism. 
Only a single case of isolated nasal duplication has been reported by Ghosh et al.  To the knowledge of the authors, this is only the second reported case of diprosopus dirrhinus from India. Furthermore, the combination of anomalies described in a patient has not been reported in the literature to date.
| References|| |
|1.||Picaud A, Nlome-Nze AR, Engongha-Beka T, Ogowet-Igumu N. Early prenatal diagnosis of diprosopic syncephalic joined twins. Rev Fr Gynecol Obstet 1990;85:379-81. |
|2.||Kotrikova B, Hassfeld S, Steiner HH, Hähnel S, Krempien R, Mühling J. Operative correction and follow-up of craniofacial duplication. Plast Reconstr Surg 2007;119:985-91. |
|3.||Machin GA. Conjoined twins: Implications for blastogenesis. Birth Defects Orig Artic Ser 1993;29:141-79. |
|4.||Wu J, Staffenberg DA, Mulliken JB, Shanske AL. Diprosopus: A unique case and review of the literature. Teratology 2002;66:282-7. |
|5.||DeRuiter C. Diprosopus (craniofacial duplication). Embryo Project Encyclopedia (2011-08-16). ISSN: 1940-5030. Available from: http://www.embryo.asu.edu/handle/10776/2286. [Last accessed on 2014 July 23]. |
|6.||Ghosh P, Dada K, Gupta S, Jamwal N, Daljit R. Double nose. J Laryngol Otol 1971;85:963-9. |
[Figure 1], [Figure 2]