Diprosopus (partially duplicated head) associated with anencephaly: a case report.
Craniofacial duplication (diprosopus) is a rare form of conjoined twin. A 16 year old mother with a twin pregnancy delivered one normally formed baby boy and one diprosopus male. The malformed baby was 33 weeks of gestation with a single trunk, normal limbs and various degrees of facial duplication. Of the following structures there were two of each: noses, eyes, ears (and one dimple), mouths, tongues and, with bilateral central cleft lips and cleft palates. This was associated with holoprosencephaly and craniorachischisis. Internal organs showed no duplication. There were multiple congenital anomalies including diaphragmatic hernia, small lungs, two lobes of the right lung, ventricular septal defect, small adrenal gland and small left kidney with short ureter. The body also had a short neck, small chest cavities and kyphosis. X-ray revealed duplication of the vertebral column. The case presented here represents a type II of diprosopia of Rating (1933) and is the least common type reported. We also reviewed 22 recently reported cases of diprosopus. In addition to facial duplication, anencephaly, neural tube defect and cardiac malformations represent the more common congenital abnormalities associated with diprosopus. The pathogenesis of diprosopus is not well understood. Factors that play a role in diprosopus are probably similar to those factors (genetic, environmental and abnormal placental circulation) which affect monozoygotic twins as observed in this case report. Early ultrasonography diagnosis of diprosopus permits one to consider a vaginal therapeutic abortion.
Department of Pathology, Mayday University Hospital, Thornton Heath, UK.
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A
al Muti Zaitoun
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J
Chang
M
M
Booker
D000015:Abnormalities, Multiple / Q000473:pathology*; D000293:Adolescent; D000757:Anencephaly / Q000473:pathology*; D019465:Craniofacial Abnormalities / Q000473:pathology*; D004200:Diseases in Twins; D017809:Fatal Outcome; D005260:Female; D005315:Fetal Diseases / Q000473:pathology; D006257:Head / Q000002:abnormalities / Q000000981:diagnostic imaging; D006801:Humans; D008297:Male; D011247:Pregnancy; D014428:Twins, Conjoined / Q000473:pathology*; D016216:Ultrasonography, Prenatal