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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.

Gross and microscopic study of twelve human anencephalics revealed existence of the forebrain with variable growth in all specimens, meaning thereby closure of the neural tube at its cephalic extremity. This is supported by the findings of choroid plexus, pineal tissue and rudimentary pallium within the anterosuperior margin of a median dorsal opening situated behind the forebrain mass. The opening bears an intimate relation to the undue dorsal convexity of the sphenooccipital junction, and leads cranially to a cavity representing the third and lateral ventricles. This opening is situated in the posterior part of the roof of diencephalon, and seems to be the primary site of disturbance causing anencephaly. Weakness of the diencephalic roof is indicated by its epithelial nature and its ballooning in cyclopia and cebocephaly. A corresponding potential weakness in the overlying tissues is evidenced by the sagittal fontanelle of early fetal life and by congenital scalp and skull defects of the parietooccipital region.

Perirenal brown adipose tissue was studied in 49 liveborn anencephalic infants of appropriate weight, whose lifespan ranged from 2 hours to 6 days. An additional series of 187 full-term infants was used as control. Morphological evidence of active and marked BAT lipolysis was found in large number of anencephalic infants suggesting that activation of BAT metabolism leading to lipolysis in the newborn does not depend on a diencephalic pathway but on peripheral mechanisms. Image analysis study did not sustain significant differences in the intrauterine storage of lipids in brown adipose tissue cells for the anencephalic and control infants who died within 24 hours of birth.

A case of cebocephaly associated with anencephaly, preauricular appendages and single umbilical artery in a stillborn full term female fetus is described. The changing nomenclature of the median faciocerebral defects, which has led to an apparent confusion, is discussed. It is felt that paucity of literature on cebocephaly is partly due to missed diagnosis of these cases in the absence of a competent teratologist.