Craniopagus twins

Craniopagus twins
Craniopagus twins
Classification and external resources
ICD-9-CM	759.4
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Craniopagus twins are conjoined twins who are fused at the cranium. This condition occurs in about 10-20 babies in every million births in the United States. Among this small group, cephalic conjoining, or craniopagus twinning, represents the rarest of congenital abnormalities only accounting for 2-6% of all conjoined twins. Additionally, conjoined twins are genetically identical and always share the same sex. The union in craniopagus twins may occur on any portion of the calvarium, but does not include either the face or the foramen magnum. The thorax and abdomen are separate and each twin has its own ubilicus and umbilical cord. The union may involve the entire diameter of the head or only a small portion. This suggests that although there are many different kinds of variabilities already known in the scientific community, there are an infinite amount of variations that can occur. Most of these variations are based on the rotation of one twin's skull to the other and in fact, the different phenotypic sub-groups of craniopagus twins are based on all these rotational conformations. Each of these factors (rotation, spot of union) affect the development of the brain, the vascular system within the brain and overall wellness of life both of the twins have outside the womb. Relatively few craniopagus twins survive the perinatal period- approximately 40% of conjoined twins are stillborn and an additional 33% die within the immediate perinatal period, usually from organ abnormalities and failure. However 25% of craniopagus twins survive and can be considered for a surgical separation and several attempts occur yearly worldwide. In the last-half century the many advances in medicine including brain imaging, neuro-anaesthesia and neurosurgical techniques have proven that a successful outcome is possible following separation of total craniopagus twins.

There are two categories of craniopagus twins:

The exact nature of how conjoined twins develop inutero remains unclear. Embryologists have traditionally attributed identical twinning as "splitting or fission" of either the inner cell mass of pleuripotential cells or early embryonic disc at 13–14 days of gestation just before the primitive streak. Some theorists suggested that conjoined twins develop as a result of the failed fusion of a single fertilized ovum. However a new hypothesis suggests that cranial fusion occurs between two separate embryos prior to the end of the 4th week of gestation. This is able to happen because the cranial neuropore is still open which is responsible for the ultimate fusion and formation of the brain stem and central nervous system. Furthermore, this secondary fusion of embryonic discs could implicate that intact skin will not fuse to other intact skin, including the ectoderm of the embryo. This means that two embryonic discs could only unite in locations where the ectoderm is absent. Moreover, the fusion occurs from neural folds of two separate, dorsally oriented embryonic discs, and the union can occur only after the ectoderm is disrupted to allow the neural and surface ectodermal layers to separate from each other. The union in craniopagus twins may happen at any portion of the calvarium. The juncture can involve either the entire diameter of the head or any portion of the head and can be positioned at a multitude of rotational angles. In fact, craniopagus twins are rarely found in a symmetrical union. Apart from this, the vertebral axes may have a straight line. Despite this, the angle of the vertebrae is the ultimate dictator in how the individuals heads actually face. The majority of twins face either the same way or the exact opposite direction. Many reviews suggest a practical four-category system that breaks down the craniopagus twins on the basis of vertical or angular configuration or on the basis if there were shared dural venous sinuses. This scheme was applied to 64 cases and has adequately described sets of twins for over the last 86 years.

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