Cyclopia

Cyclopia (from Greek Κύκλωπες, Kreisäugige ) is a rare craniofacial malformation, merge with the fetus in the two eyes investments in a bony eye socket (orbit ). In an incomplete fusion of the eyes in an orbit is called a synophthalmia. This disorder is usually associated with a holoprosencephaly, and then provides the worst form of expression dar. Due to the strong malformations are not Cyclops viable. Cyclopia occurs both in humans and in animals.

Epidemiology

The cyclopia is a rare malformation. The prevalence of 1:100,000 births. About 60% of children are born alive. Female children predominate, with almost 60%. There are no regional clusters.

Causes

Approximately 30% of cases there is a chromosomal aberration, primarily trisomy 13, above. Significant links with factors such as high age at childbirth the mother or twin and multiple pregnancies do not exist. Teratogenic factors during pregnancy may be disorders of cholesterol biosynthesis, viral diseases, maternal consumption of alcohol or gestational diabetes. There are case reports of cyclopia as a result of a teratogenic effect of drugs.

In sheep, the Veratrum californicum in Alkaloidwirkung of consumption can trigger a cyclopia by the pregnant ewe. Treatment with fenbendazole in sheep can lead already in double dose to cyclopia.

Formation

A cyclopia has its origin in a faulty circuit or failure of the neural tube. Due to the missing or incomplete separation of the rudimentary eyes nose in embryonic development can not walk down and remains as Proboscis called proboscis Annex exist above the eye. A cyclopia is due to the underlying neural tube defect is often associated with other severe malformations.

Clinical appearance

Patients with cyclopia vary significantly according to severity on a median Monophthalmie, synophthalmia or anophthalmia. There is a trunk- like extension on the front, the proboscis is called. This is the original nose system, and can be pronounced nose-shaped. The nose may be duplicated, being single standing or entirely absent. If it is present, it can be provided with only one opening. The rest of the physical development of the child may be age-appropriate and unobtrusive.

Diagnostics

The disease can be diagnosed sonographically from the 14th week of pregnancy. In the ultrasound image of the face is typically found only a Orbitaanlage. There has also been further for a alobäre holoprosencephaly typical changes. A cytogenetic investigation eg amniocentesis can detect in the context of prenatal diagnosis of chromosomal aberration where appropriate underlying.

Therapy and prophylaxis

No treatment is possible. Children with cyclopia are not viable. You die within the first week of life. With a diagnosis during pregnancy is an indication for termination of pregnancy. If there is a genetic defect or a chromosomal aberration, a Genetic counseling of parents should be.

In veterinary medicine, a precise identification of the mode of inheritance and a subsequent culling of affected animals from the breeding pool should be done. If there is an exogenous teratogenic cause, the substance ( for example, contaminated feed ) should be identified and the further supply are prevented.

History and Cultural Aspects

The name is derived from the Cyclopia Cyclops. Here are figures from Greek mythology. Characteristic of Cyclops is a single eye on the forehead. Even if the meaning of the word from the ancient Greek word Κύκλωπες ( Kreisäugige ) derives Cyclops in art were often depicted with normal almond-shaped eyes. The most famous Cyclops of Greek mythology meets the giant Polyphemus, son of Poseidon, Odysseus in the Homeric epic, the Odyssey.

The origin of the Zyklopenglaubens seems to go back to prehistoric and ancient discoveries of fossil elephant skull. When an elephant skull centrally located nostril can easily be mistaken for a single orbit, as in elephant 's eyes are not limited by a bony eye socket. This theory would explain why Cyclops in mythology are often big time.

Another possible origin for the Cyclops is the mythological processing of human cyclopia.