Cyclopia (named after the Greek mythology characters cyclopes ), also known as alobar holoprosencephaly , is the most extreme form of holoprosencephaly and is a congenital disorder (birth defect) characterized by the failure of the embryonic prosencephalon to properly divide the orbits of the eye into two cavities. Its incidence is 1 in 16,000 in born animals and 1 in 200 in miscarried fetuses .
19-587: Typically, the nose is either missing or not functional. This deformity (called proboscis ) forms above the center eye and is characteristic of a form of cyclopia called rhinencephaly or rhinocephaly. Most such embryos are either naturally miscarried or are stillborn upon delivery . Although cyclopia is rare, several cyclopic human babies are preserved in medical museums (e.g. The Vrolik Museum , Amsterdam, Trivandrum Medical College). Some extreme cases of cyclopia have been documented in farm animals (horses, sheep, pigs, goats, and sometimes chickens). In such cases,
38-439: A proboscis is a blind-ended, tube-like structure, commonly located in the middle of the face. It is commonly seen in severe forms of holoprosencephaly that include cyclopia and is usually the result of abnormal development of the nose. Proboscis formation is classified in four general types: holoprosencephalic proboscis, lateral nasal proboscis, supernumerary probosci, and disruptive proboscis. A holoprosencephalic proboscis
57-494: A Le-Fort II osteotomy first, followed by an extension along the maxillary bone. External distraction devices may also be used to provide additional facial height and sufficient midfacial vertical length for nasal reconstruction. Lengthening the maxilla has two advantages: it provides enough height for nasal cavity reconstruction and achieves suitable aesthetic proportions. Surgical treatment methods for arrhinia can involve either staged reconstruction or simultaneous reconstruction of
76-581: A pediatrician and researcher associated with the National Institute of Dental Health , made a significant observation while studying these patients. He noted that individuals with congenital arrhinia often exhibited problems related to genital and reproductive hormones. In his comprehensive report in 1981, Dr. Bosma described two unrelated males initially reported by plastic surgeon Dr. George Gifford et al. in 1972. These patients presented with congenital arrhinia, eye defects, and genital abnormalities, such as
95-550: A small penis and undescended testes at birth, with no spontaneous sexual maturation. It is noteworthy that nearly every individual with congenital arrhinia appears to be the first and only one affected within their respective families. Nonetheless, there have been a few reports of multiple cases within the same family, with one of the earliest examples being described by Klaus Ruprecht and Frank Majewski in 1978. They reported on two German sisters who exhibited congenital arrhinia along with eye defects. Treatment focuses on identifying
114-421: A supernumerary olfactory placode. A disruptive proboscis occurs if an hamartoneoplastic lesion (benign growths such as are found in disorders like neofibromatosis and tubular sclerosis) arises in the prosencephalon (forebrain) of the embryo in its early stages of development. Arrhinia Arrhinia (alternatively spelled " arhinia ") is the congenital partial or complete absence of the nose at birth. It
133-427: Is hellebore , which has been suggested as a "natural" treatment for vomiting , cramps , and poor circulation – three conditions which may be present in the early stages of pregnancy. Cyclopia occurs when certain proteins are inappropriately expressed , causing the brain to stay whole, rather than developing two distinct hemispheres. This leads to the fetus having one optic lobe and one olfactory lobe , resulting in
152-470: Is alternatively spelled "arhinia." The phenomenon of congenital arrhinia, which refers to the complete absence of the nose from birth, was initially documented in French literature during the 1800s. Over time, a few additional cases of individuals born with congenital arrhinia, some with accompanying eye defects and others without, were reported in medical literature from the early to mid-1900s. Dr. James Bosma,
171-501: Is an extremely rare condition, with few reported cases in the history of modern medicine. It is generally classified as a craniofacial abnormality . The cause of arrhinia is not known. One study of the literature found that all cases had presented a normal antenatal history. Arrhinia is associated with the following conditions and syndromes: The term " arrhinia " is derived from the Greek roots "a-" (absence) and "rhinos" (nose); it
190-505: Is found in holoprosencephaly (a condition in which the forebrain of the embryo fails to develop into two hemispheres as it should). In cyclopia or ethmocephaly, proboscis is an abnormally formed nose. In cyclopia , a single eye in the middle of the face is associated with arrhinia (absence of the nose) and usually with proboscis formation above the eye. In ethmocephaly , two separate hypoteloric eyes (eyes placed very close together) are associated with arrhinia and proboscis formation above
209-417: The maxillozygomatic consoles and towards the medial end of the inferior orbital rim . A horizontal arm connects the two sides, and then the maxilla is carefully fractured, creating a wide median nasal cavity that extends to the upper portion of the rhinopharynx . If the maxillary height is sufficient, the patient may only need a Le-Fort II osteotomy. However, if it is not high enough, the patient will require
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#1733084772684228-517: The Hadith, would have only one eye. Mainstream Islamic scholars consider the prophecy as referring to a one-eyed man, not a cyclops. One infant whose image was circulated in 2014 was claimed to be Israeli , but was actually Bolivian and from 2008. The baby girl from India born in 2006 (see notable cases above) also had her image used. [REDACTED] Media related to Cyclopia (medicine) at Wikimedia Commons Proboscis (anomaly) In teratology ,
247-515: The eye and nose malformations of cyclopia. The Sonic Hedgehog (SHH) gene regulator is involved in the separation of the single eye field into two bilateral fields. Although not proven, it is thought that SHH emitted from the prechordal plate suppresses Pax6 , which causes the eye field to divide into two. If the SHH gene is mutated, the result is cyclopia, a single eye in the center of the face (Gilbert, 2000). First , That it had no sign of any Nose in
266-476: The eye. In cebocephaly , no proboscis formation occurs, but a single-nostril nose is present. A lateral proboscis, also known as proboscis lateralis or lateral nasal proboscis, is a tubular proboscis-like structure and represents incomplete formation of one side of the nose; it is found instead of a nostril. The olfactory bulb is usually rudimentary on the side involved in the malformation. The tear duct, nasal bone, nasal cavity, vomer (the small thin bone separating
285-449: The left and right nasal passages), maxillary sinus, ethmoidal sinuses, and another nasal structure known as the cribriform plate cells are often missing on this side as well. Ocular hypertelorism (eyes set far apart) may be present. The proboscis lateralis is a rare nasal anomaly. A supernumerary proboscis, or accessory proboscis, is found when both nostrils are formed and there is a proboscis in addition to it. Accessory proboscis arise from
304-530: The nature of the anomalies through various imaging methods, including MRI and CAT scan , and surgical correction to the extent possible. In severe cases, temporary airway devices, such as tracheostomy tubes , may be utilized to facilitate breathing until surgical intervention is deemed appropriate. Surgical procedures should only be carried out by a team of medical experts, including otolaryngologists , plastic surgeons, and prosthodontists . The arrhinia reconstruction process primarily involves two main parts:
323-590: The nose and mouth fail to form, or the nose grows from the roof of the mouth, obstructing airflow and resulting in suffocation shortly after birth. Genetic defects or toxins can misdirect the embryonic forebrain-dividing process. One highly teratogenic alkaloid toxin that can cause cyclopia is cyclopamine or 2-deoxyjervine, found in the plant Veratrum californicum (also known as corn lily or false hellebore). Grazing animals are most likely to ingest this plant and induce cyclopia in offspring. People sometimes accidentally ingest false hellebore while pregnant thinking it
342-414: The reconstruction of the nasal cavity and the reconstruction of the external nose. There are two methods for reconstructing the nasal cavity. In the first method, two separate nasal cavities are created using a dental drill, and then silicone tubes are used to keep the newly formed nasal passages open. The second method involves a Le Fort II maxillary osteotomy , which involves making an incision through
361-525: The usual place, nor had it any, in any other place of the Head, unless the double Bagg CC that grew out of the midst of the forehead, were some rudiment of it. Next , That the two Eyes were united into one Double Eye which was placed just in the middle of the Brow. The Islamic State used photos of babies born with cyclopia in its recruitment campaign. ISIS claimed the photos depicted Masih ad-Dajjal , who according to
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