Diprosopus
Diprosopus (from Greek διπρόσωπος, “two faces”), also known as craniofacial duplication or double face , is an extremely rare congenital disorder in which part or all of the face is duplicated on the head.
Emergence
Although Diprosopus is classically considered to be Siamese twinning , this anomaly does not usually occur due to fusion or incomplete separation of two embryos, but is a result of the hedgehog protein . This protein and its corresponding gene play an important role in the formation of facial features during embryonic development. Among other things, it regulates the width of the facial features. In excess, it leads to the broadening of facial features and the duplication of facial structures. The greater the broadening, the more structures are duplicated, often in mirror image form. This effect has been demonstrated in the laboratory by the fact that chickens in which Sonic Hedgehog pellets had been introduced into the embryo were later born with double bills. Insufficient amounts of this protein, on the other hand, lead to the opposite results, such as cyclopia , where facial structures are not sufficiently developed.
The healthy development of the brain also depends on the signaling function of the Sonic Hedgehog protein. During embryonic development, the protein directs the embryonic stem cells to specific areas that later form specialized neuronal tissues, thereby controlling the size and shape of the brain structures.
Occurrence
Diprosopus often occurs in combination with other malformations , especially anencephaly , neural tube defects, and cardiac malformations . When present, the brain can exhibit abnormalities ranging from partial or total duplication to underdevelopment of brain tissue.
Few two-faced animals have survived for long periods of time due to the abnormalities in the internal organs and brain associated with the disorder. One of the most famous was the pig Ditto. Ditto survived into adulthood, but then died of pneumonia caused by inhalation of food as a result of eating and breathing through the two existing mouth openings at the same time.
Cats
Cats with this malformation are after the Roman god Janus , who has two faces, Janus cats called. In July 2006, a six year old male named " Frank and Louie " from Millbury caught attention. In his case, only one esophagus and one trachea were functional, which made survival easier. When Frank and Louie turned twelve in September 2011, it was announced that he would appear in the Guinness Book of World Records for 2012 as the longest-surviving Janus cat. According to The Telegram of Worcester , this cat died on December 4, 2014 at the age of 15.
In June 2013 another Janus cat was reported. The kitten "Deucy" was discovered by children in Oregon . Since the mother cat did not accept her offspring, an attempt was made to raise Deucy by hand. According to a veterinarian who had examined the kitten, it was completely healthy except for this malformation and thus had a good chance of survival, but it died 2 days later.
People
Most human infants with this malformation are stillborn. Known cases of people surviving with it for more than a few minutes to hours are very rare and only a few are recorded. In 2002 and 2003, two live male infants were described in separate case reports in the medical literature . One of the infants was born with a doubled nose and double frontal lobes , two widely spaced eyes, a small, underdeveloped eye socket in the center, and a large, asymmetrical mouth. The other infant had a double upper and lower jaw, two tongues ending in the same root, a cleft lip and palate , a slightly divided nose tip, two widely spaced eyes, no cerebral bar , a doubled pituitary gland, and abnormalities in the midbrain. Because they showed only partial malformations, both children were considered candidates for surgical correction of their abnormal facial features.
Lali Singh
In 2008, the girl Lali Singh was born in India, who is now considered the most famous person with complete diprosopus. She was born on March 10, 2008 in the village of Sanai Sampūra near Delhi . The birth was delayed because of her large head and she was born in a hospital with the assistance of a perineal cut . She was one of the few infants who survived well after birth and the only known living person with a complete doubling of the face. She had two pairs of eyes, two noses and two mouths, but only one pair of ears. In her village she was seen as a reincarnation of the goddess Durga , who has three eyes, or as an incarnation of the Hindu god Ganesha .
In April 2008, parents Sushma and Vinod Singh turned down an offer from local doctors to scan their daughter with computed or magnetic resonance imaging . Without these images, it was not possible to capture the full extent of the disorder and the condition of the infant, brain and other vital structures in the head and neck. So any assessment of her viability could only be speculative, even though her family described it as functioning normally. Likewise, it is also unknown whether neurosurgeons or craniofacial surgeons could have offered feasible solutions to corrective surgery. A local doctor told reporters that the baby should be viewed as a healthy child, leading a normal life, which until then was unique among those with the disorder.
Both of Lali's middle eyes had corneal opacity due to the abnormal anatomy of the facial muscles, which made it impossible for her to completely close her eyes. Initially, the flash lightning storm was wrongly blamed for this.
The cleft palate caused problems in feeding the infant adequately under village conditions. A poor diet of sugar solution and diluted milk dripped straight down her throat with the bottle because she could not swallow properly made her condition weak and caused vomiting and infection. Admission to the hospital was delayed by discussions between her extended family and the village chief. Eventually, because of her illness and dehydration, her parents took her to the hospital against the wishes of the other relatives, where her condition began to improve under medical treatment with antibiotics and a drip of saline solution. The vomiting stopped, she drank milk again and had normal bowel movements. But six hours later, when she was exactly two months old to the day, she died of a heart attack . She was buried in her village, as is the custom in Hinduism for children who die very young. A Hindu temple was later built in her village in her memory.
Individual evidence
- ^ Definition Diprosopus , MedicineNet.com
- ↑ a b Sanjoy Majumder: 'Miracle baby' is feted in India , BBC News
- ↑ Diane Hu, Jill A. Helms: The role of Sonic hedgehog in normal and abnormal craniofacial Morphogenesis (PDF; 712 kB)
- ↑ DL Young, RA Schneider, D. Hu, JA Helms: Genetic and Teratogenic Approaches To Craniofacial Development (PDF; 2.3 MB)
- ^ Sonic Hedgehog Shapes The Brain , ScienceDaily
- ↑ A. al Muti Zaitoun, J. Chang, M. Booker: Diprosopus (partially duplicated head) associated with anencephaly: a case report . PMID 10048094
- ↑ Kutsi Koseoglu, Cantay Gok, Yelda Dayanir, Can Karaman: CT and MR Imaging Findings of a Rare Craniofacial Malformation: Diprosopus . In: American Journal of Roentgenology
- ↑ Teresita L. Angtuaco, Edgardo J. Angtuaco, J. Gerald Quirk Jr: US Case of the Day. RadioGraphics
- ^ Armand Marie Leroi (2005). Mutants: on the form, varieties and errors of the human body. Harper Perennial, New York NY, ISBN 0-00-653164-4 .
- ↑ Cherry Wilson: Two-faced cat is a record breaker . In: The Guardian
- ↑ Roderique Ngowi: Meet Frank and Louie, the longest surviving two-faced cat .
- ^ The Telegram of Worcester
- ↑ Kittens born with two faces . In: n24.de, June 12, 2013.
- ↑ Farewell, Deucy! Rare 'Janus' kitten born with two faces dies aged TWO DAYS after sparking Internet craze
- Jump up ↑ Stefan Hähnel, Peter Schramm, Stefan Hassfeld, Hans H. Steiner, Angelika Seitz: Craniofacial Duplication (Diprosopus): CT, MR Imaging, and MR Angiography Findings - Case Report . ( Memento of the original from January 2, 2012 in the Internet Archive ) Info: The archive link was inserted automatically and has not yet been checked. Please check the original and archive link according to the instructions and then remove this notice.
- ↑ a b Nick Schifrin: Two-Faced Baby Triggers Shock and Awe . abc news
- ↑ Top 10 Bizarre Medical Anomalies
