Examine individual changes

'{{TOCright}} '''Diprosopus''' ([[Greek language|Greek]] {{lang|el|διπρόσωπος}}, "two-faced", from {{lang|el|δι-}}, ''{{lang|el-latn|di-}}'', "two" and {{lang|el|πρόσωπο[ν]}}, ''{{lang|el-latn|prósopo[n]}}'' [neuter], "face", "person"; with [[Latin]] ending), also known as '''craniofacial duplication''' (cranio- from Greek {{lang|el|κρανίο}}, "skull", the other parts Latin), is an extremely rare [[congenital disorder]] whereby part or all of the [[face]] is duplicated on the [[head]].<ref name="MedicineNet">[http://www.medterms.com/script/main/art.asp?articlekey=14546 Definition of diprosopus] at MedicineNet. Accessed [[8 January]] [[2006]].</ref><ref name="BBC">[http://news.bbc.co.uk/1/hi/world/south_asia/7340091.stm 'Miracle baby' is feted in India] at [http://news.bbc.co.uk/ BBC News]. Accessed [[10 April]] [[2008]].</ref> == Development == Although classically considered conjoined twinning (which it resembles), this anomaly is not normally due to the fusion or incomplete separation of two embryos. It is the result of a protein called [[Sonic hedgehog|sonic hedgehog homolog]] (SHH). (The unlikely-sounding name of this protein was inspired by the [[Sonic the Hedgehog (character)|Sonic the Hedgehog]] videogame character<ref name="Donahue">[http://www.nature.com/gt/journal/v13/n13/full/3302748a.html Donahue JK. Gene therapy, angiogenesis, Sonic Hedgehog: Sonic The Hedgehog to the rescue?] [http://www.nature.com/gt/index.html Gene Therapy] 2006;13:998–999. doi:10.1038/sj.gt.3302748; published online [[2 March]] [[2006]].</ref> and is part of an idiosyncratic naming tradition in molecular biology research that some have criticized as frivolous.<ref name="Maclean">[http://www.nature.com/doifinder/10.1038/439266d Maclean K. Humour of gene names lost in translation to patients.] [http://www.nature.com/index.html Nature] 2006;439(7074):266. </ref><ref name="Discover">[http://discovermagazine.com/2007/feb/gene-change-names-hugo Bye-Bye, Sonic Hedgehog]. [http://discovermagazine.com/ Discover] [[1 February]] [[2007]].</ref>) The [[SHH protein]] and its corresponding gene have been found to play an important role in signaling craniofacial patterning during embryonic development. Among other things, the SHH protein governs the width of facial features. In excess it leads to widening of facial features and to duplication of facial structures.<ref name="Hu">[http://dev.biologists.org/cgi/reprint/126/21/4873.pdf Hu D and Helms JA. The role of Sonic Hedgehog in normal and abnormal craniofacial morphogenesis.] [http://dev.biologists.org/ Development] 1999;126:4873-84. Accessed [[9 April]] [[2008]].</ref> The greater the widening, the more of the structures are duplicated, often in a mirror image form. This has been demonstrated in the laboratory by introducing pellets of the SHH protein into chicken embryos, resulting in chickens with duplicate beaks. Insufficient amounts of that protein lead to opposite conditions such as [[cyclopia]] where facial features are insufficiently developed.<ref name="Young">[http://crobm.iadrjournals.org/cgi/reprint/11/3/304.pdf Young DL, Schneider RA, Hu D, Helms JA. Genetic and teratogenic approaches to craniofacial development.] [http://crobm.iadrjournals.org/ Crit Rev Oral Biol Med.] 2000;11(3):304-17. Accessed [[10 April]] [[2008]]. </ref> Healthy brain development is also dependent on the signaling function of the SHH protein.<ref name="Sciencedaily">[http://www.sciencedaily.com/releases/2001/03/010312072303.htm Sonic Hedgehog shapes the brain]. [http://www.sciencedaily.com/ ScienceDaily] [[13 March]] [[2001]]. </ref> During embryonic development, the SHH protein directs embryonic cells to organize in specific areas that later become specialized neural tissues, thus controlling the size and shape of brain structures. == Occurrences == Diprosopus often occurs in combination with other [[congenital disorder]]s, particularly [[anencephaly]], [[neural tube|neural tube defect]] and [[cardiac malformation]]s.<ref name="Zaitoun">[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=10048094&dopt=Abstract al Muti Zaitoun A, Chang J, Booker M. Diprosopus (partially duplicated head) associated with anencephaly: a case report. Pathol Res Pract. 1999;195(1):45-50.] Accessed at PubMed [[8 January]] [[2006]].</ref> When present, the brain may show abnormalities ranging from partial to complete duplication of brain structures, and/or underdevelopment of brain tissues.<ref name="Koseoglu">[http://www.ajronline.org/cgi/content/full/180/3/863 Koseoglu K, Gok C, Dayanir Y, Karaman C. CT and MR imaging findings of a rare craniofacial malformation: diprosopus.] [http://www.ajronline.org/ Am J Roentgenol] 2003; 180:863-4.] Accessed [[8 January]] [[2006]].</ref><ref name="Angtuaco">[http://radiographics.rsnajnls.org/cgi/content/full/19/1/260?ijkey=2601e2122235b9b3247f3345374c2457c6af9f3f&keytype2=tf_ipsecsha Angtuaco TL, Angtuaco EJ, Quirk JG. US case of the day.] [http://radiographics.rsnajnls.org/ Radiographics] 1999;19:260-63. Accessed [[20 April]] [[2008]].</ref> Few two-faced animals have survived due to associated [[internal organ]] abnormalities and brain abnormalities. One of the most famous was Ditto the pig.<ref name="Leroi">Leroi Armand-Marie (2005). ''Mutants: On the Form, Varieties and Errors of the Human Body.'' New York: HarperPerennial. (ISBN 0-00-653164-4)</ref> Ditto was raised to adulthood, but died of [[pneumonia]] caused by food inhalation when breathing through one muzzle while eating with the other. In July 2006, a 6-year-old two-faced cat called "Frank and Louie" from [[Millbury]], USA received publicity. In this latter case, only one [[esophagus]] (and possibly only one [[Vertebrate trachea|trachea]]) was functional and this aided survival. Most human infants with diprosopus are stillborn. Known instances of humans with diprosopus surviving for longer than minutes to hours past birth are very rare; only a few are recorded. In 2002 and 2003, two living male infants with partial diprosopus were described in the medical literature in separate case reports.<ref name="Hähnel">[http://radiology.rsnajnls.org/cgi/content/full/226/1/210 Hähnel S, Schramm P, Hassfeld S et al. Craniofacial duplication (diprosopus): CT, MR imaging, and MR angiography findings - case report.] [http://radiology.rsnajnls.org/ Radiology] 2003;226:210-13. Accessed [[20 April]] [[2008]].</ref><ref name="Wu">[http://www.newyorkcraniofacialsurgery.com/Resources/diprosopus.pdf Wu J, Staffenberg DA, Mulliken JB, Shanske AL. Diprosopus: A unique case and review of the literature. Teratology 2002; 66:282–87.] Accessed [[20 April]] [[2008]].</ref> One infant was born with duplication of the nose and the cerebral frontal lobes, two widely spaced eyes, a small, underdeveloped central eye socket, and a large, asymmetric mouth. The other infant was born with duplication of the upper and lower jaw, two tongues ending in the same base, [[Cleft lip and palate|cleft palate]], a slightly divided tip of the nose, and two widely spaced eyes, as well as absence of the [[corpus callosum]], duplication of the pituitary gland and stalk, and abnormalities in the midbrain. Because they were born with a milder, partial form of diprosopus, both infants were considered candidates for surgical correction of their abnormal facial features. ===Lali Singh=== In [[2008]], a baby girl born in India, Lali Singh, became the most recently known person to have the condition diprosopus.<ref name="BBC"/><ref name="ABC">[http://www.abcnews.go.com/Health/Story?id=4549608&page=1 Two-Faced Baby Triggers Shock and Awe] [[American Broadcasting Company|ABC]] report. Accessed [[21 April]] [[2008]].</ref> She was born [[10 March]] [[2008]] to a lower-caste family who live in Sanai Sampūra village near [[Delhi]]; the birth was delayed by [[dystocia]] caused by her large head, and she was born in a hospital with an [[episiotomy]]. She was one of the very few infants with diprosopus to survive well past birth. She may have been the only known living individual with complete facial duplication. Her facial features included two pairs of eyes, two noses, and two mouths (but only one pair of ears). Lali Singh, daughter of Sushma and Vinod Singh, lived in the Indian village of [[Saini Sunpura]]. There, she was seen as the reincarnation of the goddess [[Durga]], who has three eyes. It was also thought that Lali was an incarnation of the Hindu god [[Ganesh]]. As at [[April 2008]], Sushma and Vinod Singh had declined an offer from local doctors to evaluate their daughter through [[CAT scan|CT]] or [[Magnetic Resonance Imaging|MRI]] scanning. Without diagnostic imaging, it was not possible to know the full extent to which the child's condition might have affected her brain and other vital structures in her head and neck. Thus, any estimation of her ability to survive or even thrive could only be speculative, though Lali's family described her as functioning normally. It is also unknown whether neurosurgeons or craniofacial surgeons, if consulted, would have had feasible solutions to offer with respect to corrective surgery. A local doctor told reporters that the baby should be considered a healthy child who currently lives a normal life, a previously unknown occurrence among sufferers of the disorder.<ref name="ABC"/> Lali's two middle eyes suffered from [[corneal opacity]] due to abnormal anatomy of the facial muscles, which prevented her from properly closing those eyes. (Before, it was wrongly blamed on [[camera flash]]es.) [[Cleft palate]] caused difficulty feeding her under village conditions. A poor diet of bottle-fed sugar solution and diluted milk, allowed to drip down her throat because she could not suck properly because of the cleft palate, weakened her condition, and vomiting and infection started. Admission to hospital was delayed by discussion (including taking her back home from hospital) among her [[extended family]] and her village's [[headman]]. Finally her parents, alarmed at her illness and [[dehydration]], defied her other relatives and took her back to hospital, where under proper medical treatment including [[antibiotic]] and a [[saline drip]] she started to improve, and stopped vomiting, and started drinking milk and defecating normally; but 6 hours later, at two months old to the day, she died of a [[heart attack]]. <ref>[[Channel 4]] UK television program ''[[Body Shock]]'', 10 pm to 11.10 pm Tuesday 16 September 2008</ref> She was buried in her village, as is usual in Hinduism with children who die very young. Later a [[Hindu temple|temple]] was built at the village in her memory. ====External links==== *http://www.channel4.com/science/microsites/B/bodyshock/girl_two_faces/index.html ==See also== *[[conjoined twins]] *[[Janus (mythology)]], a Roman two-faced god *[[Durga]], a three-eyed goddess of the Hindu religion, in India *[[cyclopia]] *[[polycephaly]] ==References== <references/> ==External links== *[http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pubmed&pubmedid=16313388 It's all in your head: new insights into craniofacial development and deformation]. Tapadia et al, J Anat. 2005 November; 207(5): 461–477. *[http://www.nytimes.com/2006/11/12/weekinreview/12schwartz.html ‘Sonic Hedgehog’ sounded funny, at first]. New York Times, [[12 November]] [[2006]]. *[http://www.learner.org/channel/courses/biology/units/gendev/experts/incardona.html Rediscovering biology: Unit 7, Genetics of development. Expert interview transcripts, interview with John Incardona, PhD]. explanation of the discovery and naming of the sonic hedgehog gene *[http://www.genenames.org/data/hgnc_data.php?hgnc_id=10848 Page for sonic hedgehog homolog (SHH)] at [http://www.hugo-international.org/ The Human Genome Organisation (HUGO)] *[http://www.ucsf.edu/daybreak/1998/06/08_campeye.html This little piggie didn't go to the circus] Daybreak (UCSF's Electronic Daily), Campus Eye column, 8 June 1998 *[http://www.skewsme.com/twofacedkitten.html Two-faced kitten has image problem] at Channel4000 *[http://www.oregonnews.com/article/20050616/NEWS/50616015 Two-faced kitten shocks owner, veterinarian] at OregonNews.com <!--*[http://wt.us.publicus.com/apps/pbcs.dll/article?AID=/20060721/NEWS/607210688/0/FRONTPAGE Cat sees world from both sides] article about 6-year-old 2-faced cat 404? --> <!--*[http://news.yahoo.com/s/afp/20080406/hl_afp/healthindiababy Healthy baby in India born with two faces] 404 --> *[http://ca.news.yahoo.com/s/capress/080408/koddities/india_two_faced_baby Baby born with 2 faces in north India, villagers worship her as a goddess] *[http://www.msnbc.msn.com/id/24012024/ Baby with 2 faces born in north India] MSNBC *[http://cache.boston.com/resize/bonzai-fba/Reuters_Photo/2008/04/10/1207842162_9109/539w.jpg Image:Diprosopus] [[Category:Congenital disorders]] [[Category:Facial features]] [[ar:ثنائية الوجه]] [[ia:Diprosopo]] [[zh-yue:雙面人]]'