Diprosopus (
GreekGreek , an independent branch of the Indo-European family of languages, is the language of the Greeks. Native to the southern Balkans, it has the longest documented history of any Indo-European language, spanning 34 centuries of written records. In its ancient form, it is the language of classical...
, "two-faced", from ,
, "two" and ,
[neuter], "face", "person"; with
LatinLatin is an Italic language originally spoken in Latium and Ancient Rome. Through the Roman conquest, Latin spread throughout the Mediterranean and a large part of Europe...
ending), also known as
craniofacial duplication (cranio- from Greek , "skull", the other parts Latin), is an extremely rare
congenital disorderCongenital disorder involves defects in or damage to a developing fetus. It may be the result of genetic abnormalities, the intrauterine environment, errors of morphogenesis, or a chromosomal abnormality. The outcome of the disorder will further depend on complex interactions between the pre-natal...
whereby part or all of the face is duplicated on the head.
Although classically considered conjoined twinning (which it resembles), this anomaly is not normally due to the fusion or incomplete separation of two embryos.
Diprosopus (
GreekGreek , an independent branch of the Indo-European family of languages, is the language of the Greeks. Native to the southern Balkans, it has the longest documented history of any Indo-European language, spanning 34 centuries of written records. In its ancient form, it is the language of classical...
, "two-faced", from ,
, "two" and ,
[neuter], "face", "person"; with
LatinLatin is an Italic language originally spoken in Latium and Ancient Rome. Through the Roman conquest, Latin spread throughout the Mediterranean and a large part of Europe...
ending), also known as
craniofacial duplication (cranio- from Greek , "skull", the other parts Latin), is an extremely rare
congenital disorderCongenital disorder involves defects in or damage to a developing fetus. It may be the result of genetic abnormalities, the intrauterine environment, errors of morphogenesis, or a chromosomal abnormality. The outcome of the disorder will further depend on complex interactions between the pre-natal...
whereby part or all of the face is duplicated on the head.
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 homologSonic hedgehog homolog is one of three proteins in the mammalian signaling pathway family called hedgehog, the others being desert hedgehog and Indian hedgehog . SHH is the best studied ligand of the hedgehog signaling pathway. It plays a key role in regulating vertebrate organogenesis, such as...
(SHH). (The unlikely sounding name of this protein was inspired by the
Sonic the Hedgehog, trademarked Sonic The Hedgehog, is a video game character and the protagonist of the eponymous video game series released by Sega, as well as in numerous spin-off comics, cartoons and books. The first game in the franchise was released on June 23, 1991, in order to provide Sega with a mascot to...
videogame character and is part of an idiosyncratic naming tradition in molecular biology research that some have criticized as frivolous.)
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. 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
cyclopiaCyclopia is a rare form of holoprosencephaly and is a congenital disorder 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 250 in embryos...
where facial features are insufficiently developed.
Healthy brain development is also dependent on the signaling function of the SHH protein. 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 disorderCongenital disorder involves defects in or damage to a developing fetus. It may be the result of genetic abnormalities, the intrauterine environment, errors of morphogenesis, or a chromosomal abnormality. The outcome of the disorder will further depend on complex interactions between the pre-natal...
s, particularly
anencephalyAnencephaly is a cephalic disorder that results from a neural tube defect that occurs when the cephalic end of the neural tube fails to close, usually between the 23rd and 26th day of pregnancy, resulting in the absence of a major portion of the brain, skull, and scalp...
,
neural tube defectIn the developing vertebrate, the neural tube is the embryo's precursor to the central nervous system, which comprises the brain and spinal cord...
and cardiac malformations. When present, the brain may show abnormalities ranging from partial to complete duplication of brain structures, and/or underdevelopment of brain tissues.
Few two-faced animals have survived due to associated internal organ abnormalities and brain abnormalities. One of the most famous was Ditto the pig. Ditto was raised to adulthood, but died of
pneumoniaPneumonia is an inflammatory illness of the lung. Frequently, it is described as lung parenchyma/alveolar inflammation and abnormal alveolar filling with fluid ....
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
MillburyMillbury may refer to a location in the United States:* Millbury, Massachusetts* Millbury, Ohio...
, USA received publicity. In this latter case, only one
esophagusThe esophagus or oesophagus , sometimes known as the gullet, is an organ in vertebrates which consists of a muscular tube through which food passes from the pharynx to the stomach. The word esophagus is derived from the Latin œsophagus, which derives from the Greek word oisophagos , lit...
(and possibly only one
tracheaThe trachea, or windpipe, is a tube that connects to the pharynx or larynx, allowing the passage of air to the lungs. It is lined with pseudostratified ciliated columnar epithelium cells with mucosal goblet cells which produce mucus...
) 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. 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 palate, a slightly divided tip of the nose, and two widely spaced eyes, as well as absence of the
corpus callosumThe corpus callosum is a structure of the mammalian brain in the longitudinal fissure that connects the left and right cerebral hemispheres. It facilitates communication between the two hemispheres. It is the largest white matter structure in the brain, consisting of 200-250 million contralateral...
, 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. She was born 10 March, 2008 to a lower-caste family who live in Sanai Sampūra village near
DelhiDelhi, known locally as Dilli , and also by the official name National Capital Territory of Delhi , is the largest metropolis by area and the second-largest metropolis by population in India...
; the birth was delayed by
dystociaDystocia is an abnormal or difficult childbirth or labour. Approximately a fifth of human labors have dystocia. Dystocia may arise due to incoordinate uterine activity, abnormal fetal lie or presentation, absolute or relative cephalopelvic disproportion, or a massive fetal tumor such as a...
caused by her large head, and she was born in a hospital with an
episiotomyAn episiotomy is a surgical incision through the perineum made to enlarge the vagina and assist childbirth. The incision can be midline or at an angle from the posterior end of the vulva, is performed under local anaesthetic and is sutured closed after delivery...
. 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
DurgaIn Hinduism, the Goddess Durga or Maa Durga "one who can redeem in situations of utmost distress"...
, who has three eyes. It was also thought that Lali was an incarnation of the Hindu god Ganesh.
As as April 2008, Sushma and Vinod Singh had declined an offer from local doctors to evaluate their daughter through CT or
MRIMagnetic Resonance Imaging , or nuclear magnetic resonance imaging , is primarily a medical imaging technique most commonly used in radiology to visualize the internal structure and function of the body...
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.
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 flashes.)
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 familyThe term Extended family has several distinct meanings. First, it is used synonymously with consanguineous family or joint family. Second, in societies dominated by the conjugal family or nuclear family, it is used to refer to kindred who does not belong to the conjugal family. Often there could be...
and her village's headman. Finally her parents, alarmed at her illness and
dehydrationDehydration is defined as excessive loss of body water. It is literally the removal of water from an object. In physiological terms, it entails a relative deficiency of water molecules in relation to other dissolved solutes...
, defied her other relatives and took her back to hospital, where under proper medical treatment including
antibioticIn common usage, an antibiotic is a substance or compound that kills or inhibits the growth of bacteria...
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.
She was buried in her village, as is usual in Hinduism with children who die very young.
Later a
templeA Hindu temple or Mandir is a place of worship for followers of Hinduism. It can be a separate structure or a part of a building. A picture of most temples is the presence of murtis of the Hindu deity to whom the temple is dedicated. They are usually dedicated to one primary deity, called the...
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
Conjoined/Siamese twins are whose bodies are joined in utero. A rare phenomenon, the occurrence is estimated to range from 1 in 50,000 births to 1 in 200,000 births, with a somewhat higher incidence in Southwest Asia and Africa. Approximately half are stillborn, and a smaller fraction of pairs...
- Janus (mythology)
In Roman mythology, Janus was the god of gates, doors, doorways, beginnings and endings. His most prominent remnant in modern culture is his namesake, the month of January, which begins the new year. He is most often depicted as having two faces or heads, facing in opposite directions...
, a Roman two-faced god
- Durga
In Hinduism, the Goddess Durga or Maa Durga "one who can redeem in situations of utmost distress"...
, a three-eyed goddess of the Hindu religion, in India
- cyclopia
Cyclopia is a rare form of holoprosencephaly and is a congenital disorder 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 250 in embryos...
- polycephaly
Polycephaly is a condition of having more than one head. The term is derived from the stems poly- meaning 'many' and kephal- meaning "head", and encompasses bicephaly and dicephaly . A variation is an animal born with two faces on a single head, a condition known as diprosopus...
External links