Unique medical findings

Diprosopus also known as craniofacial duplication (cranio- from Greek κρανίον, “skull”, the other parts Latin), is an extremely rare congenital disorder 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. It is the result of a protein called sonic hedgehog homolog (SHH). (The unlikely-sounding name of this protein was inspired by theSonic the Hedgehog 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 cyclopia 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.

Diprosopus often occurs in combination with other congenital disorders, particularly anencephalyneural tube defect 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 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 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. 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 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. She was born March 10, 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 godGanesh.

As of April 2008, Sushma and Vinod Singh had declined an offer from local doctors to evaluate their daughter through CT or 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.

 

The baby girl was born in the rural Nagar district of Uttar Pradesh, 50km north east of New Delhi.  Her parents, Vinod and Susham Singh from a village called Saini, said their little girl was “a gift from God”.  “Excited villagers claim she is the reincarnation of the Indian God Ganesha and celebrated her arrival with clapping, cheering and offerings of gifts and money.”

They were initially uncertain whether the baby would have normal functions but say so far she is “doing well” and eating from both of her two mouths.  She also opens and shuts all four eyes at the same time.

See for update on Lali see:  https://frontiersinneuroscience.wordpress.com/2011/03/19/baby-with-facial-duplication/

 

Ischiopagi

Ischiopagi is the medical term used for conjoined twins (Class V) united at the pelvis.

It specifically describes a condition involving the fused lower half of the two bodies, with spines conjoined end-to-end at a 180° angle. Ischiopagic twins have four arms; two, three or four legs; and typically one external genitalia and anus.

Lakshmi Tatama is an Indian girl born in 2005 in a village in Araria districtBihar, having “4 arms and 4 legs.” She was actually one of a pair of ischiopagus conjoined twins where one twin was headless due to its head atrophying and chest underdeveloping in the womb. The result looked like one child with four arms and four legs.

Her father, Shambhu, and mother, Poonam, were day laborers who earned less than 40 Rupees per day, and were unable to afford a separation surgery for their daughter. The daughter was named after Lakshmi, the multi-limbed Hindu goddess of wealth. She was sometimes an object of worship as an incarnation of the goddess Lakshmi; when she was 2 years old, she was known all over India. At one point, a circus had offered the couple a sum of money to buy Lakshmi as a sideshow, which forced them into hiding. At the time of being found by Dr. Sharan Patil, she was suffering from an infected pressure ulcer at the neck end of the parasitic twin and continuous fevers.

Lakshmi is the second child of the family. Her older brother is named Mitelesh.

Poonam said in a television program that a few weeks before giving birth to Lakshmi, she had a dream which told her to build a temple to the goddess Lakshmi. Subsequently, Lakshmi was born during the “Festival of Lakshmi”.

In a 40 hour operation, doctors successfully removed the lifeless body from Lakshmi Tatma, who was hailed as a reincarnation of Vishnu.

Since the operation Lakshmi has successfully taken her first steps.

Her mother Poonam Tatma said she believed her daughter was “a miracle”.

Before the operation, while being treated for the ulcer and the fever, she was again sometimes an object of worship as an incarnation of the goddess Lakshmi. She could drag herself around, although impeded by the trailing parasitic twin.

She was the subject of a surgery carried out by Dr. Sharan Patil and 30 other physicians which included Chief Anesthetist Dr. Yohannan John at Sparsh Hospital in BangaloreKarnataka.

The twins’ two pelvises formed a single combined ring. Each twin had one working kidney. Lakshmi had a second kidney which was necrotic. The autosite‘s feet were affected by clubfoot.

Her abdominal aorta gave off iliac branches to the autosite’s legs and continued as a main trunk artery which gave off iliac branches to the parasite’s legs and continued, and finally forked into the parasite’s subclavian arteries.

The parasitic twin’s spine and abdomen merged with Lakshmi’s body. The twins’ backbones were joined end-to-end and nerves were entangled. Lakshmi could not crawl normally or walk, but she could drag herself around somewhat. Doctors surmised early on that without the operation, she would not be able to live into her teens. The surgery began on Tuesday, 6 November 2007, at 7 am IST (1:30 AM UTC), and was planned to last 40 hours at the most. An estimated cost of over USD$625,000 was paid entirely by the hospital’s charitable wing Sparsh Foundation. A team of more than 30 surgeons worked in shifts. The surgery lasted for 27 hours. The doctors gave Lakshmi a 75-80% chance of survival during the surgery.

The steps of the operation were:

  1. (8 hours): Abdominal operation: remove the parasite’s abdominal organs.
  2. Remove the autosite’s necrotic kidney and replace it with the parasite’s kidney. Tie off the blood vessels that supplied the parasite.
  3. Move the reproductive system and the urinary bladder into the autosite.
  4. (6 to 8 hours) Amputate the parasite’s legs at the hip joints: this caused heavy bleeding. Cut the joined backbone: the nervous system around the join was found to be extremely chaotic, and care had to be taken to avoid causing paralysis.
  5. Separation, at 12.30 am on 7 November 2007. The combined pelvic ring was divided through or near the parasite’s hip joints and not at the pubic symphyses. The remaining incomplete pelvic ring was cut and bent to make the ends meet, and not left as an open part-circle.
  6. External fixation to hold the parts of the pelvis in place. This caused the pelvis to close in 3 weeks to the normal position.
  7. (4 hours): Suturing. Operation completed at 10 am on 7 November 2007.

After the operation, the camera showed the amputated parasite and its legs laid out so that for a while they looked like a separate human form.

Lakshmi’s recovery so far has been swift and satisfactory. Within a week after the surgery, the doctors held a press conference showing Lakshmi being carried by her father. Her feet were still bandaged. She was in the hospital for a month after the operation.

Afterwards, she and her family moved to Sucheta Kriplani Shiksha Niketan in Jodhpur in Rajasthan, where Lakshmi joined a school for disabled children and her father got a job on that school’s farm.

As of February 2008, a later operation is planned to bring her legs closer together. Another operation may be needed to rebuild pelvic floor muscles.

The last view of her in the first television program showed her making an effort to walk, with her thighs fastened together with a spacer to keep her pelvic ring in place while it heals, and casts on her shins and legs.

The second television program showed her recovering well. It was found that cutting the conjoined vertebral column in the separation operation did not cause paralysis. She was operated on for clubfoot. X-ray showed that extra bone between the pubic symphysis (parts of the parasitic twin’s ischium andpubis bones) had been absorbed, or were not ossified. Slight scoliosis was found, but was correctable by a minor operation; her mother was unwilling for her to have to wear a spinal brace through all childhood.

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