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Sirenomelia



Sirenomelia or Mermaid Syndrome is a very rare congenital deformity in which the legs are fused together, giving the appearance of a mermaid. This condition is found in approximately one out of every 70,000 live births[1] (about as rare as conjoined twins) and is usually fatal within a day or two of birth because of complications associated with abnormal kidney and bladder development and function. It results from a failure of normal vascular supply from the lower aorta in utero. Sirenomelia is associated with maternal diabetes.[citation needed]

There may be a connection to VACTERL association.

This disorder was formerly thought to be an extreme case of Caudal Regression Syndrome; however, it was reclassified to be considered a separate condition.

Victims were often exhibited as Fiji mermaids in wunderkammers and sideshows.[citation needed]

Additional recommended knowledge

Contents

Notable cases

Only a handful of patients who did not have the usual kidney and bladder complications have survived this condition, three of them being:

Milagros Cerrón

Sirenomelia
Classification & external resources
Newborn Milagros Cerrón
ICD-10 Q87.2
ICD-9 759.89
MeSH D004480

Milagros Cerrón Arauco (born April 27 2004 in Huancayo, Peru). Although most of Milagros’ internal organs, including her heart and lungs, are in perfect condition, she was born with serious internal defects, including a deformed left kidney and a very small right one located very low in her body. In addition, her digestive and urinary tracts and her genitals share a single tube.

A four-hour operation to insert silicone bags between her legs to stretch the skin was successfully completed on February 8 2005. A successful operation to separate her legs to just above the knee took place May 31, 2005 in a "Solidarity Hospital" in the district of Surquillo in Lima. The procedure, however, was so intensive that she became traumatised to the degree of losing her ability to form proper speech patterns, leaving her nearly mute. As yet it is not known if this is a physiological, or simply psychological condition. However, at Milagros's second birthday, her mother reported that she knew more than 50 words. A second operation to complete the separation up to the groin took place on September 7 2006.[2] A few weeks later, she took her first steps. [3]

Recently, her doctor Luis Rubio said he was pleased with the progress Milagros had made, but cautioned that she still needed 10 to 15 years of rehabilitation and more operations before she could lead a normal life. Particularly, she will require reconstructive surgery to rebuild her rudimentary anus, urethra and genitalia.

Milagros' parents are from a poor village in Peru's Andes Mountains; the Solidarity Hospital has given a job to her father Ricardo Cerrón so that the family can remain in Lima, while the City of Lima has pledged to pay for many of the operations.[4]


Tiffany Yorks

Tiffany Yorks of the United States (born 1988) underwent successful surgery to correct her rare congenital defect, in order to separate her legs. She is the longest surviving sirenomelia patient to date.

Shiloh Pepin

Shiloh Pepin of Kennebunkport, Maine in the United States [5]

References

  1. ^ Kallen B, Castilla EE, Lancaster PA, Mutchinick O, Knudsen LB, Martinez-Frias ML, Mastroiacovo P, Robert E (1992). "The cyclops and the mermaid: an epidemiological study of two types of rare malformation". J Med Genet 29 (1): 30-5. PMID 1552541.
  2. ^ BBC News 24, 7 September 2006,"Mermaid" girl's legs separated; http://news.bbc.co.uk/1/hi/health/5325082.stm
  3. ^ Daily Mail September 27 2006, "Mermaid" girl takes her first unaided steps; http://www.dailymail.co.uk/pages/live/articles/news/worldnews.html?in_article_id=407134&in_page_id
  4. ^ BBC News 28 September 2006, "Mermaid" girl takes first steps; http://news.bbc.co.uk/2/hi/americas/5381616.stm
  5. ^ "Girl with Mermaid syndrome defies the odds", Portsmouth Herald, 2 August 2007
 
This article is licensed under the GNU Free Documentation License. It uses material from the Wikipedia article "Sirenomelia". A list of authors is available in Wikipedia.
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