Sirenomelia

Sirenomelia

---

Quote:

Sirenomelia, alternatively known as 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 100,000 live births (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. Maternal diabetes has been associated with caudal regression syndrome and sirenomelia, although this association is not generally accepted. VACTERL-H is an expanded form of the VACTERL association that concludes that this diagnosis is a less severe form of sirenomelia. The disorder was formerly thought to be an extreme case of Caudal regression syndrome; however, it was reclassified to be considered a separate condition. 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 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, urinary tracts and genitals share a single tube. This birth defect occurs during the gastrulation week (week 3) of embyrological development. Gastrulation establishes the three germ layers: ectoderm, mesoderm and endoderm. It seems that complications such as defects in the urogenital system as mentioned above can be possibly due to malformations in the intermediate mesoderm. 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 traumatized 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 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.(BBC NEWS | Health | 'Mermaid' girl's legs separated) A few weeks later, she took her first steps. 'Mermaid' girl takes her first unaided steps | Mail Online) 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. BBC NEWS | World | Americas | 'Mermaid' girl takes first steps) Tiffany Yorks Tiffany Yorks of the United States (born May 7 1988) underwent successful surgery in order to separate her legs. She is the longest surviving sirenomelia patient to date. Shiloh Pepin Shiloh Pepin was born in Kennebunkport, Maine in August, 1999, with her lower extremities fused, a missing bladder, uterus, colon and vagina, with only one partial kidney and one ovary. Her parents initially anticipated she could expect only a few months of life. An initial kidney transplant at 4 months of age, lasted a number of years, and in 2007 a second kidney transplant was successful. She attends Consolidated Elementary School. Shiloh is the only one of the three survivors of Sirenomelia without surgery for separation of the conjoined legs

This pic shows a Japanese baby born with Sirenomelia in 2006

__________________

Now I'm Nothing
(Scum o' the earth)

source : http://www.documentingreality.com/forum/f149/sirenomelia-14733/

The Most Innocent Victims of Drugs

By Tibor Rozsahegyi

According to the National Association for Perinatal Addiction Research and Education (NAPARE), about 1 out of every 10 newborns in the U.S. is exposed in the womb to one or more illicit drugs. The most frequent ingredient in the mix is cocaine. In major cities of the USA such as New York, Los Angeles, Detroit and Washington many hospitals report that the percentage of newborns showing the effects of drugs is 20% or even higher. By the latest estimates, more than 1 million women use cocaine in the USA.

Anomalies associated with maternal cocaine use during pregnancy

Anomalies associated with maternal cocaine use during pregnancy can range from minor ones i.e., fetal cocaine syndrome: low birth weight, prematurity, irritability, microcephaly (baby's head is abnormally small), large fontanelles (the spaces where bones of the skull come together, but are not completely joined), prominent glabella (protrusion of the area between the eyes), marked periorbital and eyelid edema, low nasal bridge (a flattening of the top part of the nose), short nose, and small toenails, to major ones, i.e., segmental intestinal atresia (narrowing or absence of a portion of the intestine), sirenomelia (the term comes from "siren" or "mermaid" because of the characteristic fusion of the lower extremities that results from a failure in the development of a normal vascular supply to lower extremities), limb-body wall complex (presence of an abdominal wall defect, a short umbilical cord, abnormal curvature of the spine, limb anomaly, and craniofacial defect) and limb reduction defects (which involve missing tissue or bone in any part of a limb or limbs and can range in severity from missing fingers and toes to the complete absence of one or both arms and/or legs), congenital anomalies of the genitourinary system in infants, prune belly anomaly (when the intestinal pattern is evident through the thin protruding abdominal wall in the infant), neonatal necrotizing enterocolitis, neonatal myocardial infarction and myocardial calcification). Let us focus one of the most rare but most horrific anomalies associated with maternal cocaine use during pregnancy, gastroschisis.

Gastroschisis

Gastroschisis is a congenital disorder in which a defect is present in the wall of the abdomen of the embryo. Typically there is a small abdominal cavity with herniated abdominal organs that usually appear on the right side of the abdomen. There is no membranous sac covering the organs. The intestines may be swollen and look shortened due to exposure to the liquid that surrounds the fetus during pregnancy. Since the 1970s noteworthy trends have been observed in gastroschisis prevail. Overall, gastroschisis rates have increased over time in several regions around the world. Prevalence of gastroschisis displays wide variation by geographic location, both within and between countries. One study indicated that gastroschisis was more likely to occur in rural areas than urban ones. Birth prevalence in the United States for gastroschisis ranges between 1.22 and 5.11 per 10,000 live births (National Birth Defects Prevention Network).

Risk factors

Most studies focusing on the maternal age have found much higher rates of gastroschisis for very young mothers. The gender of the infant is associated with the risk for gastroschisis. Males are more likely than females to have gastroschisis. Maternal alcohol use has been linked to higher rates of gastroschisis, as has recreational drug use (cocaine, amphetamine, or LSD). Nevertheless scientific evidence indicates that not all people are equally susceptible to birth defects. Genetic and nutritional factors may combine with other environmental factors to increase the risk. This combination of factors makes it extremely difficult to conduct epidemiologic studies in populations of people when the entire collection of risk factors is not well understood or identified.

Cocaine and gastroschisis

Cocaine causes blood vessels to constrict, reducing the vital flow of oxygen and other nutrients. Because fetal cells multiply fleetly in the first months of the pregnancy, the proper blood supply of the embryo is set back by the mother's early and continuous use of cocaine. The heavy maternal cocaine use during the later months of pregnancy can lead to an embolism, or clot, that lodges in a fetal vessel and completely disrupts the blood supply to an organ or limb. The result: different kind of deformities (shriveled arm or leg, missing section of intestine or kidney etc. Fortunately such bold defects are very rare. Thus the link between maternal cocaine use and increased gastroschisis risk is of particular interest because cocaine is a vasoconstrictor. One hypothesis offered for the etiology of gastroschisis is that it is a vascular disruption defect.

Diagnosis

The possibility of prenatal diagnosis either through echosonogram or any other method available allows the mother to be referred to an adequate center where a caesarean section or induced natural birth can be performed before term (as natural birth is recommended and just as safe as with a normal baby), preferably within 2 weeks of term, and allow the immediate surgery to be performed on the newborn. The main cause for lengthy recovery periods in patients is the time taken for the infants' bowel function to return to normal. The morbidity is closely related to the presence of other malformations and complications of the wound or the intestine. Patients frequently require more than one surgery. The fetal abdominal wall can be seen by ultrasound from 9 postmenstrual weeks although the defect cannot not been confidently diagnosed until after the 12th week of pregnancy.

Treatment and Prognosis

There is a small risk of a chromosomal abnormality and other defects cannot be always excluded either, women who have a fetus with a gastroschisis an amniocentesis, also referred to as amniotic fluid test or AFT have been offered to check the karyotype. AFT is a medical procedure used in prenatal diagnosis of chromosomal abnormalities and fetal infections, in which a small amount of amniotic fluid contains fetal tissues, is extracted from the amnion or amniotic sac surrounding the fetus, and the fetal DNA is examined for genetic abnormalities. Most women are to be subjected to additional surveillance with ultrasound biophysical tests and or cardiotocographs (CTG) during the third trimester. In spite of all best efforts taken by doctors about 30% of women will labor prematurely (before 37 weeks gestation). The good news is most women will not have another fetus with gastroschisis (although there is a 3 - 5% reported recurrence risk). For babies born with gastroschisis, the defect requires immediate surgery and intensive hospital care. Some babies with gastroschisis born to have strongly damaged bowel. Besides, they can have blockages in the bowel. If these blockages have been present for a long time, they can cause parts of the bowel to be greatly distended. Even when these blockages are relieved after birth it can sometimes take many months for the bowel to function normally. So it can take many months for the baby to tolerate milk. They have a long stay in hospital and require intravenous feeding. Fortunately most babies do not have these types of serious problems and feeding can be established within 7 - 21 days of age. Most babies with gastroschisis are discharged within 3-4 weeks of birth. Nowadays 90% of the neonates could survive due to the current advances in surgical techniques and intensive care management.

http://www.rosehillpublishing.webs.com

Article Source: http://EzineArticles.com/?expert=Tibor_Rozsahegyi

Details About Sirenomelia (Mermaid Syndrome)

By Tauqeer Ul Hassan

Sirenomelia or Mermaid Syndrome is a very irregular congenital deformity in which the legs of the patient merged together in such a way so as to appear like a mermaid's tail.

This syndrome is very rare and occurs in one out of 100,000 live births. The first couple of days after birth are very critical due to its complications, which arise from the abnormal bladder development, kidney and function. It has been seen that more than half of sirenomelia cases result in stillbirth and this condition is more common in identical twins than in single births or fraternal twins. Although, often-maternal diabetes is associated with sirenomelia and caudal regression syndrome, this thought is opposed by number of sources.

This condition is very critical with high mortality rate, but yet few patients without typical kidney and bladder complications have able to survive. Following are given the brief history of three such patients:

Milagros Cerrón

She was born in Huancayo, Peru on April 27, 2004. Although, most of her internal organs such as heart and lungs are in good condition, her several internal organs were defective. She was born with a deformed left kidney and the other small one located extremely low in her body. The same tube was shared for her urinary, digestive tracts and genitals. These defects are caused in the urogential system by malformations in the intermediate mesoderm.

Silicon bags were inserted between her legs to stretch the skin through a successful operation in February 8, 2005. An operation was conducted on May 31, 2005 for the sake of separating her legs. Another operation was conducted in association with the previous operation to complete the process of separation on September 7, 2006. Milagros took her first steps couple of weeks after the successful operation.

However, the doctor of Milagros warned her that she still needed 10 to 15 years of therapy before she will be able to live a normal life and these extended periods of therapy includes many critical operations and surgeries.

Tiffany Yorks

Tiffany Yorks born in United States on may 7, 1988 and she is currently the longest surviving patient with sirenomelia. Before she was one, she went through several surgeries for her legs to be separated. No matter how successful the surgeries were, she is still not strong enough to get around, as her fragile legs don't support her much. She normally uses crutches or wheelchair.

Shiloh Pepin

She was born in August 4, 1999 in Kennebunkport, Maine, United States. She was born with her lower extremities fused, without uterus and bladder, no vagina except 6 inches of the large colon and just one quarter of kidney and ovary. At the age of three, her natural kidney failed and a successful kidney transplant was performed. In 2007, second kidney transplant was performed. Unfortunately, she died of severe pneumonia at Maine Medical Centre, Portland, Maine at the age of ten on October 23, 2009.

You might want to learn more about Mermaid Syndrome [http://www.mermaidsyndrome.org/].

Article Source: http://EzineArticles.com/?expert=Tauqeer_Ul_Hassan

Milagros Cerrón

Mermaid baby first steps

Peru's 'Miracle Baby' Walks on Her Own

Peru's 'Miracle Baby' Walks on Her Own

Friday, April 20, 2007 (04-20) 19:13 PDT LIMA, Peru (AP) --

Peru's "miracle baby" walked around her nursery school yard Friday, ducking in and out of a plastic playhouse seven months after undergoing an operation to fully separate her fused legs.

Milagros Cerron, whose first name means "miracles" in Spanish, was born with a rare congenital defect known as sirenomelia, or "mermaid syndrome," which left her legs connected from her heels to her groin.

Dr. Luis Rubio, head of the medical team that separated Milagros' legs, invited reporters to see her progress on Friday. He said doctors have successfully reconstructed the child's hips, knees and ankles and that she is "doing well physically."

But Rubio said Milagros — who is called "the little mermaid" by Peruvians — will need another operation in about two years to reconstruct and repair her urinary and sexual organs.

"We've gotten past the first stage, but it's not the last," Rubio said. "There's a long way to go."

Milagros, who turns 3 years old next week, now takes ballet classes and runs around the playground with her classmates.

In June 2005 doctors successfully performed risky surgery to separate her legs to above her knees. The operation seven months ago was to separate the remaining four inches of fused tissue just below the groin.

Rubio has said Tiffany Yorks, a 17-year-old American, is the only other person known to have undergone successful surgery to correct the rare congenital defect, which occurs in one out of every 70,000 births and is almost always fatal within days of birth.

Milagros' family comes from a poor village in the Andes mountains but Lima's municipal government has agreed to pay for her medical care.

Sirenomelia [Mermaid syndrome]

Sirenomelia

Sirenomelia, alternatively known as 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(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. Maternal diabetes has been associated with caudal regression syndrome and sirenomelia, although this association is not generally accepted.

VACTERL-H is an expanded form of the VACTERL association that concludes that this diagnosis is a less severe form of sirenomelia.The disorder was formerly thought to be an extreme case of Caudal regression syndrome; however, it was reclassified to be considered a separate condition.

Notable cases

Milagros Cerrón
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, urinary tracts and 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 traumatized 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 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. A few weeks later, she took her first steps.

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.


Tiffany Yorks
Tiffany Yorks of the United States (born May 7 1988) underwent successful surgery in order to separate her legs. She is the longest surviving sirenomelia patient to date.

Shiloh Pepin
Shiloh Pepin was born in Kennebunkport, Maine in 1999, with her lower extremities fused, a missing bladder, uterus, colon and vagina, with only one partial kidney and one ovary. Her parents initially anticipated she could expect only a few months of life. An initial kidney transplant at 4 months of age, lasted a number of years, and in 2008 a second kidney transplant was successful. She attends Consolidated Elementary School. Shiloh is the only one of the three survivors of Sirenomelia without surgery for separation of the conjoined legs.