Sirenomelia: developmental features and prognosis for mermaid syndrome

The term "sirenomelia" comes from the Greek "seiren", which means a fairy-tale character, a siren, seducer and "melos" - a limb, part of the body.

Mermaid syndrome is an extremely rare congenital anomaly.

Because of the fused legs, the little ones are like a mermaid. The prognosis for the disease is disappointing. Treatment practically does not lend itself to treatment.

The disease is a series of abnormalities in newborns:

• fusion of the lower extremities( the bones either grow togethersoft tissues);
• absence of rectum, bladder, sacral spine;
• absence or deviation of the external and internal genitalia;
• fused lower limbs;
• underdevelops the gastrointestinal tract;
• bilateral renal adenogenesis.

In children born with an anomaly, there is only one umbilical artery, instead of the two prescribed, as well as large trunks of arteries of the lower extremities, hypoplasia of the aortic bifurcation. Lower mesenteric and renal arteries may be absent. At the same time there is a violation of the blood supply, which develops a terrible disease.

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Causes of pathology

Until now, doctors and scientists can not find out the causes of sirenomelia. But there are several theories to which the declined:

• anomaly in the genes of the newborn's parents;
• alcohol abuse;
• disease of the expectant mother during pregnancy;
• insufficient blood supply to the lower limbs of the fetus.

Environmental factors that affect the development of the mermaid syndrome are not defined. But 16% of cases are registered in pregnant women, who are ill with diabetes mellitus.

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Some believe that syrenomelia is a multifactorial anomaly, which should be several reasons that led to the formation of a deviation.

Degree of severity of mermaid syndrome

There are two degrees of severity of sirenomelia:

1. Soft degree of .In this case, the soft tissues of the feet merge into one. The lower extremities can be fixed both completely and at the ankles. The development of the main leg bones is absolutely correct. Using the operation, you can correct the defect of the lower limbs. Other lesions are determined with x-rays and with special tests.
2. Heavy Duty .This degree of deformation in a newborn is so neglected that one does not want to present this picture. Two legs are absolutely poorly developed and fully fused. The structure of the foot can be completely absent, and along the entire lower limb there are only two bones.

Diagnosis in the uterine period

In the womb of the future mother, from the first weeks of the conception of the fetus, the congenital fusion of the lower extremities develops. Already at 11 weeks, using ultrasound, diagnose mermaid syndrome.

With fetal ultrasound in a pregnant woman, there is a lack of water, the embryo does not see the kidneys and there is a defect in the lower extremities. With the diagnosis of fetal "sirenomelia" in the early stages of pregnancy, self-abortion can occur.

Chances are minimal

If the newborn survives, surgery is performed to separate the lower extremities, sometimes a series of operations is necessary.

Since a child is born with deviations not only of the legs, but also of the internal organs, surgical treatment is necessary. Other methods of therapy only support the patient's condition.

According to statistics, 1: 100000 babies are born with an anomaly, newborns with a bleeding are terminally lethal during labor or before them, only 50% are born alive.

Only live-born babies do not live long, only a couple of days, in rare cases a few months. This factor is explained by an abnormality of the internal organs, such as renal failure and absence of the bladder.

Not( happy) stories of surviving

But there are always exceptions. There are three stories about patients who struggled for their lives, they managed to grow and develop for several years:

1. So, in America, a girl was born in 1999 with a pathology, her name was Shilo Pepin .To continue her life, she was given two operations by
   

   Shilo Pepin

   on kidney transplantation, and she grew up with fused limbs all her life, since the peculiarity of their construction did not allow surgical intervention. At 10 years old Sheila died of pneumonia.

2. In 2004, a baby Milagros Cerron was born in Peru. She had abnormalities in the digestive tract, kidneys, genitalia and urethra. In 2 years, an operation was performed to separate the limbs. A few weeks after the successful series of operations, the girl took the first steps. Also, the child was operated on to restore internal organs.
3. American girl Tiffany Yorks - the oldest "mermaid".She was born in 1988, and is still alive. She was given several operations to divide her legs and restore the working capacity of internal organs. All surgical interventions were successful, despite this, the girl's legs are weak, she has to move with crutches or a stroller.

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