What do you need to know about sirenomelia syndrome?

What do you need to know about sirenomelia syndrome?

Sirenomelia 1 General Discussion. Sirenomelia, which is also known as mermaid syndrome, is an extremely rare congenital developmental disorder characterized by anomalies of the lower spine and the lower limbs. 2 Signs & Symptoms. 3 Affected Populations. 4 Standard Therapies.

What is the ICD9 code for sirenomelia syndrome?

The deformity occurs among the infants and is associated with caudal repression. Affected newborns do not survive more than 24 hours after birth. This disorder is also referred to by a number of other names, such as: The ICD9 code for this syndrome is 759.89.

How is sirenomelia related to congenital heart defects?

Congenital heart defects and respiratory complications such as severe underdevelopment of the lungs (pulmonary hypoplasia) can also be associated with sirenomelia. The exact cause of sirenomelia is unknown. Researchers believe that both environmental and genetic factors may play a role in the development of the disorder.

What is the treatment for sirenomelia in newborns?

Sirenomelia Treatment. Treatment is applicable for newborns who survive even 24 hours after delivery. Affected newborns having functioning kidneys are recommended for surgical procedures and management. Surgical operations to rebuild the gastrointestinal and urinary outlet tracts are essential for such patients.

How is sirenomelia a multifactorial genetic disorder?

Most likely, sirenomelia is multifactorial, which means that several different factors may play a causative role. In addition, different genetic factors may contribute to the disorder in different people (genetic heterogeneity). The environmental factors that play a role in the development of sirenomelia are unknown.

Which is a gene associated with sirenomelia in mice?

In animal models, several genes have been found to cause or be associated with sirenomelia. The srn ( siren) gene is observed to cause hindlimb fusion in homozygous mice. Mice with knockouts or mutations in both tsg1 and bmp7 will also have hindlimb fusion.

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What do you need to know about sirenomelia syndrome? Sirenomelia 1 General Discussion. Sirenomelia, which is also known as mermaid syndrome, is an extremely rare congenital developmental disorder characterized by anomalies of the lower spine and the lower limbs. 2 Signs & Symptoms. 3 Affected Populations. 4 Standard Therapies. What is the ICD9 code for…