Health Information Management

Sirenomelia sequence

JustCoding News: Outpatient, September 23, 2009

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Sirenomelia sequence is a very rare congenital anomaly in which babies are born with either one lower extremity or two legs fused together. Approximately 300 cases have been reported. Other names for this condition include mermaid malformation, fused legs and feet, sirenomelus, and mermaid syndrome.

Males are three times as likely as females to have the condition. Most cases end in fetal death due to other associated anomalies.

Types/stages
Symelia, the root word for sirenomelia, refers to the fusion of the lower extremities. It is classified according to three types:

  • Apus: This indicates no feet, one tibia, and one femur.
  • Unipus: This indicates one foot with two femurs, two tibias, and two fibulas.
  • Dipus: This, the most common type, indicates fusion of two feet and two legs, giving the appearance of a flipper. Dipus is often referred to as mermaid syndrome.

Signs and symptoms
A fetus with this condition sometimes has many associated anomalies, including:

  • A single umbilical artery
  • Imperforate anus
  • Cardiac anomalies
  • Abdominal wall defects
  • Malformed thorax
  • Lower sacral/vertebral defects

The fetus also may have genitourinary system agenesis, meaning either ambiguous or no external genitalia. Renal agenesis and lung hypoplasia are some of the most severe associated anomalies.

Treatment
Physicians often diagnose sirenomelia sequence due to unexplained oligo-anhydramnios found during the second trimester of pregnancy. Although there may be many other possible causes of oligo-anhydramnios, sirenomelia sequence can be differentiated by the anomaly of the legs. Due to the prognosis, physicians often recommend termination of pregnancy.

Documentation and coding
Due to the infrequency with which patients survive the condition after birth, there is no diagnosis more specific. Even in ICD-10, sirenomelia sequence continues to be a nonspecific diagnosis code.

ICD-9-CM coding

  • Other and unspecified congenital anomalies, other specified anomalies, other: code 759.89

ICD-10-CM coding

  • Congenital malformation syndromes predominantly involving limbs: code Q87.2



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