Health Information Management

Zellweger syndrome

JustCoding News: Inpatient, March 17, 2010

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Zellweger syndrome is one of a group of genetic disorders called peroxisomal diseases. It affects brain development and the growth of myelin sheath, the fatty covering that insulates nerve fibers in the brain.

Zellweger syndrome is an inherited disorder. It occurs in one out of every 50,000 to 100,000 births. It affects both males and females. Both parents must carry the defective gene to pass it on to their child. Under these circumstances, each child has a 25% chance of being born with Zellweger syndrome.

Signs and symptoms
The most common features of Zellweger syndrome include:

  • An enlarged liver
  • High levels of iron and copper in the blood stream
  • Vision disturbances

Some affected infants show prenatal growth failure.

Symptoms at birth may include:

  • Lack of muscle tone
  • Inability to move
  • Glaucoma

Other symptoms may include unusual facial characteristics, mental retardation, seizures, and an inability to suck/swallow. Jaundice and gastrointestinal bleeding also may occur.

The prognosis for individuals with Zellweger syndrome is poor. There is no cure, nor is there a standard course of treatment. Death usually occurs by the age of six months, often resulting from respiratory distress, gastrointestinal bleeding, or liver failure.

Documentation and coding
Reference, Syndrome, Zellweger in the Alphabetic Index.

ICD-9-CM coding

  • Peroxisomal disorders: code 277.86

ICD-10-CM coding

  • Zellweger syndrome: code E71.510
  • Zellweger-like syndrome: code E71.541

Additional documentation
In ICD-10-CM there is a specific code for this syndrome as well as conditions that are described as Zellweger-like.

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