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ICD10 code of Miller-Dieker syndrome and ICD9 code

What is the ICD10 code for Miller-Dieker syndrome? And the ICD9 code for Miller-Dieker syndrome?

ICD9 and ICD10 codes of Miller-Dieker syndrome

Miller-Dieker syndrome is a rare genetic disorder characterized by a smooth brain surface (lissencephaly) and developmental delays. The ICD-10 code for Miller-Dieker syndrome is Q04.3. In the previous coding system, the ICD-9 code for this condition was 742.2. It is important to consult with a healthcare professional for accurate diagnosis and coding.


Miller-Dieker syndrome, also known as lissencephaly type 1, is a rare genetic disorder characterized by abnormal brain development resulting in smooth brain surfaces instead of the normal convoluted appearance. This condition is caused by a deletion of genetic material on chromosome 17, specifically the 17p13.3 region.

In terms of coding, Miller-Dieker syndrome is classified under the International Classification of Diseases, Tenth Revision (ICD-10). The specific ICD-10 code for Miller-Dieker syndrome is Q04.3. This code falls under the category of "Congenital malformations of the nervous system" (Q00-Q07) and specifically refers to "Other congenital malformations of brain." This code is used to identify cases of Miller-Dieker syndrome in medical records and healthcare databases, allowing for accurate statistical tracking and research.

On the other hand, the International Classification of Diseases, Ninth Revision (ICD-9) was the previous coding system used before the implementation of ICD-10. The corresponding ICD-9 code for Miller-Dieker syndrome is 742.2. This code falls under the category of "Other specified anomalies of brain" (742) and specifically refers to "Lissencephaly and pachygyria." Similar to the ICD-10 code, the ICD-9 code is used to identify and classify cases of Miller-Dieker syndrome in medical records and databases.

It is important to note that the transition from ICD-9 to ICD-10 occurred on October 1, 2015, and healthcare providers and institutions are now required to use the ICD-10 coding system for diagnosis coding purposes.
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