This document discusses several congenital malformations of the nervous system, including anencephaly, acephaly, microcephaly, congenital hydrocephalus, agyria, lissencephaly, and spina bifida. Anencephaly is the absence of a major portion of the brain, skull, and scalp that occurs during embryonic development. Microcephaly is a condition where the brain does not develop properly, resulting in a smaller than normal head. Congenital hydrocephalus is a condition where there is abnormal accumulation of cerebrospinal fluid within the brain, typically causing increased pressure inside the skull. Spina bifida is a birth defect where there is incomplete closing of the backbone and membranes

1. CONGENITAL
MALFORMATIONS OF THE
NERVOUS SYSTEM

2. INTRODUCTION
 Anencephaly is the absence of a major portion of
the brain, skull, and scalp that occurs
during embryonic development. It is a cephalic
disorder that results from a neural tube defect that
occurs when the rostral (head) end of the neural
tube fails to close, usually between the 23rd and 26th
day following conception

3.  Acephaly absence of the head, especially as a
congenital malformation.
 Microcephaly is a medical condition in which
the brain does not develop properly resulting in a
smaller than normal head.

4. CONGENITAL HYDROCEPHALUS
 Hydrocephalus is a condition in which there is
an abnormal accumulation of cerebrospinal
fluid (CSF) within the brain.
 This typically causes increased pressure inside the
skull.
 In babies there may be a rapid increase in head size.
Other symptoms may include vomiting,
sleepiness, seizures, and downward pointing of the
eyes

5.  Agyria
Congenital lack or underdevelopment of the convol
utional pattern of the cerebral cortex.
 Lissencephaly is a set of rare brain disorders where
the whole or parts of the surface of the brain appear
smooth.

6. SPINA BIFIDA
 Spina bifida is a birth defect where there is
incomplete closing of the backbone and membranes
around the spinal cord.
 There are three main types: spina bifida
occulta, meningocele, and myelomeningocele.
 The most common location is the lower back, but in
rare cases it may be the middle back or neck

7.  Use additional code for any associated paraplegia
 congenital absence of the spinal cord is known as
amyelia
 defective development of the spinal cord is known as
atelomyelia

8. ICD-10-CM GUIDELINES
 Anencephaly and similar malformations- Q00
 Encephalocele-Q01
 Microcephaly- Q02
 Congenital hydrocephalus-Q03
 Other congenital malformations of brain- Q04

9.  Spina bifida- Q05
 Other congenital malformations of spinal cord-Q06
 Other congenital malformations of nervous system-
Q07
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