Template:ICD-11 Neural anomalies table

ICD-11 LA00-LA0Z Structural developmental anomalies of the nervous system

LA00.0 Anencephaly - a neural tube defect, characterized by the total or partial absence of the cranial vault and the covering skin, the brain being missing or reduced to a small mass. Most cases are stillborn, although some infants have been reported to survive for a few hours. In most cases autopsy findings reveal absence of adrenal glands. Anencephaly is likely to be multifactorial, the result of gene-environment interactions. Familial cases with a seemingly autosomal recessive mode of inheritance have been described but most cases are sporadic. Folic acid and zinc deficiencies, as well as maternal obesity, have been shown to be risk factors. LA00.1 Iniencephaly - a rare form of neural tube defect in which a malformation of the cervico-occipital junction is associated with a malformation of the central nervous system. The cardinal features are occipital bone defect, partial or total absence of cervicothoracic vertebrae, fetal retroflexion of the head and characteristic absence of the neck. It is associated with malformations of the central nervous (spina bifida and/or anencephaly), gastrointestinal (omphalocele) and cardiovascular systems. LA00.2 Acephaly LA00.3 Amyelencephaly - Amyelencephaly is the absence of both the brain and spinal cord. LA01 Cephalocele - failure of the skull to correctly close during the antenatal period. This condition is characterized by herniation of the meninges. This condition may present with herniation of brain, or developmental delay. Confirmation is through observation of herniated meninges by imaging. LA02 Spina bifida - LA02.0 Spina bifida cystica - failure of the neural tube to correctly develop during the antenatal period. This condition is characterized by nerve damage and the presence of meningoceles on the back. This condition may present with physical or mental impairment. LA02.00 Myelomeningocele with hydrocephalus - failure of the neural tube to correctly develop during the antenatal period. This condition is characterized by nerve damage and hydrocephalus. This condition may also present with syringomyelia, hip dislocation, headache, nausea, vomiting, blurry vision, balance problems, bladder control problems, meningitis, or mental impairment. LA02.01 Myelomeningocele without hydrocephalus - failure of the neural tube to close completely during fetal development. This condition is characterized by nerve damage. This condition may also present with syringomyelia, hip dislocation, headache, nausea, vomiting, blurry vision, balance problems, bladder control problems, meningitis, or mental impairment. LA02.02 Myelocystocele - failure of the neural tube to close completely during fetal development. The condition is characterized by skin covered lumbosacral masses, an arachnoid lined meningocele that is directly continuous with the spinal subarachnoid space, and a low lying hydromyelic spinal cord that traverses the meningocele and expands into a large terminal cyst. This condition can present with neural damage and consequent impairment of function below the site of the myelocystocele. LA02.1 Spina bifida aperta - failure of the neural tube to correctly develop during the antenatal period. This condition is characterized by nerve damage originating from a known location in the spine, signified by the presence of a meningocele or myelomeningocele. This condition may present with physical or mental impairment. LA03 Arnold-Chiari malformation type II - failure of the brain and spinal cord to correctly develop during the antenatal period. This condition is characterized by extension of both cerebellar and brain stem tissue into the foramen magnum. This condition may present with partial or complete absence of the cerebellar vermis, myelomeningocele, neck pain, balance problems, muscle weakness, limb numbness, dizziness, vision problems, difficulty swallowing, ringing in the ears, hearing loss, vomiting, insomnia, depression, or impairment of motor skills. LA04 Congenital hydrocephalus - failure of the brain to correctly develop during the antenatal period. This condition is characterized by a rapid increase in head circumference or an unusually large head size due to excessive accumulation of cerebrospinal fluid in the brain. This condition may also present with vomiting, sleepiness, irritability, downward deviation of the eyes, or seizures. Confirmation is through observation of cerebrospinal fluid within cerebral ventricles by imaging. LA05 Cerebral structural developmental anomalies LA05.0 Microcephaly LA05.1 Megalencephaly LA05.2 Holoprosencephaly LA05.3 Corpus callosum agenesis LA05.4 Arhinencephaly LA05.5 Abnormal neuronal migration LA05.6 Encephaloclastic disorders LA05.7 Brain cystic malformations LA06 Cerebellar structural developmental anomalies LA06.0 Dandy-Walker malformation LA06.1 Hypoplasia or agenesis of cerebellar hemispheres LA06.2 Focal cerebellar dysplasia LA07 Structural developmental anomalies of the neurenteric canal, spinal cord or vertebral column