ICD-11 Codes
for LA00.00 - Craniorachischisis

Teodor Jurukovski January 1, 1970

Fact checked by: Teodor Jurukovski

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Understanding Craniorachischisis (ICD-11 Code LA00.00)

Craniorachischisis, identified by the ICD-11 code LA00.00, represents the most severe form of neural tube defect. This congenital malformation occurs when the neural tube fails to close completely during antenatal development, typically around the 27th intrauterine day. It is characterised by the brain and spinal cord remaining open to varying degrees. This condition combines anencephaly, which involves the absence of the cranial vault and brain, with extensive spina bifida extending along the vertebral column. The posterior neuropore's failure to close results in unfused vertebrae and exposed spinal cord, often accompanied by skull and skin defects. Craniorachischisis is a rare and serious condition.

Causes and Risk Factors for Craniorachischisis

The exact cause of Craniorachischisis (ICD-11 code LA00.00) is considered multifactorial, involving a complex interplay of genetic and non-genetic factors. Changes in genetic material (DNA) are implicated. Environmental influences and deficiencies, such as folic acid and zinc deficiencies during pregnancy, are also considered risk factors. Research suggests that interactions between specific genes, like Vangl2 and Scribble (Scrb1), and environmental factors can lead to severe neural tube defects, including craniorachischisis.

Diagnosing Craniorachischisis

The diagnosis of Craniorachischisis (LA00.00) is often made prenatally. Advanced imaging techniques, such as first-trimester ultrasound scans, can detect this condition as early as 11 weeks of gestation. Prenatal diagnosis allows for informed discussions regarding pregnancy management. In some cases, it may be diagnosed alongside other congenital anomalies, such as pentalogy of Cantrell.

Prognosis and Management Considerations

Craniorachischisis (ICD-11 code LA00.00) is invariably a lethal condition. Fetuses diagnosed with this defect have a high likelihood of miscarriage or death shortly after birth. Clinical consequences, if survival occurs, include severe motor and sensory deficits, chronic infections, and disturbances in bladder function. Due to its severity, management discussions often revolve around pregnancy termination or palliative care following diagnosis.

Synonyms and Alternative Terms for Craniorachischisis

Craniorachischisis is also known by several other terms, including:

• Anencephaly with rachischisis
• Cranial Rachischisis
• Rachischisis

These terms are often used interchangeably in clinical literature and documentation when referring to the condition classified under ICD-11 code LA00.00.

Documentation and Coding for Craniorachischisis

For accurate medical record-keeping and billing, the specific ICD-11 code for Craniorachischisis is LA00.00. This code precisely identifies the condition characterised by the failure of the neural tube to close completely, resulting in absence of the skull, extensive vertebral and skin defects, and absence of the brain. Proper documentation should reflect the clinical findings associated with this severe neural tube defect. Using the correct ICD-11 code ensures precise reporting for healthcare services.