ICD-11 Codes
for LA01 - Cephalocele

Teodor Jurukovski January 1, 1970

Fact checked by: Teodor Jurukovski

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Understanding Cephalocele (ICD-11 Code LA01)

Cephalocele, identified by ICD-11 code LA01, is a rare neurological birth defect that occurs when the skull fails to close completely during prenatal development. This condition is characterised by the herniation of the meninges, and often brain tissue, through an opening in the skull. It can be associated with developmental delays and is classified under structural developmental anomalies of the nervous system.

What Causes Cephalocele?

The primary cause of cephalocele (ICD-11 code LA01) is the failure of the skull to close properly during fetal development. While the exact mechanisms are complex, research suggests potential disturbances in mesodermal development or neural tube closure. Adequate folic acid intake during early pregnancy is recognised as a factor that can help reduce the risk of neural tube defects, including cephalocele.

Diagnosing Cephalocele

The diagnosis of cephalocele (ICD-11 code LA01) is typically confirmed through medical imaging. Prenatal diagnosis can often be made using ultrasonography. Postnatally, computed tomography (CT) scans and magnetic resonance imaging (MRI) are essential for visualising the herniated meninges and brain tissue, and for assessing the extent of the defect.

Signs and Symptoms of Cephalocele

Cephalocele (ICD-11 code LA01) typically presents as a sac-like protrusion extending from an opening in the skull. The size of these lesions can vary significantly. In infants, other signs may include poor feeding, increased muscle tone, and difficulties with temperature regulation.

Other Names for Cephalocele

Cephalocele (ICD-11 code LA01) is known by several other clinical terms. These include encephalocele, meningocele, cranial meningocele, cranium bifidum, meningoencephalocele, and hydroencephalomeningocele, among others.

Managing Cephalocele

Surgical intervention is the primary treatment for cephalocele (ICD-11 code LA01). Ideally, surgery is performed during infancy to reposition the protruding brain and meninges back into the cranial cavity and to close the skull defect. The surgical goals focus on preserving functional brain tissue and ensuring watertight closure. In cases where hydrocephalus (excess fluid in the brain) is present, a ventriculoperitoneal shunt may be required.

Coding and Documenting Cephalocele

For accurate medical billing and record-keeping, cephalocele is coded using the ICD-11 code LA01. This code falls under structural developmental anomalies of the nervous system. Documentation should reflect the clinical presentation, diagnostic findings from imaging studies, and the management plan, utilising terms such as encephalocele or meningocele as appropriate.