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2025 ICD-10-CM code Q01

Encephalocele. Includes: Arnold-Chiari syndrome, type III, encephalocystocele, encephalomyelocele, hydroencephalocele, hydromeningocele, cranial, meningocele, cerebral, meningoencephalocele.

Use additional codes to specify the location of the encephalocele (Q01.0-Q01.9).Code any associated conditions, such as hydrocephalus or Arnold-Chiari malformation, separately.

Medical necessity for encephalocele repair is typically established by the presence of the defect itself, as it poses significant risks to the patient's health and development.Surgical intervention is often necessary to protect the exposed brain tissue, prevent infection, and improve cosmetic outcomes.Additional medical necessity may be established for related conditions such as hydrocephalus or seizures.

Diagnosis and management of encephaloceles typically involves a multidisciplinary team including neurosurgeons, pediatricians, geneticists, and other specialists.Surgical repair is often necessary to place the protruding brain tissue back into the skull and close the defect.Long-term follow-up care may be required to address potential complications such as hydrocephalus, developmental delays, and seizures.

In simple words: An encephalocele is a birth defect where part of the brain pushes out through an opening in the skull, forming a sac-like structure. It is a type of neural tube defect.

Encephalocele is a neural tube defect characterized by a sac-like protrusion of the brain and its surrounding membranes through an opening in the skull.This can occur in various locations on the head, including the frontal, occipital, parietal, nasal, and orbital regions.The sac may contain only meninges (meningocele) or meninges and brain tissue (encephalocele or meningoencephalocele).

Example 1: A newborn infant presents with a sac-like protrusion on the back of their head. Imaging studies confirm the diagnosis of occipital encephalocele containing brain tissue and meninges., A fetus is diagnosed with a frontal encephalocele during a routine prenatal ultrasound.The parents are counseled about the condition and potential treatment options., A child with a repaired nasofrontal encephalocele experiences developmental delays and requires ongoing therapies.

Documentation should include detailed descriptions of the encephalocele's location, size, and contents.Imaging studies (e.g., ultrasound, MRI) are crucial for confirming the diagnosis and characterizing the defect.Operative reports should document the surgical procedure performed, including the extent of the repair and any complications encountered.

** Excludes1: Meckel-Gruber syndrome (Q87.8). Excludes2: inborn errors of metabolism (E70-E90).

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