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2025 ICD-10-CM code Q01.1

Nasofrontal encephalocele; a protrusion of brain tissue through a defect in the skull at the fronto-nasal junction.

Codes from this chapter (Q00-Q99) are not for use on maternal records.Always ensure that the appropriate code reflects the specific type and location of the encephalocele.

Surgical repair of a nasofrontal encephalocele is medically necessary to prevent infection (meningitis), neurological damage, and cosmetic deformity.The severity of the condition and the presence of associated anomalies influence the urgency of intervention.

Neurosurgery, neurosurgical subspecialties (e.g., pediatric neurosurgery, craniofacial surgery), plastic surgery, potentially ophthalmology (if nasolacrimal duct involvement), radiology (for imaging interpretation).

IMPORTANT:Q01 includes: Arnold-Chiari syndrome, type III; encephalocystocele; encephalomyelocele; hydroencephalocele; hydromeningocele, cranial; meningocele, cerebral; meningoencephalocele. Excludes1: Meckel-Gruber syndrome (Q61.9)

In simple words: Nasofrontal encephalocele is a birth defect where part of the brain or its covering pushes out through a hole in the skull near the nose.It needs surgery to fix.

Q01.1, in the ICD-10-CM classification, denotes a nasofrontal encephalocele.This is a congenital anomaly characterized by the herniation of intracranial contents, including brain tissue, through a defect in the skull at the junction of the frontal and nasal bones. The hernial sac is positioned superiorly or anteriorly to the nasal bone.This condition results from incomplete closure of the neural tube during fetal development. Associated conditions may include hydrocephalus and other neurological abnormalities.Accurate diagnosis typically involves imaging studies such as ultrasound, CT, and MRI to assess the extent of the herniation and the presence of any associated intracranial anomalies. Treatment usually involves surgical repair to close the defect and address any associated complications.

Example 1: A newborn presents with a visible mass at the bridge of the nose. Imaging confirms a nasofrontal encephalocele containing brain tissue. Surgical repair is planned to prevent infection and neurological damage., A pregnant woman undergoes an ultrasound that reveals a fetal nasofrontal encephalocele.Genetic counseling is offered, and further imaging is performed to assess for associated abnormalities.Postnatal surgical intervention is planned., A child with a history of nasofrontal encephalocele repair presents for follow-up.Neurological examination and imaging are performed to monitor for complications such as hydrocephalus or seizures.

Prenatal ultrasound images, postnatal CT or MRI scans demonstrating the encephalocele and its contents, surgical reports, neurological examination findings, and any documentation of associated congenital anomalies or syndromes.

** The severity of nasofrontal encephaloceles varies widely; some may be small and asymptomatic, while others can be large and associated with significant neurological deficits.Always correlate the code with detailed clinical documentation and imaging findings.

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