Encephalocele (Q01)
ICD-10 code Q01 is specifically used to classify different types of encephalocele, a rare congenital condition where brain tissue protrudes through a skull defect. This section covers all forms of encephalocele, helping healthcare professionals record the exact location and type for diagnosis and treatment.
This ICD-10 code range includes detailed subcodes such as Q01.0 for frontal encephalocele and Q01.1, which covers nasofrontal encephalocele; also known as frontoethmoidal, nasopharyngeal, or nasal encephalocele. The code Q01.2 represents occipital encephalocele, a variant linked with conditions like Chiari malformation type III and occipital meningocele. For encephaloceles located in other less common sites such as the orbit or temporal region, Q01.8 applies. If the exact location is not documented, Q01.9 is used for unspecified encephalocele cases. Using these specific codes ensures precise documentation of encephalocele anomalies, which is essential for proper medical care and research.
Instructional Notations
Includes
This note appears immediately under a three character code title to further define, or give examples of, the content of the category.
- Arnold-Chiari syndrome, type III
- encephalocystocele
- encephalomyelocele
- hydroencephalocele
- hydromeningocele, cranial
- meningocele, cerebral
- meningoencephalocele
Type 1 Excludes
A type 1 excludes note is a pure excludes note. It means "NOT CODED HERE!" An Excludes1 note indicates that the code excluded should never be used at the same time as the code above the Excludes1 note. An Excludes1 is used when two conditions cannot occur together, such as a congenital form versus an acquired form of the same condition.
- Meckel-Gruber syndrome Q61.9
Clinical Terms
The following clinical terms provide additional context, helping users better understand the clinical background and common associations for each diagnosis listed in this section. Including related terms alongside ICD-10-CM codes supports coders, billers, and healthcare professionals in improving accuracy, enhancing documentation, and facilitating research or patient education.
Cerebrospinal Fluid Otorrhea
Discharge of cerebrospinal fluid through the external auditory meatus or through the eustachian tube into the nasopharynx. This is usually associated with CRANIOCEREBRAL TRAUMA (e.g., SKULL FRACTURE involving the TEMPORAL BONE;), NEUROSURGICAL PROCEDURES; or other conditions, but may rarely occur spontaneously. (From Am J Otol 1995 Nov;16(6):765-71)
Encephalocele
Brain tissue herniation through a congenital or acquired defect in the skull. The majority of congenital encephaloceles occur in the occipital or frontal regions. Clinical features include a protuberant mass that may be pulsatile. The quantity and location of protruding neural tissue determines the type and degree of neurologic deficit. Visual defects, psychomotor developmental delay, and persistent motor deficits frequently occur.
Sinus Pericranii
Rare vascular anomaly involving a communication between the intracranial and extracranial venous circulation via diploe, the central spongy layer of cranial bone. It is often characterized by dilated venous structures on the scalp due to abnormal drainage from the intracranial venous sinuses. Sinus pericranii can be congenital or traumatic in origin.
