Congenital hydrocephalus (Q03)
Congenital hydrocephalus, coded under ICD-10 Q03, is a condition present at birth characterized by abnormal accumulation of cerebrospinal fluid within the brain's ventricles. These codes specifically identify different types and causes of this disorder.
The ICD-10 code Q03.0 covers malformations of the aqueduct of Sylvius, an important passageway for cerebrospinal fluid; this includes synonyms such as aqueduct of Sylvius anomaly and congenital stenosis of the aqueduct of Sylvius. Code Q03.1 is used for atresia of the foramina of Magendie and Luschka, linked with the well-known Dandy-Walker syndrome. These foramina are key exit points for cerebrospinal fluid flow. Q03.8 encompasses other types of congenital hydrocephalus not specified by the prior codes. Lastly, Q03.9 denotes unspecified congenital hydrocephalus and covers a wide range of related synonyms, including hydrocephalus with endocardial fibroelastosis and obstructive hydrocephalus. These codes facilitate precise medical documentation and billing for congenital hydrocephalus, guiding diagnosis and treatment planning.
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.
- hydrocephalus in newborn
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.
- Arnold-Chiari syndrome, type II Q07.0
- acquired hydrocephalus G91
- hydrocephalus due to congenital toxoplasmosis P37.1
- hydrocephalus with spina bifida Q05.0 Q05.4
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 Rhinorrhea
Discharge of cerebrospinal fluid through the nose. Common etiologies include trauma, neoplasms, and prior surgery, although the condition may occur spontaneously. (Otolaryngol Head Neck Surg 1997 Apr;116(4):442-9)
Dandy-Walker Syndrome
A congenital abnormality of the central nervous system marked by failure of the midline structures of the cerebellum to develop, dilation of the fourth ventricle, and upward displacement of the transverse sinuses, tentorium, and torcula. Clinical features include occipital bossing, progressive head enlargement, bulging of anterior fontanelle, papilledema, ataxia, gait disturbances, nystagmus, and intellectual compromise. (From Menkes, Textbook of Child Neurology, 5th ed, pp294-5)
Endocardial Fibroelastosis
A condition characterized by the thickening of ENDOCARDIUM due to proliferation of fibrous and elastic tissue, usually in the left ventricle leading to impaired cardiac function (CARDIOMYOPATHY, RESTRICTIVE). It is most commonly seen in young children and rarely in adults. It is often associated with congenital heart anomalies (HEART DEFECTS CONGENITAL;) INFECTION; or gene mutation. Defects in the tafazzin protein, encoded by TAZ gene, result in a form of autosomal dominant familial endocardial fibroelastosis.
Hydrocephalus
Excessive accumulation of cerebrospinal fluid within the cranium which may be associated with dilation of cerebral ventricles, INTRACRANIAL HYPERTENSION; HEADACHE; lethargy; URINARY INCONTINENCE; and ATAXIA.
Hydrocephalus, Normal Pressure
A form of compensated hydrocephalus characterized clinically by a slowly progressive gait disorder (see GAIT DISORDERS, NEUROLOGIC), progressive intellectual decline, and URINARY INCONTINENCE. Spinal fluid pressure tends to be in the high normal range. This condition may result from processes which interfere with the absorption of CSF including SUBARACHNOID HEMORRHAGE, chronic MENINGITIS, and other conditions. (From Adams et al., Principles of Neurology, 6th ed, pp631-3)
Walker-Warburg Syndrome
Rare autosomal recessive lissencephaly type 2 associated with congenital MUSCULAR DYSTROPHY and eye anomalies (e.g., RETINAL DETACHMENT; CATARACT; MICROPHTHALMOS). It is often associated with additional brain malformations such as HYDROCEPHALY and cerebellar hypoplasia and is the most severe form of the group of related syndromes (alpha-dystroglycanopathies) with common congenital abnormalities in the brain, eye and muscle development.
