2024 ICD-10-CM Diagnosis Code Q03.9

Congenital hydrocephalus, unspecified

ICD-10-CM Code:
ICD-10 Code for:
Congenital hydrocephalus, unspecified
Is Billable?
Yes - Valid for Submission
Chronic Condition Indicator: [1]
Code Navigator:

Code Classification

  • Congenital malformations, deformations and chromosomal abnormalities
    • Congenital malformations of the nervous system
      • Congenital hydrocephalus

Q03.9 is a billable diagnosis code used to specify a medical diagnosis of congenital hydrocephalus, unspecified. The code is valid during the current fiscal year for the submission of HIPAA-covered transactions from October 01, 2023 through September 30, 2024. The code is exempt from present on admission (POA) reporting for inpatient admissions to general acute care hospitals.

Unspecified diagnosis codes like Q03.9 are acceptable when clinical information is unknown or not available about a particular condition. Although a more specific code is preferable, unspecified codes should be used when such codes most accurately reflect what is known about a patient's condition. Specific diagnosis codes should not be used if not supported by the patient's medical record.

Approximate Synonyms

The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:

  • Abnormal blue sclerae
  • Agenesis of cerebellum
  • Aqueduct of Sylvius anomaly
  • Arrested hydrocephalus
  • Beemer Ertbruggen syndrome
  • Central obesity
  • Cerebellum agenesis with hydrocephaly
  • Cerebral degeneration due to congenital hydrocephalus
  • Cerebrospinal fluid rhinorrhea
  • Cerebrospinal fluid rhinorrhea due to hydrocephalus
  • Congenital anomaly of sclera
  • Congenital deformity of lumbosacral region
  • Congenital elevation of scapula
  • Congenital endocardial fibroelastosis
  • Congenital hydrocephalus
  • Congenital hydrocephalus, low insertion of umbilicus syndrome
  • Congenital hypoplasia of cerebrum
  • Congenital kyphosis
  • Congenital kyphosis of thoracic spine
  • Congenital malformation of the meninges
  • Congenital obstructive hydrocephalus
  • Congenital stenosis of aqueduct of Sylvius
  • Cranial cerebrospinal fluid leak
  • Craniosynostosis with Dandy-Walker malformation and hydrocephalus syndrome
  • Dandy-Walker syndrome
  • Endocardial fibroelastosis
  • External hydrocephalus
  • Game Friedman Paradice syndrome
  • Hydrocephalus
  • Hydrocephalus associated with congenital aqueduct stenosis
  • Hydrocephalus with endocardial fibroelastosis and cataract syndrome
  • Hydrocephalus with obesity and hypogonadism syndrome
  • Hydrocephalus, blue sclera, nephropathy syndrome
  • Hydrocephalus, costovertebral dysplasia, Sprengel anomaly syndrome
  • Hydrocephalus, tall stature, joint laxity syndrome
  • Hypoplasia of corpus callosum
  • Internal hydrocephalus
  • Kyphosis of thoracic spine
  • L1 syndrome
  • Leak of cranial cerebrospinal fluid due to hydrocephalus
  • Macroencephaly
  • Mega cisterna magna
  • Megalencephaly, polymicrogyria, postaxial polydactyly, hydrocephalus syndrome
  • Obstructive hydrocephalus
  • Port-wine nevi, mega cisterna magna, hydrocephalus syndrome
  • Port-wine stain of skin
  • Thoracic dysplasia and hydrocephalus syndrome
  • VACTERL syndrome with hydrocephalus
  • X-linked hydrocephalus syndrome

Clinical Classification

Clinical Information

  • 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)
  • 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.
  • 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.
  • 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)
  • Congenital Hydrocephalus

    hydrocephalus that is present at birth.
  • External Hydrocephalus

    abnormal increase of cerebrospinal fluid in the subdural space of the brain.
  • Endocardial Fibroelastosis

    a rare disorder characterized by diffuse thickening of the endocardium. it presents with unexplained heart failure.
  • TAFAZZIN wt Allele|BTHS|Barth Syndrome Gene|CMD3A|Cardiomyopathy, Dilated 3A (X-Linked) Gene|EFE|EFE2|Endocardial Fibroelastosis 2 Gene|G4.5|LVNCX|TAZ|TAZ1|Tafazzin, Phospholipid-Lysophospholipid Transacylase wt Allele|Taz1

    human tafazzin wild-type allele is located in the vicinity of xq28 and is approximately 10 kb in length. this allele, which encodes tafazzin protein, plays a role in phospholipid metabolism, including cardiolipin remodeling. mutations in the gene are associated with barth syndrome, dilated cardiomyopathy (dcm), hypertrophic dcm, endocardial fibroelastosis and left ventricular noncompaction.
  • Congenital Kyphosis

    an abnormally increased curvature of the thoracic portion of the spine that is present at the time of birth.
  • Cerebrospinal Fluid Rhinorrhea

    discharge of cerebrospinal fluid through the nose.

Index to Diseases and Injuries References

The following annotation back-references for this diagnosis code are found in the injuries and diseases index. The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10-CM code(s).

Present on Admission (POA)

Q03.9 is exempt from POA reporting - The Present on Admission (POA) indicator is used for diagnosis codes included in claims involving inpatient admissions to general acute care hospitals. POA indicators must be reported to CMS on each claim to facilitate the grouping of diagnoses codes into the proper Diagnostic Related Groups (DRG). CMS publishes a listing of specific diagnosis codes that are exempt from the POA reporting requirement. Review other POA exempt codes here.

CMS POA Indicator Options and Definitions

POA IndicatorReason for CodeCMS will pay the CC/MCC DRG?
YDiagnosis was present at time of inpatient admission.YES
NDiagnosis was not present at time of inpatient admission.NO
UDocumentation insufficient to determine if the condition was present at the time of inpatient admission.NO
WClinically undetermined - unable to clinically determine whether the condition was present at the time of inpatient admission.YES
1Unreported/Not used - Exempt from POA reporting. NO

Convert Q03.9 to ICD-9-CM

  • ICD-9-CM Code: 742.3 - Congenital hydrocephalus
    Approximate Flag - The approximate mapping means there is not an exact match between the ICD-10 and ICD-9 codes and the mapped code is not a precise representation of the original code.

Code History

  • FY 2024 - No Change, effective from 10/1/2023 through 9/30/2024
  • FY 2023 - No Change, effective from 10/1/2022 through 9/30/2023
  • FY 2022 - No Change, effective from 10/1/2021 through 9/30/2022
  • FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021
  • FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
  • FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
  • FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
  • FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
  • FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016. This was the first year ICD-10-CM was implemented into the HIPAA code set.


[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.