# Congenital Hydrocephalus

- Category: chronic
- Review status: reviewed (every claim checked against a document named on this page)
- Sources: Hydrocephalus - StatPearls (NCBI Bookshelf NBK560875) - https://www.ncbi.nlm.nih.gov/books/NBK560875/ · ICPC-3 (WONCA International Classification of Primary Care, 3rd edition) class ND55.00 - condition scope only, no dose · No dose - referral pathway, no medicine given in primary care
- Verified date: 2026-08

## Verified against

- No dose - referral pathway, no medicine given in primary care

## Treatment metadata

- No drug therapy in primary care (Referral & Advice)

## Complete treatment card

```text
CONGENITAL HYDROCEPHALUS
Sources: Hydrocephalus - StatPearls (NCBI Bookshelf NBK560875) -
         https://www.ncbi.nlm.nih.gov/books/NBK560875/ · ICPC-3 (WONCA International Classification
         of Primary Care, 3rd edition) class ND55.00 - condition scope only, no dose · No dose -
         referral pathway, no medicine given in primary care
Review status: REVIEWED against the source listed above  (2026-08)

IS IT THIS? - reference only, to read alongside your own examination
  SYMPTOMS - what the patient reports (2)
    - Infants may show delayed growth and sexual maturation, a high-pitched cry, irritability or
      drowsiness, vomiting, and seizures  [drowsiness · excessive crying · irritability · seizures ·
      vomiting]
    - Additional features include stiff limbs, reluctance to move the neck, poor feeding, breathing
      difficulty, and delayed developmental milestones  [breathlessness · poor feeding]
  SIGNS - what you find (5)
    - An abnormally large head is a key sign of the congenital form
    - A tense, bulging fontanelle with separated skull sutures and thin, shiny scalp with visible
      veins may be seen  [bulging fontanelle]
    - A cracked-pot sound heard on skull percussion is called the Macewen sign
    - Downward eye deviation from pressure on the tectal plate produces an impaired upward gaze
    - Acute hydrocephalus causing brain herniation presents with a dilated, non-reactive pupil on
      the affected side, autonomic dysfunction, loss of brainstem reflexes, and coma, and needs
      emergency neurosurgery  [coma]
  TESTS (7)
    - Genetic testing and counseling may be recommended for the congenital forms
    - Cranial ultrasound through the open fontanelle can assess the ventricles and track progression
      in infants
    - An emergency head CT is the first imaging choice in the acute form to assess ventricle size
    - Ballooning of the frontal horns and third ventricle on CT is called the Mickey Mouse sign and
      can indicate obstruction at the aqueduct
    - MRI is the preferred study since it shows the back of the brain better and can distinguish
      tumors and degenerative disease
    - Skull X-ray may show erosion of the sella turcica or a beaten-copper appearance but is rarely
      needed given better imaging options
    - CSF analysis can support the diagnosis and rule out an ongoing infection
  IF NOT THIS - what else fits (3)
    - Idiopathic intracranial hypertension raises pressure without any identifiable structural
      cause, unlike hydrocephalus
    - Secondary brain atrophy from autoimmune disease, HIV, or chemotherapy can mimic hydrocephalus
      on imaging
    - Other structural mimics include brainstem gliomas, frontal lobe epilepsy or syndromes,
      intracranial hemorrhage, migraine, CNS lymphoma, pituitary tumors, and subdural hematoma or
      empyema
  Source  StatPearls "Hydrocephalus" - disease-level clinical article
  Status  traced to the source above

1. NO DRUG THERAPY IN PRIMARY CARE (REFERRAL & ADVICE)    [1st line]
   Adult    Excess cerebrospinal fluid accumulation from birth causing rapid head enlargement;
            definitive treatment is neurosurgical shunting, so the GP's role is early recognition
            through serial head circumference measurement and urgent paediatric neurosurgery
            referral. - Refer, with advice
   Peds     Children follow the same pathway: recognise and refer. No primary-care medicine is
            implied.
   Source   No dose - referral pathway, no medicine given in primary care
   Why      Excess cerebrospinal fluid accumulation from birth causing rapid head enlargement;
            definitive treatment is neurosurgical shunting, so the GP's role is early recognition
            through serial head circumference measurement and urgent paediatric neurosurgery
            referral.
   Caution  No medicine is prescribed for this in primary care - this entry is for recognition and
            referral. Anything given is decided by the service it is referred to.
            RED FLAG - Rapidly increasing head circumference, a bulging or tense fontanelle, or
            sunsetting eyes, irritability or poor feeding suggesting raised intracranial pressure:
            refer urgently.

Prices are indicative (dataset snapshot 2026-06); verify with the pharmacy.
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