Hydrocephalus is a condition characterized by an abnormal accumulation of cerebrospinal fluid (CSF) within the ventricular system of the brain, leading to increased intracranial pressure. Clinical manifestations vary based on age of onset, chronicity, and underlying etiology.
In infants, the cranial sutures are not yet fused, allowing for compensatory skull expansion in response to increased CSF volume. Key features include:
As skull sutures close, symptoms shift from macrocephaly to signs of raised intracranial pressure:
Adults with hydrocephalus can present with acute or chronic forms.
(“Wet, Wobbly, and Wacky” triad)
NPH is a distinct clinical entity presenting with the classic Hakim’s Triad:
Age Group | Key Features |
---|---|
Infants | Enlarged head, bulging fontanelle, sunsetting sign, irritability, poor feeding |
Children | Headache, nausea, vomiting, ataxia, cognitive decline |
Adults | Acute: Severe headache, Cushing’s triad. Chronic: Gait disturbances, dementia, incontinence |
Elderly (NPH) | “Wet, Wobbly, Wacky” triad: Gait disturbance, cognitive decline, incontinence |
Early recognition and management of hydrocephalus, particularly in pediatric cases, are crucial to prevent long-term neurological impairment.
see Blindness from hydrocephalus.
Hydrocephalus can also cause death.
Infants commonly present with progressive macrocephaly whereas children older than 2 years generally present with signs and symptoms of intracranial hypertension.
One child with headaches, diagnosed with hydrocephalus, who played wind instruments. The patient manifested that their headaches worsened with the efforts made during playing their musical instruments. Martínez-Lage et al. hypothesize that the headaches might be influenced by increases in their intracranial pressure related to Valsalva maneuvers and had serious doubts on if we should advise our young patients about giving up playing their music instruments 1).