Symptoms and Signs of Hydrocephalus
The clinical presentation of hydrocephalus varies dramatically by age, with infants showing progressive head enlargement, bulging fontanelle, and splayed sutures, while older children present with signs of elevated intracranial pressure including headache, vomiting, lethargy, and behavioral changes. 1
Infants (Open Fontanelles)
Primary Clinical Signs
- Rapidly enlarging head circumference (>2 cm in <7 days is particularly concerning) 1
- Progressive splaying of the sagittal suture - the most reliable indication of increased pressure 1
- Bulging, tense anterior fontanelle 1
- Distended scalp veins 2
- "Setting sun" sign (downward deviation of eyes) 3
Associated Symptoms
- Apnea and bradycardia episodes 1
- Lethargy and decreased activity 1
- Feeding intolerance 1
- Vomiting 2
- Hypotonia 1
- Abnormal eye movements 1
Critical Caveat for Infants
Half of infantile hydrocephalus cases with documented raised intraventricular pressure are asymptomatic, making serial head circumference measurements and imaging essential for diagnosis 2. The clinical signs (head circumference, fontanelle fullness, suture splaying) show limited reliability when assessed by multiple practitioners of varied skill, but become reliable when the same experienced practitioner follows the same infant serially 1.
Children (Closed Fontanelles, >2 Years)
Primary Symptoms
- Headache (often worse in morning) 2, 4
- Vomiting (most common symptom) 2
- Behavioral changes 2
- Drowsiness/lethargy 2
- Irritability 4
Clinical Signs
- Papilledema or optic atrophy 3
- Nystagmus 3
- Spastic lower limbs 3
- Cognitive impairment (performance IQ worse than verbal IQ) 3
Important Warning
A quarter of children with cerebrospinal fluid shunts and documented raised intraventricular pressure have no clinical signs, emphasizing that absence of symptoms does not exclude hydrocephalus 2. Head size may remain normal if fontanelles are closed 3.
Unusual Presentations
The presentation can be sufficiently variable or even absent to justify direct intracranial pressure measurement when hydrocephalus is suspected 2. Documented unusual manifestations include:
- Torticollis with screaming episodes (in infant with normal head circumference and development) 5
- Progressive facial vein dilatation 5
- Macular rash and sweating 2
- Hypothalamic dysfunction (short stature, obesity, delayed puberty, diabetes insipidus) 3
There are no fewer than 33 different documented clinical signs of hydrocephalus, highlighting the protean nature of this condition 2.
Presentation Patterns in Neonatal Intraventricular Hemorrhage
For preterm infants developing posthemorrhagic hydrocephalus, three distinct patterns occur 1:
- Catastrophic: Rapid deterioration over minutes to hours (grave prognosis)
- Saltatory: Stuttering course over hours to days with decreased alertness, hypotonia, tight popliteal angle, abnormal eye movements, respiratory difficulties
- Clinically silent: Many cases detected only by surveillance ultrasonography; unexplained hematocrit decline may be the only clue
Diagnostic Approach
Clinical diagnosis must combine physical examination findings with serial imaging, as clinical symptoms and signs alone are unreliable 1. When clinical and radiological findings are doubtful or unusual, direct long-term intracranial pressure monitoring should always be contemplated as it is the only method to reveal with certainty whether disturbed CSF dynamics require intervention 5.
The determination of symptomatic hydrocephalus versus ventricular dilation from hydrocephalus ex vacuo is made based on clinical symptoms and signs in combination with imaging, not imaging alone 1.