Hydrocephalus

Premature infants

Full-term infants

Older children

Apnea

Irritability

Headache

Bradycardia (i.e., low heart rate)

Vomiting

Tense fontanelle

Drowsiness

Lethargy

Distended scalp veins

Macrocephaly

Diplopia

Globoid head shape

Distended scalp veins

Papilledema

Rapid head growth

Frontal bossing

Lateral rectus palsy

Macewen’s sign

Hyperreflexia/clonus

Poor head control

Lateral rectus palsy

“Setting-sun” sign

Full-Term Infants

The common causes of hydrocephalus in full-term infants include aqueductal stenosis, Dandy-Walker syndrome, arachnoid cysts, tumors, and cerebral malformations. Symptoms include irritability, vomiting, and drowsiness. Signs include macrocephaly, a convex and full anterior fontanelle, distended scalp veins, cranial suture splaying, frontal bossing, cracked pot sound on percussing over dilated ventricles (positive Macewen’s sign), poor head control, and the “setting-sun” sign, in which the eyes are inferiorly deviated (Table 6.1).

Older Children

Hydrocephalus after infancy is usually secondary to trauma or tumors. The predominant symptom is usually a dull and steady headache, which typically occurs upon awakening. It may be associated with lethargy, and often improves after vomiting. The headaches slowly increase in frequency and severity over days or weeks. Other common complaints include blurred or double vision, decreased school performance and behavioral disturbances (Table 6.1).

Diagnosis

Hydrocephalus can be diagnosed by cranial ultrasonography in infants with open scalp fontanelles, and by CT and MR imaging, which will demonstrate increased ventricular size, as well as the site of pathological obstruction if present (e.g., tumors that obstruct the ventricles and produce ventriculomegaly).

Treatment

The treatment of hydrocephalus can be divided into non-surgical approaches and surgical approaches, which in turn can be divided into non-shunting or ventricular shunting procedures. The goals of any successful management of hydrocephalus are: (1) optimal neurological outcome and (2) preservation of cosmesis. The radiographic finding of normal-sized ventricles should not be considered the goal of any therapeutic modality.

Non-surgical Options

There is no non-surgical medical treatment that definitively treats hydrocephalus effectively. Historically, acetazolamide and furosemide have been used to treat hydrocephalus. Although both agents can decrease CSF production for a few days, they do not significantly reduce ventriculomegaly, and can lead to potential side effects such as lethargy, poor feeding, tachypnea, diarrhea, and electrolyte imbalances. While acetazolamide has been used historically to treat premature infants with PHH, recent studies have shown it to be ineffective in avoidance of ventricular shunt placement and to be associated with increased neurological morbidity.

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