Premature infant male born at 24 weeks and 5 days gestation by imminent SVD. Once brought to the resuscitation room, the infant was pale, limp and had no respiratory effort. Heart rate was initially greater than 60 but then dropped severely. Chest compressions were performed for approximately 1 minute. Intubation was successful on the 4th attempt at 6 minutes of life and heart rate improved. The infant was then transferred to the NICU for further care. Bilateral intraventricular hemorrhages caused eventual posthemorrhagic hydrocephalus in the infant and were treated with a ventriculoperitoneal shunt.

Hydrocephalus occurs when the cerebral ventricular system contains excessive amount of cerebrospinal fluid and is dilated because of the increased pressure. This usually occurs when there is an imbalance between the production and absorption of the CSF. Prevalence of congenital and infantile hydrocephalus is estimated at 0.48-0.81 per 1000 live and stillbirths and may be due to multiple etiologies.

CSF if normally produced primarily by the choroid plexus, circulates through the ventricular system, and then is absorbed into the systemic circulation. The flow of CSF is from the choroid plexus to the lateral ventricles, to the third ventricle, to the fourth ventricle and then finally absorbed via the arachnoid villi into the venous channels of the sagittal sinus and into the systemic circulation. Hydrocephalus most commonly occurs due to a mechanical or function obstruction of this CSF pathway. Other causes are impaired absorption or excess production of CSF, but these mechanisms are much less common. The obstruction within the ventricular system leads to increased pressure and then rapid enlargement of the ventricular system, which leads the signs and symptoms commonly associated with hydrocephalus.

Hydrocephalus and the newborn and infant population is mainly diagnosed through signs, as children are not able to voice symptoms associated with hydrocephalus. Some signs and symptoms seen in this population is excessive head growth on serial measurements of head circumference on growth curves (although significant dilation can occur before head growth becomes abnormal), full or distended anterior fontanel, frontal bossing, and the scalp veins may appear dilated and prominent.

The diagnosis of hydrocephalus is suspected in infants whose head circumference is enlarged at birth or when serial measurements cross percentiles in standard growth curves. It is then confirmed with neuroimaging. Ultrasonography is the preferred technique for initial examination in children due to its portability and avoidance of ionizing rays.

Nonsurgical treatments for hydrocephalus include diuretics, fibrinolysis, and serial lumbar punctures. These treatment methods have significant complications and are less effective than surgical treatments, thus surgical treatments are preferred. Surgical drainage of the CSF via a reservoir or placement of a ventriculoperitoneal shunt (VSP) is often the first form of treatment. However, complications often occur with a VSP, most commonly from infections or mechanical failure. Approximately forty percent of VSPs will fail within the first year of placement. An endoscopic third ventriculostomy (ETV) is another procedure, which connects the third ventricle to the subarachnoid space and is commonly used as an alternative to shunt revision. Success of an ETV depends on the cause of hydrocephalus and the previous complications.

Survival from hydrocephalus without treatment is very poor, but with treatment the survival rate increases to 89-95%. Outcomes depend on etiology, complications and associated abnormalities. Seizures are a common complication of infantile hydrocephalus and usually led to both a poor functional and cognitive outcome. A study in the United Kingdom followed 155 children with shunted hydrocephalus for 10 years or until death showed that 59% of the survivors until school age attended a normal school. However, children with hydrocephalus caused by infection or an intraventricular hemorrhage such as the case described above were more likely to need a special school than those with congenital hydrocephalus.

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