Presentation of Slit Ventricle Syndrome in Children

Symptoms

• Elevated ICP symptoms: These are symptoms of shunt malfunction that include headache, lethargy, vomiting, and irritability.  Signs include increasing head circumference and papilledema.
• Low ICP symptoms: Symptoms of low ICP include headache (worse with upright posture), usually progressing as the day goes on and improving with recumbency.

Variable patterns of presentation

Rekate and associates (9, 10, 15) describe five clinical presentations of patients with shunt headache differentiated by history, symptoms, and ICP monitoring results:

• Intermittent shunt malfunction: Episodic high-pressure symptoms are the mainstay, sometimes associated with activity.
• Elevated ICP with functioning shunt: This has been termed cephalocranial disproportion. An example is seen in shunted children with craniofacial syndromes. Headaches, vomiting, and papilledema can lead to visual loss elevated ICP is left untreated.
• Elevated ICP with malfunctioning shunt (normal volume hydrocephalus): This has been termed normal volume hydrocephalus, or shunt pseudotumor. The patient may have morning headaches unrelieved by analgesics or visual changes similar to cephalocranial disproportion symptoms above.
• Low ICP: This has been attributed to shunt overdrainage. Low ICP symptoms progress during the day with the headache improved with recumbency. Rarely, cranial nerve 6 palsies can occur.
• Headache unrelated to shunt function: Not uncommonly there will be a family history of migraines, episodic headache, or headaches relieved by rest.

Time for evolution

• Variable: Onset can be either insidious or rapid, requiring urgent evaluation in the ER. A history of recurrent visits to the ER and multiple surgical interventions is common in this patient population.

Intervention

Stabilization

Few nonsurgical interventions alleviate symptoms.

• Steroids: Steroids can help temporarily prior to surgical intervention. 
• Hydration: IV fluids can reduce symptoms in the child with overdrainage or low ICP.

Preparation for definitive intervention, nonemergent

• Planned evaluation: The problem can be approached systematically.  Generally this will include a course of ICP monitoring, so the patient will need to be prepared for surgery and a monitored bed space arranged.

Preparation for definitive intervention, emergent

• Symptomatic elevated ICP: Emergent surgery may be required for shunt revision or for the addition of a new shunt in the setting of raised ICP.  Preparation would be the same as for any shunt revision (3).

Admission orders

• HOB, positioning, and activity: Admission to the ICU or a monitored unit is often warranted to monitor the child closely. The HOB is elevated to encourage venous return and lower the ICP.
• Vital signs: Close monitoring of vital signs to look for bradycardia, hypertension, or respiratory compromise is essential (Cushing’s triad).