Hydrocephalus with interactive quiz session

1.What is the meaning of hydrocephalus?

2.Causes of Hydrocephalus:

3. Pathophysiology of Hydrocephalus:

4.Clinical Features of hydrocephalus:

5.Management of Hydrocephalus:

6.Prognosis of Hydrocephalus:

7.Idiopathic Intracranial Hypertension (IIH): Meaning, Clinical features & management

8.References of Hydrocephalus

9. Interactive Quiz on Hydrocephalus:

1.What is meaning of hydrocephalus?

Hydrocephalus refers to an abnormal accumulation of cerebrospinal fluid (CSF) in the brain, leading to increased pressure and ventricular enlargement. If this occurs before the sutures in the skull fuse, it results in an enlarged head size1,2.

2.Causes of Hydrocephalus:

3. Pathophysiology of Hydrocephalus:

CSF is produced by the choroid plexus in the ventricles at a rate of 20 mL/hr through ultrafiltration and active secretion. It flows through the ventricular system and is absorbed by arachnoid granulations into venous sinuses. Hydrocephalus is categorized as obstructive when there is a blockage in CSF flow within the ventricular system and communicating when there is increased production or impaired absorption without obstruction.1

4.Clinical Features of hydrocephalus:

Newborns with hydrocephalus may have an enlarged head, which can complicate delivery and necessitate a Cesarean section. Signs include a bulging anterior fontanelle, rapid head growth (>1 cm every two weeks), and widened cranial sutures (>0.5 cm beyond the first two weeks). After suture fusion, symptoms of raised intracranial pressure (ICP) manifest. A MacEwen sign (cracked pot sound on percussion) suggests suture separation, while the setting sun sign (upward gaze palsy) indicates pressure on the quadrigeminal plate1,2.

Differential diagnoses include rickets, haemolytic anemia, achondroplasia, familial macrocephaly, megalencephalic, and neurometabolic disorders (e.g., Tay-Sachs, Canavan, Alexander disease). Diagnostic imaging includes cranial ultrasound (for open fontanelles), CT scan, and MRI to assess ventricular enlargement and underlying causes. Mildly enlarged ventricles due to cerebral atrophy should not be misdiagnosed as hydrocephalus2.

5.Management of Hydrocephalus:

Untreated hydrocephalus results in excessive head enlargement, cortical thinning, and eventually fatal ICP elevation. While medical interventions provide temporary relief, CSF diversion through a ventriculoperitoneal (VP) shunt is the standard treatment. The shunt redirects CSF from the ventricles to the peritoneal cavity and often requires multiple revisions as the child grows. Complications include shunt infections (leading to meningitis), shunt displacement or obstruction, and abdominal complications (peritonitis, abscesses). An alternative for obstructive hydrocephalus is endoscopic third ventriculostomy (ETV), which allows CSF drainage into the 

5.Management of Hydrocephalus:

Untreated hydrocephalus results in excessive head enlargement, cortical thinning, and eventually fatal ICP elevation. While medical interventions provide temporary relief, CSF diversion through a ventriculoperitoneal (VP) shunt is the standard treatment. The shunt redirects CSF from the ventricles to the peritoneal cavity and often requires multiple revisions as the child grows. Complications include shunt infections (leading to meningitis), shunt displacement or obstruction, and abdominal complications (peritonitis, abscesses). An alternative for obstructive hydrocephalus is endoscopic third ventriculostomy (ETV), which allows CSF drainage into the basal cisterns1,2.

6.Prognosis of Hydrocephalus:

Despite treatment, nearly two-thirds of affected children experience cognitive and motor impairments2.

7.Idiopathic Intracranial Hypertension (IIH)

IIH, previously termed benign intracranial hypertension or pseudotumor cerebri, is characterized by elevated ICP with normal or reduced ventricular size and normal CSF composition. It primarily affects adolescents and adults, particularly females with obesity. The exact mechanism remains unclear, but proposed factors include increased CSF production, impaired absorption, or elevated cerebral venous pressure, often associated with endocrine disorders, anemia, vitamin A derivatives, tetracyclines, steroids, or hormonal contraceptives 1,2.

Clinical Features

Symptoms include headache, diplopia (double vision), and blurred vision. A false localizing sign, sixth cranial nerve palsy, may also occur. Papilledema and visual disturbances require urgent evaluation2.

Management

Treatment options include:

• Medications: Acetazolamide, glycerol, corticosteroids

• Lumbar punctures: To relieve pressure

• Surgical interventions: Optic nerve sheath decompression or shunt placement 1.2

8.References

1. Ghai OP. Essential Paediatrics. 10th ed. New Delhi: CBS Publishers; 2022.

2. Hockenberry MJ, Wilson D. Wong’s Essentials of Paediatric Nursing. 11th ed. St. Louis: Elsevier; 2021.

3. Aylward SC, Aronowitz C, Roach ES. Pediatric intracranial hypertension. Pediatr Neurol. 2017;66:32-43.

4. Fenichel GM. Management of increased intracranial pressure. In: Clinical Pediatric Neurology, 7th ed. Saunders; 2013. p. 89-112.

5. Kumar R. Approach and management of children with raised intracranial pressure. J Pediatr Crit Care. 2015;2:13.

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9. Interactive Quiz on Hydrocephalus: