Current concepts of hydrocephalus and its management

Hydrocephalus as a clinical entity is usually defined as the presence of an excess amount of water (CSF, cerebrospinal fluid) resulting in increased pressure inside the head with variable clinico- pathological implications. Hydrocephalus is a multifaceted clinical entity with multiple etiological factors resulting in the altered state of CSF dynamics, protean clinical manifestations, varied neuro-radiological presentations and multitudes of available treatment strategies for satisfactory palliation but as yet no one full proof risk free curative treatment available. Basically there is disturbance of one or more of the normal physiological mechanisms involved in the production, circulation and absorption of the CSF. There is a plethora of classifications and terminologies to clinically define it. However, the each designated term defines its one or more aspects well but some other aspects largely remain unaccountable or unexpressed and therefore, such terms are mainly semantic and remain short of their objectives. It is commonest in the early age group with complex issues (3-4 cases per 1000 live births): fetal-neonatal-infantile period associated with intracranial hemorrhages and congenital malformations in significant number of cases. In other pediatric age groups, it is fairly common with and without congenital malformations, obstructive pathologies for the CSF circulation including congenital problems, acquired CNS infections and hemorrhages as well as developing neoplastic conditions. Trauma remains a rare cause of hydrocephalus. The symptoms and signs are combinations of increased volume of the head or the CSF containing intracranial spaces as compared to the normal subjects as well as increased intracranial pressure and their combined deleterious effects on the anatomy (brain parenchyma and intracranial spaces) and physiology of the brain (cerebral irritation phenomena, developmental delays, as well as imminent or progressive raised intracranial pressure effects). In young adults and middle ages, the etiology of the hydrocephalus is usually simple and more straight forward, and in a great majority of cases it is mainly due to obstructive pathologies(tumors, infections, trauma, hemorrhage, etc) affecting the ventricular system or subarachnoid spaces. These patients are commonly present with tetrad of clinical symptomatology: headaches, vomiting, visual obscuration and papilledema. Interestingly in the elderly, there lies a great challenge to prove the entity and then to find its etiology. However in the literature, there is some proof to say and support the concept that intermittently, there is a mild increase in the intracranial pressure with or without ventricular enlargement and associated cerebral atrophy as a main culprit for the triad of its symptoms (ataxia, dementia and incontinence). Following clinical evaluation, irrespective of age, then the initial assessment is performed with variable combinations of ultrasound of the head, the CT scans of the head and spine, and the MRI scans of the cranio-spinal region. The laboratory results are needed where indicated as in cases of meningitis, brain hemorrhage, etc. Patient's clinical status, neuro-imaging findings and parents' perceptions are main determinants of the further management of these cases.Management comprises mainly the clinical observations in the border line cases. However, the medical therapy is advised for the mild degrees of hydrocephalus and the surgical therapy remains gold standard treatment for the patients with moderate to severe grades of proven hydrocephalus where raised ICP is well demonstrated and the patient is likely to be benefitting with the operative interventions mainly for the diversion of the CSF under increased pressure. The surgical procedures are not without risks mainly such as hemorrhage, infection, obstruction, shunt failures, etc. Considerable morbidities and mortality still exist despite remarkable advancement in the neuro-radiological procedures(Modern Ultrasound procedures, CT Head, MRI scans), improvement in surgical procedures with cautions and care, modern anesthetic techniques and sophisticated post operative care and the periodic OPD follow ups. There are many surgical methods but currently ventricular-peritoneal shunt and third ventriculostomy are in vogue being associated with lower rates of morbidities and mortality. Untreated patients have extremely poor prognosis with large head, thin cerebral parenchyma, and moribund clinical state and largely generate great anxiety and frustration in parents on one side and medical faculty on the other.