- 1 Introduction
- 2 Causes
- 3 Classification.
- 4 Pathophysiology - Change in CSF dynamics.
- 5 Clinical features
- 6 Management
- 7 Physiotherapy Management
- 8 References
Hydrocephalus is an abnormal build-up of cerebrospinal fluid(CSF) within and around the brain, which can due to excess fluid production, obstruction to its flow, and inadequate absorption If left untreated, the excess fluid can cause increase the pressure put on the skull and brain, which can be damaging.
The etiology of hydrocephalus can be congenital or acquired. Hydrocephalus due to any obvious external cause is termed as congenital hydrocephalus whereas hydrocephalus secondary to any condition such as hemorrhage is known as acquired hydrocephalus. The most common cause of acquired hydrocephalus in infants is hemorrhage due to prematurity. In addition to this, the other causes include neoplasm, an infection most often bacterial meningitis, intrauterine infections with enterovirus, lymphocytic choriomeningitis, toxoplasmosis, and CMV. Infantile hydrocephalus is associated with certain medications taken during pregnancy such as anti-depressants, misoprostol, and metronidazole. hydrocephalus which is not caused by any of the above-mentioned extrinsic causes it is regarded as congenital hydrocephalus with some underlying genetic pathology. X-linked hydrocephalus is associated with aqueductal stenosis, which forms one of the most common hereditary forms of hydrocephalus affecting 10% of males with isolated idiopathic hydrocephalus. In addition to these causes, neural tube defects also cause hydrocephalus.
Classification of hydrocephalus has always been controversial , the first first attempt for the classification of hydrocephalus was made by Walter Dandy in 1913. The current classification with relation to the latest evidences hydrocephalus can be classified as communicating hydrocephalus and non communicating hydrocephalus.
Communicating hydrocephalus is further divided into obstructive and non obstructive types.
Communicating hydrocephalus results when there is impedance of cerebrospinal fluid after it exits the ventricles. Communicating hydrocephalus can be obstructive in nature where there is obstruction at some point between basal cisternae and arachnoid granulations. This obstruction can be due to conditions like subarachnoid hemorrhage , meningitis , congenital or acquired pathologies.
Location of the obstruction and corresponding causes of obstruction
|Lateral, 3rd and
|3rd ventricle at foramen
|Cerebral aqueduct||Aqueduct stenosis
|4th ventricle||Dandy Walker malformation
|Impaired absorption at the
Non obstructive communicating hydrocephalus includes normal pressure hydrocephalus which can be idiopathic or secondary following trauma.
Non communicating hydrocephalus occurs when there is intra ventricular obstruction of CSF flow , therefore non communicating hydrocephalus is always of obstructive type. This obstruction in the CSF flow can be due to conditions such as TORCH INFECTIONS or congenital anomalies, The sites of obstruction includes Foramen of Munro and cerebral aqueduct.
Pathophysiology - Change in CSF dynamics.
With the development of hydrocephalus, there is an alteration in CSF dynamics. The CSF exchange between the lateral ventricles and the third ventricles is compromised. With the development of hydrocephalus the Foramen of Munro undergoes dilation. Both the lateral ventricle and the third ventricle becomes monoventricule , however the cerebral aqueduct retains its function. Also there is exaggerated motion of CSF through cerebral aqueduct. In addition to this CSF pulsatility( i.e with every heart beat the arterial blood volume increases briefly and venous blood and CSF are pushed out of the cranium which leads to the pulsatile flow of CSF)is also disturbed. These changes lead to compromise in the unidirectional flow of CSF from the site of production to the site of absorption.
Neurological consequences, postnatally, include developmental delay, failure to thrive, apnea, bradycardias, Patients with aqueductal stenosis (AS), regardless of type of postnatal treatment or intervention, showed high incidence of epilepsy, developmental delay, cerebral palsy, and attention deficit hyperactivity disorders.
Clinical features of hydrocephalus widely vary according to the age of the patient and the rate of progression.
- Clinical features of hydrocephalus before the closure of the cranial sutures (<2 years of age ) include macrocephaly, increasing head circumference of the head , sunsetting sign, distend scalp veins , developmental delay, poor feeding and regression.
- After the closure of cranial sutures , the acute presentation includes, headache, vomiting, drowsines , papilleodem,a and coma in extreme cases.
- Chronic presentation and clinical features of normal pressure hydrocephalus include incontinence cognitive impairments and gait disturbances
The major neuropsychological deficits include deficits in visuoperceptual and visuomotor functions. This deficit depends on the severity and etiology of hydrocephalus. Corpous callosum abnormalities result from stretching of collosal fibers and other white cortical tracts contribute to these deficits. Children with hydrocephalus presents with a higher verbal IQ( intelligence quotient ) than performance IQ.
Non - Surgical Management.
Non surgical management is a symptomatic treatment for hydrocephalus. Medications such as Acetazolamide or
Isosorbide is used temporarily to reduce the CSF production, also increases CSF absorption by producing hyperosmotic diuresis.
In premature infants hydrocephalus develops due to intraventricular hemorrhage , which causes obstruction in the aarchnoid granulations by the products which are broken down due to hemorrhage. In such cases, lumbar puncture is one form of non-surgical in premature infants with periventricular dilation following periventricular hemorrhage. Removal of CSF with blood and protein approximately 10-15 ml/kg normal resorption of CSF to prevent the development of hydrocephalus after periventricular hemorrhage.
Despite few studies support this method, other studies revealed the repeated lumbar puncture did not reduce the need for shunting of cerebrospinal fluid and also this method did not decrease the likelihood of death or disability in these patients.
Babies born or children who have developed with hydrocephalus usually require swift treatment to reduce the pressure on their brain. If the condition is not treated, the increase in pressure will damage the brain. Both congenital and acquired hydrocephalus can be treated with shunt surgery or neuroendoscopy.
Shunt surgery consists of inserting a thin tube (a shunt) in the brain, which is used to remove excess fluid from the brain. There are two major types of shunt surgeries namely ventriculoatrial shunt , ventriculo peritoneal shunt and lumboperitoneal shunt.
Ventriculo Atrial shunt.
This shunt drains the CSF from the cerebral ventricle to the right atrium of the heart. After placing the ventricular side of the catheter by a neurosurgeon, following the neck is dissected with a parallel longitudinal incision to the sternocleidomastoid muscle and the branch of ithe nternal jugular vein which is compatible twiththe diameter of the shunt is selected and the distal end is placed which drains the fluid into the right atrium.
With the proximal catheter being inserted into the cerebral ventricle , the distal catheter is placed into the peritoneal cavity.
The one end of the catheter is inserted into the lumbar theca either percutaneously or through an open operation and the other end of the catheter is inserted into the peritoneal cavity.
Even though shunting is one of the most common surgical approaches for management , however it presents with the following complications :
- Thoracic occlusion
- Ventricular occlusion
- Shunt sepration
- Kinked Tubing
- CSF fistula
- Migrating catheter , distal as well as ventricular
- Subdural Haematoma
- Ventricular Hemorrage
- Surfacing shunt
- infection such as meningitis , peritonitis extending along the channel
- Following a shunt surgery , some patients may experience headache and vomiting in standing and sitting position this is known as low pressure state which can be gradually resolved by increased fluid in take and gradual mobilisation
Endoscopic third ventriculostomy (ETV)
ETV is a surgical method that is somewhat helpful in avoiding certain shunt-related complications such as shunt obstruction, infection, and over drainage. This is the treatment of choice in obstructive hydrocephalus where a burr hole is created at the base of the third ventricle using a flexible or rigid endoscope for the fluid to drain in the basal cisternae.
Regardless of the different surgical management, children with hydrocephalus still have some disabilities. Therefore, early involvement with physiotherapists via different methods of rehabilitation is essential, whether surgical or non-surgical management is required. Additionally, successful shunting is usually related to more obvious and rapid improvements in rehabilitation efforts. Specific treatment procedures are numerous, functional training for activities of daily living; therapeutic exercise; manual techniques such as mobilization and stretching; and therapeutic modalities.
Physiotherapy goals are aimed at:
- Improving functional skills and reducing secondary impairment, such as obesity, contractures, and fractures, which could delay developmental skills. Furthermore, physiotherapists can work with children in their home and in the hospital or clinic, depending on their medical conditions and age.
- Motor control (co-ordination of muscles and limbs), learning theories, and development are factors that contribute to occurrence of motor behaviour (how the muscles and limbs react to movement, control, development, and learning). These factors include not only the central nervous system (brain and spinal cord) as the driving force, but also biomechanical (human movement principles), psychological, social, and environmental components.
- Teaching and practicing skills under these theories is task-oriented (specific to everyday movements e.g. sitting to standing), which should be intermittent and repetitive. A high level of learning occurs via a child’s problem solving instead of by the therapist's hands-on facilitation. It is also important that emphasis is placed on family centred care and treatment in natural environments.
- The mutual goal is usually to increase functional activity, which in turn, decreases disability.
These goals should be achieved by:
- Promoting physical milestones of achievement such as sitting, crawling, standing
- Optimising mobility independence
- Improving balance and coordination via exercise
- Stretching tight muscles via exercise
- Strengthening weak muscles via exercise
- Increasing quality of life and confidence
- Improving endurance and exercise tolerance
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