Hydrocephalus

Original Editor - Shaimaa Eldib Top Contributors - Shaimaa Eldib

Introduction

Hydrocephalus is an abnormal build-up of fluid within and around the brain, which can due to excess fluid production, obstruction to its flow, and inadequate absorption[1] If left untreated, the excess fluid can cause increase the pressure put on the skull and brain, which can be damaging[2]. There are two different types of hydrocephalus, which can affect children, these are congenital and acquired.

Congenital Hydrocephalus

Congenital hydrocephalus is present from birth and can be caused when a baby is born prematurely (prior to week 37 of pregnancy). Premature birth can lead to bleeding in the infant’s brain, which could blow the flow of fluid in the brain, leading to enlargement of the brain and skull[3]. It is also known to develop when babies are born with other serious health conditions, such as spina bifida. Furthermore, it can also be caused via the change of a genetic material known as X chromosome, as well as rare genetic disorders (e.g. Danny Walker malformation), and arachnoid cysts, which are fluid- filled sacs found between the spinal cord or the brain, and the arachnoid membrane (one of three membranes surrounding the spinal cord and brain)[4]. However, in many cases the cause of congenital hydrocephalus is unknown[4].

Symptoms

Congenital hydrocephalus often has distinct physical features and symptoms, including[5],[6],[3]:

  • Abnormally large head
  • Tense or bulging fontanelle (the soft spot on top of a baby’s head)
  • Thin and shiny scalp with veins easily noticeable
  • Leg muscles may seem rigid and be prone to uncontrollable muscle contractions (spasms)
  • Eyes appear to be looking down (known as the ‘setting-sun sign’ due to the eyes resembling the sun setting below the horizon)
  • Feeding difficulties (at high risk for malnutrition)
  • Poor general health- high risk for infections
  • Increased irritability
  • Increased drowsiness/ lethargic

Acquired Hydrocephalus

Acquired hydrocephalus develops in adults or children and usually develops following illness or injury. If the condition progresses, the headaches might become continuous and if left untreated, hydrocephalus can be life-threatening. It is possible that acquired hydrocephalus could be caused in several possible ways, such as bleeding inside the brain (subarachnoid haemorrhage)[4], blood clots inside the blood vessels of the brain (venous thrombosis), an infection of the protective membranes surrounding the spinal cord and the brain (meningitis), brain tumours, head injury, or stroke. Furthermore, it is also possible for a baby to be born with narrowed brain passageways, which restrict brain fluid flow, but no symptoms are caused until several years later[3].

Symptoms

Acquired hydrocephalus symptoms include[6],[5]:

  • Headaches
  • Neck pain
  • Feeling and being sick (sometimes worse in the morning)
  • Drowsiness (can progress to a coma)
  • Mental state changes (confusion)
  • Blurred or double vision
  • Walking difficulty
  • Uncontrollable bladder (urinary incontinence)
  • In some cases uncontrollable bowel (bowel incontinence)

Medical Management

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[7].

Shunt Surgery

Shunt surgery consists of inserting a thin tube (a shunt) in the brain, which is used to remove excess fluid from the brain. The fluid passes through the shunt to a different body part, usually the abdomen (the belly) area, and is then absorbed into the blood stream. The shunt has a valve for the control fluid flow and to make sure it drains gradually and not too quickly, the valve can be felt as a lump under the skin of the scalp[7],[8]. Shunt surgery is carried out by a specialist in surgery of the brain and nervous system (neurosurgeon). A general anaesthetic will be given before the operation so that the child will be asleep throughout the procedure, which usually takes between one to two hours. After the operation, a child will need to stay in the hospital for a few days to recover. Once the shunt has been fitted, there is a risk that there could be shunt malfunction, such as blockage or infection of the shunt, leading to the need for essential additional treatment[7],[8]. Some symptoms of shunt malfunction are headaches, sickness, confusion, drowsiness, redness, increased temperature, neck stiffness, abdominal pain, and irritability[7].

Endoscopic third ventriculostomy (ETV)

ETV involves creating a hole in the bottom of the brain, which allows trapped fluid to discharge to the surface of the brain where it can then be absorbed. However, an ETV is not suitable for everyone, but it may be a possible treatment option if the excess fluid in the brain is resulting from a blockage, known as obstructive hydrocephalus. The fluid will then be able to drain out via the hole and avoiding any blockage from occurring. A general anaesthetic is given prior the operation and the neurosurgeon will then create a small hole the skull of the child and use a small camera device (endoscope) to look inside brain chambers. A small hole will then be created inside the brain with the help of the endoscope. This procedure will take approximately one hour[7],[8].

There is reduced risk of infection following ETV surgery compared with the shunt, but there is still a chance that the ETV’s may block months or years after surgery. However, there are some risks associated with ETV, such as hole closure, inability of the brain to absorb excess fluid, or bleeding in the brain (usually minor)[7]

Physiotherapy Management

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[9]. Specific treatment procedures are numerous, functional training for activities of daily living; therapeutic exercise; manual techniques such as mobilization and stretching; and therapeutic modalities[10].

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[11].
  • 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[10].
  • 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[10].

These goals should be achieved by[12]:

  • 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

References

  1. SHEPHERD, R.B., 1995. Physiotherapy in Paediatrics. 3rd ed. Oxford: Butterworth Heinemann.
  2. SOCCI, D.J., BJUGSTAD, K.B., JONES, H.C., PATTISAPU, J.V. and ARENDASH, G.W., 1999. Evidence that oxidative stress is associated with the pathophysiology of inherited hydrocephalus in the h-tx rat model. Experimental Neurology. January, vol. 155, no. 1, pp. 109–117.
  3. 3.0 3.1 3.2 SCHRANDER-STUMPEL, C. and FRYNS, J.P, 1998. Congenital hydrocephalus: Nosology and guidelines for clinical approach and genetic counselling, European Journal of Paediatrics. May, vol. 157, no. 5, pp. 355-362.
  4. 4.0 4.1 4.2 NHS CHOICES., 2014. [online]. [viewed 13 October 2014] Available from: http://www.nhs.uk/Conditions/Hydrocephalus/Pages/Causes.aspx
  5. 5.0 5.1 MAYO CLINIC., 2014. [online]. [viewed 17 October 2014] Available from: http://www.mayoclinic.org/diseases-conditions/hydrocephalus/basics/symptoms/con-20030706
  6. 6.0 6.1 NHS CHOICES., 2014. [online]. [viewed 13 October 2014] Available from: http://www.nhs.uk/Conditions/Hydrocephalus/Pages/Symptoms.aspx
  7. 7.0 7.1 7.2 7.3 7.4 7.5 NHS CHOICES., 2014. [viewed 15 October 2014] Available from: http://www.nhs.uk/Conditions/Hydrocephalus/Pages/Treatment.aspx
  8. 8.0 8.1 8.2 MAYO CLINIC., 2014. [online]. [viewed 17 October 2014] Available from: http://www.mayoclinic.org/diseases-conditions/hydrocephalus/basics/treatment/con-20030706
  9. BONTKE, C.F., ZASLER, N.D. and BOAKE, C. 1996. Rehabilitation of the head-injured patient. New York, NY: McGraw-Hill.
  10. 10.0 10.1 10.2 INSTEP PHYSICAL THERAPY., 2014. [viewed 15 October 2014] Available from: http://www.instepphysio.ca/Neurorehabilitation/Neurorehabilitation/a~3553--c~343920/article.html
  11. KARIMZADEH, P. 2012. Management of hydrocephalus. Hydrocephalus [online]. Available from: http://www.intechopen.com/books/hydrocephalus/management-of-hydrocephalus-
  12. MANCHESTER PHYSIO., 2014 [viewed 15 October 2014] Available from: http://www.manchesterphysio.co.uk/what-we-treat/paediatric-physiotherapy/hydrocephalus.html