History

Fact Explanation
Large head Production of cerebro-spinal fluid is mainly by the choroid plexus and a small proportion of CSF may be produced by ventricular ependyma and brain parenchyma. Main processes involve in the production of CSF are filtration across the endothelium and active transport of sodium by the choroidal epithelia. CSF is largely formed in the lateral ventricles, and then passes through foramen of Monroe to the 3rd ventricle, then to the 4th ventricle through the aqueduct of Sylvius finally exits through foramen of Magendi and foramen of Luschka. Daily production is around 500 ml/day. [1] Absorption of CSF is mainly via the arachnoidal villi and granulations and rest is from the brain parenchyma, choroid plexus or by the lymphatic channels in the region of the cribriform plate. Hydrocephalus is due to the dilatation of the cerebral ventricular with accumulation of excess cerebrospinal fluid (CSF) within the ventricular system and cisterns of the brain either due to increased production pr decreased absorption of CSF. Aetiology would be vary such as congenital, acquired, infective, and secondary hydrocephalus. [1] Before the cranial sutures are fused this excess accumulation may cause disproportionate head growth. [1,2]
Headache [1] Main two types of hydrocephalus are communicative and obstructive types/ non-communicating hydrocephalus. Impaired absorption in the venous system, causes communicating hydrocephalus. [3] Dandy Walker syndrome is a variety of hydrocephalus in association with agenesis of the cerebellar vermis with cystic dilation of the 4th ventricle. [1] There can be adverse effects due to compression of the brain parenchyma and increased intracranial pressure (ICP) which may cause headache. [1,4]
Vomiting [4] This is more prominent in the morning. [4]
Visual problems Visual problems can occur due to the hydrocephalus. [7] Blurred vision may be due to the papilledema an double vision in sixth nerve palsy.
Poor feeding, irritability and reduced activity [1] These are the non specific symptoms [1] that most infants and young children presenting with.
Drowsiness Significant increase in the intracranial pressure is associated with impaired consciousness. [1]
Problems in walking and abnormal moments Walking problems rae due to the pyramidal tract involvement by the hydrocephalus. Chorea may result due to the pressure on tracts of the nigrostriatal pathway or the cortico‐striato‐pallido‐thalamo‐cortical circuit. [6]
Elderly primiparous mother Congenital hydrocephalus is found in higher incidence in these mothers. [1]
Family history It is seen in males and is due to the X-linked recessive inheritance causing aqueductal stenosis . [1] Affected females are usually carriers.
Urinary incontinence, urgency, frequency Gradual progression of the disease may involve the frontal lobes [5] , causing problems in normal micturition pattern. [8]
History of central nervous system infection Inflammation will be settle with scarring that produce obstruction to CSF absorption and out flow. [1]
References
  1. VENKATARAMANA NK. Hydrocephalus Indian scenario - A review J Pediatr Neurosci [online] 2011 Oct, 6(Suppl1):S11-S22 [viewed 27 August 2014] Available from: doi:10.4103/1817-1745.85704
  2. SHANNON MW, NADLER HL. X-linked hydrocephalus. J Med Genet [online] 1968 Dec, 5(4):326-328 [viewed 27 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1468680
  3. SCARFF JE. Treatment of hydrocephalus: an historical and critical review of methods and results J Neurol Neurosurg Psychiatry [online] 1963 Feb, 26(1):1-26 [viewed 27 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC495530
  4. REKATE HL. A consensus on the classification of hydrocephalus: its utility in the assessment of abnormalities of cerebrospinal fluid dynamics Childs Nerv Syst [online] 2011 Oct, 27(10):1535-1541 [viewed 27 August 2014] Available from: doi:10.1007/s00381-011-1558-y
  5. WOESSNER H, VIBHUTE P, BARRETT K. Acute Loss of Bladder Control in a Stroke of the Frontal Cortex Neurohospitalist [online] 2012 Oct, 2(4):129-131 [viewed 19 September 2014] Available from: doi:10.1177/1941874412450715
  6. VOERMANS NC, SCHUTTE PJ, BLOEM BR. Hydrocephalus induced chorea J Neurol Neurosurg Psychiatry [online] 2007 Nov, 78(11):1284-1285 [viewed 19 September 2014] Available from: doi:10.1136/jnnp.2006.112128
  7. BLOCK J. Visual impairment in "los pressure" hydrocephalus. J Neurol Neurosurg Psychiatry [online] 1971 Feb, 34(1):107 [viewed 19 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC493706
  8. SHPRECHER D, SCHWALB J, KURLAN R. Normal Pressure Hydrocephalus: Diagnosis and Treatment Curr Neurol Neurosci Rep [online] 2008 Sep, 8(5):371-376 [viewed 19 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2674287

Examination

Fact Explanation
Bulging of the fontanellae [1] Due to the increased intracranial pressure. [2,4]
Wide separation of the cranial sutures [1] Due to the increased pressure inside the cranial cavity, specially in infants whose skull is not yet fused. [1,4]
Prominence of scalp veins [1] Prominent in infants. [1]
“setting sun” of the eyes Due to the pressure on the mid-brain tectum by CSF in the suprapineal recess. [1,4]
Papilledema Diagnosis is difficult in infants, and therefore is an unreliable sign. [1] Common in adults. [1]
Cognitive impairment, poor concentration and behavioral changes Due to the insidious development of hydrocephalus. [1,2,4]
Sixth nerve palsy Is an associated visual abnormality to increased intracranial pressure. [1]
Bradycardia, hypertension and irregularities in breathing pattern Due to significant elevation of ICP. [1]
Meningomyelocele and spina bifida Hydrocephalus is associated with spina bifida and meningomyelocele. [3]
References
  1. VENKATARAMANA NK. Hydrocephalus Indian scenario - A review J Pediatr Neurosci [online] 2011 Oct, 6(Suppl1):S11-S22 [viewed 27 August 2014] Available from: doi:10.4103/1817-1745.85704
  2. REKATE HL. A consensus on the classification of hydrocephalus: its utility in the assessment of abnormalities of cerebrospinal fluid dynamics Childs Nerv Syst [online] 2011 Oct, 27(10):1535-1541 [viewed 27 August 2014] Available from: doi:10.1007/s00381-011-1558-y
  3. HARCOURT RB. Ophthalmic complications of meningomyelocele and hydrocephalus in children. Br J Ophthalmol [online] 1968 Sep, 52(9):670-676 [viewed 19 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC506666
  4. SHPRECHER D, SCHWALB J, KURLAN R. Normal Pressure Hydrocephalus: Diagnosis and Treatment Curr Neurol Neurosci Rep [online] 2008 Sep, 8(5):371-376 [viewed 19 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2674287

Differential Diagnoses

Fact Explanation
Brainstem Gliomas [2] Brainstem gliomas [2] are usually occur in the brain between the aqueduct of Sylvius and fourth ventricle. This will usually have the features of space occupying lesion such as headache, vomiting, seizures associated with dysarthria, dysphagia, gait disturbances, drowsiness and behavioural changes.
Frontal Lobe Epilepsy Characteristic feature of frontal lobe involvement is the occurrence of seizures. Other manifestations may be deffer according to the site of involvement of the brain such as involvement of the medial frontal, cingulate gyrus, orbitofrontal, or frontopolar regions causes complex behavioral events, primary motor cortex causes simple partial motor seizures with clonic or myoclonic movements, supplementary motor area causes unilateral or asymmetrical, bilateral tonic posturing etc. Excessive daytime sleepiness is a feature of nocturnal frontal lobe epilepsy. [3]
Encephalitis Encephalitis is an acute, usually diffuse, inflammatory process of the brain. About 90% of cases are associated with herpes simplex virus (HSV-1). They present with the fever, headache, and clouding of consciousness which may be associated with seizures and focal neurology in some patients. Encephalitis associated with meningitis : meningoencephalitis. [1]
References
  1. KENNEDY P. VIRAL ENCEPHALITIS: CAUSES, DIFFERENTIAL DIAGNOSIS, AND MANAGEMENT J Neurol Neurosurg Psychiatry [online] 2004 Mar, 75(Suppl 1):i10-i15 [viewed 11 August 2014] Available from: doi:10.1136/jnnp.2003.034280
  2. VRIES EP, DE VISSER M, TROOST D. Supratentorial meningeal spread from brainstem glioma. J Neurol Neurosurg Psychiatry [online] 1989 Aug, 52(8):1011-1013 [viewed 27 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1031847
  3. CHENG JY, WALLACE DM, LOPEZ MR, CARRAZANA EJ. Nocturnal Frontal Lobe Epilepsy Presenting as Excessive Daytime Sleepiness J Family Med Prim Care [online] 2013, 2(1):101-103 [viewed 19 September 2014] Available from: doi:10.4103/2249-4863.109969

Investigations - for Diagnosis

Fact Explanation
Computer tomography (CT scan) Used to evaluate the dilated ventricular system and localize exact site of blockage of flow to CSF. [1] Findings that favours hydrocephalus are enlargement of the temporal horns of the lateral ventricles, enlargement of the 3rd ventricle, with the enlargement of the rest of the ventricular system. Depend on the site it can be further divided into monoventricular, biventricular, triventricular (3rd and both lateral ventricles),and pan ventricularinvolving all the ventricles.
Magnetic Resonance Imaging (MRI) Can be used to evaluate the Chiari malformation [2] , cerebellar or periaqueductal tumors causing hydrocephalus. It can also be used to differentiate normal pressure hydrocephalus from cerebral atrophy. [3]
Ultrasound scan Investigation and monitoring of the infant with an open fontanella is best done using the ultrasound. It can be used to evaluate the supratentorial ventricular system, hematomas or other ventricular masses causing hydrocephalus. [1]
Measurement of the occipito-frontal circumference Measurement of the occipito-frontal circumference and plotting it on a centile chart is a sensitive test. Sequential measurements are needed to assess the condition. [1]
Plain X-rays of skull Large size skull with different shapes of vault, sutural separation, cranio lacunae, flat anterior cranial fossa may be evident on X-ray. [1]
References
  1. VENKATARAMANA NK. Hydrocephalus Indian scenario - A review J Pediatr Neurosci [online] 2011 Oct, 6(Suppl1):S11-S22 [viewed 27 August 2014] Available from: doi:10.4103/1817-1745.85704
  2. INCECıK F, OZLEM HM, ALTUNBASAK S. Optic pathway glioma, scoliosis, Chiari type 1 malformation, and syringomyelia in a patient with neurofibromatosis type 1 J Neurosci Rural Pract [online] 2013 Aug, 4(Suppl 1):S141-S143 [viewed 19 September 2014] Available from: doi:10.4103/0976-3147.116473
  3. SHPRECHER D, SCHWALB J, KURLAN R. Normal Pressure Hydrocephalus: Diagnosis and Treatment Curr Neurol Neurosci Rep [online] 2008 Sep, 8(5):371-376 [viewed 19 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2674287

Investigations - Fitness for Management

Fact Explanation
Electroencephalogram [1] There is high incidence of seizures in hydrocephalus which can give rise to EEG abnormalities [1] :either focal or diffuse.
Visual evoked potential (VEP) and brainstem auditory evoked response (BAER) increased ICP, decreased cerebral flow, herniation of upper brainstem or congenital anomalies may cause auditory or visual system abnormalities. [1]
References
  1. VENKATARAMANA NK. Hydrocephalus Indian scenario - A review J Pediatr Neurosci [online] 2011 Oct, 6(Suppl1):S11-S22 [viewed 27 August 2014] Available from: doi:10.4103/1817-1745.85704

Investigations - Followup

Fact Explanation
Occipito-frontal circumference Measurement of occipito-frontal circumference and plotting it on a centile chart over the first 2–3 years of life, is helpful in detecting the hydrocephalus at an early stage. [1]
References
  1. VENKATARAMANA NK. Hydrocephalus Indian scenario - A review J Pediatr Neurosci [online] 2011 Oct, 6(Suppl1):S11-S22 [viewed 27 August 2014] Available from: doi:10.4103/1817-1745.85704

Investigations - Screening/Staging

Fact Explanation
Magnetic Resonance Imaging (MRI) Can be used to evaluate the Chiari malformation, cerebellar or periaqueductal tumors causing hydrocephalus. [1]
Cerebral angiography Is used to evaluate Galen malformations, major venous anomalies, hyperplasia, occlusion and elongation of cerebral arteries. [1]
References
  1. VENKATARAMANA NK. Hydrocephalus Indian scenario - A review J Pediatr Neurosci [online] 2011 Oct, 6(Suppl1):S11-S22 [viewed 27 August 2014] Available from: doi:10.4103/1817-1745.85704

Management - General Measures

Fact Explanation
Monitoring the vital signs Heart rate, respiratory rate, blood pressure, level of consciousness and neurological parameters like pupils, motor response need to be monitored. Glasgow Coma Scale (GCS) is of value in checking level of consciousness. [2]
Monitoring the intracranial pressure Not needed in every patient. Important in patients with symptoms and signs of increased intracranial pressure. [3]
Management of seizures Anticonvulsants should be continued and close follow-up should be done.
Follow up As they are vulnerable for complications such as hearing loss, vision problems, growth retardation, learning disability [1] they need to be followed up with hearing, vision and growth assessment.
Educating the parents They should be educated on the condition, complications, treatment options and complications of the shunts. [3] Advice should be given about the symptoms of the shunt infection and to seek immediate medical attention in a case of infection.
References
  1. VENKATARAMANA NK. Hydrocephalus Indian scenario - A review J Pediatr Neurosci [online] 2011 Oct, 6(Suppl1):S11-S22 [viewed 27 August 2014] Available from: doi:10.4103/1817-1745.85704
  2. SHPRECHER D, SCHWALB J, KURLAN R. Normal Pressure Hydrocephalus: Diagnosis and Treatment Curr Neurol Neurosci Rep [online] 2008 Sep, 8(5):371-376 [viewed 19 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2674287
  3. STARK GD, DRUMMOND MB, PONEPRASERT S, ROBARTS FH. Primary ventriculo-peritoneal shunts in treatment of hydrocephalus associated with myelomeningocele Arch Dis Child [online] 1974 Feb, 49(2):112-117 [viewed 19 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1648735

Management - Specific Treatments

Fact Explanation
Osmotic diuretics and acetazolamide (inhibitor of carbonic anhydrase) Carbonic anhydrase is an enzyme necessary for the formation of CSF. However, the effects are not sustained. Used as a temporary measure in post-hemorrhagic hydrocephalus. [1]
Ventriculoperitoneal shunt Ventriculoperitoneal shunt is the gold standard of treatment for hydrocephalus. [1,2] There is a proximal catheter located in the cerebral ventricles and a distal catheter draining into selected site of CSF absorption, that are connected by a reservoir and differential pressure valves such as slit valves, miter valves, ball and spring, and diaphragm valves. Placement of distal catheter is usually in the peritoneal cavity and less commonly in the right atrium, pleural cavity and the gall bladder. [1]
Endoscopic techniques This is an alternative method for ventriculoperitoneal shunt which is currently a popular surgical procedure for hydrocephalus. [1] Endoscopic 3rd ventriculostomy is done for aqueductal stenosis. Other procedures that can be done via endoscope are eptostomy, extirpation of choroid plexus, removal of parasitic cysts, removal of migrated shunts and arachnoid cysts.
Management of complications due to the shunts Infections, blockage, disconnection, migration, relative shortening of length, displacement are the potential mechanical complications of the stents. In addition, over drainage can cause subdural hematoma, subdural collections, low-pressure headaches, secondary craniostenosis, and cranial deformity. Ascites, loculations, hydrocele, perforation of the stomach, large and small bowel, gall bladder and vagina are asssociated extracranial complications. Symptoms and signs such as pyrexia, meningismus, may be associated with shunt infection which is mainly due to coagulase-negative staphylococci, and less commonly due to Staphylococcus epidermidis and Staphylococcus aureus. Examination of CSF needs for the dignosis and antimicrobial therapy is necessary for a good outcome.[1]
References
  1. VENKATARAMANA NK. Hydrocephalus Indian scenario - A review J Pediatr Neurosci [online] 2011 Oct, 6(Suppl1):S11-S22 [viewed 27 August 2014] Available from: doi:10.4103/1817-1745.85704
  2. SHANNON MW, NADLER HL. X-linked hydrocephalus. J Med Genet [online] 1968 Dec, 5(4):326-328 [viewed 27 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1468680