Hydrocephalus, defined as the accumulation of cerebrospinal fluid (CSF) in the brain, may occur in the setting of obstructed CSF flow throughout the ventricular system, in which case the term obstructive hydrocephalus is used. Signs and symptoms depend on the age of the patient and the underlying cause. A comprehensive clinical and imaging workup is necessary to make the diagnosis and determine the causes of CSF obstruction.
Presentation
Obstructive hydrocephalus is broadly defined as an abnormal accumulation of CSF in the brain due to obstruction of CSF flow [1] [2] [3]. The obstruction can occur at virtually any point of the CSF pathway, including the ventricular system (the lateral, 3rd and 4th ventricle, as well as the foramen of Monroe and the Sylvian aqueduct) and the subarachnoid space, and numerous etiologies have been established [2]. Intracranial hemorrhage (particularly in neonates and infants), infections (bacterial meningitis, but also intrauterine infections by toxoplasma gondii, cytomegalovirus and enteroviruses), and tumors are some of the more common causes, while progressive enlargement of the 4th ventricle (known as Dandy-Walker malformation), Arnold-Chiari malformation of the cerebellum, and a range of genetic disorders are also described as causative agents of obstructive hydrocephalus [2] [3] [4] [5]. In infants and neonates, a disproportionate head growth, bulging fontanelles, markedly wider separation of the cranial sutures, and paresis of upward gaze (the "setting sun" of the eyes) are typical signs of hydrocephalus that may be accompanied by poor feeding, irritability, and a delayed maturation [2] [3] [5]. On the other hand, headaches, vomiting, visual deficits, and drowsiness are encountered in adults and older children in the setting of hydrocephalus, whereas hypertension, bradycardia and respiratory difficulties signify severe obstruction of CSF flow [2] [4]. In addition to the acute symptomatology, hydrocephalus might also cause chronic symptoms, such as behavioral and cognitive changes, delayed puberty, and defects of higher brain functions [3] [4]. In either case, a prompt diagnosis could significantly reduce the risk for the patient, and might even be life-saving.
Entire Body System
- Disability
Sequelae are disabling for patients and lead to a greater demand for healthcare infrastructure and search for treatment options. [ncbi.nlm.nih.gov]
Because hydrocephalus may lead to both short-term and long-term disability, early recognition is vital. [symptoma.com]
The condition is characterized by aqueductal stenosis, severe intellectual disabilities and in half of the affected children, an adduction thumb deformity. [chop.edu]
Many individuals and their families are satisfied when shunt surgery results in reduced disability or dependence than he or she had before surgery, or prevention of further neurological deterioration. [hopkinsmedicine.org]
- Poor Feeding
We report the case of a 2-month-old child who presented with poor feeding and lethargy and was noted to have a fixed downward gaze. Head computed tomography revealed a posterior fossa mass that was pathologically consistent with a medulloblastoma. [journals.lww.com]
feeding, irritability, and a delayed maturation. [symptoma.com]
feeding, irritability and drowsiness. [headwayabiaustralia.org]
feeding Seizures Eyes fixed downward (sunsetting of the eyes) Deficits in muscle tone and strength Poor responsiveness to touch Poor growth Toddlers and older children Among toddlers and older children, signs and symptoms may include: Physical signs [mayoclinic.org]
Symptoms in infants include poor feeding, irritability, reduced activity, and vomiting. [emedicine.medscape.com]
Respiratoric
- Respiratory Distress
Beyond this point, there continues to be a small risk of complications from early delivery including cerebral palsy, intraventricular hemorrhage, necrotizing enterocolitis and respiratory distress syndrome. [chop.edu]
Cardiovascular
- Hypertension
Two patients presented with profound decreases in neurological status subsequent to development of malignant hypertension. [ncbi.nlm.nih.gov]
Eyes
- Diplopia
A 46-year-old man developed intermittent headache, diplopia, and visual obscuration for two months. Funduscopic examination showed optic disk swelling in both eyes. [ncbi.nlm.nih.gov]
On physical exam, the patient had normal mental status but had difficulty with tandem gait, truncal ataxia, and mild left sixth nerve palsy with diplopia. [cureus.com]
Musculoskeletal
- Macrocephaly
Progressive macrocephaly prompted an MRI confirming triventricular hydrocephalus secondary to aqueductal stenosis. [ncbi.nlm.nih.gov]
Before the bony sutures of the skull have fused in a child, hydrocephalus may present as progressive and abnormal enlargement of the head (macrocephaly). [casemed.case.edu]
Macrocephaly may result from an underlying brain problem (eg, Alexander disease or Canavan disease), or it may be a benign, sometimes inherited, feature characterized by an increased amount of CSF surrounding a normal brain. [msdmanuals.com]
Neurologic
- Headache
Cyst removal successfully treated the patient's headaches without necessitating permanent cerebrospinal fluid diversion. [ncbi.nlm.nih.gov]
- Meningism
Neurosarcoid psychiatric symptoms are usually associated with diffuse meningeal enhancement from meningitis. [ncbi.nlm.nih.gov]
One of the potential complications of meningitis is hydrocephalus as in this example; this can occur following viral, bacterial, mycobacterial or even sarcoid meningitis. Treatment is generally with a VP shunt. [svuhradiology.ie]
- Irritability
Children present with headache, vomiting, lethargy, irritability, esotropia, or paralysis of upward gaze. Seizures may occur. [msdmanuals.com]
[…] and neonates, a disproportionate head growth, bulging fontanelles, markedly wider separation of the cranial sutures, and paresis of upward gaze (the "setting sun" of the eyes) are typical signs of hydrocephalus that may be accompanied by poor feeding, irritability [symptoma.com]
Other symptoms may include vomiting, sleepiness, irritability, downward deviation of the eyes (also called "sunsetting"), and seizures. [medicinenet.com]
Signs of a shunt malfunction include headaches, vision problems, irritability, fatigue, personality change, loss of coordination, difficulty in waking up or staying awake, a return of walking difficulties, mild dementia or incontinence. [aans.org]
Symptoms common in most patients (regardless of cause or age) may include: Irritability Nausea and/or vomiting Headache Seizures Personality changes Cognitive changes (e.g., problems with memory, the ability to think/reason, or difficulty paying attention [neurosurgicalassociatespc.com]
- Papilledema
A 40-year-old African American man presented with papilledema, diplopia, and headache. Magnetic resonance imaging revealed "aqueductal stenosis" without abnormal enhancement or obstructive lesion. [ncbi.nlm.nih.gov]
[…] children and adults include the following: Slowing of mental capacity, cognitive deterioration Headaches (initially in the morning) Neck pain, suggesting tonsillar herniation Vomiting, more significant in the morning Blurred vision: A consequence of papilledema [emedicine.medscape.com]
Papilledema may be evident 2. In children whose cranial sutures have yet to fuse, there is a rapid enlargement of the head circumference 2. [radiopaedia.org]
Signs and symptoms of hydrocephalus include emesis (especially in the morning), decline in cognitive ability, headaches, papilledema, ataxia and defect in upward gaze. Infant with hydrocephalus Case 3: Imaging findings: Figure 3. [stritch.luc.edu]
This finding is called papilledema and can be found on a careful eye examination. If obstructive hydrocephalus is caused by a tumor or other mass, the patient may present with other neurological symptoms which are associated with the tumor itself. [nervous-system-diseases.com]
- Intracranial Hemorrhage
Nevertheless, it is seldom considered in intracranial hemorrhage even in cases involving obstruction of the CSF circulation. [ncbi.nlm.nih.gov]
Intracranial hemorrhage (particularly in neonates and infants), infections (bacterial meningitis, but also intrauterine infections by toxoplasma gondii, cytomegalovirus and enteroviruses), and tumors are some of the more common causes, while progressive [symptoma.com]
Intracranial hemorrhage after spine surgery. J Neurosurg Spine 2013;19:370-80. 16. Khalatbari MR, Khalatbari I, Moharamzad Y. Intracranial hemorrhage following lumbar spine surgery. Eur Spine J 2012;21:2091-6. 17. Brockmann MA, Groden C. [asianjns.org]
Hydrocephalus that results from head trauma, brain tumors, intracranial hemorrhage, or meningitis. The abnormal buildup of cerebrospinal fluid in the ventricles of the brain. [icd10data.com]
Workup
Because hydrocephalus may lead to both short-term and long-term disability, early recognition is vital. Physicians should obtain a detailed patient history that will reveal when did the symptoms start and how did they progress, which is particularly important in neonates and younger children. If the physical examination reveals prominent cranial changes and detect respiratory, but also cardiac abnormalities, a valid clinical suspicion toward hydrocephalus as the underlying cause of symptoms can be made. Imaging studies, however, are the cornerstone in confirming hydrocephalus and the exact type, but also in identifying the condition that induced it. Because of its accessibility, non-invasiveness, and reliability in the youngest population with an open fontanelle, ultrasonography should be readily used in neonates, as it provides a direct view of the ventricular system and reveals the presence of a mass (eg. hematoma) that obstructs normal CSF flow [2] [4]. Otherwise, computed tomography (CT) and magnetic resonance imaging (MRI) are recommended during the diagnostic workup [1] [3] [4] [6]. Some of the most common features encountered in these studies are ventricular dilation and edema of the periventricular white matter (as a result of spinal fluid resorption), but their ability to confirm the exact location of the obstructive lesion is vital for both diagnostic and therapeutic purposes [2] [3] [6]. Additionally, diffuse-weighted (DW) MRI imaging has proven to be of even greater benefit when patients are suspected to suffer from hydrocephalus, and this technique should be employed whenever possible [6].
Microbiology
- Toxoplasma Gondii
Intracranial hemorrhage (particularly in neonates and infants), infections (bacterial meningitis, but also intrauterine infections by toxoplasma gondii, cytomegalovirus and enteroviruses), and tumors are some of the more common causes, while progressive [symptoma.com]
Treatment
OBSTRUCTIVE: hydrocephalus due to giant basilar artery (BA) aneurysm is a rare finding, and endoscopic treatment has not been reported. [ncbi.nlm.nih.gov]
Prognosis
We assessed epidemiology, clinical and imaging findings, neurosurgical management, and prognosis of adult patients with posterior fossa metastasis-associated obstructive hydrocephalus. [ncbi.nlm.nih.gov]
Etiology
Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology. [icd10coded.com]
The diagnosis of hydrocephalus is not a challenge presently, but the exact definition of this entity and its etiology is often difficult. Early detection of the etiology o f hydrocephalus is vital for accurate and early treatment. [content.iospress.com]
At admission she presented with a subcutaneous infection of the shunt catheter and an abdominal mass of unknown etiology. [ncbi.nlm.nih.gov]
Epidemiology
We assessed epidemiology, clinical and imaging findings, neurosurgical management, and prognosis of adult patients with posterior fossa metastasis-associated obstructive hydrocephalus. [ncbi.nlm.nih.gov]
Neurology and Neurosurgery Czech and Slovak Ophthalmology Czech and Slovak Psychiatry Czech Geriatric Review Czech Gynaecology Czech Rheumatology Czech Dental Journal Czech Urology Czech-Slovak Dermatology Czecho-Slovak Pathology Czech-Slovak Pediatrics Epidemiology [prolekare.cz]
Epidemiology There is some evidence that the incidence of paediatric hydrocephalus has declined in many developed countries. [patient.info]
Estimating mortality from tuberculous meningitis in a community: Use of available epidemiological parameters in the Indian context. Indian J Tub 2000;47:9-12. 2. Tandon PN. Tuberculous meningitis (cranial and spinal). [neurologyindia.com]
Pathophysiology
We discuss the pathophysiological mechanism of this complication. [ncbi.nlm.nih.gov]
Although hydrocephalus is typically referred to as either being "obstructive" or "communicating", this can lead to confusion as to the underlying cause of ventriculomegaly as the terms are referring to different aspects of the underlying pathophysiology [radiopaedia.org]
Pharmacotherapy: A Pathophysiologic Approach. 7th ed. New York, NY: McGraw-Hill Medical; 2008:1744. 3. Kandasamy J, Jenkinson MD, Mallucci CL. Contemporary management and recent advances in paediatric hydrocephalus. BMJ. 2011;343:146-151. 4. [uspharmacist.com]
We comment on the possible pathophysiological mechanisms that may result in this dissociation in ventricular and lumbar CSF composition. Conclusions. [scielo.isciii.es]
ICH, a rare but potentially fatal complication after spinal surgery, is known to be associated with excessive CSF loss. [14], [15], [16] The postulated pathophysiology of ICH is that an increase in the transluminal venous pressure or downward cerebellar [asianjns.org]
Prevention
Bilateral neurological symptoms suggest diffuse axonal damage and normalization of the intracranial pressure should be performed on the early onset of clinical detorioration in order to prevent axonal injury. [ncbi.nlm.nih.gov]
References
- Kartal MG, Algin O. Evaluation of hydrocephalus and other cerebrospinal fluid disorders with MRI: An update. Insights Imaging. 2014;5(4):531-541.
- Venkataramana NK. Hydrocephalus Indian scenario – A review. Journal of Pediatric Neurosciences. 2011;6(Suppl1):S11-S22.
- Tully HM, Dobyns WB. Infantile hydrocephalus: a review of epidemiology, classification and causes. Eur J Med Genet. 2014;57(8):359-368.
- Porter RS, Kaplan JL. Merck Manual of Diagnosis and Therapy. 19th Edition. Merck Sharp & Dohme Corp. Whitehouse Station, N.J; 2011.
- Aster, JC, Abbas, AK, Robbins, SL, Kumar, V. Robbins basic pathology. Ninth edition. Philadelphia, PA: Elsevier Saunders; 2013
- Uluğ AM, Truong TN, Filippi CG, et al. Diffusion imaging in obstructive hydrocephalus. AJNR Am J Neuroradiol. 2003;24(6):1171-1176.