Hydrocephalus denotes enlargement of the ventricular system due to excessive cerebrospinal fluid (CSF) accumulation. In the setting of communicating hydrocephalus, the resorption of CSF is impaired, causing various neurologic symptoms, macrocephaly, and dementia, depending on the age of onset and severity. Ultrasonography, computed tomography or magnetic resonance imaging are used in different patient populations. Lumbar drainage or insertion of shunts are the main forms of treatment.
Presentation
Lethargy, vomiting, irritability and a prominent, bulging fontanelle are main signs of hydrocephalus in infants, whereas headaches and reduced vision may be reported at a later age [2] [7]. Papilledema, both learning and cognitive disorders, as well as the appearance of precocious puberty, may be seen [2] [7], together with gait disturbance, urinary incontinence, dementia and a range of other neurological deficits [1].
Entire Body System
- Falling
I had a fall in 2014. I was down at our waterfront,… Submit an appointment request on our patient portal or contact our New Jersey and Pennsylvania campuses to speak with a patient advocate. [princetonbrainandspine.com]
An obstructive component in larger masses is often also present) additionally, other conditions with large ventricles fall into this group although they are often not thought of as hydrocephalus. [radiopaedia.org]
[…] referred to only as 'hydrocephalus') can be categorised in three different ways: 1.Congenital or Acquired 2.Non-communicating (Obstructive) or Communicating 3.Internal or External Since these describe different aspects of the condition, each case will fall [hydrocephalus.allanach.dk]
They may have difficulty going up and down stairs and curbs, and they fall frequently as a result. Gait disturbance often is the most obvious first symptom. [aans.org]
[…] incontinence worsens due to indifference of the patient to their recurrent urinary symptoms Additionally, the gait disturbances can make the way to the toilet more difficult, leading to further incontinence related issues Wacky : dementia Wobbly : frequent falls [amboss.com]
- Disability
Please enable JS and disable any ad blocker [cairn.info]
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]
They used the Vellore grading system proposed by us [Table 2] and found that all patients in grade IV were left with severe disability or died. [neurologyindia.com]
Children with hydrocephalus are more likely to have various developmental disabilities. [drugs.com]
- Surgical Procedure
CT or MRI (and ultrasound for infants ) are important diagnostic procedures. Treatment involves surgical insertion of a shunt, which drains excess CSF into another area of the body – usually the peritoneum. [amboss.com]
Other surgical procedures - choroid plexectomy, choroid plexus coagulation and endoscopic cerebral aqueductoplasty have all been helpful in some cases. [patient.info]
Other surgical procedures include, in some cases, choroid plexectomy, third ventriculostomy, and ventricular reservoir. Ventricular reservoir is basically a catheter inserted into a ventricle of the brain to draw CSF. [encyclopedia.com]
Gastrointestinal
- Fecal Incontinence
A sixty-six-year-old man presented with headache, left hemiparesis, right crural monoparesis, and urinary and fecal incontinence. A picture consistent with cerebral angiitis was seen angiographically. [ncbi.nlm.nih.gov]
Musculoskeletal
- Macrocephaly
Physical examination can reveal an enlarged head (macrocephaly) and a bulging, prominent fontanelle. [symptoma.com]
A 39-month-old male had macrocephaly and communicating hydrocephalus secondary to superior vena cava (SVC) obstruction which was a postoperative complication of an intraatrial baffle operation (Mustard procedure) performed at age 10 months. [ncbi.nlm.nih.gov]
These symptoms are often accompanied by specific clinical manifestations, such as macrocephaly in congenital hydrocephalus, or changes in vital signs resulting from brainstem compression. [amboss.com]
Urogenital
- Incontinence
In the first case, a 67-year-old woman presented with progressive gait disturbance, incontinence, and memory problems. [ncbi.nlm.nih.gov]
Initially, only ↑ urgency and frequency of micturition → urge incontinence → later in the disease, incontinence worsens due to indifference of the patient to their recurrent urinary symptoms Additionally, the gait disturbances can make the way to the [amboss.com]
- Urinary Incontinence
We experienced a case of glioblastoma multiforme which exhibited dementia, gait disturbance, headache, and urinary incontinence six months after subtotal removal of the tumor. [ncbi.nlm.nih.gov]
This ventricular distension leads to the classic presentation of urinary incontinence, dementia, and abnormal gait. [amboss.com]
Papilledema, both learning and cognitive disorders, as well as the appearance of precocious puberty, may be seen, together with gait disturbance, urinary incontinence, dementia and a range of other neurological deficits. [symptoma.com]
Neurologic
- Communicating Hydrocephalus
The traditional theory of communicating hydrocephalus has implicated the bulk flow component of CSF motion; that is, hydrocephalus is generally understood as an imbalance between CSF formation and absorption. [ncbi.nlm.nih.gov]
- Stroke
Partial correlation was used to determine which of these parameters were most significantly associated with aqueductal stroke volume (ASV). [ncbi.nlm.nih.gov]
Causes can include head injuries, strokes, infections, tumors, and bleeding in the brain. [icdlist.com]
Página 311 - The National Institute of Neurological Disorders and Stroke rt-PA Stroke Study Group. Tissue plasminogen activator for acute ischemic stroke. [books.google.es]
Powered Surgical Instruments Revascularization Devices for Acute Ischemic Stroke Shunts Spinal Cord Neurostimulation Systems Spine Robotics Surgical Imaging Systems Surgical Navigation Systems [medtronic.com]
- Confusion
Although conventionally classified as either "obstructive and nonobstructive" or "communicating and noncommunicating," none of the classification schemes were without confusion and errors. [ncbi.nlm.nih.gov]
Communicating hydrocephalus is commonly used as the opposite of obstructive hydrocephalus which leads to much unnecessary confusion, as most causes of communicating hydrocephalus do have an element of obstruction to normal CSF flow / absorption. [radiopaedia.org]
- Intracranial Hemorrhage
Abstract Communicating hydrocephalus may occur spontaneously in elderly patients or occur as a complication of meningitis or intracranial hemorrhage, typically as a result of fibrosis along the route of cerebrospinal fluid (CSF) flow. [ncbi.nlm.nih.gov]
Causes of this include: Meningitis Intracranial hemorrhage (brain bleed) Traumatic brain injury Cancer Patients typically complain of headaches, vision impairment, double-vision, altered mental status and seizures. [pacificneuroscienceinstitute.org]
Neither infant had an intracranial hemorrhage on post-ECMO CT scan. During their follow-up, both infants were noted to have an abnormal increase in head circumference after 6 months of age with a diagnosis of CH on cranial CT scan. [nature.com]
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]
- Bulging Fontanelle
Physical examination can reveal an enlarged head (macrocephaly) and a bulging, prominent fontanelle. [symptoma.com]
Communicating Hydrocephalus Picture – Showing Bulging fontanel, Lateral ventricle, Third ventricle, Aqueduct of Sylvius and the Fourth ventricle. [heydoctor.org]
Diagnosis Examination in infants may reveal the following findings: Head enlargement (head circumference ≥98th percentile for age), especially crossing percentiles on the growth chart Dysjunction/splaying of sutures Dilated scalp veins Tense/bulging fontanelle [emedicine.medscape.com]
Signs that are indicative of bleeding include: stupor, respiratory difficulty, seizures, unstable vital signs, and a bulging fontanelle. Several pharmacological agents can be used to prevent intraventricular haemorrhage. [hydrocephalus.allanach.dk]
Workup
The diagnosis of hydrocephalus can be made during regular prenatal assessment with fetal ultrasonography [8], while CT or MRI can confirm ventricular enlargement in infants and older age groups [2]. An increased head circumference or a bulging fontanelle are definite signs of increased intracranial pressure, which signifies the need for a detailed physical examination at birth and during subsequent visits, especially if infants develop symptoms suggestive of hydrocephalus [2].
Treatment
The goal of treatment is to stabilize the levels of CSF in the ventricular system and relieve the pressure to the brain parenchyma, which may be performed by lumbar drainage and placement of ventricular shunts in severe cases [2] [3]. Endoscopic third ventriculostomy (ETV) is considered as an effective therapeutic procedure [10], but complications such as infection and malfunction may be life-threatening, further strengthening the role of an early diagnosis [2].
Prognosis
The importance of an early diagnosis is emphasized across numerous reports [1] [2] [8], as the condition may cause long-term consequences that can severely impair the quality of life.
Etiology
Intraventricular hemorrhage (IVH) as a result of prematurity is the most notable cause of communicating hydrocephalus [3] [4], but numerous disorders can induce abnormal accumulation of CSF [4] [5] [6]:
- Infections - Enterovirus acquired in utero, cytomegalovirus, and lymphocytic choriomeningitis virus.
- Drugs - Metronidazole, misoprostol, and several antidepressants.
- Tumors - Vestibular schwannomas and glioblastoma multiforme are documented as causes of communicating hydrocephalus [6] [7].
- Brain trauma and neurosurgery.
Epidemiology
The prevalence rates of hydrocephalus as a congenital malformation is estimated to occur in about 4.65 per 10,000 births [8], whereas rates among infants rise to 32 per 10,000 births [4]. Use of antidepressants during the first trimester, multiple gestations, and maternal diabetes were established as risk factors for congenital hydrocephalus [1] [9].
Pathophysiology
Cerebrospinal fluid (CSF) is produced at several sites along the central nervous system - the choroid plexus, brain parenchyma, spinal cord and ependymal lining of the ventricles [1]. The CSF has a unidirectional flow from lateral ventricles into the third and fourth ventricles through the foramen of Monro and the Sylvian aqueduct, respectively, followed by entrance into the subarachnoid space, where it is absorbed into the jugular venous system [1]. In the communicating hydrocephalus, there is a lack of reabsorption of CSF. In non-communicating hydrocephalus open link, there is an obstruction to the CSF-flow, that is not observed with this type. The end-result is the accumulation of CSF within the ventricular system and subsequent dilation of the ventricles, leading to increased pressure in the brain parenchyma, loss of brain cells and the appearance of numerous neurological deficits that can cause the stroke or even death [1].
Prevention
Avoidance of drugs that were proposed to increase the risk (metronidazole, misoprostol, antidepressants) and a thorough prenatal assessment in order to identify hydrocephalus early on may be effective preventive strategies.
Summary
Hydrocephalus is a condition characterized by abnormal concentrations of cerebrospinal fluid due to numerous congenital, neoplastic, or infectious diseases. It causes enlargement of the ventricular system and two main types exist - non-communicating (also known as obstructive), in which occlusion causes increased pressure; and communicating, characterized by impaired resorption or flow of CSF [1]. Recent studies have proposed a different classification, however, dividing hydrocephalus into acute (or obstructive) and chronic that is further divided into communicating and chronic obstructive [1]. Meningeal infection is considered to be the most important event in the pathogenesis of communicating hydrocephalus [2]. The clinical presentation varies across different age groups, ranging from macrocephaly and a bulging fontanelle in infants, induction of precocious puberty in girls, to numerous neurologic disorders in children and adults, such as gait disturbances, learning and cognitive deficits, but also dementia [1] [2]. The diagnosis can be made by ultrasonography in neonates and infants, whereas computed tomography (CT) or magnetic resonance imaging (MRI) are recommended for older age groups [2]. Lumbar drainage and ventricular shunting are currently the mainstays of therapy [2] [3].
Patient Information
Hydrocephalus is a clinical term that describes an increased amount of cerebrospinal fluid in the brain. Normally, the cerebrospinal fluid serves as a "cushion" for the brain and as a transporter of various nutrients across the central nervous system and hydrocephalus occurs either when its flow is obstructed or when its absorption is impaired, which is the hallmark of communicating hydrocephalus. The most common cause of communicating hydrocephalus is inflammation of the meninges (for ex. in meningitis or hemorrhage into the ventricular system). The condition is primarily encountered in infants and children and headaches, irritability, lethargy and vomiting are principal symptoms. Physical examination can reveal an enlarged head (macrocephaly) and a bulging, prominent fontanelle. If the diagnosis is not made early on, the increased pressure in the brain can lead to brain cell death and consequences such as learning disorders and various neurological deficits. For this reason, it is imperative to conduct thorough check-ups during prenatal visits, as physicians can detect hydrocephalus with ultrasonography before birth. In older children, computed tomography or magnetic resonance imaging are used for confirmation. In order to treat hydrocephalus, it is necessary to remove excess cerebrospinal fluid, either through drainage from the lumbar spine or by placement of a shunt into the skull and the ventricles.
References
- Kartal MG, Algin O. Evaluation of hydrocephalus and other cerebrospinal fluid disorders with MRI: An update. Insights Imaging. 2014;5(4):531-541.
- Porter RS, Kaplan JL. Merck Manual of Diagnosis and Therapy. 19th Edition. Merck Sharp & Dohme Corp. Whitehouse Station, N.J; 2011.
- Huttner HB, Schwab S, Bardutzky J. Lumbar drainage for communicating hydrocephalus after ICH with ventricular hemorrhage. Neurocrit Care. 2006;5(3):193-196.
- Xu H. New concept of the pathogenesis and therapeutic orientation of acquired communicating hydrocephalus. Neurol Sci. 2016;37(9):1387-1389.
- Tully HM, Dobyns WB. Infantile hydrocephalus: a review of epidemiology, classification and causes. Eur J Med Genet. 2014;57(8):359-368.
- Al Hinai Q, Zeitouni A, Sirhan D, et al. Communicating Hydrocephalus and Vestibular Schwannomas: Etiology, Treatment, and Long-Term Follow-Up. J Neurol Surg B Skull Base. 2013;74(2):68-74.
- Onuma K, Ishikawa E, Matsuda M, Hirata K, Osuka S, Yamamoto T, et al. Clinical characteristics and neuroimaging findings in 12 cases of recurrent glioblastoma with communicating hydrocephalus. Neurol Med Chir (Tokyo). 2013;53(7):474-481.
- Garne E, Loane M, Addor MC, Boyd PA, Barisic I, Dolk H. Congenital hydrocephalus - prevalence, prenatal diagnosis and outcome of pregnancy in four European regions. Eur J Paediatr Neurol. 2010;14(2):150-155.
- Munch TN, Rasmussen ML, Wohlfahrt J, Juhler M, Melbye M. Risk factors for congenital hydrocephalus: a nationwide, register-based, cohort study. J Neurol Neurosurg Psychiatry. 2014;85(11):1253-1259.
- Fabiano AJ, Doyle K, Grand W. Delayed stoma failure in adult communicating hydrocephalus after initial successful treatment by endoscopic third ventriculostomy: case report. Neurosurgery. 2010;66(6):E1210-1211.