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Communicating Hydrocephalus

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].

Precocious Puberty
  • 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]
Blue Sclera
  • Signs of connective tissue disorder included: osteopenia, pathological fracture, yellow/grey discolored teeth, blue sclerae and easy bruising. Laboratory investigations failed to reveal the cause of fetal hydrops or collagen abnormality.[ncbi.nlm.nih.gov]
Myopathy
  • Some common pathogenetic mechanism may have been involved in the development of both the myopathy and the hydrocephalus.[ncbi.nlm.nih.gov]
Proximal Muscle Weakness
  • The gait disorder improved with ventriculoperitoneal shunting, but proximal muscle weakness remained. Biopsy specimens of muscle and nerve disclosed typical lysosomal inclusions in both tissues, as well as selective loss of unmyelinated axons.[ncbi.nlm.nih.gov]
Osteopenia
  • Signs of connective tissue disorder included: osteopenia, pathological fracture, yellow/grey discolored teeth, blue sclerae and easy bruising. Laboratory investigations failed to reveal the cause of fetal hydrops or collagen abnormality.[ncbi.nlm.nih.gov]
Brachydactyly
  • He had hypertelorism, orbital hypoplasia without proptosis, brachydactyly, frontal and temporal bossing of the skull, central hypotonia, communicating hydrocephalus, and severe delay in psychomotor development.[ncbi.nlm.nih.gov]
Leg Pain
  • A 17-month-old girl presented to the authors' clinic with a 6-month history of back and leg pain, gait regression, constipation, and marked lumbar hyperlordosis due to a PMAVF. A brain MRI study also demonstrated advanced hydrocephalus.[ncbi.nlm.nih.gov]
Hypertelorism
  • Another male has communicating hydrocephalus and hypertelorism.[ncbi.nlm.nih.gov]
  • He had hypertelorism, orbital hypoplasia without proptosis, brachydactyly, frontal and temporal bossing of the skull, central hypotonia, communicating hydrocephalus, and severe delay in psychomotor development.[ncbi.nlm.nih.gov]
Bulging Fontanelle
  • 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.[symptoma.com]
  • Communicating Hydrocephalus Picture – Showing Bulging fontanel, Lateral ventricle, Third ventricle, Aqueduct of Sylvius and the Fourth ventricle.[heydoctor.org]
  • 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].

Cerebral Angiitis
  • A picture consistent with cerebral angiitis was seen angiographically. After systemic angiitis and CNS infection were excluded, the diagnosis of isolated angiitis of the central nervous system was made.[ncbi.nlm.nih.gov]

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]:

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].

Sex distribution
Age distribution

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

Article

  1. Kartal MG, Algin O. Evaluation of hydrocephalus and other cerebrospinal fluid disorders with MRI: An update. Insights Imaging. 2014;5(4):531-541.
  2. Porter RS, Kaplan JL. Merck Manual of Diagnosis and Therapy. 19th Edition. Merck Sharp & Dohme Corp. Whitehouse Station, N.J; 2011.
  3. Huttner HB, Schwab S, Bardutzky J. Lumbar drainage for communicating hydrocephalus after ICH with ventricular hemorrhage. Neurocrit Care. 2006;5(3):193-196.
  4. Xu H. New concept of the pathogenesis and therapeutic orientation of acquired communicating hydrocephalus. Neurol Sci. 2016;37(9):1387-1389.
  5. Tully HM, Dobyns WB. Infantile hydrocephalus: a review of epidemiology, classification and causes. Eur J Med Genet. 2014;57(8):359-368.
  6. 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.
  7. 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.
  8. 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.
  9. 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.
  10. 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.

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Last updated: 2018-06-21 20:22