Hydrocephalus - The Causes and risk factors

Hydrocephalus, also known as "water in the brain" is defined as complex and multifactorial neurological disorders of accumulation of cerebrospinal fluid (CSF) in the cavity of brain of that can lead to intracranial pressure inside the side, resulting of brain trauma, stroke, infection, tumor, etc.
The Causes and risk factors
A. Causes
Although the actual causes of  Hydrocephalus is unknown, some researchers suggested that
1. Genetic defect
Congenital hydrocephalus id associated with dysfunctional motile cilia represent the underlying pathogenetic mechanism in 8 of the 12 lines (Ulk4, Nme5, Nme7, Kif27, Stk36, Dpcd, Ak7, and Ak8).(3). Other researchers suggested that Although hydrocephalus is usually a sporadic disorder, there exist some rare but well delineated syndroms, in which recurrence is to be expected. The here presented Bickers-Adams-Syndrome of sex-linked hydrocephalus is caused by congenital aqueductal stenosis(4).

2.  Meningitis
Hydrocephalus is associated with the complications of tuberculous meningitis (TBM) occurred in up to 85% of children with the disease. It is more severe in children than in adults. It could be either of the communicating type or the obstructive type with the former being more frequently seen(5).

3. Neural tube defect
Congenital hydrocephalus is a relatively uncommon abnormality in population malformation surveys accounting for between four and ten out of every 10,000 births. In the review of Sixty cases of  28 with NTD (group 1) and 32 without NTD (group 2). The groups were similar in terms of maternal and child variables at birth and hospitalization days during the 1st year of life. The mortality (including intrauterine deaths and deaths of babies with malformations incompatible with life that characterize a very poor prognosis) until 1 year of age was 36% in group 1 and 59% in group 2 (p = 0.077). The rate of cardiac malformations was higher in the group without NTD (p = 0.015). The length of hospital stay after birth (1st admission) was significantly higher in the group with NTD (p = 0.007)(6).

5. Intraventricular hemorrhage
Intraventricular hemorrhage and posthemorrhagic hydrocephalus are common causes of neonatal morbidity and mortality among preterm and low-birth weight infants (PT-LBWIs), but Intraventricular hemorrhage in PT-LBWIs remains a significant problem, particularly when it is associated with PHH leading to long-term neurological impairment and decreased survival rate(7).

6. PTB Deficiency
Polypyrimidine tract-binding protein (PTB) is a well-characterized RNA-binding protein and known to be preferentially expressed in neural stem cells (NSCs) in the central nervous system. Dr. Shibasaki T and the research team at the Center for Experimental Medicine and Systems Biology. showed that PTB depletion in the dorsal telencephalon is causally involved in the development of HC and that PTB is important for the maintenance of AJs in the NSCs of the dorsal telencephalon(8).

6.  Tumors
Tumor-associated hydrocephalus is common in primary pediatric brain tumors. In the study of a total of 56.7% of cases presented hydrocephalus, including hydrocephalus that occurred at tumor diagnosis (51.5%), and hydrocephalus developed after tumor diagnosis (5.1%). At tumor diagnosis, the hydrocephalus was mainly obstructive type (98%) and rarely communicating type (1.9%). Definite shunting procedures in this series comprised of ventriculoperitoneal (VP) shunt in 54.4%, endoscopic third ventriculostomy (ETV) in 10.9%, subduroperitoneal (SP) shunt in 4.8%, septostomy in 0.7%, lumboperitoneal shunt in 0.6%, and ventriculoatrial shunt in 0.1% of patients with hydrocephalus(9).

7. Traumatic head injury
Post-traumatic hydrocephalus (PTH) is considered a frequent complication after severe head injury (HI). External hydrocephalus is a well-established entity in infants which is benign and usually resolves without shunting [1, 2]. The term “External Hydrocephalus” has also been used to describe the presence of extra ventricular cerebrospinal fluid (CSF) collections accompanied by hydrocephalus, particularly in cases of adults suffering from aneurysmal subarachnoid hemorrhage and severe head injuries [3–6]. Several other terms have been used to describe this entity [7] which has lead to confusion about this disease(10).


8. Subarachnoid hemorrhage 
Acute hydrocephalus as a consequence of subarachnoid hemorrhage is a relatively frequent problem. Dr. Suarez-Rivera O. at the National Institute of Neurology and Neurosurgery, Mexico City, indicated that that acute hydrocephalus is present in 20% of patients with subarachnoid hemorrhage. One third of them may be asymptomatic on admission; 50% of those who have clinical hydrocephalus recover spontaneously within the first 24 h(11)

9. Etc.

B. Risk factors
1. Family history

Family studies suggested that the risk to siblings of a child with uncomplicated congenital hydrocephalus, where the anatomical site is not specified, is about 1 in 50 (1 in 40 for males, 1 in 80 for females). With aqueduct stenosis, the risk to brothers of affected boys is 1 in 22, to sisters only 1 in 50. The risks to siblings of sisters is less. Probably less than 2% of uncomplicated hydrocephalus has an X-linked basis and such an aetiology should be suspected if the special clinical features are present, there is more than one male sibling affected, or there are affected male relatives on the mother's side(12).

2. Nontumoral aqueductal stenosis, nontectal tumor and tectal glioma
Hydrocephalus was most commonly due to nontumoral aqueductal stenosis (43%), nontectal tumor (25%), or tectal glioma (13%). Five patients (16%) had multicystic/loculated hydrocephalus(13).

3. Preterm and low-birth weight infants
Preterm and low-birth weight infants are associated with increased risk of  intraventricular and/or periventricular hemorrhage (IVH and PVH, respectively) of that can lead to Hydrocephalus(14).

4. Other factors
Although the precise nature of the neuropsychological deficits in hydrocephalus are not completely known, several factors such as etiology, raised intracranial pressure, ventricular size, and changes in gray and white matter tissue composition as well as shunt treatment complications have been shown to influence cognition. In fact, the presence of complications and other brain abnormalities in addition to hydrocephalus such as infections, trauma, intraventricular hemorrhage, low birthweight, and asphyxia are important determinants of the ultimate cognitive status, placing the child at a high risk of cognitive impairment(15).

5. Etc.
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Sources
(4) http://www.ncbi.nlm.nih.gov/pubmed/3907171
(5) http://www.ncbi.nlm.nih.gov/pubmed/19770534
(6) http://www.ncbi.nlm.nih.gov/pubmed/17934294
(7) http://www.ncbi.nlm.nih.gov/pubmed/16256848
(8) http://www.ncbi.nlm.nih.gov/pubmed/22705452
(9) http://www.ncbi.nlm.nih.gov/pubmed/21928036
(10) http://www.hindawi.com/journals/crim/2010/743784/
(11) http://www.ncbi.nlm.nih.gov/pubmed/9586937
(12) http://www.ncbi.nlm.nih.gov/pubmed/6524107
(13) http://www.ncbi.nlm.nih.gov/pubmed/22702328
(14) http://www.ncbi.nlm.nih.gov/pubmed/22116446
(15) http://www.ncbi.nlm.nih.gov/pubmed/11883667