Congenital Hydrocephalus

Categories: Bone diseases, Fetal diseases, Genetic diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Congenital Hydrocephalus

MalaCards integrated aliases for Congenital Hydrocephalus:

Name: Congenital Hydrocephalus 54 60 38 56
Hydrocephalus 54 74
Hydrocephalus, Nonsyndromic, Autosomal Recessive 1 74
Hydrocephalus Adverse Event 74
Ventriculomegaly 54
Hydrocephaly 54


Orphanet epidemiological data:

congenital hydrocephalus
Prevalence: 6-9/10000 (United States),1-5/10000 (Europe);


External Ids:

KEGG 38 H01677
ICD10 34 Q03
ICD10 via Orphanet 35 Q03.0 Q03.1 Q03.8 more
UMLS via Orphanet 75 C0020256
Orphanet 60 ORPHA2185
SNOMED-CT via HPO 70 230745008

Summaries for Congenital Hydrocephalus

NIH Rare Diseases : 54 Congenitalhydrocephalusis when a child is born with an excessive accumulation of cerebrospinal fluid (CSF) in the brain. CSF is a clear fluid that surrounds the brain and spinal cord. This excess fluid causes an abnormal widening of spaces in the brain called ventricles (ventriculomegalia) and can create a harmful pressure on brain tissue. Symptoms of hydrocephalus vary and may include an unusually large head with thin, transparent scalp, bulging forehead with increased spaces between the bones of the skull (fontanelles), and a downward gaze. Other symptoms may include seizures, abnormal reflexes, slow heartbeat and respiratory rate, headaches, vomiting, irritability, weakness, and visual problems.  It is caused by genetic and non-genetic factors. The most common cause of congenital hydrocephalus are variations (mutations) in the  L1CAM gene, where there is a narrow passageway between the third and fourth ventricles (aqueductal stenosis). Other causes include mutations in many other genes, brain and/or spinal cord malformations, infections, bleeding inside the cavities of the brain (intraventricular hemorrhage), trauma, exposition to certain drugs (teratogens) or a congenital tumor of the brain. Congenital hydrocephalus can be an isolated malformation or be part of a syndrome where there are other associated malformations. It is most often treated by surgically inserting a shunt system to transport the excess CSF and allow for re-absorption. If left untreated, blindness and continuing mental deterioration may occur. Hydrocephalus may be subdivided according to the particular defect that exists in the brain and whether the cerebrospinal fluid pressure is high or normal: Communicating hydrocephalus is when there is no blockage (obstruction) in the ventricules but the fluid is not absorbed readily, or there is too much fluid  to be absorbed.  Noncommunicating (obstructive) hydrocephalus is when there is a blockage of the CSF causing widening (dilation) of the pathways that are located upstream of the block, resulting in an increased  pressure inside the brain.  There are also 2 other forms of hydrocephalus that usually affect only adults:Normal-pressure hydrocephalus is where the ventricules are expanded but the pressure inside the nervous system is normal.  Hydrocephalus ex-vacuo occurs when stroke or traumatic injury cause damage to the brain and the brain tissue may shrink.  Hydrocephalus may also be classified in congenital or acquired. Acquired hydrocephalus develops at the time of birth or at some point afterward and may be caused by injury or disease.

MalaCards based summary : Congenital Hydrocephalus, also known as hydrocephalus, is related to hydrocephalus, congenital, 1 and hydrocephalus, and has symptoms including seizures, tremor and back pain. An important gene associated with Congenital Hydrocephalus is CCDC88C (Coiled-Coil Domain Containing 88C), and among its related pathways/superpathways are Axon guidance and Cell adhesion molecules (CAMs). The drugs Dexamethasone acetate and Dexamethasone have been mentioned in the context of this disorder. Affiliated tissues include brain, testes and spinal cord, and related phenotypes are hydrocephalus and behavior/neurological

Related Diseases for Congenital Hydrocephalus

Diseases in the Hydrocephalus family:

Hydrocephalus, Autosomal Dominant Hydrocephalus, Congenital, 1
Hydrocephalus Due to Congenital Stenosis of Aqueduct of Sylvius Congenital Hydrocephalus
Hydrocephalus Autosomal Recessive

Diseases related to Congenital Hydrocephalus via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 873)
# Related Disease Score Top Affiliating Genes
1 hydrocephalus, congenital, 1 33.3 CCDC88C MPDZ
2 hydrocephalus 31.3 CCDC88C FOXC1 FZD3 HYDIN L1CAM MBP
3 hydrocephalus due to congenital stenosis of aqueduct of sylvius 30.3 L1CAM MBP
4 periventricular leukomalacia 29.4 MBP TNF
5 ventriculomegaly with cystic kidney disease 12.6
6 megalencephaly-polymicrogyria-polydactyly-hydrocephalus syndrome 1 12.6
7 proliferative vasculopathy and hydranencephaly-hydrocephaly syndrome 12.6
8 chondrodysplasia with platyspondyly, distinctive brachydactyly, hydrocephaly, and microphthalmia 12.5
9 megalencephaly-polymicrogyria-polydactyly-hydrocephalus syndrome 2 12.4
10 vacterl association, x-linked, with or without hydrocephalus 12.4
11 vacterl association with hydrocephalus 12.4
12 hydrocephalus, endocardial fibroelastosis, and cataracts 12.4
13 dandy-walker malformation with sagittal craniosynostosis and hydrocephalus 12.3
14 axenfeld-rieger anomaly with partially absent eye muscles, distinctive face, hydrocephaly, and skeletal abnormalities 12.2
15 vacterl hydrocephaly 12.2
16 thoracic dysplasia-hydrocephalus syndrome 12.2
17 hydrocephalus-cleft palate-joint contractures syndrome 12.2
18 vacterl association with hydrocephaly, x-linked 12.1
19 masa syndrome 12.1
20 ventriculomegaly with defects of the radius and kidney 12.1
21 tremor, hereditary essential, and idiopathic normal pressure hydrocephalus 12.1
22 vacterl with hydrocephalus 12.1
23 hydrocephalus, autosomal dominant 12.0
24 cerebellum agenesis hydrocephaly 12.0
25 enamel hypoplasia cataract hydrocephaly 12.0
26 growth retardation hydrocephaly lung hypoplasia 12.0
27 osteochondrodysplasia, rhizomelic, with callosal agenesis, thrombocytopenia, hydrocephalus, and hypertension 11.9
28 hydrocephalus autosomal recessive 11.9
29 hydrocephalus skeletal anomalies 11.9
30 tibial aplasia ectrodactyly hydrocephalus 11.9
31 craniosynostosis-hydrocephalus-arnold-chiari malformation type i-radioulnar synostosis syndrome 11.9
32 port-wine nevi-mega cisterna magna-hydrocephalus syndrome 11.9
33 beemer ertbruggen syndrome 11.8
34 chudley-mccullough syndrome 11.8
35 daentl towsend siegel syndrome 11.7
36 cole-carpenter syndrome 1 11.7
37 cole-carpenter syndrome 11.6
38 hydrocephalus, congenital, 2, with or without brain or eye anomalies 11.4
39 hordnes engebretsen knudtson syndrome 11.4
40 pettigrew syndrome 11.4
41 melanosis, neurocutaneous 11.4
42 vater/vacterl association 11.3
43 fanconi anemia, complementation group b 11.3
44 plasminogen deficiency, type i 11.3
45 hydrocephalus, congenital, 3, with brain anomalies 11.3
46 faye-petersen-ward-carey syndrome 11.2
47 kozlowski brown hardwick syndrome 11.2
48 schwartz cohen-addad lambert syndrome 11.2
49 ependymoma 11.2
50 chromosome 8q12.1-q21.2 deletion syndrome 11.2

Graphical network of the top 20 diseases related to Congenital Hydrocephalus:

Diseases related to Congenital Hydrocephalus

Symptoms & Phenotypes for Congenital Hydrocephalus

Human phenotypes related to Congenital Hydrocephalus:

60 33
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 hydrocephalus 60 33 hallmark (90%) Very frequent (99-80%) HP:0000238

UMLS symptoms related to Congenital Hydrocephalus:

seizures, tremor, back pain, pain, headache, syncope, chronic pain, sciatica, vertigo/dizziness, sleeplessness

MGI Mouse Phenotypes related to Congenital Hydrocephalus:

# Description MGI Source Accession Score Top Affiliating Genes
1 behavior/neurological MP:0005386 10.23 EML1 FZD3 HYDIN ISLR2 L1CAM MBP
2 cellular MP:0005384 10.21 EML1 FOXC1 FOXL1 FZD3 HYDIN L1CAM
3 growth/size/body region MP:0005378 10.15 EML1 FOXC1 FOXL1 FZD3 HYDIN L1CAM
4 mortality/aging MP:0010768 9.96 FOXC1 FOXL1 FZD3 HYDIN ISLR2 L1CAM
5 craniofacial MP:0005382 9.95 EML1 FOXC1 HYDIN L1CAM POMK STK36
6 nervous system MP:0003631 9.93 EML1 FOXC1 FZD3 HYDIN ISLR2 L1CAM
7 hearing/vestibular/ear MP:0005377 9.72 ALDH1L1 FZD3 MBP MPDZ STK36
8 reproductive system MP:0005389 9.56 FOXC1 FZD3 HYDIN L1CAM MBP POMK
9 vision/eye MP:0005391 9.17 EML1 FOXC1 FZD3 L1CAM MBP TNF

Drugs & Therapeutics for Congenital Hydrocephalus

Drugs for Congenital Hydrocephalus (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 204)
# Name Status Phase Clinical Trials Cas Number PubChem Id
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 4,Phase 3 1177-87-3
Dexamethasone Approved, Investigational, Vet_approved Phase 4,Phase 3 50-02-2 5743
Propranolol Approved, Investigational Phase 4 525-66-6 4946
Citalopram Approved Phase 4 59729-33-8 2771
Cefotaxime Approved Phase 4 63527-52-6 5742673 456256
Cefoxitin Approved Phase 4 35607-66-0 441199
Acetaminophen Approved Phase 4 103-90-2 1983
Propofol Approved, Investigational, Vet_approved Phase 4,Not Applicable 2078-54-8 4943
Lidocaine Approved, Vet_approved Phase 4,Not Applicable 137-58-6 3676
Dexmedetomidine Approved, Vet_approved Phase 4,Phase 3 113775-47-6 5311068 68602
11 Antineoplastic Agents, Hormonal Phase 4,Phase 3
12 BB 1101 Phase 4,Phase 3
13 Peripheral Nervous System Agents Phase 4,Phase 3,Phase 1,Phase 2,Not Applicable
14 Gastrointestinal Agents Phase 4,Phase 3
15 Antiemetics Phase 4,Phase 3
16 glucocorticoids Phase 4,Phase 3
17 Autonomic Agents Phase 4,Phase 3,Phase 1,Phase 2,Not Applicable
18 Hormone Antagonists Phase 4,Phase 3,Phase 2,Phase 1
19 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4,Phase 3,Phase 2,Phase 1
protease inhibitors Phase 4,Phase 3
21 HIV Protease Inhibitors Phase 4,Phase 3
22 Anti-Inflammatory Agents Phase 4,Phase 3
23 Hormones Phase 4,Phase 3,Phase 2,Phase 1
24 Analgesics Phase 4,Phase 3,Not Applicable
25 Anesthetics Phase 4,Phase 2,Phase 3,Not Applicable
26 Pharmaceutical Solutions Phase 4,Phase 2,Phase 3
27 Neurotransmitter Agents Phase 4,Phase 3,Phase 1,Phase 2,Not Applicable
28 Adrenergic Agents Phase 4,Phase 3,Phase 1,Phase 2
29 Adrenergic Antagonists Phase 4
30 Anti-Arrhythmia Agents Phase 4,Phase 3,Not Applicable
31 Antihypertensive Agents Phase 4
32 Vasodilator Agents Phase 4,Phase 2
33 Adrenergic beta-Antagonists Phase 4
34 Serotonin Uptake Inhibitors Phase 4
35 Muscarinic Antagonists Phase 4
36 Neurotransmitter Uptake Inhibitors Phase 4
37 Cholinergic Antagonists Phase 4
38 Cholinergic Agents Phase 4,Not Applicable
39 Serotonin Receptor Agonists Phase 4
40 Serotonin Agents Phase 4
41 Analgesics, Non-Narcotic Phase 4,Phase 3
42 Anti-Infective Agents Phase 4,Phase 3,Phase 2,Phase 1
43 beta-Lactams Phase 4
44 Anti-Bacterial Agents Phase 4,Phase 3,Phase 1,Phase 2
45 Antipyretics Phase 4
46 Lactams Phase 4
47 Anesthetics, General Phase 4,Phase 3
48 Hypnotics and Sedatives Phase 4,Phase 3
49 Anesthetics, Intravenous Phase 4,Phase 3
50 Central Nervous System Depressants Phase 4,Phase 3,Not Applicable

Interventional clinical trials:

(show top 50) (show all 212)
# Name Status NCT ID Phase Drugs
1 NIMIP: Non Invasive Measurement of the Intracranial Pressure Unknown status NCT01685450 Phase 4
2 The Relationships Between Gene Polymorphisms of LTA4H and Dexamethasone Treatment for Tuberculous Meningitis Unknown status NCT02588196 Phase 4 Dexamethasone acetate
3 ShuntCheck-Micro-Pumper Pediatric Clinical Outcomes Study Unknown status NCT01881711 Phase 4
4 ShuntCheck Versus Radionuclide in Evaluating Shunt Function in Symptomatic NPH Patients Completed NCT01323764 Phase 4
5 Using Propranolol in Traumatic Brain Injury to Reduce Sympathetic Storm Phenomenon Completed NCT03401515 Phase 4 Propranolol Hydrochloride 1 MG/ML
6 NIMI-NICU: Non Invasive Monitoring of the Intracranial Pressure - NeuroIntensive Care Unit Completed NCT01685476 Phase 4
7 Efficacy and Safety of Selective Serotonin Reuptake Inhibitor (SSRI) in Overactive Bladder Patients Completed NCT00902421 Phase 4 Selective serotonin reuptake inhibitors;Antimuscarinics
8 Slow Initial β-lactam Infusion With High-dose Paracetamol to Improve the Outcomes of Childhood Bacterial Meningitis Completed NCT01540838 Phase 4 Infusion with paracetamol;Bolus without paracetamol
9 Dexmedetomidine and Propofol for Pediatric MRI Sedation Recruiting NCT03513757 Phase 4 propofol;Dexmedetomidine
10 Clinical, Pharmacological and Molecular Effects of IV and Oral Acetaminophen in Adults With aSAH Terminated NCT02549716 Phase 4 IV acetaminophen;Oral acetaminophen;Oral placebo;IV placebo
11 Effect of Intrathoracic Pressure Regulation on Traumatic Brain Injury Terminated NCT01824589 Phase 4
12 Contrast Enhanced Ultrasound Evaluation of Brain Perfusion in Neonatal Post-Hemorrhagic Hydrocephalus Withdrawn NCT03061045 Phase 4 Sulfur Hexafluoride Lipid Type A Microspheres 25 MG Intravenous Powder for Suspension [LUMASON]
13 ShuntCheck Accuracy in Detecting Shunt Obstruction Normal Pressure Hydrocephalus (NPH) Patients Withdrawn NCT00793416 Phase 4
14 Multi-Center Study of ShuntCheck Withdrawn NCT00788385 Phase 4
15 Clinical Value of Noninvasive Intracranial Pressure Measurement Unknown status NCT02130219 Phase 2, Phase 3
16 A Randomised Controlled Trial of Lumbar Drainage to Treat Communicating Hydrocephalus After Severe Intraventricular Hemorrhage Completed NCT01041950 Phase 2, Phase 3
17 PET Imaging of Brain Amyloid in Normal Pressure Hydrocephalus Completed NCT01053312 Phase 3 [18F] Flutemetamol
18 A Precision and Accuracy Study of the Codman Valve Position Verification (VPV) System. Completed NCT00196196 Phase 3
19 Quantitative Characterization of Safe Irrigation for Ventricular Shunt Catheters Completed NCT02651337 Phase 3
20 Intraoral 30% Glucose Effect In Newborns Completed NCT02325583 Phase 3 30% Glucose;Midazolam
21 Impact of Ventricular Catheter Used With Antimicrobial Agents on Patients With a Ventricular Catheter Completed NCT00286104 Phase 3
22 Premature Infants in Need of Transfusion (PINT) Completed NCT00182390 Phase 3
23 Efficacy and Safety of Everolimus (RAD001) in Patients of All Ages With Subependymal Giant Cell Astrocytoma Associated With Tuberous Sclerosis Complex (TSC)(EXIST-1) Completed NCT00789828 Phase 3 Everolimus;Placebo
24 EARLYdrain - Outcome After Early Lumbar CSF-drainage in Aneurysmal SAH Completed NCT01258257 Phase 3
25 High Versus Low Dose of Caffeine for Apnea of Prematurity Completed NCT02103777 Phase 3 Caffeine citrate
26 Caffeine for Apnea of Prematurity (CAP) Completed NCT00182312 Phase 3 Caffeine citrate injection
27 Ultra-early Tranexamic Acid After Subarachnoid Hemorrhage. Recruiting NCT02684812 Phase 2, Phase 3 Tranexamic Acid
28 Leukotriene A4 Hydrolase Stratified Trial of Adjunctive Corticosteroids for HIV-uninfected Adults With Tuberculous Meningitis Recruiting NCT03100786 Phase 3 Dexamethasone
29 Follow up Study to Assess Long Term Safety and Outcomes in Infants and Children Born to Mothers Participating in Retosiban Treatment Studies Recruiting NCT02292784 Phase 2, Phase 3 Retosiban;Atosiban;Placebo
30 International Society of Paediatric Oncology (SIOP) PNET 5 Medulloblastoma Recruiting NCT02066220 Phase 2, Phase 3 Reduced-intensity maintenance chemotherapy;Maintenance chemotherapy
31 Randomized Controlled Trial of Shunt vs ETV/CPC for PIH in Ugandan Infants Active, not recruiting NCT01936272 Phase 3
32 The CSF Shunt Entry Site Trial Active, not recruiting NCT02425761 Phase 3
33 Preterm Erythropoietin Neuroprotection Trial (PENUT Trial) Active, not recruiting NCT01378273 Phase 3 Epo
34 Transfusion of Prematures Trial Active, not recruiting NCT01702805 Phase 3
35 A Randomized Trial to Prevent Congenital Cytomegalovirus (CMV) Active, not recruiting NCT01376778 Phase 3 CMV hyperimmune globulin
36 Erythropoietin for the Repair of Cerebral Injury in Very Preterm Infants Active, not recruiting NCT02076373 Phase 3 recombinant human Erythropoietin;Placebo
37 Neuroprotectant for Hypertensive Intracerebral Hemorrhage Not yet recruiting NCT03546283 Phase 3 Placebos;Cattle Encephalon Glycoside and Ignotin
38 Isoflurane-induced Neuroinflammation in Children With Hydrocephalus Terminated NCT02512809 Phase 3 Isoflurane;Dexmedetomidine
39 Positron Emission Tomography Imaging of Brain Amyloid in Normal Pressure Hydrocephalus Terminated NCT01092546 Phase 3 [18F]Flutemetamol
40 Normal Pressure Hydrocephalus Treatment by Acetazolamide Terminated NCT02163330 Phase 3 Acetazolamide;sugar pill
41 The Tilburg Vasospasm Study Terminated NCT01407614 Phase 2, Phase 3
42 A Randomized Study Comparing the Efficacy and Safety of Retosiban Versus Atosiban for Women in Spontaneous Preterm Labour Terminated NCT02292771 Phase 3 Retosiban;Atosiban;Placebo matching retosiban;Placebo matching atosiban
43 Radiation Therapy and Stereotactic Radiosurgery With or Without Temozolomide or Erlotinib in Treating Patients With Brain Metastases Secondary to Non-Small Cell Lung Cancer Terminated NCT00096265 Phase 3 Erlotinib Hydrochloride;Temozolomide
44 A Phase III Efficacy and Safety Study of Intravenous Retosiban Versus Placebo for Women in Spontaneous Preterm Labor Terminated NCT02377466 Phase 3 Retosiban IV infusion;Placebo IV infusion
45 Intraventricular Tissue Plasminogen Activator (tPA) in the Management of Aneurysmal Subarachnoid Hemorrhage Unknown status NCT01098890 Phase 2 Tissue Plasminogen Activator;Placebo
46 A Study Comparing Two Treatments for Infants With Hydrocephalus Completed NCT00652470 Phase 2
47 Study to Evaluate Efficacy of Shunt Operation for Idiopathic Normal Pressure Hydrocephalus Completed NCT00221091 Phase 2
48 Determination of Levels of Micafungin in Neonates Suffering From Systemic Candidiasis and/or Candida Meningitis Completed NCT03421002 Phase 2 Micafungin
49 Overcoming Membrane Transporters to Improve CNS Drug Delivery - Improving Brain Antioxidants After Traumatic Brain Injury Completed NCT01322009 Phase 1, Phase 2 Probenecid and N-acetyl cysteine;Placebo
50 Cilengitide in Treating Younger Patients With Recurrent or Progressive High-Grade Glioma That Has Not Responded to Standard Therapy Completed NCT00679354 Phase 2 cilengitide

Search NIH Clinical Center for Congenital Hydrocephalus

Genetic Tests for Congenital Hydrocephalus

Anatomical Context for Congenital Hydrocephalus

MalaCards organs/tissues related to Congenital Hydrocephalus:

Brain, Testes, Spinal Cord, Bone, Eye, Pituitary, Kidney

Publications for Congenital Hydrocephalus

Articles related to Congenital Hydrocephalus:

(show top 50) (show all 3191)
# Title Authors Year
Palliative cerebrospinal fluid shunting for leptomeningeal metastasis-related hydrocephalus in patients with lung adenocarcinoma: A single-center retrospective study. ( 30629621 )
Obstructive hydrocephalus associated with spontaneous dural arteriovenous fistula: A case report. ( 30946337 )
Hydrocephalus due to a cavernoma-like lesion of an obliterated cerebral arteriovenous malformation treated by embolization and radiosurgery. ( 30885872 )
Clinical Features of and Risk Factors for Hydrocephalus in Childhood Bacterial Meningitis. ( 30373442 )
Management of subdural effusion and hydrocephalus following decompressive craniectomy for posttraumatic cerebral infarction in a patient with traumatic brain injury: a case report. ( 30813919 )
Risk factors for the development of posttraumatic hydrocephalus after unilateral decompressive craniectomy in patients with traumatic brain injury. ( 30827885 )
An elderly patient with Alzheimer's disease, normal pressure hydrocephalus and traumatic brain injury: presented with behavioral symptoms similar to behavioral variant frontotemporal dementia. ( 30475083 )
"Intramedullary capillary hemangioma presenting with hydrocephalus and spastic paraparesis in a two-month-old infant". ( 30685376 )
Hydrocephalus-induced neurogenic stunned myocardium and cardiac arrest in a child: completely reversed with CSF diversion. ( 31003226 )
AQP1 Overexpression in the CSF of Obstructive Hydrocephalus and Inversion of Its Polarity in the Choroid Plexus of a Chiari Malformation Type II Case. ( 31039249 )
Adverse event following live attenuated chikungunya vaccine in a cynomolgus macaque with pre-existing chronic hydrocephalus. ( 30945306 )
Lumboperitoneal and Ventriculoperitoneal Shunt Surgery for Posthemorrhagic Communicating Hydrocephalus: A Comparison. ( 30947009 )
Comparative Study Between Laparoscopic and Open Techniques for Insertion of Ventriculperitoneal Shunt for Treatment of Congenital Hydrocephalus. ( 30307809 )
A novel ISLR2-linked autosomal recessive syndrome of congenital hydrocephalus, arthrogryposis and abdominal distension. ( 30483960 )
Congenital hydrocephalus in a Belgian draft horse associated with a nonsense mutation in B3GALNT2. ( 30705458 )
Comparative study of intracisternal kaolin injection techniques to induce congenital hydrocephalus in fetal lamb. ( 30805823 )
Results of oral prednisolone administration or ventriculoperitoneal shunt placement in dogs with congenital hydrocephalus: 40 cases (2005-2016). ( 30888275 )
Trim71/lin-41 Links an Ancient miRNA Pathway to Human Congenital Hydrocephalus. ( 30975633 )
Visualizing flow in an intact CSF network using optical coherence tomography: implications for human congenital hydrocephalus. ( 30996265 )
Susceptibility Weighted Imaging (SWI) Recommended as a Regular Magnetic Resonance Diagnosis for Vascular Dementia to Identify Independent Idiopathic Normal Pressure Hydrocephalus Before Ventriculo-Peritoneal (V-P) Shunt Treatment: A Case Study. ( 30984097 )
Comorbidity of diabetes mellitus in idiopathic normal pressure hydrocephalus: a systematic literature review. ( 30744635 )
Comparative Analysis of Subventricular Zone Glioblastoma Contact and Ventricular Entry During Resection in Predicting Dissemination, Hydrocephalus, and Survival. ( 31058968 )
Holoprosencephaly or severe hydrocephalus: T1 sequence tells the story. ( 31068345 )
Leukoencephalopathy, intracranial calcifications, cysts and SNORD118 mutation (Labrune Syndrome) with obstructive hydrocephalus. ( 30794980 )
Calgary Shunt Protocol, an adaptation of the Hydrocephalus Clinical Research Network shunt protocol, reduces shunt infections in children. ( 30797206 )
Epidemiology of pediatric hydrocephalus in Haiti: analysis of a surgical case series. ( 30797213 )
Comprehensive analysis of differentially expressed profiles of long non-coding RNAs and messenger RNAs in kaolin-induced hydrocephalus. ( 30797995 )
Clinical, Radiological, and Demographic Details of Benign External Hydrocephalus: A Population-Based Study. ( 30808532 )
Optic disc cupping characteristics of normal pressure hydrocephalus patients with normal-tension glaucoma. ( 30816215 )
Diverticular enlargement of the foramen of Luschka and hydrocephalus in a child with Noonan syndrome. ( 30822582 )
Slit Ventricle Syndrome Leads to a 10-year history of Repetitive Transient Central Herniation Masquerading as Seizures: A Hydrocephalus Case Report. ( 30831286 )
Rhombencephalitis caused by Listeria monocytogenes with hydrocephalus and intracranial hemorrhage: A case report and review of the literature. ( 30842967 )
Prevalence of C9ORF72 Expansion in a Large Series of Patients with Idiopathic Normal-Pressure Hydrocephalus. ( 30861516 )
Effects of minocycline on epiplexus macrophage activation, choroid plexus injury and hydrocephalus development in spontaneous hypertensive rats. ( 30862302 )
Healthcare Economics of Hydrocephalus After Aneurysmal Subarachnoid Hemorrhage in the United States. ( 30864050 )
Hydrocephalus Due to Idiopathic Fourth Ventricle Outflow Obstruction. ( 30865115 )
Systematic analysis and identification of unexpected interactions from the neuroprotein drug interactome in hydrocephalus pharmacological intervention. ( 30866733 )
The Added Value of Magnetic Resonance Imaging Cisternography and Ventriculography as a Diagnostic Aid in Pediatric Hydrocephalus. ( 30870836 )
Lower Iron Levels Predict Acute Hydrocephalus Following Aneurysmal Subarachnoid Hemorrhage. ( 30872197 )
Ventriculostomy and endoscopic biopsy of pineal mass with hydrocephalus and unexpected definitive diagnosis. ( 30878484 )
Comparison of Complication and Revision Rates after Frontal versus Parietal Approach for Ventricular Shunt Placement in Idiopathic Normal Pressure Hydrocephalus. ( 30878745 )
Treatment of normal pressure hydrocephalus resolves dorsal midbrain syndrome. ( 30879220 )
Measurement of changes in optic nerve sheath diameter using ultrasound and computed tomography scan before and after the ventriculoperitoneal shunt surgery in patients with hydrocephalus - A prospective observational trial. ( 30880478 )
Background Risk Factors Associated with Shunt Intervention for Possible Idiopathic Normal Pressure Hydrocephalus: A Nationwide Hospital-Based Survey in Japan. ( 30883349 )
Revisiting the Cerebrospinal Fluid Biomarker Profile in Idiopathic Normal Pressure Hydrocephalus: The Bologna Pro-Hydro Study. ( 30883350 )
Hydrocephalus in neurocysticercosis: challenges for clinical practice and basic research perspectives. ( 30885859 )
Benign External Hydrocephalus in Children: A Condition in Search of an Etiology. ( 30890296 )
Cerebral blood flow and Alzheimer's disease-related biomarkers in cerebrospinal fluid in idiopathic normal pressure hydrocephalus. ( 30916850 )
Upward movement of cerebrospinal fluid in obstructive hydrocephalus-revision of an old concept. ( 30919042 )
Health Care Expenditures of Medicare Beneficiaries with Normal Pressure Hydrocephalus. ( 30928595 )

Variations for Congenital Hydrocephalus

Expression for Congenital Hydrocephalus

Search GEO for disease gene expression data for Congenital Hydrocephalus.

Pathways for Congenital Hydrocephalus

Pathways related to Congenital Hydrocephalus according to KEGG:

# Name Kegg Source Accession
1 Axon guidance hsa04360
2 Cell adhesion molecules (CAMs) hsa04514
3 Tight junction hsa04530

GO Terms for Congenital Hydrocephalus

Cellular components related to Congenital Hydrocephalus according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cell surface GO:0009986 9.02 FZD3 ISLR2 L1CAM MBP TNF

Biological processes related to Congenital Hydrocephalus according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 multicellular organism development GO:0007275 9.56 FOXC1 FOXL1 FZD3 HYDIN ISLR2 L1CAM
2 negative regulation of mitotic cell cycle GO:0045930 9.26 FOXC1 TNF
3 positive regulation of axon extension GO:0045773 9.16 ISLR2 L1CAM
4 brain development GO:0007420 9.1 EML1 FOXC1 FZD3 HYDIN POMK STK36

Molecular functions related to Congenital Hydrocephalus according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 DNA binding, bending GO:0008301 8.62 FOXC1 FOXL1

Sources for Congenital Hydrocephalus

9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
33 HPO
34 ICD10
35 ICD10 via Orphanet
39 LifeMap
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
56 Novoseek
59 OMIM via Orphanet
63 PubMed
71 SNOMED-CT via Orphanet
73 Tocris
75 UMLS via Orphanet
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