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 bone, 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 819)
# 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 30.2 L1CAM MBP
4 ventriculomegaly with cystic kidney disease 12.6
5 megalencephaly-polymicrogyria-polydactyly-hydrocephalus syndrome 1 12.6
6 proliferative vasculopathy and hydranencephaly-hydrocephaly syndrome 12.5
7 chondrodysplasia with platyspondyly, distinctive brachydactyly, hydrocephaly, and microphthalmia 12.5
8 vacterl association, x-linked, with or without hydrocephalus 12.4
9 vacterl association with hydrocephalus 12.4
10 hydrocephalus, endocardial fibroelastosis, and cataracts 12.4
11 dandy-walker malformation with sagittal craniosynostosis and hydrocephalus 12.3
12 megalencephaly-polymicrogyria-polydactyly-hydrocephalus syndrome 2 12.3
13 axenfeld-rieger anomaly with partially absent eye muscles, distinctive face, hydrocephaly, and skeletal abnormalities 12.2
14 vacterl hydrocephaly 12.2
15 thoracic dysplasia-hydrocephalus syndrome 12.2
16 hydrocephalus-cleft palate-joint contractures syndrome 12.2
17 vacterl association with hydrocephaly, x-linked 12.1
18 masa syndrome 12.1
19 ventriculomegaly with defects of the radius and kidney 12.1
20 vacterl with hydrocephalus 12.0
21 hydrocephalus, autosomal dominant 12.0
22 cerebellum agenesis hydrocephaly 12.0
23 enamel hypoplasia cataract hydrocephaly 12.0
24 growth retardation hydrocephaly lung hypoplasia 12.0
25 hydrocephaly corpus callosum agenesis diaphragmatic hernia 12.0
26 tremor, hereditary essential, and idiopathic normal pressure hydrocephalus 11.9
27 osteochondrodysplasia, rhizomelic, with callosal agenesis, thrombocytopenia, hydrocephalus, and hypertension 11.9
28 hydrocephalus autosomal recessive 11.9
29 hydrocephalus craniosynostosis bifid nose 11.9
30 hydrocephalus growth retardation skeletal anomalies 11.9
31 hydrocephalus skeletal anomalies 11.9
32 tibial aplasia ectrodactyly hydrocephalus 11.9
33 craniosynostosis-hydrocephalus-arnold-chiari malformation type i-radioulnar synostosis syndrome 11.9
34 port-wine nevi-mega cisterna magna-hydrocephalus syndrome 11.9
35 beemer ertbruggen syndrome 11.7
36 chudley-mccullough syndrome 11.7
37 daentl towsend siegel syndrome 11.7
38 cole-carpenter syndrome 1 11.7
39 cole-carpenter syndrome 11.6
40 brachial amelia, cleft lip, and holoprosencephaly 11.5
41 hordnes engebretsen knudtson syndrome 11.4
42 melanosis, neurocutaneous 11.3
43 vater/vacterl association 11.3
44 chromosome 8q12.1-q21.2 deletion syndrome 11.2
45 faye-petersen-ward-carey syndrome 11.2
46 kozlowski brown hardwick syndrome 11.2
47 schwartz cohen-addad lambert syndrome 11.2
48 ependymoma 11.2
49 porencephaly 11.2
50 hydromyelia 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 203)
# 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 456256 5742673
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
Dexmedetomidine Approved, Vet_approved Phase 4,Phase 3 113775-47-6 68602 5311068
Lidocaine Approved, Vet_approved Phase 4,Not Applicable 137-58-6 3676
11 Peripheral Nervous System Agents Phase 4,Phase 3,Phase 1,Phase 2,Not Applicable
12 Anti-Inflammatory Agents Phase 4,Phase 3
13 HIV Protease Inhibitors Phase 4,Phase 3
14 Hormones Phase 4,Phase 3,Phase 2,Phase 1
protease inhibitors Phase 4,Phase 3
16 Autonomic Agents Phase 4,Phase 3,Phase 1,Phase 2,Not Applicable
17 BB 1101 Phase 4,Phase 3
18 Antineoplastic Agents, Hormonal Phase 4,Phase 3
19 Hormone Antagonists Phase 4,Phase 3,Phase 2,Phase 1
20 Antiemetics Phase 4,Phase 3
21 Gastrointestinal Agents Phase 4,Phase 3
22 glucocorticoids Phase 4,Phase 3
23 Hormones, Hormone Substitutes, and Hormone Antagonists 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 Antihypertensive Agents Phase 4
28 Adrenergic Agents Phase 4,Phase 3,Phase 1,Phase 2
29 Anti-Arrhythmia Agents Phase 4,Phase 3,Not Applicable
30 Neurotransmitter Agents Phase 4,Phase 3,Phase 1,Phase 2,Not Applicable
31 Adrenergic beta-Antagonists Phase 4
32 Adrenergic Antagonists Phase 4
33 Vasodilator Agents Phase 4,Phase 2
34 Neurotransmitter Uptake Inhibitors Phase 4
35 Serotonin Receptor Agonists Phase 4
36 Serotonin Agents Phase 4
37 Serotonin Uptake Inhibitors Phase 4
38 Cholinergic Agents Phase 4,Not Applicable
39 Cholinergic Antagonists Phase 4
40 Muscarinic Antagonists Phase 4
41 Analgesics, Non-Narcotic Phase 4,Phase 3
42 Anti-Infective Agents Phase 4,Phase 3,Phase 1,Phase 2
43 beta-Lactams Phase 4
44 Antipyretics Phase 4
45 Anti-Bacterial Agents Phase 4,Phase 3,Phase 1,Phase 2
46 Lactams Phase 4
47 Central Nervous System Depressants Phase 4,Phase 3,Not Applicable
48 Anesthetics, Intravenous Phase 4,Phase 3
49 Hypnotics and Sedatives Phase 4,Phase 3
50 Anesthetics, General Phase 4,Phase 3

Interventional clinical trials:

(show top 50) (show all 211)
# 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 Recruiting 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 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
49 Cilengitide in Treating Younger Patients With Recurrent or Progressive High-Grade Glioma That Has Not Responded to Standard Therapy Completed NCT00679354 Phase 2 cilengitide
50 Determination of Levels of Micafungin in Neonates Suffering From Systemic Candidiasis and/or Candida Meningitis Completed NCT03421002 Phase 2 Micafungin

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, Bone, Spinal Cord, Pituitary, Eye, Lung

Publications for Congenital Hydrocephalus

Articles related to Congenital Hydrocephalus:

(show top 50) (show all 1827)
# Title Authors Year
Palliative cerebrospinal fluid shunting for leptomeningeal metastasis-related hydrocephalus in patients with lung adenocarcinoma: A single-center retrospective study. ( 30629621 )
Hydrocephalus due to a cavernoma-like lesion of an obliterated cerebral arteriovenous malformation treated by embolization and radiosurgery. ( 30885872 )
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 )
"Intramedullary capillary hemangioma presenting with hydrocephalus and spastic paraparesis in a two-month-old infant". ( 30685376 )
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 )
Comorbidity of diabetes mellitus in idiopathic normal pressure hydrocephalus: a systematic literature review. ( 30744635 )
Murine MPDZ-linked hydrocephalus is caused by hyperpermeability of the choroid plexus. ( 30518636 )
Quality of life and physician-reported developmental, cognitive, and social problems in children with benign external hydrocephalus-long-term follow-up. ( 30523438 )
Surgical treatment of post-inflammatory hydrocephalus. Analysis of 101 cases. ( 30564912 )
Reply to the comments on "Unusual case of idiopathic normal pressure hydrocephalus initially presenting as Parinaud's syndrome?" ( 30609228 )
Unusual case of idiopathic normal pressure hydrocephalus initially presenting as Parinaud's syndrome? ( 30609229 )
Lumbar drain trial outcomes of normal pressure hydrocephalus: a single-center experience of 254 patients. ( 30611143 )
Diagnosis and Management for Secondary Low- or Negative-Pressure Hydrocephalus and a New Hydrocephalus Classification Based on Ventricular Pressure. ( 30611954 )
Idiopathic normal-pressure hydrocephalus. ( 30617229 )
Prenatal brain imaging for predicting need for postnatal hydrocephalus treatment in fetuses that had neural tube defect repair in utero. ( 30620440 )
Correction: Age-specific global epidemiology of hydrocephalus: Systematic review, metanalysis and global birth surveillance. ( 30629693 )
Ventricular shape evaluation on early ultrasound predicts post-hemorrhagic hydrocephalus. ( 30631137 )
Treatment of multi-loculated hydrocephalus using endoscopic cyst fenestration and endoscopic guided VP shunt insertion. ( 30637466 )
Repeated Cerebrospinal Fluid Removal Procedure in Older Patients With Idiopathic Normal Pressure Hydrocephalus Ineligible for Surgical Treatment. ( 30638833 )
Exosomes populate the cerebrospinal fluid of preterm infants with post-haemorrhagic hydrocephalus. ( 30639393 )
Isolated antenatal hydrocephalus after foetal exposure to misoprostol : a teratogenic effect of the cytotec® ? ( 30639481 )
Prognostic Model for Chronic Shunt-Dependent Hydrocephalus After Aneurysmal Subarachnoid Hemorrhage. ( 30639492 )
Obstructive sleep apnea in patients with idiopathic normal-pressure hydrocephalus. ( 30640151 )
Reply: Letter to The Editor on "Idiopathic normal-pressure hydrocephalus and obstructive sleep apnea are frequently associated: A prospective cohort study" Journal of the Neurological Sciences 395 (2018) 164-168. ( 30641246 )
Opposing CSF hydrodynamic trends found in the cerebral aqueduct and prepontine cistern following shunt treatment in patients with normal pressure hydrocephalus. ( 30665428 )
Studies of Hydrocephalus Associated With Long-term Spaceflight May Provide New Insights Into Cerebrospinal Fluid Flow Dynamics Here on Earth. ( 30673794 )
A prospective study on gait dominant normal pressure hydrocephalus. ( 30676642 )
Shunted hydrocephalus: who has more chances to get rid of the shunt? ( 30684709 )
Obstructive hydrocephalus secondary to a perianeurysmal cyst: a case report. ( 30688108 )
[11C]PIB PET Is Associated with the Brain Biopsy Amyloid-β Load in Subjects Examined for Normal Pressure Hydrocephalus. ( 30689567 )
Hydrocephalus in a rat model of Meckel Gruber syndrome with a TMEM67 mutation. ( 30705305 )
Normal Pressure Hydrocephalus. ( 30707192 )
A rare case of negative pressure hydrocephalus: a plausible explanation and the role of transmantle theory. ( 30710718 )
Neonatal Hyponatremia in the Setting of Untreated Progressive Hydrocephalus: Case Report. ( 30716495 )
Prospective multicenter studies in pediatric hydrocephalus. ( 30717034 )
TROPHY registry study design: a prospective, international multicenter study for the surgical treatment of posthemorrhagic hydrocephalus in neonates. ( 30726526 )
Dynamic balance measurements can differentiate fallers from non-fallers in patients with idiopathic normal pressure hydrocephalus. ( 30731067 )
Intracranial endodermal cyst presenting with nonobstructive hydrocephalus: A case report. ( 30732152 )
Olfactory event-related potentials in a functionally anosmic patient with arrested hydrocephalus. ( 30732509 )
Characterization of a novel rat model of X-linked hydrocephalus by CRISPR-mediated mutation in L1cam. ( 30738385 )
The Computerized General Neuropsychological INPH Test revealed improvement in idiopathic normal pressure hydrocephalus after shunt surgery. ( 30738407 )
Cerebrospinal Fluid Biomarkers in Idiopathic Normal Pressure Hydrocephalus versus Alzheimer's Disease and Subcortical Ischemic Vascular Disease: A Systematic Review. ( 30741681 )
Complications associated with ventriculoperitoneal shunts in dogs and cats with idiopathic hydrocephalus: A systematic review. ( 30747447 )
First-in-Human Experience With Integration of a Hydrocephalus Shunt Device Within a Customized Cranial Implant. ( 30753624 )
Hemodynamically self-corrected δadc analysis in idiopathic normal pressure hydrocephalus. ( 30760003 )
Recent advances in rational diagnosis and treatment of normal pressure hydrocephalus: A critical appraisal on novel diagnostic, therapy monitoring and treatment modalities. ( 30767741 )

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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