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 53 59 37 55
Hydrocephalus 53 73
Hydrocephalus, Nonsyndromic, Autosomal Recessive 1 73
Hydrocephalus Adverse Event 73
Ventriculomegaly 53
Hydrocephaly 53


Orphanet epidemiological data:

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


External Ids:

Orphanet 59 ORPHA2185
ICD10 via Orphanet 34 Q03.1 Q03.8 Q03.9 more
UMLS via Orphanet 74 C0020256
KEGG 37 H01677
ICD10 33 Q03
SNOMED-CT via HPO 69 230745008

Summaries for Congenital Hydrocephalus

NIH Rare Diseases : 53 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 and Dexamethasone acetate have been mentioned in the context of this disorder. Affiliated tissues include brain, bone and spinal cord, and related phenotypes are hydrocephalus and cellular

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 757)
# Related Disease Score Top Affiliating Genes
1 hydrocephalus, congenital, 1 33.1 CCDC88C MPDZ
2 hydrocephalus 31.7 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 12.5
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 12.3
9 hydrocephalus, endocardial fibroelastosis, and cataracts 12.3
10 dandy-walker malformation with sagittal craniosynostosis and hydrocephalus 12.3
11 megalencephaly-polymicrogyria-polydactyly-hydrocephalus syndrome 2 12.3
12 axenfeld-rieger anomaly with partially absent eye muscles, distinctive face, hydrocephaly, and skeletal abnormalities 12.2
13 vacterl hydrocephaly 12.2
14 thoracic dysplasia-hydrocephalus syndrome 12.2
15 hydrocephalus-cleft palate-joint contractures syndrome 12.2
16 vacterl association with hydrocephaly, x-linked 12.1
17 masa syndrome 12.1
18 ventriculomegaly with defects of the radius and kidney 12.1
19 vacterl with hydrocephalus 12.0
20 hydrocephalus, autosomal dominant 12.0
21 cerebellum agenesis hydrocephaly 12.0
22 enamel hypoplasia cataract hydrocephaly 12.0
23 growth retardation hydrocephaly lung hypoplasia 12.0
24 hydrocephaly corpus callosum agenesis diaphragmatic hernia 12.0
25 tremor, hereditary essential, and idiopathic normal pressure hydrocephalus 11.9
26 osteochondrodysplasia, rhizomelic, with callosal agenesis, thrombocytopenia, hydrocephalus, and hypertension 11.9
27 hydrocephalus autosomal recessive 11.9
28 hydrocephalus craniosynostosis bifid nose 11.9
29 hydrocephalus growth retardation skeletal anomalies 11.9
30 hydrocephalus skeletal anomalies 11.9
31 tibial aplasia ectrodactyly hydrocephalus 11.9
32 craniosynostosis-hydrocephalus-arnold-chiari malformation type i-radioulnar synostosis syndrome 11.9
33 port-wine nevi-mega cisterna magna-hydrocephalus syndrome 11.9
34 beemer ertbruggen syndrome 11.7
35 chudley-mccullough syndrome 11.7
36 daentl towsend siegel syndrome 11.7
37 cole-carpenter syndrome 1 11.6
38 cole-carpenter syndrome 11.6
39 brachial amelia, cleft lip, and holoprosencephaly 11.4
40 hordnes engebretsen knudtson syndrome 11.4
41 vacterl association with hydrocephalus 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 ependymoma 11.2
48 porencephaly 11.2
49 primary ciliary dyskinesia 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:

59 32
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 hydrocephalus 59 32 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 cellular MP:0005384 10.18 EML1 FOXC1 FOXL1 FZD3 HYDIN L1CAM
2 behavior/neurological MP:0005386 10.17 EML1 FZD3 HYDIN L1CAM MBP MPDZ
3 growth/size/body region MP:0005378 10.1 EML1 FOXC1 FOXL1 FZD3 HYDIN L1CAM
4 craniofacial MP:0005382 9.91 EML1 FOXC1 HYDIN L1CAM POMK STK36
5 nervous system MP:0003631 9.9 EML1 FOXC1 FZD3 HYDIN L1CAM MBP
6 hearing/vestibular/ear MP:0005377 9.65 ALDH1L1 FZD3 MBP MPDZ STK36
7 reproductive system MP:0005389 9.56 FOXC1 FZD3 HYDIN L1CAM MBP POMK
8 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 198)
# Name Status Phase Clinical Trials Cas Number PubChem Id
Dexamethasone Approved, Investigational, Vet_approved Phase 4,Phase 3 50-02-2 5743
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 4,Phase 3 1177-87-3
Propranolol Approved, Investigational Phase 4 525-66-6 4946
Citalopram Approved Phase 4 59729-33-8 2771
Cefoxitin Approved Phase 4 35607-66-0 441199
Acetaminophen Approved Phase 4 103-90-2 1983
Cefotaxime Approved Phase 4 63527-52-6 456256 5742673
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 Hormone Antagonists Phase 4,Phase 3,Phase 2,Phase 1
12 Peripheral Nervous System Agents Phase 4,Phase 3,Phase 1,Phase 2,Not Applicable
13 Antineoplastic Agents, Hormonal Phase 4,Phase 3
14 Autonomic Agents Phase 4,Phase 3,Phase 1,Phase 2,Not Applicable
15 Hormones Phase 4,Phase 3,Phase 2,Phase 1
16 Anti-Inflammatory Agents Phase 4,Phase 3
17 Antiemetics Phase 4,Phase 3
18 BB 1101 Phase 4,Phase 3
19 HIV Protease Inhibitors Phase 4,Phase 3
protease inhibitors Phase 4,Phase 3
21 glucocorticoids Phase 4,Phase 3
22 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4,Phase 3,Phase 2,Phase 1
23 Gastrointestinal Agents Phase 4,Phase 3
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 Anti-Arrhythmia Agents Phase 4,Phase 3
28 Adrenergic Agents Phase 4,Phase 3,Phase 1,Phase 2
29 Antihypertensive Agents Phase 4
30 Adrenergic beta-Antagonists Phase 4
31 Adrenergic Antagonists Phase 4
32 Neurotransmitter Agents Phase 4,Phase 3,Phase 1,Phase 2,Not Applicable
33 Vasodilator Agents Phase 4,Phase 2
34 Muscarinic Antagonists Phase 4
35 Cholinergic Agents Phase 4,Not Applicable
36 Serotonin Agents Phase 4
37 Cholinergic Antagonists Phase 4
38 Serotonin Uptake Inhibitors Phase 4
39 Neurotransmitter Uptake Inhibitors Phase 4
40 Analgesics, Non-Narcotic Phase 4,Phase 3
41 beta-Lactams Phase 4
42 Anti-Bacterial Agents Phase 4,Phase 3,Phase 1,Phase 2
43 Antipyretics Phase 4
44 Lactams Phase 4
45 Anti-Infective Agents Phase 4,Phase 3,Phase 1,Phase 2
46 Anesthetics, Intravenous Phase 4,Phase 3
47 Central Nervous System Depressants Phase 4,Phase 3,Not Applicable
48 Hypnotics and Sedatives Phase 4,Phase 3
49 Anesthetics, General Phase 4,Phase 3
Glycopyrrolate Phase 4 596-51-0 3494

Interventional clinical trials:

(show top 50) (show all 203)
# 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 The CSF Shunt Entry Site Trial Recruiting NCT02425761 Phase 3
28 Ultra-early Tranexamic Acid After Subarachnoid Hemorrhage. Recruiting NCT02684812 Phase 2, Phase 3 Tranexamic Acid
29 Leukotriene A4 Hydrolase Stratified Trial of Adjunctive Corticosteroids for HIV-uninfected Adults With Tuberculous Meningitis Recruiting NCT03100786 Phase 3 Dexamethasone
30 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
31 International Society of Paediatric Oncology (SIOP) PNET 5 Medulloblastoma Recruiting NCT02066220 Phase 2, Phase 3 Reduced-intensity maintenance chemotherapy;Maintenance chemotherapy
32 Randomized Controlled Trial of Shunt vs ETV/CPC for PIH in Ugandan Infants Active, not recruiting NCT01936272 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, Bone, Spinal Cord, Testes, Heart, Eye, Lung

Publications for Congenital Hydrocephalus

Articles related to Congenital Hydrocephalus:

(show top 50) (show all 938)
# Title Authors Year
Identifying infant hydrocephalus in the emergency department with transfontanellar POCUS. ( 30366745 )
Letter: Lumboperitoneal Shunts for the Treatment of Idiopathic Normal Pressure Hydrocephalus: A Comparison of Small-Lumen Abdominal Catheters to Gravitational Add-On Valves in a Single Center. ( 30395341 )
In Reply: Lumboperitoneal Shunts for the Treatment of Idiopathic Normal Pressure Hydrocephalus: A Comparison of Small-Lumen Abdominal Catheters to Gravitational Add-On Valves in a Single Center. ( 30476334 )
Cognitive disorders in normal pressure hydrocephalus with initial parkinsonism in comparison with de novo Parkinson's disease. ( 30091839 )
Spontaneous Ventriculostomy into the Subdural Space in a Neonate with Congenital Hydrocephalus. ( 29627843 )
Congenital Hydrocephalus: GA^mez-LA^pez-HernA!ndez syndrome. An underdiagnosed Syndrome. A clinical case. ( 29664509 )
Trends in the prevalence of congenital hydrocephalus in 14 cities in Liaoning province, China from 2006 to 2015 in a population-based birth defect registry from the Liaoning Women and Children's Health Hospital. ( 29581857 )
Prevalence and trend of isolated and complicated congenital hydrocephalus and preventive effect of folic acid in northern China, 2005-2015. ( 29388147 )
Bi-allelic mutations of CCDC88C are a rare cause of severe congenital hydrocephalus. ( 29341397 )
Acalvaria with external hydrocephalus: an uncommon case. ( 30073388 )
Atlantoaxial Subluxation Secondary to Unstable Os Odontoideum in a Patient With Arrested Hydrocephalus Due to Congenital Aqueductal Stenosis: A Case Report. ( 30364750 )
Clinical Features of and Risk Factors for Hydrocephalus in Childhood Bacterial Meningitis. ( 30373442 )
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 )
Monocyte depletion attenuates the development of posttraumatic hydrocephalus and preserves white matter integrity after traumatic brain injury. ( 30383765 )
Fetal gunshot brain injury leading to late postnatal hydrocephalus. ( 30149472 )
Preterm neuroimaging and neurodevelopmental outcome: a focus on intraventricular hemorrhage, post-hemorrhagic hydrocephalus, and associated brain injury. ( 30166622 )
Development of Posttraumatic Hydrocephalus Requiring Ventriculoperitoneal Shunt After Decompressive Craniectomy for Traumatic Brain Injury: a Systematic Review and Meta-analysis of Retrospective Studies. ( 30061770 )
Gulliver's world: Persistent lilliputian hallucinations as manifestation of Charles Bonnet syndrome in a case of cataract and normal pressure hydrocephalus. ( 30405266 )
Management of hydrocephalus and subdural hygromas in pediatric patients after decompression of Chiari malformation type I: case series and review of the literature. ( 30028271 )
Ventriculoperitoneal Shunts Equipped with On-Off Valves for Intraventricular Therapies in Patients with Communicating Hydrocephalus due to Leptomeningeal Metastases. ( 30110924 )
Ventricular Volume Dynamics During the Development of Adult Chronic Communicating Hydrocephalus in a Rodent Model. ( 30217783 )
Deep Brain Stimulation Surgery for Parkinson Disease Coexisting With Communicating Hydrocephalus: A Case Report. ( 30532732 )
Altered folate binding protein expression and folate delivery are associated with congenital hydrocephalus in the hydrocephalic Texas rat. ( 29798726 )
De Novo Mutation in Genes Regulating Neural Stem Cell Fate in Human Congenital Hydrocephalus. ( 29983323 )
Genetics Sheds New Light on Congenital Hydrocephalus Biology. ( 30048611 )
Consequences of Severe Epileptic Attack in a 3-year-old Girl with Congenital Hydrocephalus. ( 30166852 )
Human Genetics and Molecular Mechanisms of Congenital Hydrocephalus. ( 30205212 )
Comparative Study Between Laparoscopic and Open Techniques for Insertion of Ventriculperitoneal Shunt for Treatment of Congenital Hydrocephalus. ( 30307809 )
A Distinct Metabolite Profile Correlates with Neurodegenerative Conditions and the Severity of Congenital Hydrocephalus. ( 30364991 )
Congenital Hydrocephalus, Corpus Callosum Agenesis, and Prosencephalic Cyst with Supernumerary Nostril: A Neurocristopathy. ( 30459905 )
A novel ISLR2-linked autosomal recessive syndrome of congenital hydrocephalus, arthrogryposis and abdominal distension. ( 30483960 )
Novel KDM6A splice-site mutation in kabuki syndrome with congenital hydrocephalus: a case report. ( 30509212 )
Association of Severe Hydrocephalus With Congenital Zika Syndrome. ( 30452526 )
Cryptococcal meningitis in an immunocompetent patient with obstructive hydrocephalus: A case report. ( 30195720 )
Recurrent posterior reversible encephalopathy syndrome with cerebellar involvement leading to acute hydrocephalus. ( 29990959 )
Posterior reversible encephalopathy syndrome (PRES) due to neuroblastoma in a child presenting with acute hydrocephalus. ( 29079894 )
Cancer Dissemination, Hydrocephalus, and Survival After Cerebral Ventricular Entry During High-Grade Glioma Surgery: A Meta-Analysis. ( 29790976 )
Management of hydrocephalus in infants with severe hemophilia A: report of 2 cases. ( 30485223 )
Choroid Plexus Hyperplasia with Intractable Ascites and a Resulting Communicating Hydrocele following Shunt Operation for Hydrocephalus. ( 30157489 )
Combined effects of aquaporin-4 and hypoxia produce age-related hydrocephalus. ( 30293570 )
Parkinsonian Patient with Comorbid Normal Pressure Hydrocephalus : Clinical Usefulness of Diagnosis Supported by Neurological Findings. ( 30298345 )
Loss of perivascular aquaporin-4 in idiopathic normal pressure hydrocephalus. ( 30306658 )
Comparing the Risk of Shunt-Dependent Hydrocephalus in Patients with Ruptured Intracranial Aneurysms Treated by Endovascular Coiling or Surgical Clipping: An Updated Meta-Analysis. ( 30308338 )
Role of palliative CSF diversion in patients with intracranial metastatic disease and symptomatic hydrocephalus. ( 30317279 )
Metastatic craniocervical hemangiopericytoma presenting with hydrocephalus and papilledema - Case review. ( 30318074 )
Extended Combined Neonatal Treatment With Erythropoietin Plus Melatonin Prevents Posthemorrhagic Hydrocephalus of Prematurity in Rats. ( 30319361 )
Parent report on quality of life in post-haemorrhagic hydrocephalus. ( 30320432 )
Observations from Social Media Regarding the Symptomatology of Adult Hydrocephalus Patients. ( 30321673 )
Efficacy and safety of programmable compared with fixed anti-siphon devices for treating idiopathic normal-pressure hydrocephalus (iNPH) in adults - SYGRAVA: study protocol for a randomized trial. ( 30333067 )
Endoscopic third ventriculostomy and ventriculoperitoneal shunt for patients with noncommunicating hydrocephalus: A PRISMA-compliant meta-analysis. ( 30334940 )

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

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 9.5 FOXC1 FOXL1 FZD3 HYDIN L1CAM STK36
2 negative regulation of mitotic cell cycle GO:0045930 9.16 FOXC1 TNF
3 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 ExPASy
19 FMA
28 GO
29 GTR
32 HPO
33 ICD10
34 ICD10 via Orphanet
38 LifeMap
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
55 Novoseek
58 OMIM via Orphanet
62 PubMed
70 SNOMED-CT via Orphanet
72 Tocris
74 UMLS via Orphanet
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