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Hydrocephalus, congenital communicating, 1(HYDCC1; HYC4)

MedGen UID:
1684770
•Concept ID:
C5231454
•
Disease or Syndrome
Synonym: HYDROCEPHALUS, CONGENITAL, 4
 
Gene (location): TRIM71 (3p22.3)
 
Monarch Initiative: MONDO:0032862
OMIM®: 618667

Clinical features

From HPO
Communicating hydrocephalus
MedGen UID:
1058
•Concept ID:
C0009451
•
Disease or Syndrome
A form of hydrocephalus in which there is no visible obstruction to the flow of the cerebrospinal fluid between the ventricles and subarachnoid space.
See: Feature record | Search on this feature
Seizure
MedGen UID:
20693
•Concept ID:
C0036572
•
Sign or Symptom
A seizure is an intermittent abnormality of nervous system physiology characterized by a transient occurrence of signs and/or symptoms due to abnormal excessive or synchronous neuronal activity in the brain.
See: Feature record | Search on this feature
Ventriculomegaly
MedGen UID:
480553
•Concept ID:
C3278923
•
Finding
An increase in size of the ventricular system of the brain.
See: Feature record | Search on this feature
Neurodevelopmental delay
MedGen UID:
868344
•Concept ID:
C4022738
•
Finding
Neurodevelopmental delay (NDD) refers to delays in the maturation of the brain and central nervous system; infants and young children with NDD may experience delays in the development of one or more skills including gross motor abilities, fine-motor coordination, language abilities and ability to solve increasingly complex problems.
See: Feature record | Search on this feature

Professional guidelines

PubMed

Surgery or Endovascular Treatment in Patients with Anterior Communicating Artery Aneurysm: A Systematic Review and Meta-Analysis.
Sattari SA, Shahbandi A, Lee RP, Feghali J, Rincon-Torroella J, Yang W, Abdulrahim M, Ahmadi S, So RJ, Hung A, Caplan JM, Gonzalez F, Tamargo RJ, Huang J, Xu R
World Neurosurg 2023 Jul;175:31-44. Epub 2023 Apr 1 doi: 10.1016/j.wneu.2023.03.111. PMID: 37011760
Management of posthaemorrhagic ventricular dilatation.
Whitelaw A, Aquilina K
Arch Dis Child Fetal Neonatal Ed 2012 May;97(3):F229-3. Epub 2011 Feb 2 doi: 10.1136/adc.2010.190173. PMID: 21289015
Surgical management of adult hydrocephalus.
Bergsneider M, Miller C, Vespa PM, Hu X
Neurosurgery 2008 Feb;62 Suppl 2:643-59; discussion 659-60. doi: 10.1227/01.neu.0000316269.82467.f7. PMID: 18596440

Recent clinical studies

Etiology

Surgery or Endovascular Treatment in Patients with Anterior Communicating Artery Aneurysm: A Systematic Review and Meta-Analysis.
Sattari SA, Shahbandi A, Lee RP, Feghali J, Rincon-Torroella J, Yang W, Abdulrahim M, Ahmadi S, So RJ, Hung A, Caplan JM, Gonzalez F, Tamargo RJ, Huang J, Xu R
World Neurosurg 2023 Jul;175:31-44. Epub 2023 Apr 1 doi: 10.1016/j.wneu.2023.03.111. PMID: 37011760
Loculated hydrocephalus: is neuroendoscopy effective and safe? A 90 patients' case series and literature review.
Noris A, Giordano F, Peraio S, Lenge M, Mura R, Macconi L, Barzaghi R, Genitori L
Childs Nerv Syst 2023 Mar;39(3):711-720. Epub 2022 Nov 29 doi: 10.1007/s00381-022-05747-6. PMID: 36443473Free PMC Article
Communicating Hydrocephalus Following Treatment of Cerebellopontine Angle Tumors.
Kim J, Woo B, Ji S, Hwang K, Kim YH, Han JH, Kim CY
World Neurosurg 2022 Sep;165:e505-e511. Epub 2022 Jun 26 doi: 10.1016/j.wneu.2022.06.088. PMID: 35760327
New concept of the pathogenesis and therapeutic orientation of acquired communicating hydrocephalus.
Xu H
Neurol Sci 2016 Sep;37(9):1387-91. Epub 2016 Apr 26 doi: 10.1007/s10072-016-2589-7. PMID: 27115894
Multiloculated hydrocephalus.
Zuccaro G, Ramos JG
Childs Nerv Syst 2011 Oct;27(10):1609-19. Epub 2011 Sep 17 doi: 10.1007/s00381-011-1528-4. PMID: 21928027

Diagnosis

Idiopathic normal pressure hydrocephalus: historical context and a contemporary guide.
Carswell C
Pract Neurol 2023 Feb;23(1):15-22. Epub 2022 Sep 26 doi: 10.1136/pn-2021-003291. PMID: 36162853
Communicating chronic hydrocephalus: A review.
Roblot P, Mollier O, Ollivier M, Gallice T, Planchon C, Gimbert E, Danet M, Renault S, Auzou N, Laurens B, Jecko V
Rev Med Interne 2021 Nov;42(11):781-788. Epub 2021 Jun 16 doi: 10.1016/j.revmed.2021.05.018. PMID: 34144842
Defining a Taxonomy of Intracranial Hypertension: Is ICP More Than Just a Number?
Kofke WA, Rajagopalan S, Ayubcha D, Balu R, Cruz-Navarro J, Manatpon P, Mahanna-Gabrielli E
J Neurosurg Anesthesiol 2020 Apr;32(2):120-131. doi: 10.1097/ANA.0000000000000609. PMID: 31135572Free PMC Article
Multiloculated hydrocephalus.
Zuccaro G, Ramos JG
Childs Nerv Syst 2011 Oct;27(10):1609-19. Epub 2011 Sep 17 doi: 10.1007/s00381-011-1528-4. PMID: 21928027
Mucopolysaccharidosis VI.
Valayannopoulos V, Nicely H, Harmatz P, Turbeville S
Orphanet J Rare Dis 2010 Apr 12;5:5. doi: 10.1186/1750-1172-5-5. PMID: 20385007Free PMC Article

Therapy

Surgery or Endovascular Treatment in Patients with Anterior Communicating Artery Aneurysm: A Systematic Review and Meta-Analysis.
Sattari SA, Shahbandi A, Lee RP, Feghali J, Rincon-Torroella J, Yang W, Abdulrahim M, Ahmadi S, So RJ, Hung A, Caplan JM, Gonzalez F, Tamargo RJ, Huang J, Xu R
World Neurosurg 2023 Jul;175:31-44. Epub 2023 Apr 1 doi: 10.1016/j.wneu.2023.03.111. PMID: 37011760
Communicating Hydrocephalus Following Treatment of Cerebellopontine Angle Tumors.
Kim J, Woo B, Ji S, Hwang K, Kim YH, Han JH, Kim CY
World Neurosurg 2022 Sep;165:e505-e511. Epub 2022 Jun 26 doi: 10.1016/j.wneu.2022.06.088. PMID: 35760327
Management of posthaemorrhagic ventricular dilatation.
Whitelaw A, Aquilina K
Arch Dis Child Fetal Neonatal Ed 2012 May;97(3):F229-3. Epub 2011 Feb 2 doi: 10.1136/adc.2010.190173. PMID: 21289015
Surgical management of adult hydrocephalus.
Bergsneider M, Miller C, Vespa PM, Hu X
Neurosurgery 2008 Feb;62 Suppl 2:643-59; discussion 659-60. doi: 10.1227/01.neu.0000316269.82467.f7. PMID: 18596440
Adult communicating hydrocephalus.
Pickard JD
Br J Hosp Med 1982 Jan;27(1):35, 37-8, 40, passim. PMID: 7037092

Prognosis

Hydrocephalus in Neurosarcoidosis: Clinical Course, Radiographic Accompaniments, and Experience with Shunting.
El Sammak S, Lec BM, Bou GA, Wagstaff WV, Lawson EC, Hutto SK
Mult Scler Relat Disord 2023 Nov;79:105040. Epub 2023 Sep 28 doi: 10.1016/j.msard.2023.105040. PMID: 37783195
Idiopathic normal pressure hydrocephalus: historical context and a contemporary guide.
Carswell C
Pract Neurol 2023 Feb;23(1):15-22. Epub 2022 Sep 26 doi: 10.1136/pn-2021-003291. PMID: 36162853
Hydrocephalus Associated with Posterior Fossa Tumors: How to Manage Effectively?
Muthukumar N
Neurol India 2021 Nov-Dec;69(Supplement):S342-S349. doi: 10.4103/0028-3886.332260. PMID: 35102986
Magnetic Resonance Imaging of Normal Pressure Hydrocephalus.
Bradley WG Jr
Semin Ultrasound CT MR 2016 Apr;37(2):120-8. Epub 2016 Jan 20 doi: 10.1053/j.sult.2016.01.005. PMID: 27063662
Mucopolysaccharidosis VI.
Valayannopoulos V, Nicely H, Harmatz P, Turbeville S
Orphanet J Rare Dis 2010 Apr 12;5:5. doi: 10.1186/1750-1172-5-5. PMID: 20385007Free PMC Article

Clinical prediction guides

Medical, Neurologic, and Neuromusculoskeletal Complications.
Woo JE, Azariah A, Reed EA, Gut N
Phys Med Rehabil Clin N Am 2024 Feb;35(1):127-144. Epub 2023 Jul 27 doi: 10.1016/j.pmr.2023.06.024. PMID: 37993183
Hydrocephalus in Neurosarcoidosis: Clinical Course, Radiographic Accompaniments, and Experience with Shunting.
El Sammak S, Lec BM, Bou GA, Wagstaff WV, Lawson EC, Hutto SK
Mult Scler Relat Disord 2023 Nov;79:105040. Epub 2023 Sep 28 doi: 10.1016/j.msard.2023.105040. PMID: 37783195
Idiopathic normal pressure hydrocephalus: historical context and a contemporary guide.
Carswell C
Pract Neurol 2023 Feb;23(1):15-22. Epub 2022 Sep 26 doi: 10.1136/pn-2021-003291. PMID: 36162853
Hydrocephalus Associated with Posterior Fossa Tumors: How to Manage Effectively?
Muthukumar N
Neurol India 2021 Nov-Dec;69(Supplement):S342-S349. doi: 10.4103/0028-3886.332260. PMID: 35102986
Endoscopic third ventriculostomy and cerebrospinal fluid shunting for pure communicating hydrocephalus in children.
Usami K, Ishisaka E, Ogiwara H
Childs Nerv Syst 2021 Sep;37(9):2813-2819. Epub 2021 Jun 8 doi: 10.1007/s00381-021-05242-4. PMID: 34100098

Recent systematic reviews

Surgery or Endovascular Treatment in Patients with Anterior Communicating Artery Aneurysm: A Systematic Review and Meta-Analysis.
Sattari SA, Shahbandi A, Lee RP, Feghali J, Rincon-Torroella J, Yang W, Abdulrahim M, Ahmadi S, So RJ, Hung A, Caplan JM, Gonzalez F, Tamargo RJ, Huang J, Xu R
World Neurosurg 2023 Jul;175:31-44. Epub 2023 Apr 1 doi: 10.1016/j.wneu.2023.03.111. PMID: 37011760
Communicating hydrocephalus after radiosurgery for vestibular schwannomas: does technique matter? A systematic review and meta-analysis.
De Sanctis P, Green S, Germano I
J Neurooncol 2019 Nov;145(2):365-373. Epub 2019 Oct 16 doi: 10.1007/s11060-019-03305-w. PMID: 31621039
Predictors of Shunt-dependent Hydrocephalus After Aneurysmal Subarachnoid Hemorrhage? A Systematic Review and Meta-Analysis.
Xie Z, Hu X, Zan X, Lin S, Li H, You C
World Neurosurg 2017 Oct;106:844-860.e6. Epub 2017 Jun 23 doi: 10.1016/j.wneu.2017.06.119. PMID: 28652120
Is clipping better than coiling in the treatment of patients with oculomotor nerve palsies induced by posterior communicating artery aneurysms? A systematic review and meta-analysis.
Zheng F, Dong Y, Xia P, Mpotsaris A, Stavrinou P, Brinker G, Goldbrunner R, Krischek B
Clin Neurol Neurosurg 2017 Feb;153:20-26. Epub 2016 Dec 11 doi: 10.1016/j.clineuro.2016.11.022. PMID: 28006728
Is endoscopic third ventriculostomy superior to shunts in patients with non-communicating hydrocephalus? A systematic review and meta-analysis of the evidence.
Rasul FT, Marcus HJ, Toma AK, Thorne L, Watkins LD
Acta Neurochir (Wien) 2013 May;155(5):883-9. Epub 2013 Mar 3 doi: 10.1007/s00701-013-1657-5. PMID: 23456239

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