U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Noncommunicating hydrocephalus

MedGen UID:
108198
Concept ID:
C0549423
Disease or Syndrome
Synonyms: Hydrocephalus, Obstructive; Obstructive Hydrocephalus
SNOMED CT: Non-communicating hydrocephalus (82346000); Obstructive hydrocephalus (230746009)
 
HPO: HP:0010953
Monarch Initiative: MONDO:0001896

Definition

A form of hydrocephalus in which the flow of cerebrospinal fluid (CSF) within the cerebral ventricular system or in the outlets of the CSF to the arachnoid space is obstructed. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVNoncommunicating hydrocephalus

Conditions with this feature

Ciliary dyskinesia, primary, 43
MedGen UID:
1684675
Concept ID:
C5231466
Disease or Syndrome
Primary ciliary dyskinesia-43 (CILD43) is a disorder characterized by a defect in motile cilia and ciliary clearance resulting in the onset of respiratory insufficiency soon after birth, and associated with recurrent upper and lower respiratory infections with chronic progressive lung disease. Patients with this disorder also develop significant obstructive hydrocephalus requiring shunting in infancy, although adult onset of neurologic symptoms may occur. Other more variable features include infertility and about a 50% chance of situs inversus or other left-right asymmetry defects. The disorder is considered to be a type of ciliopathy known as 'reduced generation of multiple motile cilia' (RGMC) (summary by Wallmeier et al., 2019). For a discussion of genetic heterogeneity of primary ciliary dyskinesia, CILD1 (244400).
Coffin-Siris syndrome 12
MedGen UID:
1782096
Concept ID:
C5444111
Disease or Syndrome
Coffin-Siris syndrome-12 (CSS12) is a neurodevelopmental disorder characterized by global developmental delay with variably impaired intellectual development, speech and language delay, and behavioral abnormalities, such as autism or hyperactivity. Affected individuals may have hypotonia and poor feeding in infancy. There are variable dysmorphic facial features, although most patients do not have the classic hypoplastic fifth digit/nail abnormalities that are often observed in other forms of CSS (Barish et al., 2020). For a general phenotypic description and a discussion of genetic heterogeneity of Coffin-Siris syndrome, see CSS1 (135900).
Intellectual developmental disorder, autosomal dominant 65
MedGen UID:
1787923
Concept ID:
C5543371
Disease or Syndrome
Autosomal dominant intellectual developmental disorder-65 (MRD65) is characterized by delayed motor and speech acquisition, variably impaired intellectual development, and behavioral abnormalities. Affected individuals also have dysmorphic facial features. Brain imaging may be normal or may show abnormalities, including cerebellar hypoplasia, poor development of the corpus callosum, dysmorphic hippocampus, and polymicrogyria. Feeding difficulties, hypotonia, and seizures may also be observed (Duncan et al., 2020).
Hydrocephalus, congenital, 5, susceptibility to
MedGen UID:
1840908
Concept ID:
C5830272
Finding
Congenital hydrocephalus-5 (HYC5) is an autosomal dominant condition characterized by hydrocephalus associated with aqueductal stenosis apparent from birth. Some patients may have neurodevelopmental delay, seizures, or structural brain abnormalities (Furey et al., 2018). For a discussion of genetic heterogeneity of congenital hydrocephalus, see 233600.

Professional guidelines

PubMed

Park YS
Neurol Med Chir (Tokyo) 2022 Sep 15;62(9):416-430. Epub 2022 Aug 27 doi: 10.2176/jns-nmc.2022-0100. PMID: 36031350Free PMC Article
Oertel JM, Mondorf Y, Schroeder HW, Gaab MR
Acta Neurochir (Wien) 2010 Feb;152(2):229-40. Epub 2009 Aug 26 doi: 10.1007/s00701-009-0494-z. PMID: 19707715
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

Linares Torres J, Ros López B, Iglesias Moroño S, Ros Sanjuán Á, Selfa Rodríguez A, Cerro Larrazábal L, Casado Ruiz J, Arráez Sánchez MÁ
Neurocirugia (Astur : Engl Ed) 2022 May-Jun;33(3):111-119. Epub 2021 Apr 9 doi: 10.1016/j.neucie.2021.04.002. PMID: 35526943
Krejčí O, Krejčí T, Mrůzek M, Večeřa Z, Šalounová D, Lipina R
World Neurosurg 2021 Apr;148:e425-e435. Epub 2021 Jan 12 doi: 10.1016/j.wneu.2021.01.008. PMID: 33444837
Spennato P, Ruggiero C, Aliberti F, Nastro A, Mirone G, Cinalli G
World Neurosurg 2013 Feb;79(2 Suppl):S22.e21-6. Epub 2012 Feb 10 doi: 10.1016/j.wneu.2012.02.005. PMID: 22381847
Erşahin Y
Childs Nerv Syst 2007 Feb;23(2):143-50. Epub 2006 Oct 13 doi: 10.1007/s00381-006-0227-z. PMID: 17053938
Jones RF, Stening WA, Brydon M
Neurosurgery 1990 Jan;26(1):86-91; discussion 91-2. doi: 10.1097/00006123-199001000-00012. PMID: 2294483

Diagnosis

Maller VV, Gray RI
Semin Ultrasound CT MR 2016 Apr;37(2):109-19. Epub 2015 Dec 17 doi: 10.1053/j.sult.2015.12.004. PMID: 27063661
Agarwal A, Bathla G, Kanekar S
Semin Ultrasound CT MR 2016 Apr;37(2):100-8. Epub 2016 Feb 23 doi: 10.1053/j.sult.2016.02.007. PMID: 27063660
Emery SP, Greene S, Hogge WA
Fetal Diagn Ther 2015;38(2):81-5. Epub 2015 May 13 doi: 10.1159/000382015. PMID: 25997519
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
Erşahin Y
Childs Nerv Syst 2007 Feb;23(2):143-50. Epub 2006 Oct 13 doi: 10.1007/s00381-006-0227-z. PMID: 17053938

Therapy

Park YS
Neurol Med Chir (Tokyo) 2022 Sep 15;62(9):416-430. Epub 2022 Aug 27 doi: 10.2176/jns-nmc.2022-0100. PMID: 36031350Free PMC Article
Jiang L, Gao G, Zhou Y
Medicine (Baltimore) 2018 Oct;97(42):e12139. doi: 10.1097/MD.0000000000012139. PMID: 30334940Free PMC Article
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
Erşahin Y
Childs Nerv Syst 2007 Feb;23(2):143-50. Epub 2006 Oct 13 doi: 10.1007/s00381-006-0227-z. PMID: 17053938
Rush JL, Foltz EL
J Neurosurg 1977 Mar;46(3):385-90. doi: 10.3171/jns.1977.46.3.0385. PMID: 839265

Prognosis

Park YS
Neurol Med Chir (Tokyo) 2022 Sep 15;62(9):416-430. Epub 2022 Aug 27 doi: 10.2176/jns-nmc.2022-0100. PMID: 36031350Free PMC Article
Linares Torres J, Ros López B, Iglesias Moroño S, Ros Sanjuán Á, Selfa Rodríguez A, Cerro Larrazábal L, Casado Ruiz J, Arráez Sánchez MÁ
Neurocirugia (Astur : Engl Ed) 2022 May-Jun;33(3):111-119. Epub 2021 Apr 9 doi: 10.1016/j.neucie.2021.04.002. PMID: 35526943
Jiang L, Gao G, Zhou Y
Medicine (Baltimore) 2018 Oct;97(42):e12139. doi: 10.1097/MD.0000000000012139. PMID: 30334940Free PMC Article
Maller VV, Gray RI
Semin Ultrasound CT MR 2016 Apr;37(2):109-19. Epub 2015 Dec 17 doi: 10.1053/j.sult.2015.12.004. PMID: 27063661
Agarwal A, Bathla G, Kanekar S
Semin Ultrasound CT MR 2016 Apr;37(2):100-8. Epub 2016 Feb 23 doi: 10.1053/j.sult.2016.02.007. PMID: 27063660

Clinical prediction guides

Linares Torres J, Ros López B, Iglesias Moroño S, Ros Sanjuán Á, Selfa Rodríguez A, Cerro Larrazábal L, Casado Ruiz J, Arráez Sánchez MÁ
Neurocirugia (Astur : Engl Ed) 2022 May-Jun;33(3):111-119. Epub 2021 Apr 9 doi: 10.1016/j.neucie.2021.04.002. PMID: 35526943
Balédent O, Capel C, Metanbou S, Bouzerar R
Acta Neurochir Suppl 2021;131:303-306. doi: 10.1007/978-3-030-59436-7_56. PMID: 33839862
Jiang L, Gao G, Zhou Y
Medicine (Baltimore) 2018 Oct;97(42):e12139. doi: 10.1097/MD.0000000000012139. PMID: 30334940Free PMC Article
Castro BA, Imber BS, Chen R, McDermott MW, Aghi MK
Neurosurgery 2017 Mar 1;80(3):421-430. doi: 10.1227/NEU.0000000000001263. PMID: 27218235Free PMC Article
Kasarskis EJ, Tibbs PA, Lee C
Neurosurgery 1988 Apr;22(4):770-2. doi: 10.1227/00006123-198804000-00029. PMID: 3287215

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Consumer resources

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...