Abnormal Development - Congenital Hydrocephalus
|Embryology - 18 Feb 2019 Expand to Translate|
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- 1 LA04 Congenital hydrocephalus
- 1.1 International Classification of Diseases (ICD10)
- 1.2 Introduction
- 1.3 Some Recent Findings
- 1.4 Aqueduct of Sylvius
- 1.5 Arnold-Chiari syndrome
- 1.6 Treatment
- 1.7 References
- 1.8 External Links
- 1.9 Glossary Links
LA04 Congenital hydrocephalus
A disease caused by failure of the brain to correctly develop during the antenatal period. This condition is characterized by a rapid increase in head circumference or an unusually large head size due to excessive accumulation of cerebrospinal fluid in the brain. This condition may also present with vomiting, sleepiness, irritability, downward deviation of the eyes, or seizures. Confirmation is through observation of cerebrospinal fluid within cerebral ventricles by imaging.
|International Classification of Diseases ICD-11 20 Developmental anomalies (beta draft)|
|ICD-11 Beta Draft - NOT FINAL, updated on a daily basis, It is not approved by WHO, NOT TO BE USED for CODING except for agreed FIELD TRIALS.
Chapter 20 Developmental anomalies, only a few examples of the draft ICD-11 Beta coding and tree structure for "structural developmental anomalies" within this section are shown in the table below.
|Mortality and Morbidity Statistics - 20 Developmental Anomalies|
|Structural Developmental Anomalies
|Multiple developmental anomalies or syndromes|
|Chromosomal anomalies, excluding gene mutations|
|Conditions with disorders of intellectual development as a relevant clinical feature|
|LD6Y Other specified developmental anomalies
LD6Z Developmental anomalies, unspecified
|CD-11 Beta Draft - NOT FINAL, updated on a daily basis, It is not approved by WHO, NOT TO BE USED for CODING except for agreed FIELD TRIALS.|
International Classification of Diseases (ICD10)
ICD10 - Q03 Congenital hydrocephalus Incl.: hydrocephalus in newborn Excl.: Arnold-Chiari syndrome (Q07.0) hydrocephalus: acquired (G91.-) due to congenital toxoplasmosis (P37.1) with spina bifida (Q05.0-Q05.4)
- Q03.0 Malformations of aqueduct of Sylvius Aqueduct of Sylvius: anomaly obstruction, congenital stenosis
- Q03.1 Atresia of foramina of Magendie and Luschka Dandy-Walker syndrome
- Q03.8 Other congenital hydrocephalus
- Q03.9 Congenital hydrocephalus, unspecified
This is a defect of cerebrospinal fliud (CSF) flow, excess fluid production or impaired fluid absorption and can be congenital or acquired. Estimated incidence of 1 in 1000 live births the condition leads to enlarged ventricles and head, separated skull cranial sutures and fontanelles. Obstruction of CSF flow can occur at any time (prenatally or postnatally) and leads to accumulation of within the ventricles. The time of onset will have different effects and should be compared to the equilivant neurological events that are occuring.
Ventricular obstruction usually occurs at the level of the cerebral aqueduct (narrowest site), but can occur elsewhere, and can be caused by viral infection or zoonotic disease.
Some Recent Findings
|More recent papers|
This table allows an automated computer search of the external PubMed database using the listed "Search term" text link.
<pubmed limit=5>Congenital Hydrocephalus</pubmed>
Aqueduct of Sylvius
Progressive obliteration of the aqueduct of Sylvius in hydrocephalus
ICD - Q07 Other congenital malformations of nervous system - Q07.0 Arnold-Chiari syndrome
Magnetic Resonance Image Sagittal T1-weighted image showing Chiari II malformation.
Ventriculoperitoneal (VP) shunting is a surgical procedure that commenced in the 1960's to treat hydrocephalus and relieve increased pressure inside the skull due to excess cerebrospinal fluid (CSF) on the brain. A catheter is placed inside of the brain ventricle, the catheter is then tunneled under the skin from the scalp down into the abdominal cavity, where the other end opens into the peritoneal cavity. The catheter contains a valve (located in the skin behind the ear) that opens when pressure builds up around the brain. Complication can include: infections (8-10%), catheter blockage, over-drainage and movement of the catheter.
- Links: Medline Plus article
An endoscopic surgical technique where a small perforation is made in the thinned floor of the third ventricle, allowing cerebrospinal fluid (CSF) out of the blocked ventricular system and into the interpeduncular cistern 9 (Cisterna interpeduncularis) that is also a normal CSF space. This was an early surgical technique, reported as early as 1922, that has revived with modern magnetic resonance imaging (MRI) and surgical endoscopic techniques that result in less damage. Due to the location of the surgery complications can include: bleeding, endocrine disfunction, and diabetes insipidus.
- Bokhari R & Baeesa S. (2012). Remote cerebellar hemorrhage due to ventriculoperitoneal shunt in an infant: a case report. J Med Case Rep , 6, 222. PMID: 22846583 DOI.
- Krishna Pillai M, Kariyattil R, Rubene L, Kumar R, Govindaraju V & Kochummen K. (2018). Spontaneous Ventriculostomy into the Subdural Space in a Neonate with Congenital Hydrocephalus. Pediatr Neurosurg , , . PMID: 29627843 DOI.
- Yamasaki M, Nonaka M, Bamba Y, Teramoto C, Ban C & Pooh RK. (2012). Diagnosis, treatment, and long-term outcomes of fetal hydrocephalus. Semin Fetal Neonatal Med , 17, 330-5. PMID: 23089488 DOI.
- Ortega E, Muñoz RI, Luza N, Guerra F, Guerra M, Vio K, Henzi R, Jaque J, Rodriguez S, McAllister JP & Rodriguez E. (2016). The value of early and comprehensive diagnoses in a human fetus with hydrocephalus and progressive obliteration of the aqueduct of Sylvius: Case Report. BMC Neurol , 16, 45. PMID: 27067115 DOI.
- Elgamal EA. (2012). Natural history of hydrocephalus in children with spinal open neural tube defect. Surg Neurol Int , 3, 112. PMID: 23087828 DOI.
- Whiley SP, Mantokoudis G, Ott D, Zimmerman H & Exadaktylos AK. (2012). A review of full-body radiography in nontraumatic emergency medicine. Emerg Med Int , 2012, 108129. PMID: 23243508 DOI.
Search Bookshelf: Congenital Hydrocephalus
10 Most Recent - Congenital Hydrocephalus
Note - This sub-heading shows an automated computer PubMed search using the listed sub-heading term. References appear in this list based upon the date of the actual page viewing. Therefore the list of references do not reflect any editorial selection of material based on content or relevance. In comparison, references listed on the content page and discussion page (under the publication year sub-headings) do include editorial selection based upon relevance and availability. (More? Pubmed Most Recent)
<pubmed limit=10>Congenital Hydrocephalus</pubmed>
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Cite this page: Hill, M.A. (2019, February 18) Embryology Abnormal Development - Congenital Hydrocephalus. Retrieved from https://embryology.med.unsw.edu.au/embryology/index.php/Abnormal_Development_-_Congenital_Hydrocephalus
- © Dr Mark Hill 2019, UNSW Embryology ISBN: 978 0 7334 2609 4 - UNSW CRICOS Provider Code No. 00098G