Abnormal Development - Congenital Hydrocephalus: Difference between revisions
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:'''Links:''' [[International_Classification_of_Diseases_-_XVII_Congenital_Malformations|XVII Congenital Malformations]] | [[International Classification of Diseases]] | :'''Links:''' [[International_Classification_of_Diseases_-_XVII_Congenital_Malformations|XVII Congenital Malformations]] | [[International Classification of Diseases]] | ||
==Introduction== | ==Introduction== | ||
[[File:Congenital_hydrocephalus_MRI01.jpg|thumb|MRI Congenital hydrocephalus. | [[File:Congenital_hydrocephalus_MRI01.jpg|thumb|MRI Congenital hydrocephalus.{{#pmid:22846583|PMID22846583}}]] | ||
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. | 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. | 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. | ||
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* '''Diagnosis, treatment, and long-term outcomes of fetal hydrocephalus''' | * '''Diagnosis, treatment, and long-term outcomes of fetal hydrocephalus'''{{#pmid:23089488|PMID23089488}} "This study analyzed 156 cases of fetal hydrocephalus treated at Osaka National Hospital from 1992 to 2011 to review current methods for diagnosing and treating fetal hydrocephalus, and for estimating its clinical outcome. This was a retrospective study of a single institute (Osaka National Hospital). Of 156 cases in total, 37% were diagnosed as isolated ventriculomegaly, 50% as another type of malformation (36 cases of myelomeningocele, six of holoprosencephaly, three of Dandy-Walker syndrome, one case of Joubert syndrome, 12 of arachnoid cyst, nine of encephalocele, three of atresia of Monro and eight of corpus callosum agenesis, and 13% as secondary hydrocephalus. Diagnoses were made between 13 and 40 weeks of gestation (average 27 weeks). Diagnosis was made before 21 weeks of gestation in 24% of cases, from the first day of 22 weeks to the sixth day of 27 weeks in 27%, and after the first day of 28 weeks in 49%. With the exclusion of 17 aborted cases and 40 cases in which the patients were too young to evaluate or lost during follow-up, the final outcome was analyzed for 90 cases. Of these, 17% of the patients died, 21% showed severe retardation, 13% moderate retardation, 26% mild retardation, and 23% showed a good outcome. The long-term outcome was mostly influenced by the basic disease and accompanying anomaly. The time of diagnosis showed no correlation with outcome. Hydrocephalus associated with arachnoid cyst, atresia of Monro, and corpus callosum agenesis, and hydrocephalus due to fetal intracranial hemorrhage, resulted in good outcomes. By contrast, holoprosencephaly, hydrocephalus associated with encephalocele, syndromic hydrocephalus, and hydrocephalus due to fetal virus infection led to poor outcomes. For accurate diagnosis and proper counseling, established protocols are important for the diagnosis and treatment of fetal hydrocephalus, including not only fetal sonography, fetal magnetic resonance imaging, and TORCH (toxoplasma, rubella, cytomegalovirus, herpes simplex) screening test, but also chromosomal and gene testing." | ||
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! More recent papers | ! More recent papers | ||
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| [[File:Mark_Hill.jpg|90px|left]] {{Most_Recent_Refs}} | | [[File:Mark_Hill.jpg|90px|left]] {{Most_Recent_Refs}} | ||
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| [[File:Hydrocephalus aqueduct of Sylvius 01.jpg|600px]] | | [[File:Hydrocephalus aqueduct of Sylvius 01.jpg|600px]] | ||
Progressive obliteration of the aqueduct of Sylvius in hydrocephalus | Progressive obliteration of the aqueduct of Sylvius in hydrocephalus{{#pmid:27067115|PMID27067115}} | ||
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* '''a''' Ultrasound of fetal patient at 36 GW showing dilation of the lateral ventricles (arrows). | * '''a''' Ultrasound of fetal patient at 36 GW showing dilation of the lateral ventricles (arrows). | ||
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[[File:Chiari II malformation MRI02.jpg|400px]] | [[File:Chiari II malformation MRI02.jpg|400px]] | ||
Magnetic Resonance Image Sagittal T1-weighted image showing Chiari II malformation. | Magnetic Resonance Image Sagittal T1-weighted image showing Chiari II malformation.{{#pmid:23087828|PMID23087828}} | ||
==Treatment== | ==Treatment== | ||
===Ventriculoperitoneal Shunt=== | ===Ventriculoperitoneal Shunt=== | ||
[[File:Ventriculoperitoneal shunt 01.jpg|thumb|alt=Ventriculoperitoneal shunt|Ventriculoperitoneal shunt | [[File:Ventriculoperitoneal shunt 01.jpg|thumb|alt=Ventriculoperitoneal shunt|Ventriculoperitoneal shunt{{#pmid:23243508|PMID23243508}}]] | ||
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. | 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. | ||
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===Reviews=== | ===Reviews=== | ||
{{#pmid:22069421}} | |||
===Search PubMed=== | ===Search PubMed=== |
Revision as of 12:04, 11 April 2018
Embryology - 19 Apr 2024 Expand to Translate |
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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) |
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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
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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
Introduction
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
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More recent papers |
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This table allows an automated computer search of the external PubMed database using the listed "Search term" text link.
More? References | Discussion Page | Journal Searches | 2019 References | 2020 References Search term: Congenital Hydrocephalus <pubmed limit=5>Congenital Hydrocephalus</pubmed> |
Aqueduct of Sylvius
Progressive obliteration of the aqueduct of Sylvius in hydrocephalus[3] |
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Arnold-Chiari syndrome
ICD - Q07 Other congenital malformations of nervous system - Q07.0 Arnold-Chiari syndrome
Magnetic Resonance Image Sagittal T1-weighted image showing Chiari II malformation.[4]
Treatment
Ventriculoperitoneal Shunt
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
Third Ventriculostomy
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.
References
- ↑ 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.
- ↑ 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.
Reviews
Venkataramana NK. (2011). Hydrocephalus Indian scenario - A review. J Pediatr Neurosci , 6, S11-22. PMID: 22069421 DOI.
Search PubMed
Search Bookshelf: Congenital Hydrocephalus
Search Pubmed: Congenital Hydrocephalus | Ventriculoperitoneal Shunt
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. (2024, April 19) Embryology Abnormal Development - Congenital Hydrocephalus. Retrieved from https://embryology.med.unsw.edu.au/embryology/index.php/Abnormal_Development_-_Congenital_Hydrocephalus
- © Dr Mark Hill 2024, UNSW Embryology ISBN: 978 0 7334 2609 4 - UNSW CRICOS Provider Code No. 00098G