Talk:Abnormal Development - Congenital Hydrocephalus

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Cite this page: Hill, M.A. (2021, April 13) Embryology Abnormal Development - Congenital Hydrocephalus. Retrieved from


Removed - 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
Links: XVII Congenital Malformations | International Classification of Diseases

PMC Images - Ventriculoperitoneal Shunt


0.1T magnetic resonance image in the study of experimental hydrocephalus in rats. Accuracy of the method in the measurements of the ventricular size

Acta Cir Bras. 2012 Nov;27(11):768-72.

Castro SC, Machado HR, Catalão CH, Siqueira BA, Simões AL, Lachat JJ, Lopes Lda S. Source Department of Surgery and Anatomy, School of Medicine of Ribeirao Preto, USP.


PURPOSE: To investigate the accuracy of 1.0T Magnetic Resonance Imaging (MRI) to measure the ventricular size in experimental hydrocephalus in pup rats. METHODS: Wistar rats were subjected to hydrocephalus by intracisternal injection of 20% kaolin (n=13). Ten rats remained uninjected to be used as controls. At the endpoint of experiment animals were submitted to MRI of brain and killed. The ventricular size was assessed using three measures: ventricular ratio (VR), the cortical thickness (Cx) and the ventricles area (VA), performed on photographs of anatomical sections and MRI. RESULTS: The images obtained through MR present enough quality to show the lateral ventricular cavities but not to demonstrate the difference between the cortex and the white matter, as well as the details of the deep structures of the brain. There were no statistically differences between the measures on anatomical sections and MRI of VR and Cx (p=0.9946 and p=0.5992, respectively). There was difference between VA measured on anatomical sections and MRI (p<0.0001). CONCLUSION: The parameters obtained through 1.0T MRI were sufficient in quality to individualize the ventricular cavities and the cerebral cortex, and to calculate the ventricular ratio in hydrocephalus rats when compared to their respective anatomic slice.

PMID 23117608

Natural history of hydrocephalus in children with spinal open neural tube defect

Surg Neurol Int. 2012;3:112. doi: 10.4103/2152-7806.101801. Epub 2012 Sep 28.

Elgamal EA. Source Neurosurgery Division, King Khalid University Hospital, King Saud University, Riyadh, Saudi Arabia.


BACKGROUND: The long-term prognosis of patients with Spinal Open Neural Tube Defect (SONTD)-associated hydrocephalus is not well known. This study was conducted to ascertain the incidence and natural history of hydrocephalus in patients with SONTD. METHODS: All 82 patients with SONTD referred to Neurosurgery/Spina Bifida Clinics at King Khalid University Hospital, Riyadh, Saudi Arabia (January 1995 - July 2010) were studied and followed for a period of 1-16 years. Patients were divided into three groups: Group "A" with active hydrocephalus treated with ventriculoperitoneal shunt (VPS), or endoscopic third ventriculostomy (ETV); Group "B" with compensated hydrocephalus; and Group "C" with no hydrocephalus. Timing of shunt insertion, complications of treatment and status of hydrocephalus were analyzed. RESULTS: The mean age of the 82 patients was 7.4 years (range 1-16 years). Group "A" included 59 (72%) patients, Group "B" 7 (8.5%) patients, and Group "C" 16 (19.5%) patients. Chiari malformation type II was found in 71 (86.6%) patients, 57 of whom (80%) were in Group "A" with active hydrocephalus. They were treated by VPS (51 patients) and ETV (8 patients). The shunts were revised or replaced in 10 (19.6%) patients due to obstruction or infection. Primary ETV failed in 3/8 patients, and treated by VPS. None of those in Groups "B" or "C" required treatment for hydrocephalus during the follow up. CONCLUSION: Hydrocephalus affects the majority of patients with SONTD who have Myelomeningocele (MMC) and CM II and requires close surveillance and prompt management. Children with SONTD should routinely undergo MRI examination of brain and craniocervical junction to clarify ventricular size, and the presence of CM II.

PMID 23087828;year=2012;volume=3;issue=1;spage=112;epage=112;aulast=Elgamal

Elgamal EA. Natural history of hydrocephalus in children with spinal open neural tube defect. Surg Neurol Int [serial online] 2012 [cited 2012 Dec 6];3:112. Available from:

© 2012 Elgamal; This is an open-access article distributed under the terms of the Creative Commons Attribution License (, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Guidelines for management of idiopathic normal pressure hydrocephalus: second edition

Neurol Med Chir (Tokyo). 2012;52(11):775-809.

Mori E, Ishikawa M, Kato T, Kazui H, Miyake H, Miyajima M, Nakajima M, Hashimoto M, Kuriyama N, Tokuda T, Ishii K, Kaijima M, Hirata Y, Saito M, Arai H. Source Department of Behavioral Neurology and Cognitive Neuroscience, Tohoku University Graduate School of Medicine.

Abstract Among the various disorders manifesting dementia, gait disturbance, and urinary incontinence in the elderly population, idiopathic normal pressure hydrocephalus (iNPH) is becoming of great importance. After the publication of the first edition of the Guidelines for Management of Idiopathic Normal Pressure Hydrocephalus in 2004 (the English version was published in 2008), clinical awareness of iNPH has risen dramatically, and the number of shunt surgeries has increased rapidly across Japan. Clinical and basic research on iNPH has increased significantly, and more high-level evidence has since been generated. The second edition of the Japanese Guidelines was thus published in July 2011, to provide a series of timely evidence-based recommendations related to iNPH. The revision of the Guidelines has been undertaken by a multidisciplinary expert working group of the Japanese Society of Normal Pressure Hydrocephalus in conjunction with the Japanese Ministry of Health, Labour and Welfare research project on "Studies on the epidemiology, pathophysiology, and treatment of normal pressure hydrocephalus." This English version of the second edition of the Guidelines was made to share these ideas with the international community and to promote international research on iNPH. PMID 23183074

Diagnosis, treatment, and long-term outcomes of fetal hydrocephalus

Semin Fetal Neonatal Med. 2012 Oct 19. pii: S1744-165X(12)00086-8. doi: 10.1016/j.siny.2012.07.004.

Yamasaki M, Nonaka M, Bamba Y, Teramoto C, Ban C, Pooh RK. Source Department of Neurosurgery, Osaka National Hospital, National Hospital Organization, Osaka, Japan; Institute for Clinical Research, Osaka National Hospital, National Hospital Organization, Osaka, Japan. Electronic address:


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. Copyright © 2012 Elsevier Ltd. All rights reserved.

PMID 23089488


Hydrocephalus Indian scenario - A review

J Pediatr Neurosci. 2011 Oct;6(Suppl 1):S11-22. doi: 10.4103/1817-1745.85704.

Venkataramana NK. Source Department of Neurosurgery, Advanced Neuroscience Institute, BGS Global Hospital, Bangalore, India.


Hydrocephalus is a common clinical problem seen in pediatric neurosurgical practice. Hydrocephalus involves dilatation of the cerebral ventricular system with corresponding, compressive effects on the parenchyma. It can be communicative or obstructive types. Congenital, acquired, infective, and secondary hydrocephalus have different clinical features with different modality of treatments. Ventriculoperitoneal shunt is the gold standard of treatment. Endoscopic 3(rd) ventriculostomy is rapidly gaining prominence as an alternative. Various kinds of hydrocephalus, their pathophysiology, treatment and complications are reviewed.

PMID 22069421


Lumbar peritoneal shunt

Neurol India. 2010 Mar-Apr;58(2):179-84.

Yadav YR, Parihar V, Sinha M. Source Neurosurgery Unit, NSCB Medical College, Jabalpur, Madhya Pradesh, India.


A lumbar peritoneal (LP) shunt is a technique of cerebrospinal fluid (CSF) diversion from the lumbar thecal sac to the peritoneal cavity. It is indicated under a large number of conditions such as communicating hydrocephalus, idiopathic intracranial hypertension, normal pressure hydrocephalus, spinal and cranial CSF leaks, pseudomeningoceles, slit ventricle syndrome, growing skull fractures which are difficult to treat by conventional methods (when dural defect extends deep in the cranial base or across venous sinuses and in recurrent cases after conventional surgery), raised intracranial pressure following chronic meningitis, persistent bulging of craniotomy site after operations for intracranial tumors or head trauma, syringomyelia and failed endoscopic third ventriculostomy with a patent stoma. In spite of the large number of indications of this shunt and being reasonably good, safe, and effective, very few reports about the LP shunt exist in the literature. This procedure did not get its due importance due to some initial negative reports. This review article is based on search on Google and PubMed. This article is aimed to review indications, complications, results, and comparison of the LP shunt with the commonly practiced ventriculoperitoneal (VP) shunt. Shunt blocks, infections, CSF leaks, overdrainage and acquired Chiari malformation (ACM) are some of the complications of the LP shunt. Early diagnosis of overdrainage complications and ACM as well as timely appropriate treatment especially by programmable shunts could decrease morbidity. Majority of recent reports suggest that a LP shunt is a better alternative to the VP shunt in communicating hydrocephalus. It has an advantage over the VP shunt of being completely extracranial and can be used under conditions other than hydrocephalus when the ventricles are normal sized or chinked. More publications are required to establish its usefulness in the treatment of wide variety of indications.

PMID 20508332