Abnormal Development - Congenital Hydrocephalus

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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 Beta Draft - 20 Developmental anomalies  
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  
  • Structural developmental anomalies of the nervous system  
    • LA00 Anencephaly or similar anomalies
    • LA01 Cephalocele
    • LA02 Spina bifida
    • LA03 Arnold-Chiari malformation type II
    • LA04 Congenital hydrocephalus
    • LA05 Cerebral structural developmental anomalies
    • LA06 Cerebellar structural developmental anomalies
    • LA07 Structural developmental anomalies of the neurenteric canal, spinal cord or vertebral column
    • LA0Y Other specified structural developmental anomalies of the nervous system
    • LA0Z Structural developmental anomalies of the nervous system, unspecified
  • Structural developmental anomalies of the eye, eyelid or lacrimal apparatus
    • LA10 Structural developmental anomalies of ocular globes
    • LA11 Structural developmental anomalies of the anterior segment of eye
    • LA30 Structural developmental anomalies of lens or zonula
    • LA31 Structural developmental anomalies of the posterior segment of eye
    • LA32 Structural developmental anomalies of eyelid, lacrimal apparatus or orbit
    • LA3Y Other specified structural developmental anomalies of the eye, eyelid or lacrimal apparatus
    • LA3Z Structural developmental anomalies of the eye, eyelid or lacrimal apparatus, unspecified
  • Structural developmental anomalies of the ear  
    • LA40 Structural anomaly of eustachian apparatus
    • LA41 Minor anomalies of pinnae
    • LA42 Structural developmental anomalies of ear causing hearing impairment
    • LA43 Otocephaly
    • LA44 Accessory auricle
    • LA4Y Other specified structural developmental anomalies of the ear
    • LA4Z Structural developmental anomalies of the ear, unspecified
  • Structural developmental anomalies of the face, mouth or teeth
    • LA50 Structural developmental anomalies of teeth and periodontal tissues
    • LA51 Structural developmental anomalies of mouth or tongue
    • Clefts of lip, alveolus or palate
    • LA70 Congenital velopharyngeal incompetence
    • LA71 Facial clefts
    • LA72 Facial asymmetry
    • LA73 Macrocheilia
    • LA74 Microcheilia
    • LA75 Compression facies
    • LA76 Pierre Robin syndrome
    • LC20 Dermoid cyst
    • LA7Y Other specified structural developmental anomalies of the face, mouth or teeth
    • LA7Z Structural developmental anomalies of the face, mouth or teeth, unspecified
  • Structural developmental anomalies of the neck  
  • Structural developmental anomalies of the respiratory system  
  • Structural developmental anomalies of the circulatory system  
    • Structural developmental anomaly of heart and great vessels
      • LB00 Congenital heart or great vessel related acquired abnormality
      • LB01 Congenital anomaly of atrioventricular or ventriculo-arterial connections
      • LB02 Congenital anomaly of the mediastinal veins Congenital anomaly of atria or atrial septum
      • LB20 Congenital anomaly of atrioventricular valves or septum
      • LB21 Congenital anomaly of ventricles and ventricular septum
      • LB22 Functionally univentricular heart
      • LB23 Congenital anomaly of ventriculo-arterial valves and adjacent regions
      • LB24 Congenital anomaly of great arteries including arterial duct
      • LB25 Anomalous position-orientation of heart
      • LB26 Total mirror imagery
      • LB27 Left isomerism
      • LB28 Congenital anomaly of coronary arteries
      • LB29 Structural developmental anomalies of the pericardium
      • LB2Y Other specified structural developmental anomaly of heart and great vessels
      • LB2Z Structural developmental anomaly of heart and great vessels, unspecified
    • LB30 Structural developmental anomalies of the peripheral vascular system
      • LB30.1 Capillary malformations
      • LB30.2 Lymphatic malformations
        • LB30.21 Macrocystic lymphatic malformation
        • LB30.22 Microcystic lymphatic malformation
        • LB30.23 Cystic hygroma in fetus
        • BD23.1 Primary lymphoedema
            • EK91 Yellow nail syndrome
            • LC5F.26 Noonan syndrome
        • LB30.2Y Other specified lymphatic malformations
        • LB30.2Z Lymphatic malformations, unspecified
      • LB30.3 Peripheral venous malformations
      • LB30.4 Peripheral arteriovenous malformations
      • LB30.5 Peripheral arterial malformations
      • LB30.6 Pulmonary arteriovenous fistula
      • LB30.Y Other specified structural developmental anomalies of the peripheral vascular system
      • LB30.Z Structural developmental anomalies of the peripheral vascular system, unspecified
    • LB3Y Other specified structural developmental anomalies of the circulatory system
    • LB3Z Structural developmental anomalies of the circulatory system, unspecified
  • Structural developmental anomalies of the diaphragm, abdominal wall or umbilical cord  
  • Structural developmental anomalies of the digestive tract  
  • Structural developmental anomalies of the liver, biliary tract, pancreas or spleen  
  • Structural developmental anomalies of the urinary system  
  • Structural developmental anomalies of the female genital system  
  • Structural developmental anomalies of the male genital system  
  • Structural developmental anomalies of the breast  
  • Structural developmental anomalies of the skeleton  
  • Structural developmental anomalies of the skin  
  • Structural developmental anomalies of the adrenal glands  
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.

See also International Classification of Diseases

ICD-10 Links: XVII Congenital Malformations | System Tables | XVI Perinatal Period | XV Pregnancy Childbirth | Abnormal Development | Reports

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


MRI Congenital hydrocephalus.[1]

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.

Links: Neural Abnormalities | Ventricular Development

Neural Links: Introduction | Ventricular System | Stage 22 | Gliogenesis | Fetal | Medicine Lecture - Neural | Lecture - Ectoderm | Lecture - Neural Crest | Lab - Early Neural | Neural Crest | Sensory | Abnormalities | Folic Acid | Iodine Deficiency | Fetal Alcohol Syndrome | Postnatal | Postnatal - Neural Examination | Histology | Historic Neural | Category:Neural

Some Recent Findings

  • Diagnosis, treatment, and long-term outcomes of fetal hydrocephalus[2] "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."

Aqueduct of Sylvius

Hydrocephalus aqueduct of Sylvius 01.jpg

Progressive obliteration of the aqueduct of Sylvius in hydrocephalus[3]

  • a Ultrasound of fetal patient at 36 GW showing dilation of the lateral ventricles (arrows).
  • b MRI at 33 GW. The third ventricle (3°) and posterior horns of the lateral ventricles (ph) are dilated.
  • c MRI at 33 weeks. Stenosis of the SA is shown.
  • d Detailed magnification of the area framed in C showing stenosis of the SA. 4°, fourth ventricle.
  • e CT at 39 GW (or the 2nd PN day). The lateral (LV) and third (3°) ventricles are dilated.
  • f MRI of the brain on the 5th postnatal day, sagittal T2 imaging. The SA is obliterated

Arnold-Chiari syndrome

ICD - Q07 Other congenital malformations of nervous system - Q07.0 Arnold-Chiari syndrome

Chiari II malformation MRI02.jpg

Magnetic Resonance Image Sagittal T1-weighted image showing Chiari II malformation.[4]


Ventriculoperitoneal Shunt

Ventriculoperitoneal shunt
Ventriculoperitoneal shunt[5]

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

Subarachnoid cistern

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.


  1. Rakan Bokhari, Saleh Baeesa Remote cerebellar hemorrhage due to ventriculoperitoneal shunt in an infant: a case report. J Med Case Rep: 2012, 6;222 PubMed 22846583 | PMC3443652 | J Med Case Rep.
  2. Mami Yamasaki, Masahiro Nonaka, Yohei Bamba, Chika Teramoto, Chiaki Ban, Ritsuko K Pooh Diagnosis, treatment, and long-term outcomes of fetal hydrocephalus. Semin Fetal Neonatal Med: 2012, 17(6);330-5 PubMed 23089488
  3. 27067115</pubmed>> Eduardo Ortega, Rosa I Muñoz, Nelly Luza, Francisco Guerra, Monserrat Guerra, Karin Vio, Roberto Henzi, Jaime Jaque, Sara Rodriguez, James P McAllister, Esteban Rodriguez 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: 2016, 16(1);45 PubMed 27067115
  4. Essam A Elgamal Natural history of hydrocephalus in children with spinal open neural tube defect. Surg Neurol Int: 2012, 3;112 PubMed 23087828 | PMC3475879 | Surg Neurol Int.
  5. S P Whiley, G Mantokoudis, D Ott, H Zimmerman, A K Exadaktylos A review of full-body radiography in nontraumatic emergency medicine. Emerg Med Int: 2012, 2012;108129 PubMed 23243508


N K Venkataramana Hydrocephalus Indian scenario - A review. J Pediatr Neurosci: 2011, 6(Suppl 1);S11-22 PubMed 22069421

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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)

José Ascención Arenas-Ruiz, Horus Martinez-Maldonado, Vicente Gonzalez-Carranza, Samuel Torres-García, Fernando Chico-Ponce de Leon Endoscopic ventriculo-cisterno-ventricular approach in the treatment of bilateral trapped temporal horn related to fungal infection in a child: case report and review of the literature. Childs Nerv Syst: 2018; PubMed 29557511

Mohammad Sadegh Masoudi, Marziye Rasafian, Zahra Naghmehsanj, Fariborz Ghaffarpasand Intraperitoneal cerebrospinal fluid pseudocyst with ventriculoperitoneal shunt. Afr J Paediatr Surg: 2018, 14(3);56-58 PubMed 29557353

Rebecca Korth, Renate Schepker [Brain X-radiation for Childhood Epilepsy, Hydrocephalus or Mental Retardation? Research at Tuebingen University, 1940-1946]. [Röntgenbestrahlung des Gehirns zur Behandlung von kindlicher Epilepsie, Hydrocephalus oder Schwachsinn? Forschungen an der Tübinger Universitätsklinik von 1940-1946.] Prax Kinderpsychol Kinderpsychiatr: 2017, 66(7);498-515 PubMed 29557315

Jonathan Roth, Haggai Benvenisti, Shlomi Constantini Watertight dural closure in pediatric craniotomies - is it really necessary? World Neurosurg: 2018; PubMed 29551726

Pierre-Olivier Champagne, Michel W Bojanowski Cranio-cervical junction meningiomas without hydrocephalus presenting solely with syncope: report of two cases. World Neurosurg: 2018; PubMed 29551725

Danica M Sutherland, Pavithra Aravamudhan, Terence S Dermody An Orchestra of Reovirus Receptors: Still Searching for the Conductor. Adv. Virus Res.: 2018, 100;223-246 PubMed 29551138

Nabeel Alshafai, Rodolfo Maduri, Mrigank Shail, Domenico Chirchiglia, David Meyronet, Francesco Signorelli Surgical approach for suprasellar hemangioblastomas preserving the pituitary stalk: Review of the literature and report of a further case. Clin Neurol Neurosurg: 2018, 168;147-152 PubMed 29550691

Karen Brastad Evensen, Michael O'Rourke, Fabrice Prieur, Sverre Holm, Per Kristian Eide Non-invasive Estimation of the Intracranial Pressure Waveform from the Central Arterial Blood Pressure Waveform in Idiopathic Normal Pressure Hydrocephalus Patients. Sci Rep: 2018, 8(1);4714 PubMed 29549286

Haiyong He, Wensheng Li, Chaofeng Liang, Lun Luo, Bo Hou, Huasheng Yang, Ying Guo Eyebrow incision for combination supraorbital minicraniotomy with orbital osteotomy: application to cranio-orbital lesions. World Neurosurg: 2018; PubMed 29548951

Vinayak Narayan, Hima Pendharkar, Bhagavatula Indira Devi, Dhananjaya I Bhat, Dhaval P Shukla Aggressive management of vasospasm with direct intra-arterial nimodipine therapy. Neurol India: 2018, 66(2);416-422 PubMed 29547164

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Cite this page: Hill, M.A. (2018, March 21) Embryology Abnormal Development - Congenital Hydrocephalus. Retrieved from https://embryology.med.unsw.edu.au/embryology/index.php/Abnormal_Development_-_Congenital_Hydrocephalus

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