Congenital Marked Communicated Hydrocephalus

Case History: A 3-month-old make presented with marked skull enlargement and widening sutures and fontanelles.

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FIGURE  A

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FIGURE B

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FIGURE C

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FIGURE D

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FIGURE E

Findings: MDCT (Figs. A, B, C) showing marked dilatation of the ventricular system of the brain. Reduced brain mantel effaced against the skull. 3D reconstructed images (Figs. D, E) showing thinning expanded skull bone with very widened sutures and fontanelles.

Diagnosis: Congenital marked communicated hydrocephalus

Discussion: Hydrocephalus occurs in approximately one out of 500 births. The following are the primary reasons why hydrocephalus occurs:
• Blockage of the CSF flow inside of the head
• Problems absorbing the CSF
• Overproduction of CSF (rare)

Hydrocephalus can occur either as a condition present at birth (congenital), or it can be acquired later in life. When hydrocephalus isn't related to a known genetic cause, it's thought that many factors, both genetic and environmental, contribute to the condition. In a small percentage of babies, a single gene defect on the X chromosome, or another chromosome, is responsible for the condition. If a child has been born with hydrocephalus not known to be caused by a single gene defect, the chance of it occurring in another child is 1 to 5 percent.

Causes of acquired hydrocephalus may include the following:
• Tumor
• Infection
• Prematurity
• Bleeding inside the head
• Birth injury
• Abnormal blood vessel formation inside of the head
• Trauma

References
1. Hydrocephalus. American Association of Neurological Surgeons. http://www.aans.org/Patient%20Information/Conditions%20and%20Treatments/Hydrocephalus.aspx. Accessed June 11, 2011.
2. Hydrocephalus fact sheet. National Institute of Neurological Disorders and Stroke. http://www.ninds.nih.gov/disorders/hydrocephalus/detail_hydrocephalus.htm. Accessed June 10, 2011.
3. Fishman MA. Hydrocephalus. http://www.uptodate.com/home/index.html. Accessed May 10, 2011. 4. Diagnosis of hydrocephalus. Hydrocephalus Association. https://www.hydroassoc.org/hydrocephalus-education-and-support/learning-about-hydrcephalus/diagnosis-of-hydrocephalus. Accessed June 11, 2011.

Doaa Ibrahim, MD in radio-diagnosis, Zagazig University Hospitals and TechnoScan Centers in Egypt