13 month old female Jason Dowling Radiology Elective April 21, 2005
Presentation • 13 month old female with no significant PMH • HPI: Pt presents with 2-3wk h/o increased head circumference, fussiness, lethargy, and refusal to stand. • PMI: NSVD without hydrocephalous at birth, otitis media, RSV infection • FH: brittle bone disease • Labs: CBC and BMP normal • Work-up: CT at outside hospital showed non-communicating hydrocephalous involving lateral and 3rd ventricle.
Radiology - sagital T1 • Findings: dilatation of the lateral and third ventricles with periventricular fluid • normal fourth ventricle • distal aqueduct stenosis
Hospital Course • Pt received a endoscopic third ventricular ventriculostomy • MRI showed marked decrease in ventricular size post-op
Aqueduct of Sylvius Stenosis • Most common cause of hydrocephalus (20% of cases) • Incidence: 0.5-1.0 per 1000 births • Can be congenital or acquired following inflammatory or neoplastic event • X-linked recessive form (rare) • Associated with spina bifida
Radiographic findings • Marked enlargement of the lateral ventricles, with interstitial edema • Outpouching of the suprasellar recesses of the third ventricle • “Ventricularization” of the proximal aqueduct just above the level of obstruction • Normal size and configuration of the fourth ventricle Differntial Diagnosis • Congenital malformation • Post-infectious • Post-hemorrhage • Tumors • Other causes of hydrocephalus: communicating hydrocephalus, malformations (Chiari type II, Dandy-Walker, Vein of Galen)
References • Behrman: Nelson Textbook of Pediatrics. 17th ed. Elsevier: 2004. Pg 1990. • Grainger & Allison's Diagnostic Radiology: A Textbook of Medical Imaging, 4th ed. 2001 Churchill Livingstone, Inc. pg 2403. • Nard, JA. Abnormal head size and shape. In: Gartner, JC, Zitelli, BJ. Common Chronic Symptoms in Pediatrics, Mosby, St. Louis, 1997.