Hydrocephalus with chromosome 9 tetrasomy and persistent left superior vena cava treated by ventriculo-femoro-atrial shunt: a case report

Shimozato Rin, Takeshita Tomonori, Nagamine Tomoaki

doi:10.34544/jspn.48.3_295

<p>Introduction: We report a case of hydrocephalus caused by diffuse villous hyperplasia of the choroid plexus (DVHCP) associated with chromosome 9 tetrasomy. Fluid management in the patient, although difficult, was done by a ventriculo-femoro-atrial (VFA) shunt. Case: The patient was a young girl aged 6 years and 1 month who had a birth weight of 3402 g and a gestational age of 40 weeks. She presented with a failed ventriculo-atrial (VA) shunt five years after its placement. Ventricular enlargement was not noted at birth, but marked hydrocephalus was confirmed by MRI six months later. A ventriculo-peritoneal (VP) shunt was then placed. Six months later, it became difficult to control the fluid in the abdomen; hence, 9 months after the initial operation, her VP shunt was replaced with a VA shunt, which also failed about 5 years after its placement. This was due to a thrombus in the atrial catheter that deviated to the superior vena cava. Although an attempt was made to reconstruct the VA shunt using the left internal jugular vein, stable placement of the catheter was difficult because of the persistent left superior vena cava (PLSVC). Shunt dysfunction and infection reoccurred. Finally, a VFA shunt placement was performed after choroid plexus ablation to obtain spinal fluid control. Conclusion: In childhood hydrocephalus, shunt replacement or rerouting is unavoidable. VFA shunts, although rarely utilized, can be an option for drainage routes.</p>

Hydrocephalus with chromosome 9 tetrasomy and persistent left superior vena cava treated by ventriculo-femoro-atrial shunt: a case report

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