Bobble-head Doll Syndrome Associated with the III Ventricular Cyst: Three cases in children 7 years after CVP or CVA shunting

Abstract

The III ventricular cyst, accompanied by head bobbing, was operated on for the first time by Benton in 1965. He and his co-workers described this phenomenon as the “bobble-head doll syndrome.” The authors reevaluate in this report three cases treated with their own surgical technique—cystoventriculoperitoneal (CVP) or cystoventriculoatrial (CVA) shunting—7 years ago. In all cases a Cordis-Hakim Standart Valve 30–45 mm H₂O was used. In the first case (a 4, 5-year-old girl), the CVP shunt was removed after 16 months due to suppuration around the device after trauma of the scalp. Nevertheless, the communication between the cyst and ventricle continued and the bobbing has never returned. In the second case (a 10-year-old girl), a pseudocyst at the end of the peritoneal catheter was accidentally found and evacuated after 4, 5 years. In the third case (a 17-year-old boy), was the postoperative course uneventful. The follow-up for 7 years of all cases showed good results from the clinical point of view and CT scans. Using peroperative CSF cyst and ventricular tensimetry, ventriculo- and cystography and/or CT scanning the authors also tried to elucidate the pathophysiology of the bobble-head doll syndrome. In their opinion, head bobbing, as a unique head manifestation of this syndrome, is caused simultaneously by the changes in pressure and metabolism in both the nuclei and tracts around the III ventricle. The restoration of CSF circulation and metabolism can initiate either complete cessation or considerable abatement of the bobble-head doll syndrome symptomatology-nodding, disorders of the thalamus, hypothalamus, limbic system and visual tract. Reviewing the literature, 39 cases were operated upon and 4 were treated without surgery.