Bobble-head doll syndrome successfully treated with an endoscopic ventriculocystocisternostomy

Case report and review of the literature

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✓The bobble-head doll syndrome (BHDS) is characterized by a back-and-forth movement of the head with a frequency of 2 to 3 Hz, which increases during walking and excitement and decreases during concentration. The head movements are accompanied by macrocephaly, ocular disturbances, psychomotor retardation, and sometimes endocrine dysfunction. The BHDS is frequently associated with a suprasellar arachnoid cyst. The authors present the case of a 4-year-old patient with BHDS; an endoscopic cystoventriculostomy was performed by fenestrating a cyst in the suprasellar region. After wide fenestration of the cyst wall that was protruding and obstructing the foramen of Monro, the cyst was entered with the endoscope and a small, natural, valvelike communication of the cyst with the basal prepontine cistern was seen close to the basilar artery. This communication was widened by balloon dilation. After completion of the ventriculocystocisternostomy, the cyst collapsed and the obstruction of the aqueduct was resolved. In view of the source mechanism and cerebrospinal fluid dynamics of the suprasellar arachnoid cyst, a ventriculocystocisternostomy is an important treatment option for BHDS arising from a suprasellar cyst. Three years after treatment, the head bobbing had resolved completely and psychomotor development was improving. Delay of diagnosis and treatment of this condition can cause permanent neurological dysfunction and psychomotor retardation. The authors recommend early ventriculocystocisternostomy as a physiologically based treatment for BHDS originating from a suprasellar cyst.

Article Information

Address reprint requests to: Eveline E. O. Hagebeuk, M.D., Department of Child Neurology, Emma Children Hospital/AMC (G8-205), P.O. Box 22660, 1100 DD Amsterdam, the Netherlands. email: e.e.hagebeuk@amc.uva.nl.

© AANS, except where prohibited by US copyright law.

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Figures

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    A T1-weighted sagittal MR image obtained at initial presentation in 1998, demonstrating a large suprasellar arachnoid cyst.

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    A T2-weighted axial MR image obtained at initial presentation in 1998, demonstrating a large hydrocephalus with the suprasellar arachnoid cyst.

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    A T2-weighted sagittal MR image obtained 2 years after surgery in 2001, demonstrating a residual suprasellar arachnoid cyst and an unchanged large hydrocephalus. A flow-void signal through the aqueduct is visible. A flow-void signal is also apparent through the suprasellar arachnoid cyst and basal prepontine cistern, demonstrating a communication between them.

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