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[The bobble head doll syndrome]
Insights
Bobble head doll syndrome in a child was treated with shunts. A cysto-peritoneal shunt improved head movements, growth, and motor development by reducing third ventricle cyst size.
Area of Science:
- Neurology
- Pediatrics
- Neurosurgery
Background:
- Bobble head doll syndrome (BHDS) is a rare movement disorder characterized by involuntary, rhythmic, side-to-side or front-to-back head movements.
- It is often associated with structural brain abnormalities, particularly in the third ventricle.
- This condition can lead to significant developmental delays and neurological deficits.
Observation:
- A 4.1-year-old girl presented with typical bobble head doll syndrome (2-3 Hz head oscillations).
- She exhibited retarded motor development and growth.
- Cranial computed tomography (CT) revealed a third ventricular cyst and hydrocephalus of the lateral ventricles.
Findings:
- Initial management with a ventricular-cardial low-pressure shunt for hydrocephalus provided temporary relief.
- Repeated aspiration of the third ventricular cyst via an Ommaya reservoir was ineffective for long-term symptom management.
- Surgical intervention with a cysto-peritoneal shunt resulted in cyst size reduction (confirmed by CT) and notable improvement in the patient's symptoms.
Implications:
- Cysto-peritoneal shunting is an effective treatment for managing third ventricular cysts causing bobble head doll syndrome.
- This intervention can lead to significant clinical improvement in patients with BHDS and associated developmental delays.
- Early diagnosis and appropriate surgical management are crucial for optimizing outcomes in children with this rare condition.
Abstract:
A 4,1 year old girl with bobble head doll syndrome is described. The child showed the typical 2-3 per second anterior-posterior movements of the head. The girl had a retarded motor development and retarded growth. On CT scan we found a cyst within the third ventricle and a hydrocephalus of the lateral ventricles. To reduce the hydrocephalus we implanted a ventricular-cardial low pressure shunt system. Repeated emptying of the cyst via an Ommaya reservoir had no long-term effect. Implantation of a cysto-peritoneal shunt-system led to a reduction of the cyst (determined by CT scan) and an improvement of the symptoms.