Intraventricular baclofen and bilateral pallidotomy in a pediatric patient with acquired generalized dystonia and refractory status dystonicus: illustrative case

Abstract

Background: Status dystonicus (SD) is a life-threatening emergency in pediatric patients with severe dystonia, often requiring intensive care. Surgical interventions, such as deep brain stimulation, intrathecal baclofen (ITB), or pallidotomy, are considered for refractory cases, but evidence for intraventricular baclofen (IVB) and bilateral pallidotomy (BP) in children is limited. We report a novel combined approach using IVB followed by BP in a 3-year-old boy with refractory SD secondary to hypoxic-ischemic encephalopathy.

Case Description: A 3-year-old male developed SD and spastic quadriparesis following cardiorespiratory arrest due to airway obstruction. Despite aggressive medical management (levodopa, clonazepam, tetrabenazine, oral baclofen, sedatives), SD persisted, requiring prolonged mechanical ventilation. An ITB test (100 mcg) reduced the modified Ashworth scale from 5 to 3 and dystonic episodes from 8 to 2/day. IVB was placed via a third ventricle catheter with a pump delivering 100-1050 mcg/day, achieving spasticity control but fluctuating dystonia response. Due to recurrent SD, poor prognosis, and malnutrition, BP was performed using stereotactic microelectrode guidance and radiofrequency lesions (80°C, 60s). Postoperative MRI confirmed precise pallidal lesions. The patient achieved full SD resolution, was weaned from ventilation, and stabilized at home with tracheostomy and gastrostomy.

Conclusions: Bilateral pallidotomy and IVB should be considered in the treatment of refractory SD, particularly for patients with contraindications for DBS and concurrent severe spasticity. The literature review  support this findings, providing an overview that can guide palliative management in similar cases. Further studies are needed to establish comprehensive guidelines for surgical management of SD in children.