Objective: To present management with intraventricular baclofen followed by bilateral pallidotomy in a 3-years-old child who presents a refractory status dystonicus secondary to hypoxic-ischemic encephalopathy

Background: Refractory Status dystonicus (SD) is a life-threatening emergency, surgical treatments such as deep brain stimulation (DBS) have demonstrated efficacy, alternatives include bilateral pallidotomy (BP) for patients with cognitive impairment, young age, cachectic state, follow‐up limitations, failure or contraindications for DBS; Intrathecal baclofen (ITB) has reports benefit in a small number of patients; and intraventricular baclofen (IVB) when ITB is not feasible

Method: A previously healthy 3-year-old boy presented with secondary severe hypoxic-ischemic encephalopathy after cardiorespiratory arrest due to upper airway obstruction, which triggered SD with severe spastic quadriparesis refractory to medical treatment and prolonged mechanical ventilation.  The patient required IVB, after a positive intrathecal baclofen test, (reduction of modified Ashworth scale score from 5 to 3 generalized and 8 to 2 episodes per day of SD), and reducing the dose of sedative drugs by 50%. IVB was performed through neuronavigation until a 850 mcg/day within the first two weeks, with good spasticity but fluctuating dystonia response. After a surgical review of adequate pump patency with contrast infusion testing, a bilateral pallidotomy was considered due to the poor neurological prognosis and malnutrition status

Results: The preoperative score on the Burke Fahn Marsden rating scale was 96 points. After BP the initial response was partial, but gradually the patient achieved resolution of the SD and weaning from mechanical ventilation. The final dose of IVB baclofen is 1050 mcg/day.

Conclusion: 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

Fig1. 3th Intraventricular catheter IVB

Figure 2. Baclofen pump in abdominal region

Figure 3. Status dystonicus in pediatric ICU

Figure 4. MRI showing BP in a T2

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MDS Abstracts - https://www.mdsabstracts.org/abstract/intraventricular-baclofen-and-bilateral-pallidotomy-in-a-pediatric-patient-with-acquired-generalized-dystonia-and-refractory-status-dystonicus/