Risk factors and results of hemispherotomy reoperations in children.

OBJECTIVE: The goal of this study was to perform an analysis of a single-center experience with hemispherotomy reoperations for refractory hemispheric pediatric epilepsy due to persistence of seizures after initial surgery. The authors also identify possible anatomical and neurophysiological reasons for hemispherotomy failure, as well as risk factors and surgical options for this subgroup of patients. METHODS: A review was performed of the medical records in 18 consecutive cases in which candidates for redo hemispherotomy were treated between 2003 and 2018 at the authors' epilepsy surgery center. Fourteen patients underwent reoperation due to seizure recurrence and were studied herein, whereas in 3 the initial surgical procedure was stopped because of uncontrollable bleeding, and the remaining patient refused to undergo a reoperation in spite of seizure recurrence and went on to have a vagus nerve stimulation device placed. RESULTS: Among the 14 patients whose seizures recurred and in whom reoperations were done, the etiology of epilepsy consisted of 7 cases with malformations of cortical development (50%), 5 cases of Rasmussen encephalitis (35.8%), 1 case of porencephaly (7.1%), and 1 case of Sturge-Weber syndrome (7.1%). Eleven patients had radiological evidence of incomplete disconnection. After reoperation, 6 patients were Engel class IA, 1 was Engel II, 5 were Engel III, and 2 were Engel IV, within a mean follow-up of 48.4 months. CONCLUSIONS: Patients with malformations of cortical development have a higher risk of seizure recurrence, and these malformations comprised the main etiology in the reoperation series. Failure of an initial hemispherotomy usually occurs due to incomplete disconnection and needs to be extensively assessed. Outcomes of reoperation are most often favorable, with acceptable complication rates.

Cranial morcellation decompression for refractory idiopathic intracranial hypertension in children.

BACKGROUND: Primary idiopathic intracranial hypertension (PIIH) in children is rare and has a poorly understood pathophysiology. It is characterized by raised intracranial pressure (ICP) in the absence of an identified brain lesion. Diagnosis is usually confirmed by the measurement of a high cerebrospinal fluid (CSF) opening pressure and exclusion of secondary causes of intracranial hypertension. Refractory PIIH may lead to severe visual impairment. The purpose of this study was to evaluate a cranial morcellation decompression (CMD) technique as a new surgical alternative to stabilize intracranial pressure in PIIH. MATERIALS AND METHODS: A literature review was carried out, disclosing only 7 pediatric cases of PIIH treated with surgical skull expansion. In addition, we describe here one case of our own experience treated by CMD. CONCLUSIONS: CMD surgery is a safe and effective option to control refractory PIIH in selected patients.

Hypertrophic olivary degeneration in children after posterior fossa surgery. An underdiagnosed condition.

BACKGROUND: Hypertrophic olivary degeneration (HOD) is a rare transsynaptic form of degeneration occurring after injury to the dentato-rubro-olivary pathway ("Guillain-Mollaret triangle"). The majority of studies have described HOD resulting from posterior fossa (PF) hemorrhage or infarction. HOD in patients undergoing PF surgery has not been well characterized. These lesions are rare and symptomatic children with HOD are even more uncommon. The purpose of this study was to evaluate HOD that develops after PF operations in children. MATERIALS AND METHODS: A literature review was carried out describing 37 pediatric cases of HOD in 13 articles. In addition, two new cases of our own experience were included. CONCLUSIONS: HOD is a rare complication related after PF tumors surgery and symptoms may be misdiagnosed with pediatric cerebellar mutism syndrome. Children with HOD usually do not develop palatal tremor but ataxia is common.