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Choreoathetosis after cardiac surgery with hypothermia and extracorporeal circulation
J L Gherpelli1, E Azeka, A Riso
1Department of Child Neurology, Clinics Hospital of the University of São Paulo Medical School, SP, Brazil.
Insights
Choreoathetoid movements occurred in children after heart surgery using extracorporeal circulation (ECC). While some children recovered fully, others had lasting neurological issues, highlighting the need for further research.
Area of Science:
- Pediatric Neurology
- Cardiovascular Surgery
- Neuroscience
Background:
- Congenital cyanotic heart defects often require surgical intervention.
- Hypothermia and extracorporeal circulation (ECC) are standard techniques in cardiac surgery.
- Post-operative neurological complications can occur following complex cardiac procedures.
Purpose of the Study:
- To describe the clinical presentation and outcome of choreoathetoid movements in children post-cardiac surgery.
- To investigate potential causes and effective treatments for this neurological syndrome.
- To emphasize the need for further research into the pathogenesis of post-operative choreoathetosis.
Main Methods:
- Observational study of eleven pediatric patients (4-48 months) with congenital cyanotic heart defects.
- Detailed clinical assessment of neurological symptoms, including onset, characteristics, and duration.
- Review of diagnostic tests such as CT scans, cerebrospinal fluid analysis, and EEG.
- Evaluation of symptomatic treatment with haloperidol and benzodiazepines.
Main Results:
- Choreoathetoid movements developed in 11 children 2-12 days after cardiac surgery with hypothermia and ECC.
- Affected children exhibited limb, facial, and tongue movements, causing severe dysphagia.
- Of nine survivors, three had persistent movements, while six experienced complete regression within 1-4 weeks.
- Diagnostic tests showed no specific findings related to the movements.
- Symptomatic treatment provided temporary improvement but did not alter disease course.
Conclusions:
- Post-cardiac surgery choreoathetoid syndrome is an emerging neurological entity.
- The pathogenesis remains unclear, necessitating further investigation.
- Preventive strategies are crucial to avoid potentially permanent neurological sequelae.
Abstract:
Eleven children, 4-48 months old, with congenital cyanotic heart defects developed choreoathetoid movements 2-12 days after cardiac surgery with hypothermia and extracorporeal circulation (ECC). The abnormal movements mainly involved the limbs, facial musculature, and tongue, leading to a severe dysphagia. The symptoms had an acute onset, after a period of apparent neurologic normality, and had a variable outcome. Of the nine children that survive, three had abnormal movements when last seen (41 days to 12 months of follow-up). The other six children had a complete regression of the choreoathetoid movements 1-4 weeks after onset. No specific finding was observed in the CT scans, cerebrospinal fluid examination, or EEG that could be related to the abnormal movements. Symptomatic therapy with haloperidol with or without benzodiazepines led to symptomatic improvement in six children, although there was no evidence that this treatment modified the evolution of the disease. The authors conclude that the choreoathetoid syndrome after cardiac surgery with deep hypothermia and ECC is an ill-defined entity requiring additional study to better understand its pathogenesis so that preventive measures can be taken to avoid a condition that can lead to permanent and incapacitating neurologic sequelae.
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