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Anaesthetic management of children with tuberous sclerosis
Ze'ev Shenkman1, Mark A Rockoff, Elizabeth A Eldredge
1Department of Anaesthesia, Children's Hospital, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA. shlomsh@netvision.net.il
Insights
Tuberous sclerosis (TS) patients with intractable seizures often have coexisting heart and kidney issues. Pre-surgical evaluation for these conditions is crucial for managing TS complications.
Area of Science:
- Pediatric Neurology
- Cardiology
- Nephrology
Background:
- Tuberous sclerosis (TS) is a genetic disorder characterized by hamartomatous growths affecting multiple organ systems.
- Commonly presents with skin and brain lesions, but systemic involvement is frequent.
- Neurological manifestations, particularly intractable seizures, are a significant concern in pediatric TS.
Purpose of the Study:
- To evaluate the perioperative course of children with Tuberous Sclerosis (TS) and medically intractable seizures.
- To identify the prevalence of cardiac and renal involvement in these patients.
- To highlight potential perioperative complications associated with TS.
Main Methods:
- Retrospective review of 24 children with TS and intractable seizures.
- Analysis of perioperative data, including cardiac and renal evaluations.
- Documentation of major perioperative complications.
Main Results:
- Cardiac rhabdomyoma identified in 11/18 patients; congenital heart defects in 6.
- Renal TS diagnosed in 10/17 patients, with one case of azotemia and four of hypertension.
- Major perioperative complications included one death, five seizure recurrences, and two bradyarrhythmias.
Conclusions:
- Patients with TS and neurological disorders frequently exhibit coexisting cardiac and renal disease.
- Pre-surgical evaluation for cardiac and renal conditions is recommended for patients with TS.
- Early identification and management of systemic involvement can improve perioperative outcomes.
Background:
Tuberous sclerosis (TS) is a hamartomatous disease that usually presents with cutaneous and intracranial lesions, but can also affect other organ systems.
Methods:
In this report, we retrospectively reviewed the perioperative course of 24 children with TS who had medically intractable seizures as the primary disease process.
Results:
Cardiac rhabdomyoma was detected in 11 of 18 patients who had a cardiac evaluation, and coexisting congenital heart defects were diagnosed in six. Ten of 17 patients who had a renal evaluation were diagnosed with renal TS, presenting with azotaemia in one and hypertension in four. The major perioperative complications in the 52 anaesthetics, included death (a neonate after cardiac rhabdomyoma resection), seizures (five patients) and bradyarrhythmias (two patients).
Conclusions:
Patients with TS and neurological disorders frequently have coexisting cardiac and renal disease as well. Patients with TS should be evaluated for these organ specific disorders prior to surgery.