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Anaesthetic management of children with tuberous sclerosis
Author(s) -
Shenkman Ze'ev,
Rockoff Mark A.,
Eldredge Elizabeth A.,
Korf Bruce R.,
Black Peter Mch. L.,
Soriano Sulpicio G.
Publication year - 2002
Publication title -
pediatric anesthesia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.704
H-Index - 82
eISSN - 1460-9592
pISSN - 1155-5645
DOI - 10.1046/j.1460-9592.2002.00917.x
Subject(s) - tuberous sclerosis , medicine , rhabdomyoma , perioperative , heart disease , disease , epilepsy , pediatrics , kidney disease , surgery , radiology , psychiatry
Summary 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.