Abstract
We report the anaesthetic management of an eight-year-old asthmatic boy with Bartter’s syndrome who had bilateral orchidopexy with caudal epidural analgesia. Bartter’s syndrome is a rare congenital disorder characterized by hypokalaemic hypochloraemic metabolic alkalosis, hyperaldosteronism, hyperreninaemia and hyperplasia of the juxtaglomerular apparatus of the kidneys. Characteristically, although these patients are normotensive they may be hypovolaemic. They may have unstable baroreceptor responses and show marked resistance to vasopressors. Hence, fluid, acid-base and electrolyte imbalances along with haemodynamic instability pose particular problems in their anaesthetic management. Previous case reports have described the management of these patients with general anaesthesia, our patient had his orchidopexy with caudal epidural analgesia using plain bupivacaine 0.5%. The patient was haemodynamically stable throughout surgery and was comfortable with caudal analgesia as the sole anaesthetic. Hypovalaemia, acid-base status and electrolyte imbalance were treated before instituting caudal epidural analgesia. We present this case report which describes the anaesthetic considerations in the light of the pathophysiology of Bartter’s syndrome.
Résumé
Le syndrome de Bartter est une affection congénitale rare caractérisée par une acidose métabolique hypochlohydrique avec hypokaliémie, hyperaldostéronisme, hyperréninémie associées à l’hyperplasie de l’appareil juxtaglomérulaire rénal. Cette observation décrit la gestion anesthésique d’un garçon asthmatique porteur d’un syndrome de Bartter soumis à une orchidopexie bilatérale sous anesthésie caudale. Bien qu’ib soient typiquement normotensifs, parfois ces patients sont en même temps hypovolémiques. Ils présentent des réponses baroréceptrices variables et une résistance marquée aux vasopresseurs. Par conséquent, les désiquilibres hydriques, acidobasiques et électrolytiques sont associés à l’instabilité hémodynamique et, pour cette raison, posent des problèmes particuliers lors de l’anesthésie. Des observations antérieures font état d’une gestion sous anesthésie générale mais dans ce cas-ci, l’orchidopexie a été réalisée sous anesthésie régionale caudale à la bupivacaïne 0,5%. Le patient a été stable sous le plan hémodynamique et confortable avec l’anesthésie caudale seule. L’hypovolémie, l’équilibre acidobasique et le dérangement électrolytique ont été traités avant l’initiation de l’anesthésie caudale. Les auteurs présentent cette observation qui décrit certaines particularités de l’anesthésie propres à la physiopathologie du syndrome de Bartter.
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Kannan, S., Delph, Y. & Moseley, H.S.L. Anaesthetic management of a child with Bartter’s syndrome. Can J Anaesth 42, 808–812 (1995). https://doi.org/10.1007/BF03011183
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DOI: https://doi.org/10.1007/BF03011183