Anesthetic management of cesarean delivery for a parturient with Wilson's disease

Abstract

Rationale:Wilson's disease (WD), or hepatolenticular degeneration, is an autosomal recessive disorder with a prevalence of 1:50,000 to 1:100,000 live births.Patient concerns:A 26-year-old primipara with WD was admitted to our hospital, due to awaiting delivery. Her main symptoms were slightly higher total bile acid (TBA) and bilateral depressed edema of lower limbs.Diagnosis:She was at 38 weeks and 4 days of gestation with a 15-year history of WD, controlled with penicillamine in the past and replaced by zinc preparations from three months before pregnancy.Outcomes:General anesthesia was successfully administered for a female with WD undergoing cesarean delivery.Lesson:General anesthesia can be administered in an asymptomatic primigravida with WD. Appropriate anesthetics choice can effectively minimize the rates of complications and sequelae.

Wilson Hastalığı Olan Gebede Sezaryen Ameliyatında Spinal Anestezi Uygulaması

“…Bu hastalarda fertilizasyon anormallikleri, obstetrik sorunların daha çok yaşanması ve gebelik süresince multidisipliner tıbbi takibini gerektirmesinde, kıymetli gebeliğe sahip olmalarının etkisinin de olduğu şüphe götürmez (9)(10)(11)(12)(13)(14) . Wilson hastalarında özellikle hastalığın komplike olduğu gebelerde normal vajinal doğuma göre sezaryen tercih edilmektedir (6,(10)(11)(12)(13)(14) . Üstüner ve ark.…”

Perioperative anesthetic management for cesarean delivery of severe Wilson’s disease with liver failure: a case report

“…WD shows abnormal copper accumulation in the liver and brain due to a decrease in ceruloplasmin, which is the transport protein bound to serum copper. ATP7B mutation on chromosome 13q14 gene has been identified as the causative gene [1,2]. Generally, the treatment of WD is by pharmaceutical means that reduce copper absorption or promote copper excretion.…”

“…Wilson's disease (WD) is a rare autosomal recessive disorder whose main symptom is abnormal copper accumulation mainly in the liver and brain [1,2]. WD shows a wide range of clinical manifestations, including hepatic disease, neurological abnormality, psychiatric disorder, and Kayser-Fleischer rings (copper deposition on the periphery of the cornea) [3].…”

“…Several reports on pregnant patients with WD have been reported, wherein patients with well-controlled WD have had successful pregnancy outcomes with continued disease treatment and close monitoring [1,2,[4][5][6][7]. In recent retrospective study of pregnancy in WD, liver dysfunction during pregnancy has been observed in 6% of patients, and it had normalized after delivery [7].…”

Wilson disease – Challenge for safe anesthesia!

“…Although use of different anesthetic techniques including general anesthesia as well as regional anesthesia have been reported in patients of Wilson's disease but there is no clear consensus regarding the safest technique. [345] General anesthesia has certain disadvantages as it may aggravate the already impaired hepatic function due to reduction in total hepatic blood flow and toxic effects of anesthetic drugs on liver function. In other way, deranged hepatic function can adversely alter the whole pharmacokinetics of several anesthetics drugs including muscle relaxants, analgesics and sedatives.…”