Anaesthetic Management with Homocystinuria

Abstract

Homocystinuria is a metabolic and multisystemic disorder often caused by cystatyonin β-sentase deficiency. We have noticed mental retardation, skeletal system malformation, oculer disorders, hypoglycemia and severe thromboembolic complication in this disease. Patients with homocystinüria often needs oculer surgery under general anesthesia. A good preoperative preperation, avoiding hypoglycemia and thromboemboli perioperatively and having precautions about these help to reduce the anesthetic or surgical complication. In our document, we aimed to report our anaesthetic approaches that we applied to two patients with homocystinuria for oculer surgery under general anesthesia. It is possible to prevent the expected complications by the help of precautions that are taken in the preoperative period. No complication about thromboemboli or hypoglycemia is observed during operation or postoperative period in both patients

Keywords

• Homocystinuria, general anesthesia, thromboembolism

HOMOSİSTİNÜRİDE ANESTEZİK YAKLAŞIM

Abstract

Homosistinüri otozomal resesif geçiş gösteren, metiyonin metabolizmasında rol oynayan enzim ya da enzimlerin defektinden kaynaklanan ve birden çok sistemik bozuklukla seyreden, metabolik bir hastalıktır. Kliniğinde mental retardasyon, iskelet anomalileri, gözle ilgili problemler, hayatı tehdit eden tromboembolik komplikasyonlar görülür. Homosistinürili olgularda genel anestezi altında oküler cerrahiye sıklıkla ihtiyaç duyulmaktadır. Preoperatif iyi bir hazırlık yapılması, perioperatif tromboemboli ve hipogliseminin önlenmesi ve bunlara yönelik tedbirlerin alınması ile anestezi ve cerrahinin neden olabileceği komplikasyonlar azaltılabilir. Yazımızda homosistinüri tanısı konulmuş iki olgunun oküler cerrahisi sırasında uyguladığımız anestezik yaklaşımları bildirmeyi planladık. Operasyon öncesi ve perioperatif dönemde gerekli önlemleri alarak uyguladığımız anestezi sonucunda her iki olgumuzda da intraoperatif ve postoperatif tromboembolizm, hipoglisemi veya başka bir komplikasyon izlenmemiştir

Keywords

• Homosistinüri, genel anestezi, tromboembolizm

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