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PREOPERATIVE AND ANESTHETIC CONTROL IN A PATIENT WITH CLASSIC HOMOCYSTINURIA

D. Bolado López De Andújar1 , D. Fajardo Puig2 , S. Gutiérrez Barrera1 ,

C. Díez-Picazo Garot3 , C. Bodas Folguera4 , M. caballero Sánchez5 ,

L.  Rojo Sanz5 , L. Solana López5

1 12 de Octubre University Hospital, Faculty of Medicine, UCM; 2 Department of Bio- chemistry and Molecular Biology, Faculty of Medicine, UCM; 3 Gregorio Marañón Uni- versity Hospital, Faculty of Medicine, UCM; 4 CIEMAT, Faculty of Pharmacy, UCM; 5 Department of Biochemistry and Molecular Biology, Faculty of Pharmacy, UCM

Introduction: Classic homocystinuria is a pathology characterized by a mutation in the enzyme cystathionine-beta-synthase that produces an accumulation of homocysteine in plasma which increases hemodynamic risk in a basal situation and even more in surgery.

Methods: This is a 10-year-old male with a history of classic homocystin- uria and recurrent convulsive episodes who is diagnosed of cataracts. Dur- ing the 72 hours prior to surgery, the patient is kept hyperhydrated and 5 mg of promethazine and intravenous phenytoin are administered one hour before. Results: The patient is monitored and 0.1 mg of glycopyrrolate, 2 mg of mor- phine, 70 mg of monosodium thiopental and 2.5 mg of vecuronium bro- mide were administered. Anaesthesia is maintained with 0.5% halothane

and nitrous oxide, and low molecular weight dextran (Lomodex 70) is admin- istered to improve venous return, along with intravenous phenytoin to prevent convulsions. For extubating, 25 mg of tramadol was administered with 2 mg of ondansetron. The residual neuromuscular block was eliminated with 1 mg of neostigmine and 0.2 mg of glycopyrrolate.

Conclussions: When operating a patient with this type of metabolic disease, a special surgical algorithm must be considered because if we maintain a standard hydration protocol the risk of hemodynamic instability increases. The fact of taking into account the metabolopathy of the patient makes vary the chosen anaesthetic protocol. It’s essential that the surgical team knows perfectly the patient’s underlying disease before entering the operating room.

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