The Greek e-journal of Perioperative Medicine

3-11

Authors: Sivrioğlu D, Tuç Ö, Ersoy Z, Çekmen N, Torgay A

Glycogen storage disease type Ia (GSD-Ia; Von Gierke disease) is an inherited disease caused by glucose-6-phosphatase deficiency. Perioperative management of patients with GSD has important implications for anaesthesiologists due to different system involvements. In the case reported here, an 8-year-old patient with GSD type-Ia underwent liver transplantation (LT) for hepatic adenoma that had malignant transformation, severe hypoglycemia and metabolic acidosis, occurred in this patient intraoperatively. The metabolic complications were managed successfully by administering balanced volume of glucose and sodium bicarbonate intravenously (iv). Although liver transplantation is an important treatment option in these patients, anesthesia management requires a multidisciplinary approach, comprehensive perioperative assessment and close intraoperative follow-up for successful outcomes.

19-23

Authors: Kandemir E, Çekmen N, Torgay A, Haberal M

Glycogen storage disease type 1a is a rare inborn error of metabolism. It causes severe fasting intolerance and lactic acidosis due to the deficiency of glucose-6-phosphatase enzyme. Anesthetic management of these patients is very difficult due to their course with different system involvements. Our case report evaluates the anesthetic management and considerations of a glycogen storage disease type 1a patient with air embolism during liver transplantation. Although orthotopic liver transplantation is an important treatment option in these patients, anesthesia management requires a multidisciplinary approach and care should be taken in terms of perioperative complications.

31-36

Authors: Tuç Ö, Bihorac E, Ersoy Z, Çekmen N

Hereditary angioedema (HAE) is a life-threatening rare autosomal dominant (AD) disorder caused by C1-esterase inhibitor (C1-INH) deficiency or dysfunction. HAE is not related to mast cell and histamine pathway; as a result, it does not respond to antihistamines, glucocorticoids, or epinephrine. Attacks may occur spontaneously or after a trauma. The upper airway swelling and laryngeal edema cause asphyxia, which can be life-threatening. Treatment of HAE includes C1-INH concentrate, recombinant human C1-INH, bradykinin B2-receptor antagonists, recombinant plasma kallikrein inhibitors, fresh frozen plasma (FFP) and androgen steroids. In this case we report anesthetic management of a 62 years old female patient with HAE, undergoing endoscopy and colonoscopy, for which highly selective α2 adrenergic receptor agonist dexmedetomidine intravenous (IV) infusion was used. We administered icatibant (bradykinin B2-receptor antagonist) for prophylaxis, and FFP was prepared in case of use. Anesthetic management and preoperative preparation are critical for the patient with HAE. Difficult airway management should be kept in mind in case of airway edema and early intubation.