

e-Journal – Issue 2022d
• LETTER FROM THE EDITORS
• ANAESTHETIC MANAGEMENT IN A PEDIATRIC PATIENT WITH MALIGNANT TRANSFORMATION OF HEPATIC ADENOMA IN GLYCOGEN STORAGE DISEASE TYPE 1 (VON-GIERKE) FOR LIVER TRANSPLANTATION: A CASE REPORT.
• NON-OPERATING ROOM ANESTHESIA (NORA) MANAGEMENT AND VACTERL SYNDROME: A CASE REPORT.
• ANESTHETIC MANAGEMENT OF A GLYCOGEN STORAGE DISEASE TYPE 1A WITH AIR EMBOLISM DURING LIVER TRANSPLANTATION.
• INTRAOPERATIVE DIAGNOSIS OF AN ASCENDING


Letter from the Editors
Dear colleagues The fourth issue of the Greek e-journal of Perioperative Medicine for 2022 presents various case reports concerning perioperative medicine. The case of Sivrioğlu D et al. presents the perioperative management of an 8-year-old patient with Glycogen storage disease type Ia (GSD type-Ia), with malignant transformation of hepatic adenoma, that underwent liver transplantation. The authors note that the anesthetic management of these patients has important implications for


Anaesthetic Management In A Pediatric Patient With Malignant Transformation Of Hepatic Adenoma In Glycogen Storage Disease Type 1 (Von-Gierke) For Liver Transplantation: A Case Report
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.


Non-Operating Room Anesthesia (NORA) Management And VACTERL Syndrome: A Case Report
VACTERL syndrome presents many challenges related to anesthesia management due to multiple congenital malformations that may include vertebral, cardiac, tracheo-esophageal, renal and extremity anomalies.


Anesthetic Μanagement Of A Glycogen Storage Disease Type 1a With Air Embolism During Liver Transplantation
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.


Intraoperative diagnosis of an ascending Aorta dissection in a patient with gradually collapsing cardiovascular system: An example of how the role of perioperative transesophageal echocardiography (TEE) becomes crucial.
Although Computed Tomographic Angiography (CTA) is the established gold standard test for the detection of an aortic dissection, many researches and studies have proofed and established that transesophageal echocardiogram (TEE) can rule in or rule out the diagnosis of this catastrophic disorder, with greater accuracy.


Anesthesia management of a patient with hereditary angioedema under Dexmedetomidine: A Case Report.
Hereditary angioedema (HAE) is a life-threatening rare autosomal dominant (AD) disorder caused by C1-esterase inhibitor (C1-INH) deficiency or dysfunction.


Anesthetic Management In A Patient With Crigler-Najjar Syndrome Undergoing A Dental Procedure: A Case Report
Crigler Najjar Syndrome (CNS) is an ultra-rare autosomal recessive inherited disorder caused by a mutation in the uridine glucronyl 5’-diphosphate-glucronyl transferase (UGT) gene.


e-Journal – Issue 2022b
• ΕΠΙΣΤΟΛΗ ΤΗΣ ΣΥΝΤΑΞΗΣ
• ΜΑΙΕΥΤΙΚΗ ΑΙΜΟΡΡΑΓΙΑ ΚΑΙ ΔΙΑΤΑΡΑΧΕΣ ΑΙΜΟΣΤΑΣΗΣ: ΠΕΡΙΕΓΧΕΙΡΗΤΙΚΗ ΔΙΑΧΕΙΡΙΣΗ ΑΙΜΟΘΕΡΑΠΕΙΑΣ
• ΔΙΕΓΧΕΙΡΗΤΙΚΗ ΔΙΑΧΕΙΡΙΣΗ ΤΗΣ ΑΛΓΑΙΣΘΗΣΙΑΣ: ΙΑΤΡΙΚΗ ΚΑΙ ΒΙΟΗΘΙΚΗ ΔΙΑΣΤΑΣΗ
• HYPERTONIC SALINE RESUSCITATION IN ACUTE INTRAOPERATIVE HEMORRHAGE: CASE SERIES PRESENTATION
• CHOLECY