Abstract
Background: Hemoglobinopathies are common genetic disorders affecting the synthesis of one of the globin chains of hemoglobin molecule. Laparoscopic cholecystectomy is the standard procedure for treatment of patients with normal hemoglobin and symptomatic gall stones, but doubt is still to date regarding safety of this procedure in patients with hemoglobinopathy. Aims:1. To assess the safety of laparoscopic cholecystectomy for hemoglobinopathic patients, 2. To describe the perioperative events that might happen before, during or after this procedure. Patients and Methods: This is a record-based comparative study conducted over a period of seven years, involved 62 hemoglobinopathic patients and 148 patients with normal hemoglobin variant. All underwent laparoscopic cholecystectomy and comparison done regarding perioperative events. Results: The overall complication rate was 56.5% in hemoglobinopathic patients and 21.6% in the other group and the difference was statistically significant (P- value ˂0.001). Vaso-occlusive crisis occurred in 9 patients (14.5%), respiratory complications (atelectasis and bronchitis) in (9.7%), acute chest syndrome in (4.8%) and hemolysis in (4.8%). Conclusions: 1. Laparoscopic cholecystectomy is safe in well prepared hemoglobinopathic patients but it is associated with significantly higher rate of disease related complications, namely acute chest syndrome, hemolysis and vaso-occlusive crises.2- Hemoglobinopathic patients require special pre, intra and post-operative care which should be offered by the surgeon, the physician and the anesthetist
Highlights
Hemoglobinopathies are one of the most common autosomal recessive disorders worldwide;[1] mainly present in two patterns, either qualitative disorders (HbS) in which valine replacing glutamine in the 6th position of Beta chain, or quantitative of either Alpha or Beta chain.[2]
Laparoscopic cholecystectomy is safe in well prepared hemoglobinopathic patients but it is associated with significantly higher rate of disease related complications, namely acute chest syndrome, hemolysis and vaso-occlusive crises.2- Hemoglobinopathic patients require special pre, intra and post-operative care which should be offered by the surgeon, the physician and the anesthetist
Hemoglobin S- Beta-Thalassemia disease constitutes the biggest group of the studied hemoglobinopathic patients (29 cases) (46.8%), followed by patients with sickle cell disease (16 cases) (25.8%)
Summary
Hemoglobinopathies are one of the most common autosomal recessive disorders worldwide;[1] mainly present in two patterns, either qualitative disorders (HbS) in which valine replacing glutamine in the 6th position of Beta chain, or quantitative (reduction or deletion) of either Alpha or Beta chain.[2]. These patients may need surgical treatment so that surgeons and anesthetists have to be familiar with the physiological abnormalities and the high possibility of perioperative complications.[4] The incidence of cholelithiasis differs in different types of hemoglobinopathies; 4-85% in sickle cell anemia, 29% in Sickle Cell trait, 12% in Hemoglobin S-Beta-thalassemia disease and 30-. All patients were admitted one day before surgery except those with hemoglobin level less than 9 g/dl These cases were admitted earlier for blood transfusion. Patients were managed in the surgical wards (few cases were admitted to the intensive care unit), they received intravenous fluids, antibiotics and analgesia. They were discharged home when they were fully ambulated and started oral diet. P value of less than 0.05 was considered significant
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