No abstract

The effect of hypothermic cardiopulmonary bypass techniques on the sickling process was evaluated in patients with sickle cell hemoglobin. It was presumed that intraoperative hemolysis, as identified by hemoglobinuria, reflected increased sickling. Data of 43 patients with sickle cell traits and 2 with sickle cell disease, who were operated on under cardiopulmonary bypass and cold cardioplegic arrest in a tertiary center from the beginning of 1995 to the end of 2004, were retrospectively analyzed. A mean nasal temperature of 30.8 degrees C +/- 2.1 degrees C was achieved. Three patients with sickle cell trait developed intraoperative hemoglobinuria, albeit with normal surrogate values for hemolysis. However, they had significantly lower mean hemoglobin levels during cardiopulmonary bypass compared to those sickle cell patients who did not exhibit hemoglobinuria (hemoglobin, 6.0 +/- 0.2 vs. 7.4 +/- 0.9 g x dL(-1), p < 0.01). Total drainage and blood transfusion requirements in patients with normal and sickle cell hemoglobin were similar. It was concluded that hypothermic cardiopulmonary bypass with cold cardioplegia is safe in sickle cell patients. Maintenance of adequate hemoglobin levels during cardiopulmonary bypass may be important to avoid triggering a sickling process.

Methemoglobinemia could cloud a turbulent postoperative course in infant cardiac surgery by interfering with the diagnosis of various critical conditions. Issues surrounding the treatment with methylene blue in the presence of glucose-6-phosphate dehydrogenase (G-6-P-D) deficiency are highlighted.

Premature male ischiopagus tetrapus twins weighing 2.2 kg were born unexpectedly. They required initial ventilation for respiratory distress syndrome and an emergency laparotomy for intestinal obstruction at 10 days of age. At 8 months of age, formal separation was carried out. Primary skin closure was possible without the use of any tissue expander. Anaesthesia was supplemented with continuous intra- and post-operative epidural analgesia, with great benefit. Both twins are alive and well 18 months after surgery. Details of the anatomical findings and preparation for surgery are discussed.

Premature male ischiopagus tetrapus twins weighing 2.2 kg were born unexpectedly. They required initial ventilation for respiratory distress syndrome and an emergency laparotomy for intestinal obstruction at 10 days of age. At 8 months of age, formal separation was carried out. Primary skin closure was possible without the use of any tissue expander. Anaesthesia was supplemented with continuous intra- and post-operative epidural analgesia, with great benefit. Both twins are alive and well 18 months after surgery. Details of the anatomical findings and preparation for surgery are discussed.