Common indications for surgery in sickle cell disease and considerations
| Surgical indication . | Surgery . | Surgical considerations . |
|---|---|---|
| Severe avascular necrosis | Total hip replacement27,28 | - High risk of periprosthetic and wound infection and respiratory complications - Monitor temperature, fluid balance, and Hb - Postoperative prophylactic anticoagulation for a minimum of 10-14 days and up to 35 days; LMWH is recommended - Early mobilization - Chest physiotherapy with incentive spirometry |
| Tonsillar hypertrophy, pediatric OSA, and/or recurrent tonsillitis | ENT surgery such as tonsillectomy and adenoidectomy14,29 | - At increased risk of nocturnal hypoxia pre- and postoperatively - Monitor for dehydration and postoperative oral fluid intake |
| Cholelithiasis | Cholecystectomy30 | - Use least invasive technique (laparoscopic) - Monitor for pulmonary complications (atelectasis, acute chest syndrome) and vaso-occlusive crisis of the liver |
| Splenic sequestration | Splenectomy31,32 | - Use least invasive technique (laparoscopic) - At risk for postsplenectomy infection; immunize with pneumococcal, meningococcal, Haemophilus influenza at least 10-14 days before surgery or postoperatively if emergency surgery is required - Consider prophylactic anticoagulation to prevent postsplenectomy portal vein system thrombosis |
| Priapism | Surgical shunt or penile prosthesis33 | - At risk for postoperative erectile dysfunction, penile gangrene, and perineal abscess |
| Pregnancy complications (eg, preeclampsia) | Caesarian section26,34 | - Hydrate and maintain spO2 > 94% - Consider transfusion to Hb 10 g/dL - Monitor fluid balance to avoid volume overload - Postpartum prophylactic anticoagulation for both vaginal and Caesarean deliveries for 6 weeks starting 6-12 hours after all delivery (no sooner than 4 hours after epidural catheter removal). LMWH and warfarin are not contraindicated with breastfeeding. |
| Moyamoya disease | Cerebral revascularization35 | - At risk for reduced cerebral blood flow due to general anesthesia - Aggressive fluid hydration prior to surgery - Utilize intraoperative EEG |
| Valvular disease | Cardiac surgery36 | - At risk for hemorrhage, stroke, renal failure, and acute chest syndrome - For valve replacement, at risk of hemolysis, thrombosis, and infection - Use incentive spirometry, anticoagulation, and prophylactic antibiotics - Maintain normothermia to minimize vasoconstriction and sickling |
| Surgical indication . | Surgery . | Surgical considerations . |
|---|---|---|
| Severe avascular necrosis | Total hip replacement27,28 | - High risk of periprosthetic and wound infection and respiratory complications - Monitor temperature, fluid balance, and Hb - Postoperative prophylactic anticoagulation for a minimum of 10-14 days and up to 35 days; LMWH is recommended - Early mobilization - Chest physiotherapy with incentive spirometry |
| Tonsillar hypertrophy, pediatric OSA, and/or recurrent tonsillitis | ENT surgery such as tonsillectomy and adenoidectomy14,29 | - At increased risk of nocturnal hypoxia pre- and postoperatively - Monitor for dehydration and postoperative oral fluid intake |
| Cholelithiasis | Cholecystectomy30 | - Use least invasive technique (laparoscopic) - Monitor for pulmonary complications (atelectasis, acute chest syndrome) and vaso-occlusive crisis of the liver |
| Splenic sequestration | Splenectomy31,32 | - Use least invasive technique (laparoscopic) - At risk for postsplenectomy infection; immunize with pneumococcal, meningococcal, Haemophilus influenza at least 10-14 days before surgery or postoperatively if emergency surgery is required - Consider prophylactic anticoagulation to prevent postsplenectomy portal vein system thrombosis |
| Priapism | Surgical shunt or penile prosthesis33 | - At risk for postoperative erectile dysfunction, penile gangrene, and perineal abscess |
| Pregnancy complications (eg, preeclampsia) | Caesarian section26,34 | - Hydrate and maintain spO2 > 94% - Consider transfusion to Hb 10 g/dL - Monitor fluid balance to avoid volume overload - Postpartum prophylactic anticoagulation for both vaginal and Caesarean deliveries for 6 weeks starting 6-12 hours after all delivery (no sooner than 4 hours after epidural catheter removal). LMWH and warfarin are not contraindicated with breastfeeding. |
| Moyamoya disease | Cerebral revascularization35 | - At risk for reduced cerebral blood flow due to general anesthesia - Aggressive fluid hydration prior to surgery - Utilize intraoperative EEG |
| Valvular disease | Cardiac surgery36 | - At risk for hemorrhage, stroke, renal failure, and acute chest syndrome - For valve replacement, at risk of hemolysis, thrombosis, and infection - Use incentive spirometry, anticoagulation, and prophylactic antibiotics - Maintain normothermia to minimize vasoconstriction and sickling |
EEG, electroencephalogram; ENT, ear, nose, and throat; spO2, oxygen saturation.