Hemolysis Following Intraoperative Cell Salvage Replacement in a Scoliosis Patient With Sickle Cell Trait: A Case Report.

MINI: Intraoperative cell salvage (ICS) is used to reduce blood loss in scoliosis spinal fusion. We report one case of hemolysis induced acute renal failure following ICS. This is the first reported case of acute renal failure associated with hemolysis following ICS in a scoliosis patient with sickle cell trait. STUDY DESIGN: Case report. OBJECTIVE: To describe a novel presentation of acute renal failure associated with hemolysis after intraoperative cell salvage (ICS) in a neuromuscular scoliosis patient with sickle cell trait (SCT). SUMMARY OF BACKGROUND DATA: Hemolysis-associated acute renal failure after ICS in patients with SCT has not been previously reported. Sickle cell disease is regarded as a relative contraindication for ICS due to the risk of red blood cell sickling in the hypoxic cell saver reservoir. A previous case series demonstrated successful ICS reinfusion after elective caesarean section in two patients with SCT. However, a decision to not reinfuse ICS collected blood due to increased sickling after blood processing was reported in general surgery. METHODS: A 14-year-old female with Group I neuromuscular scoliosis underwent a navigated T3-S1posterior spinal instrumentation fusion. Three hundred milliliters of blood collected by ICS was reinfused intraoperatively along with two units of packed red blood cells. RESULTS: Postoperatively, the patient had delayed emergence from the general anesthetic and gross hematuria was observed in the urinary catheter bag. The patient was transferred to the intensive care unit and was treated successfully for hemolysis-induced renal failure. CONCLUSION: Given the potential seriousness of hemolysis-associated acute renal failure associated with ICS, we recommend against the use of ICS in patients with SCT. LEVEL OF EVIDENCE: 5.

Vitreoretinal management and surgical outcomes in proliferative sickle retinopathy: a case series.

PURPOSE: To report the outcomes of current vitreoretinal surgical management of proliferative sickle retinopathy and to compare current methods to previous studies. DESIGN: A retrospective, interventional case series. METHODS: Fifteen eyes of 14 patients with proliferative sickle retinopathy were managed with vitreoretinal surgery over a 12-year period at a single institution. RESULTS: Nine patients had a sickle cell-hemoglobin C (SC) profile, 1 was sickle cell-beta(+) thalassemia (S beta(+)-thal), and 4 were sickle cell trait (AS). All 15 eyes underwent pars plana vitrectomy (PPV): 6 for vitreous hemorrhage (VH), 1 for epiretinal membrane (ERM), and an additional 8 for tractional retinal detachment (RD) and/or rhegmatogenous RD. In addition, an encircling scleral buckle (SB) was used in 2 cases. In 7 cases, 20 gauge PPV was used; 23 gauge was used in 3; and 25 gauge was used in 5. All 7 eyes with VH or ERM had improved vision postoperatively. Four of the 8 patients with traction and/or rhegmatogenous RD developed recurrent detachments and required a second operation. All retinas were attached at last follow-up, and visual acuity was 20/400 or better in all eyes. No cases of anterior segment ischemia were encountered. CONCLUSIONS: Anterior segment ischemia is no longer a common occurrence in eyes undergoing surgery for proliferative sickle retinopathy. Although PPV has replaced the use of SB in many situations, an encircling SB may still be used in this population when necessary. Surgery for VH and ERM generally results in favorable outcomes, but eyes undergoing surgery for traction/rhegmatogenous RD carry a more guarded prognosis.

Sickle cell and renal transplant: a national survey and literature review.

Sickle cell disease is an inherited, structural hemoglobin defect with multisystemic sequelae including renal failure. Patients with sickle cell disease are thought to benefit from renal transplant, but the long-term outcomes in such patients are unclear and have not been supported by any large prospective studies. Similarly, the renal morbidity and outcome after transplant in patients with sickle cell trait is also unclear. There is little evidence concerning living donation in donors with sickle cell disease or sickle cell trait, either for the donor health or for the graft outcome, and there are no United Kingdom guidelines. The aim of this study is to review the evidence surrounding renal transplant in recipients and donors with sickle syndromes and to determine the attitudes and current practices of United Kingdom transplant centers to performing such operations.

Retinal photocoagulation for proliferative sickle cell retinopathy: a prospective clinical trial with new sea fan classification.

PURPOSE: To compare the clinical outcome of stage III proliferative sickle cell retinopathy (PSR) treated by peripheral retinal scatter photocoagulation to natural course disease. METHODS: Long-term follow-up of 101 patients enrolled in a prospective trial of photocoagulation for PSR has been completed. Among 202 eyes of 101 patients enrolled at the University Eye Clinic of Créteil, 73 eyes showed a stage III PSR, which the authors further divided into five new grades (A, B, C, D, E) considering size, hemorrhage, fibrosis, and visible vessels. Grading was based on a three-mirror fundus examination, 360 degrees color photographs, and fluorescein angiography. Mean follow- up was 4 years. RESULTS: Thirty-eight treated eyes and 35 untreated eyes were included in this study. The evolution was not statistically significant between treated and untreated groups concerning flat sea fan <1 MPS disc area (grade A) or elevated sea fan with partial fibrosis (grade C). Progression and regression were compared between the two groups for grade B, resulting statistically significant (p<0.05). Nine complications (13%) were observed, which only occurred in untreated patients with elevated sea fan and hemorrhage (grade B) or complete fibrosed sea fan with well defined vessels (grade E) (p<0.05). CONCLUSIONS: These data suggest that patients with grade A or C new sea fan classification should not be initially treated but observed.

Prognostic significance of early vaso-occlusive complications in children with sickle cell anemia.

Sickle cell anemia (SS) is highly phenotypically variable, and early predictors of outcome could guide clinical care. To determine whether early vaso-occlusive complications predicted subsequent adverse outcomes in the Dallas Newborn Cohort, we studied all members with SS or sickle-beta0-thalassemia who presented in their first year of life and had 5 years or more of follow-up. We defined 3 potential early predictors: hospitalizations in the first 3 years of life for (1) painful events other than dactylitis, (2) dactylitis, and (3) acute chest syndrome (ACS). We studied the associations of these predictors with the following late adverse outcomes (occurring after the third birthday): death, first overt stroke, use of disease-modifying therapy, and hospitalizations for pain events and ACS. None of the early events predicted death or stroke. Early pain and ACS both predicted a modest, temporary increase in the number of later painful episodes, but early ACS strongly increased the odds of more frequent ACS throughout childhood. Dactylitis had limited utility as a predictor. Although we still lack a useful prognostic framework for young children with SS, those who experience early ACS might be candidates for higher risk interventions to mitigate or cure their disease.

Fast-track cardiac anesthesia in patients with sickle cell abnormalities.

UNLABELLED: We conducted a retrospective review of 10 patients with sickle cell trait (SCT) and 30 patients (cohort control) without SCT undergoing first-time coronary artery bypass graft surgery with cardiopulmonary bypass. Demographic, perioperative management, and outcome data were collected. Both groups were matched according to age, weight, duration of surgery, and preoperative hemoglobin (Hb) concentration. Distribution of gender, medical conditions, pharmacological treatment, and preoperative left ventricular function were similar between the groups. The comparisons were analyzed in respect to postoperative blood loss and transfusion rates, as well as duration of intubation, intensive care unit, and hospital length of stay (LOS). All patients underwent fast-track cardiac anesthesia. A combination of cold crystalloid and blood cardioplegia was used. The lowest nasopharyngeal temperature was 33 degrees C. There were no episodes of significant hypoxemia, hypercarbia, or acidosis. None of the patients had sickling crisis during the perioperative period. The postoperative blood loss was 687 +/- 135 vs 585 +/-220 mL in the SCT and control groups, respectively. The trigger for blood transfusion during cardiopulmonary bypass was hematocrit <20% and Hb <75 g/L postoperatively. Three SCT patients (30%) and 10 control patients (33%) received a blood transfusion. Median extubation time was 4.0 vs 3.9 h; intensive care unit LOS was 27 vs 28 h; and hospital LOS was 6.0 vs 5.5 days in the SCT and control groups, respectively. There were no intraoperative deaths. One patient in the SCT group died from multiorgan failure 2 mo after surgery. IMPLICATIONS: Fast-track cardiac anesthesia can be used safely in patients with sickle cell trait undergoing first-time coronary artery bypass graft surgery. Extubation time and intensive care unit and hospital length of stay are comparable to those of matched controls, and blood loss and transfusion requirements are not increased. A hematocrit of 20% seems to be a safe transfusion trigger during cardiopulmonary bypass in these patients.

Septic complications after splenectomy for sickle cell sequestration crisis.

Patients with sickle cell disease (SCD) are predisposed to infections. There is a paucity of recent information on the incidence of post-splenectomy infectious complications in these patients. The purpose of this study was to determine whether splenectomy increases infectious complications in SCD. Twenty-nine patients with SCD had splenectomy for sequestration crises at our hospital between 1988 and 1992; 16 of them received all of their follow-up care at our institution. These 16 charts were reviewed for infectious-related admissions, hospital days, days of i.v. antibiotics, positive cultures, and episodes of sepsis. For each patient, these parameters in the pre- and postoperative period were compared and expressed as number per year. The mean age at time of splenectomy was 2.5 +/- 0.4 years and the mean follow-up was 4.5 +/- 0.4 years. There was no significant difference in the pre- and postoperative periods for admissions, hospital days, days of i.v. antibiotics, positive cultures, or episodes of sepsis per year. There were also no operative deaths. The incidence of pre-splenectomy sepsis was 0.04 +/- 0.03 episodes per year compared to 0.09 +/- 0.04 (P = ns) episodes/year after splenectomy. Sepsis occurred at an average of 20.8 (range 2-30) months postoperatively; Streptococcus pneumoniae was the most common causative organism. The total mortality after splenectomy in SCD patients was 3.4% (1/29) over a nearly 5-year period. Although infections are common in children with SCD, there was no increase in infections or episodes of sepsis in SCD patients who underwent splenectomy.

Priapism in sickle cell disease: the case for early implantation of the penile prosthesis.

OBJECTIVES: Recurrent ischemic priapism in sickle cell (SS) patients often leads to impotence. Blockage of venous outflow by sickle cells leads to anoxia of the cavernosal smooth muscle with subsequent replacement of the erectile tissue with dense fibrosis. This study evaluates the efficacy of early penile prosthesis implantation in patients with priapism associated with sickle cells disease. METHODS: Our recent experience includes 6 SS patients with impotence resulting from repeated episodes of priapism and 1 SS patient who suffered penile autoamputation from prolonged tricorporeal low flow priapism. The average age was 26 years. Six patients underwent excision of fibrotic cavernosal sinusoidal tissue and placement of a malleable prosthesis. The autoamputation case was managed with a one-stage penile reconstruction using a forearm free-flap and a two-piece inflatable penile prosthesis. RESULTS: All patients in our series are currently potent. Because of infection, 1 patient required removal and subsequent reinsertion of his prosthesis, and another has required revision. CONCLUSION: Potency in the young SS patient with recurrent episodes of ischemic priapism may best be managed by a penile prosthesis. Early implantation may lessen the psychological trauma of repeated priapisms, and reduce the technical difficulties and complications associated with penile prosthetic insertion in the presence of dense fibrosis.

Myocardial revascularization in a sickle-cell trait patient.

A 56-year-old man with confirmed sickle-cell trait AS underwent successful triple-vessel myocardial revascularization with internal mammary artery and vein conduits. Variations in routine technique included limitation in hypothermia (32 degrees C), avoidance of intraoperative autologous salvage, hypoxia, severe anemia (< 25% hematocrit), acidosis and postoperative reinfusion of mediastinal drainage.

Acceleration in linear growth after splenectomy for hypersplenism in homozygous sickle cell disease.

Chronic hypersplenism in homozygous sickle cell (SS) disease markedly increases haemolysis and the resulting erythropoietic expansion is likely to have a high metabolic cost. Splenectomy for hypersplenism in SS disease is followed by highly significant changes in haematological indices and an increase in height, but not weight, velocity. This pattern is similar to that observed in the trichuris dysentery syndrome after treatment, and differs from the increases in both height and weight velocity that follow nutritional supplementation of severely malnourished children. It is postulated that accelerated linear growth after the reduction in erythropoietic stress may implicate a specific nutrient deficiency in hypersplenic children with SS.

Acceleration in linear growth after splenectomy for hypersplenism in homozygous sickle cell disease

Chronic hypersplenism in homozygous sickle cell (SS) disease markedly increases haemolysis and the resulting erythropoietic expansion is likely to have a high metabolic cost. Splenectomy for hypersplenism in SS disease is followed by highly significant changes in haematological indices and an increase in height, but not weight, velocity. This pattern is similar to that observed in the trichuris dysentery syndrome after treatment, and differs from the increase in both height and weight velocity that follow nutritional supplementation of severely malnourished children. It is postulated that accelerated linear growth after the reduction in erythropoietic stress may implicate a specific nutrient deficiency in hypersplenic children with SS (AU)

Renal medullary carcinoma. The seventh sickle cell nephropathy.

Over the last 22 years, we have encountered 34 examples of a highly aggressive neoplasm with a microscopic morphology that is highly predictive of finding sickled erythrocytes in the tissue. With the exception of one patient, all are believed to have had sickle cell trait or, in one case, hemoglobin SC disease. These 33 patients are the subject of this report and, where their race was known, they were all blacks between the ages of 11 and 39 years. Between the ages of 11 and 24 years, males predominated by 3 to 1. Beyond age 24, however, the tumors occurred equally in men and women. The dominant tumor mass was in the medulla and ranged from 4 to 12 cm in diameter. Mean size was 7 cm; median, 6 cm. Peripheral satellites in the renal cortex and pelvic soft tissues, as well as venous and lymphatic invasion, were usually present. The lesions exhibited a reticular, yolk sac-like, or adenoid cystic appearance, often with poorly differentiated areas in a highly desmoplastic stroma admixed with neutrophils and usually marginated by lymphocytes. The tumors had usually metastasized when first discovered, and none was confined to the kidney at the time of nephrectomy. The mean duration of life after surgery was 15 weeks. These tumors probably arise in the calyceal epithelium in or near the renal papillae, the same site that produces the more familiar picture of unilateral hematuria in patients with sickle cell trait. We have concluded that renal medullary carcinoma represents another example of renal disease associated with sickle cell disorders. The other six are unilateral hematuria, papillary necrosis, nephrotic syndrome, renal infarction, inability to concentrate urine, and pyelonephritis.

Use of a tourniquet in patients with sickle-cell disease.

Fifteen patients, 13 male and two female, known to be carrying the sickle-cell gene (12 HbSS and 3 HbAS), who were undergoing operations requiring a bloodless field, were included in the study. Of the 12 with HbSS, seven had haemoglobin A1 component of between 11 and 27%, three had fetal haemoglobin ranging from 5.7 to 29% and the remaining two had increased haemoglobin A2 concentrations suggesting a beta non-thalassaemia combination. All had a tourniquet applied to the appropriate limb and were given general anaesthesia with moderate hyperventilation throughout the procedure. The tourniquet inflation time was 61.7 +/- 27.5 min. The mean PaO2 remained above 200 mmHg, mean PaCO2 was less than 37 mmHg, and pH ranged between 7.40 and 7.45. There were no clinically important changes in BP or ECG. All patients made uneventful recoveries and none developed sickle-cell crises. It is suggested that it is safe to use tourniquet in patients with sickle-cell disease provided optimum acid-base status and oxygenation are maintained throughout the procedure.

Partial splenectomy in sickle cell syndromes.

Partial splenectomy was carried out in four children with homozygous sickle cell disease and eight children with sickle cell beta thalassaemia. It was performed in order to preserve splenic contribution to the host defence against infections while suppressing hypersplenism or the risk of recurrence of acute splenic sequestration. Indications for this surgical operation were acute splenic sequestration (n = 1), hypersplenism (n = 5), and acute splenic sequestration and hypersplenism (n = 6). Surgery was uneventful in 11 patients. A significant reduction of blood requirements and a significant decrease of the number of hospitalisations/patient/year were observed after splenectomy. No recurrence of hypersplenism or acute splenic sequestration occurred and no severe infection was noticed during the follow up period after surgery (mean (SD) 4.2 (2.8) years; range 6 months-7 years). Mean haemoglobin concentration and leucocyte and platelet counts increased after surgery. The benefit of partial splenectomy compared with total splenectomy to treat acute splenic sequestration or hypersplenism in sickle cell disease is discussed.

Otolaryngologic manifestations of sickle cell disease.

Sickle cell disease is a common hereditary hematologic disorder characterized by the presence of a structurally abnormal hemoglobin molecule. Physicochemical properties of sickle hemoglobin result in a chronic hemolytic anemia and in vaso-occlusive episodes with ischemic injury of many tissues. In patients with sickle cell disease the otolaryngologist may be confronted with lesions affecting the ear, the bones and soft tissues, and the nervous system. Surgery and anesthesia also present special problems. An outline and discussion of these clinical concerns are presented.