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.

Acute splenic complications and implications of splenectomy in hemoglobin SC disease.

Splenectomy indications and outcome were evaluated in 124 adults with hemoglobin SC disease (Hb SC). Twelve patients (9.6%) required splenectomy. There was a significant difference between the splenectomy group and the non-splenectomy group, respectively, regarding Hb levels (median 7.2 g/dL vs. 12.5 g/dL, P < 0.0001), platelet counts (median 146 x 10(6)/L vs. 275 x 10(6)/L, P = 0.031), palpable spleen rate (66% vs. 16%, P = 0.0003%), acute chest syndrome frequency (75% vs. 12%P = 0.0004) and cholecystectomy rate (66% vs. 13%, P = 0.0004). No significant morbidity or mortality occurred postsplenectomy. There is a subgroup of Hb SC patients requiring splenectomy, in which splenectomy is effective. Although it appears to be safe regarding short-term complications of surgery, long-term adverse effects such as infections have to be evaluated cautiously.

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.

Limb amputation in hemoglobin SC disease after application of ice and elevation.

A 31-year-old man with hemoglobin SC disease was admitted with acute chest syndrome, treated with antibiotics and ventilator support. His restrained right wrist and hand became swollen and cool, thought due to infiltration of an intravenous line. The hand was elevated, ice packs applied around the clock, and prophylactic low molecular weight heparin given. The arm quickly turned gangrenous. In spite of attempted thrombectomy and red blood cell exchange transfusion, amputation was required. Cold, elevation, and measures that reduce blood flow are known precipitators of sickling and crises. Vasoocclusion of the extremities to the point of gangrene is rarely reported in sickle cell disorders, and this case highlights the dangers of measures limiting blood flow in such patients.

Acute splenic sequestration crisis in an adult with sickle beta-thalassemia.

Acute splenic sequestration crisis (ASSC) is a major cause of morbidity and mortality in children with sickle cell disease. Reports of ASSC in adults with sickle beta-thalassemia (S-beta(thal)) are rare and consist of isolated case reports comprising a total of seven patients, three of whom died during the crisis. We report a 22-year-old man with S-beta(thal) who developed ASSC 1 day after suffering multiple blunt trauma. Systemic inflammatory response to severe blunt trauma may have precipitated ASSC in our patient. ASSC in adults with S-beta(thal) is a potentially life-threatening complication with a high risk of recurrence. Splenectomy is recommended after the first attack of ASSC in adults with S-beta(thal).

Infectious complications of implantable venous access devices in patients with sickle cell disease.

PURPOSE: To evaluate the incidence of implantable venous access device infection in patients with sickle cell disease. MATERIALS AND METHODS: The authors performed a retrospective search of their hospital's information system from January 1, 1996 to December 31, 2001 to identify hospital admissions with ICD-9 codes related to sickle cell anemia. This search yielded 2703 admissions in 293 patients. A search of the radiology information system identified 23 of these patients who had placement of an implantable venous access device. Excluding two patients who were lost to follow-up, the population of this study included eight men and 13 women aged 23 to 62 years old (mean, 37 years). A total of 30 implantable venous access devices (25 venous ports, five tunneled catheters) were placed by interventional radiologists. Cases of device infection were identified based on clinical data, microbiology, reports of device removal, and clinical follow-up. Infections were defined according to the Centers for Disease Control criteria for catheter-related bloodstream infection. The incidence of infection, organism, and time from device placement to infection was determined. RESULTS: In 21 patients with 30 devices, 18 device infections (60%) occurred in 12 patients (57%) involving 15 venous ports and three tunneled catheters. There were a total of 12389 days of catheter use and a rate of 1.5 infections per 1000 catheter days. Infections occurred from 16 to 1542 days (mean, 349 days) after device placement. Blood, wound, and catheter tip cultures yielded solitary organisms in 13 cases and mixed organisms in four cases. Staphylococcus aureus was the most common pathogen (59%). One patient was considered infected based on clinical signs and purulent discharge from the port site, despite negative cultures after partial antibiotic treatment. One patient died of sepsis resulting from an infected port. CONCLUSION: This study shows a high incidence of infection associated with placement of implantable venous access devices in patients with sickle cell disease. Therefore, the authors avoid placing these devices in this patient population.

The effect of hydroxyurea on vasculopathy in a child with sickle cell disease.

We report serial CNS findings in a girl with sickle cell disease and stroke. Religious considerations precluded transfusion and bone marrow transplantation; therefore, she received single-agent hydroxyurea therapy for almost 6 years. MR angiography showed that vascular patency improved, although diffuse cerebral atrophy slowly worsened. Hydroxyurea can be effective in treating vasculopathy, but it might not prevent the progression of parenchymal damage in advanced disease.

Surgery in patients with hemoglobin SC disease. Preoperative Transfusion in Sickle Cell Disease Study Group.

While surgery is commonly required for complications related to hemoglobin SC (HbSC) disease, little is known about the perioperative complications or the indications for preoperative transfusion in this group. We describe the patient characteristics, preoperative transfusion regimens, and outcome in 92 patients with HbSC and sickle-variants undergoing elective surgery. Thirty-eight percent of the patients were transfused preoperatively. Patients transfused were more likely to have been hospitalized in the year prior to the surgery and scheduled for abdominal procedures. Abdominal and ear, nose and throat procedures were the most common surgeries in our study. The overall complication rate was 18% and sickle cell-related complications occurred in 9% of patients. In patients undergoing intra-abdominal procedures, the incidence of sickle cell-related complications was significantly higher in those patients not transfused prior to their surgery (35 vs. 0%). There were two deaths. We recommend selective use of preoperative transfusion in patients with HbSC disease undergoing surgery. Transfusion appears to be beneficial in abdominal cases but is not necessary with minor procedures such as myringtomy.

Laparoscopic versus open cholecystectomy in children.

Twenty-one consecutive laparoscopic cholecystectomies (LC) were compared with 29 consecutive open cholecystectomies (OC). Sickle-cell disease (SCD) was the most common reason for cholecystectomy in both groups. The average length of operative time for LC was significantly longer than that of OC (P=0.0149). In 1 patient there was conversion from LC to OC due to severe adhesions. Common bile duct (CBD) stones were diagnosed in 8 (27.6%) of the OC group; in 4 of them the diagnosis was made preoperatively by ultrasound, in 4 by intraoperative cholangiogram. All 8 patients required CBD exploration, and 2 had additional transduodenal sphincteroplasties. In the LC group 5 patients (23.8%) had CBD stones. All had (ERCP) endoscopic retrograde cholangiopancreatography sphincterotomy, and stone extraction followed by LC. ERCP is a necessary adjunct to treatment if LC is to be contemplated. Six patients in the OC group developed complications, while only 4 patients in the LC group developed minor complications. The length of hospitalization after LC was significantly shorter than after OC (P=0.0150). LC is the procedure of choice in the management of cholelithiasis in children, especially those with SCD.

[Hemoglobinopathies and retinopathies in Lomé UHC]. / Hémoglobinopathies et rétinopathies au CHU de Lomé.

BACKGROUND: There are few publications concerning retinal complications of hemoglobinopathies in our country. We studied recruited patients to evaluate these complications. MATERIAL AND METHODS: From October 1993 to August 1995, 66 patients were recruited among clinical hematology out patients; all of them underwent acetate electrophoretic diagnosis and fluorescein angiographic examination. RESULTS: The mean age of the patients was 26,92 years, ranging from 5 years to 50 years; the genotypic forms were 34 SC (51.51%), 15 SS (22.72%), 11 AC (16.66%), 2 AS (3.03%), 2 CC (3.03%), and 2 A2F (3.03%). Thirty eight patients (57.57%) had retinopathies. The majority were proliferative retinopathies in 55,26% cases and in 44,73% non proliferarive retinopathies. These retinopathies were essentially present with SC (26 cases for 34 SC, 76.47%), predominantly proliferative forms (21 cases for 26 SC, 80.76%). CONCLUSION: Retinal complications are common in Togolese sickle cell patients; clinical course usually leads to proliferations. Early screening could help improve prevention, including laser photocoagulation.