Splenic complications of the sickling syndromes and the role of splenectomy.

PURPOSE: To analyze the authors' experience with splenectomy for sickling disorders and evaluate the indications, complications, and outcome. PATIENTS AND METHODS: Over a period of 10 years (1987-1997), 113 patients with sickling disorders (100 with sickle cell disease and 13 with sickle-beta-thalassemia) had splenectomy at the authors' hospital as part of their management. The indications for splenectomy were hypersplenism (26 patients), major splenic sequestration crisis (MSSC) (23 patients), minor recurrent splenic sequestration crisis (MRSSC) (50 patients), splenic abscess (12 patients), and massive splenic infarction (2 patients). RESULTS: Splenectomy in patients with sickle cell disease (SCD) and sickle-beta-thalassemia (S-beta-Thal) was beneficial in reducing their transfusion requirements and its attendant risks, eliminating the discomfort from mechanical pressure of the enlarged spleen, and avoiding the risks of acute splenic sequestration crisis. It also was curative for patients with splenic abscess and massive splenic infarction. Twenty-four patients with SCD (24%) had splenectomy and cholecystectomy caused by concomitant gallstones. There was no mortality, and the postoperative morbidity was 7%. CONCLUSIONS: With careful perioperative management, splenectomy is both safe and beneficial in a select group of patients with SCD and S-beta-Thal.

Splenectomy for hematological diseases: The Qatif Central Hospital experience.

BACKGROUND: In the Eastern Province of Saudi Arabia, an area known for various hemoglobinopathies, splenectomy is performed rather frequently. This study is an analysis of our experience with splenectomy performed for various hematological disorders between 1988 and 1997, outlining the indications, complications and outcome. PATIENTS AND METHODS: This is a retrospective analysis of all patients who had splenectomy at our hospital during this period. One hundred and forty-three patients were treated for various hematological disorders at our hospital. These disorders included sickle cell disease (SCD) (100 patients), sickle ss-thalassemia (S-ss-thal) (13 patients), ss-thalassemia major (15 patients), Hb H disease (3 patients), idiopathic thrombocytopenic purpura (ITP) (5 patients), Gaucherâs disease (2 patients), hereditary spherocytosis (1 patient), autoimmune hemolytic anemia (1 patient), thalassemia intermediate (2 patients) and chronic myeloid leukemia (1 patient). RESULTS: The indications for splenectomy in those with SCD and S-ss-thal were: hypersplenism (26 patients), major splenic sequestration crisis (23 patients), minor recurrent splenic sequestration crisis (50 patients), splenic abscess (12 patients), and massive splenic infarction (2 patients). Splenectomy in these patients was beneficial in reducing their transfusion requirements and its attendant risks, eliminating the discomfort from mechanical pressure of the enlarged spleen, avoiding the risks of acute splenic sequestration crisis, and managing splenic abscess. For those with thalassemia, total splenectomy was beneficial in reducing their transfusion requirements, while partial splenectomy was beneficial only as a temporary measure, as regrowth of splenic remnant in these patients subsequently led to increase in their transfusion requirements. Those with ITP, hereditary spherocytosis, and autoimmune hemolytic anemia showed excellent response following splenectomy. There was no mortality, and the postoperative morbidity was 5.6%. CONCLUSION: With careful perioperative management, splenectomy is both safe and beneficial in a selected group of patients with hematological diseases.

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.

Highly selective devascularization for bleeding oesophagogastric varices.

Between 1975 and 1984, 419 patients with bleeding oesophagogastric varices were subjected to the simplified operation of highly selective devascularization. All but three were available for follow-up at 5-10 years. The overall mortality rates for urgent and elective operation were 8 and 2 per cent respectively. The overall recurrent bleeding rates at 1, 5 and 10 years were 8 per cent, 13 per cent (15 per cent of survivors) and 17 per cent (24 per cent of survivors). The overall survival rates at 1, 5 and 10 years were 87, 76 and 57 per cent. Recurrent bleeding was usually controlled by endoscopic sclerotherapy and less often by reoperation. Highly selective devascularization controlled bleeding in emergency and elective situations without compromising hepatic function.

Randomized controlled trial of injection sclerotherapy for bleeding oesophageal varices--an interim report.

Oesophageal varices are the commonest cause of acute upper gastrointestinal bleeding in Egypt, due to the prevalence not only of schistosomiasis but also chronic hepatitis. Poor results of conventional treatment and shunt surgery led us to evaluate injection sclerotherapy, using fibreoptic endoscopy. In a controlled trial, 108 patients were randomly allocated to injection sclerotherapy or to conventional treatment (medical measures, with modified splenectomy and oesophagogastric devascularization in selected cases). We report the results in the first 108 patients, with a follow-up of 1-35 months. Fifty-three patients received injection sclerotherapy; 5 died (2 of recurrent bleeding) and 5 others had recurrent bleeding but were controlled by further injections. Thirty-six of the 55 control patients underwent surgery; 5 died (2 of recurrent bleeding) and 2 others developed recurrent bleeding. Further bleeding occurred in 12 of the 19 patients who were managed by medical measures alone, with 7 dying. These early results indicate that injection sclerotherapy can be effective in urgent and elective situations and that it appears to have advantages over conventional medical and surgical treatments.