Splenectomy in Gaucher's disease. Apropos of 2 cases, one of which was preceded by embolization

Two cases of Gaucher's disease type I are reported Splenectomy was indicated because of hypersplenism and massive splenomegaly. In one case hypersplenism was treated with pre-operative selective embolization because of the volume of the spleen (20 kg). The embolization corrected the thrombopenia but not the size of the spleen. Four years after operation for case 1 and eight months for case two, there is an improvement in the clinical status.     

Gaucher's disease

This is a review of the classification, pathophysiology, and clinical manifestations of Gaucher's disease. The orthopedic manifestations of the disease include bone pain syndromes, pathologic fractures, and avascular necrosis of the femoral head in children and adults.     

Spinal cord decompression for Gaucher's disease

We report an individual with Type I nonneuronopathic Gaucher's disease who experienced the rare complication of spinal cord compression secondary to a sclerotic vertebral fracture. He successfully underwent anterolateral spinal cord decompression and spinal fusion despite the severity of his generalized skeletal disease.     

Splenectomy for non-haematological metastatic malignant disease

Purpose  

Multiple cases and small series of patients who have undergone splenectomy for metastatic malignant disease have been reported. This study examines the outcome of patients with metastatic malignant disease to the spleen treated by splenectomy at a tertiary oncology centre and a review of cases published in the last 10 years.     

Splenectomy for hematologic disease. Mini-invasive versus traditional technique]

BACKGROUND: This study aimed to compare the safety, efficacy and clinical benefits of laparoscopic splenectomy (LS) to open splenectomy (OS) in patients with hematologic disorders. METHODS: EXPERIMENTAL DESIGN: prospective study; SETTING: II Department of Surgery, Santa Maria Nuova Hospital, Reggio Emilia and III Department of Surgery, Santo Spirito Hospital Pescara; PATIENTS: 48 consecutive adult patients underwent splenectomy; 30 patients under-went LS and 18 OS. Perioperative characteristics, outcomes, complications and costs were comparatively analyzed. RESULTS: Mean age was 35.3 years in the LS group, and 40.8 in the OS group. Mean spleen size was 11.7 cm in the LS group and 15.2 cm in the OS group. Accessory spleens were found in 5 patients in the LS group and in 4 patients in the OS group; 4 conversions to laparotomy occurred in the LS group. A total of 4 complications occurred in 3 patients of the LS; 9 complications occurred in 5 patients of OS group. Mean surgical time was 141.5 minutes for LS and 89.7 minutes for OS (p<0.005). Mean postsurgical stay was 5.8 days in the LS group and 8.5 days in the OS group (p<0.005). Response rates were similar in both groups. CONCLUSIONS: LS is comparable to OS in terms of efficacy and safety and it is associated with a shorter hospital stay. LS should become the technique of choice for treatment of intractable benign hematologic disease.     

Splenectomy for hematologic disease. The UCLA experience with 306 patients.

Between 1956 and 1981, 306 splenectomies for hematologic diseases were performed at the UCLA Medical Center. Of these operations, more than 75% were performed for therapeutic reasons to control anemia, thrombocytopenia, neutropenia, or painful symptoms of splenomegaly. Of the 65 patients who had idiopathic thrombocytopenic purpura, 77% showed an excellent response, and of the 39 patients who had hereditary spherocytosis, 90% responded. Other diseases with predictably good response rates were autoimmune hemolytic anemias, Felty's syndrome, and hairy cell leukemia. Forty patients with Hodgkin's disease had splenectomies for diagnostic purposes the last 10 years. The overall morbidity and mortality were 24% and 6%, respectively, the most common complications being pneumonia, wound infections, and local postoperative bleeding, and the most common cause of death being sepsis. The review supports the thesis that in carefully selected patients, therapeutic splenectomy can have desirable palliative effects and that diagnostic splenectomy has a sufficiently low risk to warrant its consideration in patients with Hodgkin's disease.     

Partial splenectomy in Gaucher's disease

In 11 children with hypersplenism due to Gaucher's disease, partial splenectomy was planned with the aim to prevent the development of postsplenectomy sepsis and also to slow the advance of the disease in the rest of the reticuloendothelial system by permitting continuing accumulation of the beta-glucocerebroside in the remaining splenic tissue. In seven children, partial splenectomy was performed successfully, the weight of the splenic tissue removed ranging from 400 to 3,680 g. The postoperative course was uneventful and the average duration of hospitalization was 12 days. In subsequent follow-up, isotope scanning demonstrated continuing growth of the splenic remnant and there were no episodes of postsplenectomy sepsis nor evidence of increased accumulation of beta-glucocerebroside in the liver or bones. These children showed a marked improvement in the growth curve and dramatic improvement in the hematologic picture. Of the four remaining children, in two, partial splenectomy was followed by complete removal of the remaining spleen due to necrosis, whereas in two, total splenectomy was performed since the huge spleens were extensively infarcted. Our experience suggests that partial splenectomy is the treatment of choice in the management of young patients with hypersplenism due to Gaucher's disease.     

Risk factors for osteonecrosis in patients with type 1 Gaucher's disease.

This study investigated 51 patients with Type 1 Gaucher's disease clinically and radiographically for the presence of osteonecrosis. Twenty-five female and 26 male patients with a mean age of 37 years were evaluated retrospectively for osteonecrosis of the proximal and distal femur, proximal tibia, and proximal humerus. All patients were examined before enzyme replacement therapy. Gender, age at diagnosis, prior splenectomy, hematocrit, platelet count, acid phosphatase level, radiographs of the long bones, and magnetic resonance quantitative chemical shift imaging of the spine were analyzed to see if any of these values or findings were associated with the presence of osteonecrosis. Splenectomy was an independent risk factor for the presence of osteonecrosis in three of the four major sites and was a multivariate risk factor for osteonecrosis of the proximal femur and tibia. Male gender was the other significant multivariate risk factor for osteonecrosis of the humerus and distal femur when all sites were taken into account.     

Long-term follow-up of partial splenectomy in Gaucher's disease.

Seven children with Gaucher's disease who underwent partial splenectomy were followed for 7 to 8 years. None of the children had systemic sepsis or symptoms related to liver enlargement. Bone crisis occurred in only two children who had experienced bone crisis prior to partial splenectomy. This contrasts with the development of bone crises in five of six children who underwent total splenectomy at a similar age and who had previously been free of bone symptoms. Partial splenectomy should be regarded as a temporary solution in the treatment of hypersplenism and the mechanical compression related to the huge spleen. In 71% of the patients, massive enlargement and a severe to moderate degree of pancytopenia occurred again after 3 to 8 years; total splenectomy was required in three patients.     

Total hip arthroplasty in Gaucher's disease. Long-term prognosis

Avascular necrosis of the femoral head is a cause of morbidity in patients with Gaucher's disease. Total hip arthroplasty (THA) is recommended, but there is controversy about the long-term value of this procedure. In eight patients treated with 15 THAs, 11 (73%) have remained fully mobile and asymptomatic up to 14 years following surgery. These long-term follow-up observations are evidence in favor of THA for treatment of hip joint problems associated with Gaucher's disease.