RESUMO
Context: Gaucher's disease (GD) is a rare inherited metabolic disorder caused by the defective activity of glucocerebrosidase. Enzyme replacement therapy (ERT) and substrate reduction therapy are the treatment of choice. Total splenectomy has a role when the child develops complications of massive splenomegaly. There are only a few case series of partial splenectomy in GD in the pediatric age group. Aims: To study the role, technical feasibility, and challenges of partial splenectomy in children with GD with hypersplenism. Materials and Methods: Retrospective review of children of GD who had partial splenectomy between February 2016 and April 2018. Demographics, clinical findings, laboratory, operative details, transfusion requirements, and perioperative, immediate, and late complications were retrieved. Clinical courses after discharge were obtained from follow-up data. Results: Eight children with GD underwent partial splenectomy between 2016 and 2018. The median age at surgery was 3 years and 6 months (range -2 years to 8 years). Five children underwent partial splenectomy successfully, of which one child required postoperative ventilatory support for 48 h owing to lung atelectasis. Three children underwent completion splenectomy due to bleed from the cut surface of the splenic remnant. One of the children who underwent completion splenectomy expired on the postoperative day 5 due to refractory shock with multi-organ dysfunction. Conclusion: Partial splenectomy has a definite role in selected children who present with massive splenomegaly with mechanical effects and/or hypersplenism while awaiting ERT.
RESUMO
BACKGROUND: Even though the role of thoracoscopy is well defined in Stage 2 empyema thoracis and is very popular, its role in the management of advanced empyema is still unclear. The technical difficulties and the potential complications are the principal reasons for the hesitancy in attempting video assisted thoracoscopic surgery (VATS) in advanced stages. METHODS: We prospectively studied the safety, feasibility and effectiveness of VATS for decortication in Stage 3 empyema. RESULTS: In the 61 cases that we attempted VATS over the last 7â¯years, we could complete the procedure in 45 patients (73.77%). Four children among them required re-do procedure later for persistent problems. Conversion to thoracotomy was needed in 16 patients (26.23%). The post-operative hospital stay of patients who underwent primary VATS decortication was significantly less when compared to patients requiring conversion (pâ¯<â¯0.0001). CONCLUSION: Thoracoscopy is a safe, feasible and effective option even in advanced empyema thoracis and should be offered in centers with adequate expertise and set up. LEVEL OF EVIDENCE: Level II.
