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
Introduction: Testis is essential for male sexual hormone production, fertility, and psychological well-being of a male. In the unfortunate event of testicular loss, placement of a testicular prosthesis perhaps will offer a sense of well-being, improved body image, and overall confidence in the growing child. Aims: The aim is to evaluate the feasibility and assessment of outcomes following concurrent placement of testicular prosthesis in children following orchiectomy. Materials and Methods: This is a cross-sectional study conducted by reviewing the reports of patients who underwent simultaneous insertion testicular prosthesis following orchiectomy for various indications between January 2014 and December 2020 at tertiary hospitals in Bengaluru. Children <18 years were included in this study. A transscrotal approach was preferred in cases where a transscrotal orchiectomy was done. Transinguinal approach was preferred in children undergoing prosthesis insertion as an isolated procedure. The size of the prosthesis was chosen based on the age of the child and the size of the scrotum. Outcomes were assessed on follow-up. Results: A total of 29 children underwent prosthesis insertion (25 unilateral and four bilateral). The mean standard deviation age was 5.58 (3.92) years. The indications for prosthesis insertion were cryptorchidism with atrophic testis (22), torsion (3), Leydig cell tumor (2), and severely virilized Congenital adrenal hyperplasia (CAH) (2). Of these, three children (9%) had complications (wound gaping in two and wound infection in one) that needed removal of the implant. The mean duration of follow-up was 49.23 months. All the parents reported a good outcome, and none of the children who underwent prosthesis placement needed a change during this follow-up. Conclusion: Concurrent placement of a testicular prosthesis is technically easy and a safe procedure, achieves satisfactory cosmetic appearance with minimal morbidity.