Clinical outcomes of splenectomy in children: report of the splenectomy in congenital hemolytic anemia registry.

The outcomes of children with congenital hemolytic anemia (CHA) undergoing total splenectomy (TS) or partial splenectomy (PS) remain unclear. In this study, we collected data from 100 children with CHA who underwent TS or PS from 2005 to 2013 at 16 sites in the Splenectomy in Congenital Hemolytic Anemia (SICHA) consortium using a patient registry. We analyzed demographics and baseline clinical status, operative details, and outcomes at 4, 24, and 52 weeks after surgery. Results were summarized as hematologic outcomes, short-term adverse events (AEs) (≤30 days after surgery), and long-term AEs (31-365 days after surgery). For children with hereditary spherocytosis, after surgery there was an increase in hemoglobin (baseline 10.1 ± 1.8 g/dl, 52 week 12.8 ± 1.6 g/dl; mean ± SD), decrease in reticulocyte and bilirubin as well as control of symptoms. Children with sickle cell disease had control of clinical symptoms after surgery, but had no change in hematologic parameters. There was an 11% rate of short-term AEs and 11% rate of long-term AEs. As we accumulate more subjects and longer follow-up, use of a patient registry should enhance our capacity for clinical trials and engage all stakeholders in the decision-making process.

Development of a multi-institutional clinical research consortium for pediatric surgery.

BACKGROUND: Multicenter clinical research studies in pediatric surgery have been largely limited to relatively small case-series and retrospective reviews because of the rarity of many of the diseases we treat and difficulty coordinating and executing multi-institutional studies. Creation of a collaborative research network can provide the needed patient population and infrastructure to perform high quality multi-institutional studies. METHODS: In 2013, eleven academic pediatric surgery centers within the United States formed a research consortium to develop and conduct multicenter clinical research projects to advance the practice of pediatric surgery. RESULTS: We present our process for creating, developing, and maintaining this consortium including initial regional geographic limitation, charter development with by-laws and procedures for adopting studies, and research infrastructure including a central website for study monitoring and central reliance institutional review board process. CONCLUSION: Our model could be reproduced or adapted by other institutions to develop or strengthen other research collaboratives. LEVEL OF EVIDENCE: Type of study: retrospective, IV.

Hematologic outcomes after total splenectomy and partial splenectomy for congenital hemolytic anemia.

PURPOSE: The purpose of this study was to define the hematologic response to total splenectomy (TS) or partial splenectomy (PS) in children with hereditary spherocytosis (HS) or sickle cell disease (SCD). METHODS: The Splenectomy in Congenital Hemolytic Anemia (SICHA) consortium registry collected hematologic outcomes of children with CHA undergoing TS or PS to 1 year after surgery. Using random effects mixed modeling, we evaluated the association of operative type with change in hemoglobin, reticulocyte counts, and bilirubin. We also compared laparoscopic to open splenectomy. RESULTS: The analysis included 130 children, with 62.3% (n=81) undergoing TS. For children with HS, all hematologic measures improved after TS, including a 4.1g/dl increase in hemoglobin. Hematologic parameters also improved after PS, although the response was less robust (hemoglobin increase 2.4 g/dl, p<0.001). For children with SCD, there was no change in hemoglobin. Laparoscopy was not associated with differences in hematologic outcomes compared to open. TS and laparoscopy were associated with shorter length of stay. CONCLUSION: Children with HS have an excellent hematologic response after TS or PS, although the hematologic response is more robust following TS. Children with SCD have smaller changes in their hematologic parameters. These data offer guidance to families and clinicians considering TS or PS.

Excision of large cystic ovarian tumors: combining minimal invasive surgery techniques and cancer surgery--the best of both worlds.

BACKGROUND/PURPOSE: Cystic ovarian lesions can be massive, and preoperative evaluations can often not distinguish benign from malignant tumors. Up to 57% of malignant ovarian tumors have a cystic component. We present an approach to these neoplasms that adheres to oncologic principles using minimally invasive techniques. METHODS: A 5-cm Pfannensteil incision is performed followed by peritoneal washings. The mass is identified and dried. Dermabond (Ethicon, Johnson & Johnson, New Jersey) is applied to an area of the capsule (measuring 3 x 3 cm) and to a sterile plastic ultrasound bag. The bag is then applied directly to the exposed capsule. BioGlue (Cyrolife Inc, Kennesaw, GA) is then injected into and around the bag/mass interface and allowed to solidify. A veress needle decompresses the cyst, and the ovary is delivered out of the peritoneal cavity for either cystectomy or an oophorectomy. Routine surveillance of the omentum, lymph nodes, contralateral ovary, and peritoneal surface is then performed. RESULTS: Nine female patients (mean age, 14.1 +/- 2 years) were treated. All had normal alpha-fetoprotein and human chorionic gonadotropin. Computed tomographic scans demonstrated cystic lesions ranging from 8.9 to 27 cm in diameter (17.1 +/- 2.6 cm2). Operative procedures were: 4 salpingooophorectomies and 5 unilateral oophorectomies. In a single case, the contralateral ovary had a suspicious lesion, which was biopsied. No tumors spills occurred. The pathology included 2 simple cysts, 3 serous cyst adenomas, 3 mature cystic teratomas, and 1 immature teratoma with grade 2 to 3 immature elements. Peritoneal washings were negative. All patients were discharged within 48 hours and are well 15 months to 3.1 years postoperatively. CONCLUSIONS: The containment of the ovarian cyst with the application of surgical adhesives and a plastic sleeve offers a significant advancement in our ability to safely treat these lesions. This approach markedly reduces the length of the surgical incision while insuring the prevention of peritoneal contamination with cystic fluid.

Excellent outcome in patients with stage I germ cell tumors of the testes: a study of the Children's Cancer Group/Pediatric Oncology Group.

BACKGROUND/PURPOSE: The aim of this study was to correlate outcomes in patients with stage I testicular germ cell tumors with compliance to surgical guidelines and to confirm previous single-institution experiences that show excellent disease-free survival rates when treated with orchiectomy alone. METHODS: Sixty-three patients were entered into this intergroup study (Children's Cancer Group 8881/Pediatric Oncology Group 9048) between 1990 and 1996. Surgical guidelines for orchiectomy included an inguinal approach, early vascular control of the cord structures, and a hemiscrotectomy if a transscrotal violation occurred. Failure of tumor marker normalization or subsequent elevation suggested advanced disease requiring further surgery and chemotherapy. Survival curves were compared using the log-rank test. RESULTS: The median age was 16 months (range, 1 month to 5.6 years). Progression of disease occurred in 11 patients. The 6-year event-free survival (EFS) was 78.5% + 7%. The overall 6-year survival rate was 100%. Adherence to all guidelines was confirmed in only 43 patients (69%). Overall, there was no statistical significance in event-free survival rates between patients who had a correct surgical approach and all patients who had surgical violations; however, 4 patients with transscrotal violations showed a significantly higher rate of disease recurrence. CONCLUSIONS: Patients with stage I germ cell tumors of the testes have excellent survival rates when treated with surgery alone. Despite numerous surgical guideline violations, event-free survival in this multiinstitutional study was similar to previous single-institution reports. All patients with relapse or progression of their disease appear to be cured with further surgical excision and chemotherapy.