[Splenic surgery in hematological diseases : Indications and surgical technique]. / Chirurgie der Milz bei hämatologischen Erkrankungen : Indikationen und operative Technik.

BACKGROUND: Splenic surgery in hematological disorders requires a well-weighted decision on the indications because the medical treatment has rapidly changed in recent years due to new pharmaceutical approaches. OBJECTIVE: Summary of the indications, surgical procedures and perioperative management regarding operative interventions on the spleen in hematological disorders. MATERIAL AND METHODS: Selective literature search and summary of reviews and guideline recommendations. RESULTS: In hematological disorders surgical procedures of the spleen (splenectomy and partial splenectomy) are an important part of the repertoire in the treatment. In recent years the indications for surgery have become narrower because of new forms of medicinal treatment. Especially in hereditary spherocytosis, immune thrombocytopenia and symptomatic splenomegaly and hypersplenism it is still of importance. The minimally invasive splenectomy is regarded as the gold standard. The spleen has an important immune and sequestration function, therefore preoperative and postoperative infectious and thromboembolic events have to be anticipated and prevented. A close interdisciplinary cooperation with hematologists is essential for an optimal outcome of patients. CONCLUSION: The minimally invasive splenectomy and partial splenectomy are part of the surgical repertoire in the diagnostics and treatment of hematological disorders. Because of novel medicinal approaches the therapeutic protocols are continuously changing. A close cooperation with hematologists is important for the optimal evaluation of the indications and the perioperative management.

Exploring parents' experience in the decision-making process for children with haematological diseases receiving haematopoietic stem cell transplantation in China based on genograms.

PURPOSE: To explore parents' experience in the decision-making process for children with haematological diseases receiving haematopoietic stem cell transplantation (HSCT) based on genograms. DESIGN AND METHODS: A qualitative study based on genogram was conducted. Seven pairs of parents completed their genogram and a semi-structured interview. Two related donors were interviewed to discuss their experiences in the decision-making process of donating bone marrow to their loved ones. Interviews were transcribed verbatim and analysed using interpretative phenomenological analysis. RESULTS: The genogram presents the complete family structure, family member relationships and incident-related conflicts. Two superordinate themes emerged from the data analysis. The first theme was transplantation decision motivations, including four subthemes: the values of life priority, empathy based on kinship, inheritance of family blood, trust in doctors and medical science. The second theme was transplantation decision conflicts, including four subthemes: decision-making conflicts among family members, related-donor decision-making conflicts, risk-benefit game, blind optimism and insufficient cognition conflict. CONCLUSIONS: Genogram can show the hidden resources and conflicts of each family and help the care providers better understand parents' experience and the decision-making dilemmas in the decision-making process for children with haematological diseases receiving HSCT. The needs of transplant families for treatment-related information were not fully met. PRACTICE IMPLICATIONS: It is necessary for medical staff to increase psychological and informational support for donors and their families when the parents of patients make bone marrow transplant decisions. Medical staff should engage in family-focused systematic interventions to reduce the psychological burden and conflicts of decision makers.

Evaluación de los trasplantes de progenitores hematopoyéticos bajo cobertura del fondo nacional de recursos / Evaluation of hematopoietic stem cell transplants covered by the national resource fund

INTRODUCCIÓN: El trasplante de precursores hematopoyéticos (TPH) ha evolucionado durante los últimos 50 años. Implica la infusión de células progenitoras hematopoyéticas en pacientes con trastornos hematológicos malignos o no malignos con el objetivo de restablecer la función hematopoyética e inmunitaria normal. También es una opción de tratamiento potencialmente curativo en pacientes portadores de tumores sólidos (1). Los primeros trasplantes se realizaron en gemelos idénticos, pero con el advenimiento de nuevas técnicas que permitieron conocer el complejo mayor de histocompatibilidad se amplió el espectro de trasplantes a donantes relacionados y no relacionados, tanto histoidénticos como aquellos con algún grado de incompatibilidad. OBJETIVOS: Evaluar los trasplantes de precursores hematopoyéticos realizados bajo cobertura financiera del Fondo Nacional de Recursos. METODOLOGÍA: Se trata de un estudio retrospectivo, de la cohorte de pacientes en quienes se realizó un TPH bajo cobertura financiera del FNR en el periodo comprendido entre el 25 de abril 1996 al 31 de diciembre 2018. RESULTADOS: a) Unidad de análisis: Solicitudes de trasplantes. En el periodo desde el inicio cobertura de TPH del 28 de abril de 1995 al 30 de diciembre 2019, se solicitaron 3160 TPH, de los cuales se autorizaron un total de 2902, no autorizados 250 y pendientes al momento del corte 8 trámites. b) Unidad de análisis: Pacientes. Características de las cohortes, tiempos y medianas de seguimiento. En el periodo en que se analizaron efectivamente las realizaciones de TPH, del 25 de abril de 1996 al 27 de diciembre 2018 se autorizaron 2699 solicitudes, y se realizaron efectivamente 2356 TPH. DISCUSIÓN: El Trasplante de progenitores hematopoyéticos es un procedimiento terapéutico que se encuentra en expansión tal cual lo muestran los dos registros internacionales más importantes, el europeo (European Society for Blood and Marrow Transplantation, EBMT) y el americano (Center for International Blood and Marrow Transplant Research, CIBMTR). Por otra parte, con el advenimiento de nuevas terapias innovadoras, para algunas patologías ocurrió un descenso significativo del número de trasplantes realizados por año. La Leucemia Mieloide crónica y su respuesta a inhibidores de tirosina quinasa es el ejemplo más representativo. De todas formas, esta técnica continuó incrementándose. Los factores principalmente asociados a este incremento son la flexibilización de la edad de los candidatos a un trasplante, el mayor número de donantes, y la ausencia de avances significativos en algunas de sus principales indicaciones como lo son la Leucemia Aguda y los Síndromes Mielodisplásicos. Esta evaluación representa más de 20 años de experiencia adquirida en procedimientos financiados por el FNR en esta técnica. El número de autorizaciones de trasplantes por año evidencia un incremento en la última década, así como el número de trasplantes efectivamente realizados de un 12.2 % (considerando los últimos 20 años) Este incremento es a expensas de los TPH alogénicos (incremento de un 57.2 % considerando las cohortes 1999-2008 y 2009-2018), concordante con lo que también ocurre a nivel internacional. En nuestra evaluación los trasplantes autólogos muestran un ligero descenso en la última década en torno a un 8.3 %. En concordancia con esto el reporte de CIBMTR del 2018 evidenció un descenso de los TPH autólogos de un 5%, aunque se habían mantenido estables los cuatro años prévios. Se trata de una población joven, con media de edad de 39 años, mayormente proveniente del sector privado de la salud. La proporción de TPH autólogo fue de un 81.4 % y el alogénico 18.6 % en forma global. En otros registros tanto de la región como del propio registro europeo muestran mayor proporción de TPH alogénicos realizados, próximos a un 40%. En nuestra evaluación se ha mantenido en estas mismas proporciones a lo largo de los años, tanto globalmente como en los diferentes institutos de medicina altamente especializada. Destacamos en el caso del IMAE en Hospital Maciel y Asociación Española para el periodo 2014- 2018 presentaron una proporción mayor de TPH alogénicos realizados. En nuestra evaluación dentro de los TPH alogénicos, la modalidad TPH alogénico "relacionado" fue el más frecuente (67.6%). Dentro de las otras dos modalidades, el TPH Haploidentico es el segundo en proporción (20.3%), a pesar de haberse incorporado ultimo dentro de la normativa del FNR.

Long-Term Results of Total Hip Arthroplasty in Young Patients With Osteonecrosis After Allogeneic Bone Marrow Transplantation for Hematological Disease: A Multicenter, Propensity-Matched Cohort Study With a Mean 11-Year Follow-Up.

BACKGROUND: The number of young patients with hematological disease requiring total hip arthroplasty (THA) is expected to increase. We aimed to investigate the long-term THA outcomes in patients with osteonecrosis of the femoral head (ONFH) following allogeneic bone marrow transplantation (BMT) for hematological disease. METHODS: All patients who underwent THA for osteonecrosis after BMT from 1997 to 2012 were identified at 2 institutions. Using propensity scores, 75 THAs in 45 patients were matched for age, gender, body mass index, American Society of Anesthesiologists score, and year of surgery with 75 THAs in 58 patients with idiopathic ONFH without a history of hematological disease (1:1 ratio). The mean age at surgery was 36.7 years and 52% were men. Clinical and radiographic evaluations were performed and clinical scores were obtained at last follow-up. Kaplan-Meier analyses were used to compare survivorship. RESULTS: At a mean follow-up of 10.6 ± 3.5 years, clinical, radiographic, and survivorship outcomes, and the Harris hip scores were similar between both groups. The 13-year survivorship for all-cause revision was 93.4% for the BMT group and 95% for the control group (P = .928). No significant differences were observed between groups in the rates of reoperation (4% vs 5.3%, P = 1.000), 90-day readmission (all 5.3%), or overall mortality (4.4% vs 1.7%, P = .681). No hips had periprosthetic joint infection or septic loosening in either group. Osteolysis occurred in none of the BMT patients and in 2 hips (2.7%) of the control patients (P = .497). CONCLUSION: This large cohort multicenter survey at 11-year follow-up shows that contemporary cementless THA in young hematological disease patients after allogeneic BMT is not associated with a higher risk for surgical complications, revision, reoperation, readmission, and mortality compared to a matched cohort of idiopathic ONFH.

Long-term Outcomes of Sequential Hematopoietic Stem Cell Transplantation and Kidney Transplantation: Single-center Experience.

BACKGROUND: Experience with sequential hematopoietic stem cell transplant (HSCT) and kidney transplant (KT) is limited. METHODS: We conducted a retrospective observational study of adult patients who underwent both HSCT and KT at our center, with a median follow-up of 11 y. RESULTS: In our 54 patients cohort (94% autologous HSCT), 36 (67%) patients received HSCT first followed by KT, while 18 (33%) received KT before HSCT. In both groups, AL amyloidosis represented 50% of hematologic diagnosis. Only 4 patients expired due to hematologic disease relapse (2 patients in each group) and only 3 allografts were lost due to hematologic disease recurrence (HSCT first n = 1 and KT first n = 2). Overall 1, 5, and 10 y death-censored graft survival rates were 94%, 94%, and 94%, respectively, for the HSCT first group and 89%, 89%, and 75%, respectively, for the KT first group. Overall 1, 5, and 10 y patients survival rates were 100%, 97% and 90%, respectively, for the HSCT first group and 100%, 76%, and 63%, respectively, for the KT first group. CONCLUSIONS: Our study supports safety of sequential KT and HSCT, with improved overall patient survival compared to recipients of HSCT remaining on dialysis and good long-term kidney allograft outcome.

Laparoscopic Splenectomy in Children: Safety of Ligasure Usage.

INTRODUCTION: Laparoscopic splenectomy started to be more popular and nowadays it is the first choice for splenectomy in children. The main advantages of laparoscopic splenectomy over open approach are the shorter hospital stay, low complications and the cosmetic results despite the main concern of long operative time and the intra operative bleeding. AIM: The aim of this study was to report our experience with laparoscopic splenectomy in children's with hematological diseases using ligasure regarding vascular control safety, efficacy and complications rate. METHODS: A retrospective study was carried out at Queen Rania Al-Abdullah Hospital for Children over the period from January 2017 to February 2019. Total number of 18 patients with hematological diseases underwent laparoscopic splenectomy using ligasure. Demographic data, complications and outcomes were collected to assess the advantages of ligasure usage for vascular control safety. RESULTS: Out of 18 patients underwent laparoscopic splenectomy using ligasure (10 with spherocytosis, 4 with immune thrombocytopenic purpura [ITP], 3 with sickle cell anemia, 1 with hemangioma), from which number, 8 patients were males and 10 were females. Mean age was 6.7 years (ranges from 4.3 to13.7 years). Mean operative time was 107 minutes (ranges from 70-180 minutes). There was no conversion to open splenectomy and no mortality. Intra operative blood loss was insignificant. CONCLUSION: Laparoscopic splenectomy using ligature is safe with excellent results in decreasing operative time, less complications and decrease incidence of intraoperative bleeding.

Comparison of Laparoscopic Splenectomy Outcomes for Benign and Malignant Hemopathies.

Background: Laparoscopic splenectomy for malignant hemopathies has been associated with a higher morbidity than for benign hemopathies. Recent progress in medical and surgical treatment for malignant hemopathies may have improved the outcomes of laparoscopic splenectomy. The purpose of this study is to compare the outcomes of laparoscopic splenectomy for malignant hemopathies (SHM) and benign hemopathies (SHB). Materials and Methods: We retrospectively reviewed all patients with hematological diseases who underwent a non-post-traumatic laparoscopic splenectomy between 2008 and 2019. Patients who suffered splenectomy for a malignant and benign disease were divided into two groups and compared. Results: Fifty patients suffered a splenectomy for hematologic disorder, 19 patients for benign hemopathy, and 31 for malignant hemopathy. SHM group was significantly older, and had more history of abdominal surgery and significantly larger spleens (P < .05). There was no significant difference in terms of operative time (150 versus 146 minutes; P < .8) and blood loss (243 versus 402 mL; P < .26). Hospital stay for SHB and SHM groups was 5.4 and 7.6 days, respectively (P = .19). There was no significant difference in terms of early (10% versus 13%; P = 1) and late complications (0% versus 13%; P = .28). One conversion to open surgery and one perioperative death in each group (P = 1) were reported. Splenectomy effectiveness was 83% and 79% in benign hemopathy and malignant hemopathy groups (P = .91), respectively. Conclusions: Laparoscopic splenectomy for malignant hemopathy shows similar outcomes to laparoscopic splenectomy for benign hemopathy, despite older patients, larger spleens, and more important abdominal surgery history. Higher late morbidity rate after laparoscopic splenectomy for malignant hemopathy may justify a careful follow-up.

IMPACT OF HLA-DPB1 MATCHING ON CLINICAL OUTCOMES AFTER HAPLOIDENTICAL-RELATED HEMATOPOIETIC STEM CELL TRANSPLANTATION.

BACKGROUND: The impact of HLA-DPB1 compatibility and its role as a transplantation antigen in haploidentical-related hematopoietic stem cell transplant (haplo-R-HSCT) have not been established, and a negative effect on survival has been suggested. OBJECTIVE: The objective of the determine was to study the frequency and clinical effects of incompatibility at the HLA-DPB1 locus in the haplo-R-HSCT setting. METHODS: Clinical records and electronic files of 91 patients with a hematological disease who underwent haplo-HSCT from January 2009 to October 2017 in a university medical center were scrutinized. Overall survival (OS) was estimated by the Kaplan-Meier method; the cumulative incidence of transplant-related mortality (TRM) and relapse rates was determined. Acute graft-versus-host disease was assessed by binary logistic regression. Cox regression model with a 95% confidence interval was used to examine the association between the different variables and their effect on OS. RESULTS: Of the 91 donor-recipient pairs, 24 (26.37%) shared complete DPB1 identity, 60 (65.93%) had a mismatch at one allele, and 7 (7.70%) were mismatched at two alleles. Twenty-four different HLA-DPB1 alleles were found; the most frequent were DPB1*04:01 (34.1%) and DPB1*04:02 (27.5%). Two-year OS, the cumulative incidence of TRM and relapse was 51.3 ± 6.8%, 28 ± 6% and 60 ± 7.8% for all haplo-related transplants, respectively, with no statistical difference between HLA-DPB1 matched and partially matched patients. In Cox regression analysis, no risk factors associated with OS, TRM, or relapses were identified. CONCLUSION: HLA-DPB1 mismatching in the haplo-R-HSCT setting did not influence transplant outcomes and was clinically tolerable. A high degree of homozygosity was found.

Splenectomy in childhood for non-malignant haematologic disorders - long-term follow-up shows minimal adverse effects.

Splenectomy is considered therapeutic in various non-malignant haematologic diseases. Adverse events - specifically infections and thromboembolism - are not extensively documented in the paediatric population, maintaining the concern over risks-versus-benefits of the procedure. We studied a cohort of paediatric haematology patients undergoing splenectomy between 1977 and 2015 to determine short- and long-term complications. We summarised all the patients of the haematology clinic in our major Israeli tertiary centre undergoing splenectomy for therapeutic reasons, capturing infectious and thromboembolic events. The data of 103 patients, comprising 1657 follow-up years, were analysed. The cohort included 33 patients with transfusion-dependent thalassaemia, seven with non-transfusion-dependent thalassaemia, four with sickle-thalassaemia, 41 with hereditary spherocytosis, and 18 with immune thrombocytopenia. Standard presplenectomy vaccinations were noted in most. No typical cases of overwhelming postsplenectomy infection (OPSI) were identified, nor were typical OPSI bacteria isolated. Thalassaemics with central lines were most prone to infection and thrombosis. Beyond this subgroup, thrombotic events were anecdotal. This is the largest study to date to comprehensively analyse infectious and thrombotic complications of childhood splenectomy for the treatment of haematologic diseases. The use of splenectomy appears to be a relatively safe therapeutic option in paediatric patients with proper preoperative vaccination and follow-up care; use of central venous lines or catheters increase the risk in thalassaemic patients and should be avoided if possible.

Impact of hHLA-DPB1 matching on clinical outcomes after haploidentical-related hematopoietic stem cell transplantation

ABSTRACT Background: The impact of HLA-DPB1 compatibility and its role as a transplantation antigen in haploidentical-related hematopoietic stem cell transplant (haplo-R-HSCT) have not been established, and a negative effect on survival has been suggested. Objective: The objective of the determine was to study the frequency and clinical effects of incompatibility at the HLA-DPB1 locus in the haplo-R-HSCT setting. Methods: Clinical records and electronic files of 91 patients with a hematological disease who underwent haplo-HSCT from January 2009 to October 2017 in a university medical center were scrutinized. Overall survival (OS) was estimated by the Kaplan-Meier method; the cumulative incidence of transplant-related mortality (TRM) and relapse rates was determined. Acute graft-versus-host disease was assessed by binary logistic regression. Cox regression model with a 95% confidence interval was used to examine the association between the different variables and their effect on OS. Results: Of the 91 donor-recipient pairs, 24 (26.37%) shared complete DPB1 identity, 60 (65.93%) had a mismatch at one allele, and 7 (7.70%) were mismatched at two alleles. Twenty-four different HLA-DPB1 alleles were found; the most frequent were DPB1*04:01 (34.1%) and DPB1*04:02 (27.5%). Two-year OS, the cumulative incidence of TRM and relapse was 51.3 ± 6.8%, 28 ± 6% and 60 ± 7.8% for all haplo-related transplants, respectively, with no statistical difference between HLA-DPB1 matched and partially matched patients. In Cox regression analysis, no risk factors associated with OS, TRM, or relapses were identified. Conclusion: HLA-DPB1 mismatching in the haplo-R-HSCT setting did not influence transplant outcomes and was clinically tolerable. A high degree of homozygosity was found.

Multivariate analysis on complications of central venous access devices in children with cancer and severe disease influenced by catheter tip position and vessel insertion site (A STROBE-compliant study).

CONTEXT: Reliable long-term central venous access device (CVAD) is essential for the management of pediatric patients with cancer or chronic diseases. However, there is no general consensus for optimal catheter tip location and vessel insertion site in children. OBJECTIVE: This single center study analyzes the risk of complications associated with long-term upper body CVAD and evaluates them with respect to catheter tip location as well as vessel insertion site. DESIGN: Pediatric patients who received long-term upper body CVAD from January 2008 through April 2017 and underwent radiographic documentation of the tip location were retrospectively included in the study. Data on demographics, catheter tip location on chest x-ray, intraoperative vessel insertion sites and postoperative complications were analyzed. Catheter tip location was categorized as "high" (above the right mainstem bronchus), "medium" (at the level of the bronchus), and "low" (below the right mainstem bronchus). Distance to the carina was measured as well. RESULTS: A total of 396 patients, 74.7% suffering from cancer were included in our study (mean age 6.3 ± 0.3 years). Complications occurred in about one fourth of all patients. Catheter-related blood stream infections (BSI) (n = 40, 36.4%) were most prevalent, but catheter tip position or vessel insertion site had no impact on the risk of infections. Dislodgement (n = 27, 24.6%) and occlusion (n = 11, 10.0%) were more frequent in "high" positioned catheter tips. While there was one patient who developed arrhythmia, no case of cardiac perforation, and in particular, no catheter-related death was recorded in our series. The vessel insertion site seemed to have no influence on the complication frequency of CAVDs. CONCLUSION: The catheter tip position seems to have an impact on the catheter-related complication profile in children. To avoid complications, we recommend avoiding a "high" localization of the catheter tip above the right main bronchus. "Low" catheter tip placement was associated with the lowest dislocation rate. Given the overall low complication rate, insertion and use of CVADs in children can generally be considered as safe.

Open Versus Laparoscopic Splenectomies in Children: A Comparative Study Performed at a Public Hospital in Brazil.

Introduction: In pediatric population, the two most common indications for splenectomy include traumatic rupture and hematological diseases such as hereditary spherocytosis, idiopathic thrombocytopenic purpura, sickle cell disease, and autoimmune hemolytic anemia. Traditionally, splenectomy has been an open procedure; however, since the first laparoscopic splenectomy was reported in 1991, the minimally invasive laparoscopic approach has become increasingly popular. In most public hospitals in Brazil, where vessel sealing devices are not available, most surgeons seem to prefer open splenectomy to guarantee intraoperative safety and improved outcomes. Objectives: To compare outcomes between open and laparoscopic splenectomy in children in a public hospital in Brazil. Materials and Methods: Retrospective study conducted between January 2010 and June 2018. Patients from 0 to 14 years old who underwent open or laparoscopic splenectomy at the University Hospital of Federal University of Paraná were included. Clinical, laboratory, imaging, and surgical data were collected. Results: For 8 years, 35 patients underwent splenectomy. Mean age was 4 years old and 54% were female. Of the 35 subjects, 69% had sickle cell anemia and 23% spherocytosis. The most common indication for surgery was a previous episode of splenic sequestration. The 13 laparoscopic surgeries were performed without harmonic scalpel or other vessel sealing devices. During the laparoscopic procedure, 2 patients experienced intraoperative complications: bleeding and prolonged surgical time due to technical problems with the equipment. Mean operative time was higher in laparoscopy group than in open group (186 minutes versus 66 minutes). Oral feeds began earlier on the laparoscopic group. Postoperatively, there were more complications on the open group, and no reoperations. There was only one fatality, likely secondary to fulminant sepsis, which occurred 34 days after the surgery in a patient who was undergoing prophylactic oral antibiotics therapy. Discussion: Performing laparoscopic splenectomy without harmonic scalpel or other vessel sealing devices is feasible, but it implicates in a significantly higher surgical time. Laparoscopic splenectomy had earlier oral feeds and fewer complications than open surgery.

Favorable Outcome of Lung Transplantation for Severe Pulmonary Graft Versus Host Disease: An Australian Multicenter Case Series.

BACKGROUND: Severe pulmonary chronic graft versus host disease (GVHD) is a life-threatening complication of allogeneic hematopoietic stem cell transplantation. Few treatments influence outcome, with 5-year overall survival as low as 13%. Lung transplantation (LTx) has been reported in small numbers of patients worldwide. METHODS: We investigated the outcomes of LTx performed for this indication at 2 large Australian LTx centers. RESULTS: Eighteen patients (aged 10-64 y; median, 29.6 y) received bilateral deceased lung transplants for pulmonary chronic GVHD between 2002 and 2017. LTx was performed at a median of 8.6 years after allogeneic stem cell transplantation (range, 2-23 y) with a median interval of 16 months from the time of transplant unit review to LTx. There were 2 early infective deaths and 3 further deaths from pulmonary infection and lung allograft rejection. There were no primary disease relapses. At a median follow-up of 5 years, the 5-year overall survival post-LTx is 80% and comparable to the Australia and New Zealand registry data of 64% for LTx performed for all indications. CONCLUSIONS: From one of the largest series of deceased LTx for this indication, we conclude that it is a feasible option for selected patients with severe pulmonary GVHD. The outcomes appear superior to that of non-LTx-based therapies and similar to the survival of the general LTx population. Establishing guidance on referral triggers, patient eligibility, organ selection, prophylaxis of allograft rejection, and supportive care would assist hematopoietic and lung transplant units in optimizing resource allocation and patient outcomes.

Laparoscopic Splenectomy: Has It Become the Standard Surgical Approach in Pediatric Patients?

BACKGROUND: Splenectomy is often required in the pediatric population as part of the treatment of hematologic disorders and can be performed laparoscopically or open. We evaluated the comparative effectiveness of laparoscopic (LS) and open (OS) splenectomies using the American College of Surgeons National Surgical Quality Improvement Program Pediatric (NSQIP-P) data set. METHODS: The NSQIP-P data set was used to identify children who underwent elective splenectomy between January 2012 and December 2016. Thirty-day outcomes between OS and LS, and LS alone and concurrent LS and cholecystectomy were compared using univariate and multivariate analysis. RESULTS: Most of the splenectomies (91%) were performed laparoscopically. There was no difference in overall complications between OS (n = 60) and LS (n = 613), although OS had a higher risk of perioperative transfusion (OR 3.19, 95% CI 1.52-6.69). LS was associated with a shorter median hospital length of stay (2 versus 4 d, P < 0.001) and similar mean operative times compared to OS (120 versus 133 min, P = 0.559). There was no difference in outcomes of children undergoing LS versus LS and concurrent cholecystectomy (n = 129). CONCLUSIONS: LS has become the standard approach for elective splenectomies in the pediatric population and has minimal morbidity, and when indicated, concurrent cholecystectomies do not increase the risk of complications. LEVELS OF EVIDENCE: III.

Prognostic significance of abnormal hematological parameters in severe traumatic brain injury requiring decompressive craniectomy.

OBJECTIVE: Hematological abnormalities after severe traumatic brain injury (TBI) are common, and are associated with a poor outcome. Whether these abnormalities offer additional prognostic significance over and beyond validated TBI prognostic models is uncertain. METHODS: This retrospective cohort study compared the ability of admission hematological abnormalities to that of the IMPACT (International Mission for Prognosis and Analysis of Clinical Trials) prognostic model to predict 18-month neurological outcome of 388 patients who required a decompressive craniectomy after severe TBI, between 2004 and 2016, in Western Australia. Area under the receiver operating characteristic (AUROC) curve was used to assess predictors' ability to discriminate between patients with and without an unfavorable outcome of death, vegetative state, or severe disability. RESULTS: Of the 388 patients included in the study, 151 (38.9%) had an unfavorable outcome at 18 months after decompressive craniectomy for severe TBI. Abnormalities in admission hemoglobin (AUROC 0.594, p = 0.002), plasma glucose (AUROC 0.592, p = 0.002), fibrinogen (AUROC 0.563, p = 0.036), international normalized ratio (INR; AUROC 0.645, p = 0.001), activated partial thromboplastin time (AUROC 0.564, p = 0.033), and disseminated intravascular coagulation score (AUROC 0.623, p = 0.001) were all associated with a higher risk of unfavorable outcome at 18 months after severe TBI. As a marker of inflammation, neutrophil to lymphocyte ratio was not significantly associated with the risk of unfavorable outcome (AUROC 0.500, p = 0.998). However, none of these parameters, in addition to the platelet count, were significantly associated with an unfavorable outcome after adjusting for the IMPACT predicted risk (odds ratio [OR] per 10% increment in risk 2.473, 95% confidence interval [CI] 2.061-2.967; p = 0.001). After excluding 8 patients (2.1%) who were treated with warfarin prior to the injury, there was a suggestion that INR was associated with some additional prognostic significance (OR 3.183, 95% CI 0.856-11.833; p = 0.084) after adjusting for the IMPACT predicted risk. CONCLUSIONS: In isolation, INR was the best hematological prognostic parameter in severe TBI requiring decompressive craniectomy, especially when patients treated with warfarin were excluded. However, the prognostic significance of admission hematological abnormalities was mostly captured by the IMPACT prognostic model, such that they did not offer any additional prognostic information beyond the IMPACT predicted risk. These results suggest that new prognostic factors for TBI should be evaluated in conjunction with predicted risks of a comprehensive prognostic model that has been validated, such as the IMPACT prognostic model.

Congenital Refractory Glaucoma: A New Ophthalmic Association of Kabuki Syndrome and its Management With Glaucoma Drainage Devices.

INTRODUCTION: Kabuki syndrome (KS) is a rare congenital and polymalformative condition, traditionally associated with mental retardation, unusual facial features, and skeletal abnormalities. We hereby describe a case of bilateral congenital glaucoma associated with MLL2-mutation KS. To the best of our knowledge, this is the first association of KS with congenital glaucoma. CASE REPORT: The patient was a 3-year-old male of North African ethnicity diagnosed with KS and bilateral congenital glaucoma at the age of 3 months and the first child of a nonconsanguineous healthy couple, with no known genetic conditions within the family. The patient was referred to our tertiary glaucoma center with uncontrollable intraocular pressures between 50 and 60 mm Hg in both eyes. Past ophthalmic history included bilateral trabeculectomies and right retinal detachment with phthisis bulbi. We performed left lensectomy combined with Baerveldt tube insertion in the vitreous cavity, revealing extensive posterior synechiae. Postoperatively, he developed intense inflammation with fibrin and vitreous strand formation, requiring subsequent surgeries and subconjunctival injections of betamethasone. Despite these complications, IOP stabilized between 3 and 6 mm Hg. DISCUSSION: This case confirms congenital glaucoma as a rare association of KS, and highlights the potential high-risk nature of such cases. In our opinion, the presence of high levels of inflammation perioperatively and postoperatively is an indication for primary glaucoma drainage device surgery given the high risk of bleb scarring resulting in recurrent surgical revisions. In view of these observations, we also advocate the use of intensive postoperative anti-inflammatory therapy.

[Esplenectomía laparoscópica, una opción eficaz en un hospital de segundo nivel]. / Laparoscopic splenectomy, an efficient option in a second level hospital.

INTRODUCTION: Laparoscopic splenectomy was reserved for selected cases, however and because of the improvement in surgical technique and laparoscopic instruments, nowadays, there are few contraindications. METHOD: Retrospective and observational study of patients surgically treated of laparoscopic splenectomy secondary to hematologic diseases in a period span of 6 years. We analyzed demographic, preoperative, transoperative and postoperative variables. RESULTS: We studied eight patients, 62.5% were men with a mean age of 47.5 years. The most frequent hematologic diagnosis was idiopathic thrombocytopenic purpura (50%). The approach was laparoscopic in 100%. Median surgical time was 129.87 min and median transoperative bleeding was 396.25 cc. Median length of resected spleen was 15 cm. Median time for oral feeding was 20.62 h and total hospital stay was 4 days. There was only one reported complication. CONCLUSION: Laparoscopic splenectomy represents a safe and feasible option with rapid recovery and few complications.

INTRODUCCIÓN: La esplenectomía laparoscópica era reservada para pacientes sin esplenomegalia y enfermedades benignas; sin embargo, debido a la mejoría en la técnica y en los instrumentos de laparoscopía, actualmente las contraindicaciones son pocas. MÉTODO: Estudio retrospectivo y observacional de pacientes sometidos a esplenectomía laparoscópica por enfermedades hematológicas refractarias durante 6 años. Se analizaron variables demográficas, preoperatorias, transoperatorias y posoperatorias. RESULTADOS: Se estudiaron ocho pacientes, de los cuales el 62.5% eran hombres y la edad promedio era de 47.5 años. El diagnóstico hematológico más común fue púrpura trombocitopénica idiopática (50%). El abordaje fue laparoscópico en el 100% de los casos. El tiempo quirúrgico fue 129.87 minutos. El sangrado transoperatorio fue de 396.25 ml. El tamaño de los bazos resecados fue de 15 cm. El tiempo para el inicio de la vía oral fue de 20.62 horas y la estancia intrahospitalaria fue de 4 días. Se reportó una complicación. CONCLUSIÓN: La esplenectomía laparoscópica es un procedimiento seguro y factible, con recuperación rápida y menos complicaciones.

Is ITP really a desirable indication for teaching laparoscopic splenectomy? Cohort study.

Background: We aimed to evaluate the outcomes of laparoscopic splenectomy (LS) in patients with immune thrombocytopenic purpura (ITP) compared with operated for other indications. Methods: Retrospective cohort study of patients who underwent LS in 1998-2017. Group 1 consisted of 256 patients operated for ITP, and Group 2 of 231 operated for other indications. Primary outcome was procedure difficulty. Secondary was perioperative course (30 days of surgery). Results: Patients in Group 1 were younger (p < .001) with lower ASA (p < .001). The spleen was larger in Group 2 (p < .001). Operative time was longer in Group 2 [110 (80-150) vs. 90 (65-115) min; p < .001)]. Intraoperative blood loss was greater in Group 2 (p < .001). Conversions were higher in Group 2 (4.76 vs. 6.93%, p = .037), as were intraoperative complications (7.79 vs. 3.91%, p = .048). Morbidity, reoperations and mortality did not differ between groups (respectively, p = .899, .697, and .999). Median length of stay was similar (p = .211). Among the first 10 LSs performed by trainees, 63.66% were done for ITP. Later (after 11+ procedures) this dropped to 48.12% (p = .002). Conclusions: ITP may be a preferred indication for surgeons training for LS. Patients' characteristics and intraoperative factors allow an expectation for a relatively easy and hassle-free operation in those patients.

Red Blood Cell Disorders: Perioperative Considerations for Patients Undergoing Cardiac Surgery.

Disorders affecting red blood cells (RBCs) are uncommon yet have many important physiologic considerations for patients undergoing cardiac surgery. RBC disorders can be categorized by those that are congenital or acquired, and further by disorders affecting the RBC membrane, hemoglobin, intracellular enzymes, or excessive RBC production. A foundational understanding of the physiologic derangement for these disorders is critical when considering perioperative implications and optimization, strategies for cardiopulmonary bypass, and the rapid recognition and treatment if complications occur. This review systematically outlines the RBC disorders of frequency and relevance with an emphasis on how the disorder affects normal physiologic processes, a review of the literature related to the disorder, and the implications and recommendations for patients undergoing cardiac surgery.