Baseline characteristics of 32 patients with Gaucher disease who were treated with imiglucerase: South African data from the International Collaborative Gaucher Group (ICGG) Gaucher Registry.

BACKGROUND: Gaucher disease (GD) is a rare inherited autosomal recessive metabolic disorder with a prevalence in the general population of ~1 per 100 000. To optimise the recognition, diagnosis and management of patients with GD in South Africa (SA), it is important to have an understanding of local patterns of presentation of the disease. OBJECTIVES: To describe the baseline pretreatment characteristics of the SA cohort of patients enrolled into the International Collaborative Gaucher Group (ICGG) Gaucher Registry whowere treated with imiglucerase (Cerezyme; Sanofi Genzyme). METHODS: The ICGG Gaucher Registry is an observational, longitudinal, international database that tracks the clinical, demographic, genetic, biochemical and therapeutic characteristics of patients with GD globally, irrespective of disease severity, treatment status or treatment choice. The study population included all SA patients reported in the ICGG Gaucher Registry as of 1 May 2020. RESULTS: The registry included 49 SA GD patients, of whom 32 received imiglucerase as first primary GD therapy. All the patients had GD type 1, 59.4% were female, and mean and median ages at diagnosis were 14.7 and 9.8 years, respectively. The most common genotype was N370S/N370S (37.5%). At treatment initiation, 30.0% of patients had been splenectomised. Among patients for whom data were available, anaemia was present in one-third of non-splenectomised patients and 12.5% of those with splenectomy, and moderate or severe thrombocytopenia was reported in two-thirds of non-splenectomised patients. Bone pain was present in 30.8% and 57.1% of non- splenectomised and splenectomised patients, respectively. No bone crises were reported, and data relating to other bone complications were available for only ≤3 patients. CONCLUSIONS: Haematological findings and bone pain in this group are similar to those in the global ICGG Gaucher Registry cohort. Lack of baseline data for other bone complications limits interpretation in that regard. Clinicians who treat patients with GD are encouraged to submit accurate, complete and up-to-date information so that comprehensive data for the subset of SA GD patients can be maintained to improve recognition and diagnosis, and guide appropriate and effective use of treatment for SA patients.

Pneumococcal meningitis in adults in 2014-2018 after introduction of pediatric 13-valent pneumococcal conjugate vaccine in Japan.

We assessed the impact of the pediatric 13-valent pneumococcal conjugate vaccine (PCV13) on pneumococcal meningitis in adults in Japan in 2014-2018 by comparing epidemiological characteristics of adults with invasive pneumococcal disease with (n = 222) and without (n = 1258) meningitis. The annual incidence of pneumococcal meningitis in 2016-2018 was 0.20-0.26 cases/100,000 population. Age (p < 0.001) and case fatality rate (p = 0.003) were significantly lower in patients with meningitis than in those without meningitis. The odds of developing meningitis were higher in asplenic/hyposplenic or splenectomized patients (adjusted odds ratio [aOR] 2.29, 95% CI 1.27-4.14), for serotypes 10A (aOR 3.26, 95% CI 2.10-5.06) or 23A (aOR 3.91, 95% CI 2.47-6.19), but lower for those aged ≥ 65 years (aOR 0.59, 95% CI 0.44-0.81). PCV13 had an indirect effect on nonmeningitis, but its impact on meningitis was limited because of an increase in non-PCV13 serotypes. Of meningitis isolates, 78 (35.1%) and 3 (1.4%) were penicillin G- or ceftriaxone-resistant, respectively. We also confirmed an association of the pbp1bA641C mutation with meningitis (aOR 2.92, 95% CI 1.51-5.65).

Near Disappearance of Splenorrhaphy as an Operative Strategy for Splenic Preservation After Trauma.

BACKGROUND: Splenorrhaphy was once used to achieve splenic preservation in up to 40% of splenic injuries. With increasing use of nonoperative management and angioembolization, operative therapy is less common and splenic injuries treated operatively are usually high grade. Patients are often unstable, making splenic salvage unwise. Modern surgeons may no longer possess the knowledge to perform splenorrhaphy. METHODS: The records of adult trauma patients with splenic injuries from September 2014 to November 2018 at an urban level I trauma center were reviewed retrospectively. Data including American Association for the Surgery of Trauma splenic organ injury scale, type of intervention, splenorrhaphy technique, and need for delayed splenectomy were collected. This contemporary cohort (CC) was compared to a historical cohort (HC) of splenic injuries at a single center from 1980 to 1989 (Ann Surg 1990; 211: 369). RESULTS: From 2014 to 2018, 717 adult patients had splenic injuries. Initial management included 157 (21.9%) emergent splenectomy, 158 (22.0%) angiogram ± embolization, 371 (51.7%) observation, and only 10 (1.4%) splenorrhaphy. The HC included a total of 553 splenic injuries, of which 313 (56.6%) underwent splenectomy, while splenorrhaphy was performed in 240 (43.4%). Those who underwent splenorrhaphy in each cohort (CC vs HC) were compared. CONCLUSION: The success rate of splenorrhaphy has not changed. However, splenorrhaphy now involves only electrocautery with topical hemostatic agents and is used primarily in low-grade injuries. Suture repair and partial splenectomy seem to be "lost arts" in modern trauma care.

Survival and complications in patients with haemoglobin E thalassaemia in Sri Lanka: a prospective, longitudinal cohort study.

BACKGROUND: Worldwide, haemoglobin E ß-thalassaemia is the most common genotype of severe ß-thalassaemia. The paucity of long-term data for this form of thalassaemia makes evidence-based management challenging. We did a long-term observational study to define factors associated with survival and complications in patients with haemoglobin E thalassaemia. METHODS: In this prospective, longitudinal cohort study, we included all patients with haemoglobin E thalassaemia who attended the National Thalassaemia Centre in Kurunegala, Sri Lanka, between Jan 1, 1997, and Dec 31, 2001. Patients were assessed up to three times a year. Approaches to blood transfusions, splenectomy, and chelation therapy shifted during this period. Survival rates between groups were evaluated using Kaplan-Meier survival function estimate curves and Cox proportional hazards models were used to identify risk factors for mortality. FINDINGS: 109 patients (54 [50%] male; 55 [50%] female) were recruited and followed up for a median of 18 years (IQR 14-20). Median age at recruitment was 13 years (range 8-21). 32 (29%) patients died during follow-up. Median survival in all patients was 49 years (95% CI 45-not reached). Median survival was worse among male patients (hazard ratio [HR] 2·51, 95% CI 1·16-5·43), patients with a history of serious infections (adjusted HR 8·49, 2·90-24·84), and those with higher estimated body iron burdens as estimated by serum ferritin concentration (adjusted HR 1·03, 1·01-1·06 per 100 units). Splenectomy, while not associated with statistically significant increases in the risks of death or serious infections, ultimately did not eliminate a requirement for scheduled transfusions in 42 (58%) of 73 patients. Haemoglobin concentration less than or equal to 4·5 g/dL (vs concentration >4·5 g/dL), serum ferritin concentration more than 1300 µg/L (vs concentration ≤1300 µg/L), and liver iron concentration more than 5 mg/g dry weight of liver (vs concentration ≤5 mg/g) were associated with poorer survival. INTERPRETATION: Patients with haemoglobin E thalassaemia often had complications and shortened survival compared with that reported in high-resource countries for thalassaemia major and for thalassaemia intermedia not involving an allele for haemoglobin E. Approaches to management in this disorder remain uncertain and prospective studies should evaluate if altered transfusion regimens, with improved control of body iron, can improve survival. FUNDING: Wellcome Trust, Medical Research Council, US March of Dimes, Anthony Cerami and Ann Dunne Foundation for World Health, and Hemoglobal.

Splenic Abscess Complicating Bariatric Surgery : A Systematic Review.

INTRODUCTION: Bariatric surgery has become one of the most rapidly growing subspecialty performed globally, and it has been well reported to be associated with low morbidity and mortality rates. Splenic abscess is a rare but serious complication of bariatric surgery that has not been previously systematically reviewed in the literature. METHODS: The authors have performed a systematic review of the evidence that has looked into the pathophysiology, clinical presentation, and the management options of splenic abscess complicating bariatric surgery. RESULTS: This systematic review has been unsurprisingly based on level-IV evidence due to the rarity of the explored condition. The final analysis included 27 relevant reported cases. The mean age was 38 years and the mean of the time interval between the initial operation and developing splenic abscess was 72 days, with the male to female ratio being 1:1.6. Laparoscopic sleeve gastrectomy was the initial operation in 85.2% of the patients. Nearly half of the patients did not have an objective evidence of local or systemic sepsis that could explain the abscess formation. Nonsurgical management was attempted in 14 patients, with 34% success rate only. Splenectomy was needed in 41.7% of the patients. No mortality was reported. CONCLUSIONS: Splenic abscess is a rare and rather late but serious complication of bariatric surgery that could result in splenectomy in a relatively young group of patients. It is more commonly reported following laparoscopic sleeve gastrectomy. Early diagnosis with intervention in a timely manner is crucial to avoid life threatening complications.

Evaluation of splenic accumulation and colocalization of immature reticulocytes and Plasmodium vivax in asymptomatic malaria: A prospective human splenectomy study.

BACKGROUND: A very large biomass of intact asexual-stage malaria parasites accumulates in the spleen of asymptomatic human individuals infected with Plasmodium vivax. The mechanisms underlying this intense tropism are not clear. We hypothesised that immature reticulocytes, in which P. vivax develops, may display high densities in the spleen, thereby providing a niche for parasite survival. METHODS AND FINDINGS: We examined spleen tissue in 22 mostly untreated individuals naturally exposed to P. vivax and Plasmodium falciparum undergoing splenectomy for any clinical indication in malaria-endemic Papua, Indonesia (2015 to 2017). Infection, parasite and immature reticulocyte density, and splenic distribution were analysed by optical microscopy, flow cytometry, and molecular assays. Nine non-endemic control spleens from individuals undergoing spleno-pancreatectomy in France (2017 to 2020) were also examined for reticulocyte densities. There were no exclusion criteria or sample size considerations in both patient cohorts for this demanding approach. In Indonesia, 95.5% (21/22) of splenectomy patients had asymptomatic splenic Plasmodium infection (7 P. vivax, 13 P. falciparum, and 1 mixed infection). Significant splenic accumulation of immature CD71 intermediate- and high-expressing reticulocytes was seen, with concentrations 11 times greater than in peripheral blood. Accordingly, in France, reticulocyte concentrations in the splenic effluent were higher than in peripheral blood. Greater rigidity of reticulocytes in splenic than in peripheral blood, and their higher densities in splenic cords both suggest a mechanical retention process. Asexual-stage P. vivax-infected erythrocytes of all developmental stages accumulated in the spleen, with non-phagocytosed parasite densities 3,590 times (IQR: 2,600 to 4,130) higher than in circulating blood, and median total splenic parasite loads 81 (IQR: 14 to 205) times greater, accounting for 98.7% (IQR: 95.1% to 98.9%) of the estimated total-body P. vivax biomass. More reticulocytes were in contact with sinus lumen endothelial cells in P. vivax- than in P. falciparum-infected spleens. Histological analyses revealed 96% of P. vivax rings/trophozoites and 46% of schizonts colocalised with 92% of immature reticulocytes in the cords and sinus lumens of the red pulp. Larger splenic cohort studies and similar investigations in untreated symptomatic malaria are warranted. CONCLUSIONS: Immature CD71+ reticulocytes and splenic P. vivax-infected erythrocytes of all asexual stages accumulate in the same splenic compartments, suggesting the existence of a cryptic endosplenic lifecycle in chronic P. vivax infection. Findings provide insight into P. vivax-specific adaptions that have evolved to maximise survival and replication in the spleen.

Short- and long-term outcomes of 486 consecutive laparoscopic splenectomy in a single institution.

ABSTRACT: Since its introduction in 1991, laparoscopic splenectomy (LS) has become the gold standard in elective spleen surgery in many centres. However, there still lack the report of long-term outcomes of LS with the large-scale cases. The aim of the present study was to analyze the short- and long-term outcomes of LS in a single institution over 16 years, and to compare the perioperative outcomes of totally laparoscopic splenectomy (TLS) and hand-assisted laparoscopic splenectomy (HALS) for splenomegaly.Between November 2002 and December 2018, 486 consecutive patients undergoing elective LS were enrolled in this study, including 222 TLS and 264 HALS. The intraoperative, postoperative, and follow-up data were retrospectively analyzed.The 5 most common indications were hypersplenism (71.0%), immune thrombocytopenia (14.8%), splenic benign tumor (4.5%), splenic cyst (2.9%), and splenic malignant tumor (2.9%). The mean operative time, intraoperative blood loss, and length of stay were 149.4 ±â€Š63.3 minutes, 230.1 ±â€Š225.1 mL, and 6.7 ±â€Š3.2 days, respectively. The morbidity, mortality, reoperation, and conversion rate were 23.0%, 0, 0.4%, and 1.9%, respectively. Portal vein system thrombosis (PVST) was the most frequent complication with an incidence of 19.8%. The incidence of PVST in HALS was higher than that in TLS (23.9% vs 14.9%, P = .013). Compared with TLS, HALS had a shorter operative time (P = .000), lower intraoperative blood loss (P = .000), comparable conversion rate (P = .271), and morbidity (P = .922) for splenomegaly > 17.0 cm. During the follow-up period, the overall respond rate for immune thrombocytopenia was 77.8%, and the esophagogastric variceal bleeding rate was 6.9% in 320 patients with hypersplenism secondary to hepatic cirrhosis.LS is a safe, feasible, and effective procedure with satisfactory short- and long-term outcomes. HALS is a reasonable technique in patients with massive spleens.

Temporal trends and outcome of splenectomy in adults with immune thrombocytopenia in the USA.

Splenectomy is one of the treatments of immune thrombocytopenia (ITP) with a high response rate. However, it is an irreversible procedure that can be associated with morbidity in this setting. Our aim was to study the trends of splenectomy in adults with ITP, and the factors associated with splenectomy and resource utilization during these hospitalizations. We used the National (Nationwide) Inpatient Sample (NIS) to identify hospitalizations for adult patients with a principal diagnosis of ITP between 2007 and 2017. The primary outcome was the splenectomy trend. Secondary outcomes were (1) incidence of ITP trend, (2) in-hospital mortality, length of stay, and total hospitalization costs after splenectomy trend, and (3) independent predictors of splenectomy, length of stay, and total hospitalization costs. A total of 36,141 hospitalizations for ITP were included in the study. The splenectomy rate declined over time (16% in 2007 to 8% in 2017, trend p < 0.01) and so did the in-hospital mortality after splenectomy. Of the independent predictors of splenectomy, the strongest was elective admissions (adjusted odds ratio [aOR]: 22.1, 95% confidence interval [CI]:17.8-27.3, P < 0.01), while recent hospitalization year, older age, and Black (compared to Caucasian) race were associated with lower odds of splenectomy. Splenectomy tends to occur during elective admissions in urban medical centers for patients with private insurance. Despite a stable ITP hospitalization rate over the past decade and despite listing splenectomy as a second-line option for management of ITP in major guidelines, splenectomy rates consistently declined over time.

Risk factors and predictors of treatment responses and complications in immune thrombocytopenia.

Management of adult patients with immune thrombocytopenia (ITP) is often unsatisfactory, due to variable efficacy of treatment, risk of life-threatening bleeding if disease control is poor, and side effects associated with treatment. Lack of data on the platelet count threshold associated with bleeding and infection risk associated with ITP treatment limits risk/benefit clinical decision making. We reviewed medical records of all ITP patients who were admitted to our hospital between 2012 and 2017 to evaluate the platelet count threshold for bleeding, infection burden associated with treatment, and real-world efficacy of second-line treatment. We demonstrated fair discrimination between platelet count and occurrence of bleeding, with 15 × 109/L being the optimal cut-off for predicting any bleeding while 20 × 109/L had the highest negative predictive value for severe bleeding. In multivariable analyses, patients who were treated with corticosteroids for at least 2 months were 5.3 times as likely to have an infection. In addition, rituximab response was strongly associated with response to frontline corticosteroids and infection was associated with older age ≥ 65 years and corticosteroid dependence. If corticosteroids are initiated, physicians should aim for the shortest duration of treatment before switching to effective second-line agents for hemostatic platelet counts.

Risk Factors for Failure of Splenic Angioembolization: A Multicenter Study of Level I Trauma Centers.

BACKGROUND: Angioembolization (AE) is an adjunct to nonoperative management (NOM) of splenic injuries. We hypothesize that failure of AE is associated with blood transfusion, grade of injury, and technique of AE. METHODS: We performed a retrospective (2010-2017) multicenter study (nine Level I trauma centers) of adult trauma patients with splenic injuries who underwent splenic AE. Variables included patient physiology, injury grade, transfusion requirement, and embolization technique. The primary outcome was NOM failure requiring splenectomy. Secondary outcomes were mortality, complications, and length of stay. RESULTS: A total of 409 patients met inclusion criteria; only 33 patients (8%) required delayed splenectomy. Patients who failed received more blood in the first 24 h (P = 0.009) and more often received massive transfusion (P = 0.01). There was no difference in failure rates for grade of injury, contrast blush on computed tomography, and branch embolized. After logistic regression, transfusion in the first 24 h was independently associated with failure of NOM (P = 0.02). Patients who failed NOM had more complications (P = 0.002) and spent more days in the intensive care unit (P < 0.0001), on the ventilator (P = 0.0001), and in the hospital (P < 0.0001). Patients who failed NOM had a higher mortality (15% versus 3%, P = 0.007), and delayed splenectomy was independently associated with mortality (odds ratio, 4.2; 95% confidence interval, 1.2-14.7; P = 0.03). CONCLUSIONS: AE for splenic injury leads to effective NOM in 92% of patients. Transfusion in the first 24 h is independently associated with failure of NOM. Patients who required a delayed splenectomy suffered more complications and had higher hospital length of stay. Failure of NOM is independently associated with a fourfold increase in mortality.

Splenic Primary Solid Tumors : Does a Preoperative Histopathology Diagnosis Really Matter?

BACKGROUND: The present study aims to present a case series of patients who underwent splenectomy for splenic primary solid tumors without preoperative histopathologic diagnosis. METHODS: From 2013 to 2019, 12 patients underwent splenectomy for solid primary splenic tumors at 3 different academic medical centers. All electronic medical records were retrospectively reviewed. RESULTS: Seven (58.3%) patients were women, and 5 (41.6%) were male. The median age was 48 years (range: 25-72 years). In 8 (66.6%) patients, a conventional approach was performed. In 2 (16.6%), a hand-assisted laparoscopic surgery procedure was completed, and in other 2 (16.6%) patients, a laparoscopic approach was auspiciously achieved. Median operative time was 135 minutes (range: 60-210 minutes), and median blood loss was 500 mL (range: 200-1500 mL). Procedure-related morbidity was found in 2 (16.6%) patients, and the mortality rate was 0%. The final histopathologic diagnosis was lymphoma in 5 (41.6%) patients, lymphangioma in 3 (25%) patients, hamartoma in 2 (16.6%) patients, angiosarcoma, and sclerosing angiomatoid nodular transformation (SANT) in 1 (8.3%) case each. CONCLUSION: Splenectomy should be the treatment of choice when encountering a primary splenic tumor without the need for preoperative fine-needle aspiration biopsy, avoiding the complications this technique entails.

Efficacy evaluation of different conservative treatments for blunt spleen rupture.

BACKGROUND: This study aimed to provide a reference for the clinical treatment of patients with spleen ruptures by analyzing and discussing the clinical effects of the conservative treatment. METHODS: The clinical data of 93 patients with blunt spleen rupture treated in the First Affiliated Hospital of University of Science and Technology of China from April 2015 to April 2018 were retrospectively analyzed. Among them, 84 cases were treated conservatively and 9 cases were treated surgically. The general information of conservative treatment and surgical treatment were compared. The relationship between different conservative treatment methods and CT classification of spleen rupture and the changes of abdominal drainage were analyzed. RESULTS: The CT classification grade and trauma score of patients with spleen rupture in surgical treatment were higher than those in conservative treatment group (P<0.05). A total of 90.3% patients were treated conservatively. Among them, 7.1% (83.4% were in CT classification of spleen injury grade 1-2) were from the observation group, 14.3% (83.3% were in CT classification of spleen injury grade 1-2) were from abdominal drainage group, 3.6% were from splenic artery embolization group, and 75% (9.5% were in CT classification of spleen injury grade 2, 77.8% in grade 3 and 12.7% in grade 4) were from splenic artery embolization plus abdominal drainage group. There was no significant difference in the total amount of abdominal drainage on day 1, day 2 and day 3, and the CT classification of spleen rupture (P>0.05). However, there significant differences on the amount of abdominal drainage among day 1, day 2 and day 3 (P<0.05). Meanwhile, 2 complications occurred in the splenic artery embolization plus abdominal drainage group. CONCLUSIONS: Conservative treatment is feasible in blunt spleen rupture patients of CT classification grade of 1-4 with stable hemodynamical. Splenic rupture patients of CT classification grade 4-5 with instable hemodynamical should be treated surgically.

The spectrum of splenic complications in patients with sickle cell disease in Africa: a systematic review.

The majority of the global population of sickle cell disease (SCD) patients resides in Africa. Individuals with this condition are at great risk of serious infections and early mortality secondary to splenic dysfunction without preventative measures. This review investigated the spectrum of splenic complications encountered in SCD among populations in Africa. We systematically searched several databases for all articles published through March 3, 2020. We included 55 studies from 14 African countries. This review reveals the difference in frequency of splenic complications in SCD in Africa when compared with their counterparts in the United State and Europe. While several studies (n = 45) described splenomegaly with a prevalence of 12% to 73% among children, and 4% to 50% among adults with HbSS, the reported prevalence for acute splenic sequestration crisis (n = 6 studies) and hypersplenism (n = 4 studies) was <10% and <5% respectively. A total of 30 surgical splenectomy was reported across eight studies. Only two (3.7%) studies provided data on spleen function. A conflicting pattern was observed amongst studies that evaluated the relationship between splenomegaly and the presence of bacterial and malaria infections. This review reveals the paucity of studies describing the role of SCD-induced splenic dysfunction in morbidity and infection related mortality in Africa.

Outcomes of Hepatosplenic T-Cell Lymphoma: The Mayo Clinic Experience.

BACKGROUND: Hepatosplenic T-cell lymphoma (HSTCL) is a rare subtype of peripheral T-cell lymphoma accounting for less than 1% of non-Hodgkin lymphomas. It is generally associated with poor prognosis. PATIENTS AND METHODS: We performed a cohort study of patients with HSTCL treated at the Mayo Clinic between 1996 and 2020 exploring the clinical characteristics and therapeutic outcomes. RESULTS: Twenty-two cases of HSTCL were identified with a median (range) age at diagnosis of 45.5 (15.5-80.6) years and a male predominance (15/22, 68.2%). Clinical characteristics include massive splenomegaly in 16 patients (73%), hepatic involvement in 13 (59%), and chronic immunosuppressed state in 8 (36%). Phenotypically, lymphoma cells had gamma/delta T-cell receptor expression in 18 (82%) and alpha/beta in 4 patients. Cytogenetic abnormalities included isochromosome 7q (i7q) in 8 (62%) of 13 and trisomy 8 in 4 (44%) of 9. The median (range) follow-up of surviving patients was 33 (2.5-137) months. The median progression-free and overall survival were 9.5 months (95% CI, 1.8, 16.3) and 12.4 months (95% CI, 4.9, 18.5), respectively. Long-term survival was seen in 4 (18%) of 22 patients, with survival of 55, 74, 95, and 137 months. Moreover, 3 of 4 long-term survivors had splenectomy as part of initial treatment, and 2 of 4 long-term survivors received an allogeneic hematopoietic cell transplant (allo-HCT). CONCLUSION: Liver involvement and chronic immunosuppression were associated with shorter survival. Although splenectomy and allo-HCT have anecdotal benefit in the literature, our data do not show a statistically significant benefit of splenectomy and/or allo-HCT, likely as a result of our small sample size.

National trends in bowel and upper abdominal procedures in ovarian cancer surgery.

OBJECTIVES: In the United States, trends in the initial treatment approach for ovarian cancer reflect a shift in paradigm toward the increased use of neoadjuvant chemotherapy and interval cytoreductive surgery. The aim of this study was to evaluate the trends in surgical cytoreductive procedures in ovarian cancer patients who underwent either primary or interval cytoreductive surgery. METHODS: This retrospective, population-based study examined patients with stage III/IV ovarian cancer diagnosed between January 2000 and December 2013 identified using SEER-Medicare. Small or large bowel resection, ostomy creation, and upper abdominal procedures were identified using relevant billing codes and compared over time. A 1:1 primary and interval cytoreductive propensity matched cohort was created using demographic and clinical variables. 30-day complications and the use of acute care services were compared. RESULTS: A total of 5417 women were identified. 34% underwent bowel resections, 16% ostomy creation, and 8% upper abdominal procedures. There was an increase in bowel resections and upper abdominal procedures from 2000 to 2013 in patients who underwent primary cytoreductive surgery. Compared with patients who received primary cytoreduction, patients who underwent interval cytoreductive surgery were less likely to undergo bowel resection (OR=0.50; 95% CI [0.41, 0.61]) or ostomy creation (OR=0.48; 95% CI [0.42, 0.56]). Upper abdominal procedures did not differ between groups. For patients who underwent primary cytoreductive surgery, these procedures were associated with intensive care unit stay (4.6% vs <2%, P<0.01). In both primary and interval cytoreductive surgery patients, the receipt of bowel and upper abdominal procedures was associated with multiple 30-day postoperative complications and higher rates of readmission and emergency room visits. CONCLUSIONS: The performance of upper abdominal procedures in ovarian cancer patients increased from 2000 to 2013. Interval cytoreductive surgery was associated with decreased likelihood of bowel surgery. In matched primary and interval cytoreductive surgery cohorts, the receipt of these procedures were associated with the increased likelihood of postoperative complications and use of acute care services.

Left-sided portal hypertension after pancreatoduodenectomy with resection of the portal/superior mesenteric vein confluence. Results of a systematic review.

BACKGROUND: Pancreatoduodenectomy with synchronous resection of the portal vein/superior mesenteric vein confluence may result in the development of left-sided portal hypertension. Left-sided portal hypertension presents with splenomegaly and varices and may cause severe gastrointestinal bleeding. The aim of the study is to review the incidence, treatment, and preventive strategies of left-sided portal hypertension. METHODS: A systematic literature search was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement to identify all studies published up to September 30, 2019 reporting data on patients with left-sided portal hypertension after pancreatoduodenectomy with venous resection. RESULTS: Eight articles including 829 patients were retrieved. Left-sided portal hypertension occurred in 7.7% of patients who had splenic vein preservation and 29.4% of those having splenic vein ligation. Fourteen cases of gastrointestinal bleeding owing to left-sided portal hypertension were reported at a mean interval of 28 months from pancreatoduodenectomy. Related mortality at 1 month was 7.1%. Treatment of left-sided portal hypertension consisted of splenectomy in 3 cases (21%) and colectomy in 1 (7%) case, whereas radiologic, endoscopic procedures or conservative treatments were effective in the other cases (71%). CONCLUSION: Left-sided portal hypertension represents a potentially severe complication of pancreatoduodenectomy with venous resection occurring at greater incidence when the splenic vein is ligated and not reimplanted. Left-sided portal hypertension-related gastrointestinal bleeding although rare can be managed depending on the situation by endoscopic, radiologic procedures or operative intervention with low related mortality.

Post-operative pancreatic fistula following splenectomy with or without distal pancreatectomy at cytoreductive surgery in advanced ovarian cancer.

OBJECTIVE: Splenectomy with or without distal pancreatectomy may be necessary at time of cytoreductive surgery to achieve complete cytoreduction in advanced ovarian cancer. However, these procedures have been associated with peri-operative morbidity. The aims of this study were to determine the incidence of distal pancreatectomy among patients undergoing splenectomy during cytoreductive surgery for advanced ovarian cancer and to determine the incidence, management, treatment, and prognosis of patients with post-operative pancreatic fistula. METHODS: Retrospective cohort study of all consecutive patients with FIGO stage IIIC-IVB ovarian, fallopian tube, or primary peritoneal cancer who underwent splenectomy with or without distal pancreatectomy, during primary, interval, or secondary cytoreductive surgery between January 2007 and December 2017. All histologic subtypes were included; patients with borderline ovarian tumor and those undergoing emergency surgery were excluded from analysis. Univariate analyses for survival were generated by Kaplan-Meier survival curves and log-rank (Mantel-Cox) tests for statistical significance. Patients who underwent surgery for recurrence were excluded from survival analysis. Inter-group statistics were performed using Student's t-test for continuous variables, and chi-square test and Fisher's exact test for categorical variables. RESULTS: A total of 156/804 (19.4%) women underwent splenectomy, and of these 22 (14.1%) patients had distal pancreatectomy. Of patients who underwent splenectomy only, 2/134 (1.5%) developed grade B post-operative pancreatic fistula and 6/22 (27.3%) patients who underwent distal pancreatectomy developed grade B and C post-operative pancreatic fistula. Five (83.3%) of six of these patients were symptomatic. Distal pancreatectomy patients had a higher risk of developing post-operative pancreatic fistula when compared with patients who underwent splenectomy only (63.7% vs 9.7%, p=0.0001). Median length of hospital stay was longer in patients with post-operative pancreatic fistula: 16.5 (range 7-38) days compared with 10 (range 7-15) days (p=0.019). There was no progression-free survival (p=0.42) and disease-specific survival (p=0.33) difference between patients undergoing splenectomy with or without distal pancreatectomy. CONCLUSION: Clinically relevant post-operative pancreatic fistula is a relatively frequent complication (27.3%) following distal pancreatectomy and it is a possible complication after splenectomy only (1.5%).

Report of a 'consensus' on the lines of therapy for primary immune thrombocytopenia in adults, promoted by the Italian Gruppo di Studio delle Piastrine.

Despite the publication in 2009 of a paper on 'terms and definitions of immune thrombocytopenia' (ITP), some unresolved issues remain and are reflected by the disagreement in the treatment suggested for primary ITP in adults. Considering that these disagreements could be ascribed to non-shared goals, we generated a 'consensus' on some terms, definitions, and assertions useful for classifying the different lines of treatment for primary ITP in adults according to their indications and goals. Agreement on the appropriateness of the single assertions was obtained by consensus for the following indicators: 1. classification of four 'lines of therapy'; 2. acceptance of the expression 'sequences of disease' for the indications of the respective four lines of treatment; 3I . practicability of splenectomy; 3Ib . acceptance, with only some exceptions, of a 'timing for elective splenectomy of 12 months'; and 4a-d . 'goals of the four lines of therapy.' On the basis of the consensus, a classification of four lines of treatment for primary ITP in adults was produced. In our opinion, this classification, whose validity is not influenced by the recently published new guidelines of the American Society of Hematology (ASH) and reviews, could reduce the disagreement that still exists regarding the treatment of the disease.

Increased long-term pneumonia risk for the trauma-related splenectomized population - a population-based, propensity score matching study.

BACKGROUND: Splenectomy is the life-saving treatment for high-grade spleen trauma. Splenectomized patients are at a significant infection risk. However, the trauma-induced splenectomy results in less incidence of postsplenectomy infection than the hematologic disorder. We conducted a large-scale study to identify the infection rate and management strategy in trauma-related splenic injuries. METHODS: We included patients with the diagnosis of spleen injury in Taiwan from January 2003 to December 2013 by using the National Health Insurance Database and divided them into spleen preserved and splenectomized groups. The demographic factors including age, sex, hospital level, year of injury, trauma mechanism, associated injuries, whether injury severity score ≧16, and comorbidities were extracted. A 1:1 propensity score match was performed, and we analyzed the long-term outcome as the presence of infection-related disease (septicemia, pneumonia, and meningitis) after spleen trauma. The multivariate logistic regression analysis was used to identify the risk factor for each outcome. RESULTS: During the 11 years included in this study, a total of 8,897 patients with spleen trauma were identified. A total of 3,520 (39.6%) patients were splenectomized, and 5,377 (60.4%) were spleen preserved. After propensity score matching, 3,099 pairs of patients were enrolled for further analysis. In univariate analysis, the incidence of pneumonia is significantly higher in the splenectomized group (8.5% vs 7.0%, P = .037). There was no significant difference in septicemia and meningitis between the 2 groups. In multivariate analysis, splenectomy is an independent risk factor for pneumonia in long-term follow-up. CONCLUSION: Compared with the spleen preserved group, splenectomy is related to an increased likelihood of long-term pneumonia onset but not to an increase in the possibility of other infections.

Splenic trauma, the way forward in reducing splenectomy: our 15-year experience.

INTRODUCTION: The aim of this study was to study radiological assessment, management and outcome of traumatic splenic injury over 15 years in a UK district general hospital. METHOD: A retrospective database was established including all splenic injury cases from June 2002 to June 2017 by searching the clinical electronic database. We searched the radiological database for computed tomography reported phrases 'spleen injury', 'laceration', 'haematoma', 'trauma'. We interrogated theatre records for operations coded as splenectomy and cross-referenced this with pathology. Records were reviewed for demographics, vital observations, documentation of American Association for the Surgery of Trauma (AAST) grading of splenic injury, subsequent management and outcomes. RESULTS: There were 126 patients identified with traumatic splenic injury, with male to female ratio three to one. Operative management was undertaken in 54/126 (43%) patients and selective non-operative management in the remaining. Splenic artery embolisation was undertaken in 5/126 (4%) and 2/126 underwent splenorrhaphy. Computed tomography was undertaken in 109/126 (87%) patients and AAST grading was reported in 18 (17%) patients. AAST grade reporting did not improve significantly when comparing the first 7.5 years with the latter (2/30, 7%; 16/79, 20%), respectively; p = 0.09). Selective non-operative management increased significantly over the studied period (14/34, 42%; 58/93, 62%; p = 0.04). The overall hospital mortality was 10.3%. DISCUSSION AND CONCLUSION: AAST grade reporting of splenic injury has remained sub-optimal over 15 years. Despite progression towards selective non-operative management, operative intervention remained unacceptably high, with splenectomy being the main therapeutic modality. Standardisation through an integrated multidisciplinary diagnostic and management pathway offers the optimal strategy to reduce trauma-induced splenectomy.