OBJECTIVE: To delineate the natural history of splenic complications other than loss of splenic function in children with sickle cell disease (SCD), we performed a retrospective chart review of patients with SCD treated at the Texas Children\'s Hospital.
METHODS: We determined the dates of diagnoses of splenic complications, the number of acute splenic sequestration crises (ASSC), and hydroxyurea treatment in pediatric patients with SCD. We also examined the association of hydroxyurea therapy with the onset and severity of ASSC.
RESULTS: The cumulative prevalence of splenic complications was 24.7% for splenomegaly, 24.2% for ASSC, 9.6% for hypersplenism, and 5.6% for splenectomy. The cumulative prevalence of splenic complications was highest in patients with hemoglobin Sβ0 (69.2%), intermediate in hemoglobin SS (33.3%), low in hemoglobin SC (9.0%), and non-existent in hemoglobin Sβ+. The overall event rate of ASSC was 8.3 per 100 patient-years. The event-rate was 28.4 for hemoglobin Sβ0, 10.9 for hemoglobin SS, and 3.5 for hemoglobin SC. Patients with hemoglobin SS and hemoglobin Sβ0 on hydroxyurea therapy had a significantly higher occurrence of ASSC than those who were not, with event rates of 14.2 and 3.1, respectively. The event rate was also higher for children who started hydroxyurea before age 2 years than for those who started after this age (19.8 and 9.2, respectively).
CONCLUSIONS: The prevalence and severity of splenic problems vary widely between different sickle cell genotypes, with hemoglobin Sβ0 having the most severe complications. Hydroxyurea therapy is associated with increased incidence of ASSC, particularly when initiated before 2 years of age.

BACKGROUND: Although postoperative portal vein thrombosis (PVT) is a frequent complication of splenectomy, few studies have examined PVT after simultaneous hepatectomy and splenectomy (HS). The aim of this study was to clarify the risk factors for and characteristics of PVT after HS.
METHODS: This retrospective observational study included 102 patients, including 76 with liver cirrhosis (LC) and 26 without, who underwent HS between April 2004 and April 2021. The incidence and location of postoperative PVT detected on contrast-enhanced CT 1 week after surgery were analyzed. In addition, pre- and intraoperative parameters were compared between patients with postoperative PVT and those without in order to determine risk factors for PVT after HS.
RESULTS: Among the 102 patients, 29 (28.4 %), including 32.9 % with LC and 15.4 % without LC, developed PVT after surgery. Among the 29 patients with PVT, 21 (72.4 %), 4 (13.8 %), and 4 (13.8 %) developed thrombus in the intrahepatic portal vein only, extrahepatic portal vein only, and both the extra- and intrahepatic portal veins, respectively. Multivariable analysis showed that preoperative splenic vein dilatation was an independent risk factor for PVT after HS (odds ratio: 1.53, 95 % confidence interval: 1.156-2.026, P = 0.003).
CONCLUSIONS: Our results suggest that splenic vein dilatation is an independent risk factor for PVT after simultaneous HS, and that PVT after HS occurs more frequently in the intrahepatic portal vein. After HS for cases with dilated splenic veins, we should pay particular attention to the PVT development in the intrahepatic portal vein regardless of the type of liver resection.

Introduction Beta thalassemia is a hemoglobinopathy characterized by defective production of the beta chain of hemoglobin, leading to irreversible destruction of RBCs, splenomegaly, pancytopenia, and a requirement for multiple transfusions. This condition necessitates iron chelation therapy, and splenectomy is often performed to manage hypersplenism. Methods This report includes a series of seven diagnosed cases of beta thalassemia with hypersplenism, all of whom underwent open splenectomy. Preoperative transfusions were administered to achieve target hemoglobin and platelet counts of 9 g/dL and 50,000/µL, respectively. Results The study included seven patients diagnosed with beta thalassemia, all of whom underwent open splenectomy. Among these, three patients also had concomitant cholecystectomy due to the presence of gallstones. The primary indication for performing splenectomy was hypersplenism. Preoperative transfusions were administered to ensure target hemoglobin levels of 9 g/dL and platelet counts of 50,000/µL. All patients were successfully discharged with minimal morbidity and no reported mortality. The longest follow-up period observed in this series was 10 months post-splenectomy, which limited the assessment of long-term effects. Conclusion Open splenectomy for hypersplenism in patients with beta thalassemia appears to be a safe and effective procedure with minimal short-term morbidity and no mortality observed in this series. However, due to the limited follow-up duration, the long-term effects of splenectomy in these patients could not be evaluated. Further studies with longer follow-up are needed to assess the long-term outcomes of splenectomy in beta thalassemia patients.

OBJECTIVE: This study evaluated portal hypertension (PHT) and its predictors among native liver survivors (NLS) of biliary atresia (BA) after Kasai portoenterostomy (KPE).
METHODS: This was a multicenter study using prospectively collected data. The subjects were patients who remained transplant-free for 5 years after KPE. Their status of PHT was evaluated and variables that predicted PHT were determined by regression analysis and receiver operating characteristic (ROC) curve.
RESULTS: Six centers from East Asia participated in this study and 320 subjects with KPE between 1980 to 2018 were analyzed. The mean follow-up period was 10.6 ± 6.2 years. At the 5th year after KPE, PHT was found in 37.8% of the subjects (n = 121). Patients with KPE done before day 41 of life had the lowest percentage of PHT compared to operation at older age. At 12 months after KPE, PHT + ve subjects had a higher bilirubin level (27.1 ± 11.7 vs 12.3 ± 7.9 µmol/L, p = 0.000) and persistent jaundice conferred a higher risk for PHT (OR = 12.9 [9.2-15.4], p = 0.000). ROC analysis demonstrated that a bilirubin level above 38 µmol/L at 12 months after KPE predicted PHT development (sensitivity: 78%, specificity: 60%, AUROC: 0.75).
CONCLUSIONS: In BA, early KPE protects against the development of PHT among NLSs. Patients with persistent cholestasis at one year after KPE are at a higher risk of this complication. They should receive a more vigilant follow-up.
METHODS: Level III.

UNASSIGNED: Partial splenic embolization (PSE) combined with transarterial chemoembolization (TACE) has been reported in treatment of hepatocellular carcinoma (HCC) with cirrhotic hypersplenism and thrombocytopenia. However, efficacy and safety of repeated PSE when required are unclear. This study aims to investigate post-procedural changes in peripheral blood cell and hepatic function, progression-free survival (PFS), and safety of HCC patients with hypersplenism received TACE and repeated PSE compared to those received TACE alone.
UNASSIGNED: This retrospective study included 102 HCC patients with hypersplenism who received TACE (n = 73) or TACE+PSE (n = 29) from January 2014 to December 2021. Changes in peripheral blood cell and hepatic function were investigated at 1 week, 2, 6, 12, 18, and 24 months. TACE procedure sessions and adverse events were recorded. PFS and prognostic factors were analyzed.
UNASSIGNED: Despite response to initial PSE being limited, repeated PSE increased platelet (PLT) again, which peaked at 18 months. It also continued to improve red blood cell (RBC) and hemoglobin, which showed significant differences in changes from baseline between two groups until 24 months, as well as Child-Pugh scores at 12 and 18 months. Mean TACE procedure sessions were significantly higher in TACE+PSE group than that in TACE alone group (4.55 vs 3.26, P = 0.019). TACE+PSE group had longer median PFS (19.4 vs 9.5 months, P = 0.023) than TACE alone group, where PSE was an independent protective factor (HR, 0.508; P = 0.014). Initial and repeated PSE showed no significant differences in safety.
UNASSIGNED: Repeated PSE is effective in increasing PLT again and improving RBC, hemoglobin and liver function. It contributed to performing serial TACE procedures thereafter. TACE combined with repeated PSE has significantly longer PFS than TACE alone, where PSE was an independent protective factor. Moreover, the safety of repeated PSE was comparable to initial PSE.

暂无摘要。

Splenic artery embolization plays an important role in the management of various medical and surgical conditions that are non-traumatic in etiology, in addition to its well-established and widely discussed role in managing splenic trauma. In nontraumatic emergencies of catastrophic bleeding originating from the spleen or splenic artery, splenic artery embolization can be effective in achieving hemostasis as a definitive management, temporary stabilizing measure, or preoperative optimization technique. In addition to emergency clinical conditions, splenic artery embolization can be performed electively as an alternative to splenectomy for managing patients with hypersplenism. Herein, we report 6 cases of splenic artery embolization performed at our center to highlight its various indications. This article aims to demonstrate the role of splenic artery embolization in different clinical scenarios and the considerations behind the techniques employed through illustrative cases.

UNASSIGNED: Partial splenic artery embolization (PSAE) is an alternative treatment modality for managing hypersplenism secondary to portal hypertension. We are presenting a case series of patients with portal hypertension who underwent PSAE for symptomatic hypersplenism.
UNASSIGNED: We included patients with portal hypertension who underwent PSAE from January 2022 to December 2022. Patients\' characteristics and procedure related complications were noted. Data were analyzed for improvement in the hematological parameters.
UNASSIGNED: A total of 11 (7 women, median age 34 [18-56] years) patients were included. Three patients were cirrhotic (hepatitis B-2, metabolic dysfunction-associated steatotic liver disease -1) and 8 were non-cirrhotic (extra-hepatic portal vein obstruction-5, Non cirrhotic portal fibrosis-3). Splenic artery aneurysm was concomitantly present in 5 cases. Technical success was achieved in all cases. Post embolization, hemoglobin, white blood cells and platelet counts improved at 4 weeks, 12 weeks and 24 weeks along with symptomatic improvement. All patients had post-embolization syndrome. One patient developed transient ascites and secondary bacterial peritonitis which was managed conservatively. One patient died due to splenic abscess and septicemia.
UNASSIGNED: Although, hematological parameters and symptoms improve post procedure, PSAE is associated with major complications and should be performed judiciously in selected cases only. Graphical abstract is presented in Figure 1.

Background: Splenectomy has been performed for various indications from haematological diseases to benign cysts and tumours, and for splenic traumatic injuries. However, there has been a steady decline in splenectomies in the last 20 years. The aim of this study is to establish the reasons behind this decline in splenectomy and to analyse them based on indication, type of splenectomy, and manner of approach (open, laparoscopic or robotic). Material and Methods: This is a retrospective study of a single centre experience of all the splenectomies, both total and partial, performed in the Department of General Surgery of Fundeni Clinical Institute (Bucharest) between 2002 and 2023. Only surgeries for primary splenic diseases were selected, splenic resections as part of other major operations were not included. Results: Between 2002 and 2023, 876 splenectomies were performed in the Department of General Surgery of Fundeni Clinical Institute (Bucharest). Most splenectomies (n=245) were performed for immune thrombocytopenic purpura (ITP), followed by benign tumours and cysts (n=136), lymphoma (n=119), hypersplenism due to cirrhosis (n=107) and microspherocytosis (n=95). Other indications included myelodysplastic syndrome (n=39), trauma (n=35), thalassemia (n=22), leukaemia (n=18) and also there were 60 splenectomies that were performed for hypersplenism of unknown cause. There were 795 total splenectomies (TS) and 81 partial splenectomies (PS). There was a decline in the number of splenectomies both TS and PS for all these indications, most notably in the case of ITP, microspherocytosis and hypersplenism due to cirrhosis with no splenectomies performed for these indications since 2020. Conclusion: With the development of new lines of treatment, advances in interventional radiology and in surgery with the spleen parenchyma sparing options, the need for total splenectomy has been greatly reduced which is reflected in the decline in the number of splenectomies performed in the last 20 years in our clinic.

BACKGROUND: Partial splenic embolization (PSE) has been proposed to treat the consequences of hypersplenism in the context of portal hypertension, especially thrombocytopenia. However, a high morbidity/mortality rate has made this technique unpopular. We conducted a multicenter retrospective nationwide French study to reevaluate efficacy and tolerance.
METHODS: All consecutive patients who underwent PSE for hypersplenism and portal hypertension in 7 tertiary liver centers between 1998 and 2023 were included.
RESULTS: The study population consisted of 91 procedures in 90 patients, with a median age of 55.5 years [range 18-83]. The main cause of portal hypertension was cirrhosis (84.6 %). The main indications for PSE were (1) an indication of medical treatment or radiological/surgical procedure in the context a severe thrombocytopenia (59.3 %), (2) a chronic hemorrhagic disorder associated with a severe thrombocytopenia (18.7 %), and (3) a chronic pain associated with a major splenomegaly (9.9 %). PSE was associated with a transjugular intrahepatic portosystemic shunt in 20 cases. Median follow-up after PSE was 41.9 months [0.5-270.5]. Platelet count increased from a median of 48.0 G/L [IQR 37.0; 60.0] to 100.0 G/L [75.0; 148]. Forty-eight patients (52.7 %) had complications after PSE; 25 cases were considered severe (including 7 deaths). A Child-Pugh B-C score (p < 0.02) was significantly associated with all complications, a history of portal vein thrombosis (p < 0.01), and the absence of prophylactic antibiotherapy (p < 0.05) with severe complications.
CONCLUSIONS: Our results strongly confirm that PSE is very effective, for a long time, although a quarter of the patients experienced severe complications. Improved patient selection (exclusion of patients with portal vein thrombosis and decompensated cirrhosis) and systematic prophylactic antibiotherapy could reduce morbidity and early mortality in the future.