Laparoscopic splenectomy for hereditary spherocytosis—preliminary report

Splenectomy is considered standard surgical therapy in hereditary spherocytosis. The procedure is indicated in patients with severe anemia, recurrent hemolytic, and aplastic crises. The aim of the study was to assess treatment outcomes in patients with hereditary spherocytosis who underwent total or partial laparoscopic splenectomy. Fifteen patients aged 4–17 yr underwent laparoscopic splenectomy from 2009 to 2012. Partial and total splenectomies were performed (five and 10 children, respectively). Hematologic parameters, liver function tests, and splenic volume before and after the surgery were analyzed retrospectively. Total follow‐up was 1–30 months. Hospitalization and operating time were similar in both groups. In partial splenectomy group, branches of splenic arteries gave better blood supply than short gastric vessels. In both groups, hematologic parameters were improved. Postoperative markedly elevated platelet count was maintained up to 6 months, and after that, platelet count gradually decreased to normal values. Bilirubin level was decreased in early postoperative period; however, it increased later to achieve levels lower than in preoperative period. No severe general infections were observed in both groups. Laboratory parameters (hemoglobin and bilirubin concentrations and RBC) after the surgery improved in all patients, and the effect was maintained during 12 months of follow‐up. Platelet count increased significantly after the surgery and was maintained at high levels during the next 6 months. However, it returned to preoperative levels within a year after the surgery. Our study showed that partial splenectomy was not inferior to total splenectomy. However, full assessment requires longer follow‐up and larger group of patients.     

Surgery of the spleen

A normally functioning spleen is critical in providing adequate immune protection and in regulating blood homeostasis. Whereas primary disorders of the spleen can attenuate these important functions, absence of the spleen, most commonly as a result of surgical excision, carries the grave and lifetime risk of devastating systemic sepsis. With some historical exceptions, splenic surgery has classically involved removal of the entire organ at open surgery, either following traumatic injury or to supplement the medical management of haematological disorders. Performed primarily for this latter indication, laparoscopy has emerged over the last two decades as the strongly favoured approach for elective splenectomy, with very large splenic size being one of the few remaining reasons for a planned open approach. Even in skilled hands, laparoscopic splenectomy can prove technically demanding, where careful patient selection and recognition of major complications including haemorrhage, portal system thrombosis and pancreatic injury, requires considerable experience. Whether performed under emergency or elective circumstances and undertaken as an open or laparoscopic procedure, an optimal surgical outcome depends on the successful collaboration between surgeon, anaesthetist, haematologist and radiologist in the preoperative and postoperative phases. Finally, it is paramount that in patients undergoing splenectomy, sufficient attention is given to providing effective lifelong prophylaxis against post-splenectomy infection.     

Unusual association of Axenfeld-Rieger syndrome and wandering spleen:A case report

BACKGROUND Axenfeld-Rieger syndrome(ARS)is an autosomal dominant genetic disease characterized by ocular developmental disorders and its association with torsion of wandering spleen(WS)has not been reported to date to the best of our knowledge.This study aimed to describe a rare case of ARS observed at our emergency department.CASE SUMMARY A 25-year-old female presented with a constant lower abdominal pain of increasing severity.Diagnostic computed tomography with intravenous contrast material showed a non-homogenously enhanced splenic parenchyma with a twisted vascular pedicle.Further,an emergent laparoscopic exploration was performed,and an ischemic spleen without its normal ligamentous attachments was noted.Notably,the spleen did not regain its normal vascularity after detorsion;thus,we performed the laparoscopic total splenectomy.The postoperative course was uneventful,and the patient was discharged on the 5th postoperative day.This case demonstrates a rare association of WS and ARS.CONCLUSION Early diagnosis of WS in the emergency department is important to prevent pedicle torsion or splenic necrosis and to avoid splenectomy.     

Transjugular intrahepatic portosystemic shunt versus open splenectomy and esophagogastric devascularization for portal hypertension with recurrent variceal bleeding

BACKGROUND: Transjugular intrahepatic portosystemic shunt (TIPS) and open splenectomy and esophagogastric devascu-larization (OSED) are widely used to treat patients with portal hypertension and recurrent variceal bleeding (PHRVB). This study aimed to compare the effectiveness between TIPS and OSED for the treatment of PHRVB.METHODS: The data were retrospectively retrieved from 479 cirrhotic patients (Child-Pugh A or B class) with PHRVB, who had undergone TIPS (TIPS group) or OSED (OSED group) between January 1, 2010 and October 31, 2014.RESULTS: A total of 196 patients received TIPS, whereas 283 underwent OSED. Within one month after TIPS and OSED, the rebleeding rates were 6.1% and 3.2%, respectively (P=0.122). Significantly lower incidence of pleural effusion, splenic vein thrombosis, and pulmonary infection, as well as higher hepatic encephalopathy rate, shorter postoperative length of hospital stay, and higher hospital costs were ob-served in the TIPS group than those in the OSED group. Dur-ing the follow-up periods (29 months), significantly higher incidences of rebleeding (15.3% vs 4.6%, P=0.001) and hepatic encephalopathy (17.3% vs 3.9%, P=0.001) were observed in the TIPS group than in the OSED group. The incidence of in-stent stenosis was 18.9%. The survival rates were 91.3% in the TIPS group and 95.1% in the OSED group. The long-term liver function did not worsen after either TIPS or OSED.CONCLUSION: For the patients with liver function in the Child-Pugh A or B class, TIPS is not superior over OSED in terms of PHRVB treatment and rebleeding prevention.     

手术治疗肝豆状核变性并脾功能亢进的疗效评价

目的：评价脾切除术联合胃底贲门周围血管离断术治疗肝豆状核变性并脾功能亢进的疗效。方法148例肝豆状核变性并脾功能亢进患者行脾切除术联合胃底贲门周围血管离断术治疗,检测患者的血常规、肝功能变化,评定手术疗效。结果手术后1周,患者血小板（PLT）、白细胞（WBC）较手术前显著升高（P ＜0．05）,甚至超过正常范围;手术后1月的 PLT、WBC水平则显著回落,恢复正常水平。谷丙转氨酶、谷草转氨酶、总胆红素术前处于较高水平,术后1周较术前进一步升高（P ＜0．05）,而术后1月时其水平较术前及术后1周均有显著下降（P ＜0．05）。结论脾切除术联合胃底贲门周围血管离断术治疗肝豆状核变性并脾亢,对恢复外周血细胞数量、保护肝功能均有显著作用。     

Splenectomy for the treatment of thrombotic thrombocytopenic purpura

Plasma exchange is the treatment of choice for patients with thrombotic thrombocytopenic purpura (TTP) and results in remission in >80% of the cases. Treatment of patients who are refractory to plasma therapy or have relapsing disease is difficult. Splenectomy has been a therapeutic option in these conditions but its value remains controversial. We report on a series of 33 patients with TTP who were splenectomised because they were plasma refractory (n = 9) or for relapsed disease (n = 24). Splenectomy generated prompt and unmaintained remissions in all except five patients, in whom remission was delayed (n = 4) or who died with progressive disease (n = 1). Four postoperative complications occurred: one pulmonary embolism and three surgical complications. Median follow-up after splenectomy was 109 months (range 28-230 months). The overall postsplenectomy relapse rate was 0.09 relapses/patient-year and the 10-year relapse-free survival (RFS) was 70% (95% CI 50-83%). In the patients with relapsing TTP, relapse rate fell from 0.74 relapses/patient-year before splenectomy to 0.10 after splenectomy (P < 0.00001). Two patients died from first postsplenectomy relapse. Although these results are based on retrospective data and that the relapse rate may spontaneously decrease with time, we conclude that splenectomy, when performed during stable disease, has an acceptable safety profile and should be considered in cases of plasma refractoriness or relapsing TTP to reach durable remissions and to reduce or prevent future relapses.     

31例腹腔镜下脾切除的临床分析

目的：探讨完全腹腔镜下脾切除的手术技巧和临床经验。方法回顾性分析该院从2003年1月至2015年4月进行完全腹腔镜脾切除术31例,其中,肝硬化脾功能亢进12例,脾多发性血管瘤5例,脾淋巴瘤3例,脾囊肿3例,陈旧性脾破裂、脾血肿2例,特发性血小板减少症6例,术中LigaSure处理脾胃韧带,结扎脾动脉,采用丝线结扎或腔内直线切割闭合器个体化处理脾蒂。结果31例腹腔镜脾切除术,无中转开腹患者。其中,13例逐一分离、丝线结扎脾门血管后离断脾蒂,18例采用腔内直线切割闭合器离断脾蒂,手术时间80～240 min ,平均（130±35）min ,术中出血量60～500 mL ,平均（150±80）mL ,平均住院时间8．3d,无手术死亡及胰瘘并发症发生病例。结论在开腹脾切除的基础上,术前仔细评估,选择合适病例,利用不同腹腔镜器械的优势,个体化方式处理脾蒂,腹腔镜脾切除术安全实用。     

Venous thromboembolism and hypercoagulability in splenectomized patients with thalassaemia intermedia

Thromboembolic phenomena have been described in patients with thalassaemia intermedia and major, although there are relatively few epidemiological data on the overall frequency of these complications. To obtain more insight into the risk and mechanism of venous thromboembolism in thalassaemia, the aims of this study were: (i) to establish retrospectively the prevalence of thromboembolic events in a large group of adults with thalassaemia intermedia and major during a follow up period of 10 years; (ii) to measure in subgroups of these patients sensitive markers of activation of coagulation and fibrinolysis enzymes; and (iii) to look for possible procoagulant mechanisms. A high prevalence of thromboembolic events was found, particularly in splenectomized patients with thalassaemia intermedia (29%). These patients had high plasma levels of markers of coagulation and fibrinolysis activation. Furthermore, thalassaemic red cells and erythroid precursors from splenectomized patients with thalassaemia intermedia had an enhanced capacity to generate thrombin. To evaluate the role of splenectomy per se on procoagulant activity, we evaluated the capacity to form thrombin in healthy individuals who had been splenectomized for trauma. They produced the same amount of thrombin as non-splenectomized controls. In conclusion, the results of this study show the existence of a hypercoagulable state in splenectomized patients with thalassaemia intermedia and that their red and erythroid cells are capable of acting as activated platelets in thrombin generation.     

Thoracic splenosis: A diagnosis by history and imaging

Abstract:   Thoracic splenosis (TS) is autoimplantation of ectopic splenic tissue in the thoracic cavity that occurs following splenic injury. The majority of cases of TS are asymptomatic and are diagnosed in the course of an evaluation of incidentally discovered pulmonary lesions. Some cases may be difficult to diagnose, especially if features suggesting TS are not recognized. This may lead to an extensive work-up and unnecessary invasive diagnostic procedures including thoracotomy. This case report describes a 40-year-old man, who was diagnosed incidentally with TS, several years after the initial injury. Multiple, asymptomatic, left-sided pleural based lesions associated with a history of thoracoabdominal injury and splenectomy are the key points that should alert suspicion of TS, which can then simply be confirmed by a Tc-99m sulphur colloid radionuclide scan. Most patients are treated conservatively unless they are symptomatic. Physicians must recognize the key features suggesting a diagnosis of TS, order appropriate imaging studies and avoid unnecessary invasive diagnostic procedures.