Rescue splenic artery embolization in an adult patient of sickle cell disease presented with acute splenic sequestration crisis.

BACKGROUND: Splenic sequestration crisis is a potentially fatal complication of sickle cell disease, mainly seen in young children. Only a few case series describe the acute splenic sequestration crisis in adults and its management, which primarily consists of supportive care and, in some cases, splenectomy. Splenic artery embolization has seldom been described in sickle cell disease. This is probably the first case in which an adult with sickle cell disease presented with an acute splenic sequestration crisis was managed successfully through splenic artery embolization. RESULTS: This 22-year-old female, a known case of sickle cell disease, presented with severe pain in the abdomen and low-grade intermittent fever for two days, secondary to an acute splenic sequestration crisis. The diagnosis of acute splenic sequestration was made based on clinical and blood parameters, ultrasonography, and computed tomography. Even with adequate supportive care and blood transfusions, the patient's condition worsened with a rapid fall in the hemoglobin and total platelet count. Considering splenectomy to be a high-risk procedure for this patient, a decision of rescue splenic artery embolization was taken, which was successful. CONCLUSION: Splenic artery embolization may be considered a lifesaving procedure in patients with acute splenic sequestration, where the risk of splenectomy can be high. Adequate post-procedure supportive care is vital for preventing complications.

Prevention and treatment of infection in patients with an absent or hypofunctional spleen: A British Society for Haematology guideline.

Guidelines for the prevention and treatment of infection in patients with an absent or dysfunctional spleen were published by the British Committee for Standards in Haematology in 1996 and updated in 2002 and 2011. With advances in vaccinations and changes in patterns of infection, the guidelines required updating. Key aspects included in this guideline are the identification of patients at risk of infection, patient education and information and immunisation schedules. This guideline does not address the non-infective complications of splenectomy or functional hyposplenism (FH). This replaces previous guidelines and significantly revises the recommendations related to immunisation. Patients at risk include those who have undergone surgical removal of the spleen, including partial splenectomy and splenic embolisation, and those with medical conditions that predispose to FH. Immunisations should include those against Streptococcus pneumoniae (pneumococcus), Neisseria meningitidis (meningococcus) and influenza. Haemophilus influenzae type b (Hib) is part of the infant immunisation schedule and is no longer required for older hyposplenic patients. Treatment of suspected or proven infections should be based on local protocols and consider relevant anti-microbial resistance patterns. The education of patients and their medical practitioners is essential, particularly in relation to the risk of serious infection and its prevention. Further research is required to establish the effectiveness of vaccinations in hyposplenic patients; infective episodes should be regularly audited. There is no single group ideally placed to conduct audits into complications arising from hyposplenism, highlighting a need for a national registry, as has proved very successful in Australia or alternatively, the establishment of appropriate multidisciplinary networks.

Colosplenic fistula diagnosis and management: a case series and review of literature.

BACKGROUND: A colosplenic fistula (CsF) is an extremely rare complication. Its diagnosis and management remain poorly understood, owing to its infrequent incidence. Our objective was to systematically review the etiology, clinical features, diagnosis, management, and prognosis to help clinicians gain a better understanding of this unusual complication and provide aid if it is to be encountered. METHODS: A systematic review of studies reporting CsF diagnosis in Ovid MEDLINE, Ovid EMBASE, Scopus, Web of Science, and Wiley Cochrane Library from 1946 to June 2022. Additionally, a retrospective review of four cases at our institution were included. Cases were evaluated for patient characteristics (age, sex, and comorbidities), CsF characteristics including causes, symptoms at presentation, diagnosis approach, management approach, pathology findings, intraoperative complications, postoperative complications, 30-day mortality, and prognosis were collected. RESULTS: Thirty patients with CsFs were analyzed, including four cases at our institution and 26 single-case reports. Most of the patients were male (70%), with a median age of 56 years. The most common etiologies were colonic lymphoma (30%) and colorectal carcinoma (17%). Computed tomography (CT) was commonly used for diagnosis (90%). Approximately 87% of patients underwent a surgical intervention, most commonly segmental resection (81%) of the affected colon and splenectomy (77%). Nineteen patients were initially managed surgically, and 12 patients were initially managed nonoperatively. However, 11 of the nonoperative patients ultimately required surgery due to unresolved symptoms. The rate of postoperative complications was (17%). Symptoms resolved with surgical intervention in 25 (83%) patients. Only one patient (3%) had had postoperative mortality. CONCLUSIONS: Our review of 30 cases worldwide is the largest in literature. CsFs are predominantly complications of neoplastic processes. CsF may be successfully and safely treated with splenectomy and resection of the affected colon, with a low rate of postoperative complications.

Gastrosplenic fistula due to splenic lymphoma: two case reports and review of the literature.

BACKGROUND: Gastrosplenic fistula is a rare and potentially fatal complication of various conditions. Lymphoma is the most common cause. It can occur spontaneously or after chemotherapy. Gastrosplenic fistula diagnosis can be confused with a splenic abscess because of the presence of air into the mass. The computed tomography identification of the fistulous tract is the key to a right diagnosis. Treatment modalities include surgical resection, chemotherapy, or a combination of both. CASE PRESENTATION: Here we report two patients with gastrosplenic fistula due to diffuse large B cell lymphoma. The first patient was a 54-year-old Caucasian woman with an enormous primary splenic diffuse large B cell lymphoma leading to the development of a spontaneous fistula in the stomach. The second patient was a 48-year-old Caucasian male patient with an enormous splenic diffuse large B cell lymphoma complicated by fistula after chemotherapy. Both patients died of septic shock several days after surgery. CONCLUSION: Gastrosplenic fistula is a rare complication with a poor-prognosis, for which surgery is currently the preferred treatment.

Size of Splenic Subcapsular Hematoma Is Associated With Varying Outcomes of Nonoperative Management.

The presence of a splenic subcapsular hematoma (SCH) has been associated with higher rates of failure of nonoperative management (FNOM) in patients with blunt splenic injury (BSI), with rates up to 80%. We hypothesized that contemporary rates are lower. A retrospective review was conducted of patients admitted with BSI to a level I trauma center (2016-2021). Patients with SCH who had FNOM were compared to those who did not. There were 661 BSI patients, of which 102 (15.4%) had SCH. Among the SCH patients, 8 (7.8%) had FNOM. Failure of nonoperative management was higher in patients who had a SCH measuring 15 mm or greater. To the best of our knowledge, this is the largest study to date examining the relationship between SCH and FNOM. The presence of a SCH alone is not associated with a high risk for FNOM contrary to previous literature. However, SCH thickness was larger in those who failed.

[Diagnostics and treatment of splenic abscesses]. / Diagnostik und Therapie des Milzabszesses.

A splenic abscess is a rare disease found in less than 1% of all autopsy studies. Several different diseases are associated as a predisposing factor, such as septic bacteremia due to endocarditis or diverticulitis, previous splenic trauma, immunosuppressive medication or diseases. The reported mortality in the literature is up to 24.5% in correctly diagnosed and treated cases. The diagnostic work-up primarily comprises sonography and computed tomography as well as a percutaneous puncture for determination of the pathogen. In most cases, a percutaneous interventional drainage treatment is sufficient and a splenectomy is necessary only in refractory cases.

Safety and Effectiveness of Sclerotherapy for Nonparasitic Splenic Cysts: A Systematic Review and Meta-Analysis.

PURPOSE: To assess the reported safety and effectiveness of sclerotherapy for the treatment of nonparasitic splenic cysts through a systematic review and meta-analysis. MATERIALS AND METHODS: A systematic search of PubMed MEDLINE, Embase, Web of Science, and the Cochrane Library through July 2023 was performed. Studies including at least 5 patients reporting percutaneous sclerotherapy of nonparasitic splenic cysts, initial and posttreatment cyst size, clinical symptoms as well as adverse events (AEs), and recurrence rates were included. A 0-8-point scale for case reports and case series was used to assess bias. Data were analyzed using random-effects meta-analysis. RESULTS: Twenty-three of 833 citations were selected for full-text assessment, and 7 studies were included for a total of 99 patients. The methodological quality of the studies included scored 3-7. Composite analysis demonstrated 38% (95% CI, 23%-55%) rate of recurrence after treatment with significant heterogeneity; however, when assessed for a cyst size of <8 cm, recurrence dropped to 7% (95% CI, 2%-20%). Residual symptoms after treatment completion were present in 17% (95% CI, 7%-33%). Intraprocedural and postprocedural AE rates were 6% (95% CI, 3%-13%) and 6% (95% CI, 3%-12%) respectively. CONCLUSIONS: Sclerotherapy of splenic cysts seemed to be safe, with a high rate of recurrence for cysts ≥8 cm.

Splenic abscess due to non-operative management of splenic injury: a case report.

BACKGROUND: Splenic abscess is a rare disease, with incidence of 0.2-0.7% in previous studies. It often appears with left upper quadrant abdominal pain, fever, chills. Splenic abscess often happens because of hematogenous spreading of infections, endocarditis, angioembolization and some other rare reasons. Treatment relies on one of these two methods: percutaneous drainage or surgery. CASE PRESENTATION: A 68-year-old diabetic Asian female (Asian woman) presented with generalized abdominal pain, low blood pressure, tachycardia, fever, lethargy and elevated level of blood sugar. She had history of conservative therapy in intensive care unit due to blunt abdominal trauma and splenic injury. She had a huge splenic abscess in ultrasonography and computed tomography scan so she went under splenectomy. Our patient had a splenic abscess without performing any intervention like angioembolization. CONCLUSION: Immune compromised patients who are selected for nonoperative management after splenic injury need close follow up and evaluating about abscess formation for at least 2 weeks. Early diagnosis and treatment with two methods including percutaneous drainage or splenectomy should be considered and it depends on patient's risk factors, vital signs, general conditions and presence or absence of sepsis.

Primary non-parasitic splenic cyst: US- and fluoroscopy-guided percutaneous management by alcohol sclerosis on six patients.

PURPOSE: This manuscript aims to report on a retrospective analysis of six patients treated with combined US- and fluoroscopic-guided percutaneous alcohol sclerosis for primary non-parasitic splenic cysts. METHODS: In this retrospective analysis, three females and three males affected by primary non-parasitic splenic cysts were included. All except one were symptomatic. Preoperative cyst diameter was in mean 113 mm (range: 67-210 mm). Ethanol 96% was adopted as sclerosant agent; the amount of ethanol injected corresponded to the 20%-30% of the cystic volume. US follow-up was planned at 2/4 weeks; MR follow-up was conducted almost at 6 months after the last treatment session. Technical success was considered as cyst disappearance or reduction of the maximum diameter <50 mm; clinical success, in those symptomatic cases, was considered as symptoms resolution or marked improvement. RESULTS: Eleven procedures had been performed: one in three patients, three in two patients and two in one patient. Technical success was 83.3%; clinical success was 80%. Only one patient, with a preoperative cystic diameter of 210 mm and despite three treatment sessions, had an increase in the cystic size and did not report symptoms improvement. CONCLUSIONS: In this sample, US-guided percutaneous alcohol sclerosis was a safe and effective spleen preserving option to treat primary non-parasitic splenic cysts.

The Utility of Serial Hemoglobin Monitoring in Non-Operative Management of Blunt Splenic Injury.

BACKGROUND: The Eastern Association for the Surgery of Trauma (EAST) states there is not enough evidence to recommend a particular frequency of measuring Hgb values for non-operative management (NOM) of blunt splenic injury (BSI). This study was performed to compare the utility of serial Hgb (SHgb) to daily Hgb (DHgb) in this population. METHODS: We conducted a retrospective chart review of patients with BSI between 2013 and 2019. Demographics, comorbidities, lab values, clinical decisions, and outcomes were gathered through a trauma database. RESULTS: A total of 562 patients arrive in the trauma bay with BSI. In the NOM group, 297 were successful and 37 failed NOM. Of those that failed NOM, 8 (21.6%) changed to OM due to a drop in Hgb. 5 (62.5%) were hypotensive first, 2 (25%) were no longer receiving SHgb, and 1 (12.5%) had a repeat CT scan and was embolized. DHgb patients were not significantly different from SHgb patients in injury severity, length of stay, the largest drop in Hgb, and incidence of failing NOM. Patients taking aspirin were more likely to fall below 7 g/dl at 48 and 72 hours into admission. CONCLUSIONS: These results suggest that that trending SHgb may not influence clinical decision-making in NOM of BSI. Besides taking aspirin, risk factors for who would benefit from SHgb were not identified. Patients who received DHgb had similar injuries and outcomes than patients who received SHgb. Prospective studies are needed to evaluate the clinical utility of SHgb compared to DHgb.

Hyposplenism and Gastrointestinal Diseases: Significance and Mechanisms.

BACKGROUND: Functional hyposplenism is a recognized complication of several gastroenterological disorders, including coeliac and inflammatory bowel diseases, and is believed to contribute to the increased infection risk seen in these disorders. SUMMARY: The mechanisms of hyposplenism are poorly understood. In this article, we review possible mechanisms underlying development of functional hyposplenism and discuss implications for its management. KEY MESSAGES: Identifying functional hyposplenism is important, as it may permit earlier recognition and treatment of serious infections through patient education and vaccination.

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.

Patient with H syndrome, cardiogenic shock, multiorgan infiltration, and digital ischemia.

BACKGROUND: H syndrome (HS) is a rare autoinflammatory disease caused by a mutation in the solute carrier family 29, member 3 (SCL29A3) gene. It has a variable clinical presentation and little phenotype-genotype correlation. The pathognomonic sign of HS is cutaneous hyperpigmentation located mainly in the inner thighs and often accompanied by other systemic manifestations. Improvement after tocilizumab treatment has been reported in a few patients with HS. We report the first patient with HS who presented cardiogenic shock, multiorgan infiltration, and digital ischemia. CASE PRESENTATION: 8-year-old boy born to consanguineous parents of Moroccan origin who was admitted to the intensive care unit during the Coronavirus Disease-2019 (COVID-19) pandemic with tachypnoea, tachycardia, and oliguria. Echocardiography showed dilated cardiomyopathy and severe systolic dysfunction compatible with cardiogenic shock. Additionally, he presented with multiple organ dysfunction syndrome. SARS-CoV-2 polymerase chain reaction (PCR) and antibody detection by chromatographic immunoassay were negative. A previously ordered gene panel for pre-existing sensorineural hearing loss showed a pathological mutation in the SCL29A3 gene compatible with H syndrome. Computed tomography scan revealed extensive alveolar infiltrates in the lungs and multiple poor defined hypodense lesions in liver, spleen, and kidneys; adenopathy; and cardiomegaly with left ventricle subendocardial nodules. Invasive mechanical ventilation, broad antibiotic and antifungal coverage showed no significant response. Therefore, Tocilizumab as compassionate use together with pulsed intravenous methylprednisolone was initiated. Improvement was impressive leading to normalization of inflammation markers, liver and kidney function, and stabilising heart function. Two weeks later, he was discharged and has been clinically well since then on two weekly administration of Tocilizumab. CONCLUSIONS: We report the most severe disease course produced by HS described so far in the literature. Our patient's manifestations included uncommon, new complications such as acute heart failure with severe systolic dysfunction, multi-organ cell infiltrate, and digital ischemia. Most of the clinical symptoms of our patient could have been explained by SARS-CoV-2, demonstrating the importance of a detailed differential diagnosis to ensure optimal treatment. Although the mechanism of autoinflammation of HS remains uncertain, the good response of our patient to Tocilizumab makes a case for the important role of IL-6 in this syndrome and for considering Tocilizumab as a first-line treatment, at least in severely affected patients.

Trends in Blunt Splenic Injury Management: The Rise of Splenic Artery Embolization.

INTRODUCTION: Splenic injury is common in blunt trauma. We sought to evaluate the injury characteristics and outcomes of BSI admitted over a 10-y period to an academic trauma center. METHODS: A retrospective review of adult blunt splenic injury patients admitted between January 2009 and September 2018. RESULTS: The 423 patients meeting inclusion criteria were divided by management: Observational (OBS, n = 261), splenic surgery (n = 114 including 4 splenorrhaphy patients), SAE (n = 43), and multiple treatment modalities (3 had SAE followed by surgery and 2 OBS patients underwent splenic surgery at readmission). The most common mechanism of injury was motor vehicle collision (47.8%). The median ISS (OBS 17, SAE 22, Surgery 34) and spleen AIS (OBS 2, SAE 3, Surgery 4) were significantly different.  Complication rates (OBS 21.8%, SAE 9.3%, Surgery 45.6%) rates were significantly different, but mortality (OBS 7.3%, SAE 2.3%, Surgery 13.2%), discharge to home and readmission rates were not. Additional abdominal injuries were identified in 26.3% of the surgery group and 2.7% of OBS group. SAE rate increased from 3.0% to 28%; median spleen AIS remained 2-3. Thirty-five patients expired; 28 had severe head, chest, and/or extremity injuries (AIS ≥4). CONCLUSION: SAE rates increased over time. Splenorrhaphy rates were low. SAE was associated with relatively low rates of mortality and complications in appropriately selected patients.

Splenic Abscess Following Sleeve Gastrectomy: A Systematic Review of Clinical Presentation and Management Methods.

BACKGROUND: Splenic abscess is a rare complication following sleeve gastrectomy. METHODS: We performed a systematic review to clarify its clinical significance, presentation, and management. PubMed, Embase, MEDLINE, Google Scholar, and the Cochrane Library were searched up to the 19th of July 2020. A total of 18 patients were included, of which 11 were female and 7 were male. The mean age was 34.1 ± 12.3 years, and the mean body mass index was 45.8 ± 7.6 kg/m2. Type 2 diabetes mellitus was reported in 11.1% of patients and hypertension in 22.2%. Fever was the most common presenting symptom seen in 17 (94.4%) patients, followed by abdominal pain in 10 (55.6%). The mean duration from surgery to presentation was 98.6 ± 132.7 days (range 10-547 days). Computed tomography was used for investigations in 17/18 (94.4%) patients. Seven patients had reported leak, three reported bleeding, and 2 reported pleural effusion. Thirteen patients had unilocular abscess. All patients were treated with antibiotics. Four patients needed total parenteral nutrition, and three were given proton pump inhibitor. In total, 11 patients needed percutaneous drainage as a part of treatment and 11 patients needed total splenectomy and 1 needed partial splenectomy. CONCLUSION: Splenic abscess following sleeve gastrectomy is a rare identity. The etiology of formation of splenic abscess needs further studies. A computed tomography of the abdomen with contrast is the preferred diagnostic tool. There is no gold standard treatment for splenic abscess.

Diagnosis and clinical management of hepatosplenic schistosomiasis: A scoping review of the literature.

BACKGROUND: Hepatosplenic schistosomiasis (HSS) is a disease caused by chronic infection with Schistosma spp. parasites residing in the mesenteric plexus; portal hypertension causing gastrointestinal bleeding is the most dangerous complication of this condition. HSS requires complex clinical management, but no specific guidelines exist. We aimed to provide a comprehensive picture of consolidated findings and knowledge gaps on the diagnosis and treatment of HSS. METHODOLOGY/PRINCIPAL FINDINGS: We reviewed relevant original publications including patients with HSS with no coinfections, published in the past 40 years, identified through MEDLINE and EMBASE databases. Treatment with praziquantel and HSS-associated pulmonary hypertension were not investigated. Of the included 60 publications, 13 focused on diagnostic aspects, 45 on therapeutic aspects, and 2 on both aspects. Results were summarized using effect direction plots. The most common diagnostic approaches to stratify patients based on the risk of variceal bleeding included the use of ultrasonography and platelet counts; on the contrary, evaluation and use of noninvasive tools to guide the choice of therapeutic interventions are lacking. Publications on therapeutic aspects included treatment with beta-blockers, local management of esophageal varices, surgical procedures, and transjugular intrahepatic portosystemic shunt. Overall, treatment approaches and measured outcomes were heterogeneous, and data on interventions for primary prevention of gastrointestinal bleeding and on the long-term follow-up after interventions were lacking. CONCLUSIONS: Most interventions have been developed on the basis of individual groups' experiences and almost never rigorously compared; furthermore, there is a lack of data regarding which parameters can guide the choice of intervention. These results highlight a dramatic need for the implementation of rigorous prospective studies with long-term follow-up in different settings to fill such fundamental gaps, still present for a disease affecting millions of patients worldwide.

Splenic abscess diagnosed following relapsing sterile peritonitis in a peritoneal dialysis patient: A case report with literature review.

Peritoneal dialysis (PD)-related peritonitis is sometimes complicated with other infections; however, few cases of splenic abscess have been reported. We present the case of a 64-year-old PD patient with complicated splenic abscesses diagnosed following relapsing sterile peritonitis. After PD induction, he presented with turbid peritoneal fluid and was diagnosed with PD-related peritonitis. A plain abdominal computed tomography (CT) did not reveal any intra-abdominal focus of infection. After empiric intravenous antibiotics, the peritoneal dialysate was initially cleared, with a decrease in dialysate white blood cells (WBC) to 20/µL. However, WBC and C-reactive protein (CRP) levels remained elevated. A contrast-enhanced abdominal CT showed two areas of low-density fluid with no enhancement in a mildly enlarged spleen, making it difficult to distinguish abscesses from cysts. Due to relapsing sterile peritonitis, we performed an abdominal ultrasonography, and suspected splenic abscesses due to rapid increase in size. Repeated imaging tests were useful in establishing a diagnosis of splenic abscesses. Considering the persistent elevation of WBC and CRP levels, imaging findings, and episodes of relapsing peritonitis, we comprehensively formed the diagnosis, and performed a splenectomy as a rescue therapy. We should consider the possibility of other infectious foci with persistent inflammation after resolving PD-related peritonitis.