Gastric rupture caused by intragastric perforation of splenic artery aneurysm: a case report and literature review.

BACKGROUND: The rupture of splenic artery pseudoaneurysm (SAP) is life-threatening disease, often caused by trauma and pancreatitis. SAPs often rupture into the abdominal cavity and rarely into the stomach. CASE PRESENTATION: A 70-year-old male with no previous medical history was transported to our emergency center with transient loss of consciousness and tarry stools. After admission, the patient become hemodynamically unstable and his upper abdomen became markedly distended. Contrast-enhanced computed tomography performed on admission showed the presence of a splenic artery aneurysm (SAP) at the bottom of a gastric ulcer. Based on the clinical picture and evidence on explorative tests, we established a preliminary diagnosis of ruptured SAP bleeding into the stomach and performed emergency laparotomy. Intraoperative findings revealed the presence of a large intra-abdominal hematoma that had ruptured into the stomach. When we performed gastrotomy at the anterior wall of the stomach from the ruptured area, we found pulsatile bleeding from the exposed SAP; therefore, the SAP was ligated from inside of the stomach, with gauze packing into the ulcer. We temporarily closed the stomach wall and performed open abdomen management, as a damage control surgery (DCS) approach. On the third day of admission, total gastrectomy and splenectomy were performed, and reconstruction surgery was performed the next day. Histopathological studies of the stomach samples indicated the presence of moderately differentiated tubular adenocarcinoma. Since no malignant cells were found at the rupture site, we concluded that the gastric rupture was caused by increased internal pressure due to the intra-abdominal hematoma. CONCLUSIONS: We successfully treated a patient with intragastric rupture of the SAP that was caused by gastric cancer invasion, accompanied by gastric rupture, by performing DCS. When treating gastric bleeding, such rare causes must be considered and appropriate diagnostic and therapeutic strategies should be designed according to the cause of bleeding.

Spontaneous rupture of a mycotic splenic artery pseudoaneurysm secondary to histoplasmosis: a case report.

BACKGROUND: A splenic artery pseudoaneurysm is a rare pathology that occurs mainly secondary to pancreatitis, abdominal trauma, peptic ulcers, pancreatic and gastric cancers, and infections. It is best diagnosed using computed tomography angiography and typically treated using endovascular embolization and, in some cases, open or laparoscopic surgery. In this report, we present a case of a ruptured mycotic splenic artery pseudoaneurysm containing Histoplasma capsulatum, which to our knowledge is the first case to report a mycotic splenic artery pseudoaneurysm of this nature. CASE PRESENTATION: We report a case of a 42-year-old white male with past medical history of Hepatitis C and IV drug abuse who presented to the Emergency Department with a 24-h history of severe diffuse abdominal pain. He was tachycardic and peritonitic on exam. Work-up demonstrated leukocytosis and lactic acidosis. Computed tomography of the abdomen and pelvis with intravenous contrast showed hemoperitoneum and active extravasation of contrast from the splenic artery into the splenic hilum, associated with a surrounding hematoma measuring 5.3 × 5.0 cm, concerning for ruptured splenic artery pseudoaneurysm. The patient was taken emergently for exploratory laparotomy, where a large intraperitoneal hematoma was evacuated. A ruptured splenic artery pseudoaneurysm was identified, isolated, and controlled, followed by completion splenectomy. Final pathology demonstrated a 3.0 × 1.3 × 0.3 cm pseudoaneurysm wall and a 14 × 9.5 × 5.5 cm spleen containing multiple necrotizing granulomata positive for the presence of Histoplasmosis species. The patient recovered appropriately and was discharged on post-operative day five. CONCLUSIONS: This case demonstrates a successful approach to a ruptured mycotic splenic artery pseudoaneurysm resulting in a positive outcome. It is a unique case as it highlights, to our knowledge, the first report of splenic artery aneurysm secondary to Histoplasma capsulatum infection. This report helps further the understanding of the pathophysiology as well as the natural history of mycotic splenic pseudoaneurysms.

When Ascites Is Not Ascites!

Patients presenting with ascites should be properly evaluated to differentiate potential etiologies. Then, based on the evaluation, we can tailor more accurate treatment plans for patients. Cirrhosis is the most common cause, and others include cancer, heart failure, and, in our case, rarely a visceral artery rupture. Rupture of the splenic artery aneurysm can be lethal and should be considered as a possible differential in a patient with no previous history of heart failure, cancer, or cirrhosis. Our patient was identified after an initial misdiagnosis of possible ascites secondary to cirrhosis. However, input from an interventional radiologist led to proper identification and tailored management. Early treatment is crucial to prevent complications, including death.

Treatment strategy for splenic artery aneurysms and novel classification based on imaging.

OBJECTIVE: The incidence of splenic artery aneurysms (SAAs) has increased with advances in imaging techniques, necessitating a comprehensive classification to guide treatment strategies. This study aims to propose a novel classification system for SAAs based on aneurysm characteristics and to review treatment outcomes at our center. METHODS: This retrospective study included 113 patients with SAAs admitted to Peking Union Medical College Hospital from January 2019 to December 2023, assessed using computed tomography angiography or digital subtraction angiography. A new classification system was devised based on the aneurysm location, morphology, integrity, and parent artery anatomy. Treatment strategies were determined based on these characteristics, with interventions ranging from endovascular therapy to laparoscopic and open surgery. Patients were followed up after the intervention to assess mortality, complications, reinterventions, and aneurysm-related outcomes. RESULTS: The study cohort of 113 patients with 127 SAAs had a predominance of female patients (63.7%) and a mean age of 52.7 years. The SAAs were classified into five types, with type I being the most common. The intervention techniques varied across types, with sac embolization, covered stent implantation, and artery embolization being the most frequently used. The overall technical success rate was 94.7%, with perioperative complication and reintervention rates of 25.0% and 0.9%, respectively, and no deaths within 30 days after the intervention. The median follow-up duration was 21 months, with overall complications rate of 3.5% and no aneurysm-related complications or deaths. CONCLUSIONS: The proposed classification system effectively guides the selection of treatment strategies for SAAs, incorporating key anatomical and morphological features. This system facilitated high technical success and low complication rates, underscoring the importance of tailored techniques in managing SAAs.

Endovascular Stent-Graft Repair of True and False Aneurysms of the Splenic Artery.

OBJECTIVE: In treatment of aneurysms (SAAs) and pseudoaneurysms (SAPs) of the splenic artery, endovascular coil embolization is the approach most commonly used as it is minimally invasive and safe. However, it carries a significant rate of primary failure (up to 30%) and might be complicated by splenic infarction. The use of stent grafts might represent a valuable alternative when specific anatomical criteria are respected. We report a comprehensive review on technical and clinical outcomes achieved in this setting. Methods: We performed a comprehensive review of the literature through the MedLine and Cochrane databases (from January 2000 to December 2023) on reported cases of stenting for SAAs and SAPs. Outcomes of interest were clinical and technical success and related complications. The durability of the procedure in the long-term was also investigated. Results: Eighteen papers were included in the analysis, totalling 41 patients (n = 20 male 48.8%, mean age 55.5, range 32-82 years; n = 31, 75.6% SAAs). Mean aneurysm diameter in non-ruptured cases was 35 mm (range 20-67 mm), and most lesions were detected at the proximal third of the splenic artery. Stent grafting was performed in an emergent setting in n = 10 (24.3%) cases, achieving immediate clinical and technical success rate in 90.2% (n = 37) of patients regardless of the type of stent-graft used. There were no procedure-related deaths, but one patient died in-hospital from septic shock and n = 2 (4.9%) patients experienced splenic infarction. At the last available follow-up, the complete exclusion of the aneurysm was confirmed in 87.8% of cases (n = 36/41), while no cases of aneurysm growing nor endoleak were reported. None of the patients required re-intervention during follow-up. Conclusions: When specific anatomical criteria are respected, endovascular repair of SAAs and SAAPs using stent grafts appears to be safe and effective, and seems to display a potential advantage in respect to simple coil embolization, preserving the patient from the risk of end-organ ischemia.

Segmental arterial mediolysis leading to spontaneous rupture of splenic artery and fatal hemorrhage in pregnancy.

We report an unexpected death of a 22-year-old primigravida who was admitted to the hospital with sudden abdominal pain two days before a scheduled delivery. During an emergency caesarean section due to intrauterine asphyxia, intraabdominal bleeding was observed with no apparent source of bleeding. Newly formed blood clots in the subdiaphragmatic space and arterial bleeding near the splenic hilum required a surgery on the next day. Hemorrhagic shock led to multiple organ failure on the fourth day of admission. The autopsy revealed ruptured splenic artery at the pancreatic tail and near the splenic hilum. Microscopically, different stages of segmental arterial mediolysis were observed in partially thinned and aneurysmatic artery.

Emergent method for management of splenic artery aneurysms rupture: A case report.

INTRODUCTION AND IMPORTANCE: Although endovascular therapy is becoming more used for the treatment of splenic artery aneurysms (SAAs) instead of open surgery, there is limited information available on the emergent hybrid approach, selectively. We present our experience of hybrid therapy using an emergent endovascular balloon for inflow control and open resection. CASE PRESENTATION: A 34-year-old woman was brought to the emergency room after it was reported that she had a pseudoaneurysm in her splenic artery at a different medical facility. The patient was hemodynamically stable. Then we underwent a combination of endovascular and open procedures, using balloon proximal control and open aneurysm resection. She was discharged from hospital on the fifth postoperative day after the operation. CLINICAL DISCUSSION: There is no agreement on how to treat SAA patients. Endovascular procedures such as endovascular intervention are also being used, minimizing the risks of surgery and shortening the patient's hospital stay, but complications remain. We propose to try SAA's emergency hybrid strategy operation with a good prognosis and fewer complications. CONCLUSION: It seems that, compared to open surgery alone when endovascular procedures were impossible, elective hybrid procedures are more secure and efficient in stable patients and could make the operation easier without more dissection for proximal control of splenic artery.

Robotic resection for splenic artery aneurysm associated with neurofibromatosis type 1: a case report.

BACKGROUND: Neurofibromatosis type 1 is an autosomal-dominant disease characterized by café-au-lait spots and neurofibromas, as well as various other symptoms in the bones, eyes, and nervous system. Due to its connection with vascular fragility, neurofibromatosis type 1 has been reported to be associated with vascular lesions, such as aneurysms. However, there have been few reports of abdominal visceral aneurysms associated with neurofibromatosis type 1. Furthermore, there have been no reports of robotic treatment of aneurysms associated with neurofibromatosis type 1. In this report, we describe the case of a patient with neurofibromatosis type 1 with a splenic artery aneurysm who was successfully treated with robotic surgery. CASE PRESENTATION: This report describes a 41-year-old Asian woman with a history of neurofibromatosis type 1 who was referred to our hospital for evaluation of a 28 mm splenic artery aneurysm observed on abdominal ultrasound. The aneurysm was in the splenic hilum, and transcatheter arterial embolization was attempted; however, this was difficult due to the tortuosity of the splenic artery. Thus, we suggested minimally invasive robotic surgery for treatment and resection of the splenic artery aneurysm with preservation of the spleen. The postoperative course was uneventful, and the patient was discharged on the eighth day after surgery. At 1 year of follow-up, the patient was doing well, with no evidence of recurrence. CONCLUSION: We encountered a rare case of splenic artery aneurysm in a patient with neurofibromatosis type 1 who was successfully treated with robotic surgery. There is no consensus on treatment modalities for neurofibromatosis-related aneurysms, and endovascular treatment is considered safe and effective; however, surgery remains an important treatment modality. Especially in patients with stable hemodynamic status, robotic surgery may be considered as definitive treatment. To our knowledge, this is the first successfully treated case of a splenic artery aneurysm in a patient with neurofibromatosis type 1.

Endovascular Strategies and Outcomes for Aberrant Splenic Artery Aneurysms.

OBJECTIVES: Aberrant splenic artery aneurysms (ASAAs) located at the splenomesenteric trunk (SMT) and the celiacomesenteric trunk have a close anatomical relationship with the superior mesenteric artery (SMA). The aim of this study was to review our institutional experience of endovascular treatment for ASAAs and evaluate the long-term outcomes. METHODS: A retrospective review of patients with ASAAs who underwent endovascular treatment between December 2006 and December 2022 was performed. The demographics of the patients, aneurysm characteristics, treatment strategies, perioperative and long-term outcomes, and complications were analyzed. RESULTS: A total of 29 patients with ASAAs were endovascularly treated at our institution. The SMT variant occurred in the majority of the patients. All ASAAs were characterized by eccentric growth and extremely short inflow arteries. Only 1 patient's inflow artery of the aneurysm exceeded 1 cm in length. Thirteen patients were treated by coil embolization alone. Four patients received bare stent-assisted coil embolization. A combination of coil embolization and covered stent placement across the orifice of the aberrant splenic artery was performed in the remaining 12 cases. Coil migration into the SMA occurred in 2 patients during the operation. Technical success was achieved in all patients. With a median duration of 63 (34-101) months of follow-up, no intestinal ischemia, aneurysm-related death, aneurysm rupture, or sac enlargement occurred. Three cases of aneurysm sac reperfusion were observed, and 1 patient underwent reintervention with secondary embolization. Asymptomatic occlusion of the covered stent was detected in 1 patient at 2 years. CONCLUSIONS: Endovascular treatment is a safe, effective, and durable option for ASAAs. Inflow embolization might be difficult to achieve in ASAAs and poses a high risk of coil migration into the SMA. Long-term observation indicates that reasonable use of the covered stent could achieve reliable inflow artery exclusion in ASAAs without intestinal complications. CLINICAL IMPACT: Aberrant splenic artery aneurysm (ASAA) is an extremely rare entity. This study reported a large sample size of ASAAs treated by endovascular techniques with long-term follow-up. The ASAA was characterized by an extremely short inflow artery and a close anatomical relationship with the superior mesenteric artery (SMA). Endovascular treatment is a safe, effective, and durable option for ASAAs. Inflow embolization might be difficult to achieve in ASAAs and pose a high risk of coil migration into the SMA. Long-term observation indicates that reasonable use of the covered stent could achieve reliable inflow artery exclusion in ASAAs without intestinal complications.

The natural history and long-term follow-up of splenic artery aneurysms.

OBJECTIVE: Although splenic artery aneurysms (SAAs) are the most common visceral aneurysm, there is a paucity of literature on the behavior of these entities. The objective of this study was to review the natural history of patients with SAA. METHODS: This single-institution, retrospective analysis studied patients with SAA diagnosed by computed tomography imaging between 2015 and 2019, identified by our institutional radiology database. Imaging, demographic, and clinical data were obtained via the electronic medical record. The growth rate was calculated for patients with radiologic follow-up. RESULTS: The cohort consisted of 853 patients with 890 SAAs, of whom 692 were female (81.2%). There were 37 women (5.3%) of childbearing age (15-50 years). The mean age at diagnosis was 70.9 years (range: 28-100 years). Frequently observed medical comorbidities included hypertension (70.2%), hypercholesterolemia (54.7%), and prior smoking (32.2%). Imaging indications included abdominal pain (37.3%), unrelated follow-up (28.0%), and follow-up of a previously noted visceral artery aneurysm (8.6%). The mean diameter at diagnosis was 13.3 ± 6.3 mm. Anatomic locations included the splenic hilum (36.0%), distal splenic artery (30.3%), midsplenic artery (23.9%), and proximal splenic artery (9.7%). Radiographically, the majority were saccular aneurysms (72.4%) with calcifications (88.5%). One patient (38-year-old woman) was initially diagnosed at the time of rupture of a 25 mm aneurysm; this patient underwent immediate endovascular intervention with no complications. The mean clinical follow-up among 812 patients was 4.1 ± 4.0 years, and the mean radiological follow-up among 514 patients was 3.8 ± 6.8 years. Of the latter, 122 patients (23.7%) experienced growth. Aneurysm growth rates for initial sizes <10 mm (n = 123), 10 to 19 mm (n = 353), 20 to 29 mm (n = 34), and >30 mm (n = 4) were 0.166 mm/y, 0.172 mm/y, 0.383 mm/y, and 0.246 mm/y, respectively. Of the entire cohort, 27 patients (3.2%) eventually underwent intervention (81.5% endovascular), with the most common indications including size/growth criteria (70.4%) and symptom development (18.5%). On multivariate analysis, only prior tobacco use was significantly associated with aneurysm growth (P = .028). CONCLUSIONS: The majority of SAAs in this cohort remained stable in size, with few patients requiring intervention over a mean follow-up of 4 years. Current guidelines recommending treatment of asymptomatic aneurysms >30 mm appear appropriate given their slow progression. Despite societal recommendations for intervention for all SAAs among women of childbearing age, only a minority underwent vascular surgical consultation and intervention in this series, indicating that these recommendations are likely not well known in the general medical community.

A case of multifocal sclerosis angiomatoid nodular transformations of the spleen occurring after partial splenic infarction with transcatheter arterial embolization for splenic artery aneurysm.

A 48-year-old woman underwent transcatheter arterial embolization (TAE) for a splenic artery aneurysm, which resulted in a partial splenic infarction in the middle lobe. Five years after TAE, a 20-mm diameter mass in the noninfarcted area of the spleen was detected on imaging, which grew to 25 mm in diameter after 6 months. MRI after gadolinium administration showed a 35 × 34 mm mass within the superior pole and 15 × 12 mm mass within the inferior pole. The patient underwent laparoscopic splenectomy and had an uneventful postoperative recovery. No evidence of recurrence was observed during the 2-year follow-up period after surgery. The mass was pathologically confirmed to be sclerosing angiomatoid nodular transformation (SANT) of the spleen. While some studies hypothesize that SANT is a response to vascular injury or trauma, to the best of our knowledge, there have been no previous reports of SANT occurring after procedures directly affecting splenic blood flow. Additionally, multifocal SANTs are reported to be very rare, accounting for only 4.7% of all reported SANTs of the spleen. We highlight a rare course of SANT of the spleen and discuss the possible relationship between blood flow abnormalities and the appearance of SANT.

Giant splenic artery aneurysm: case report.

True splenic artery aneurysms are exceedingly rare and the medical literature contains only a limited number of reports on this pathology. Presently, there remains a lack of consensus regarding the optimal management and treatment approaches for patients in this category. Over the course of the last century, significant changes have occurred in the realm of surgical options, transitioning from open and endovascular procedures to the more advanced laparoscopic and robotic interventions. The propensity for these aneurysms to rupture underscores the need for timely intervention. The risk of rupture is notably elevated in patients harboring giant splenic artery aneurysms. In this report, we present the case of a 55-year-old woman diagnosed with a giant splenic artery aneurysm measuring 12x12 cm in diameter. She presented with notable weakness, discomfort, and pain in the left subcostal area. In response to her complaints and after thorough evaluation, we opted for a surgical procedure encompassing distal pancreatic resection in conjunction with splenectomy and resection of the giant splenic artery aneurysm.

Os aneurismas verdadeiros da artéria esplênica são extremamente raros, e há um número limitado de relatos sobre essa condição na literatura médica. Atualmente, não há consenso sobre as abordagens ideais de manejo e tratamento para pacientes que se enquadram nessa categoria. Ao longo do século passado, ocorreram mudanças significativas no domínio das opções cirúrgicas, passando de procedimentos abertos e endovasculares para intervenções laparoscópicas e robóticas mais avançadas. A propensão à ruptura do aneurisma ressalta a necessidade de intervenção em tempo oportuno. O risco de ruptura é notavelmente elevado em pacientes com aneurismas gigantes da artéria esplênica. Neste relato, apresentamos o caso de uma mulher de 55 anos diagnosticada com aneurisma gigante de artéria esplênica medindo 12x12 cm de diâmetro. A paciente apresentava fraqueza notável, desconforto e dor na região subcostal esquerda. Em resposta às suas queixas e após avaliação minuciosa, optamos por um procedimento cirúrgico que incluiu pancreatectomia distal associada a esplenectomia e ressecção do aneurisma gigante da artéria esplênica.

Endovascular Stent Graft Placement and Coil Embolization for Splenic Artery Aneurysm with an Anatomical Variant.

A 62-year-old woman with abdominal pain was diagnosed with a splenic artery aneurysm (SAA) and an anatomical variant in the splenic artery (SA) arising from the superior mesenteric artery (SMA) as its first branch. To treat the SAA, the draining artery and a small branch of the SAA were embolized, and then small-diameter stent grafts were deployed from SMA orifice, covering the aberrant origin of the SA and preserving the second branch of SMA. Intraoperative angiography confirmed successful exclusion of the SAA without endoleak or arterial dissection. The stent graft was patent and the aneurysm had shrunk 3.5 years after the operation.

A Rare Case of Splenic Artery Aneurysm With Unusual Combination of Pancytopenia and Massive Splenomegaly in a Tertiary Care Hospital.

Aneurysms of the splenic artery leading to extrahepatic portal hypertension are sporadic and infrequently encountered. They typically manifest as a consequence of thrombus or embolus formation. A splenic artery aneurysm (SAA) represents a localized expansion in the diameter of the splenic artery and is one of the most prevalent forms of visceral artery aneurysms. This artery dilation is primarily attributed to pancreatitis, trauma, or atherosclerosis, commonly affecting elderly patients. Patients affected by this condition typically remain asymptomatic, except for an audible bruit over the aneurysm site, unless a rupture occurs. In the event of a rupture, early indicators include abdominal pain, hemoperitoneum, and a positive Kerr sign, all indicative of SAA rupture. Most SAAs are incidentally discovered, with CT angiography being the preferred diagnostic tool. We present the case of a 38-year-old female (gravida 1, para 1) with a previous full-term normal delivery, who presented to a rural tertiary care hospital with a two-week history of left-sided abdominal pain. A CT scan of the abdomen revealed a solitary aneurysm in the distal portion of the splenic artery, accompanied by perisplenic fluid collection resulting in splenomegaly. Given the critical risk of rupture, which can result in life-threatening bleeding, prompt and accurate diagnosis assumes paramount significance. It is worth noting that the diagnosis of SAA often occurs incidentally due to its asymptomatic nature in its early stages. We document this unique occurrence of extrahepatic SAA contributing to pancytopenia, portal hypertension, and extensive splenomegaly to provide valuable insights for medical professionals in recognizing and managing such presentations. This awareness can help prevent unnecessary diagnostic and therapeutic interventions.

Rupture of Splenic Artery Aneurysm in Patient with ACTN2 Mutation.

Here, we report a case of splenic artery aneurysm rupture in a patient with known heterozygosity mutation of the ACTN2 gene (variant c.971G > A p.Arg324Gln). The patient came to our emergency department with epigastric pain radiating to the lumbar area, with an absence of peritonism signs. An abdominal computed tomography angiography showed a ruptured huge (5 cm) splenic artery aneurysm. Therefore, the patient underwent emergency endovascular coil embolization with complete aneurysm exclusion. The postoperative course was uneventful, until postoperative day five when the patient developed a symptomatic supraventricular tachycardia in the absence of echocardiographic alterations. The signs and symptoms disappeared after three days of medical management. The patient was discharged on the 14th postoperative day in good clinical condition under verapamil and anti-platelet therapy. Although ACTN2 mutation was associated with cardiac and peripheral vascular disease occurrence, to the best of our knowledge, the present case is the first report of a visceral (splenic) aneurysm directly linked with this rare mutation.

Hybrid Approach for Treatment of a Symptomatic Giant Splenic Artery Aneurysm.

A 56-year-old female presented with a symptomatic giant fusiform mid-splenic artery aneurysm (7.3 x 6.4 cm). The patient underwent hybrid management of the aneurysm with endovascular embolization of the aneurysm and inflow splenic artery followed by laparoscopic splenectomy with control and division of the outflow vessels. The patient had an uneventful post-operative course. This case demonstrates the safety and efficacy of an innovative, hybrid management of a giant splenic artery aneurysm with endovascular embolization and laparoscopic splenectomy that spares the pancreatic tail.

Endovascular management of incidentally discovered splenic arteriovenous fistula resulting from ruptured splenic aneurysm: Case report and review of the literature.

Splenic arteriovenous fistulas (SAVFs) are rare vascular anomalies, which have a described association with splenic artery aneurysms. Treatment options include surgical fistula excision, splenectomy, or percutaneous embolization. Here we present a unique case of endovascular repair of a splenic arteriovenous fistula (SAVFs) associated with a splenic aneurysm. A patient with past medical history of early-stage invasive lobular carcinoma was referred to our interventional radiology practice to discuss an incidentally discovered splenic "vascular malformation" discovered during magnetic resonance imaging of the abdomen and pelvis. Arteriography demonstrated smooth dilatation of the splenic artery, with a fusiform aneurysm which had fistulized to the splenic vein. There were high flows and early filling of the portal venous system. The splenic artery, immediately proximal to the aneurysm sac, was catheterized using a microsystem and embolized using coils and N-butyl cyanoacrylate. Complete occlusion of the aneurysm and resolution of the fistulous connection was achieved. The patient was discharged home the following day, without complication. Associated splenic artery aneurysms and SAVFs are rare occurrences. Timely management is necessary to prevent adverse sequelae such as aneurysm rupture, further enlargement of the aneurysmal sac, or portal hypertension. Endovascular treatment, including n-Butyl Cyanoacrylate glue and coils, offers a minimally invasive treatment option, with facile recovery and low morbidity.

A ruptured splenic artery aneurysm treated by transcatheter arterial embolization using n-butyl cyanoacrylate-Lipiodol-Iopamidol.

N-butyl cyanoacrylate, one of embolic materials, is usually used as a mixture with Lipiodol (N-butyl cyanoacrylate-Lipiodol mixture). N-butyl cyanoacrylate-Lipiodol-Iopamidol was developed by adding a nonionic iodine contrast agent (Iopamiron) to N-butyl cyanoacrylate-Lipiodol mixture. N-butyl cyanoacrylate-Lipiodol-Iopamidol has lower adhesiveness than N-butyl cyanoacrylate-Lipiodol mixture and the ability to form a single large droplet. We report the case of a 63-year-old man with a ruptured splenic artery aneurysm treated by transcatheter arterial embolization using N-butyl cyanoacrylate-Lipiodol-Iopamidol. He was referred to the emergency room because of sudden onset of upper abdominal pain. A diagnosis was established using contrast-enhanced computed tomography and angiography. Emergency transcatheter arterial embolization was performed, and the ruptured splenic artery aneurysm was successfully embolized using a combination of coil framing and N-butyl cyanoacrylate-Lipiodol-Iopamidol packing. This case demonstrates the usefulness of a combination of coil framing and N-butyl cyanoacrylate-Lipiodol-Iopamdol packing for the embolization of aneurysms.

Splenic artery pathology presentation, operative interventions, and outcomes in 88 patients with vascular Ehlers-Danlos syndrome.

OBJECTIVE: Vascular Ehlers-Danlos syndrome (VEDS) is rare and associated with arteriopathies. The aim of this study is to investigate the presentation, operative interventions, and outcomes of splenic arterial pathology in a population of more than 1500 individuals with genetically confirmed VEDS due to pathogenic COL3A1 variants. METHODS: Cross-sectional analysis of 1547 individuals was performed. The data were assembled by harmonizing data from three overlapping cohorts with genetically confirmed VEDS: the VEDS Collaborative Natural History Study (N = 242), a single-center cohort (N = 75), and the University of Washington Collagen Diagnostic Lab cohort (N = 1231). Duplicates were identified and removed. Patients were selected for analysis if they had splenic artery aneurysm (SAA), pseudoaneurysm, dissection, thrombosis, or rupture. Demographics, COL3A1 variants, interventions, and outcomes were analyzed. Comparisons by splenic artery rupture were made. RESULTS: A total of 88 patients presented between 1992 and 2021 with splenic artery pathology (5.7% of the cohort; mean age at diagnosis, 37 ± 11.1 years; 50% male). One-third were diagnosed with VEDS prior to the splenic artery pathology diagnosis, and 17% were diagnosed post-mortem. Most had a positive family history (61%). Most had COL3A1 variants associated with minimal normal collagen production (71.6%). Median follow up was 8.5 years (interquartile range, 0.9-14.7 years). Initial presentation was rupture in 47% of the cases. Splenic artery rupture overall was 51% (n = 45), including four cases of splenic rupture. There were no major differences in VEDS-related manifestations or COL3A1 variant type by rupture status. SAA was noted in 39% of the cases. Only 12 patients had splenic artery diameter documented in 12 cases with a median diameter of 12 mm (interquartile range, 10.3-19.3 mm). A total of 34 patients (38.6%) underwent 40 splenic arterial interventions: 21 open surgical, 18 embolization, and one unknown procedure. More than one splenic artery intervention was performed in five cases (14.7%). Open repair complications included arteriovenous fistula (n = 1), intestinal or pancreatic injury (n = 1 each), and four intraoperative deaths. There were no deaths or access site complications related to splenic artery embolization. Four patients (23.5%) developed a new SAA in the remaining splenic artery post embolization. All-cause mortality was 35% (n = 31), including 22 related to a ruptured splenic artery. CONCLUSIONS: Splenic arteriopathy in VEDS is associated with variants that affect the structure and secretion of type III collagen and frequently present with rupture. Rupture and open repair are associated with high morbidity and mortality, whereas embolization is associated with favorable outcomes. Suggest repair considerations at SAA diameter of 15 mm. Long-term follow-up is indicated as secondary splenic arteriopathy can occur.