Massive left pulmonary artery aneurysm with a co-existing patent ductus arteriosus in a five-year-old female child: A case report.

Pulmonary Artery Aneur ysm (PAA), whether congenital or acquired, is a rare diagnostic find ing com pare d to aor tic aneur ysms. There have been fe w cases where PA As were documented as a complication of untreated Patent Ductus Ar teriosus (PDA) due to long-standing Pulmonary Arterial H ypertension (PAH). However, it is quite rare for a case of PAA to be reported with co-existing PDA without PAH. This report highlights a case of a five -year-old girl who was presented with palpitations, easy fatigability, fever, c yanos is, and vomiting. A Chest X-ray s howed mo derate cardiomega ly. A PDA of 6 mm was diagnosed on Transthoracic E chocardiog rap hy ( TTE ) and a large cavity con necte d with LPA raised suspicion of a possible LPA aneur ysm. A Chest CT scan confirm ed the diagnosis of a saccular aneurysm, originating from the distal part of the main Left Pulmonary Artery (LPA) just proximal to the point of bifurcation into lobar branches, measuring 7.5x6.5 cm. During surgery, the aneurysm was opened, emptied with suction and closed without resecting the aneur ysmal walls. The patient had an uneventful post-op course and is doing well during regular interval follow up visits.

Renal artery pseudoaneurysm following robot assisted nephron sparing surgery: two case reports.

BACKGROUND: Renal artery pseudoaneurysm following partial nephrectomy is a rare entity, the incidence of this entity is more common following penetrating abdominal injuries, percutaneous renal interventions such as percutaneous nephrostomy(PCN) or Percutaneous nephrolithotomy (PCNL). Although rare, renal artery pseudoaneurysm can be life threatening if not managed timely, they usually present within two weeks postoperatively with usual presenting complains being gross haematuria, flank pain and/or anaemia. CASE PRESENTATION: We report case of two female patients 34 and 57 year old respectively of South Asian ethnicity, presenting with renal artery pseudoaneurysm following left sided robot assisted nephron sparing surgery for interpolar masses presenting clinically with total, painless, gross haematuria with clots within fifteen days postoperatively and their successful treatment by digital subtraction angiography and coil embolization. CONCLUSION: Renal artery aneurysm is a rare fatal complication of minimally invasive nephron sparing surgery however considering the preoperative and intraoperative risk factors for its development and prompt suspicion at the outset can be life saving with coil embolization of the bleeding arterial aneurysm.

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.

Reconstructive endovascular treatment of petrous ICA pseudoaneurysm in skull base osteomyelitis: a hidden catastrophe.

Skull base osteomyelitis can be more life-threatening in immunocompromised patients and patients with diabetes. Here, we present a case of a petrous internal carotid artery pseudoaneurysm resulting from skull base osteomyelitis in a diabetic male in his 50s. This case report highlights the need to be conscious of the various complications associated with skull base osteomyelitis, be proficient in detecting them and treat them as early as possible for better outcomes. After adequate control of the disease process with medical treatment, immediate management of the aneurysm with balloon angioplasty and stenting was done. Acknowledging the trivial nasal and ear bleed, radiological evaluation is necessary to rule out rare complications like pseudoaneurysms in a diagnosed case of skull base osteomyelitis. In the discussion, we have cited the various treatment methods and similar cases of pseudoaneurysm caused by osteomyelitis. Currently, the patient continues to live a disease and disability-free life.

I saw the sign: He felt a pang and we saw the Yin-Yang (sign).

The yin-yang sign, also known as the Pepsi sign, is used to describe the classic appearance of bidirectional blood flow within an aneurysm or pseudoaneurysm sac on color Doppler ultrasound. The corresponding spectral Doppler finding is a "to-and-fro" waveform, caused by inflow to the aneurysm/pseudoaneurysm sac during systole and outflow during diastole. It is important to recognize this sign in order to quickly identify the presence of an aneurysm or pseudoaneurysm and prevent complications such as expansion and rupture.

Surgical treatment for right-side aortic arch concomitant with Kommerell's diverticulum: techniques selection and follow-up results.

BACKGROUND: Right-side aortic arch concomitant with Kommerell's diverticulum (KD) is a rare and complex ailment, and there is no consensus on the optimal strategy to deal with this congenital anomaly. We retrospectively analyzed and summary of the cases treated in our center with individual treatment methods for different situations. METHODS: Between September 2018 and December 2021, 10 patients experienced surgical therapy at our institution who presented with a Kommerell's diverticulum arising from an aberrant subclavian artery from the right-side aortic arch. Four main surgical techniques were applied to those patients: 1. total arch replacement with frozen elephant trunk implantation (n = 2); 2. hybrid procedure combining open arch repair and endovascular intervention (n = 1); 3. total endovascular repair using thoracic endovascular aortic repair (TEVAR) with or without left subclavian artery (LSCA) revascularization (n = 6); 4. direct repair underwent endoaneurysmorrhaphy. Clinical characteristics and outcomes were collected. RESULTS: The mean age of these 10 patients was 56.5 years (range 29-79 years) and only 1 woman. The pathology includes aortic dissection (n = 6) and aneurysm (n = 4). The mean diverticulum size was 41.4 [24.2-56.8] mm. There were no in-hospital deaths, and the median hospital stay was 22 [15-43] days. During the follow-up period (21.4 months, 1-44 months), one died of an unknown cause and one died of esophageal fistula. Two patients underwent second-stage endovascular intervention for distal lesion. And none of the patients had endoleak during the follow-up period. CONCLUSIONS: Each of the procedures we have mentioned here has its advantages and disadvantages; individualized treatment should meet the appropriate indications. A single-branched stent graft is feasible and effective in the treatment of aortic disease combined with Kommerell's diverticulum.

Fibromuscular dysplasia of the coronary arteries: An unusual case of sudden death and review of the literature.

Fibromuscular dysplasia of the coronary is an uncommon coronary defect with a range of pathological alterations and unpredictable clinical description that can cause sudden death. We present an autopsy case of sudden cardiac death due to a rupture of a coronary artery aneurysm in a 59-year-old woman. Postmortem autopsy revealed two huge saccular aneurysms located at the right coronary artery, one of which was ruptured leading to a fatal hemopericardium. Histopathological examination revealed coronary artery fibromuscular dysplasia with fibromyxoid dissociation of the media causing saccular aneurysms. The involvement of coronary arteries in fibromuscular dysplasia with aneurysmal features has been rarely reported in the literature and is most likely an underdiagnosed finding. Due to the little number of published studies, the etiology is not fully understood and data on pathogenesis, risk factors, manifestation, disease course, and mortality are still unclear, which is a gap that needs to be filled in order to avoid under-diagnosis of the disease. Our case report aimed to discuss the mechanisms of sudden death attributed to coronary fibromuscular dysplasia.

Enlarging aneurysm with paracentral acute middle maculopathy in idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) - a case report.

INTRODUCTION: Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome is a rare clinical entity affecting young healthy individuals. Treatment primarily involves pan retinal photocoagulation (PRP) to capillary non perfusion areas. Intravitreal anti-VEGF or steroids are given in the presence of macula edema. Oral steroids do not alter the course of the disease. Arterial occlusions have been reported in IRVAN. METHODS: Retrospective case review. RESULT: A twenty seven year old male presented to us with mild blurring of vision for one week. His BCVA was OU 20/20. Anterior segment examination was normal. Fundus examination showed bilateral disc aneurysm with OS arterial aneurysm along the inferior arcade. Fundhus fluorescein angiography and OCT angiography were confirmatory of the disc and retinal aneurysm. Capillary non perfusion (CNP) areas were noted in the periphery. Two days later he presented with paracentral scotoma in his left eye which was confirmed by Amsler chart. Fundus, OCT and OCTA were confirmatory of Paracentral Acute Middle Maculopathy (PAMM). The retinal aneurysm had increased in size from 333 micron diameter to 566 micron diameter. Panretinal photocoagulation to the CNP areas was done and intravitreal antiVEGF was given. At 6 months follow up, retinal aneurysm had disappeared. DISCUSSION: Our case describes a unique event with sudden increase in size of the aneurysm leading to acute blockage in the deep capillary plexus thus being the first report of PAMM in IRVAN. The patient was treated with PRP and intravitreal anti-VEGF for the enlarging aneurysm which reduced in size within a week.

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.

Hughes-Stovin syndrome-An important differential diagnosis in patients with suspected chronic thromboembolic pulmonary hypertension : A case report.

Hughes-Stovin syndrome (HSS) is a rare vasculitis of unknown etiology. The disease is characterized by pronounced inflammation and damage to the vessel walls, with subsequent widespread vascular thrombosis and the formation of pulmonary artery aneurysms that can lead to fatal hemoptysis. This disorder can be mistaken for other conditions, such as chronic thromboembolic pulmonary disease (CTEPD) without or with pulmonary hypertension at rest (CTEPH).We report the case of a 20-year-old female with HSS, which was misdiagnosed as CTEPH and subsequently treated with anticoagulants, which led to severe hemoptysis and eventually death of the patient. This case highlights the challenges of diagnosing HSS at early stages of the disease.HSS should be considered in young patients with signs of large vessel vasculitis in combination with thrombotic occlusions of pulmonary arteries, with or without aneurysms of the pulmonary arteries, and particularly, if there are no risk factors for thromboembolic disease.

Sudden unexpected death of a young adult due to subarachnoid hemorrhage associated with polyarteritis nodosa: Clinicopathological appearance and literature review.

A 28-year-old male was found dead in his bedroom. There were no anomalies in his birth and medical history, and there was no family history of sudden unexpected death (SUD). Autopsy showed subarachnoid hemorrhage (SAH) with basilar top inflammatory pseudoaneurysm rupture accompanied by fibrinoid necrosis in the aneurysm wall. Active and healed arteritides in small- to medium-sized arteries were identified in the brain, heart, and systemic connective tissue, which was consistent with polyarteritis nodosa (PAN). Furthermore, pneumatosis cystoides intestinalis was observed in the ascending colon. Hepatitis B virus infection and antineutrophil nuclear antibodies were negative. Genetic investigation using whole-exome sequencing showed no mutations among autoinflammatory-related genes, including UBA1, MEFV, and ADA2. SAH due to rupture of a pseudoaneurysm formed by PAN was considered as the cause of death in the present case. Although myocardial ischemia linked to coronary arteritis is a recognized trigger for SUD in PAN, our study showed that rupture of inflammatory pseudoaneurysm in the cerebral artery can also cause SUD in younger subjects with PAN, even if prodromal symptoms are not evident before death.

Pilot Study on Feasibility and Outcome of a Nerve-Preserving Aortoiliac Exposure Technique.

BACKGROUND: Dysfunctional ejaculation is a common complication following open aortoiliac aneurysm surgery. It may occur in 49-63% of patients and is caused by iatrogenic damage to the sympathetic lumbar splanchnic nerves and superior hypogastric plexus. A nerve-preserving operative technique based on a unilateral right-sided approach to the abdominal aorta, was implemented in clinical practice. The aim of this pilot study was to establish the safety and feasibility of the technique, and whether a sympathetic pathway and ejaculatory function was preserved. METHODS: Patients were asked to fill out questionnaires preoperatively, and 6 weeks, 6 months, and 9 months postoperatively. The International Index of Erectile Function, Cleveland Clinic Incontinence Score (CCIS), Patient assessment of constipation symptoms (Pac-Sym), and International Consultation on Incontinence Questionnaire on male lower urinary tract symptoms were used. Surgeons were asked to complete a technical feasibility questionnaire. RESULTS: Twenty-four patients undergoing aortoiliac aneurysm surgery were included. The nerve-sparing phase of the procedure added an average of 5-10 min of operating time and was technically feasible in twenty-two patients. No major complications occurred during nerve-sparing exposure. Fifteen of twenty-four patients were sexually active at some point throughout the study. No postoperative loss of ejaculation was seen in sexually active patients. CCIS, Pac-sym, International Index of Erectile Function, and Incontinence Questionnaire on male lower urinary tract symptoms scores remained similar throughout the study. CONCLUSIONS: Nerve-preserving aortoiliac reconstruction surgery is safe and feasible. Ejaculatory function is preserved. Given the low number of patients in the study, further research is needed to provide robust data.

Prosthetic Conduit Use Does Not Adversely Impact Outcomes after Open Repair of Popliteal Artery Aneurysms.

BACKGROUND: Single segment, greater saphenous vein (GSV) conduit is considered the optimal bypass conduit among patients undergoing bypass surgery for peripheral artery disease (PAD). While this data has been extrapolated to patients undergoing bypass for popliteal artery aneurysms (PAAs), the pathophysiology of PAA is inherently different when compared to PAD, and the impact of conduit type on long-term outcomes after open repair of PAA remains unclear. METHODS: A multicenter database of five regional hospitals was retrospectively reviewed for all patients with PAA undergoing open surgical repair. Data were collected on demographic information, operative details, medications, and postoperative outcomes. Kaplan-Meier curves were used to compare freedom from major adverse limb events (MALE) following GSV versus prosthetic bypass. Cox proportional hazards model was used to identify patient-level characteristics associated with MALE, which was defined as major ipsilateral limb amputation or reintervention for graft patency. RESULTS: From 1999 to 2020, a total of 101 patients with PAA underwent open exclusion and bypass surgery. Median follow-up period was 4.2 years (interquartile range, 1.3-7.4 years), and complete data were available for 99 (98.0%) patients. The majority of patients were male (99.0%) and Caucasian (93.9%). Only 11.1% of procedures were emergent, with the remainder (88.9%) being elective. All patients underwent medial exposure with a below-knee popliteal bypass target (100%). Bypass conduits included GSV (69.7%), prosthetic conduit (28.3%), and 2 (2.0%) alternative conduits (one spliced arm vein, one cryopreserved vein). Patients undergoing prosthetic bypass were older (72 vs. 66 years, P = 0.001) and had similar rates of medical comorbidities. Compared with the GSV group, patients with prosthetic conduits were more frequently placed on postoperative anticoagulation (60.7% vs. 23.2%, P < 0.001). Conduit type did not impact postoperative complication rates (P = NS each). MALE rates were low overall (19.2% at 2 years), and similar when stratified by conduit type (log rank P = 0.47). On multivariable analysis, emergent bypass was associated with MALE (hazard ratio [HR] 5.73, 95% confidence interval [CI] 2.07-15.85, P < 0.001). Prosthetic conduit usage (HR 1.00, 95% CI, 0.40-2.51, P = 0.99) and postoperative anticoagulation (HR 1.02, 95% CI 0.42-2.50, P = 0.97) were not associated with MALE. CONCLUSIONS: Open repair of PAA is associated with excellent long-term outcomes. Prosthetic bypass is a comparable alternative to autogenous conduit for below-knee popliteal bypass targets, and lack of suitable GSV should not prohibit open surgical repair when indicated.

Aneurysmal Disease in Patients With Takayasu Arteritis.

OBJECTIVE: Takayasu arteritis (TA) leads to stenotic disease. Aneurysmal lesions are rarer. This study assessed the main characteristics of aneurysmal disease in a Canadian cohort of patients with TA. METHODS: This monocentric retrospective study included patients with TA followed at the Mount Sinai Hospital Vasculitis Clinic in Toronto. Diagnosis of TA was based on clinical findings and/or satisfied the 1990 American College of Rheumatology classification criteria. RESULTS: Seventy-four patients were included. At any time, aneurysmal disease was found in 23 (31%) patients. Median disease duration was 9.0 (IQR 7.0-19.0) years. Prior hypertension (P = 0.02), fever (P = 0.04), and seizure disorders (P = 0.03) were more common. Limb claudication was less frequent (P = 0.01). Persistent and/or new aneurysms were demonstrated in 22/23 patients at follow-up. Thoracic aorta aneurysm (13/22) was most common, followed by abdominal aorta (8/22), subclavian (7/22), and carotid (6/22) artery disease. Aortic valve regurgitation was more frequent (9/23 vs 3/48; P = 0.001). Twenty-one patients had been treated with glucocorticoids (median 6.1 years [IQR 3.7-8.1]). Methotrexate, azathioprine, and leflunomide were repeatedly used. Infliximab (7/23) was used more often (P = 0.04), whereas tocilizumab was received by only 4 patients with aneurysmal disease (P = 0.01). Patients with aneurysms suffered more frequent relapses (2.0 [IQR 0.0-4.0] vs 1.0 [IQR 0.0-2.0], P = 0.04). CONCLUSION: Aneurysmal disease was found in a significant proportion of patients with TA. Given that aneurysms may carry a risk of rupture, and are associated with a higher rate of relapse, this finding should be reported systematically in TA studies.

Prenatal Diagnosis of Ductus Arteriosus Anomalies: A Single-Center Study.

To evaluate the fetal ductus arteriosus anomalies diagnosed by fetal echocardiography. The perinatal outcomes and associated cardiac and genetic anomalies are also explored. The fetal echocardiography records of 2366 fetuses were evaluated retrospectively. Thirty-seven pregnancies prenatally diagnosed with ductus arteriosus anomalies and evaluated after delivery were enrolled in the study. Perinatal and obstetric outcomes were analyzed. The incidence of ductus arteriosus anomaly in our series was 1.5% (37/2366). The most frequent ductus arteriosus anomaly detected was right-sided ductus arteriosus followed by aneurysm, constriction and bilateral ductus arteriosus with an incidence of 51.3%, 27.1%, 18.9% and 2.7%, respectively. There were 19 fetuses with right-sided ductus arteriosus, of which 15 had tetralogy of Fallot. There were 2 chromosomal anomalies (22q11 microdeletion) in this group. Of the 7 fetuses with ductus arteriosus constriction, 3 (3/7, 42.9%) died in-utero. There were 2 (2/10, 20%) neonatal deaths due to hypoplastic left heart syndrome in the ductus arteriosus aneurysm group. Various types of ductus arteriosus anomalies can be diagnosed prenatally. Perinatal outcomes mostly dependent on the type of the ductus arteriosus anomaly and accompanying cardiac malformations.