Renal Autotransplantation for Uncontrolled Hypertension in Nonatherosclerotic Renal Artery Stenosis-2 Case Reports and a Brief Review of the Literature.

Fibromuscular dysplasia is the most common cause of renovascular hypertension in young adults under 40 years old. It is potentially amenable to renal artery angioplasty, which frequently normalizes blood pressure. However, limited options exist if angioplasty is not technically possible, or restenosis occurs. Here, we describe 2 patients who presented with hypertension secondary to renal artery stenosis. In the first case, a young adult with hypertension secondary to renal artery stenosis (fibromuscular dysplasia), developed restenosis 11 weeks after an initially successful renal artery angioplasty. In the second case, a patient with neurofibromatosis type 1 was diagnosed with hypertension secondary to renal artery stenosis. Angioplasty was not possible due to multiple branch occlusions. Both individuals went on to have successful renal autotransplantations, which ultimately cured their hypertension. In this article, we review the background, indications, and blood pressure outcomes in relation to renal autotransplantation in nonatherosclerotic renal artery stenosis.

[Management of the patient with renal artery fibromuscular dysplasia: clinical case].

Fibromuscular dysplasia (FMD) is a rare disease that affects small and medium-sized arteries. Clinical manifestations of FMD depend on its localization. In many cases, FMD of renal arteries (RA) is associated with arterial hypertension. Young age, particularly of female patients, suspected RA dissection or kidney infarction, absence of atherosclerosis or presence of FMD in other arteries of such patients evidence for RA FMD. In invasive treatment of hemodynamically significant stenoses, transluminal balloon angioplasty (TBA) of renal arteries is preferrable. Taking into account initial alterations of the vascular wall and unevenness of the lumen of the affected blood vessel, stent implantation is associated with an increased risk of complications and is recommended only if ballooning complications develop. An open reconstructive surgery is indicated in complicated narrowing anatomy, a high risk of the endovascular treatment, or after failure of the endovascular intervention. This article presents a clinical case of a young female patient with RA FMD and renovascular arterial hypertension who successfully underwent renal TBA with a drug-coated balloon.

Fibromuscular dysplasia with recurrence after "long-term" following percutaneous transcatheter renal angioplasty: two case reports with a review of 26 patients.

BACKGROUND: Fibromuscular dysplasia (FMD) often causes renal artery stenosis with renovascular hypertension. Recent clinical outcomes encourage percutaneous transluminal renal angioplasty (PTRA) to treat FMD; however, the necessary follow-up period remains unclear. Moreover, previous studies have not revealed the difference in the period until recurrence between two major types of FMD-multifocal and focal. CASE PRESENTATION: We describe two patients with multifocal FMD who developed hypertension during their teenage years and had recurrence of FMD > 10 years after PTRA. We further examined the types of FMD and age of onset in 26 patients who underwent PTRA. The period until recurrence of multifocal FMD was longer than that of focal FMD. Moreover, patients with early-onset multifocal FMD are likely to have a delayed recurrence after PTRA compared to other types. CONCLUSIONS: Our report suggests that patients with multifocal FMD, especially those with onset at an early age, may need long-term follow-up for at least ≥ 10 years.

Ex-Vivo Repair of Complex Hilar Renal Artery Aneurysms and Auto-Transplantation of Solitary Kidney.

Renal artery aneurysms (RAA) are rare and challenging to repair. We present a case of a 48-year-old female with solitary right kidney who had complex aneurysms near the renal hilum. CT angiogram showed fibromuscular dysplasia (FMD) features of the renal artery and 2 saccular aneurysms measuring 2.3 cm and 1 cm. An additional small lower pole renal artery added to the complexity of the case. Ex-vivo aneurysm repair was performed after the kidney was removed and flushed with preservation solution. This was followed by auto-transplantation to the right external iliac vessels. The patient did well postoperatively without need for dialysis with serum creatinine returning to normal 5 weeks after.

Fibromuscular dysplasia of mesenteric arteries: a rare cause of multiple bowel resections-a case report and literature review.

BACKGROUND: Fibromuscular dysplasia (FMD) is a type of unexplained nonatherosclerotic vascular disease that usually involves the renal and internal carotid arteries and rarely involves the mesenteric artery. Mesenteric artery FMD is difficult to distinguish from Crohn's disease (CD) and Behcet's disease (BD) solely based on symptoms. Patients with mesenteric artery FMD can present with an acute abdomen, but case reports of patients who have a long medical history and undergo multiple bowel resections are extremely rare. CASE PRESENTATION: The patient was a 45-year-old woman with an 11-year history of intermittent lower abdominal pain and fever. At the age of 34 years, she underwent right hemicolectomy and appendectomy due to an acute abdomen. She suffered from oral ulcers between 34 and 36 years old. A clinical diagnosis of presumed CD was made by the age of 41, and she was treated with mesalazine; however, the effect was poor. At the age of 42, she came to our centre, and based on her atypical symptoms and examination results, we thought she had CD. Hence, she was treated with glucocorticoids for 3 years. However, when she was 45, due to steroid dependence, thalidomide tablets were added. Unfortunately, she suffered from another episode of intestinal obstruction. Therefore, she underwent enterectomy. The postoperative histopathological diagnosis was mesenteric artery FMD. She no longer underwent pharmacotherapy after the surgery. Although she did not have any of her previous symptoms and postoperative colonoscopy showed no signs of recurrence, splenomegaly and abnormal routine blood results were still present. CONCLUSIONS: Patients with mesenteric artery FMD can present with an acute abdomen. In addition, the symptoms and endoscopic manifestations of mesenteric artery FMD may appear similar to CD and BD. Hence, it is difficult to make a clear clinical diagnosis and proceed with treatment. Mesenteric artery FMD often requires surgical pathology to confirm its diagnosis. For patients who suffer from this disorder, surgery may be the best choice to improve the patient's quality of life.

Iliac Arteries Dissection with a Rapid Dilatation as Debut of Fibromuscular Dysplasia.

We report a case of a 38-year-old male diagnosed with fibromuscular dysplasia (FMD) and a dissection of both common iliac arteries without aortic involvement. It was revealed after an inguinal hematoma and a pelvic pain, which are not the typical FMD presentation. Surgical treatment was performed after a rapid iliac growth in the first month control computed tomography angiography. Although the clinical course of this entity is relatively benign, rupture of the common iliac artery has also been described.

Spontaneous Rupture of Mesenteric Vasculature Associated with Fibromuscular Dysplasia in a 28-Year-Old Male.

Spontaneous rupture of mesenteric vasculature associated with fibromuscular dysplasia is an unreported phenomenon. We describe a case in a 28-year-old male with a history of chronic abdominal pain who presented to our facility in hemorrhagic shock secondary to a ruptured transverse mesocolon middle colic aneurysm status postemergent transverse colectomy. He was found to have chronic vertebral, renovisceral, and iliac aneurysms as well as acute superior and inferior mesenteric artery dissection and chronic bilateral vertebral artery dissections. He subsequently developed disseminated intravascular coagulopathy, resulting in saddle pulmonary embolus as well as right renal artery and splenic artery thrombosis. Ultimately, the patient expired.

Surgical Treatment of Carotid Webs in Symptomatic Young Adults.

BACKGROUND: Carotid webs are possibly responsible for ischemic stroke in young adults. We aim to report a case series study about surgical treatment of this uncommon condition. METHODS: We performed a retrospective analysis on clinical, imaging, pathological, operative data, and outcomes about consecutive patients affected with carotid ischemic stroke due to carotid webs in a single institution. RESULTS: During a three-year period, a total of 123 patients were treated for carotid stroke, in five cases (4%) (median age 48.4 years, range 44-53, 2 men and 3 women, median NIHSS score 8.4, range 4-17) due to a carotid web. Patients were all Caucasian excepting for one. No severe comorbidities were detected. Preoperatively thrombectomy of middle cerebral artery was needed in three cases. Surgical treatment consisted in web resection and carotid end-to-end anastomosis (3 cases) or web excision and patch angioplasty (2 cases). In-hospital NIHSS score improved postoperatively of more than 2 points (median 3.6, range 2-7) in all cases. No major complications were detected. One patient experienced a local hematoma treated conservatively. At a mean follow-up of 18 months (range 1-35 months), no recurrent stroke nor myocardial infarction nor death were remarked. CONCLUSIONS: In young patients presenting with ischemic stroke without neither conventional stroke risk factors nor other possible sources of embolism, a carotid web should be suspected. Surgery guarantees excellent results in prevention of recurrences.

Carotid webs management in symptomatic patients.

BACKGROUND: Atypical fibromuscular dysplasia (AFMD), also known as carotid web, is a rare underdiagnosed shelf-like fibrous tissue arising from the posterior carotid artery bulb that is a cause of cryptogenic stroke of the anterior cerebral vascularization. Despite the recurrence and severity of strokes caused by embolization associated with AFMD, there are no recommendations on the best strategy to manage single and bilateral lesions, which have unsatisfactory outcomes when treated with medical treatment exclusively. METHODS: From January 2016 to April 2019, 365 patients were operated on for a carotid stenosis in our institution. This cohort included 11 patients (3%), with a median age of 41 years (range, 39-51 years), referred by a stroke unit, treated for a symptomatic (10 strokes and 1 recurrent transient ischemic attack) AFMD lesion. Preoperative workup revealed a contralateral similar lesion in 45% of patients (5/11), which all also underwent surgery during a subsequent hospitalization. The diagnosis was confirmed by histologic examination when open surgery was performed. The 30-day and 1-year outcomes were retrospectively reviewed. RESULTS: Of the 16 AFMD lesions operated, 13 were treated by open surgery (2 by classic endarterectomy and 11 by internal carotid resection-anastomosis) and 3 by carotid artery stenting, respectively, with a mean delay of 85.5 days and 20.5 days after the latest stroke. There was one complication after stenting (external iliac rupture) that was treated by a covered stent, and no perioperative complications after open surgery. The follow-ups at 30 days and 1 year were uneventful for all patients, without any deaths or stroke recurrences. CONCLUSIONS: Symptomatic AFMD is a rare cause of cryptogenic stroke. Bilateral lesions are frequent. Early intervention is associated with favorable perioperative and 1-year outcomes. Open surgery is the first-line therapeutic option in this young patient population.

Carotid Web Misdiagnosis.

BACKGROUND: A carotid web (CW), an atypical fibromuscular dysplasia, is rare and may cause ischemic stroke. It is challenging to recognize a CW promptly and treat it accordingly. We report a case of an initially misdiagnosed CW. CASE DESCRIPTION: A 48-year-old man with recurrent cerebral infarction was transferred to our hospital for bypass surgery. Imaging performed at our facility showed multiple old infarct lesions and cerebral tissue hypoperfusion in the occluded left middle cerebral artery territory. Bypass surgery was performed without perioperative complications. While searching for the cause of his stroke, we found a thin intraluminal filling defect along the posterior wall of the left carotid bulb just beyond the carotid bifurcation on sagittal maximal intensity projection images, axial thin-cut images, and volume rendering images. The defect was diagnosed as a CW. It was also detected on follow-up ultrasonography. Owing to our initial unawareness, we did not photograph the carotid bulb during preoperative digital subtraction angiography and postoperative computed tomography angiography. Although the patient was treated with bypass, the persistence of the factors underlying the CW may induce further thrombosis and subsequent occlusion of his ipsilateral anterior cerebral artery. CONCLUSIONS: Clinicians should be aware of CW as a potential cause of ischemic stroke. Head and neck computed tomography angiography is a reliable imaging method to detect CWs.

Endovascular Treatment of Concomitant Carotid Fibromuscular Dysplasia and Atherosclerotic Disease After Failed Open Surgical Treatment.

We describe a case of concomitant fibromuscular dysplasia (FMD) and atherosclerotic internal carotid artery (ICA) disease in a symptomatic patient. Sixty-eight-year-old female presented for evaluation of a transient ischemic attack. Imaging revealed severe proximal >80% ICA stenosis with severe FMD to mid and distal ICA. Planned hybrid approach with left carotid endarterectomy (CEA) and balloon angioplasty was aborted intraoperatively due to lack of back bleeding. Open gradual rigid dilation was then performed with resumption of back bleeding. Completion angiogram revealed widely patent flow through CEA patch with no residual defects. Patient awoke from operation neurologically intact. At 6-month follow-up, carotid duplex revealed severe recurrent ICA stenosis. Endovascular intervention was performed with balloon dilation to the proximal and mid-ICA with stenting of a moderate 60% ostial common carotid stenosis. She recovered well from procedure with 3-month carotid duplex showing widely patent common carotid stent and ICA with no areas of stenosis. Informed consent has been obtained from the patient for publication of the case report and accompanying images.

A Systematic Literature Review of Patients With Carotid Web and Acute Ischemic Stroke.

Background and Purpose- Carotid web (CW) is a rare form of focal fibromuscular dysplasia defined as an abnormal shelf-like projection of intimal fibrous tissue into the carotid bulb. It is theorized that CW leads to ischemic stroke secondary to blood flow stasis and subsequent embolization. The natural history and optimal management of CW are unclear. To address this knowledge gap, we performed a systematic literature review (SLR) of CW. Methods- Our librarians performed a SLR for CW and related terminology. Patient-level demographics, stroke risk factors, neuroimaging findings, stroke recurrence or stroke free-duration, and treatment modality were extracted. We used descriptive statistics to characterize our results. When specific patient-level metrics were not reported, the denominators for reporting percentage calculations were adjusted accordingly. Results- Our literature search produced 1150 articles. Thirty-seven articles including 158 patients (median age 46 years [range 16-85], 68% women, 76% symptomatic) met entry criteria and were included in our SLR. Of the symptomatic CW patients: 57% did not have stroke risk factors, 56% who received medical therapy had recurrent stroke (median 12 months, range 0-97), and 72% were ultimately treated with carotid revascularization (50% carotid stenting, 50% carotid endarterectomy). There were no periprocedural complications or recurrent strokes in carotid revascularization patients. Conclusions- CW leads to ischemic stroke in younger patients without conventional stroke risk factors. We found a high stroke recurrence rate in medically managed symptomatic CW patients, whereas carotid revascularization effectively prevented recurrent stroke. Our findings should be interpreted with caution because of risk of publication and reporting bias.

Surgical management of internal carotid artery aneurysm near the skull base.

Extracranial carotid artery aneurysms are rare. The most common location is the common carotid artery near the bifurcation. The mid to distal internal carotid artery is the second most common location. We are reporting the case of a 64-year-old woman who was admitted to our department for management of an asymptomatic left internal carotid artery aneurysm. Physical examination revealed a pulsatile mass, and imaging confirmed the aneurysm diagnosis. Computed tomography angiography detailed a 28mm×3cm×6cm aneurysm of the left cervical internal carotid artery with tortuous outflow the aneurysm sac. Open repair was undertaken. Exposure with incision anterior to the sternocleidomastoid was performed although extended more superiorly than usual because of the distal aneurysm location. After carotid clamping, the aneurysm was resected and an end-to-end anastomosis with prosthesis was performed. After closure, the patient was extubated demonstrating baseline neurologic function. Histologic examination of the arterial wall confirmed the diagnosis of fibromuscular dysplasia.