Endovascular and medical therapy of May-Thurner syndrome: Case series and scoping literature review.

INTRODUCTION: May-Thurner syndrome has been recognized as a cause of chronic venous insufficiency and a trigger for venous thromboembolism. There is no consensus about the definition, diagnosis, and therapeutic approach. We are aiming to describe its characteristics and a scoping literature review. METHODS: A retrospective review of patients with May-Thurner syndrome from March 2010 to May 2018 and scoping literature review were made. RESULTS: Seven patients were identified. All patients were female with a median age of 36 (20-60) years. The median time from the first symptom to diagnosis was 3.41 (0.01-9) years. The primary clinical presentation was post-thrombotic syndrome (4 patients). Six patients had at least one risk factor for deep venous thrombosis. All patients underwent angioplasty with stent; patients with acute deep venous thrombosis, furthermore mechanic thrombectomy with or without catheter-directed thrombolysis were done. There were three complications (one patient, lymphedema, and two venous stent thrombosis). Scoping review results were descriptively summarized. CONCLUSION: May-Thurner syndrome has a varied spectrum of clinical presentation, and clinical awareness is paramount for diagnosis. Its principal complication is the post-thrombotic syndrome, which is associated with high morbidity. There is no consensus on the antithrombotic treatment approach.

[Cerebral embolism as an atypical presentation of May Thurner syndrome]. / Embolia cerebral como presentación atípica de síndrome de May Thurner.

May-Thurner syndrome is an anatomic abnormality that predisposes patients to increase risk of paradoxical embolism and stroke. It consists of chronic compression of the left common iliac vein by the overlying right common iliac artery and lumbar spine. This may lead to deep venous thrombosis and paradoxical embolism that could provoke cerebral ischemia in patients with a cardiac shunt from right-to-left. Embolic cerebral ischemic event is reported in a 30-year-old man after sexual intercourse. Further studies revea led suggestive findings of May-Thurner syndrome coupled with a patent foramen ovale and a factor V Leiden thrombophilia. He was placed on anticoagulation therapy and has not had any recurrent events.

El síndrome de May-Thurner es una anormalidad anatómica que consiste en la comprensión cronica de la vena iliaca común izquierda, entre la arteria ilíaca común derecha y la columna vertebral, que puede predisponer a la trombosis venosa profunda. Es una entidad rara, y es infrecuente que provoque una embolia paradojal cerebral. Se presenta el caso de una isquemia cerebral de origen embólico, en un varón de 30 años, luego de actividad sexual. Los estudios realizados revelaron síndrome de May-Thurner asociado con un foramen oval permeable y una trombofilia por factor V de Leiden. Fue tratado con anticoagulantes orales y no ha presentado recurrencias.

Embolia cerebral como presentación atípica de síndrome de May Thurner

El síndrome de May-Thurner es una anormalidad anatómica que consiste en la comprensión cronica de la vena iliaca común izquierda, entre la arteria ilíaca común derecha y la columna vertebral, que puede predisponer a la trombosis venosa profunda. Es una entidad rara, y es infrecuente que provoque una embolia paradojal cerebral. Se presenta el caso de una isquemia cerebral de origen embólico, en un varón de 30 años, luego de actividad sexual. Los estudios realizados revelaron síndrome de May-Thurner asociado con un foramen oval permeable y una trombofilia por factor V de Leiden. Fue tratado con anticoagulantes orales y no ha presentado recurrencias.

May-Thurner syndrome is an anatomic abnormality that predisposes patients to increase risk of paradoxical embolism and stroke. It consists of chronic compression of the left common iliac vein by the overlying right common iliac artery and lumbar spine. This may lead to deep venous thrombosis and paradoxical embolism that could provoke cerebral ischemia in patients with a cardiac shunt from right-to-left. Embolic cerebral ischemic event is reported in a 30-year-old man after sexual intercourse. Further studies revea led suggestive findings of May-Thurner syndrome coupled with a patent foramen ovale and a factor V Leiden thrombophilia. He was placed on anticoagulation therapy and has not had any recurrent events.

Systematic review of May-Thurner syndrome with emphasis on gender differences.

OBJECTIVE: May-Thurner syndrome (MTS) is increasingly recognized as a frequent source of leg swelling and a precipitating factor for venous thromboembolism. This paper is a systematic review of the English literature on MTS with an analysis focusing on gender differences in presentation and treatment. METHODS: A systematic review of the English literature between April 1967 and December 2014 was performed using the following terms: "May-Thurner syndrome," "Cockett syndrome," and "iliac vein compression syndrome." After review, there were 174 articles in the analysis. We first analyzed all presented cases, followed by a gender comparison if case reports and case series had detailed description. Asymptomatic patients with just anatomic compression without symptoms were excluded. Statistical differences between data sets were assessed using χ2 test and Student t-test. RESULTS: There were 1569 patients with MTS after exclusion of articles based on our criteria. The female to male ratio was 2:1 (976 [67.1%] vs 480 [32.9%]). Women presented at a younger age compared with men (38.7 ± 14.0 years vs 46.2 ± 16.9 years; P = .02). Gender comparison at presentation, which was available for 254 patients, showed that men had significantly more reported leg swelling (92.7% vs 80.8%; P = .037) and more leg pain (88% vs 74.3%; P = .045) compared with women. There was no difference in the reported proportion of patients presenting with deep venous thrombosis between the two groups (88.9% vs 81.7%; P = .14). However, women were significantly more likely to have a pulmonary embolus on presentation compared with men (9.9% vs 1.6%; P = .035). Treatment modalities included endovascular interventions without thrombolysis (53%) or with thrombolysis (33.2%), open surgery (6.8%), and medical management (7%). Endovascular treatment was more common than surgical or medical treatment (P < .001). Because of lack of granularity in the data, it was not possible to distinguish treatment methods between female and male patients. There was no statistically significant difference in complication rate between men and women based on the articles that provided that information (P = .34). However, open procedures had significantly higher complications compared with endovascular interventions (P = .021). CONCLUSIONS: Based on the reported literature, MTS is more common in women and is at least twice as frequent in women as in men. Men tend to have more pain and swelling in the legs, whereas women tend to be younger and more likely to have a pulmonary embolus on presentation. MTS and iliac vein compression are sometimes used interchangeably in an inaccurate manner.

Presentación atípica de síndrome infrecuente: absceso como inicio de síndrome de May-Thurner / Atypical presentation in an uncommon syndrome: abscess as debut of May-Thurner syndrome

Resumen Objetivo Presentar un caso de inicio atípico de síndrome de May-Thurner, enfermedad vascular poco frecuente. Caso clínico Se reporta el caso de una paciente de 23 años con absceso de pie izquierdo y extenso edema de dicha extremidad. El dímero D y la ecotomografía doppler color venosa descartan evento trombótico agudo. El estudio complementario con angioTC objetiva compresión del origen de la vena ilíaca común izquierda, presencia de venas colaterales y diferente grado de insuficiencia venosa en dicha extremidad, hallazgos compatibles con el síndrome de May-Thurner. Conclusión El síndrome de May-Thurner representa una causa inusual y de presentación variable en enfermedad venosa de extremidad inferior izquierda en mujeres jóvenes.

ABSTRACT Aim To present a case of an atypical debut of May-Thurner syndrome, uncommon vascular disease. Case report We report a case of a 23 year old female with an abscess of the left foot and extensive edema of the left lower limb. D-Dimmer test and Venous Doppler ultrasound discards an acute trombotic event. Further studies with CT angiogram concludes the compression in the origin of the left common iliac vein compatible with May-Thurner syndrome. Conclusion May-Thurner syndrome represent an uncommon and variable cause of venous disease of the left lower limb in young female patients.

Pyoderma gangrenosum associated with left iliac vein compression syndrome: presentation of difficult diagnosis.

Pyoderma gangrenosum is a rare neutrophilic dermatosis of unknown etiology, of which the most frequent clinical manifestations are ulcers. The diagnosis difficulty is, among other things, to rule out other causes of ulcers, since it is considered a diagnosis of exclusion. Skin ulcerations may also occur in the iliac vein compression syndrome, which, like pyoderma gangrenosum, mainly affects young women. Because they have such similar characteristics, the presence of vascular disease may hinder the diagnosis of concurrent pyoderma gangrenosum. Because of the clinical relevance of ulcerated lesions and scars, the early diagnosis and treatment of this condition is considered extremely important. We report a case in which the two diseases were associated, hampering the diagnosis of pyoderma gangrenosum.

[May Thurner syndrome with ischemic cerebrovascular accident and permeable oval foramen]. / Síndrome de May Thurner con accidente cerebrovascular isquémico y foramen oval permeable.

The association of cerebral ischemic attack with patent foramen ovale has not been extensively studied, and frequently the site of origin of embolism is not detected despite routine studies. We present the case of a young patient with ischemic stroke and permeable oval foramen in the context of May Thurner syndrome. The May Thurner syndrome is an entity scarcely studied in the medical literature and it has also been infrequently related to ischemic vascular cerebral accident, but in patients with permeable oval foramen without evidence of the emboligen source, it is interesting to rule it out as a cause of paradoxical embolism.

Síndrome de May Thurner con accidente cerebrovascular isquémico y foramen oval permeable / May Thurner syndrome with ischemic cerebrovascular accident and permeable oval foramen

La asociación de accidente cerebral isquémico con foramen oval permeable, no ha sido extensamente estudiada, y frecuentemente el sitio de origen de la embolia no se detecta a pesar de los estudios de rutina. Se presenta el caso de un paciente joven con accidente cerebral vascular isquémico y foramen oval permeable en el contexto de síndrome de May Thurner. El síndrome de May Thurner es una entidad poco estudiada en la literatura médica y además se lo ha relacionado poco con accidente cerebral vascular isquémico, pero en pacientes con foramen oval permeable sin evidencia de la fuente embolígena, es interesante descartarlo como causa de embolia paradojal.

The association of cerebral ischemic attack with patent foramen ovale has not been extensively studied, and frequently the site of origin of embolism is not detected despite routine studies. We present the case of a young patient with ischemic stroke and permeable oval foramen in the context of May Thurner syndrome. The May Thurner syndrome is an entity scarcely studied in the medical literature and it has also been infrequently related to ischemic vascular cerebral accident, but in patients with permeable oval foramen without evidence of the emboligen source, it is interesting to rule it out as a cause of paradoxical embolism.

New Vein Compression Entities in Patients with Unexplained Leg Swelling.

BACKGROUND: This retrospective study identifies often overlooked anatomical sites for nonthrombotic venous outflow obstruction (NTVO) in patients with unexplained lower extremity edema and pain. METHODS: We reviewed the charts of 75 consecutive patients experiencing symptoms of unexplained lower extremity edema with pain that were unexplained by ultrasound, computed tomography angiography (CTA), and magnetic resonance imaging (MRI), who subsequently underwent venography in an outpatient medical office from 2010 to 2014. We categorized venograms based on the presence or absence of NTVO lesions and calculated prevalence of each at specific sites. The patients with NTVO lesions showing >50% stenosis on venography were then treated with angioplasty and/or stenting. After intervention, we documented subjective levels of pain and edema. RESULTS: Of the 75 venograms reviewed, physicians classified 52 as normal and 23 as showing evidence of compression, including 9 with May-Thurner syndrome and 14 with anatomical compressions at previously underreported sites. These 14 compression sites occurred at the following: iliofemoral vein at the inguinal ligament region (n = 7, 50%), external iliac vein at the iliac artery bifurcation (n = 1, 7.1%), both inguinal ligament region and iliac artery bifurcation (n = 4, 28.6%), and popliteal vein at the popliteal fossa (n = 2, 14.3%). Nine of the 14 patients (64.3%) reported total or near total resolution of lower extremity pain and edema at follow-up between 1 and 7 months (mean = 5.3 ± 2 months, median = 6 months) after balloon angioplasty and/or stent. Five with failed primary interventions underwent subsequent stenting and/or angioplasty and reported total or near total resolution of pain and clinical resolution of edema. CONCLUSIONS: This study provides evidence to broaden the disease profile of venous compression syndromes to other sites such as the hypogastric artery, inguinal ligament, and popliteal fossa. The results support previous research that suggests increased incidence of NTVO exists among patients with unexplained lower extremity edema and pain. In an effort to encourage further exploration, we developed a diagnostic algorithm to support a critical and systematic review of patients with lower extremity edema and pain that may go unexplained using traditional diagnostic measures, including ultrasound, CTA, and MRI alone.

Pyoderma gangrenosum associated with left iliac vein compression syndrome: presentation of difficult diagnosis

Abstract: Pyoderma gangrenosum is a rare neutrophilic dermatosis of unknown etiology, of which the most frequent clinical manifestations are ulcers. The diagnosis difficulty is, among other things, to rule out other causes of ulcers, since it is considered a diagnosis of exclusion. Skin ulcerations may also occur in the iliac vein compression syndrome, which, like pyoderma gangrenosum, mainly affects young women. Because they have such similar characteristics, the presence of vascular disease may hinder the diagnosis of concurrent pyoderma gangrenosum. Because of the clinical relevance of ulcerated lesions and scars, the early diagnosis and treatment of this condition is considered extremely important. We report a case in which the two diseases were associated, hampering the diagnosis of pyoderma gangrenosum.

[May-Thurner syndrome as a cause of neurovascular compression of the pudendal nerve]. / Síndrome de May-Thurner como causa de compresión neurovascular del nervio pudendo.

May-Thurner syndrome is a clinical condition that results from narrowing of the left common iliac vein lumen due to pressure from the right common iliac artery as it crosses anterior to it. We describe an atypical case of May-Thurner syndrome in a 23-year-old woman that presented only continuous pain in pudendal zone without vascular symptoms. The Doppler ultrasound, nuclear magnetic resonance and others complementary analyses show the presence of a pelvic venous congestion syndrome and we hypothesized that this condition produced a neuropathic compression of the pudendal nerve in Alcock's canal. Patient was treated with the technique of pudendal nerve blockade by trans-gluteal via. An important reduction in pain of pudendal zone was showed.