1/13. A 10-year-old boy with marfan syndrome exhibiting cerebrovascular abnormalities.A young male with marfan syndrome, diagnosed at the age of 10 years, presented with conspicuous elongation and tortuosity of the internal carotid, middle cerebral, vertebral and basilar arteries on cranial magnetic resonance and computed tomography angiography. There is a little mention of cerebral blood vessel examinations in the guidelines of the American Academy of pediatrics for marfan syndrome. Guidelines may be provided for the evaluation of cerebrovascular system for the patients with marfan syndrome who have family history of marfan syndrome as well as a family history of death from subarachnoid hemorrhage.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/13. Emergency management of spontaneous coronary artery dissection.Six cases of spontaneous coronary arteries dissection are reported. In one patient, triple vessel spontaneous coronary artery dissection was identified. Another patient presented spontaneous left main coronary artery dissection. In one case we found the spontaneous dissection of the left anterior descending artery associated with distal aortic arch dissection. These conditions are very rare and may present a surgical dilemma. Causative factors and underlying pathology are clarified. Prompt diagnosis and surgical intervention is safe and effective. Early recognition of left main coronary artery dissection or three-vessel dissection is essential because urgent coronary artery bypass grafting may be life saving.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
3/13. Symptomatic enlarged cervical anterior epidural venous plexus in a patient with marfan syndrome.A 38-year-old man with marfan syndrome presented with headache and neck pain. MR imaging revealed a large enhancing mass in the cervical anterior epidural space. Cervical laminectomy with biopsy of the lesion revealed a large engorged anterior epidural venous plexus (AEVP). marfan syndrome may predispose the patient to enlargement of AEVP secondary to a vessel wall abnormality.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/13. Arch-first technique used with commercial T-graft to treat subacute type-A aortic dissection in patient with marfan syndrome.Staged repair of extensive thoracic aortic aneurysms puts certain patients at risk of rupture. We report the case of a patient with marfan syndrome who presented with subacute type-A aortic dissection and a large descending aortic aneurysm. We used the arch-first technique with a commercially available Dacron T-graft. A clamshell incision was used for exposure. A button of arch vessels was anastomosed to the T-graft. Antegrade cerebral perfusion was established through the side branch. The distal end of the graft was anastomosed to the descending aorta and the proximal end to a composite graft. The duration of cerebral ischemia was 30 minutes; antegrade cerebral perfusion lasted 52 minutes. The patient experienced no neurologic dysfunction and was discharged with no major deficit. This technique shortens brain-ischemia time and is a good option if the risk of rupture of the descending component of an extensive thoracic aortic aneurysm is high. To the best of our knowledge, this is the 1st reported case in which the arch-first technique has been used with a commercially available T-graft to treat subacute type-A aortic dissection in a patient with marfan syndrome.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/13. Cerebellar hematoma in a patient with marfan syndrome.BACKGROUND: marfan syndrome is a connective tissue disorder affecting many structures, including the skeleton, lungs, eyes, heart and blood vessels. It is an autosomal dominant inherited disorder due to a mutation of a gene encoding fibrillin-1, which affects connective tissue. Few case reports have associated marfan syndrome with vascular malformations of the brain and spinal cord. In this regard, association with intracranial aneurysm has been vaguely proposed. CASE REPORT: We report here a patient with marfan syndrome who was admitted because of a sudden loss of consciousness. The patient underwent computed tomography (CT) examination, which disclosed a right intracerebellar hematoma. Cerebral angiogram did not demonstrate aneurysm or arteriovenous malformation (AVM), or evidence of any other vascular lesions or neoplasms in the posterior fossa. Conservative treatment was undertaken. The clinical course was uneventful and after 6 weeks the patient was discharged free of symptoms. CONCLUSIONS: Although patients with marfan syndrome are at high risk of vascular abnormalities, a clear association with cerebral aneurysm has not yet been established. Our experience and the contrasting reports available in the medical literature strongly warrant further studies in order to better clarify this controversial association.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
6/13. Cardiac transplantation in neonatal marfan syndrome -- a life-saving approach.marfan syndrome is a connective tissue disease with typical clinical signs and cardiac involvement. Its appearance in the neonatal period has a bad prognosis due to incompetence of all cardiac valves with subsequent congestive heart failure. Conservative management usually fails, the children die during their first year of life. We report on a girl with neonatal marfan syndrome who suffered from regurgitance of all cardiac valves, enlarged ventricles, and dilated great arteries. She was NYHA class IV. At the age of six months she underwent heart transplantation. To prevent aneurysm formation and dissection of the great vessels, the whole aortic arch and pulmonary trunk were replaced as well.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
7/13. Prenatal marfan syndrome: report of one case and review of the literature.OBJECTIVES: Our objective was to describe the features of prenatal marfan syndrome. methods: Doppler fetal echocardiograms were performed. The morphology and rhythm of the fetal heart were examined sequentially. RESULTS: The case was referred because of cardiomegaly and dilated great vessels. Sequential Doppler echocardiographic evaluation led to the diagnosis of prenatal marfan syndrome. The main features are cardiomegaly, dysplastic atrioventricular valves with tricuspid regurgitation and dilated great vessels, which can be aneurysmal at their origin. The fetus died in utero at 39 weeks of gestation because of cardiac failure. Pathological study confirmed the Marfan habitus and complications. Molecular genetic study showed a de novo point mutation in exon 26 of the FBN1 gene. CONCLUSION: We report a case of prenatal marfan syndrome diagnosed by sequential evaluation of the cardiac signs, which are essential for prenatal diagnosis. The prognosis seems as poor as the neonatal one. The prenatal diagnosis is essential for adequate counselling.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
8/13. Endovascular graft for late iatrogenic vascular complication after anterior spinal instrumentation: a case report.STUDY DESIGN: Case report. OBJECTIVE: To describe an endovascular treatment option for (late) vascular complications after anterior spinal instrumentation. SUMMARY OF BACKGROUND DATA: Severe progressive scoliosis is a well-known feature in Marfan's disease. Although overall complication rate after surgical correction of the spinal deformity is rather high, late iatrogenic complications are less frequently identified. methods: A 40-year-old woman with Marfan's disease reported to our outpatient clinic with dyspnoe d'effort, 20 years after anterior spinal instrumentation of a thoracolumbar scoliosis. Routine screening identified a saccular aneurysm of the descending thoracic aorta. A contrast-enhanced CT scan revealed that the most proximal screw of the construct had perforated the vessel wall and caused a false aneurysm. An endovascular approach to the problem was chosen; by a transfemoral approach, an AneuRx endovascular graft was successfully implanted. RESULTS: The patient had an uneventful postoperative course and was discharged within 5 days. At 5-year follow-up, the patient still has no clinical complaints, nor radiographic leakage nor recurrence of the false aneurysm at contrast-enhanced CT screening. CONCLUSION: Use of an endovascular graft is an adequate alternative for treatment of late vascular complications after anterior spinal instrumentation.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
9/13. Internal carotid artery aneurysm and Marfan's syndrome.Aneurysms of the extracranial carotid artery are most commonly caused by atherosclerosis or trauma but may also have unusual causes, such as Marfan's syndrome. Although aneurysmal changes in the extracranial carotid vessels usually are due to extension of aortic dissection into the carotid system, isolated aneurysms may occasionally complicate Marfan's syndrome. The authors report a case of Marfan's syndrome in which the patient, a 45-year-old woman, presented with an asymptomatic mass in the right side of the neck at the level of the carotid bifurcation. An isolated internal carotid artery aneurysm was identified. The aneurysm was resected using an interposition vein graft over an outlying shunt. Histologic examination confirmed the typical cystic medial necrosis with loss of elastic fibres and an increase of mucoid material in the media. The patient's recovery was smooth and at 2-year follow-up there were no signs of recurrent aneurysm formation.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
10/13. Aortic 125I-albumin transport in patients with Marfan's syndrome and annuloaortic ectasia.The morphologic, biochemical, and mechanical abnormalities of connective tissue fibrous proteins in Marfan's syndrome have been well studied, and their role in cardiovascular complications is well accepted. Less is known, however, about the state of the amorphous components of the aortic connective tissue. In the course of a study of transmural transport in blood vessels, we have had the opportunity to study dystrophic aorta from two young men who survived elective surgery; both with aortic insufficiency (AI) histologically compatible with Marfan's syndrome. One had recurrent chronic dissecting aneurysm (RCDA) as well. The aorta of the first (but not the second) was histologically compatible with Marfan's syndrome. Fresh specimens of intact ascending aorta were incubated in Krebs solution, pH 7.4, containing 125I-labelled bovine serum albumin for 2 h at 37 degrees C. The samples were then frozen, and serially sectioned in the plane of the lumenal surface. The radioactivity of the 20-micron thick sections was then determined, and expressed as a tissue/labelled solution concentration ratio. Transmural profiles of these ratios revealed no difference between the aorta of the RCDA patient with non-specific aortic dystrophy, and that of a 70-year-old man undergoing aortocoronary bypass. However, in the patient with aortic histology compatible with Marfan's syndrome, the average media concentration ratio was 5-fold less (4% vs. 20%).- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
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