1/21. Combined heart transplantation and resection of dissecting aneurysm of ascending aorta and aortic arch: a case report.A 21-year-old male patient had suffered from palpitation and exertional dyspnea since October, 1997. He was admitted to our hospital, and a series of examinations were performed. Chest computed tomography (CT) revealed marked dilatation of the ascending aorta (about 7.5 cm at the proximal portion) and aortic annulus, an intimal flap in the ascending aorta and aortic arch was also noted. cardiac catheterization revealed the pulmonary capillary wedge pressure was 33 mmHg, pulmonary artery pressure was 47/38 mmHg with a mean of 35.4. The cardiac index was 1.01 l/min/m2. Poor left ventricular contractility was shown by a left ventricular ejection fraction (LVEF) of 13.8% and a right ventricular ejection fraction (RVEF) of 5.13% by a radionuclide angiogram (rna) study. Under the diagnosis of dilated cardiomyopathy and dissecting aortic aneurysm of the ascending aorta and aortic arch, he was put on a waiting list for heart transplantation. On November 11, 1997 he received heart transplantation. Resection of the dissecting aneurysm of the ascending aorta and the aortic arch and replacement with a 26 mm Vascutek graft were performed first under deep hypothermia and retrograde cerebral perfusion. Then while he was rewarming up, heart implantation was performed. He was discharged 30 days after surgery and has been doing well since then. As far as we know, no literature regarding combined heart transplantation and resection of a dissecting aneurysm of the ascending aorta and aortic arch has been reported.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
2/21. Hypotony caused by scleral buckle erosion in marfan syndrome.PURPOSE: To describe hypotony caused by erosion of the conjunctiva and sclera by a silicone scleral buckle. methods: Interventional case report. A 33-year-old man with marfan syndrome presented with hypotony maculopathy and a collapsed globe 17 months after repair of retinal detachment with a silicone sponge and silicone encircling band. RESULTS: Examination in the operating room revealed extrusion of the buckle through the conjunctiva and full-thickness scleral erosion. The silicone buckle was removed, and the scleral defect was closed with interrupted 8-0 nylon sutures. Postoperative glaucoma was treated with cyclophotocoagulation. Eight months after scleral repair, visual acuity was RE: 20/40, intraocular pressure was 10 mm Hg, and the retina was attached. CONCLUSION: Full-thickness scleral erosion secondary to a silicone exoplant causing hypotony is a rare long-term complication in patients with thin sclera.- - - - - - - - - - ranking = 0.5keywords = pressure (Clic here for more details about this article) |
3/21. epidemiology and outcome of postural headache management in spontaneous intracranial hypotension.BACKGROUND AND OBJECTIVES: Spontaneous intracranial hypotension is a postural headache syndrome unrelated to dural puncture. Due to the apparent failure of epidural blood patch to relieve headache in spontaneous intracranial hypotension, we investigated the epidemiologic features and treatment outcomes of this condition. methods: The clinical findings and management of 22 cases (21 published 1 reported) of spontaneous intracranial hypotension were analyzed retrospectively. The study population was stratified by age and sex; continuous variables were compared for differences by t-tests; categorical variables were compared by Fisher exact tests. Significant differences were identified by P values of.05 or less. RESULTS: The mean age of the study population was 43 /- 16 years, with a female:male ratio of 3.4:1.0. Females with spontaneous intracranial hypotension were younger (P =.050) than males. Men presented with tinnitus (P =.021) and visual field defects (P =.009) more often than women. Meningeal enhancement on contrast magnetic resonance imaging was the most consistent radiographic finding. Radionuclide cisternography showed thoracolumbar dural leaks in 7 of 9 patients. cerebrospinal fluid opening pressure was low in all patients (33.13 /- 31.02 mm H(2)O). Epidural blood patch was performed in 8 patients, repeated in 3 patients, failed in 3 patients, and offered only transient improvement in 5 patients. CONCLUSIONS: Spontaneous intracranial hypotension was more common in women than men, was not uniformly responsive to epidural blood patch, and had significant comorbidities. The management of postural headache in spontaneous intracranial hypotension by other techniques to restore cerebrospinal fluid dynamics and prevent its leakage should be investigated.- - - - - - - - - - ranking = 0.5keywords = pressure (Clic here for more details about this article) |
4/21. Functional pulmonary atresia in neonatal Marfan's syndrome: successful treatment with inhaled nitric oxide.Functional pulmonary atresia is characterized by a structurally normal pulmonary valve not opening during right ventricular ejection. We report this rare condition in a premature newborn of a twin pregnancy, in which fetal echocardiography findings were consistent with critical pulmonary stenosis. After birth, features of neonatal Marfan's syndrome were noted. echocardiography showed a morphologically normal but immobile pulmonary valve with continuous regurgitation. Right ventricular pressure was subsystemic. In this case, initial treatment with nitric oxide, followed by pharmacological duct closure, was successful. Differentiating between anatomic and functional pulmonary valve atresia may be difficult. The echocardiographic criteria are discussed.- - - - - - - - - - ranking = 0.5keywords = pressure (Clic here for more details about this article) |
5/21. Unexpected tracheomalacia in marfan syndrome during general anesthesia for correction of scoliosis.IMPLICATIONS:We experienced an unexpected tracheomalacia exhibiting CO2 retention and an increased peak inspiratory pressure after beginning an operation; it was confirmed with fiberoptic bronchoscopy. If there are no problems in the circuit or tube when the patient shows CO2 retention and increases in peak inspiratory pressure, the fiberoptic bronchoscope is helpful- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
6/21. Aortic root diameter and nasal intermittent positive airway pressure treatment in Marfan's syndrome.We report on a patient with Marfan's syndrome, with coexistent obstructive sleep hypopnea (OSH) and restrictive lung disease, complicated by respiratory insufficiency, who was successfully treated with nasal intermittent positive airway pressure (NIPPV) and oxygen. NIPPV therapy turned out to be effective on arterial gas exchange and well tolerated. Moreover, progressive dilatation of the aortic root was attenuated during NIPPV, but could, however, not be reversed. We hypothetized that (at least partially) a decrease of nocturnal intrathoracic pressures could explain this.- - - - - - - - - - ranking = 3keywords = pressure (Clic here for more details about this article) |
7/21. Bilateral popliteal aneurysms complicating adult polycystic kidney disease in a patient with a marfanoid habitus.A man born in 1944 presented with an episode of macroscopic haematuria during a urinary tract infection in 1988. He was unusually tall at 2 metres. An intravenous pyelogram and an abdominal ultrasound disclosed the presence of bilaterally enlarged polycystic kidneys and a polycystic liver. There was a family history of renal disease. plasma creatinine (180 micro mol/l) and blood pressure (150/100 mm Hg) were both raised. Despite good blood pressure control his renal function declined progressively and he started renal dialysis treatment in 1995. He received a renal allograft in 1996. In 1994 he had noticed a painful swelling behind his left knee. Computed tomography with contrast showed a large popliteal aneurysm. This was replaced with a vein graft. The right popliteal artery showed milder changes, and this was repaired in 1999. Popliteal aneurysms develop most often in older vasculopaths with multiple risk factors; connective tissue disorders have rarely been associated with their presence in younger patients. Polycystic kidney disease has been associated with several aneurysms, most notably cerebral, but not popliteal. The patient's marfanoid habitus also may have played a part. This case emphasises the mixed aetiology of popliteal aneurysms.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
8/21. Severe spontaneous intracranial hypotension and marfan syndrome in an adolescent.We describe a 14-year-old girl with marfan syndrome who presented with severe postural headache. Dural ectasia was demonstrated radiologically. cerebrospinal fluid (CSF) pressure was immeasurable on formal measurement. Radionucleide cisternography failed to demonstrate a CSF leak. We consider that the underlying fibrillinopathy in marfan syndrome rendered the dura sufficiently permeable to CSF leakage to cause the low CSF pressure headache. The patient was treated successfully with epidural autologous blood patch.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
9/21. Orthostatic blood pressure control in Marfan's syndrome.A 33-year old male patient, with Marfan's syndrome, reported symptoms of orthostatic intolerance and fatigue as a longstanding problem. Orthostatic cardiovascular examination showed poor orthostatic tolerance, with a rise in heart rate and a fall in arterial blood pressure and cerebral blood flow velocity. Self-discovered physical counterpressure manoeuvers improved symptoms, related to a substantial increase in arterial pressure and cerebral perfusion. When orthostatic complaint are reported by patients with Marfan's syndrome, physical counterpressure manoeuvers should be advised to reduce symptoms of postural hypotension.- - - - - - - - - - ranking = 4keywords = pressure (Clic here for more details about this article) |
10/21. Asymmetric pigmentary glaucoma in a patient with Marfan's syndrome.BACKGROUND: No relationship between Marfan's syndrome and pigmentary glaucoma has previously been described in the ophthalmic literature. We describe the case of a patient with Marfan's syndrome who had bilateral pigment dispersion syndrome and asymmetrical glaucoma. methods: A 34-year-old man with Marfan's syndrome developed pigment dispersion bilaterally. In the right eye, elevated intra-ocular pressure was associated with marked glaucomatous excavation of the right disc and corresponding visual field loss. A localised zonular dehiscence was present at the 6 o'clock position in the right eye. RESULTS: Ultrasound biomicroscopy showed marked iris concavity in the right eye, increased iridolenticular contact and superior subluxation of the right lens, resulting in increased dispersion of pigment unilaterally. This explains the asymmetric pattern of the glaucomatous damage. CONCLUSIONS: Asymmetrical progression of pigmentary glaucoma is uncommon and should prompt a search for some other mechanical factor, which might increase the iridozonular contact on one side or reduce it on the other. The association between the two syndromes in this patient was most likely mechanical due to reduced fibrillin expression throughout the eye and particularly in the iris and the lens zonules, resulting in loss of iris tensile strength and marked iris concavity as well as zonular weakness and partial lens subluxation.- - - - - - - - - - ranking = 0.5keywords = pressure (Clic here for more details about this article) |
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