Chromatic pupillometry in patients with retinitis pigmentosa.

OBJECTIVE:: To evaluate the chromatic pupillary response as a means of assessing outer and inner retinal function in patients with retinitis pigmentosa (RP). DESIGN:: Evaluation of diagnostic technology. PARTICIPANTS:: Thirty-two patients with RP and visual loss and 43 normal subjects. METHODS:: Patients were tested with a chromatic pupillometer using red and blue lights (1, 10, and 100 cd/m(2)), and their pupil responses were compared with those from 43 normal subjects (reported previously). Visual field and electroretinography (ERG) results were examined and compared with the pupil responses. MAIN OUTCOME MEASURES:: The percent pupil contraction of the transient response to a low-intensity (1 cd/m(2)) blue light and high-intensity (100 cd/m(2)) red light and the sustained response to a high-intensity blue light was calculated for 1 eye of each subject. RESULTS:: The pupil responses to red and blue light at all intensities were recordable in all patients except 1, whose pupil responded only to bright blue light. There was a significant difference of the pupil response between patients with RP and normal subjects in testing conditions that emphasized rod (1 cd/m(2) blue light) or cone (100 cd/m(2) red light) contribution (P<0.001). Patients with a non-recordable scotopic ERG showed significantly reduced pupil responses (P<0.001) to low-intensity blue light (1 cd/m(2)). Patients with a non-recordable or abnormal photopic ERG showed significantly reduced pupil responses (P<0.05) to high-intensity red light (100 cd/m(2)). Patients with a nonrecordable ERG had the most visual field loss and reduced pupil responses. Unexpectedly, patients with RP showed a slower re-dilation of the pupil after termination of bright blue light compared with red light, a pattern not observed in normal subjects. CONCLUSIONS:: Pupil responses to red and blue light stimuli weighted to favor cone or rod input are significantly reduced in patients with RP but are still recordable in patients having a non-recordable ERG. In addition, outer photoreceptor disease appears to unmask a post-illumination pupillary constriction to bright blue light, most likely mediated by intrinsic activation of melanopsin ganglion cells. Chromatic pupillometry provides a novel, noninvasive method for following retinal functional status, particularly in patients with severe RP and non-recordable ERG. FINANCIAL DISCLOSURE(S):: Proprietary or commercial disclosure may be found after the references.

Suprapineal recess: an alternate site for third ventriculostomy? Case report.

This 30-year-old woman presented with clinical symptoms and signs of intracranial hypertension and Parinaud syndrome secondary to ventriculoperitoneal shunt dysfunction. Magnetic resonance (MR) imaging revealed gross triventricular hydrocephalus with a large suprapineal recess due to aqueductal stenosis. Using an endoscopic approach, a ventriculostomy was performed within the floor of the dilated suprapineal recess. Following this procedure the patient experienced alleviation of all her neurological symptoms and signs. Postoperative MR imaging and cerebrospinal fluid flow studies demonstrated a functioning ventriculostomy. The anatomy of the suprapineal recess and its suitability for endoscopic ventriculostomy are discussed.

Sténose de l'artère rénale: indications pour une revascularisation [Atherosclerotic renal stenosis: indications for revascularisation]

La sténose de l'artère rénale (SAR) est souvent associée à une maladie athéromateuse diffuse et, en conséquence, à une morbidité et une mortalité cardiovasculaires accrues. Le nombre de revascularisations de l'artère rénale a considérablement augmenté ces dernières années. Mais les succès rapportés par cette procédure, par rapport à un traitement médical seul, semblent modestes tant sur le contrôle de la pression artérielle que sur la progression de l'insuffisance rénale. La mise en évidence d'une SAR ne représente pas systématiquement une indication à une revascularisation. Plusieurs critères doivent être pris en compte, dont la localisation de la sténose, son retentissement hémodynamique, la fonction rénale, la sévérité de l'hypertension artérielle et la facilité avec laquelle le traitement antihypertenseur parvient à normaliser la pression artérielle. Atherosclerotic renal artery stenosis is often associated with diffuse atherosclerotic disease and consequently an increased cardiovascular morbidity and mortality. Despite evidence of only moderate clinical benefit in comparison with medical treatment to control the blood pressure and to prevent renal failure, renal endovascular revascularisation has become more and more popular. The decision to treat an atherosclerotic renal stenosis by revascularisation should be taken only after a close examination of the hemodynamic impact of the stenosis, the renal function, the severity of hypertension and the quality of blood pressure control achieved by the medical treatment

Hepato-atrial anastomosis, the "other Senning operation" for treatment of Budd-Chiari syndrome.

A now 36-year-old woman developed a suprahepatic inferior vena cava stenosis, 9 years after liver transplantation for extensive liver echinococcosis. The lesion was treated by percutaneous angioplasty and stenting. Five years later, recurrence of echinococosis with intrastent stenosis together with clinical symptoms, prompted surgical treatment. Hepato-atrial anastomosis was performed under cardiopulmonary bypass with good result.

Cardiovascular influences of alpha1b-adrenergic receptor defect in mice.

BACKGROUND: The alpha1-adrenergic receptors (alpha1-ARs) play a key role in cardiovascular homeostasis. However, the functional role of alpha1-AR subtypes in vivo is still unclear. The aim of this study was to evaluate the cardiovascular influences of alpha1b-AR. METHODS AND RESULTS: In transgenic mice lacking alpha1-AR (KO) and their wild-type controls (WT), we evaluated blood pressure profile and cardiovascular remodeling induced by the chronic administration (18 days via osmotic pumps) of norepinephrine, angiotensin II, and subpressor doses of phenylephrine. Our results indicate that norepinephrine induced an increase in blood pressure levels only in WT mice. In contrast, the hypertensive state induced by angiotensin II was comparable between WT and KO mice. Phenylephrine did not modify blood pressure levels in either WT or KO mice. The cardiac hypertrophy and eutrophic vascular remodeling evoked by norepinephrine was observed only in WT mice, and this effect was independent of the hypertensive state because it was similar to that observed during subpressor phenylephrine infusion. Finally, the cardiac hypertrophy induced by thoracic aortic constriction was comparable between WT and KO mice. CONCLUSIONS: Our data demonstrate that the lack of alpha1b-AR protects from the chronic increase of arterial blood pressure induced by norepinephrine and concomitantly prevents cardiovascular remodeling evoked by adrenergic activation independently of blood pressure levels.

Circadian and wake-dependent effects on the pupil light reflex in response to narrow-bandwidth light pulses.

PURPOSE: Nonvisual light-dependent functions in humans are conveyed mainly by intrinsically photosensitive retinal ganglion cells, which express melanopsin as photopigment. We aimed to identify the effects of circadian phase and sleepiness across 24 hours on various aspects of the pupil response to light stimulation. METHODS: We tested 10 healthy adults hourly in two 12-hour sessions covering a 24-hour period. Pupil responses to narrow bandwidth red (635 ± 18 nm) and blue (463 ± 24 nm) light (duration of 1 and 30 seconds) at equal photon fluxes were recorded, and correlated with salivary melatonin concentrations at the same circadian phases and to subjective sleepiness ratings. The magnitude of pupil constriction was determined from minimal pupil size. The post-stimulus pupil response was assessed from the pupil size at 6 seconds following light offset, the area within the redilation curve, and the exponential rate of redilation. RESULTS: Among the measured parameters, the pupil size 6 seconds after light offset correlated with melatonin concentrations (P < 0.05) and showed a significant modulation over 24 hours with maximal values after the nocturnal peak of melatonin secretion. In contrast, the post-stimulus pupil response following red light stimulation correlated with subjective sleepiness (P < 0.05) without significant changes over 24 hours. CONCLUSIONS: The post-stimulus pupil response to blue light as a marker of intrinsic melanopsin activity demonstrated a circadian modulation. In contrast, the effect of sleepiness was more apparent in the cone contribution to the pupil response. Thus, pupillary responsiveness to light is under influence of the endogenous circadian clock and subjective sleepiness.

Laparoscopic surgery for coeliac artery compression syndrome: current management and technical aspects.

Objectives: The study aims to assess the feasibility and midterm outcome of trans-peritoneal laparoscopy for coeliac artery compression syndrome (CACS).Design: Retrospective chart review involving four European vascular surgery departments and two surgical teams.Materials and methods: charts for patients who underwent laparoscopy for symptomatic CACS between December 2003 and November 2009 were reviewed. Preoperative computed tomography (CT) angiography and postoperative duplex scan and/or CT angiography were performed.Results: Eleven consecutive patients (nine women) with a median age of 52 years (interquartile range: 42.5-59 years) underwent trans-peritoneal laparoscopy for CACS. All patients had a history of postprandial abdominal pain; weight loss exceeded 10% of the body mass in eight cases. Preoperative CT angiography revealed coeliac trunk stenosis >70% in all cases. One patient had additional aortitis and inferior mesenteric artery occlusion, while another patient presented with an occluded superior mesenteric artery. Two conversions occurred (one difficult dissection and one aorto-hepatic bypass needed for incomplete release of CACS). The median blood loss was 195 ml (range: 50-900 ml) and median operative time was 80 min (interquartile range: 65-162.5 years). Symptoms improved immediately in 10/11 patients (no residual stenosis) while one remained unchanged despite a residual stenosis treated by a percutaneous angioplasty. Symptoms reappeared in one patient due to coeliac axis occlusion. The mean follow-up period was 35 +/- 23 months (range: 12-78 months).Conclusion: Our study demonstrates that trans-peritoneal laparoscopy for treating median arcuate ligament syndrome is safe and feasible. Additional patients and a longer follow-up are needed for long-term assessment of this laparoscopic technique. (C) 2011 European Society for Vascular Surgery. Published by Elsevier Ltd. All rights reserved.

Esophageal and pharyngeal strictures: report on 1,862 endoscopic dilatations using the Savary-Gilliard technique

Treatment of symptomatic pharyngeal and esophageal strictures requires endoscopic dilatation. The Savary-Gilliard bougienage was developed by our department and has been used since 1980 for this purpose. We report our experience using this technique. The records of patients seen from January 1, 1963 to December 31, 2005, who had pharyngeal and esophageal strictures needing dilatation, were reviewed. The prevalence of different etiologies, and the incidence of complications using the Savary-Gilliard dilators were assessed. Efficiency of dilatation was assessed over a 17-year segment of this period, using number of dilatations and time intervals between dilatations until resolution of symptoms as outcome measures. Of the 2,652 pharyngeal and esophageal strictures reviewed, 90% were of organic origin (45% benign and 55% malignant stenoses), and 10% were of functional etiology. The most common etiologies were peptic strictures before the era of proton pump inhibitors, and postoperative anastomotic strictures thereafter. A total of 1,862 dilatations using the Savary-Gilliard technique were analyzed. Complication and mortality rates were 0.18 and 0.09% for benign and 4.58 and 0.81% for malignant etiologies, respectively. The number of dilatations per stricture and the time interval between different sessions were dependent on the type of strictures, varying from 1 to 23 dilatations and 7 days to 16 years, respectively. Pharyngeal and esophageal dilatations using the Savary-Gilliard technique were safe when used together with fluoroscopy. Overall, the efficiency of the dilatation procedure was good, but some types of strictures (e.g., caustic, post-surgical and/or post radiotherapy) were refractory to treatment and required repeated dilatations.

Palliative portal vein stent placement in malignant and symptomatic extrinsic portal vein stenosis or occlusion.

This article evaluates the results of portal vein (PV) stent placement in patients with malignant extrinsic lesions stenosing or obstructing the PV and causing symptomatic PV hypertension (PVHT). Fourteen patients with bile duct cancer (n = 7), pancreatic adenocarcinoma (n = 4), or another cancer (n = 3) underwent percutaneous transhepatic portal venous stent placement because of gastroesophageal or jejunal varices (n = 9), ascites (n = 7), and/or thrombocytopenia (n = 2). Concurrent tumoral obstruction of the main bile duct was treated via the transhepatic route in the same session in four patients. Changes in portal venous pressure, complications, stent patency, and survival were evaluated. Mean +/- standard deviation (SD) gradient of portal venous pressure decreased significantly immediately after stent placement from 11.2 mmHg +/- 4.6 to 1.1 mmHg +/- 1.0 (P < 0.00001). Three patients had minor complications, and one developed a liver abscess. During a mean +/- SD follow-up of 134.4 +/- 123.3 days, portal stents remained patent in 11 patients (78.6%); stent occlusion occurred in 3 patients, 2 of whom had undergone previous major hepatectomy. After stent placement, PVHT symptoms were relieved in four (57.1%) of seven patients who died (mean survival, 97 +/- 71.2 days), and relieved in six (85.7%) of seven patients still alive at the end of follow-up (mean follow-up, 171.7 +/- 153.5 days). Stent placement in the PV is feasible and relatively safe. It helped to relieve PVHT symptoms in a single session.

Improved growth with bioabsorbable sutures in both high- and low-pressure zones.

BACKGROUND: Compromised growth after operation remains a significant problem in the cardiovascular field. Some benefit of absorbable suture materials has been demonstrated for arterial anastomoses. However, for the low-pressure zone, few data are available. METHODS: To assess growth in high- versus low-pressure zones we transected the abdominal aorta (high-pressure zone) as well as the inferior vena cava (low-pressure zone) in 10 young mongrel dogs using for reanastomosis 7-0 nonabsorbable versus absorbable running sutures in random order. RESULTS: All animals survived and were evaluated over 12 months including body weight (gain, 212% +/- 45% for nonabsorbable versus 218% +/- 8% for absorbable; not significant), angiography, and, after elective sacrifice, detailed studies of aorta and vena cava. Systematic complication of angiographic data at 12 months showed at the suture level an area of 13.8 mm2 for nonabsorbable versus 24.3 +/- 14.4 mm2 for absorbable sutures in the high-pressure zone as compared with 12.9 +/- 4.9 mm2 for nonabsorbable versus 25.3 +/- 15.4 mm2 for absorbable sutures in the low-pressure zone. Residual lumen, calculated as a function of the area above and below the suture, accounted for 35% +/- 10% for nonabsorbable versus 92% +/- 12% for absorbable sutures (p < 0.001) in the high-pressure zone as compared with 37% +/- 13% for nonabsorbable versus 75% +/- 15% for absorbable sutures (p < 0.003) in the low-pressure zone (high versus low, not significant). Poststenotic dilatation accounted for 199% +/- 22% for nonabsorbable versus 126% +/- 43% for absorbable sutures (p < 0.01) in the high-pressure zone. In the low-pressure zone, poststenotic dilatation remained below the inflow area, and the residual poststenotic lumen accounted for 52% +/- 14% for nonabsorbable versus 77% +/- 16% for absorbable sutures (p < 0.004). Macroscopic, light, and scanning electron microscopic studies confirmed different growth patterns in high- versus low-pressure zones. CONCLUSIONS: Aortic narrowing resulted in poststenotic dilatation and unrestricted outflow path (hourglass-type stenosis). Caval narrowing was followed by restriction of poststenotic outflow path (funnel-type stenosis). Absorbable suture material allows for superior growth in both high- and low-pressure zones.

Protéinurie orthostatique et phénomène de compression de la veine rénale gauche (nutcracker syndrome) [Orthostatic proteinuria and compression of the left renal vein (nutcracker syndrome)]

INTRODUCTION: The pathogenic mechanism of orthostatic proteinuria has not yet been clearly established. OBSERVATION: In a tall, thin, 21 year-old man, isolated proteinuria was discovered during an urological control conducted one year after a bilateral orchidopexy following left testicular torsion. Proteinuria was orthostatic. Doppler examination of the kidney revealed an entrapment of the left renal vein (nutcracker phenomenon-NCP). COMMENTS: An NCP was diagnosed in a young patient presenting with orthostatic proteinuria. By provoking modifications in intraglomerular haemodynamics, the NCP may, in nearly half of the cases, be at the origin of orthostatic proteinuria. Doppler examination is the diagnostic method of choice in the screening for NCP.

Cardiac raptor deletion impairs adaptive hypertrophy, alters metabolic gene expression and causes heart failure in mice

Background: Mammalian target of rapamycin (mTOR), a central regulator of cell growth, is found in two structurally and functionally distinct multiprotein complexes called mTOR complex (mTORC)1 and mTORC2. The specific roles of each of these branches of mTOR signaling have not been dissected in the adult heart. In the present study, we aimed to bring new insights into the function of cardiac mTORC1-mediated signaling in physiological as well as pathological situations.Methods: We generated mice homozygous for loxP-flanked raptor and positive for the tamoxifen-inducible Cre recombinase (MerCreMer) under control of the α- myosin heavy chain promoter. The raptor gene encodes an essential component of mTORC1. Gene ablation was induced at the age of 10-12 weeks, and two weeks later the raptor cardiac-knockout (raptor-cKO) mice started voluntary cagewheel exercise or were subjected to transverse aortic constriction (TAC) to induce pressure overload.Results: In sedentary raptor-cKO mice, ejection fractions gradually decreased, resulting in significantly reduced values at 38 days (P < 0.001). Raptor-cKO mice started to die during the fifth week after the last tamoxifen injection. At that time, the mortality rate was 36% in sedentary (n = 11) and 64% in exercising (n = 14) mice. TAC-induced pressure overload resulted in severe cardiac dysfunction already at earlier timepoints. Thus, at 7-9 days after surgery, ejection fraction and fractional shortening values were 22.3% vs 43.5% and 10.2% vs 21.5% in raptor-cKO vs wild-type mice, respectively. This was accompanied by significant reductions of ventricular wall and septal thickness as well as an increase in left ventricular internal diameter. Moreover, ventricular weight to tibial length ratios were increased in wild-type, but not in the raptor-cKO TAC mice. Together, this shows that raptor-cKO mice rapidly developed dilated cardiomyopathy without going through a phase of adaptive hypertrophy. Expression of ANP and β-MHC was induced in all raptor-cKO mice irrespective of the cardiac load conditions. Consistent with reduced mTORC1 activity, phosphorylation of ribosomal S6 kinase and 4E-BP1 was blunted, indicating reduced protein synthesis. Moreover, expression of multiple genes involved in the regulation of energy metabolism was altered, and followed by a shift from fatty acid to glucose oxidation.Conclusion: Our study suggests that mTORC1 coordinates protein and energy metabolic pathways in the heart. Moreover, we demonstrate that raptor is essential for the cardiac adaptation to increased workload and importantly, also for normal physiological cardiac function.

Endovascular treatment of symptomatic intracranial arterial stenosis: six-year experience in a single-center series of 42 consecutive patients with acute and mid-term results.

BACKGROUND: The limitations of the medical management of symptomatic intracranial arterial stenosis encourage the development of new therapeutic strategies such as intracranial stenting. OBJECTIVE: To report and analyze the results of a series of 42 patients treated with 3 different endovascular techniques: isolated angioplasty, balloon-expandable coronary stents, and the Wingspan self-expandable intracranial stent system. METHODS: Forty-two patients presenting with symptomatic intracranial arterial stenosis were treated with one of these techniques. Computed tomography angiography was performed 6 months after the procedure, and the clinical neurological statuses were categorized using the modified Rankin Scale and the National Institutes of Health Stroke Scale. RESULTS: A total of 42 lesions were treated: 9 with isolated angioplasty, 14 with balloon-expandable coronary stents, and 19 with Wingspan self-expandable intracranial stents. The mean patient age was 62.9 years, and the mean arterial diameter stenosis was 73.9%. Technical success was achieved in 97.6% of the patients. The overall incidence of procedural complications was 21.4%, and the postoperative permanent morbidity/mortality rate was 7.1%. There were 3 cases of in-stent thrombosis (1 fatal) and 5 cases of asymptomatic restenosis (11.9%), 3 in the isolated angioplasty group and 2 in the Wingspan self-expandable intracranial stent group (mean follow-up 20.4 months). The rate of restenosis was higher in the angioplasty group (33%) than in the coronary (0%) and Wingspan stent (10.5%) groups. CONCLUSION: Endovascular treatment of symptomatic intracranial stenosis has significant overall morbidity and mortality rates. Nevertheless, the very critical natural history of severe refractory lesions and the relatively favorable postoperative evolution suggest that it should be considered the first alternative strategy in cases in which medical therapy has failed.

Investigations of a thermosensitive gel to temporarily occlude crural arteries in femoro-distal bypass surgery.

OBJECTIVES: Long occlusions in calcified crural arteries are a major cause of endovascular technical failure in patients with critical limb ischaemia. Therefore, distal bypasses are mainly performed in patients with heavily calcified arteries and with consequently delicate clamping. A new reverse thermosensitive polymer (RTP) is an alternative option to occlude target vessels. The aim of the study is to report our technical experience with RTP and to assess its safety and efficiency to temporarily occlude small calcified arteries during anastomosis time. METHODS: Between July 2010 and December 2011, we used RTP to occlude crural arteries in 20 consecutive patients with 20 venous distal bypasses. We recorded several operative parameters, such as volume of injected RTP, duration of occlusion and anastomotic time. Quality of occlusion was subjectively evaluated. Routine on-table angiography was performed to search for plug emboli. Primary patency, limb salvage and survival rates were reported at 6 months. RESULTS: In all patients, crural artery occlusion was achieved with the RTP without the use of an adjunct occlusion device. Mean volume of RTP used was 0.3 ml proximally and 0.25 ml distally. Mean duration of occlusion was 14.4 ± 4.5 min, while completion of the distal anastomosis lasted 13.4 ± 4.3 min. Quality of occlusion was judged as excellent in eight cases and good in 12 cases. Residual plugs were observed in two patients and removed with an embolectomy catheter, before we amended the technique for dissolution of RTP. At 6 months, primary patency rate was 75% but limb salvage rate was 87.5%. The 30-day mortality rate was 10%. CONCLUSIONS: This study shows that RTP is safe when properly dissolved and effective to occlude small calcified arteries for completion of distal anastomosis.

Altered high-energy phosphate metabolism predicts contractile dysfunction and subsequent ventricular remodeling in pressure-overload hypertrophy mice.

To study the role of early energetic abnormalities in the subsequent development of heart failure, we performed serial in vivo combined magnetic resonance imaging (MRI) and (31)P magnetic resonance spectroscopy (MRS) studies in mice that underwent pressure-overload following transverse aorta constriction (TAC). After 3 wk of TAC, a significant increase in left ventricular (LV) mass (74 +/- 4 vs. 140 +/- 26 mg, control vs. TAC, respectively; P < 0.000005), size [end-diastolic volume (EDV): 48 +/- 3 vs. 61 +/- 8 microl; P < 0.005], and contractile dysfunction [ejection fraction (EF): 62 +/- 4 vs. 38 +/- 10%; P < 0.000005] was observed, as well as depressed cardiac energetics (PCr/ATP: 2.0 +/- 0.1 vs. 1.3 +/- 0.4, P < 0.0005) measured by combined MRI/MRS. After an additional 3 wk, LV mass (140 +/- 26 vs. 167 +/- 36 mg; P < 0.01) and cavity size (EDV: 61 +/- 8 vs. 76 +/- 8 microl; P < 0.001) increased further, but there was no additional decline in PCr/ATP or EF. Cardiac PCr/ATP correlated inversely with end-systolic volume and directly with EF at 6 wk but not at 3 wk, suggesting a role of sustained energetic abnormalities in evolving chamber dysfunction and remodeling. Indeed, reduced cardiac PCr/ATP observed at 3 wk strongly correlated with changes in EDV that developed over the ensuing 3 wk. These data suggest that abnormal energetics due to pressure overload predict subsequent LV remodeling and dysfunction.