Klippel-Feil syndrome. When using fiberoptic bronchoscopy guide, a case report

Klippel-Feil syndrome is a rare disease with congenital musculoskeletal condition characterized by faulty segmentation of cervical vertebrae and consists of cervical vertebra fusions with limitation of head movements, short neck and low posterior hairline. In several cases the syndrome is associated with cardiovascular malformations. Patients affected by Klippel-Feil syndrome could be an anesthetic challenge, not only during cardiac surgery. We are presenting a case of Klippel-Feil Syndrome in an adult patient, who was operated on for a pulmonary valve insufficiency in a previously corrected Tetralogy of Fallot Syndrome. We are going to discuss the features of this rare syndrome

The dark atrophy with indocyanine green angiography

PURPOSE. To evaluate differences in fluorescein angiography (FA) and indocyanine green angiography (ICGA), findings between subjects affected by Stargardt disease (STGD) and atrophic AMD. METHODS. This was a consecutive, cross-sectional case series. A total of 24 eyes of 12 patients with STGD and 23 eyes of 14 patients with atrophic AMD were enrolled in the study. Patients underwent dynamic simultaneous FA and ICGA using a dual beam confocal scanning system. Images were recorded from the initial filling of choroidal and retinal vessels throughout all the phases of the angiogram. Spectral-domain optical coherence tomography (SD-OCT) and fundus autofluorescence were also executed. FA and ICGA findings in the two groups were evaluated. RESULTS. In 92% (22/24) of eyes affected by STGD, ICGA showed hypocyanescence from the areas of atrophy, more evident in the late phases. This finding, defined as ICGA-imaged ''dark atrophy,'' was present in only 13% (3/23) of the eyes affected by atrophic AMD. The remaining eyes in both groups showed iso-or mild hypercyanescence from the areas of atrophy. Eyes with ICGA-imaged dark atrophy, both in STGD and in atrophic AMD groups, did not show early obscuration of the choroidal vessels by FA. SD-OCT revealed morphologically intact choroid in STGD patients with ICGA-imaged dark atrophy. In atrophic AMD eyes with ICGA-imaged dark atrophy, SD-OCT revealed a severely thinned choroid. CONCLUSIONS. Hypocyanescence by ICGA from the areas of atrophy was more frequent in STGD compared with atrophic AMD. This finding, along with SD-OCT evidence of intact choroid, suggests a possible selective damage of the choriocapillaris in STGD. (Invest Ophthalmol Vis Sci. 2012;53:3999-4004) DOI:10.1167/iovs.11-9258 R ecessive Stargardt disease (STGD) and age-related macular degeneration (AMD) lead to progressive and severe visual acuity loss. STGD is one of the most common inherited retinal dystrophies, while AMD is the most important cause of central visual acuity loss in western countries. STGD typically appears before age 20. It exhibits simple Mendelian transmission and is caused by mutations in the ABCA4 gene. A reduction in ABCA4 activity in the photoreceptors results in the increased production and accumulation of A2E and related bisretinoids within RPE cells. 1,2 These compounds cannot be readily metabolized and have negative effects on RPE cell function and viability. 3,4 RPE cell loss might originate from several mechanisms, including photooxidative stress, 5 vascular alterations, 6,7 and deposition of toxic lipofuscin material under RPE. Fluorescein angiography (FA) and indocyanine green angiography (ICGA) are important tools for diagnostic and pathogenetic evaluation of the two diseases. In STGD, FA is helpful because it allows for the identification of the dark choroid. This finding is characterized by the absence of normal background fluorescence mainly due to the presence of RPE lipofuscin that absorbs the blue excitatory light. 8 Also, ICGA may provide useful information, in particular about the alterations of the choroid and the choriocapillaris. 10 In addition, partial absorption by retinal pigment epithelial melanin occurs. In contrast, indocyanine green absorbs and emits light in the near-infrared spectrum, and allows better penetration. Furthermore, indocyanine green is predominantly bound to plasma protein (98% compared with 60%-80% for fluorescein), and this limits its diffusion through the fenestrations of the choriocapillaris. 11 Moreover, AMD may be characterized by a presumed macular choroidal watershed filling. 12 ICGA showed diminished choroidal arterial perfusion of the macula and enlargement of choroidal veins in the pathogenesis of AMD. 13 The combination of FA and ICGA facilitates interpretation of the exam and provides more information than either FA or ICGA alone. 10 Therefore, in this study, simultaneous FA and ICGA were used to evaluate possible differences in the pathogenesis of the two clinical entities. METHODS Twenty-six consecutive patients affected by STGD and atrophic AMD

How far is too far? Exploring the indications for robotic partial nephrectomy in a highly complex kidney tumor

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Protocol of the Italian Radical Cystectomy Registry (RIC): a non-randomized, 24-month, multicenter study comparing robotic-assisted, laparoscopic, and open surgery for radical cystectomy in bladder cancer

Bladder cancer is the ninth most common type of cancer worldwide. In the past, radical cystectomy via open surgery has been considered the gold-standard treatment for muscle invasive bladder cancer. However, in recent years there has been a progressive increase in the use of robot-assisted laparoscopic radical cystectomy. The aim of the current project is to investigate the surgical, oncological, and functional outcomes of patients with bladder cancer who undergo radical cystectomy comparing three different surgical techniques (robotic-assisted, laparoscopic, and open surgery). Pre-, peri- and post-operative factors will be examined, and participants will be followed for a period of up to 24\u2009months to identify risks of mortality, oncological outcomes, hospital readmission, sexual performance, and continence

Protocol of the Italian Radical Cystectomy Registry (RIC): a non-randomized, 24-month, multicenter study comparing robotic-assisted, laparoscopic, and open surgery for radical cystectomy in bladder cancer

Background: Bladder cancer is the ninth most common type of cancer worldwide. In the past, radical cystectomy via open surgery has been considered the gold-standard treatment for muscle invasive bladder cancer. However, in recent years there has been a progressive increase in the use of robot-assisted laparoscopic radical cystectomy. The aim of the current project is to investigate the surgical, oncological, and functional outcomes of patients with bladder cancer who undergo radical cystectomy comparing three different surgical techniques (robotic-assisted, laparoscopic, and open surgery). Pre-, peri- and post-operative factors will be examined, and participants will be followed for a period of up to 24 months to identify risks of mortality, oncological outcomes, hospital readmission, sexual performance, and continence. Methods: We describe a protocol for an observational, prospective, multicenter, cohort study to assess patients affected by bladder neoplasms undergoing radical cystectomy and urinary diversion. The Italian Radical Cystectomy Registry is an electronic registry to prospectively collect the data of patients undergoing radical cystectomy conducted with any technique (open, laparoscopic, robotic-assisted). Twenty-eight urology departments across Italy will provide data for the study, with the recruitment phase between 1st January 2017-31st October 2020. Information is collected from the patients at the moment of surgical intervention and during follow-up (3, 6, 12, and 24 months after radical cystectomy). Peri-operative variables include surgery time, type of urinary diversion, conversion to open surgery, bleeding, nerve sparing and lymphadenectomy. Follow-up data collection includes histological information (e.g., post-op staging, grading, and tumor histology), short- and long-term outcomes (e.g., mortality, post-op complications, hospital readmission, sexual potency, continence etc). Discussion: The current protocol aims to contribute additional data to the field concerning the short- and long-term outcomes of three different radical cystectomy surgical techniques for patients with bladder cancer, including open, laparoscopic, and robot-assisted. This is a comparative-effectiveness trial that takes into account a complex range of factors and decision making by both physicians and patients that affect their choice of surgical technique. Trial registration: ClinicalTrials.gov , NCT04228198 . Registered 14th January 2020- Retrospectively registered

Variazioni dell'apofisi trasversa della settima vertebra cervicale: studio anatomo - radiologico su una popolazione <<segregata>>

Gli autori hanno studiato le modificazioni morfologiche delle apofisi trasverse della settima vertebra cervicale. Sono state osservate principalmente due variazioni: la mega - apofisi trasversa e la costa cervicale. Gli autori hanno riscontrato una incidenza di variazioni notevolmente superiore rispetto a quanto riportato in letteratura, suppongono che ciò sia dovuto alla relativa > geografica e socio - economica ed ipotizzano una trasmissione genetica di tale variazione del tipo autosomico recessivo

Novel diamond X-ray detectors with patterned reduced graphene oxide contacts

A novel kind of detector based on polycrystalline grade diamond substrate and Reduced Graphene Oxide (RGO) contacts is presented. This detector combines some of the good qualities of diamond (i.e. radiation hardness and almost unique combination of electric, thermal and optical properties) with low- Z contacts. This characteristic together with the possibility of patterning the electrodes with standard lithographic techniques, make this detector particularly suitable for X-ray beam monitors where the intensity and the position of the photon beam needs to be measured with minimal effect on the beam itself (i.e. in-line and highly transmissive measurement). The steps needed to realize our novel detectors together with a new Graphene Oxide (GO) rapid thermal reduction process are described and the results of preliminary X-ray tests reported

Alastrim, varíola é? Is allastrim small pox?

Este artigo trata de uma controvérsia científica havida em São Paulo entre 1910 e 1913, que opôs dois destacados médicos da cidade: Antonio Carini, diretor do Instituto Pasteur de São Paulo, e Emílio Ribas, diretor do Serviço Sanitário. A questão em jogo era a classificação de uma doença que reinava no estado. Para Carini tratava-se de varíola, para Ribas era uma entidade mórbida chamada de alastrim. A polêmica teve início na Sociedade de Medicina e Cirurgia e, posteriormente, migrou para outros fóruns, incorporando novos personagens. A apresentação e discussão dessa controvérsia objetiva contribuiu para a compreensão do processo de construção e estabilização de um consenso científico no campo das ciências biomédicas.<br>Between 1910 and 1913, two renowned physicians in the city of São Paulo found themselves engaged in a scientific controversy regarding the classification of a disease then assailing the state. Antonio Carini, director of the Instituto Pasteur de São Paulo, believed the illness to be small pox, while Emílio Ribas, director of the Serviço Sanitário, claimed it was allastrim, or milk pox. The controversy started in the Sociedade de Medicina e Cirurgia but later migrated to other forums and came to incorporate other figures as well. This presentation and discussion of the polemic is meant as a contribution to our understanding of the process by which a scientific consensus is constructed and solidified within the field of the biomedical sciences

A BRIEF ANATOMO-SURGICAL DISSECTION GUIDE TO HUMAN LOWER LIMB: RESULTS OF THE COLLABORATION BETWEEN THE UNIVERSITY OF PALERMO AND THE UNIVERSITY OF MALTA

The aim of this Article is to show methods for dissection of the lower limb. In the summer 2017 a group of students at the University of Palermo that have already passed the exam of Human Anatomy took a 4 weeks dissection course at the University of Malta - School of Medicine. The students were provided with a dissection kit, video recording equipment and cameras for taking pictures. This paper presents the results of the dissection course and a small and simple guide to young students and medical doctors who want to learn the bases of lower limb dissection