Stevens-Johnson syndrome related with Comirnaty® coronavirus disease 2019 vaccine.

Stevens-Johnson syndrome is a rare severe delayed-type hypersensitivity reaction. Even though not initially described as a side-effect of the Comirnaty® coronavirus disease 2019 (COVID-19) Vaccine, the worldwide public COVID-19 vaccination programs are uncovering this serious adverse event. We present the case of a 44-year-old woman, vaccinated with the 1st dose in July 2021, and the 2nd dose 4 weeks later. Five days after the 2nd dose, a 10 cm, circular, painful, violet/red lesion appeared on the injection site. From then on, multiple, generalized purpuric painful lesions appeared, associated with ulcers on the lips, oral cavity, nasal cavity, vulva, and vagina, oedema of the hands and feet, conjunctival erythema, blurred vision, and malaise. The patient was being treated with lamotrigine and sodium valproate (for 2 years, without interruptions or dose change) which were stopped, and the patient started treatment with systemic corticosteroids. Lymphocyte transformation test were performed and were positive for PEG2000 1 µg/mL (stimulation index [SI], 30.9), and the undiluted Comirnaty® vaccine (SI, 32.2). These tests were negative on several vaccinated controls. We can definitively show that sensitization to the vaccine and PEG2000 can occur. A more extensive evaluation and reporting is needed to know the true incidence of this life-threatening condition and possible risk factors; as not only further booster shots of this vaccine will be administered, but also new vaccines with the mRNA technology are likely to be more prevalent in the future.

Prediction of ground vibrations induced by rail traffic in Lisbon urban area.

The possibility of introducing high-speed trains in Portugal will challenge the regular train circulation of existing railways tracks. To carry this traffic, improvements and maintenance programmes will be intensified together with the evaluation of the railway performance and its consequences on neighbourhood structures, including the execution of several vibration measurement tests. One of them was performed in Lisbon's urban area for measure of ground-borne vibration due to the train traffic. It was used to develop an efficient numerical model, which allowed to study the influence of introducing high-speed vehicles on the generation and propagation of vibrations through the free field and the impact on the wayside buildings. To access the effect of introducing such trains in the existing tracks, three bi-dimensional numerical models for each measurement profile were developed. Numerical models were calibrated using the monitoring records. Then, new simulations were performed with Thalys HST train allowing to conclude that the introduction of high-speed trains will not lead to an increase of level of vibrations, for the adopted traffic conditions.

Contact Dermatitis: Overcoming Challenges of Specific Patients, Deciphering the Results and Reaching a Correct Diagnosis.

Skin lesions caused by allergic contact dermatitis are an important occupational and environmental disease. Patch testing is the gold-standard procedure used to diagnose allergic contact dermatitis.The present chapter summarizes aspects of patch testing for the diagnosis of contact allergy: important working definitions, relevance of treating contact dermatitis, materials, technique, test result and interpretation, and special consideration regarding individual factors which influence the patch test outcome or necessitate special attention.Performing and interpreting patch tests requires know-how. Knowing how to perform them and the particularities of specific cases is essential to correctly interpret the results. A correct evaluation and diagnosis will significantly impact the natural history of the disease and significantly improve the quality of life of the patient.

What Basophil Testing Tells Us About CSU Patients - Results of the CORSA Study.

Basophil testing is the most effective single approach for diagnosing type-IIb autoimmune chronic spontaneous urticaria (TIIbaiCSU). A positive basophil test has been linked to long disease duration, higher disease activity, a poor response to antihistamines and omalizumab, and a better response to cyclosporine and fenebrutinib. As of now it is unclear what other features are connected to a positive basophil test in chronic spontaneous urticaria (CSU). We aimed to identify features of basophil test-positive CSU patients. We performed a cross-sectional study of 85 CSU patients. Basophil testing was done with the basophil activation test (BAT) and the basophil histamine release assay (BHRA). Data were analysed using SPSS: Student's t-test, Chi-square test, Odds Ratio, Spearman's correlation test. Of 85 CSU patients, 44% and 28% tested positive with the BAT and BHRA, respectively. These patients showed higher disease activity and impact, lower levels of disease control and total serum IgE, as well as higher rates of having a positive autologous serum skin test (ASST), angioedema, nocturnal symptoms, symptoms for >5 days/week, and thyroid autoantibodies. The ASST, by itself, was not a good predictor of basophil test results, but it predicted a positive basophil test in up to 100% of cases when combined with angioedema, thyroid autoantibodies or low IgE. In conclusion, a positive basophil test is linked to known features of TIIbaiCSU and novel characteristics including nocturnal symptoms. Further studies on basophil test-positive and -negative CSU patients can help to better understand CSU endotypes and to develop better management approaches.

Radiographic healing patterns after tooth-borne distraction in canine model.

BACKGROUND: The osteogenesis distraction technique applied to the craniofacial skeleton is an alternative treatment for dentofacial deformities. Despite the advantages of tooth-borne distractors, few studies have evaluated their clinical implementation in sagittal dentoskeletal deformities. This study aimed provide a radiographic assessment of the effect of two different activations of tooth-borne distraction in the lengthening of the mandible in canines. MATERIAL AND METHODS: Ten male beagle dogs, approximately one year old, were used for this experimental study. Three remained as a control group and seven underwent a mandibular tooth-borne distraction protocol with single daily activation in one hemimandible and two daily activations in the other, during ten days. The consolidation period took 12 weeks. Occlusal radiographs were performed immediately pre- and postoperatively. RESULTS: After the distraction period, the host bone margins presented very well-defined outlines with regular contours. Concerning the consolidation period, between the second and fourth weeks, all hemimandibles showed small rectangular radiopaque regions with parallel orientation to the distraction axis. At the twelfth week, all hemimandibles presented an entire mineralization of the distraction gap with no axial deviations of the anterior and posterior host bone, nine of which with both margins showing corticalization. CONCLUSIONS: Radiographic analysis showed bone regeneration in order to achieve the original bone architecture, especially in the group of multiple distraction. Tooth-borne distraction allowed successful sagittal lengthening of the mandible in a canine model. Key words:Orthodontics, osteogenesis, distraction, mandibular advancement, orthodontic appliance design.

The Impact of Sacral Neuromodulation on Sexual Dysfunction.

BACKGROUND/AIMS: Sacral neuromodulation (SNM) is a well-established treatment in several urinary and bowel dysfunctions, nevertheless its role on sexual dysfunction remains unclear. We evaluate the impact of SNM on sexual function and its association with age at SNM, functional diagnosis and post-void residual urine (PVR) before SNM. METHODS: Patients who had SNM were retrospectively analyzed. Sexual function was assessed before and after treatment with the International Index of Erectile Function (IIEF-5) for men and the Female Sexual Function Index (FSFI) for women. IIEF-5 and FSFI were also associated with age at SNM, functional diagnosis, and PVR. RESULTS: Fifteen females and 9 males, with a median age of 41 years (26-72 years), median follow-up 20.7 months (2-53 months) were enrolled. IIEF-5 improved in 4 patients (p = 0.06), and FSFI total score in 5 (p = 0.2). There was significant association between functional diagnosis and FSFI total score (p = 0.05), and FSFI specific domains of arousal (p = 0.03), lubrication (p = 0.04), and satisfaction (p = 0.03), with significant improvement showed in patients with detrusor overactivity with impaired contractility. CONCLUSION: Although gains observed in IIEF-5 and FSFI were modest, our preliminary results show that SNM may have favorable impact on sexual function.

Esofagite eosinofílica numa consulta de alergia alimentar: caracterização e comparação entre idade pediátrica e idade adulta / Eosinophilic esophagitis at a food allergy appointment: characterization and comparison between pediatric and adult ages

Introdução: Esofagite eosinofílica (EoE) é uma doença inflamatória crônica do esôfago, mediada imunologicamente e caracterizada por sintomas relacionados com disfunção esofágica e infiltração da mucosa esofágica por eosinófilos (Eo). Os objetivos foram caracterizar os doentes com diagnóstico de EoE e analisar as diferenças entre doentes com diagnóstico em idade pediátrica (Cr, < 18 anos) e adulta (Ad, ≥ 18 anos). Métodos: Estudo observacional retrospetivo dos doentes seguidos no serviço de Imunoalergologia, no período de Fev/2009 a Jul/2017, com diagnóstico de EoE. Foram divididos em dois grupos, Cr e Ad, caracterizados de acordo com dados demográficos, história de atopia, sintomas, sensibilizações alimentares, IgE Total, eosinofilia, achados na endoscopia digestiva alta e biópsias. Avaliou-se a correlação entre sensibilização alimentar, clínica grave (ClinG), ou seja, idas ao serviço de urgência ou internamento por complicações de EoE ou histologia grave (HistG), biópsia com Eo > 50 e/ou microabcessos. Resultados: 74 pacientes (81% sexo masculino, média de idades 27±17 anos), 36 Cr e 38 Ad. Os sintomas mais frequentemente reportados foram, no grupo Cr disfagia (73%) e refluxo gastroesofágico (46%), enquanto no grupo Ad impactação (85%) e disfagia (56%). Foram referidos antecedentes de atopia em 96% das Cr, e 67% dos Ad. Em 77% das Cr e 69% dos Ad havia sensibilização alimentar. Os achados endoscópicos mais frequentes no grupo Cr foram estriação (65%) e placas brancas (50%), enquanto que no grupo Ad foram placas brancas (42%) e anéis esofágicos (35%). HistG (46%) associou-se a ClinG (35%), p = 0,001, nas Cr, mas o mesmo não foi objetivado no grupo Ad [ClinG (22%) e HistG (17%), p = 0,5]. Conclusão: Os nossos resultados estão de acordo com o descrito na literatura, observando-se um predomínio do sexo masculino e uma maior frequência de história de atopia e sensibilização alimentar no grupo Cr. As situações graves de impactação e estenose esofágica foram mais frequentes nos Ad, e objetivou-se uma associação de histologia grave com clínica grave, apenas nas Cr.

Background: Eosinophilic esophagitis (EoE) is an immunologically mediated chronic inflammatory disease of the esophagus characterized by symptoms related to esophageal dysfunction and eosinophilic infiltration in the esophageal mucosa. This study aimed to characterize patients with a diagnosis of EoE and analyze differences between patients with EoE diagnosed at pediatric age (Ch, <18 years) and at adult age (Ad, ≥18 years). Methods: This was a retrospective study of patients with a diagnosis of EoE who were followed in the immunoallergology department from February 2009 to July 2017. Patients were divided into Ch and Ad groups and characterized according to demographic data, history of atopy, symptoms, food sensitization, total IgE, eosinophils (Eo), upper digestive endoscopy (UDE) findings, and biopsy findings. Correlations were assessed between food sensitization, clinical severity (SClin; determined by number of ER visits or hospital admissions for EoE complications), and severe histology (SHist; defined as biopsy with Eo >50 and/or microabscesses). Results: 74 patients (81% male, mean age 27±17 years), 36 Ch and 38 Ad. The most commonly reported symptoms were dysphagia (73%) and gastroesophageal reflux (46%) in the Ch group, and impaction (85%) and dysphagia (56%) in the Ad group. History of atopy was reported in 96% of Ch vs 67% of Ad. Food sensitization was found in 77% of Ch vs 69% of Ad. The most common UDE findings were striation (65%) and white plaques (50%) in the Ch group, and white plaques (42%) and esophageal rings (35%) in the Ad group. SHist (46%) was associated with SClin (35%) in Ch (p=0.001), but not in Ad (SClin [22%] and SHist [17%], p=0.5). Conclusion: Our results are consistent with those reported in the literature, with a male predominance and a higher prevalence of atopy and food sensitization in Ch. Severe impaction and esophageal stenosis were more frequent in Ad, but an association between SHist and SClin was found only in Ch.

Adjustable Transobturator Male System after Failed Surgical Devices for Male Stress Urinary Incontinence: A Feasibility Study.

OBJECTIVES: Feasibility study to evaluate the efficacy and safety of Adjustable Transobturator Male System (ATOMS) after failed surgical devices for male stress urinary incontinence (SUI). MATERIALS AND METHODS: Thirty patients were implanted with ATOMS after they were implanted with surgical device/s previously. SUI severity was evaluated as dryness (0-1 pad/day), mild (2 pads/day), moderate (3-5 pads/day), or severe (≥6 pads/day). Change in pad-test and pad-count after adjustment, operative parameters, patient satisfaction, and number and grade of complications were investigated. RESULTS: Previous failed treatment methods were artificial urinary sphincter (AUS; n = 19), Advance (n = 10), and Virtue (n = 1). Six cases had multiple previous treatments. Preoperative SUI was mild 6 (20%), moderate 11 (36.7%), and severe 13 (43.3%). Median pad-test decreased from 435 mL baseline to 10 mL after adjustment and pad-count from 4 to 0. Dry-rate was 76.7 and 83.3% declared satisfied. Postoperative SUI distribution was mild in 3 (10%) and moderate in 4 (13.3%). No patient had urinary retention after catheter removal. Complications presented in 4 (13.3%; 3 grade-I, 1 grade-II). After a median of 24 months follow-up, no system experienced infection or urethral erosion and 1 (3.3%) was removed for inefficacy. CONCLUSION: Based on short-term efficacy and patient satisfaction, ATOMS can be a realistic alternative for male SUI after other failed systems, including AUS. The absence of urethral erosion and limited infective problems makes this alternative attractive for cases with previous failed treatments.

Airway management in a patient with Crouzon syndrome proposed to orthognathic surgery.

Airway management is one of the most important anaesthetist's skills as major complications of airway management, although rare, can be among the most life threatening in medicine. Crouzon syndrome is a rare condition with physical characteristics that can result in difficult airway manipulation. A correct preanaesthetic evaluation and a planned preinduction strategy should be designed to facilitate intubation.

Treatment of male stress urinary incontinence with the adjustable transobturator male system: Outcomes of a multi-center Iberian study.

AIM: To evaluate effectiveness and safety of the adjustable transobturator male system (ATOMS) for male stress urinary incontinence (SUI). MATERIAL AND METHODS: A retrospective multicenter study was conducted in nine Iberian institutions using a board-approved database for 215 patients intervened between 2012 and 2017, with no case excluded. Continence status, patient satisfaction, number, and grade of complications (Clavien-Dindo) and factors affecting dry rate at adjustment were evaluated. Multivariate analysis defined the population at best success rate. Incontinence recurrence due to device failure and/or explant was evaluated and Kaplan-Meier curve for durability performed. RESULTS: Adjustment was achieved at a mean 1.4 ± 1.9 fillings. Dry-rate after adjustment was 80.5% (96.2% mild and 75.3% moderate-severe), 121 (56.3%) used no pads, and 52 (24.2%) a security pad with urine loss under 10 mL. Mean basal daily pad-test and pad-count decreased from 484 ± 372.3 mL and 3.9 ± 2 pads to 63.5 ± 201.2 mL and 0.9 ± 1.5pads (both P < 0.0001). Satisfaction rate was 85.1% (94.3% mild and 82.1% moderate-severe). Factors associated to dryness were: lesser severity of SUI (P < .0001), absence of radiotherapy (P = 0.0002) and device generation (P = 0.05). Multivariate analysis revealed absence of radiation (OR = 3.12; 1.36-7.19), mild (OR = 19.61; 3.95-100), and moderate (OR = 2.48; 1.1-5.59) SUI were independent predictors. Complications presented in 33(15.35%); 66.7% grade 1, 9.1% grade 2, and 24.2% grade 3. At 24.3 ± 15 mo mean follow-up device was explanted in seven (3.25%) and SUI worsened after adjustment in nine (4.2%). Dry-rate at follow-up was 73% and durability of device in dry patients at adjustment was 89.8% (82.9-94) at 2-years. CONCLUSIONS: This study confirms ATOMS device is safe and achieves high treatment efficacy and patient satisfaction in a multicenter setting. Significantly better results are achieved in less severe and non-irradiated cases. Durability of the device is reassuring in the short-term.

Outcome Measures of Adjustable Transobturator Male System with Pre-attached Scrotal Port for Male Stress Urinary Incontinence After Radical Prostatectomy: A Prospective Study.

INTRODUCTION: The objective of this study was to report outcome measures with third-generation pre-attached scrotal port adjustable transobturator male system (ATOMS) for male stress urinary incontinence (SUI) after radical prostatectomy. METHODS: A prospective open study was conducted on consecutive patients. Evaluation included cough test, urethroscopy, filling and voiding cystometry, 24-h pad count and pad test, patient-reported outcomes (ICIQ-SF, IIQ-7, PGI, GRA, and VAS), complications according to the Clavien-Dindo system, operative results, number of adjustments, and filling of the system. RESULTS: Thirty-four patients with median pad test 510 (170-1225) ml were operated on. Preoperative SUI was mild (5.9%), moderate (17.6%), and severe (76.5%). At median 18.5 (12-26) months follow-up distribution of SUI was none (85.3%), mild (8.8%), and moderate (5.9%). Median intraoperative filling was 14 (8-17) ml, number of adjustments 1 (0-5), and total filling 17.5 (11-33.5) ml. At 3 months, median ICIQ-SF (p = 0.0001) and IIQ-7 (p < 0.0001) decreased. At 12 months, 24-h pad count and pad test decreased (both p < 0.0001), residual volume slightly increased (p = 0.018), PGI-I was 1 (1-3), GRA 6 (3-6), and 97% were satisfied with treatment. Continence (p = 0.016) and satisfaction (p = 0.09) were worse in irradiated patients. Median operative time was 67 (35-120) min, hospital stay 1 (1-3) days, and VAS for pain on postoperative day 1 was 0 (0-2). Complications presented in 14.7% (8.8% grade I and 5.9% grade III). CONCLUSION: Treatment of severe male SUI after radical prostatectomy with pre-attached scrotal port ATOMS is safe and very effective in the short term. A positive cough test before implant and intraoperative overfilling of the system may optimize patient selection and results.

3D virtual planning in orthognathic surgery and CAD/CAM surgical splints generation in one patient with craniofacial microsomia: a case report.

OBJECTIVE: In this case report, the feasibility and precision of tridimensional (3D) virtual planning in one patient with craniofacial microsomia is tested using Nemoceph 3D-OS software (Software Nemotec SL, Madrid, Spain) to predict postoperative outcomes on hard tissue and produce CAD/CAM (Computer Aided Design/Computer Aided Manufacturing) surgical splints. METHODS: The clinical protocol consists of 3D data acquisition of the craniofacial complex by cone-beam computed tomography (CBCT) and surface scanning of the plaster dental casts. The ''virtual patient'' created underwent virtual surgery and a simulation of postoperative results on hard tissues. Surgical splints were manufactured using CAD/CAM technology in order to transfer the virtual surgical plan to the operating room. Intraoperatively, both CAD/CAM and conventional surgical splints are comparable. A second set of 3D images was obtained after surgery to acquire linear measurements and compare them with measurements obtained when predicting postoperative results virtually. RESULTS: It was found a high similarity between both types of surgical splints with equal fitting on the dental arches. The linear measurements presented some discrepancies between the actual surgical outcomes and the predicted results from the 3D virtual simulation, but caution must be taken in the analysis of these results due to several variables. CONCLUSIONS: The reported case confirms the clinical feasibility of the described computer-assisted orthognathic surgical protocol. Further progress in the development of technologies for 3D image acquisition and improvements on software programs to simulate postoperative changes on soft tissue are required.

3D virtual planning in orthognathic surgery and CAD/CAM surgical splints generation in one patient with craniofacial microsomia: a case report

Objective: In this case report, the feasibility and precision of tridimensional (3D) virtual planning in one patient with craniofacial microsomia is tested using Nemoceph 3D-OS software (Software Nemotec SL, Madrid, Spain) to predict postoperative outcomes on hard tissue and produce CAD/CAM (Computer Aided Design/Computer Aided Manufacturing) surgical splints. Methods: The clinical protocol consists of 3D data acquisition of the craniofacial complex by cone-beam computed tomography (CBCT) and surface scanning of the plaster dental casts. The ''virtual patient'' created underwent virtual surgery and a simulation of postoperative results on hard tissues. Surgical splints were manufactured using CAD/CAM technology in order to transfer the virtual surgical plan to the operating room. Intraoperatively, both CAD/CAM and conventional surgical splints are comparable. A second set of 3D images was obtained after surgery to acquire linear measurements and compare them with measurements obtained when predicting postoperative results virtually. Results: It was found a high similarity between both types of surgical splints with equal fitting on the dental arches. The linear measurements presented some discrepancies between the actual surgical outcomes and the predicted results from the 3D virtual simulation, but caution must be taken in the analysis of these results due to several variables. Conclusions: The reported case confirms the clinical feasibility of the described computer-assisted orthognathic surgical protocol. Further progress in the development of technologies for 3D image acquisition and improvements on software programs to simulate postoperative changes on soft tissue are required.

Objetivo: neste relato de caso, de um paciente com microssomia craniofacial, testou-se a viabilidade e a precisão do planejamento virtual tridimensional (3D) utilizando o software Nemoceph 3D-OS (Software Nemotec SL, Madri, Espanha) para prever os resultados pós-operatórios em tecidos duros e produzir splints cirúrgicos CAD/CAM. Métodos: o protocolo clínico usado consistiu na aquisição de dados 3D do complexo craniofacial por meio de tomografia computadorizada de feixe cônico (TCFC) e digitalização dos modelos de gesso. O "paciente" criado virtualmente foi submetido à cirurgia virtual e obteve-se uma simulação dos resultados pós-operatórios nos tecidos duros. Os splintscirúrgicos foram confeccionados usando-se a tecnologia CAD/CAM, e permitiram que o planejamento cirúrgico virtual fosse transferido para a sala cirúrgica. No transoperatório, tanto os splints CAD/CAM quanto os splints cirúrgicos convencionais mostraram-se similares. Um segundo conjunto de imagens 3D foi obtido após a cirurgia, para que medidas lineares pudessem ser obtidas e comparadas com as medidas feitas na simulação virtual dos resultados pós-operatórios. Resultados: foi encontrada uma forte semelhança entre os dois tipos desplints cirúrgicos, que se adaptaram da mesma forma nas arcadas dentárias. As medidas lineares mostraram algumas discrepâncias entre os resultados cirúrgicos reais e os resultados previstos na simulação virtual 3D; porém, a análise desses resultados requer cautela, em virtude da presença de inúmeras variáveis que podem neles interferir. Conclusões: o caso relatado no presente estudo confirma a viabilidade clínica do protocolo descrito de cirúrgica ortognática assistida por computador. Porém, ainda se faz necessária uma maior evolução no desenvolvimento de tecnologias para a aquisição de imagens 3D e nos softwares que simulam as alterações pós-operatórias nos tecidos moles.

Evaluation of an anorganic bovine-derived mineral with P-15 hydrogel bone graft: preliminary study in a rabbit cranial bone model.

OBJECTIVES: The present investigation aimed to assess the bone-regenerative potential of two formulations of anorganic bovine-derived mineral bound to a P-15 (ABM/P-15) bone graft - the particulate and the hydrogel forms - in a delayed healing rabbit cranial defect model. MATERIAL AND METHODS: Ten adult male New Zealand White rabbits were used to create two 8 mm transcortical cranial defects per rabbit and each one received randomly the test material (ABM/P-15 carboxymethyl cellulose (CMC)-hydrogel graft), the standard control material (ABM/P-15 particulate graft) or remained empty as a negative control. The defects were allowed to heal for 2 and 4 weeks. Qualitative and quantitative histological outcomes were assessed on undecalcified sections. RESULTS: In the defects grafted with the test material, at both time points, there was a marked random migration of the bone substitute particles. As a consequence, the space maintenance provision was lost and new bone formation was reduced compared with the control particulate graft material. The histomorphometric analysis showed that the control material attained better results, with an average of 13.8 ± 1.9% and 18.2 ± 4.4% of new bone at 2 and 4 weeks, compared with 8.5 ± 2.4% and 13 ± 2.9% for the test material. These differences were significant at 2 weeks (P ≤ 0.05), but not at 4 weeks (P>0.05). Additionally, there was a significant difference in the total area of mineralized tissue (new bone plus particles), favoring the standard control over the test material: 43.2 ± 14.4% vs. 14.2 ± 5.3% at 2 weeks and 56.9 ± 4.2% vs. 24.2 ± 9.6% at 4 weeks, respectively. CONCLUSIONS: The test ABM/P-15 CMC-hydrogel graft material behaved in this animal model by migration of the graft particles, what determined an unpredictable osseoconduction and, consequently, a decreased quality and quantity of bone regeneration as compared with the osseopromotive behavior exhibited by the standard particulate form of the ABM/P-15 control graft. It is therefore suggested to restrain the application of the hydrogel graft form in non-contained anatomical bone defects.

[Surgical management of a dissecting aneurysm of the thoracic aorta in a patient with Marfan's syndrome - case report]. / Tratamento cirúrgico de um aneurisma dissecante da aorta torácica numa doente com síndrome de marfan - Caso clínico.

Marfan's Syndrome is a rare connective tissue disease whose surgical treatment presents several challenges. The existing criteria and recommendations on how and when to surgically approach this disease is almost exclusively confined to the root and ascending aorta, in contrast to the variability of indications on the involvement of the remaining segments of the thoracic and abdominal aorta, due to the multiplicity, morphology and topography of these involvements. This case report is related to a 39 year old woman with Marfan's syndrome, with a type B chronic dissection and a segmentar aneurysm of the proximal descending thoracic aorta with 6,1cm of maximum diameter. The clinical and imagiologic evaluation pointed out the absence of any disorder of the heart, root or ascending aorta. The patient underwent surgical management of the dissecting aneurysm through a transthoracic approach to the aneurysm, with fenestration of the distal aorta and exclusion of the aneurism and intercostal arteries. The efficacy and result of the procedure in addition to the rarity of the vascular pathology presented by the patient, even in the context of a Marfan, completely justify the presentation and disclosure of this clinical case.