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1.
Ann R Coll Surg Engl ; 102(1): e7-e11, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31530171

RESUMO

Ehlers-Danlos syndrome is a hereditary connective tissue disorder that has gastrointestinal manifestations in over 50% of its cases. We present the first case of bariatric surgery in a patient with Ehlers-Danlos syndrome and outline management challenges in the context of the relevant literature. A 56-year-old man with type IV Ehlers-Danlos syndrome and a body mass index of 41.8 kg/m2 was referred to the bariatric centre of the Churchill Hospital, Oxford, for consideration of surgery for morbid obesity. His comorbidity included type 2 diabetes, hypertension, dyslipidaemia and obstructive sleep apnoea. He underwent a laparoscopic Roux-en-Y gastric bypass. His initial recovery was uneventful and he was discharged on the first postoperative day. Six weeks later, he presented with 43.9% excess weight loss and improved glycaemic control. Three months postoperatively, however, he complained of dysphagia, regurgitation and postprandial pain. A barium meal and gastroscopy suggested the presence of a gastric diverticulum. A surgical exploration was planned. Intraoperative gastroscopy demonstrated an asymmetrical gastric pouch dilatation and the pouch was therefore refashioned laparoscopically. Despite the initial symptomatic relief, two months later he experienced retrosternal pain with progressive dysphagia. Since then, multiple endoscopic dilatations of the gastro-oesophageal junction have been performed for recurrence of symptoms. Finally, a laparoscopic hiatus hernia repair and adhesiolysis was performed resulting in complete relief of patient's symptoms. Bariatric management of patients with Ehlers-Danlos syndrome can prove challenging. The bariatric team must implement a careful management plan including a detailed consent process, a tailored surgical intervention and a follow-up focused on potential gastrointestinal manifestations.


Assuntos
Síndrome de Ehlers-Danlos/complicações , Obesidade Mórbida/cirurgia , Transtornos de Deglutição/etiologia , Diabetes Mellitus Tipo 2/complicações , Endoscopia do Sistema Digestório/métodos , Derivação Gástrica/métodos , Hérnia Hiatal/diagnóstico , Hérnia Hiatal/etiologia , Hérnia Hiatal/cirurgia , Herniorrafia/métodos , Humanos , Laparoscopia/métodos , Masculino , Pessoa de Meia-Idade , Obesidade Mórbida/complicações , Dor Pós-Operatória/etiologia , Complicações Pós-Operatórias/diagnóstico , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/cirurgia , Reoperação
2.
World Neurosurg ; 133: e473-e478, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31526884

RESUMO

BACKGROUND: Ehlers-Danlos syndrome (EDS) is a group of rare congenital disorders of connective tissue that result in tissue fragility and joint hyperextensibility. Owing to its rarity, outcomes of pediatric spine surgery in patients with EDS are poorly characterized. Although it has been suggested that complication rates are high, few studies have characterized these complications. METHODS: Pediatric National Surgery Quality Improvement Program data from 2012-2016 were analyzed. Patients with EDS undergoing spine surgery were identified along with patients without EDS undergoing the same surgeries using International Classification of Diseases, Ninth Revision and Current Procedural Terminology codes. RESULTS: Of 369,176 total patients, 279 were determined to have EDS. Of these, 56 patients underwent spine surgery; 46% were male and 54% were female (P = 0.108). Mean age at surgery was 11.59 years (P = 0.888) with a range of 1.77-17.33 years. The most common procedure was arthrodesis (n = 37). There were no differences in unplanned reoperations (n = 4, P = 0.119), wound infections or disruptions (n = 2, P = 0.670), or overall complications (n = 25, P = 0.751). Blood transfusions were required in 41% of patients with EDS, but this was not significant compared with patients without EDS undergoing the same procedures (n = 23, P = 0.580). The total amount of blood transfused (P = 0.508), length of hospital stay (P = 0.396), and total operative time (P = 0.357) were not different from control subjects. CONCLUSIONS: Pediatric patients with EDS do not appear to be at a higher risk of bleeding or other complications during spine surgery as reported in past case series. This is the largest retrospective review of its kind that has been performed in this patient population.


Assuntos
Síndrome de Ehlers-Danlos/cirurgia , Complicações Pós-Operatórias/epidemiologia , Fusão Vertebral , Adolescente , Perda Sanguínea Cirúrgica , Transfusão de Sangue , Criança , Pré-Escolar , Bases de Dados Factuais , Síndrome de Ehlers-Danlos/complicações , Feminino , Transtornos Hemorrágicos/genética , Humanos , Lactente , Tempo de Internação/estatística & dados numéricos , Masculino , Duração da Cirurgia , Complicações Pós-Operatórias/genética , Hemorragia Pós-Operatória/epidemiologia , Hemorragia Pós-Operatória/genética , Estudos Retrospectivos , Estados Unidos/epidemiologia
3.
Pediatr Clin North Am ; 67(1): 23-43, 2020 02.
Artigo em Inglês | MEDLINE | ID: mdl-31779835

RESUMO

A wide of array of patients with genetic and metabolic conditions present with orthopedic manifestations. This article discusses the most common conditions seen in a typical pediatric orthopedic practice. A few pearls are highlighted for each condition to alert practitioners to some of the pitfalls encountered when treating these often highly challenged children.


Assuntos
Doenças Musculoesqueléticas/diagnóstico , Doenças Musculoesqueléticas/etiologia , Acondroplasia/complicações , Criança , Síndrome de Down/complicações , Síndrome de Ehlers-Danlos/complicações , Doenças Genéticas Inatas/complicações , Humanos , Síndrome de Marfan/complicações , Doenças Metabólicas/complicações , Mucopolissacaridoses/complicações , Distrofia Muscular de Duchenne/complicações , Osteocondrodisplasias/complicações , Osteogênese Imperfeita/complicações , Fenótipo , Raquitismo/complicações
4.
Am Surg ; 85(10): 1162-1165, 2019 Oct 01.
Artigo em Inglês | MEDLINE | ID: mdl-31657316

RESUMO

The risk factors and associated conditions of median arcuate ligament syndrome (MALS) have not been well characterized in the literature. In this study, we aim to investigate the presentation and outcomes of MALS patients with an emphasis on the prevalence of other uncommon disorders. To this end, data of patients with MALS who underwent surgery between 2013 and 2018 were collected and compiled into a retrospective database and analyzed. Eleven patients were identified. Seven of these eleven patients underwent diagnostics to evaluate gastric emptying. Five of these seven patients (71.4%) had radiographic evidence of delayed gastric emptying. Four of the eleven patients (36.4%) were found to have anatomic abnormalities of their visceral vasculature. Two of the eleven patients (18.2%) were found to have connective tissue disorders, both with Ehlers-Danlos syndrome. Three of the eleven (27.3%) had a diagnosis of postural orthostatic tachycardia syndrome. This is the first case series reporting on an association between MALS and delayed gastric emptying. We also explored the relationship between MALS and visceral vascular abnormalities, Ehlers-Danlos syndrome, and postural orthostatic tachycardia syndrome. It is notable that these conditions are more prevalent in the MALS population than in the general population, suggesting a possible pathophysiologic relationship.


Assuntos
Síndrome de Ehlers-Danlos/complicações , Esvaziamento Gástrico , Gastroparesia/complicações , Síndrome do Ligamento Arqueado Mediano/complicações , Síndrome do Ligamento Arqueado Mediano/fisiopatologia , Síndrome da Taquicardia Postural Ortostática/complicações , Adulto , Índice de Massa Corporal , Diagnóstico Diferencial , Síndrome de Ehlers-Danlos/diagnóstico , Feminino , Gastroparesia/diagnóstico , Humanos , Tempo de Internação/estatística & dados numéricos , Masculino , Síndrome do Ligamento Arqueado Mediano/diagnóstico , Síndrome do Ligamento Arqueado Mediano/cirurgia , Cuidados Pré-Operatórios , Estudos Retrospectivos , Fatores de Risco , Resultado do Tratamento , Vísceras/irrigação sanguínea
5.
World Neurosurg ; 132: 67-68, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31479794

RESUMO

Ehlers-Danlos syndrome can be associated with cerebrospinal fluid (CSF) disturbances including recurrent CSF leak and Chiari I malformations. Persistent pseudomeningoceles are known to be associated with raised intracranial pressure. We present an unusual case of a compressive epidural CSF collection occurring after a computed tomography-guided L5 nerve root block and describe an effective management strategy.


Assuntos
Vazamento de Líquido Cefalorraquidiano/etiologia , Síndrome de Ehlers-Danlos/complicações , Injeções Epidurais/efeitos adversos , Hipertensão Intracraniana/complicações , Compressão da Medula Espinal/etiologia , Adulto , Malformação de Arnold-Chiari/complicações , Vazamento de Líquido Cefalorraquidiano/diagnóstico por imagem , Vazamento de Líquido Cefalorraquidiano/cirurgia , Feminino , Humanos , Vértebras Lombares , Bloqueio Nervoso , Radiculopatia/tratamento farmacológico , Radiculopatia/etiologia , Compressão da Medula Espinal/diagnóstico por imagem , Compressão da Medula Espinal/cirurgia , Espondilolistese/complicações , Tomografia Computadorizada por Raios X
6.
Congenit Heart Dis ; 14(5): 864-867, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31328377

RESUMO

OBJECTIVES: This study assessed the frequency and utility of echocardiographic examination in patients with all forms of Ehlers-Danlos syndrome and sought to identify clinical variables associated with an abnormal echocardiogram. DESIGN/SETTING: This was a retrospective study of all patients carrying a diagnosis of Ehlers-Danlos syndrome of any type who were evaluated by a pediatrician or pediatric subspecialist at a single tertiary medical center with an affiliated children's hospital during the period January 2013 to December 2018. PATIENTS: Chart review was performed on all patients carrying a diagnosis of Ehlers-Danlos syndrome in the electronic medical record. OUTCOME MEASURES: Data from genetics examination, cardiovascular examination where applicable, genetic test results when available, and echocardiography were recorded. RESULTS: Of 262 patients identified, echocardiography and cardiac evaluation were common occurring in 90% and 50% of patients with any form of Ehlers-Danlos syndrome. Cardiovascular complications occurred in 50% of patients with vascular Ehlers-Danlos syndrome but echocardiography was normal in all. Aortic dilation was common in classic Ehlers-Danlos syndrome but absent in hypermobile Ehlers-Danlos syndrome. Mitral valve prolapse and bicuspid aortic valve occurred at the same incidence as the general population. Cardiac symptoms were present in 12% but did not correlate with abnormal cardiac structure. Presentation with symptoms of musculoskeletal pain was inversely related to the presence of cardiac pathology. CONCLUSIONS: In light of the absence of cardiac pathology in patients with hypermobile Ehlers-Danlos syndrome, routine cardiac evaluation and echocardiography are not required for patients with hypermobile Ehlers-Danlos syndrome.


Assuntos
Ecocardiografia/estatística & dados numéricos , Síndrome de Ehlers-Danlos/diagnóstico , Cardiopatias/diagnóstico , Ventrículos do Coração/diagnóstico por imagem , Criança , Diagnóstico Diferencial , Síndrome de Ehlers-Danlos/complicações , Feminino , Cardiopatias/etiologia , Humanos , Masculino , Reprodutibilidade dos Testes , Estudos Retrospectivos
7.
Haemophilia ; 25(4): 558-566, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31329366

RESUMO

It has long been hypothesized that bleeding symptoms in people with hypermobility occur as a result of abnormalities in the collagen of the vessel wall or the connective tissues. The bleeding symptoms, particularly in the skin, have been attributed to the fragility of skin and blood vessels caused by "defective collagen wickerwork" of the reticular layer of the skin. Collagen, which forms the framework of vessel walls, is altered in many patients with Ehlers-Danlos syndrome (EDS) leading to weakening of the vessel wall or the supporting tissues. Another important function of subendothelial collagen is its interaction with platelets and von Willebrand factor, which results in the propagation of a platelet plug. Thus, abnormalities in subendothelial collagen may alter its interaction with platelets and VWF. More recently, hypermobile-EDS (hEDS) has been associated with mast cell disorders, a condition independently associated with bleeding symptoms. It has also been observed that patients with mild bleeding disorders have a more severe bleeding phenotype when they have co-existing joint hypermobility.


Assuntos
Síndrome de Ehlers-Danlos/classificação , Síndrome de Ehlers-Danlos/complicações , Hemorragia/etiologia , Contusões/etiologia , Síndrome de Ehlers-Danlos/metabolismo , Síndrome de Ehlers-Danlos/patologia , Humanos
9.
Am J Case Rep ; 20: 1057-1062, 2019 Jul 20.
Artigo em Inglês | MEDLINE | ID: mdl-31324749

RESUMO

BACKGROUND Ehlers-Danlos Syndrome (EDS) is a group of connective tissue disorders with heterogeneous clinical features associated with varying genetic mutations. EDS type IV, also known as vascular EDS (vEDS), is the rarest type but has fatal complications, including rupture of major vasculature and intestinal and uterine perforation. Intestinal perforation can be spontaneous or a consequence of long-standing constipation, a common symptom among patients with EDS. CASE REPORT We present a case of a 6-year-old boy with the previous diagnosis of vEDS who presented with colonic perforation from a stercoral ulcer. He underwent diagnostic laparoscopy and loop colostomy, with an uneventful postoperative course. Unfortunately, he developed a second colonic perforation 14 months after the initial episode and underwent total abdominal colectomy with end ileostomy. CONCLUSIONS Intestinal perforation is a well-documented and devastating complication of vEDS. However, spontaneous intestinal perforation is extremely rare in a young child. Therefore, the diagnosis of vEDS should be included in the differential diagnosis if a child presents with intestinal perforation. There is no clear guideline available for surgical management of colonic perforation in patients with vEDS, but total abdominal colectomy appears to provide the best chance of preventing recurrent perforation.


Assuntos
Doenças do Colo/diagnóstico , Doenças do Colo/etiologia , Síndrome de Ehlers-Danlos/complicações , Síndrome de Ehlers-Danlos/diagnóstico , Perfuração Intestinal/diagnóstico , Perfuração Intestinal/etiologia , Criança , Doenças do Colo/cirurgia , Diagnóstico Diferencial , Humanos , Perfuração Intestinal/cirurgia , Masculino
10.
BMC Ophthalmol ; 19(1): 159, 2019 Jul 25.
Artigo em Inglês | MEDLINE | ID: mdl-31345188

RESUMO

BACKGROUND: The Ehlers-Danlos syndromes are a heterogenous group of diseases that cause connective tissue defects. At present, there are no published reports focusing upon the neuro-ophthalmic symptoms that might occur in EDS patients after mild traumatic brain injury. The demographics and clinical course of seven patients with subclinical EDS and mild traumatic brain injury are presented. CASE PRESENTATION: This series describes patients with Ehlers Danlos Syndrome whose diagnosis was discovered in a neuro-ophthalmic clinic for brain injury. Patient demographics and neuro-ophthalmological symptoms are presented. CONCLUSIONS: Patients with subclinical EDS and brain injury may experience a slower, less complete recovery course. Heightened awareness of undiagnosed or underlying Ehlers Danlos Syndrome is important for patients and providers.


Assuntos
Concussão Encefálica/complicações , Síndrome de Ehlers-Danlos/diagnóstico , Recuperação de Função Fisiológica , Transtornos da Visão/etiologia , Acuidade Visual/fisiologia , Adulto , Idoso , Concussão Encefálica/diagnóstico , Síndrome de Ehlers-Danlos/complicações , Feminino , Humanos , Transtornos da Visão/diagnóstico , Transtornos da Visão/fisiopatologia , Adulto Jovem
11.
Phys Ther ; 99(9): 1189-1200, 2019 09 01.
Artigo em Inglês | MEDLINE | ID: mdl-31158283

RESUMO

Hypermobility spectrum disorder (HSD) and hypermobile Ehlers-Danlos syndrome (hEDS) can cause widespread or chronic pain, fatigue, and proprioceptive and coordination deficits resulting in functional restrictions. These conditions are common and often unrecognized, and patients are likely to present in physical therapy for musculoskeletal injuries, pain, or coordination deficits. Although physical therapy is considered central to managing these conditions, many patients report pain and iatrogenic injuries due to inappropriate interventions. The diagnostic classification for these conditions was revised in 2017 to supersede previous diagnostic categories of Joint Hypermobility Syndrome and Ehlers-Danlos Syndrome-hypermobility type/type III. It is now known that these conditions affect multiple body systems and not just joints and that patients require a holistic approach. This Perspective article will describe the 2017 diagnostic classification system, clinical presentation, examination, evaluation, and management of patients with HSD/hEDS. Both adult and pediatric cases are presented to illustrate the patient management concepts discussed. This knowledge can lead to more effective management of this patient population.


Assuntos
Síndrome de Ehlers-Danlos , Instabilidade Articular , Síndrome de Ehlers-Danlos/complicações , Síndrome de Ehlers-Danlos/diagnóstico , Síndrome de Ehlers-Danlos/terapia , Fadiga/etiologia , Fadiga/terapia , Humanos , Instabilidade Articular/complicações , Instabilidade Articular/diagnóstico , Instabilidade Articular/terapia , Mastocitose/complicações , Modalidades de Fisioterapia , Síndrome da Taquicardia Postural Ortostática/complicações , Prognóstico , Avaliação de Sintomas
12.
Dis Colon Rectum ; 62(7): 859-866, 2019 07.
Artigo em Inglês | MEDLINE | ID: mdl-31188187

RESUMO

BACKGROUND: Vascular Ehlers-Danlos syndrome is a rare and severe genetic condition leading to spontaneous, potentially life-threatening arterial and digestive complications. Colonic ruptures are a common feature of the disease, but clear recommendations on their management are lacking. OBJECTIVE: This study aimed to identify surgery-related morbidity and 30-day postoperative mortality after colonic perforation. DESIGN: This was a retrospective review. SETTING: A large cohort of patients with vascular Ehlers-Danlos syndrome was followed in a tertiary referral center. PATIENTS: Between 2000 and 2016, the French National Reference Centre for Rare Vascular Diseases (HEGP, AP-HP, Paris, France) followed 148 patients with molecularly proven vascular Ehlers-Danlos syndrome. MAIN OUTCOME MEASURES: The primary outcomes measured were surgery-related morbidity and 30-day postoperative mortality. RESULTS: Of 133 patients with molecularly proven vascular Ehlers-Danlos syndrome, 30 (22%) had a history of colonic perforation and 15 (50%) were males. These subjects were diagnosed with vascular Ehlers-Danlos syndrome at a younger age than patients with a history of GI events without colonic perforation (p = 0.0007). There were 46 colonic perforations, median 1.0 event per patient (interquartile range, 1.0-2.0). Reperforations occurred in 14 (47%) patients, mostly males. Surgical management consisted of Hartmann procedures or subtotal abdominal colectomies, with a nonnegligible rate of reperforation following partial colonic resection (11 patients, 41%). LIMITATIONS: The main limitations of this work are its retrospective design and that the diagnosis of vascular Ehlers-Danlos syndrome was made after colonic perforations in a majority of patients. CONCLUSION: Colonic perforations seem more severe in males, with a high rate of reperforation after Hartmann procedure. Subtotal colectomy may reduce digestive morbidity, particularly in male patients. Additional studies are required to identify other predictors of reperforation. See Video Abstract at http://links.lww.com/DCR/A937.


Assuntos
Doenças do Colo/etiologia , Doenças do Colo/cirurgia , Síndrome de Ehlers-Danlos/complicações , Perfuração Intestinal/etiologia , Perfuração Intestinal/cirurgia , Complicações Pós-Operatórias/etiologia , Adolescente , Adulto , Criança , Colectomia/efeitos adversos , Colectomia/mortalidade , Síndrome de Ehlers-Danlos/diagnóstico , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Técnicas de Diagnóstico Molecular , Recidiva , Estudos Retrospectivos , Adulto Jovem
13.
J Fr Ophtalmol ; 42(7): 722-729, 2019 Sep.
Artigo em Francês | MEDLINE | ID: mdl-31133401

RESUMO

INTRODUCTION: The goal of this study was to describe and analyze the ophthalmological manifestations found in 21 patients followed for Ehlers-Danlos Syndrome in our department. METHODS: This retrospective study analyzed 21 consecutive patients (17 women and 4 men) with Ehlers-Danlos syndrome seen in the Necker hospital, Paris, between April 2016 and November 2017. The mean age was 25.95 years (12-47). A complete evaluation was performed searching for symptoms, orthoptic evaluation and complete ophthalmologic examination with slit lamp examination of the anterior segment, pachymetry and fundus examination with fundus photography and OCT. RESULTS: Nineteen patients presented ophthalmological signs (90.5%). The most frequent ophthalmological signs were: ocular motility disorders in 15 patients (71.4%), with convergence insufficiency in 13 of them, blue sclera in 8 patients (38%) and dry eye syndrome in 7 patients (33%, with 2 patients with reduced Break-Up Time<10seconds and 5 with very reduced Break-Up Time<5seconds). Mean pachymetry was 539.25µm (365-612). One patient presented with bilateral keratoglobus (4.8%). High myopia was present in 2 patients (9.5%) and associated with retinal tears in one patient (4.8%). No patients presented with angioid streaks. DISCUSSION: In this study, the main ophthalmological sign was convergence insufficiency present in more than 60% of the patients. This highlights the importance of an orthoptic examination in patients with Ehlers-Danlos syndrome. Dry eye syndrome with tear film instability was frequent, even though the patients were young. Blue sclera was seen in 38% of the patients. We reported two patients with high myopia and one patient with keratoglobus in our cohort. No patients presented with angioid streaks, and mean pachymetry was normal in our series. CONCLUSION: An ophthalmological and orthoptic evaluation should be performed in all patients with Ehlers-Danlos syndrome to detect and treat ocular manifestations. If Ehlers-Danlos syndrome is suspected, ophthalmological examination can also provide support for the diagnosis.


Assuntos
Síndrome de Ehlers-Danlos/epidemiologia , Oftalmopatias/epidemiologia , Adolescente , Adulto , Criança , Estudos de Coortes , Síndrome de Ehlers-Danlos/complicações , Oftalmopatias/etiologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Paris/epidemiologia , Estudos Retrospectivos , Adulto Jovem
16.
Dermatol Online J ; 25(3)2019 Mar 15.
Artigo em Inglês | MEDLINE | ID: mdl-30982307

RESUMO

Elastosis perforans serpiginosa (EPS) is a rare skin disease with elimination of connective tissue fibers from dermis to epidermis. The typical presentation shows hyperkeratotic red or skin-colored papules arranged in a circinate pattern. We present a 26-year-old woman with EPS known to have vascular Ehlers-Danlos syndrome.


Assuntos
Síndrome de Ehlers-Danlos/complicações , Dermatopatias/diagnóstico , Adulto , Feminino , Humanos , Dermatopatias/complicações , Dermatopatias/patologia
17.
Am J Case Rep ; 20: 233-237, 2019 Feb 22.
Artigo em Inglês | MEDLINE | ID: mdl-30792378

RESUMO

BACKGROUND Vascular type of Ehlers-Danlos syndrome (vEDS) is a rare connective tissue disorder associated with a high prevalence rate of aortic dissection (AD). The coexistence of a pregnancy raises these rates and the diagnostic complexity of the situation. In this article, we present a different initial diagnostic approach to an acute aortic syndrome. CASE REPORT A young pregnant woman (29th week gestation) with vEDS was admitted to our clinic due to sudden tearing back pain radiating to the left arm. Four years ago, the same patient underwent a surgical aortic valve reconstruction and replace of the ascending and proximal arch of the aorta because of an acute Standford A AD. The clinical, laboratory as well as transthoracic echocardiographic findings did not reveal any objective signs of an acute aortic syndrome. Due to the relative contraindications against computed tomography imaging due to pregnancy, we conducted a transesophageal echocardiography which revealed acute progress of pre-existing AD. A follow-up computed tomography could verify our findings,showing a Standford B dissection, which was treated conservatively. After 2 weeks, due to a distal progression of dissection, our patient underwent a cesarean section. In absence of new clinical findings, the young patient was discharged the following week. CONCLUSIONS Patients with vEDS are at high risk of an AD and other life-threatening complications, especially during pregnancy. According to the guidelines of European Society of Cardiology (ESC), vEDS-patients should be thoroughly screened. In the case of pregnancy, physicians should consider frequent follow-up examinations and be prepared for diagnosis and treatment of the potential complications.


Assuntos
Aneurisma Dissecante/diagnóstico por imagem , Aneurisma Aórtico/diagnóstico por imagem , Síndrome de Ehlers-Danlos/complicações , Complicações Cardiovasculares na Gravidez/diagnóstico por imagem , Adulto , Cesárea , Angiografia por Tomografia Computadorizada , Ecocardiografia Transesofagiana , Feminino , Humanos , Gravidez
18.
Vasc Endovascular Surg ; 53(4): 343-347, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30630391

RESUMO

Ehlers-Danlos syndrome (EDS) refers to a group of genetic disorders involving the connective tissues. Type IV EDS impairs type III collagen that is responsible for vessel integrity. Patients with type IV EDS are susceptible to vascular and visceral complications, including aortic aneurysms, pseudoaneurysms, dissections, and spontaneous rupture of internal organs. Treating aneurysms with open surgery versus endovascular techniques each carry a unique risk-to-benefit ratio that must be applied to each individual carefully. We present a patient with type IV EDS who presented with a rapidly growing inferior mesenteric artery aneurysm. The patient was treated with a percutaneous endovascular technique using coils and n-butyl-cyanoacrylate glue.


Assuntos
Aneurisma/terapia , Síndrome de Ehlers-Danlos/complicações , Embolização Terapêutica/métodos , Embucrilato/administração & dosagem , Artéria Mesentérica Inferior , Adulto , Aneurisma/diagnóstico por imagem , Aneurisma/etiologia , Angiografia por Tomografia Computadorizada , Síndrome de Ehlers-Danlos/diagnóstico , Embolização Terapêutica/instrumentação , Humanos , Masculino , Artéria Mesentérica Inferior/diagnóstico por imagem , Resultado do Tratamento , Ultrassonografia de Intervenção
19.
Am J Case Rep ; 20: 39-42, 2019 Jan 10.
Artigo em Inglês | MEDLINE | ID: mdl-30626862

RESUMO

BACKGROUND Ehlers-Danlos syndrome, hypermobility type is characterized by increased extensibility, permeability, and fragility of the affected cartilaginous tissues, including the trachea, larynx, and skin. Anesthetic considerations for patients with this syndrome include intubation difficulties secondary to the collapse of fibro-elastic tissues in the trachea and a reported resistance to local anesthetics. CASE REPORT Our patient was a 22-year-old G4P0030 woman with a history of morbid obesity, seizures, Barrett's esophagus, hypermobility being evaluated for Ehlers-Danlos syndrome, and anaphylaxis to an unknown local anesthetic who was scheduled for cesarean delivery. She refused allergy testing. After rapid-sequence induction of general anesthesia, video laryngoscopy facilitated endotracheal intubation. Delivery and recovery were uneventful for the mother and child. CONCLUSIONS No guidelines for neuraxial or general anesthesia exist for patients with Ehlers-Danlos syndrome, hypermobility type. Increased rates of cervical spine instability and local anesthetic resistance have been reported in this population and should be considered when developing the anesthetic plan.


Assuntos
Anestesia Geral , Anestésicos Locais/efeitos adversos , Cesárea , Síndrome de Ehlers-Danlos/complicações , Anafilaxia/induzido quimicamente , Contraindicações de Medicamentos , Feminino , Humanos , Intubação Intratraqueal , Laringoscopia , Gravidez , Gravação em Vídeo , Adulto Jovem
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