The challenge of simultaneous major aortic surgery and pectus excavatum repair in a patient with Ehlers ‐Danlos type IV syndrome

AbstractA unique case of a patient with Ehlers ‐Danlos type IV syndrome was referred with aortic root aneurysm and previous cosmetic primary repair of pectus excavatum (PE) through a solid silicone onlay prosthesis. The patient underwent successful removal of the silicone prosthesis and one‐stage ascending aortic root replacement with concom itant Nuss procedure for repair of the PE.
Source: Journal of Cardiac Surgery - Category: Cardiovascular & Thoracic Surgery Authors: Tags: CASE REPORT Source Type: research

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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 ...
Source: Annals of the Royal College of Surgeons of England - Category: Surgery Authors: Tags: Ann R Coll Surg Engl Source Type: research
Ehlers-Danlos Syndromes (EDS) are a group of heritable connective tissue disorders with distinct genetic etiologies. Of the 13 currently recognized types of EDS, the vascular type (vEDS), is generally considered the most severe and is associated with a decreased life expectancy due to spontaneous arterial, intestinal, and or uterine rupture. Diagnosis of vascular EDS is supported by genetic testing confirming the presence of pathogenic variations in COL3A1, a type III procollagen gene. Management of vEDS is usually conservative with control of hemodynamic stress, frequent cardiovascular imaging, and, if indicated, a though...
Source: Annals of Vascular Surgery - Category: Surgery Authors: Tags: Case Report Source Type: research
​BY JENNIFER TUONG; IVAN KHARCHENKO; JEAN LUC AGARD; &AHMED RAZIUDDIN, MDA 65-year-old man who had HIV well-controlled with highly active antiretroviral therapy, hypertension, sciatica, and restless leg syndrome presented to the emergency department with left leg pain. He also had had chemotherapy and radiation for anal cancer. The patient said the pain had started 45 minutes earlier when he was sitting on the toilet.He described the pain as sore in quality and 10/10 on the pain scale. He reported that it had started in his lower back and radiated to his left leg. He said he had had no trauma or weakness to the regio...
Source: The Case Files - Category: Emergency Medicine Tags: Blog Posts Source Type: research
Despite improvements in prevention and management, aortic aneurysm repair remains a high-risk operation for patients with Marfan syndrome (MFS) and Ehlers-Danlos syndrome (EDS). The goal of this study was to examine differences in characteristics and outcomes of patients with MFS or EDS undergoing aortic aneurysm repair at teaching versus nonteaching hospitals.
Source: Annals of Vascular Surgery - Category: Surgery Authors: Tags: Clinical Research Source Type: research
Despite improvements in prevention and management, aortic aneurysm repair remains a high risk operation for patients with Marfan syndrome (MFS) and Ehlers-Danlos syndrome (EDS). The goal of this study was to examine differences in characteristics and outcomes of patients with MFS or EDS undergoing aortic aneurysm repair at teaching versus non-teaching hospitals.
Source: Annals of Vascular Surgery - Category: Surgery Authors: Source Type: research
Publication date: Available online 18 June 2018Source: Arthroscopy TechniquesAuthor(s): Jillian Karpyshyn, Erin E. Gordey, Catherine M. Coady, Ivan H. WongAbstractPosterior shoulder instability is an uncommon and challenging cause of shoulder pain and dysfunction. Surgical management has less reliable results and higher failure rates compared with techniques for anterior shoulder instability. The presence of generalized ligamentous laxity further complicates options for surgical management. If primary capsulolabral repair fails, controversy exists as to the optimal revision procedure. This technical description and video p...
Source: Arthroscopy Techniques - Category: Surgery Source Type: research
We present an all-arthroscopic technique involving extra-articular anterior and posterior glenoid bone grafting to augment a capsular repair in a patient with Ehlers-Danlos syndrome and recurrent multidirectional shoulder instability. Graft options include either distal tibial allograft or iliac crest autograft. Anterior graft placement uses a dilated far medial portal using an inside-out technique. The posterior graft is placed through a dilated posterior portal. A 1-mm edge of anterior and posterior glenoid rim is denuded of cartilage for later capsular repair, and grafts are secured flush to the osseous surface. A capsu...
Source: Arthroscopy Techniques - Category: Surgery Source Type: research
Publication date: Available online 18 June 2018Source: Arthroscopy TechniquesAuthor(s): Jillian Karpyshyn, Erin E. Gordey, Catherine M. Coady, Ivan H. WongAbstractPosterior shoulder instability is an uncommon and challenging cause of shoulder pain and dysfunction. Surgical management has less reliable results and higher failure rates compared with techniques for anterior shoulder instability. The presence of generalized ligamentous laxity further complicates options for surgical management. If primary capsulolabral repair fails, controversy exists as to the optimal revision procedure. This technical description and video p...
Source: Arthroscopy Techniques - Category: Surgery Source Type: research
We present an all-arthroscopic technique involving extra-articular anterior and posterior glenoid bone grafting to augment a capsular repair in a patient with Ehlers-Danlos syndrome and recurrent multidirectional shoulder instability. Graft options include either distal tibial allograft or iliac crest autograft. Anterior graft placement uses a dilated far medial portal using an inside-out technique. The posterior graft is placed through a dilated posterior portal. A 1-mm edge of anterior and posterior glenoid rim is denuded of cartilage for later capsular repair, and grafts are secured flush to the osseous surface. A capsu...
Source: Arthroscopy Techniques - Category: Surgery Source Type: research
Publication date: Available online 18 June 2018 Source:Arthroscopy Techniques Author(s): Jillian Karpyshyn, Erin E. Gordey, Catherine M. Coady, Ivan H. Wong Posterior shoulder instability is an uncommon and challenging cause of shoulder pain and dysfunction. Surgical management has less reliable results and higher failure rates compared with techniques for anterior shoulder instability. The presence of generalized ligamentous laxity further complicates options for surgical management. If primary capsulolabral repair fails, controversy exists as to the optimal revision procedure. This technical description and video presen...
Source: Arthroscopy Techniques - Category: Surgery Source Type: research
More News: Cardiovascular & Thoracic Surgery | Ehlers-Danlos Syndrome | Gastroschisis Repair