Spontaneous coronary artery dissection in Ehlers-Danlos syndrome

Ehlers-Danlos syndrome is a heterogeneous group of connective tissue disorders with type IV, the vascular subtype, behaving as the most severe largely due to spontaneous arterial aneurysm and dissection. In this case report we describe a spontaneous left anterior descending coronary artery dissection treated with coronary artery bypass graft in a patient with Ehlers-Danlos syndrome type IV.     

Emergency repair of type A aortic dissection in type IV Ehlers-Danlos syndrome

Ehlers-Danlos syndrome type IV is a distinctive syndrome in which thin and fragile skin, premature ageing, bruising and scarring are combined with lethal or life-threatening arterial weakness. Aortic rupture either at the aortic root and arch, or sometimes lower down the artery, are particularly characteristic. Even quite minor injury can produce dangerous vascular tearing and damage. Technical difficulties encountered in arterial repair or venous ligature are particularly worrying. The authors report the treatment of a ruptured type A aortic dissection associated with Ehlers-Danlos syndrome where the extreme fragility of the tissues and tendency to bleed posed a difficult task for the surgeon.     

Occlusion of a hepatic arterial aneurysm with gianturco coils in a patient with the Ehlers-Danlos syndrome

Successful transcatheter occlusion of a proper hepatic arterial aneurysm in a patient with type IV Ehlers-Danlos syndrome is reported. This patient also had multiple, small intrahepatic arterial aneurysms, stenosis, and vascular occlusions. A spontaneous colonic perforation brought this patient to medical attention.     

Spontaneous rupture of the spleen in type IV Ehlers-Danlos syndrome: Report of a case

The vascular type of Ehlers-Danlos syndrome, type IV, is associated with severe complications, including arterial rupture and visceral perforation. However, to our knowledge, there has been only one previous report of splenic rupture caused by a spontaneous hemorrhage in type IV Ehlers-Danlos syndrome. We report another case of this uncommon complication, occurring in a 35-year-old woman who presented after the sudden onset of acute abdominal pain. Patients should be stabilized quickly in the intensive care unit and the most timesaving surgical techniques used. Moreover, tissues must be handled with great care intraoperatively in view of their extreme fragility. Despite prompt and appropriate treatment, the prognosis is often dismal.     

A large abdominal intercostal hernia in a patient with vascular type Ehlers-Danlos syndrome: a surgical challenge

A patient with vascular type Ehlers-Danlos syndrome developed a large abdominal intercostal hernia secondary to coughing. The tissue friability and associated risks for arterial ruptures and visceral perforations in these patients make hernia repair challenging. The hernia was successfully treated using a novel approach.     

Anaesthetic implications of vascular type Ehlers-Danlos syndrome

Vascular type Ehlers-Danlos syndrome is an inherited connective tissue disease, which is typified by tissue fragility, joint hypermobility, a tendency to bleed excessively and rupture of the uterus, the bowel and arteries. Two case reports are presented which describe the anaesthetic management of patients with spontaneous bowel perforations due to vascular type Ehlers-Danlos syndrome. Both cases were associated with relatively minor problems. The implications of this condition for anaesthesia include difficulty (and risk of complications) with vascular access, a risk of spontaneous arterial rupture in the perioperative period, possible cervical spine instability and airway trauma on intubation. There is also a risk of excessive bleeding, potentially difficult neuraxial anaesthesia and an increased risk of epidural haematoma. There also may be associated cardiac or pulmonary disease. However, these two cases and the limited previously published literature suggest that such potential complications are actually rare and that anaesthesia may be conducted in a relatively standard manner in most circumstances.     

Successful management of popliteal arterial disruption in Ehlers-Danlos syndrome.

A review of the literature on Ehlers-Danlos syndrome with particular attention to vascular complications enabled us to outline an orderly diagnostic and therapeutic plan which has allowed salvage of an individual with a popliteal false aneurysm. In patients with Ehlers-Danlos syndrome and brachial, superficial femoral, or popliteal arterial disruption, the use of proximal tourniquet control to allow ligation of the disrupted artery under a no-flow, no-pressure situation is recommended. The ligations should be carried out with a large ligature or possibly with large hemoclips and/or reinforced with multiple fine atraumatic vascular sutures distally. These patients may be treated successfully for flexion contractures because of the laxness of their tissues.     

Difficult intubation during rapid sequence induction in a parturient with Ehlers-Danlos syndrome, hypermobility type

There have been several reports of resistance to local anaesthetic agents in women with Ehlers-Danlos syndrome, hypermobility type, also known as Ehlers-Danlos syndrome Type III. General anaesthesia with rapid sequence induction was performed for caesarean section due to prolonged second stage of labour, but intubation proved to be difficult. We propose that intubation difficulty probably arose from collapse of fibro-elastic tissues and adjoining C-shaped cartilages of the trachea with appropriately applied cricoid pressure. We found no other case reports of difficult intubation in patients with Ehlers-Danlos syndrome, hypermobility type. There are reports of cervical spine instability and temporomandibular joint dysfunction in patients with this syndrome suggesting a potential for difficult airway management. Additional anaesthetic problems associated with Ehlers-Danlos syndrome involve patient positioning and vascular access.     

Endovascular repair of multiple infrageniculate aneurysms in a patient with vascular type Ehlers-Danlos syndrome

Patients with vascular type Ehler-Danlos syndrome can develop aneurysms in unusual locations. We describe the case of a 33-year-old woman with vascular type Ehlers-Danlos syndrome who developed metachronous tibial artery aneurysms that were sequentially treated with endovascular means.     

Lower Limb Arterio-venous Fistula as a Late Complication of Phlebectomy in a Patient with Ehlers-Danlos Type IV

Ehlers-Danlos syndrome type IV (EDS type IV), the vascular type, results from mutations in the gene for type III procollagen (COL3A1). Affected patients are at risk for arterial, bowel, and uterine rupture. The timing, frequency and course of these events are unpredictable. We report a 50-year-old patient with previous complications of EDS type IV who presented with recurrent varicose veins that subsequent imaging identified as an arteriovenous fistula (AVF) at the site of previous phlebectomy.Patients with EDS type IV present vascular surgeons manifold management problems. A pre-existing diagnosis of EDS type IV should alert the clinician to the risk of unusual presentations, both acutely and as complications subsequent to intervention. Once identified, appropriate investigation and follow-up of these patients by a vascular surgeon is mandated.     

Type IV Ehlers-Danlos syndrome with isolated arterial involvement

Ehlers-Danlos syndrome is a connective tissue disorder characterized by skin hyperelasticity and friability, hyperextensible small articulations, vascular fragility, and ocular and visceral symptoms. Ten types of Ehlers-Danlos syndrome have been described to date. Type IV is characterized by isolated arterial involvement with the risk of rupture, making diagnosis difficult. A 25-year-old man was operated on for a ruptured left and then right iliac artery and once again for a hepatic artery aneurysm within a nine month period. The diagnosis of Ehlers-Danlos syndrome was based on clinical and histopathologic findings. An asymptomatic iliac artery aneurysm was found in the patient's 23-year-old brother.Presented at the Annual Meeting of the Societé de Chirurgie Vasculaire de Langue Française, La Grande Motte, France, May 20–21, 1988.     

Varying presentations in patients with symptomatic type IV vascular Ehlers-Danlos syndrome

Ehlers-Danlos syndrome (EDS) represents a group of inheritable connective tissue disorders. Patients with type IV or vascular EDS, autosomal dominant pattern of inheritance, may present with aneurysm formation or arterial dissection. Due to vessel fragility, operative therapy for such disorders has been reserved for compelling indications in which benefit clearly warrants risk, yet assessment of risk is largely clinical with operative decisions guided by factors such as response to previous operations and age at onset of index vascular complications. We present 2 patients with differences in their clinical presentations and outcomes and review the literature with emphasis on operative decision making.     

The Ehlers-Danlos specter revisited

Ehlers-Danlos type IV is a major concern to vascular surgeons because it is often associated with spontaneous hemorrhage from arteries containing decreased type III collagen. Five members of a family with Ehlers-Danlos type IV and a review of another family of five with Ehlers-Danlos type IV are reported. Evaluation of the recent family included clinical evaluation as well as assay of collagen production. The age range of the three involved females and two males was 7 to 52 years. The father of the affected family had a spontaneous colon perforation at age 39. His son, at age 27, had a spontaneous rupture of the iliac artery. Revascularization was accomplished with difficulty. His daughter had a large cerebral bleed. Two granddaughters, ages 7, have not had any bleeding or aneurysmal events. The amount of type III collagen was only 10% of normal in the patient with the iliac artery rupture. The three females all exhibited similarly low levels of type III collagen. The father's type III collagen level was not sufficiently low to confirm Ehlers-Danlos type IV, although he had a spontaneous colon perforation. In the other Ehlers-Danlos type IV family of five, the three surviving members had type III collagen levels as low as 5% of normal. Two family members died after spontaneous iliac rupture at ages 24 and 33. Both families exhibited an autosomal dominant inheritance pattern. Ehlers-Danlos type IV remains a challenging problem for vascular surgeons. It is transmitted as an autosomal dominant inheritance with a high degree of penetrance. Spontaneous arterial and intestinal perforations should alert the clinician to the possibility of Ehlers-Danlos type IV. Patients should be evaluated noninvasively. Arterial repairs may not be successful in these patients because the vessels are extremely friable. Assays of collagen production are advisable in establishing the diagnosis.     

Successful Coil Embolization for Spontaneous Arterial Rupture in Association with Ehlers-Danlos Syndrome Type IV: Report of a Case

When a patient with Ehlers-Danlos syndrome (EDS) presents with a vascular emergency, performing life-saving surgery can be difficult because of the profound fragility of the arterial tissue. We report the case of a 27-year-old woman with EDS in whom a spontaneous arterial rupture was successfully treated with transcatheter embolization. The patient was brought to our hospital in shock, with left lower abdominal pain. She had been diagnosed with EDS type IV following a colonic rupture 8 years earlier. An emergency angiogram revealed rupture of the left external iliac artery. The active bleeding was managed by transarterial embolization of the ruptured artery using stainless steel coils, which took 30min to achieve. The patient has not suffered any further vascular complications during the year since this procedure. Transcatheter coil embolization may be a reliable option for treating sudden arterial rupture in patients with this syndrome.     

Marfan and other connective tissue disorders: conservative and surgical considerations

Several inheritable disorders of connective tissue have clinical manifestations in the cardiovascular system. Of these, the most significant disorders are Marfan syndrome and Ehlers-Danlos syndrome type IV. The cardiovascular complication typically seen in Marfan syndrome is aneurysmal dilatation of the aortic root, which begins in the first or second decade of life and leads to premature death caused by aortic rupture, dissection, or cardiac complications of aortic regurgitation. However, the remaining aorta and its branches are vulnerable to subsequent aneurysmal degeneration, especially when involved with chronic dissection. Ehlers-Danlos syndrome type IV is characterized by extremely friable arteries that are prone to aneurysm and rupture and are uncommonly difficult to repair. The authors review the literature on these disorders and document their personal experience with their vascular complications, with emphasis on their experience with the aortic tailoring procedure in dissection of the descending thoracic aorta.     

Survival of an aortic trauma patient with Ehlers-Danlos syndrome type IV: a case report

Ehlers-Danlos syndrome type IV is the most lethal variant of that illness and is associated with fatal large vessel arterial hemorrhages. The literature reports only two survivors of elective aortic surgery and two survivors of spontaneous aortic hemorrhage. This article presents a 14-year-old boy who had aortic and vena cava blunt trauma and survived.     

Ruptured Dissecting Aneurysm in Bilateral Iliac Arteries Caused by Ehlers-Danlos Syndrome Type IV: Report of a Case

Ehlers-Danlos syndrome (EDS) is an inherited disorder of connective tissue characterized by hyperextensible skin, hypermobile joints, and ab-normalities of the cardiovascular system. Ten types and several subtypes of EDS have so far been recognized based on genetic, clinical, and biochemical characteristics. The spectrum of the disorder varies from mild to life-threatening vascular complications. EDS type IV is a particularly dangerous form with a lethal spontaneous rupture of the major arteries and aneurysmal formation. We present herein a case of a ruptured dissecting aneurysm in the bilateral iliac arteries caused by EDS type IV. A previously healthy 33-year-old man without any physical features of this connective tissue disorder experienced a metachronous vascular rupture two times. Successful synthetic bypass grafting was performed with great difficulty. The diagnosis of EDS type IV was made afterwards based on an electrophoresis analysis of a skin biopsy specimen which revealed a lack of type III collagen. Surgical intervention in cases of arterial complications in EDS type IV patients have been reported to be both difficult and frequently unsuccessful. The early clinical recognition of this syndrome is therefore of great importance due to the hazards of such surgical therapies. Received: November 16, 1999 / Accepted: July 25, 2000     

Rupture of the Abdominal Aorta in Patients with Ehlers-Danlos Syndrome

Ehlers-Danlos syndrome (EDS) is a heterogeneous inherited disorder of collagen synthesis. Type IV is frequently associated with major vascular catastrophes and challenges the vascular surgeon with its varied clinical presentation and the difficulty of vascular repair. Rupture of the abdominal aorta is one of the most serious complications and is associated with nearly 100% mortality rate. We describe here three patients with type IV EDS.     

Endovascular abdominal aortic aneurysm repair in a patient with Ehlers-Danlos syndrome

Ehlers-Danlos syndrome is a connective tissue disorder caused by abnormal collagen synthesis. Vascular complications, including aneurysm formation and spontaneous arterial perforations, are difficult to manage surgically and result in significant operative mortality due to blood vessel fragility. We describe the first reported successful endovascular abdominal aortic aneurysm repair in a patient with Ehlers-Danlos syndrome. We discuss the advantages endovascular surgery offers over open surgery in these patients. We believe that endovascular repair of abdominal aortic aneurysms preferentially over open repair merits consideration in patients with Ehlers-Danlos syndrome.     

Uncommon arteriopathies: what the vascular surgeon needs to know

Uncommon arteriopathies encompass a diverse range of diseases, including inherited collagen vascular disorders such as Marfan syndrome, Ehlers-Danlos Type IV, and pseudoxanthoma elasticum; vasculitides, including Takayasu's arteritis, extracranial giant cell arteritis, and Beh?et's disease; neurofibromatosis type 1; intimal fibromuscular dysplasia; unusual bacterial and viral infections; and drug-induced arteriopathies. Patients with uncommon arterial disorders may present to the vascular surgeon with common surgical problems, including intermittent claudication, renovascular hypertension, Raynaud's phenomenon, and aneurysmal disease. However, the disease manifestations, expected course, and outcomes may be much different than more common arterial disorders and this can have important surgical implications. This review centers around several interesting cases and the differential diagnoses that should be considered when encountering an unusual clinical presentation. Reference is made to the literature for diagnostic criteria, clinical pearls, and how to avoid pitfalls in the evaluation and management of patients with unusual arteriopathies.