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.     

Isolated Iliac Artery Aneurysm Caused by Fibromuscular Dysplasia: Report of a Case

Fibromuscular dysplasia (FMD) can develop in many different arteries, but iliac artery aneurysms are rare. A 69-year-old Japanese woman was admitted to our hospital for treatment of a right common iliac artery aneurysm. Aortography revealed aneurysms in both the right common iliac artery and the left internal iliac artery. Notably, the right common iliac artery aneurysm had a string-of-beads appearance. At surgery, the aneurysms were resected, and replaced with Y-shaped vascular prostheses. The histopathological diagnosis was fibromuscular dysplasia (FMD). We report this case of common iliac artery aneurysm caused by FMD due to its rarity.     

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.     

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.     

Bleeding Prolapsed Hemorrhoids as a Presentation of Ruptured Internal Iliac Artery Aneurysm: Report of a Case

Internal iliac artery aneurysms (IIAAs) are rare and their concealed location in the pelvis presents a diagnostic challenge. We report a case of a 79-year-old man who presented 12 years after an abdominal aortic aneurysm repair, with signs of prolapse, bleeding, hemorrhoids, and a deep vein thrombosis. His condition rapidly deteriorated, with the development of acute renal failure and obvious perianal and perineal ecchymoses, within a few days. Abdominal ultrasound and computed tomography showed a ruptured IIAA. His renal function returned to normal after surgical decompression of the aneurysm.     

Endovascular treatment for a spontaneous rupture of the posterior tibial artery in a patient with Ehlers-Danlos syndrome Type IV: Report of a case

This report presents the case of a 27-year-old woman with a history of Ehlers-Danlos syndrome Type IV (EDS-IV) who suffered from acute compartment syndrome caused by the spontaneous rupture of the posterior tibial artery. Bleeding control was achieved by endovascular embolization. A fasciotomy was successfully performed without any trouble with hemorrhaging. Endovascular treatment could therefore represent the ideal option for treating infrapopliteal lesions, particularly for EDS-IV patients.     

Abdominal Compartment Syndrome Causing Respiratory Failure During Surgery for a Ruptured Descending Thoracic Aneurysm: Report of a Case

Elevated intra-abdominal pressure causing widespread organ dysfunction is known as abdominal compartment syndrome (ACS). The subject of our case report is a 64-year-old man who underwent repair of a ruptured descending thoracic aortic aneurysm (TAA) under deep hypothermic circulatory arrest. During the operation, decompression laparotomy was required to relieve intra-abdominal hypertension causing respiratory failure, before the patient could be weaned off cardiopulmonary bypass. We report this case to alert surgeons to the fact that ACS can occur during surgery on the thoracic aorta, especially if massive fluid resuscitation is required and venous drainage for extracorporeal circulation is less than optimal. Early recognition and prompt decompression by laparotomy is essential to save the life of the patient.     

A Ruptured Pancreaticoduodenal Artery Aneurysm Associated with a Splenic Artery Aneurysm: Report of a Case

True pancreaticoduodenal artery (PDA) aneurysms are extremely rare. We report herein a case of a ruptured PDA aneurysm associated with a nonruptured splenic artery aneurysm which was successfully treated by surgery. A 55-year-old man was admitted to a local hospital complaining of sudden abdominal and back pain, and thereafter he was transferred to our university hospital. Abdominal computed tomography revealed retroperitoneal hematoma and an enhanced round spot suggesting a peripancreatic aneurysm. Emergency angiography showed a 20-mm-sized aneurysm in the inferior PDA and a 10-mm-sized aneurysm in the splenic artery. The patient underwent an emergency laparotomy with a diagnosis of a ruptured PDA aneurysm. After evacuating a large volume clot in the right retroperitoneal space and the peritoneal cavity, we detected an index finger-sized aneurysm with arterial bleeding in the right inferioposterior aspect of the pancreas. Hemostasis was obtained by oversewing the aneurysm and a ligation of the feeding arteries. A prophylactic splenectomy was performed for the nonruptured splenic artery aneurysm. This case indicates that emergency angiography is indispensable for both a definitive diagnosis and adequate surgical treatment of PDA aneurysms. Received: July 19, 2000 / Accepted: January 9, 2001     

Management of spontaneous colonic perforation in Ehlers-Danlos syndrome type IV

A 14-year-old girl with a family history of fatal colonic rupture, presented with a 2-day history of abdominal pain and signs of peritonitis. At laparotomy, a full-thickness perforation of the sigmoid colon was found, which was exteriorized as a loop colostomy. Subsequently, molecular studies of the patient’s cultured fibroblasts found a point mutation in the COL3A1 gene, confirming a diagnosis of Ehlers-Danlos syndrome type IV (EDS-IV). Four and a half years later, a total abdominal colectomy and ileoproctostomy were performed, restoring intestinal continuity. At 5 years follow-up, the patient has had no further complications. Although spontaneous colonic perforation is a well-reported manifestation of EDS-IV, a consensus on the surgical management of this complication in EDS-IV has yet to be determined. Given the high rate of reperforation in EDS-IV when the colon is left in place and the low incidence of reported small bowel and rectal perforations, subtotal colectomy is a reasonable treatment. Primary anastomosis and avoidance of an end-ileostomy was possible in this young patient, with no evidence of anastomotic leakage nor reperforation to date. Lifelong close follow-up should be continued in these patients, because the natural history of this anatomy in EDS-IV is not known.     

Aortic Dissecting Aneurysm with a Bicuspid Aortic Valve in Turner's Syndrome: Report of a Case

We herein report the performance of a successful, modified Bentall procedure and a total arch replacement for a Stanford type A chronic aortic dissection and a bicuspid aortic valve in Turner's syndrome (TS). The patient was a 45-year-old woman with 45, XO karyotype TS, who had had a history of hypertension since the age of 20. She had also been diagnosed as having a dilatation of the ascending aorta and a bicuspid aortic valve 3 years earlier. The patient became aware of back pain 6 months prior to the current admission, and was diagnosed as having a Stanford type A chronic aortic dissection and a bicuspid aortic valve with mild aortic regurgitation. One of the greatest concerns in TS is the risk of aortic dissection. Regarding the operation, aortic root replacement is one of the options for a bicuspid aortic valve so as to avoid high-risk surgical procedures in TS.     

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.     

A novel mutation in the vascular Ehlers-Danlos syndrome: a case presenting with colonic perforations

A 15-year-old girl who had chronic constipation presented with peritonitis caused by sigmoid colon perforation. After her sigmoid colon was resected and an end colostomy performed, as there were no apparent causes for perforation, she was followed-up. After the second colonic perforation proximal to the end colostomy, as the pathologic findings revealed myopathic changes, the connective tissue disorders were evaluated. Her molecular biology studies revealed an undefined missense mutation in the COL3A1 gene, confirming the diagnosis of vascular Ehlers-Danlos syndrome (EDS). As she refused a permanent stoma, total colectomy and ileorectal anastomosis were performed, but the postoperative complications resulted in a fatal progression.The typical progression of vascular EDS will be discussed with the presented case by means of a review of the English medical literature on children diagnosed with vascular EDS.     

Celiprolol Treatment in Patients with Vascular Ehlers-Danlos Syndrome

1. Download : Download high-res image (234KB)
2. Download : Download full-size image

     

主动脉夹层动脉瘤合并腹主动脉瘤的一期腔内治疗(附1例报告)

目的 :探讨胸主动脉夹层动脉瘤合并腹主动脉瘤病人作一期腔内隔绝术治疗的可行性、手术操作技巧及并发症防治原则。临床资料 :1例StanfordB型胸主动脉夹层动脉瘤合并腹主动脉及双侧髂动脉瘤的病人于 2 0 0 1年 2月在本中心接受了腔内隔绝术。术前CTA显示 :主动脉自弓降部开始出现夹层 ,一直延伸到腹主动脉分叉上6cm ,假腔的最大直径达 6 .6cm ;肾下腹主动脉瘤的最大直径为 4 .5cm ,瘤颈受夹层累及 ;双侧髂总动脉各有一直径 2 .5cm的真性动脉瘤。手术在全麻下进行 ,降主动脉植入规格为 34mm× 34mm× 1 30mm的直管型Talent移植物封闭夹层裂口 ;腹主动脉植入规格为 2 6mm× 1 4mm× 1 4 5mm的分叉型Talent移植物。将腹主动脉瘤和双侧髂动脉瘤隔绝 ,手术耗时 30 0min ,失血 30 0ml,透视 62min ,造影 5次 ,使用造影剂 2 0 0ml。术后病人恢复顺利 ,术后第 2天出ICU ,术后 30d出院。随访 1年 ,病人生活质量良好 ,复查CT示胸主动脉、腹主动脉瘤及髂动脉瘤完全封闭。结论 :腔内隔绝术的微创特点使一期治疗StandordB型主动脉夹层动脉瘤合并腹主动脉瘤成为一种比较安全的手术。术后应先处理胸主夹层处理腹主动脉瘤 ,以减少后半程手术对先前植入物的影响     

Superior Mesenteric Artery Syndrome After Successful Coil Embolization of a Ruptured Pancreaticoduodenal Artery Aneurysm: Report of a Case

Superior mesenteric artery (SMA) syndrome is a rare condition in which the third part of the duodenum is trapped between the abdominal aorta and the SMA. Any disease process decreasing the angle between the aorta and the SMA can result in external compression of the duodenum and subsequent intestinal obstruction. Aneurysms of the pancreaticoduodenal artery (PDA) and its branches are also rare and account for only 2% of all splanchnic artery aneurysms. We report a case of SMA syndrome that developed after successful coil embolization of a ruptured PDA aneurysm.