Clinical significance of retrograde flow in the vertebral artery

Although retrograde vertebral artery flow was described over 100 years ago, its relationship to symptoms remains unclear. We documented 43 patients who were found by duplex scanning to have reverse flow in the vertebral artery. Of this group, seven patients (16%) were found to have symptoms described as typical for the subclavian steal syndrome. Nearly one-third were asymptomatic. Of the remaining patients, 13 (30%) presented with nonhemispheric symptoms while nine (21%) had hemispheric symptoms. Nine patients had to and fro flow in the vertebral artery. This variant was not found in subclavian steal patients but correlated with nonhemispheric symptoms. During follow-up (mean: 19 months) none of the asymptomatic patients became symptomatic, and there were no strokes or stroke-related deaths. Surgical procedures which restored antegrade vertebral artery flow did not necessarily improve symptoms of posterior circulation ischemia. In some patients improvement in posterior circulation symptoms was noted following carotid endarterectomy. It is concluded that retrograde flow in the vertebral artery is, per se, a benign entity. Accurate selection of surgical candidates remains imprecise. It will require not only identification of vertebrobasilar disease but as yet undefined tests to assure symptoms are due to these stenoses.     

Persistent proatlantal artery associated with carotid artery stenosis treatment by percutaneous transluminal balloon angioplasty

A 58-year-old man had an asymptomatic tight stenosis of the internal carotid artery associated with a persistent proatlantal artery. This as well as other compositional arterial anomalies of the basilar artery were discovered on arteriograms. The stenosis was successfully treated by percutaneous transluminal balloon angiopfasty. Therapeutic choices are discussed in this setting because of the risk of carotid clamping in the presence of persistent carotid-basilar anastomoses. kg]Key wordsPresented at the Annual Meeting of the Société de Chirurgie Vasculaire de Langue Française, May 18–19, 1990, Nancy, France.     

Vertebral artery reconstruction: Results in 106 patients

Between January 1982 and December 1989, we performed 109 revascularizations of the vertebral artery in 106 patients. Eighty-six patients (81%) had isolated vertebrobasilar insufficiency, 18 (17%) had associated carotid and vertebrobasilar pathology while two (2%) had isolated carotid symptoms. The procedures performed included 98 revascularizations of the proximal vertebral artery and 11 reconstructions of the distal vertebral artery. One distal revascularization was required after early failure of proximal revascularization. In 36 cases (34%), a concomitant ipsilateral carotid artery revascularization procedure was performed, and in one case, an ipsilateral subclavian artery aneurysm was excised. Two patients (1.9%) died post-operatively and five patients (4.7%) had nonfatal neurologic complications. Four of these seven complications occurred after combined vertebral and carotid surgery. One hundred early follow-up arteriograms were obtained (92% of reconstructions). There were four occlusions, two of which were associated with neurologic deficits. Three patients were lost to follow-up. Mean follow-up was 48 months (4–100 months). Seven patients died in the late follow-up period (after one month). Actuarial five year survival was 91%. Overall patency at five years was 96%. The study of late neurologic events showed that 63% of patients had complete recovery, 30% improvement, and 7%, failure or aggravation of symptoms.Presented at the Annual Meeting of the Société de Chirurgie Vasculaire de Langue Française, May 18–19, 1990, Nancy, France.     

Surgical management of extracranial carotid artery aneurysms

Between 1982 and 1991 we performed eight operations on seven patients with carotid artery aneurysms. Their mean age was 52.8 years (range: 20–67 years). Five aneurysms were atherosclerotic, one was associated with Marfan's syndrome, and two were pseudoaneurysms, one occurring after Dacron patch angioplasty and the other due to tuberculosis. Seven aneurysms were treated electively; one patient underwent an emergency surgical procedure. In one case, the internal carotid artery was ligated. Seven operations were reconstructive. No intraluminal shunt was used. No perioperative deaths occurred. Regressive hemiparesis and ipsilateral Horner's syndrome developed in one patient. The follow-up period ranged from six months to nine and a half years. One patient died of myocardial infarction three months after surgery.     

Subclavian artery revascularization for myocardial ischemia

A 54-year-old woman had a secondary occlusion of the subclavian artery proximal to the internal mammary artery, which had been used for an anterior interventricular artery bypass, and was the source of recurrent angina. A left carotid-to-subclavian bypass was performed with success. This rare complication underscores the need for careful selection and surveillance of candidates for myocardial revascularization using the internal mammary artery.     

Complications of anomalous origin of the right subclavian artery: Case report and review of the literature

A 35-year-old black woman presented with thrombosis of an anomalous right subciavian artery and distal arterial embolization. Initially, her right subclavian artery was reimplanted onto the common carotid artery, and a brachial artery embolectomy plus intraoperative thrombolytic therapy were used to reopen her distal arterial circulation. When her brachial artery repair thrombosed the following day, a distal ulnar artery bypass and repeat thrombolytic therapy were required to restore arterial patency. Six months later, she returned with severe, progressive, neointimal hyperplasia of her brachial artery and a second attempt at arterial reconstruction was unsuccessful. She eventually required a right below-elbow amputation. This patient demonstrated an anomalous right subclavian artery that presented with distal embolization without an antecedent history of severe atherosclerotic disease or the development of a right subclavian artery aneurysm. A review of the medical literature relating to complications of this anomaly is provided.     

Hepatic artery aneurysm in corticosteroidtreated,adult Kawasaki's disease

We describe a single case of Kawasaki's disease (mucocutaneous lymph node syndrome) with the rare complication of a hepatic artery aneurysm which was surgically repaired. Unusual features include arterial aneurysmal formation in the hepatic arteries rather than in coronary arteries, the unusual morphology of the hepatic artery aneurysm, and the expansion of the aneurysm after corticosteroid therapy.     

Surgical management of the spinal deformity in Ehlers-Danlos syndrome type VI

Five patients with Ehlers-Danlos syndrome type VI were surgically treated for their spinal deformities. All of them were female. All but one had a double-major thoracic and lumbar curve. One had a mild scoliotic curve but severe thoracic lordosis. Anterior discectomy and fusion and posterior instrumentation was performed in a single stage in two patients, while two had anterior and posterior surgery performed in two stages. The remaining patient underwent posterior surgery only, because of the relative mildness of the deformity and adequate flexibility. Two serious vascular complications were confronted. One patient experienced avulsion of segmental arteries and rupture of iliac artery and vein during anterior surgery. Another patient had avulsion of superior gluteal artery, which happened during subperiosteal dissection to harvest iliac bone graft. Fortunately, we did not see any temporary or permanent neurological complications. The spinal fusions appeared solid radiographically, with no implant failure and loss of correction after an average follow-up of 4 years.     

Atherosclerotic occlusive disease of the superior mesenteric artery: Late results of reconstructive surgery

Between 1975 and 1988, 103 patients underwent reconstruction of the superior mesenteric artery for atherosclerotic occlusive disease. Patients undergoing revascularization with associated mesenteric infarction were excluded. There were 89 men and 14 women whose mean age was 57.2 years. Six patients were operated on emergently for impending mesenteric infarction; six patients underwent revascularization after intestinal resection for ischemic lesions; 20 patients had typical abdominal angina; 39 patients had nonspecific abdominal symptoms, and 32 patients underwent revascularization of their superior mesenteric artery for asymptomatic lesions. Revascularization of the celiac axis and inferior mesenteric artery was associated in 36 and four cases, respectively. Four patients (4%) died postoperatively. Four early occlusions (4%) were observed. During the follow-up period (mean=69 months), 18 patients died; five patients had recurrent intestinal ischemic symptoms, four of whom died. All surviving patients underwent follow-up duplex scanning, examination, and arterial or venous digitalized angiograms in selected cases. Nine patients (9%) had anatomical abnormalities: two stenoses and seven occlusions. Failure of revascularization of the superior mesenteric artery was observed in patients with severe initial intestinal ischemia. Late complications were not statistically significantly related to the different techniques of revascularization used. Presented at the Annual Meeting of the Société de Chirurgie Vasculaire de Langue Française, June 23–24, 1989, Strasbourg, France.     

Medial fibrodysplasia and aneurysm of the popliteal artery

Fibromuscular dysplasia of the popliteal artery is rare. We report a case in a 20-year-old man complicated by aneurysm revealed by recurrent synovial effusion of the right knee. Diagnosis was based on pathology reports which showed severe destruction in the media. Two other cases with histopathological documentation were found in the literature.     

Combined percutaneous transluminal angioplasty and extraanatomic bypass for symptomatic unilateral iliac artery occlusion with contralateral iliac artery stenosis

We have reviewed our experience with percutaneous transluminal angioplasty of contralateral iliac stenosis and extraanatomic bypass of the occluded iliac artery. Twenty-two men and nine women with a mean age of 65 years (range 46 to 84) presented with symptomatic iliac occlusive disease. Twenty-four (77%) had disabling claudication, four (13%) rest pain, and three (10%) ischemic tissue loss. Six (19%) had undergone previous vascular reconstructive procedures. All had an occluded iliac artery on the symptomatic side and greater than 50% stenosis of the contralateral iliac artery. Percutaneous transluminal angioplasty of the iliac stenosis was done prior to extraanatomic bypass, using polytetrafluoroethylene. There were six late deaths after discharge. The only significant complication was a femoral artery thrombosis which was corrected when the bypass graft was performed. Cumulative primary graft patency was 89% at one year and 81% at three years. The crossover graft occluded in six patients, five within 48 months of surgery, and one after nine years. One of these occluded grafts was salvaged by thrombectomy, for a secondary patency rate of 85% at three years. Two patients required aortobifemoral bypass, one an iliobifemoral bypass and one an ilioprofunda bypass. One patient operated upon for rest pain came to below-knee amputation. Mean resting ankle/brachial systolic pressure index increased significantly on the side of the iliac occlusion from 0.35 ±0.21 to 0.70 ± 0.20 (p < 0.05, paired t test) after the combined procedure. There was no significant difference in the mean resting ankle/brachial systolic pressure index on the contralateral side (0.60 ± 0.22 to 0.65 ± 0.27, ns). Combined iliac percutaneous transluminal angioplasty and femorofemoral bypass is a safe alternative to aortobifemoral bypass for selected patients with aortoiliac arterial occlusive disease. Presented at the Royal Australasian College of Surgeons, General Scientific Meeting, May 1989, Melbourne, Australia.     

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.     

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.     

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.     

True profunda femoris aneurysms: Are they more dangerous than other atherosclerotic aneurysms of the femoropopliteal segment?

Three cases of true aneurysms of the profunda femoris artery are reported along with a review of 17 other cases in the literature. These aneurysms are rare and commonly present with rapid enlargement or rupture (9/20), the risk of rupture being higher than those affecting the femoral or popliteal arteries. All patients underwent successful surgical treatment except for one who required amputation. The diagnosis of an aneurysm of the profunda femoris artery must be considered in all patients with a pulsatile swelling in the groin. Surgical treatment is mandatory, and it carries a low mortality as well as a low risk of amputation.     

Surgical complications of Ehlers-Danlos syndrome in children.

The Ehlers-Danlos syndrome is a special challenge to the surgeon because of its relative infrequency and the late onset of overt symptoms, such as hyperelastic skin. We should consider this diagnosis in every child with recurrent inguinal herniae and search for its stigmata. A child with a known Ehlers-Danlos syndrome who requires an elective operation should be carefully studied for coagulation disorders. If there is a history of past injuries or operations in which there was poor healing, the benefits of the proposed operation must be carefully weighed against possible complications. Operations must be carried out with great care to avoid tearing the skin and fascia. Meticulous attention is given to hemostasis to prevent postoperative bleeding or hematomas. Inguinal hernias in these children resemble those seen in elderly patients. The transversalis fascia is thin and the internal ring is hugely dilated. An adult-type repair, with the use of mesh or felt may result in a lower incidence of recurrence. In addition, the Ehlers-Danlos syndrome may be yet another etiology for obscure gastrointestinal bleeding in children. Specific enzyme defects have been identified in 2 of the 7 subtypes of this disease. Further, biochemical study of connective tissue defects may contribute to our knowledge of normal collagen synthesis and wound healing.     

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.     

A multi-institutional experience in vascular Ehlers-Danlos syndrome diagnosis

ObjectiveVascular Ehlers-Danlos syndrome (vEDS) is a rare disorder and 1 of 13 types of EDS. The syndrome results in aortic and arterial aneurysms and dissections at a young age. Diagnosis is confirmed with molecular testing via skin biopsy or genetic testing for COL3A1 pathogenic variants. We describe a multi-institutional experience in the diagnosis of vEDS from 2000 to 2015.MethodsThis is a multi-institutional cross-sectional retrospective study of individuals with vEDS. The institutions were recruited through the Vascular Low Frequency Disease Consortium. Individuals were identified using the International Classification of Diseases-9 and 10-CM codes for EDS (756.83 and Q79.6). A review of records was then performed to select individuals with vEDS. Data abstraction included demographics, family history, clinical features, major and minor diagnostic criteria, and molecular testing results. Individuals were classified into two cohorts and then compared: those with pathogenic COL3A1 variants and those diagnosed by clinical criteria alone without molecular confirmation.ResultsEleven institutions identified 173 individuals (35.3% male, 56.6% Caucasian) with vEDS. Of those, 11 (9.8%) had nonpathogenic alterations in COL3A1 and were excluded from the analysis. Among the remaining individuals, 86 (47.7% male, 68% Caucasian, 48.8% positive family history) had pathogenic COL3A1 variants and 76 (19.7% male, 19.7% Caucasian, 43.4% positive family history) were diagnosed by clinical criteria alone without molecular confirmation. Compared with the cohort with pathogenic COL3A1 variants, the clinical diagnosis only cohort had a higher number of females (80.3% vs 52.3%; P < .001), mitral valve prolapse (10.5% vs 1.2%; P = .009), and joint hypermobility (68.4% vs 40.7%; P < .001). Additionally, they had a lower frequency of easy bruising (23.7% vs 64%; P < .001), thin translucent skin (17.1% vs 48.8%; P < .001), intestinal perforation (3.9% vs 16.3%; P = .01), spontaneous pneumothorax/hemothorax (3.9% vs 14%, P.03), and arterial rupture (9.2% vs 17.4%; P = .13). There were no differences in mortality or age of mortality between the two cohorts.ConclusionsThis study highlights the importance of confirming vEDS diagnosis by testing for pathogenic COL3A1 variants rather than relying on clinical diagnostic criteria alone given the high degree of overlap with other forms genetically triggered arteriopathies. Because not all COL3A1 variants are pathogenic, the interpretation of the genetic testing results by an individual trained in variant assessment is essential to confirm the diagnosis. An accurate diagnosis is critical and has serious implications for lifelong screening and treatment strategies for the affected individual and family members.