Successful endovascular repair of acute type B aortic dissection in undiagnosed Ehlers-Danlos syndrome type IV.

A 61-year-old man presented with an acute type B aortic dissection for which a stent-graft was introduced. He remains complication-free 4 years onwards and has since been diagnosed with Ehlers-Danlos syndrome type IV (EDS IV). His particular mutation is predicted to result in lesser levels of normal collagen and may explain his favourable outcome from endovascular intervention. Understanding the genotype-phenotype correlation may influence the choice of therapy offered to patients with EDS IV.

Management of spontaneous and iatrogenic retroperitoneal haemorrhage: conservative management, endovascular intervention or open surgery?

BACKGROUND: Retroperitoneal haematoma is a rare clinical entity with variable aetiology, which is increasing in incidence mainly due to complications related to interventional procedures. There is no general consensus as to the best management plan for patients with retroperitoneal haematoma. METHODS: A literature review was undertaken using MEDLINE, all relevant papers on retroperitoneal haemorrhage or haematoma were used. RESULTS: The diagnosis is often delayed as symptoms are nonspecific. Retroperitoneal haematoma should be suspected in patients with significant groin, flank, abdominal, back pain or haemodynamic instability following an interventional procedure. Spontaneous haemorrhage usually occurs in patients who are anticoagulated. Multi-slice CT and arteriography are important for diagnosis. Most haemodynamically stable patients can be managed with fluid resuscitation, correction of coagulopathy and blood transfusion. Endovascular treatment involving selective intra-arterial embolisation or the deployment of stent-grafts over the punctured vessel is attaining an increasingly important role. Open repair of retroperitoneal bleeding vessels should be reserved for cases when there is failure of conservative or endovascular measures to control the bleeding. Open repair is also required if endovascular facilities or expertise is unavailable and in cases where the patient is unstable. If treated inappropriately, the mortality of patients with retroperitoneal haematoma remains high. CONCLUSION: There is a lack of level I evidence for the best management plans for retroperitoneal haematoma, and evidence is based on small cohort series or isolated case reports. Conservative management should only be reserved for patients who are stable. Interventional radiology with intra-arterial embolisation or stent-grafting is the treatment of choice. Open surgery is now rarely required.

Endoluminal repair of distal aortic arch aneurysms causing aorto-vocal syndrome.

PURPOSE: We have evaluated the efficacy of endovascular repair of distal aortic arch aneurysms (DAAA) causing recurrent laryngeal nerve palsy. MATERIAL AND METHODS: Eight patients (five male and three female) with median age of 72 years (range: 59-80) presented with left recurrent laryngeal nerve palsy associated with DAAA. All patients were considered unfit for open surgery. The median aneurysm size was 5.9 cm (range: 5-7.3). Thirteen stents were deployed: eight Gore, four Endofit and one Talent. Epidural anaesthesia was used in all patients. The left subclavian artery was covered in all and the left common carotid in three who had a preliminary right to left carotid-carotid bypass. Routine follow-up (FU) was with computed tomography (CT) at 3-6 months and yearly thereafter. RESULTS: Exclusion of the aneurysm sac was achieved in all patients. Thirty-day mortality was 0%, with no paraplegia or stroke. Early complications included: rupture of the external iliac artery (one) and common femoral artery thrombectomy (one). One patient died of unknown cause at 17 months. The mean FU in the remaining seven patients was 21 months (range: 6-51). Aneurysm size decreased in five, was unchanged in one and increased in one. Three patients had improvement in voice quality postoperatively. One patient had a recurrent type 1 endoleak which was restented twice. No late deaths have occurred. CONCLUSION: Though technically the procedures involved were more complicated, endovascular repair of DAAA causing aorto-vocal syndrome is safe and offers a realistic alternative to open surgery. Hoarseness of the voice can improve postoperatively and is associated with reduction in aortic sac diameter.

Hybrid repair of type B dissecting aneurysm with associated Kommerell's diverticulum.

Endovascular therapy has revolutionised vascular surgery. Complex open surgical procedures may be replaced with a combination of extra-anatomical reconstruction and endovascular treatment. This minimally invasive approach is associated with a lower peri-operative morbidity and mortality than open repair. We describe a novel 2-stage treatment in a patient with Type B thoracic aortic dissection with subsequent aneurysmal dilatation and the added difficulty of a concomitant aneurysmal aberrant right subclavian artery (Kommerell's diverticulum).

Transcatheter embolization in the treatment of coronary artery fistulas.

Seven patients with a coronary artery fistula underwent percutaneous transcatheter embolization (five were male and two female; the age range was 2 to 67 years [median 17]). Three patients were symptomatic. The left to right shunt ranged from 1.6 to 2.6:1. In six patients, the fistula was an isolated congenital anomaly; in one, it was acquired. The fistula arose from branches of the left (n = 5) and right (n = 2) coronary arteries and drained to the right ventricle (n = 2), right atrium (n = 2), coronary sinus (n = 1), pulmonary artery (n = 1) and a bronchial artery (n = 1). Different embolization techniques were used to occlude eight feeding arteries. The embolization materials included a detachable balloon (n = 3), coaxial embolization with platinum microcoils (n = 3), a combination of detachable balloon and microcoil (n = 1) and standard steel coils (n = 1). Satisfactory occlusion was achieved in six patients. In one case, the valve of the detachable balloon was damaged, resulting in early balloon deflation and a residual fistula. There were no associated complications in any patient. Follow-up investigation by Doppler ultrasound or coronary angiography 4 months to 4 years later showed that permanent occlusion was achieved in all six patients in whom embolization was initially successful. Transcatheter embolization should be considered the treatment of choice for coronary artery fistulas.

The management of renal transplant artery stenosis in children by percutaneous transluminal angioplasty.

Between 1967 and 1989 in this unit 262 children (age at transplantation 9 months to 17 years, mean 9.6 years) had 345 renal transplants performed. Transplant artery stenosis (TAS) was found in 30 (8.7%) as demonstrated by arteriography, performed only when there was unexplained deterioration in transplant function, hypertension that was difficult to control, or in the presence of a vascular bruit. All patients with TAS except one had received a cadaveric allograft. From 1980 onward, percutaneous transluminal angioplasty (PTA) has been available for TAS, and this was attempted on 21 occasions in 16 patients. Nine patients demonstrated angiographic improvement following the procedure, and 7 showed immediate clinical improvement. On one occasion angioplasty precipitated graft loss. Five patients underwent planned corrective surgery, 4 after unsuccessful angioplasties. Our experience suggests that PTA should be the first method of intervention for TAS. Moderate success, both in angiographic and clinical terms, can be achieved, negating the need for surgery, while failure of PTA does not preclude surgical attempts at correction.

Use of interlocking detachable coils in embolization of coronary arteriovenous fistulas.

Thirteen patients underwent transcatheter closure of coronary artery fistulas using interlocking detachable coils alone or combined with fibered coils. Complete occlusion was achieved in 92% of patients; although inadvertent embolization of coils occurred in 23% of patients, all these coils were easily retrieved using snares.

Transcatheter embolisation to control severe bleeding in fungating breast cancer.

Severe and recurrent haemorrhage from ulcerating breast or skin lesions was controlled in seven of nine women with breast cancer. Arterial catheterisation and embolisation with a variety of materials (gelfoam, polyvinyl alcohol, steel coils, 50% dextrose) was used. Local anaesthesia only was needed, allowing very ill patients to be treated. This approach is the treatment of choice for severe haemorrhage in breast cancer.

The pathology of balloon pulmonary valvoplasty.

The pulmonary valve was examined following balloon dilation of pulmonary valve stenosis in six children. Stenosis was due to commissural fusion in two. In these patients, cineangiography showed that the valve was slightly thickened and "domed" during systole. Balloon valvoplasty produced complete separation of fused commissures in one and partial separation in the other. Commissural splitting is likely to be the most common mechanism of relief of pulmonary stenosis by balloon dilation. Incomplete separation could account for the partial reduction of the gradient in most children treated with this technique. Four children had pulmonary valve dysplasia. Balloon valvoplasty did not produce haemodynamic improvement or morphological changes that were identifiable at the time of surgical correction. Tearing of pulmonary valve tissue and avulsion of pulmonary cusps were not seen in this series.

Intraluminal biopsy of a superior vena cava mass.

A number of methods have been devised for the biopsy of intracaval tumour masses but all risk damage to the cava and tumour dissemination. We report on a case in which the tumour mass was almost entirely within the superior vena cava and describe an 'endoscopic' technique for biopsy.

Short communication: tunnelled central venous catheter insertion via the internal jugular vein using a dedicated portable ultrasound device.

We describe the use of a purpose-built portable ultrasound device to assist puncture of the internal jugular vein during percutaneous insertion of tunnelled central venous catheters such as Hickman lines. In many situations the internal jugular route is safer, faster and less costly in comparison with the subclavian approach. The use of ultrasound allows an accurate initial venous puncture with fewer complications, in the hands of both experienced operators and those less familiar with the internal jugular vein approach.

Quickscan: Doppler ultrasound emulation of angiography--its value prior to arteriography in peripheral vascular disease.

The prevalence of peripheral vascular disease demands a quick, reliable, non-invasive technique for initial assessment. We have devised a new method which combines the two physical principles that (1) Doppler shift is proportional to blood velocity and (2) blood velocity is inversely proportional to arterial cross-section with the ability to track probe position using a non-contacting method. An image of the probe track and any arterial narrowing is shown superimposed on an outline of the patient. Pressure measurement, scan and graphics with final report take about 20 minutes. The accuracy of this system in peripheral vascular disease was evaluated. Thirty-one patients underwent quickscan (QS) and arteriography within an average time of 7 days. Abdominal aorta, common iliac-common femoral, superficial femoral and popliteal artery segments were graded independently as normal, significant stenosis (greater than 50% of diameter) or occluded by both techniques. Of 197 segments, QS correctly assessed 106 normal, 22 stenosed and 28 occluded segments. Four equivocal angiographic stenoses were normal on QS and three severe stenoses were graded occlusion. Fifteen segments on angiography and five on QS were not assessed. For the iliac and superficial femoral artery segments, sensitivity and specificity averaged 77% and 86%, respectively, for all grades. Aortic statistics were invalid (only one significant lesion). Six out of eight popliteal occlusions were correctly diagnosed by QS, but no popliteal stenoses were detected out of six shown on arteriography. Low numbers may contribute to this discrepancy but an improved popliteal scanning method may be necessary. We find initial QS an invaluable aid to direct percutaneous angiography and to indicate potential sites for angioplasty.

Endovascular treatment of acute type B aortic dissection.

Intervention is currently reserved in acute aortic dissection for Stanford Type A and for complications of Type B. Endovascular techniques such as fenestration of the intimal flap and stenting of vessel origins have been used to alleviate end-organ ischaemia due to compromised branches. The introduction of stent grafts has offered a realistic alternative to surgery for Type B dissections. Closure of the primary entry tear encourages thrombosis of the false lumen, which is associated with good long-term outcome. Many questions remain unanswered and randomised controlled trials need to be performed to establish the role of stent grafts in uncomplicated Type B dissections, and the use of bare stents to encourage thrombosis of the more distal false lumen. Improvements in the design and engineering of stent grafts may help to establish endoluminal repair as the first line treatment of aortic dissection.