D. Grotemeyer

Surgical Treatment for Agenesis of the Vena Cava: A Single-centre Experience in 15 Cases

OBJECTIVE Agenesis of the inferior vena cava (IVC) is a rare vascular malformation. Deep vein thrombosis (DVT) and bilateral pelvic thrombosis develop quite frequently, making surgical therapy necessary. PATIENTS AND METHODS Between 1982 and 2006, 15 patients (nine male, six female, mean age 28 standard deviance 9 years) with agenesis of the IVC (IVCA) were treated surgically because of acute or subacute DVT. These patients underwent bilateral transfemoral ante- and retrograde thrombectomy of the iliofemoral and sometimes popliteal veins and replacement of the IVC with an external ring supported PTFE-graft. Bi- or unilateral arteriovenous fistulae were created in the femoral region. The fistulae were closed, on average, 8 months after trans-arterial venography was performed. These patients were examined clinically and by duplex ultrasound imaging during follow-up to assess graft patency and to allow CEAP classification. Patients were assessed for the development of post-thrombotic syndrome (PTS). RESULTS No patient died during any part of their treatment or within 60 days. Primary patency of the venous reconstruction was 53%, secondary and long time follow-up patency was 83%. The mean duration of follow-up was 41 SD 12 months. Minor complications were observed in five cases (33%). PTS showed no progression during a follow-up of 41 SD 12 months in all patients. There was no change in the CEAP clinical stage during follow-up nor did any leg ulcer develop. CONCLUSION A surgical approach to restore venous patency is effective and appears to prevent the deterioration of CVI over time.

Renal Cysts in Living Donor Kidney Transplantation: Long-Term Follow-up in 25 Patients

INTRODUCTION The acceptance of a living donor kidney bearing cysts might implicate complications after the transplantation due to the natural history of renal cysts. We have presented our experience with transplantation of living donor kidneys containing cysts but not polycystic disease. PATIENTS AND METHODS We retrospectively reviewed donor and recipient records of all living kidney transplants performed between January 1997 and April 2008. We analyzed serum creatinine and urea levels, as well as ultrasound scans concerning cyst size and morphology at hospital discharge as well as at 12 and 24 months after transplantation. RESULTS Among 268 living kidney transplantations, we noted 25 donors with renal cysts. In the computed tomography scan reports, 19 kidneys were described to show a single and six, multiple cysts. The size of 10 single cysts was <5 mm; the other nine were a mean of 17.33 mm. Two of the multiple cyst kidneys had lesions <5 mm; in four kidneys, the mean cyst size was 27.25 mm. The renal function of the recipients was normal or almost normal at discharge with a tendency to lower levels at 12 and 24 months after transplantation. Ultrasound revealed changes in cyst diameter among 6/23 kidneys; the mean diameter increased after 12 months, namely, 8.25 mm to 11.5 mm after 24 months. The subgroup of patients with enlarged cysts showed creatinine and urea levels slightly higher than in the entire group. No aspects of malignancy were found, according to the Bosniak and Israel classification system. One suspicious cyst was tomographically confirmed to be hemorrhagic without any need for treatment. None of the living donors had any problems related to the donor nephrectomy or a need for dialysis due to renal insufficiency in the long term. In addition, the living donors who had even beforehand cystic lesions in their contralateral nonremoved kidney at the time of transplantation did not show complications upon follow-up. CONCLUSIONS In our study, 25 living donor kidneys carried cysts. Neither cyst-related complications nor dysfunction of the transplanted organs occurred. An unroofing or excision of the cyst was generally not necessary. Regular ultrasound scans and optional computed tomography scans are recommended for follow-up. Based on this experience, we concluded that kidneys presenting cystic diseases should be considered to be suitable for transplantation without a hazard to the recipients, thus extending the pool of organs.

Analysis of Calcifications in Patients with Coral Reef Aorta

BACKGROUND Coral reef aorta is a rare vascular disease with intraluminal calcifications of the dorsal part of the visceral aorta. The pathogenesis of this disease with its topographic and morphologic characteristics is unknown. The aim of our study was to investigate calcification inhibitors and the ultrastructure of calcifications in patients with coral reef aorta. METHODS Ten patients with coral reef aorta were examined. Calcified specimens were investigated by immunohistochemical techniques for the expression of the calcification inhibitors matrix gla protein (MGP) and fetuin-A. Vessel walls were also assessed by electron microscopic techniques including electron energy-lost spectroscopy, electron dispersive spectroscopy, and electron diffraction. Sera of patients were analyzed for fetuin-A, uncarboxylated MGP (ucMGP), and osteoprotegerin. RESULTS As assessed by immunohistochemistry, most MGP was detected in the vicinity of calcified regions. Serum levels of the calcification inhibitors ucMGP, fetuin-A, and osteoprotegerin were 370+/-107 nmol/L, 0.57+/-0.03 g/L, and 5.64+/-0.79 pmol/L, respectively. Ultrastructural analysis of calcified specimens showed a core-shell structure with multiple calcification nuclei. Calcifications displayed a fine-crystalline character, and elemental analysis revealed hydroxyl apatite as the chemical compound. CONCLUSION The coral reef aorta represents an extreme exophytic growth of vascular calcification with multiple nuclei which resemble typical media calcification. Positive vascular immunostaining and low serum levels of both fetuin-A and ucMGP suggest a pathophysiologic role of these calcification inhibitors in the development of coral reef aorta.

Dignity of carotid body tumors. Review of the literature and clinical experiences

INTRODUCTION Tumors of the carotid body are rare paragangliomas (incidence 0.012%) originating from sympathetic fibres of the carotid bifurcation. Growth is slow and they frequently become symptomatic through local mechanical compression of neighboring vascular and neural structures. The aim of this study is to present the diagnosis, therapy and course in patients with a carotid body tumor treated at our department of the Düsseldorf University Hospital and to discuss rates of recurrence and also dignity during the long-term follow-up. PATIENTS AND METHODS Included in this retrospective study were all patients treated for a carotid body tumor between January 1988 and June 2008. At follow-up examination the current history was recorded and a physical examination, sonography and duplex sonography were carried out. Furthermore each patient completed the questionnaires QLQ-C30 of the European Organisation for Research and Treatment of Cancer (EORTC) and the module for head and neck QLQ-H&N35 to assess quality of life. RESULTS In our collective of 36 patients consisting of 13 men (36%) and 23 women (64%) with an average age of 48.33 years (range 17-78 years), 16 patients presented with a local neck swelling and 5 patients each had difficulties swallowing or hoarseness, respectively. Preoperatively Horners syndrome was found in one patient. A total of 22 tumors were found on the right side of the neck (52.38%), 20 were found on the left side (47.62%) and 6 patients showed a bilateral carotid body tumor (16.67%), 3 of which were bilaterally excised. The other 3 patients are still under surveillance without surgery. Altogether surgery of 39 carotid body tumors was performed in 36 patients. In all 39 cases (primary surgery n=34, recurrence surgery n=5) the tumors were macroscopically excised in toto. Parts of the vagus nerve had to be resected in 3 patients (7.69% Shamblin type II n=1, Shamblin type III n=1) and resection of blood vessels was necessary during 10 operations. The survival rate after 1 year was 100%, after 2 years 96.3% and after 5 years 92.6%. A local recurrence was diagnosed in 2 patients (5.13%). In one patient a second operation was necessary and in the other patient there was a non-progressive swelling in the carotid bifurcation which had existed for 14 years and which was conservatively left untreated. Peripheral neural lesions could be found in 12% (3/25) at long-term follow-up. None of the patients showed evidence of local or remote metastasization of a carotid body tumor. CONCLUSIONS Surgical extirpation of carotid body tumors can be regarded as the only curative option with an overall mortality of 0%. Morbidity is low when applying vascular surgical techniques (2.56% for central lesions). The incidence of peripheral nervous lesions is high reflecting the radicality of the resection (64.10%) but is outweighed by the benefits. In the long-term follow-up the rate of permanent peripheral neural lesions decreased to 12%. Due to a potentially infiltrating and disseminating growth, carotid body tumors should be regarded as semi-malignant and should therefore be indicated for surgery at the time of diagnosis. Whether the incidence of carotid body tumors will rise due to increased routine diagnostic examination of the head and neck region using sonography and tomography remains to be seen.

Subclavian carotid transposition: immediate and long-term outcomes of 126 surgical reconstructions.

BACKGROUND Subclavian carotid transposition (SCT) is a safe, effective, and durable treatment in atherosclerotic disease of the proximal subclavian artery. We report about our experience in SCT in a retrospective study with a long-term outcome in 126 cases and discuss our results with the current literature. METHODS From January 1995 to December 2013, we treated 126 patients (51 men, 75 women; mean age, 60.9 years; range, 25-80 years) for proximal subclavian atherosclerotic lesions. Preoperative angiography revealed 58 stenoses (46.0%) and 68 occlusions (54.0%). We performed 126 SCTs. The following parameters were documented through a retrospective chart review: demographic data, occlusion site, preoperative symptoms, cardiovascular risk profile, coexisting cerebrovascular disease, and postoperative outcome. Continuous variables are summarized as mean. Categoric variables are expressed as frequency and percentage. Survival and patency rates were estimated using Kaplan-Meier analysis. RESULTS Four immediate occlusions and 1 hemodynamic relevant stenosis (4.0%) occurred postoperatively. Thrombectomy was successful in 2 and a carotid axillary bypass was performed in 3 cases. Three strokes occurred, one during concomitant carotid endarterectomy. The 30-day mortality was 0%. Follow-up data were obtained on 106 of 126 patients (84.0%). The mean follow-up period was 53.8 months (range, 3-159 months). Twenty-three (18.3%) late deaths occurred. Estimated survival was 121.48 ± 6.86 months (range, 3-112 months). Ninety-five percent reported continuous resolution of symptoms. Primary patency rate was 96.0% and secondary patency rate was 100% at 30 days. The long-term patency rate was 96.3% at a mean follow-up of 53.8 months. CONCLUSIONS SCT is safe, effective, and durable in the long term. SCT is a standard procedure for occlusion and stenosis. Vessel occlusions, ineffective angioplasty, and preparation for thoracic stent grafting make SCT an important procedure in the surgeons repertoire.

Dignität von Glomus-caroticum-Tumoren

INTRODUCTION Tumors of the carotid body are rare paragangliomas (incidence 0.012%) originating from sympathetic fibres of the carotid bifurcation. Growth is slow and they frequently become symptomatic through local mechanical compression of neighboring vascular and neural structures. The aim of this study is to present the diagnosis, therapy and course in patients with a carotid body tumor treated at our department of the Düsseldorf University Hospital and to discuss rates of recurrence and also dignity during the long-term follow-up. PATIENTS AND METHODS Included in this retrospective study were all patients treated for a carotid body tumor between January 1988 and June 2008. At follow-up examination the current history was recorded and a physical examination, sonography and duplex sonography were carried out. Furthermore each patient completed the questionnaires QLQ-C30 of the European Organisation for Research and Treatment of Cancer (EORTC) and the module for head and neck QLQ-H&N35 to assess quality of life. RESULTS In our collective of 36 patients consisting of 13 men (36%) and 23 women (64%) with an average age of 48.33 years (range 17-78 years), 16 patients presented with a local neck swelling and 5 patients each had difficulties swallowing or hoarseness, respectively. Preoperatively Horners syndrome was found in one patient. A total of 22 tumors were found on the right side of the neck (52.38%), 20 were found on the left side (47.62%) and 6 patients showed a bilateral carotid body tumor (16.67%), 3 of which were bilaterally excised. The other 3 patients are still under surveillance without surgery. Altogether surgery of 39 carotid body tumors was performed in 36 patients. In all 39 cases (primary surgery n=34, recurrence surgery n=5) the tumors were macroscopically excised in toto. Parts of the vagus nerve had to be resected in 3 patients (7.69% Shamblin type II n=1, Shamblin type III n=1) and resection of blood vessels was necessary during 10 operations. The survival rate after 1 year was 100%, after 2 years 96.3% and after 5 years 92.6%. A local recurrence was diagnosed in 2 patients (5.13%). In one patient a second operation was necessary and in the other patient there was a non-progressive swelling in the carotid bifurcation which had existed for 14 years and which was conservatively left untreated. Peripheral neural lesions could be found in 12% (3/25) at long-term follow-up. None of the patients showed evidence of local or remote metastasization of a carotid body tumor. CONCLUSIONS Surgical extirpation of carotid body tumors can be regarded as the only curative option with an overall mortality of 0%. Morbidity is low when applying vascular surgical techniques (2.56% for central lesions). The incidence of peripheral nervous lesions is high reflecting the radicality of the resection (64.10%) but is outweighed by the benefits. In the long-term follow-up the rate of permanent peripheral neural lesions decreased to 12%. Due to a potentially infiltrating and disseminating growth, carotid body tumors should be regarded as semi-malignant and should therefore be indicated for surgery at the time of diagnosis. Whether the incidence of carotid body tumors will rise due to increased routine diagnostic examination of the head and neck region using sonography and tomography remains to be seen.

[Ruptured abdominal aortic aneurysm : perioperative indicators for the clinical course following conventional open surgery].

OBJECTIVES Ruptured abdominal aortic aneurysms (rAAA) still represent a life-threatening vascular disease, with high mortality despite improved diagnostic tools and perioperative patient management. The aim of this study was to reveal predictors of perioperative mortality and survival after open (conventional) rAAA repair. PATIENTS AND METHODS We analyzed data from our department containing 67 patient histories and clinical notes which were collected between January 1984 and December 2004. The study patients underwent emergent surgery for rAAA. In these cases we defined 72 preoperative, 47 intraoperative, and 39 postoperative variables for further analysis. RESULTS Our results indicate that the worst survival prognosis could be defined in patients with rAAA and aneurysmatic inclusion of the iliac arteries with concomitant prolonged shock who received an aorto-iliac bypass. For these patients we calculated a cumulative 30-day survival rate of 59.7% and 1-year survival of 43.3%. An influence of age and comorbidity on the mortality rate could not be proven. Furthermore the conclusion cannot be drawn that postoperative course was influenced by intra- vs retroperitoneal rupture localization. CONCLUSION This study provides evidence that neither old patient age nor comorbidities influence the mortality of patients suffering from rAAA, for whom time-consuming case selection according to previous morbidities should therefore be omitted. Instead we recommend conventional surgical repair as soon as possible to maximize the chances of survival.

[Paradigm shift in the therapy of Wilkie's syndrome. From bowel reconstruction to transposition of the superior mesenteric artery].

The superior mesenteric artery syndrome--also known as Wilkies syndrome or as arteriomesenteric obstruction of the duodenum--is a rare condition of upper intestinal obstruction in which the third part of the duodenum is compressed by the overlying, narrow-angled superior mesenteric artery against the posterior structures. It is characterized by early satiety, recurrent vomiting, abdominal distention, weight loss and postprandial distress. When nonsurgical management is not possible or the problem is refractory, surgical intervention is necessary. Usually a reconstruction of the intestinal passage is performed. We report the first case of successful transposition of the superior mesenteric artery into the infrarenal aorta in the therapy of Wilkies syndrome.

Ösophagusnekrose nach endovaskulärer Versorgung eines rupturierten thorakalen Aortenaneurysmas

We report a case of acute esophageal necrosis after endovascular stenting for acute rupture of a thoracic aortic aneurysm into the mediastinum caused by mediastinal compartment syndrome and overstenting of intercostal arteries.ZusammenfassungWir berichten über eine akute Ösophagusnekrose, die nach endovaskulärer Therapie eines ins Mediastinum perforierten thorakalen Aortenaneurysma auftrat. Ursache ist eine Ösophagusminderdurchblutung hervorgerufen durch ein mediastinales Kompartmentsyndrom in Kombination mit dem Überstenten von Interkostalarterien.AbstractWe report a case of acute esophageal necrosis after endovascular stenting for acute rupture of a thoracic aortic aneurysm into the mediastinum caused by mediastinal compartment syndrome and overstenting of intercostal arteries.

Paradigmenwechsel in der Behandlung des Wilkie-Syndroms

The superior mesenteric artery syndrome--also known as Wilkies syndrome or as arteriomesenteric obstruction of the duodenum--is a rare condition of upper intestinal obstruction in which the third part of the duodenum is compressed by the overlying, narrow-angled superior mesenteric artery against the posterior structures. It is characterized by early satiety, recurrent vomiting, abdominal distention, weight loss and postprandial distress. When nonsurgical management is not possible or the problem is refractory, surgical intervention is necessary. Usually a reconstruction of the intestinal passage is performed. We report the first case of successful transposition of the superior mesenteric artery into the infrarenal aorta in the therapy of Wilkies syndrome.