Kidney transplantation improves survival and is indicated in Fabry's disease.

BACKGROUND: Fabry's disease (FD) is an inborn error of glycosphingolipid catabolism with progressive systemic deposition of globotriaosylceramide thereby leading to renal and cardiac failure. Current therapy involves symptomatic medical management, dialysis, enzyme replacement therapy, kidney transplantation (KTx), and more recently gene therapy. Case fatalities occur in the fourth decade of life resulting from uremia unless dialysis or KTx is undertaken. STUDY DESIGN: This is a retrospective study aimed at determining the effect of KTx on the long-term outcome of patients with FD. RESULTS: Between 1964 and 1998, ten patients with FD received KTx at our institutions. Actuarial patient and graft survivals were 100% and 90% at 5 years; 76% and 66% at 10 years. One kidney graft was lost due to rejection. Patient survival data compared favorably at 5 years with survival of FD patients on hemodialysis alone (41%, P < .05). Five patients are alive at the time of this study, and five patients died with median survival time after KTx of 128 months (range: 74-160 months). CONCLUSIONS: This study demonstrates an excellent outcome in patients with FD in the first decade after KTx. In the absence of a severe contraindication, we advocate KTx to improve the overall prognosis of patients with renal failure due to FD. Based on the data, enzyme replacement therapy after KTx seems indicated, as FD progresses posttransplant, leading to case fatalities in the second decade after KTx.

[Mesenteric cyst as the etiology of abdominal pain--a case report]. / Mesenteriale Zyste als Ursache von Abdominalschmerzen--Eine Fallbeschreibung.

INTRODUCTION: Mesenteric cysts are part of the differential diagnosis of abdominal tumors. We want to remember this diagnosis with the following case report. CASE REPORT: A 35-year-old woman was admitted for abdominal pain that had begun two weeks previously. Sonographic examination and CT scan of the abdomen showed a 14 x 12 x 3 cm abdominal tumor without any relation to the uterus, adnexa or organs of the epigastrium. Laparotomy was performed and the cystic tumor removed. DISCUSSION: Mesenteric cysts are rare. The pathogenesis is not uniform and the clinical and radiologic diagnosis is difficult. The symptoms of this condition vary from acute abdominal signs to non-specific abdominal features or incidental findings. Mesenteric cysts can be located anywhere in the mesentery from the duodenum to the rectum. The treatment of choice is resection. CONCLUSIONS: Mesenteric cysts are rare abdominal conditions. The resection of the cyst and the verification of the diagnosis is the treatment of choice.

Ultrasound scans done by surgeons for patients with acute abdominal pain: a prospective study.

OBJECTIVE: To evaluate the routine use of abdominal ultrasonography (US) in patients admitted to the surgical emergency unit with acute abdominal pain. DESIGN: Prospective study with a three-step evaluation of patients over a 12-month period. SETTING: University hospital, Switzerland. SUBJECTS: 496 patients (male/female = 234/262; mean age 45 years) who presented with acute abdominal pain. INTERVENTIONS: Every patient underwent routine investigations and had an abdominal US by the attending surgeon. MAIN OUTCOME MEASURES: Clinical diagnosis, post-ultrasonography diagnosis and final diagnosis. RESULTS: US improved the correct diagnostic rate from 348 (70%) to 414 (83%). The diagnostic accuracy for acute appendicitis and biliary tract disease improved after US from 455 (92%) to 488 (98%) and from 463 (93%) to 490 (99%), respectively; the corresponding sensitivities and specificities were 91% and 99% and 94% and 99%. CONCLUSIONS: Ultrasonography should be part of routine surgical investigation and should be mastered and used by surgeons.

[The "buried bumper syndrome"--a rare complication of percutaneous endoscopic gastrostomy]. / Das Buried Bumper-Syndrom--Eine seltene Komplikation nach perkutaner endoskopischer Gastrostomie (PEG).

The buried bumper syndrome is a rare complication of percutaneous endoscopic gastrostomy (PEG). Hereby the PEG bumper is overgrown by hypertrophic gastric mucosa and embedded into the gastric wall. This is probably a consequence of enforced tightening of the PEG tube causing an ulcer in the gastric mucosa. Endoscopically the bumper is not visible anymore. The symptoms of the buried bumper-syndrome are a poorly transporting PEG tube, a PEG tube that cannot be mobilised, secretion along the tube and upper abdominal pain. Most often an endoscopic approach to remove the bumper is successful. If not, the operative removal of the plate is necessary. After endoscopic removal of the tube an endoscopic replacement of a PEG tube is technically possible.

[Transanal endoscopic microsurgery--experiences at the Zurich University Hospital]. / Die transanale endoskopische Mikrochirurgie--Erfahrungen am USZ.

The advantages of TEM (transanal endoscoic microsurgery) are minimally invasive, exact and full thickness excision of tumors in the rectum and a very low morbidity with excellent comfort for the patient. In a retrospective study all transanal endoscopic operations at Zurich University hospital in the last 5 years have been analyzed (n = 18). 11 adenomas and 5 carcinomas of the rectum have been resected with TEM (one mucosectomy, 16 full wall resections and one segmental resection of the rectum). In the group of the carcinomas there were four preoperatively known carcinomas, one T1 carcinoma was discovered postoperatively in the analyzed tissue. Among the four known carcinomas was one T1 carcinoma, two T2 carcinomas (one of them was thought to be a T1 preoperatively) and one T3 carcinoma. One patient with T2 carcinoma wanted specifically a minimally invasive procedure, the other one with T2 carcinoma was an older patient who didn't qualify for laparotomy. The patient with T3 carcinoma also had a malignant lymphoma. The operation was tolerated well by all the patients. There was one case of peritoneal perforation treated laparscopically and one case of postoperative bleeding. An incontinence of gas in one patient disappeared after 3 months. There was no adjuvant treatment in the group of the T1 carcinomas. One patient with a postoperative T2 carcinoma did not want a chemotherapy. The other two patients with T2 and T3 were polymorbid. Among the resected adenomas there was no case of recurrence. One T2 carcinoma recurred. These results show that transanal endoscopic microsurgery (TEM) is an excellent technique to treat ademomas and T1 carcinomas of the rectum with the advantages of full thickness excision under good vision, a minimal rate of recurrence and maximal patient comfort. The indications for transanal microsurgery are rare. The techically demanding operation is not always simple and should be performed in larger centers only.

Prospective, randomized comparison of extrapleural versus epidural analgesia for postthoracotomy pain.

BACKGROUND: Thoracic epidural analgesia is considered the method of choice for postthoracotomy analgesia, but it is not suitable for every patient and is associated with some risks and side effects. We therefore evaluated the effects of an extrapleural intercostal analgesia as an alternative to thoracic epidural analgesia. METHODS: In a prospective, randomized study, pain control, recovery of ventilatory function, and pulmonary complications were analyzed in patients undergoing elective lobectomy or bilobectomy. Two groups of 15 patients each were compared: one received a continuous extrapleural intercostal nerve blockade (T3 through T6) with bupivacaine through an indwelling catheter, the other was administered a combination of local anesthetics (bupivacaine) and opioid analgesics (fentanyl) through a thoracic epidural catheter. RESULTS: Both techniques were safe and highly effective in terms of pain relief and recovery of postoperative pulmonary function. However, minor differences were observed that, together with practical benefits, would favor extrapleural intercostal analgesia. CONCLUSIONS: These results led us to suggest that extrapleural intercostal analgesia might be a valuable alternative to thoracic epidural analgesia for pain control after thoracotomy and should particularly be considered in patients who do not qualify for thoracic epidural analgesia.

Percutaneous drainage of abdominal abscesses: are large-bore catheters necessary?

OBJECTIVE: To find out whether small-bore catheters (7 F) are as effective as the 14F sump drains generally used for drainage of abdominal abscesses. DESIGN: Retrospective review. SETTING: University hospital, Switzerland. SUBJECTS: 64 patients with intra-abdominal abscesses. INTERVENTIONS: 40 were drained with 7F pigtail catheters and 24 by 14F sump drains. RESULTS: Drainage was successful in 34/40 (85%) and 20/24 (83%), respectively. There were 3 recurrences in the small-bore and 1 in the large-bore group (p=0.4). Mean drainage time was 8 (SD 5) days and 11 (SD 11) days, respectively (p=0.29). One patient (3%) developed a complication in the small-bore group and 2 (8%) in the large-bore group. 4/6 failures in the small-bore group and 1/4 failures in the large-bore group were pancreatic abscesses. CONCLUSIONS: We conclude that percutaneous drainage with small-bore catheters is as effective as drainage with bigger tubes.

Intussusception of the small bowel due to Peutz-Jeghers syndrome: a case report.

We report the case of a 45-year-old woman with an intussusception of the small bowel due to Peutz-Jeghers syndrome. Immediate laparotomy was performed, and approximately 5 cm of the small bowel had to be removed because of ischemic areas. Six other polyps were removed through buttonhole enterotomies. The Peutz-Jeghers syndrome is rare. Treatment is either surgery or a combination of surgery and intraoperative enteroscopy. Bowel resections must be kept to a minimum.

Long-term results of hepaticojejunostomy for benign lesions of the bile ducts.

BACKGROUND: Hepaticojejunostomy has been the method of choice for the treatment of benign lesions of the extrahepatic bile ducts for years. In the era of minimally invasive and interventional techniques, a review of its long-term results is necessary to set the standard with which these new techniques have to be compared. METHODS: A retrospective analysis was carried out for 51 patients (16 females, 35 males) aged 24 to 83 years (average 48 +/- 13) who had undergone hepaticojejunostomy for benign lesions at our institution between 1980 and 1989. Twelve patients had had up to 4 prior operations of their bile ducts. The main indications for operation were chronic pancreatitis (n = 33) and iatrogenic bile duct lesions (n = 15). If possible, a low end-to-side hepaticojejunostomy was performed. The Hepp-Couinaud approach was saved for high strictures and recurrences. All patients were reassessed by questionnaire at an average of 7.6 years (range 2 to 13) after the operation. RESULTS: Four Hepp-Couinaud and 47 low hepaticojejunostomies were performed. Postoperative complications were seen in 17 patients (33%), 4 of whom had a reoperation. One patient died, for a mortality rate of 2%. The hospital stay averaged 24 +/- 17 days (range 8 to 90). Late complications developed in 13 patients (25%) 2 months to 6 years after the operation. Stenosis and cholangitis necessitated reoperation in 3 cases, cholangitis without stenosis was treated in 4, and other complications were seen in 5 cases. One patient died with a liver abscess, and 12 died of causes unrelated to the operation. When questioned, 31 of 35 patients were in good or very good condition. CONCLUSIONS: Hepaticojejunostomy is a safe and reliable method for the treatment of benign lesions of the bile ducts even in young patients in need of a long-term biliary bypass.

[Detection of unexpected extrathoracic metastases in preoperative staging of non-small-cell bronchial carcinoma (NSCLC) with positron emission tomography (PET)]. / Nachweis unerwarteter extrathorakaler Metastasen beim präoperativen Staging des nicht kleinzelligen Bronchialkarzinoms (NSCLC) mittels Positronenemissionstomographie (PET).

Accurate staging of non-small cell lung cancer (NSCLC) is essential for subsequent treatment. This study was designed to evaluate the value of FDG-PET in detecting unexpected extrathoracic metastases (ETM) in patients with NSCLC qualifying for surgical treatment based on conventional staging. One hundred patients with stage IIIa or less were included and underwent clinical evaluation, chest and upper abdominal CT scan, mediastinoscopy, and routine laboratory tests. If clinical signs of EM were present additional diagnostic methods, were applied. A partial body FDG-PET was performed. All findings in the FDG-PET were confirmed histologically or radiologically. Unknown ETM were detected in 13 patients (14%) at 19 sites. Whole-body FDG-PET improves detection of unsuspected ETM in patients with NSCLC otherwise eligible for surgery. Fourteen percent of patients were understaged.

[33 years kidney transplantation in Zurich]. / 33 Jahre Nierentransplantation in Zürich.

This article summarizes our experience with renal transplantation over the past 33 years and shows progressive improvement of the clinical outcome over time. In parallel we observed an increasing proportion of elderly recipients and diabetics. The increasing shortage of cadaver grafts was compensated by using donor hearts from legally dead donors (heart no longer beating) and establishing a living donor program.

[Kidney retransplantation in relation to organ shortage]. / Nieren-Retransplantation im Zeichen des Organmangels.

This article reviews our experience with renal regrafting in the past 16 years with particular emphasis on long-term outcome and prognostic parameters. Among 1094 renal transplants there were 15.8% regrafts, and the proportion of patients awaiting a regraft procedure on our current waiting list is 33%. Graft and patient survival was similar for first and second transplants, but was significantly reduced when a third graft was performed. The main prognostic parameters were the time of function of the previous graft, mismatch for HLA-DR and the presence of panel-reactive antibodies.

Magnetic resonance-guided laparoscopic interstitial laser therapy of the liver.

BACKGROUND: There is a necessity for an imaging method during laparoscopy to get a three-dimensional access to the target. In this study we evaluated laparoscopic interstitial laser therapy of the liver under magnetic resonance imaging guidance. METHODS: Five domestic pigs underwent laparoscopy in an open-configuration magnetic resonance system. Under simultaneous real-time magnetic resonance imaging interstitial laser therapy was applied to the liver. Magnetic resonance images, macroscopic aspects of the lesions, and light microscopic findings were compared. RESULTS: The interventions could be safely performed. There was no image artifact caused by instruments or by the carbon dioxide. Dynamic gadolinium-enhanced imaging proved to significantly predict the macroscopic volume of the laser lesions. CONCLUSIONS: Magnetic resonance-guided laparoscopic interstitial laser therapy of the liver combines the advantages of minimal invasive surgery and magnetic resonance imaging. Further development should focus on laparoscopic instruments and temperature sensitive sequences.

Experimental technique for (laparoscopic) bowel anastomosis: transient endoluminally stented anastomosis (TESA).

A new technique for bowel anastomosis is presented. The principle of transient endoluminally stented anastomosis (TESA) is based on anastomosing the two bowel ends around a resorbable stent of polyglycolic acid (PGA) in seroserosal contact. To evaluate the feasibility of TESA for bowel anastomosis, laparoscopic colon anastomosis following sigma resection was performed in five juvenile pigs. Three animals were sacrificed 2 months postoperatively, and the anastomoses were examined radiologically and histologically. One animal was sacrificed at day 2, suffering from acute peritonitis due to small bowel leak but with regular colon anastomosis. One trial was terminated at the fourth postoperative day because of insufficiency of the colon anastomosis. Three animals did not have any complications during the 2-month follow-up. In these animals the colon anastomoses were not detectable radiologically at the time of death. The microscopic examination showed intact mucosal and muscular layers without foreign material. Our study demonstrates that laparoscopic application of TESA to colon anastomosis is a feasible method. These results will further stimulate our future research for an anastomosis technique avoiding remnant foreign material.

Lung herniation through a postthoracoscopy chest wall defect: demonstration with spiral CT.

The incidence of complications following thoracoscopy is approximately 10%, the most prevalent being prolonged air leak and chest pain. We report two cases of lung herniation through the chest wall defect created by thoracoscopy. Use of the Valsalva maneuver during CT scanning is recommended as a diagnostic imaging method in cases with suspected lung herniation.

Long-term complete remission of melanoma liver metastases after intermittent intra-arterial cisplatin chemotherapy and surgery.

This paper presents the case of a female patient with liver metastases of a malignant melanoma showing complete remission after 10 courses of regional, intra-arterial chemotherapy with cisplatin. The drug was administered as continuous infusion for 5 days. The daily dosage amounted to 30 mg/m2. The interval between courses was 6 weeks. Nausea and vomiting were seen after each course, while pathological serum creatinine levels only appeared after the eighth course. The only lesion in the liver still visible on CT scan after chemotherapy was removed by left hemihepatectomy. Meticulous histological examination revealed a big focus of necrotic tissue without any tumour cells. At the time of publication the patient is alive and disease-free over 9 years later.

Prospective evaluation of pretransplant blood transfusions in cadaver kidney recipients.

BACKGROUND: A beneficial effect of pretransplant transfusions on graft survival was demonstrated in the early 1970s. In the mid-1980s, however, retrospective studies showed that transfusions had lost their graft-protective effect in the cyclosporine era. During the last 10 years, deliberate transfusion pretreatment of transplant patients has been discontinued. METHODS: Within a collaborative project of 14 transplant centers, prospective recipients of cadaver kidney grafts were randomized to receive either three pretransplant transfusions or transplants without transfusions. RESULTS; The graft survival rate was significantly higher in the 205 transfusion recipients than in the 218 patients who did not receive transfusions (at 1 year: 90+/-2% vs. 82+/-3%, P=0.020; at 5 years: 79+/-3% vs. 70+/-4%, P=0.025). Cox regression analysis showed that this effect was independent of age, gender, underlying disease, prophylaxis with antilymphocyte antibodies, and preformed lymphocytotoxins. CONCLUSIONS; Transfusion pretreatment improves the outcome of cadaver kidney transplants even with the use of modern immunosuppressive regimens.

Laparoscopic Roux-en-Y choledochojejunostomy.

OBJECTIVE: Although surgical biliary bypass for nonresectable periampullary tumors is superior to endoscopic stent placement, the latter has become popular because of the "minimally invasive" approach. Laparoscopic biliary bypass would appear to offer the advantages of both. However, this technique remains technically difficult using existing instrumentation. This study investigates the efficacy of a new endoscopic device designed for rapidly completing a small-diameter intestinal anastomosis under laparoscopic guidance. METHODS: Eighteen female pigs (mean weight 35 kg, range 31 to 44) were randomly divided into three groups: animals undergoing handsewn (group H) or instrumental transient endoluminally stented anastomosis (TESA; groups P and D) laparoscopic Roux-en-Y choledochojejunostomy. For TESA two different reabsorbable stents were used, polyglycolic acid (PGA; group P) and polyurethane ester (Degrapol; group D). Blood chemistry, weight gain, and abdominal X-rays were taken weekly to document any possible migration or reabsorption of the radio-opaque stents. After 3 months, necropsy was performed. Patency of the biliary bypass and choledochojejunostomy were examined using fluoroscopy and measured by introducing graduated dilators into the anastomosis. RESULTS: Fluoroscopy revealed immediate passage of contrast through the anastomosis in all animals. Weight gain, bilirubin, and alkaline phosphatase were within normal range in all groups. Diameter of the bile duct (group H 10.7 +/- 2.9 mm/group P 9.5 +/- 3.6 mm/group D 11.0 +/- 4.6 mm) and choledochojejunostomy (group H 4.5 +/- 1.1 mm/group P 4.7 +/- 1.8 mm/group D 3.6 +/- 1.9 mm) did not differ. The time required to complete the biliary bypass was significantly decreased when TESA was applied (group H 152 +/- 13 min/group P 86 +/- 14 min, P <0.001/group D 110 +/- 20 min, P <0.002). CONCLUSIONS: Applying TESA, laparoscopic choledochojejunostomy can be performed rapidly and safely, revealing good bypass function over a period of 3 months. With regard to treatment for nonresectable periampullary tumors, TESA may offer a new therapeutic approach combining the benefits of minimally invasive endoscopic stent placement with the functional results and lower readmission of conventional Roux-en-Y choledochojejunostomy.