[Malignant epithelioid hemangioendothelioma of the liver. Results of surgical treatment strategies]. / Malignes epitheloides Hämangioendotheliom der Leber. Ergebnisse der chirurgischen Behandlung.

BACKGROUND: Malignant epithelioid hemangioendothelioma is a rare vascular tumor described mostly in soft tissue, lungs, or liver. The outcome after a wide variety of therapeutic measures, ranging from extended surgical therapy to no therapy, is reported to be variable. Therefore, we reviewed our experience with resective therapy for this rare liver tumor, including orthotopic liver transplantation. MATERIAL AND METHODS: During a period of 5 years, seven patients with the histological diagnosis of hepatic epithelia hemangioendothelioma were seen. In five of them, the liver pathology was detected at random, and all patients were prospectively followed. The therapeutic measures and course of disease are given as case reports. RESULTS: Three patients received formal liver resection and two received liver grafts, one partial and one whole. One further patient is scheduled for transplantation and one is undergoing alternative therapy, with the tumor remaining stable. All resected patients recovered quickly and are alive and well 2 months to 4 years later without signs of tumor recidivism. CONCLUSION: Anatomic liver resection, or in the case of diffuse tumor spread, orthotopic liver transplantation, show favorable long-term results for the treatment of hepatic epithelioid hemangioendothelioma. Therefore, surgical therapy is proposed as the treatment of choice for this entity.

Liver transplantation for treatment of intrahepatic Osler's disease: first experiences.

BACKGROUND: Intrahepatic Osler's disease with multiple arteriovenous malformations and high intrahepatic shunting may lead to secondary pulmonary hypertension followed by right-heart stress and insufficiency. Until now, therapy with arterial embolization, banding, or ligation of the hepatic arteries is still limited and provides unsatisfactory long-term results. Liver transplantation offers another therapeutic option. METHODS: We report on four patients with intrahepatic involvement of Osler's disease who were liver transplanted between 1995 and 1999. All patients suffered from restricted liver function and right-heart insufficiency with multiple cardiac decompensations. One patient received one course of embolization, and another received six courses of embolization and then banding of the main hepatic artery before transplantation. In both patients, the clinical symptoms improved for only a few months. RESULTS: All patients had high degrees of intrahepatic arteriovenous shunting, and cardiac output measurements were between 8.0 to 13.3 L/min preoperatively. Preoperative mean pulmonary artery pressure was between 24 to 35 mmHg. After liver transplantation, cardiac output and right-heart diameter decreased or normalized and pulmonary pressure reached the normal range after 2 months. All patients received tacrolimus and steroids for primary immunosuppression. In one case, temporary hemodialysis was necessary for 2 weeks after transplantation, but renal function recovered completely. After follow-up time of 12 to 65 months, all patients had normal graft function and good cardiopulmonary condition. CONCLUSIONS: Indication for liver transplantation should be considered in patients with intrahepatic Osler's disease, high arteriovenous shunting with right-heart stress, and restricted liver function before irreversible fixed pulmonary hypertension leads to severe right-heart insufficiency or failure. Our therapeutic regimen of early liver transplantation in the case of intrahepatic Osler's disease in four patients has promising results.

Pulmonary artery stenosis 5 years after single lung transplantation in primary pulmonary hypertension.

This is a case report about a 56-year-old female patient with primary pulmonary hypertension who underwent single, right lung transplantation. Five years postoperatively she developed signs of right heart failure. History and physical examination suggested pulmonary artery stenosis. Diagnosis was confirmed by pulmonary angiography. Percutaneous placement of a balloon expandable stent normalized pulmonary artery pressure.

Treatment of humoral rejection after heart transplantation.

BACKGROUND: Until a few years ago, the incidence of humoral rejection after heart transplantation was underestimated. These episodes were frequently very aggressive and often fatal, because the maintenance and emergency immunosuppression available at the time only inadequately covered the humoral branch of the immune response. In spite of individual case reports, the effects of blood purification procedures or cyclophosphamide in this situation can only be insufficiently estimated. METHODS: To evaluate this therapy concept, 20 dog-lymphocyte-antigen-matched dogs underwent heterotopic neck-heart transplantation. Fourteen dogs underwent transplantation after having been previously sensitized through multiple skin transplantations, 6 dogs were not sensitized (control). The animals received an induction with 3x 250 mg prednisolone, as well as triple immunosuppression (cyclosporine, azathioprine, and cortisone). Biopsy (light microscopy, immunofluorescence), intramyocardial voltage, electric myocardial impedance (>200 kHz, <10 kHz), and echocardiographic (left ventricular wall thickness, diastolic relaxation velocity) examinations were performed daily to monitor rejection. Rejection therapy was continued for 3 days according to the following regimen: apheresis, cortisone boluses (CB), and cyclophosphamide in group A1 (n = 4), apheresis and CB without cyclophosphamide in group A2 (n = 4), and CB only in group C (n = 6). The subsequent course under triple immunosuppression was then observed. RESULTS: In the sensitized animals the onset of severe humoral rejection on the fifth day deteriorated cardiac function down to 75% (70% to 80%) of the initial values. In groups A1 and A2, apheresis resulted in recovery to near-control values (89% to 94%) within two hours, and indeed to complete recovery (97% to 101%) after the second apheresis, that is, within 1 day. In group C recovery was delayed (2 days) and incomplete (84% to 91 %). After therapy was discontinued, rejection-related functional deterioration recurred immediately in group C, and from 2 to 3 days after apheresis, regardless of whether cyclophosphamide therapy was performed (group A1) or not (group A2). In the control group all animals showed a rejection-free posttransplantation course. CONCLUSIONS: By diluting inflammatory mediators, apheresis leads to a rapid improvement in cardiac function during severe humoral rejection after head transplantation. Neither apheresis nor cyclophosphamide therapy are able to have an immediate positive influence on the activation of the immune cascade and to prevent an ongoing rejection.

Intramyocardial impedance measurements for diagnosis of acute cardiac allograft rejection.

BACKGROUND: Measurements of intramyocardial impedance at high frequencies can indicate alterations in cell membranes and intracellular spaces during acute cardiac allograft rejection. METHODS: Fifteen beagle dogs underwent heterotopic heart transplantation and were immunosuppressed with cyclosporine and methyl prednisolone (MP). Impedance was determined twice daily by means of four screw-in electrodes in the right and left ventricle. Transmyocardial biopsies and the intramyocardial electrogram (IMEG) were performed as reference methods. A total of 23 rejection episodes were induced. When acute rejection was recognized histologically and through IMEG readings, the animals were treated with a bolus of 125 mg of methyl prednisolone over 5 consecutive days. Treatment of rejection was controlled by biopsy and IMEG. RESULTS: All hearts showed a uniform decrease in impedance of about 28.3%+/-5.5% immediately after transplantation, which subsequently reached a stable plateau after 7 to 8 days. Impedance values then remained unchanged as long as rejection was absent. Biopsy findings of grades 1A to 1B (ISHLT) were accompanied by a statistically significant increase in impedance of 12.2%+/-2.5%; of grades 2 to 3A of 19.2%+/-3.2% and of grades 3B to 4 of 27.0%+/-2.9%. Sensitivity was 96%, specificity 91%. Successful treatment of rejection led to a decrease of impedance to the initial levels. CONCLUSIONS: The amount of increase in impedance of high frequencies is a method to stratify acute cardiac allograft rejection into grades like histologically grading. The effectiveness of rejection treatment can also be monitored through impedance measurement. The method is also applicable for telemetric rejection monitoring by means of an implantable device.

Plastic reconstruction of an extended corrosive injury of the posterior tracheal wall with an autologous esophageal patch.

We report on a patient with an extended corrosive injury of the posterior tracheal wall and left-sided tracheo-esophageal fistula after severe inhalative trauma. Resection of the fistula and necrotic tissue was followed by reconstruction of the posterior tracheal wall with an esophageal patch. Interposition of the stomach was performed to restore upper gastro-intestinal continuity. Revision was necessary due to an anastomotic insufficiency and a recurrent fistula between the trachea and the esophago-gastrostomy on the left side. The stomach was resected and the fistula was covered with a sternocleidomastoideus muscle flap. Several weeks later interposition of the right hemicolon was performed to establish the gastro-intestinal tract and the patient recovered completely, thereafter.

Intramyocardial electrogram recordings (IMEG) for diagnosis of cellular and humoral mediated cardiac allograft rejection.

OBJECTIVE: The purpose of this study was to prove the reliability of intramyocardial electrogram (IMEG) recordings for diagnosis and treatment monitoring of (1) cellular and (2) humoral mediated allograft rejection after heart transplantation. MATERIAL AND METHODS: Fifteen beagle dogs underwent heterotopic neck-heart transplantation. Eight of them were previously sensitized through several skin transplantations. IMEG recordings were performed daily. Donor-specific antibodies (IgG, IgM) were determinated in serum daily. Transmyocardial biopsies were performed every two days. RESULTS: In the sensitized group (group I) accelerated rejection occurred under triple drug immunosuppression with cyclosporine A, azathioprine, and cortisone on the fifth postoperative day (range: 4th-5th). All episodes were detected through IMEG diagnosis. In each case rejection could be treated successfully. In the cellular mediated group (group II), the average sensitivity for rejection diagnosis of a single lead was 24% for the unipolar and 42% for the bipolar leads. When the voltages of different leads were summed up the sensitivity rose to 36% (3 unipolar), 81% (3 bipolar) and 100% (all leads). During rejection therapy the IMEG recovered within 24-48 hours. CONCLUSION: The IMEG detects cellular and humoral mediated rejection early and with high reliability. The rejection-related changes of grade 2/3a rejection in IMEG seem to follow a Ofocal patternO similar to the histology. Therefore the recording of several, preferably bipolar, electrode configurations appears to enhance diagnostic reliability.

[Mesenteric artery occlusion as a rare complication of thromboangiitis obliterans]. / Mesenterialarterienverschluss als seltene Komplikation bei Thrombangiitis obliterans.

Mesenteric artery occlusions are rare complications of Thrombangiitis obliterans (Buerger's disease). We report on a 30-year old male with Thrombangiitis obliterans and mesenteric occlusion as a complication of this disease. Because of unclear abdominal pain, laparoscopy was performed which showed small bowel infarction and reduced liver perfusion. After small bowel resection and a second examination, ischemia of the intestinum continued. Angiography was performed, which showed central occlusion of the celiac trunk and the superior mesenteric artery. Relaparotomy with the embolectomy of the superior mesenteric artery, venous bypass from the sup.mes.art. to the hepatic arteries and repeated small bowel resection was performed. The patient recovered completely and was discharged from hospital after 3 weeks. After a further admission to the hospital 3 weeks later with abdominal pain caused by acute occlusion of the right colonic artery and severe ischemia of the right hemicolon, a right hemicolectomy was performed. Now, one year after the last hospital admission, the patient shows no sign of having any abdominal problems.

Transabdominal ligation of the thoracic duct as treatment of choice for postoperative chylothorax after esophagectomy.

Postoperative chylothorax after injury of the thoracic duct during esophagectomy is a rare but severe complication which may lead to serious problems such as loss of fat and proteins, and immunodeficiency. Without treatment mortality can rise to over 50%. From 1988 to 2005, we treated 10 patients with postoperative chylothorax after 409 resections of the esophagus (2.4%). Of these 10 patients nine underwent transthoracic esophagectomy with gastric pull-up to enable an intrathoracic (n = 7) or cervical (n = 2) anastomosis and one patient received a transhiatal esophagectomy with gastric pull-up and cervical anastomosis. The average amount of postoperative chylus was 2205 mL (200-4500 mL) per day. After a median postoperative interval of 10 days, relaparotomy and transhiatal double ligation of the thoracic duct was performed in nine out of 10 patients. One patient could be managed conservatively. The average amount of chylus was reduced to 151 mL per day (90.5%). Seven patients had no complications, and three suffered from postoperative pneumonia. Two of the patients with pneumonia recovered, and one died. Discharge from hospital, after ligation of the thoracic duct, was possible after a median time of 18 days (11-52). Ligation of the thoracic duct via relaparotomy appeared to be a simple and safe method to treat postoperative chylothorax.

Hepatocellular carcinoma in association with hepatic focal nodular hyperplasia.

PURPOSE: To report the association between hepatocellular carcinoma (HCC) and hepatic focal nodular hyperplasia (FNH) and the possible impact on clinical decision-making with regard to resective approaches in patients with FNH. MATERIAL AND METHODS: We retrospectively analyzed the findings in 77 adult patients who underwent liver resections for FNH between October 1989 and September 2001 at our center. HCC within the confines of FNH was found in two patients. We demonstrate the magnetic resonance imaging (MRI) and macroscopic and microscopic findings. RESULTS: Presurgical MRI demonstrated heterogeneous signal characteristics of moderately hyperintense FNH on T2-weighted images and, after i.v. administration of superparamagnetic iron oxide particles, HCC in FNH was barely delineable. Both patients underwent successful right hemihepatectomy to remove the suspicious FNH with diameters of 12 and 14 cm; intralesional HCC diameters were 3 and 5 cm, respectively. Patients could be rapidly dismissed. However, one patient died after recurrence of HCC 1.5 years after surgery, whereas the other patient continues tumor-free 4 years after surgery. Alpha-feto-protein was normal in both patients. CONCLUSION: In FNH with rapid growth tendency and heterogenic MR appearance, surgical removal should be considered to overcome the risk of inadequate therapy in the very rare group of patients with HCC in association with FNH.

[Arterial port-catheter dislocation into the duodenum]. / Arterielle Port-Katheter-Dislokation ins Duodenum - Eine seltene Komplikation.

Arterial port-catheter dislocations into the duodenum are rare complications. We report on a 64-year-old male with sigmoid-resection for carcinoma, left hemihepatectomy and arterial port-catheter implantation into the common hepatic artery for liver metastases in both lobes and partial dislocation of the port system into the duodenum. Therefore, the port-catheter system had to be explanted after 8 cycles of cytostatic therapy and the duodenum had to be closed by a suture. Six months after surgery the patient felt well, a new singular metastasis in the right liver lobe could be treated successfully by laserinduced thermotherapy. At present the patient has no evidence for another metastasis.

A dermoid cyst in the greater omentum as a rare epigastric tumor.

A 36-year-old female presented with unspecific epigastric discomfort, without weight loss, night sweat or fever. Ultrasound and computed tomography showed a solid tumor with a diameter of 9 cm in the left upper abdomen, without any connection to the stomach, the pancreas or spleen. Laparoscopy showed a connection to parts of the greater omentum. Two days after laparoscopic resection the patient was discharged from hospital. Histology revealed the rare diagnosis of a dermoid cyst. A possible malignant degeneration of the tumor has to be considered, and therefore the tumor should be resected. Since the operation the patient is asymptomatic and without tumor recurrence.