[Giant paraesophageal herniation with intermittent prolapse into the thorax of the transverse colon]. / Monströse paraösophageale Zwerchfellhernie mit isolierter Prolabierung des Colon transversum nach intrathorakal.

HISTORY AND ADMISSION FINDINGS: A 74-year-old woman was admitted with a history of recurring dyspnea for several months. During radiological examination of the chest computed tomography demonstrated a giant paraesophageal hernia containing transverse colon with a significant amount of paracolic fat tissue. Physical examination was unremarkable. INVESTIGATIONS: Routine blood tests and abdominal ultrasound were within the normal range. Endoscopy showed a normal upper and lower gastrointestinal tract and barium swallow was normal without any esophageal motor dysfunction. The esophagogastric junction and gastric fundus were below the diaphragm. TREATMENT AND COURSE: Laparoscopy revealed the colonic herniation and mediastinal adhesiolysis, complete resection of the hernia sac and reposition of the intrathoracic migrated transverse colon were undertaken. Hiatal repair was performed by anterior and posterior hiatoplasty and construction of an anterior 180˚ semifundoplication with fundopexy. CONCLUSION: Patients with giant paraesophageal hernias often present with nonspecific cardiac and respiratory symptoms and the condition is often misdiagnosed. If it is demonstrated, a possible abdominal involvement should be looked for. Minimally invasive surgery is feasible and efficacious in this condition and in addition to being better tolerated by the patient provides a far better visualization of the intrathoracic parts of a type IV hiatal hernia to the surgeon.

Minilaparoscopy in the diagnosis of peritoneal tumor spread: prospective controlled comparison with computed tomography.

BACKGROUND: Early diagnosis of peritoneal spread in malignant disease prevents unnecessary laparotomies. Minimally invasive laparoscopy with the patient under conscious sedation is a new, easily feasible diagnostic technique. This study compares prospective and controlled diagnostic minilaparoscopy with computed tomography (CT) scan for the diagnosis of peritoneal metastases. METHODS: In this study, 56 patients with malignant disease were prospectively investigated with diagnostic minilaparoscopy and CT scan. RESULTS: The study criteria were fulfilled by 54 patients. Minilaparoscopy detected peritoneal carcinosis in 28 of 54 cases, whereas CT detected the disease in 14 of 54 cases. For 36 patients, the diagnosis could be verified by histologic examination of peritoneal biopsies or laparotomy. In this group, minilaparoscopy detected peritoneal carcinosis in 25 of 36 cases, whereas CT detected the disease in 12 of 36 cases. CONCLUSIONS: Minilaparoscopy was more sensitive than CT in detecting peritoneal carcinosis (100% vs 47.8%; p < 0.01). Considering its low grade of invasiveness and superior sensitivity, minilaparoscopy should be regarded as the procedure of choice for the early detection of peritoneal carcinosis.

Minilaparoscopy in the diagnosis of cirrhosis: superiority in patients with Child-Pugh A and macronodular disease.

BACKGROUND AND STUDY AIMS: The diagnosis of cirrhosis has prognostic and therapeutic implications, but early forms are difficult to diagnose. Laparoscopy with histology has been reported to be superior to histology alone, but is often considered to be too invasive. This study aimed to assess whether minilaparoscopy offers similarly high sensitivity coupled with only minor invasiveness. PATIENTS AND METHODS: Minilaparoscopy with biopsy was performed in 226 consecutive patients with chronic liver disease. Cirrhosis was diagnosed macroscopically primarily on the basis of nodularity in a nontumorous liver. A histological diagnosis using the modified Knodell score was made without knowledge of the macroscopic assessment. RESULTS: Biopsies from 22 patients were inadequate for histological assessment, and 16 of these were considered to be cirrhotic from macroscopic observation. Out of 204 liver biopsies, 94 (46 %) were macroscopically identified as cirrhotic; 68/204 (33 %) showed stage 5 or 6 fibrosis (incomplete or complete cirrhosis). Histological understaging occurred mainly in patients who were otherwise diagnosed as having early Child-Pugh A cirrhosis, macroscopically incomplete cirrhosis and macronodular cirrhosis; 4/204 (2 %) of patients with cirrhosis histologically were understaged macroscopically. CONCLUSIONS: Macroscopic evaluation during minilaparoscopy increases the sensitivity of detection of liver cirrhosis, compared with biopsy alone, by more than 30 %. Because of its minimal invasiveness, minilaparoscopy combined with biopsy is recommended as a superior method for the staging of chronic liver disease.

Minilaparoscopy-guided spleen biopsy in systemic disease with splenomegaly of unknown origin.

With the advent of a minimally invasive laparoscopy technique, the advantages of diagnostic laparoscopy are being rediscovered. We report here on four patients with systemic disease of unknown origin and splenomegaly, in whom minilaparoscopy-guided splenic biopsy yielded a definitive diagnosis. Four patients with unclear systemic disease were studied using diagnostic minilaparoscopy and guided spleen biopsy, after failure of diagnostic work-up. Minilaparoscopic spleen biopsy revealed the diagnosis of a B-cell non-Hodgkin's lymphoma in two cases. In one patient, who had a history of Still's disease, the spleen biopsy showed granulocytic infiltration in the spleen typical of an acute episode of Still's disease. One patient with a known immunodeficiency syndrome (stage C III) showed multiple hypodense lesions in the spleen. Biopsy allowed a diagnosis of mycobacterial infection, with identification of Mycobacterium tuberculosis. No major complications occurred in any of the four cases; post-biopsy bleeding was observed in three of the four, but was easily managed by argon plasma coagulation or application of fibrin glue, or both. We recommend the use of spleen biopsy as a diagnostic tool in splenopathy of unknown origin if previous diagnostic methods have failed to yield a definitive diagnosis.

[Safety and value of minilaparoscopy in high risk patients]. / Sicherheit und Wertigkeit der Minilaparoskopie bei Hochrisikopatienten.

INTRODUCTION: Coagulopathies and thrombocytopenia may constitute contraindications for percutaneous liver biopsy. We investigated the safety and value of visually guided liver biopsy using minilaparoscopy in patients with coagulation disorders. PATIENTS AND METHODS: We studied 50 patients requiring a liver biopsy, but whose risk of severe bleeding complications was considered too high for the following reasons: INR > 1.5 (40%), platelets 50/nl (36%) or both (18%), other coagulopathies (6%). Indications for liver biopsy were: Hepatopathy of unknown etiology (38%), fulminant liver failure (18%), virus induced hepatitis (6%) and evaluation for liver transplantation (38%). Patients underwent minimally invasive diagnostic laparoscopy and liver biopsies were obtained with a Silverman or Menghini needle. Bleeding was stopped or prevented by coagulation with the argon beamer or a monopolar probe or application of fibrin glue. RESULTS: Macroscopical assessment of the liver was possible in all patients. A liver biopsy was performed in 47/50. 46/47 biopsy specimens were large enough to allow reliable histological evaluation. The diagnostic procedure had major therapeutic consequences in 35/40 patients. No relevant bleeding from the liver biopsy site occurred. CONCLUSION: We demonstrated that laparoscopically guided liver biopsy is safe even in patients with a very high risk of bleeding complications because of coagulation disorders. It is therefore an attractive and preferable alternative to transjugular liver biopsy.

[Budd-Chiari syndrome--a rare manifestation of hereditary thrombophilia]. / Budd-Chiari Syndrom--eine seltene Manifestation der hereditären Thrombophilie.

Budd-Chiari syndrome is a rare manifestation of hereditary or acquired thrombophilia. We saw a case of Budd-Chiari syndrome in a 30-year-old woman leading to initial diagnostic difficulties. She underwent surgical side-to-side shunt and 9 weeks later an almost normal liver could be demonstrated on computerized tomography. Budd-Chiari syndrome should be considered if the Chiari triad with abdominal pain, hepatomegaly and ascites occurs in a patient. If necessary, invasive diagnostic procedures (e.g. angiography) must be performed. Therapeutic options are anticoagulative therapy and porto-systemic shunt, either as a TIPS or a surgical shunt. If severe liver failure occurs or liver cirrhosis is present, orthotopic liver transplantation is an additional option which also cures hereditary thrombophilia.

Combined therapy with azathioprine, prednisolone, and ursodiol in patients with primary sclerosing cholangitis. A case series.

BACKGROUND: No established medical therapy alters the progressive course of primary sclerosing cholangitis. OBJECTIVE: To explore the potential usefulness of combined therapy with azathioprine, steroids and ursodeoxycholic acid (UDCA) in primary sclerosing cholangitis. DESIGN: Case series. SETTING: University hospital in Mainz, Germany. PATIENTS: 15 patients with primary sclerosing cholangitis. INTERVENTION: Azathioprine (1 to 1.5 mg/kg of body weight per day), prednisolone (1 mg/kg per day initially, tapering to 5 to 10 mg per day) and UDCA (500 to 750 mg per day). MEASUREMENTS: Clinical and laboratory evaluation, liver biopsy, and endoscopic retrograde cholangiography (a >30% change in stenosis was considered significant). RESULTS: After a median observation period of 41 months (range, 3 to 81 months), liver enzyme levels declined significantly in all patients. Six of 10 patients with follow-up liver biopsies showed histologic improvement. Significant radiographic deterioration was seen in only 1 of 10 patients who had endoscopic retrograde cholangiography. In 7 patients previously treated with UDCA alone, liver enzyme levels declined significantly only after immunosuppressive therapy was added. Adverse drug reactions led to the withdrawal of study medications in 2 patients. CONCLUSIONS: Combined immunosuppressive therapy may alter the progression of primary sclerosing cholangitis. Our observations suggest a benefit from adding immunosuppressive drugs to UDCA therapy. A randomized trial is warranted.

Safety and feasibility of a new minimally invasive diagnostic laparoscopy technique.

BACKGROUND AND STUDY AIMS: Laparoscopy combined with guided liver biopsy offers many advantages in the diagnosis and staging of chronic liver diseases and is superior to other diagnostic procedures. We developed a new minilaparoscopic technique and evaluated the utility of this minimally invasive laparoscopic system in the first 320 patients who underwent diagnostic assessment for liver disease or peritoneal carcinosis. PATIENTS AND METHODS: Between July 1996 and February 1998, minilaparoscopy, with analgesia and sedation was carried out in 320 patients. It was done using a 1.9-mm optical instrument, which was inserted through the same 2.75-mm trocar as the Veress needle used for inflating the pneumoperitoneum. Thus only a single puncture of the peritoneum was required. Liver biopsies, when indicated, were obtained under laparoscopic control with the Silverman needle through a short 2-mm additional trocar when the Menghini technique was used. RESULTS: Complication rates, patient discomfort and duration of procedure were extremely low with minilaparoscopy. We observed no serious complications, two complications that could be treated conservatively and technical difficulties in eight of 320 patients, which prevented liver biopsy in 2.8%. These minor difficulties all happened during the first 40 procedures, whereas after the initial 40 examinations of each investigator no further difficulties arose. CONCLUSIONS: This new minilaparoscopic technique allows a macroscopic and histological diagnosis of liver disease with minimal invasiveness, easy handling, excellent patient tolerance, and also a high degree of safety in patients with coagulation defects. Exploratory laparoscopy is an accurate and safe method for intra-abdominal diagnosis of liver diseases and peritoneal carcinosis.

Post-infantile giant cell hepatitis in patients with primary sclerosing cholangitis and autoimmune hepatitis.

In post-infancy, multinucleated giant cell hepatitis is rare. Various conditions and diseases associated with post-infantile giant cell hepatitis have been described, but the pathogenesis remains unknown. In this paper we review the case reports of four patients (3 male, 1 female; aged 22 to 32 years) with primary sclerosing cholangitis and autoimmune hepatitis. The follow-up ranges from five to seven years. All patients showed cholestasis and repeated elevation of hepatic transaminases. Patients with viral infections, metabolic disorders and toxic influences were excluded. Histopathology of liver tissue in all four patients revealed giant cell formation with up to 20 nuclei in 20-70% of all hepatocytes. Post-infantile giant cell hepatitis was defined histopathologically. The clinical course of all four patients markedly improved after immunosuppressive treatment. Further improvement was observed with the addition of ursodeoxycholic acid. Follow-up liver biopsies during treatment showed reduced inflammation and a decreased number of giant cells. One patient, who initially was admitted to the hospital with liver cirrhosis died five years later due to a sepsis. The clinical course of the other three patients remained stable during the observation period, and no progression of liver fibrosis was recorded as long as immunosuppressive treatment was continued. Cholestasis and autoimmunity seem to be two important mechanisms triggering hepatic giant cell formation in post-infancy. In the reported cases long-lasting cholestasis in primary sclerosing cholangitis together with features of autoimmune hepatitis seem to have triggered the formation of syncytial hepatic giant cells.

Drug-induced hepatitis: a rare complication of oral anticoagulants.

Hepatotoxicity is a rare complication of coumarin anticoagulants. We present the case of a 56-year-old woman who developed a viral-hepatitis-like picture 8 months after mitral valve replacement and oral anticoagulation. Phenprocoumon-induced hepatitis was diagnosed after positive reexposure and improvement following withdrawal of the drug. There appeared to be cross-reactivity to warfarin since this drug led to a similar increase in alkaline phosphatase and gamma-glutamyl transferase after a few days of administration. Liver biopsy showed an acute viral-hepatitis-like picture. Anticoagulation was changed to a subcutaneous low molecular weight heparin and low-dose aspirin. Because of the widespread use of coumarin anticoagulants, physicians should be aware of the hepatotoxic potential of these drugs, which most frequently mimics the clinical presentation of viral hepatitis.

Rheumatoid nodules located in the penis of a methotrexate-treated patient with rheumatoid arthritis.

A case is reported in which a patient with a rheumatoid factor-negative rheumatoid arthritis developed rheumatoid nodules in the penis during treatment with methotrexate. The development of rheumatoid nodules in seronegative rheumatoid arthritis patients is extremely rare. An acceleration of rheumatoid nodules in methotrexate-treated rheumatoid arthritis patients is reported in literature. Regarding the case reported here, we propose a causal relationship between methotrexate-treatment and the development of rheumatoid nodules in our patient. Methotrexate should not be the preferential treatment for patients with rheumatoid arthritis developing rheumatoid nodules and suffering from vasculitis.