Jacques Pringot

Adult polycystic liver disease: is fenestration the most adequate operation for long-term management?

OBJECTIVE The aim of this study was to evaluate the immediate and long-term results in a retrospective series of patients with highly symptomatic adult polycystic liver disease (APLD) treated by extensive fenestration techniques. A classification of APLD was developed as a stratification scheme to help surgeons conceptualize which operation to offer to patients with APLD. SUMMARY BACKGROUND DATA Treatment options for APLD remain controversial, with partisans of fenestration techniques or combined liver resection-fenestration. METHODS Clinical symptoms, performance status, liver volume measurement by computed tomography (CT), and morbidity were recorded before surgery and after surgery. Adult polycystic liver disease was classified according to the number, size, and location of liver cysts and the amount of remaining liver parenchyma. Follow-up was obtained by clinical and CT examinations in all patients. RESULTS Ten patients with highly symptomatic APLD were operated on using an extensive fenestration technique (by laparotomy in 8 patients and by laparoscopy in 2 patients, 1 of whom conversion to laparotomy was required). The mean preoperative liver volume was 7761 cm3. There was no mortality. Postoperative morbidity occurred in 50%, mainly from biliary complications, requiring reintervention in two cases. Massive intraoperative hemorrhage occurred in one patient. During a mean follow-up time of 71 months (range, 17 to 239 months), all patients were improved clinically according to their estimated performance status. The mean postoperative liver volume was 4596 cm3, which represents a mean liver volume reduction rate of 43%. However, in type III APLD, despite absence of clinical symptoms, a significant increase in liver volume was observed in 40% of the patients. CONCLUSIONS Extensive fenestration is effective in relieving symptoms in patients with APLD. Hemorrhage and biliary complications are possible consequences of such an aggressive attempt to reduce liver volume. The procedure can be performed laparoscopically in type I APLD. A longer follow-up period is mandatory in type II APLD, to confirm the usefulness of the fenestration procedure. In type III APLD, significant disease progression was observed in 40% of the patients during long-term follow-up. Fenestration may not be the most appropriate operation for long-term management of all types of APLD.

Benign hepatocellular tumors: MRI after superparamagnetic iron oxide administration.

Objective Our goal was to describe the features of benign hepatocellular tumors on superparamagnetic iron oxide (SPIO)–enhanced MR images and to evaluate the potential role of SPIO administration in the diagnosis of these tumors. Materials and Methods The T1− and T2-weighted SE images were performed before and after SPIO administration in 19 patients with focal nodular hyperplasia (FNH) or adenomas. The lesions were counted and qualitatively assessed. In 32 tumors with a diameter of >10 mm, the contrast-to-noise ratio (CNR) and the liver and tumor enhancement were calculated. Results Thirty-seven tumors were detected on MR images: 29 before and 36 after SPIO administration (p < 0.05). The SPIO-enhanced T1-weighted images provided the highest detection rate and CNR. The central scar was most conspicuous on SPIO-enhanced T2-weighted images. Most FNHs and adenomas lost less signal than the liver, three tumors did not lose signal, and four FNHs showed a signal loss equal to or greater than that of the liver. Conclusion The SPIO-enhanced T1-weighted images increased the CNR and the detection rate of benign hepatocellular tumors. On T2-weighted images, most benign hepatocellular tumors showed a signal decrease after SPIO administration. No significant difference in signal loss was observed between FNH and adenomas.

Laparoscopic splenectomy in adults and children: experience with 31 patients.

BACKGROUND Open surgery is the standard approach for splenectomy in hematologic disorders, but a few cases of successful laparoscopic splenectomy have been reported. METHODS Thirty-one patients (18 adults, group 1; and 13 children, group 2) underwent laparoscopic splenectomy. Indications for surgery included idiopathic thrombocytopenic purpura (25 patients), congenital spherocytosis (4 patients), and hemolytic anemia (2 patients). In 97% of the patients the diameter of the spleen was less than 15 cm. RESULTS Laparoscopic splenectomy was successful in 94% of the patients; conversion to open surgery was mainly related to hemorrhage. Accessory spleen was found in 39% in group 1 and 8% in group 2. Two adults received intraoperative autotransfusion. Postoperative morbidity was minimal. The median postoperative stay was 3 days (range, 2 to 12 days) in group 1 and 2 days (range, 2 to 5 days) in group 2. CONCLUSIONS Laparoscopic splenectomy is safe in both adults and children. Adequate selection of patients (small-size spleen, splenic destruction on preoperative scanning of platelets), appropriate preparation in patients with idiopathic thrombocytopenic purpura (immunoglobulin G), and meticulous surgical technique (with routine opening of the gastrocolic ligament to search for accessory spleen) are key factors in obtaining the same long-term results as with open surgery.

Epithelioid hemangioendothelioma of the liver: MR and CT findings.

The MR imaging features in five patients with hepatic epithelioid hemangioendothelioma (EHE) were correlated with CT and pathologic findings. Two hemangioendotheliomas appeared as multiple nodular lesions with a predominantly peripheral location in the liver. In three more extensive cases, the tumors formed confluent peripheral lesions with macroscopic invasion of portal or hepatic veins (n = 3), signs of portal hypertension (n = 3), and nodular hypertrophy of uninvolved liver (n = 2). These findings, suggestive of EHE, were well demonstrated by MR imaging and CT. The internal architecture of the tumors was clearly depicted on T2-weighted MR images. Viable tumor peripheries appeared moderately hyperintense relative to liver. The center of the tumors consisted of one or several concentric zones. Hyperintense central zones were composed of loose, edematous connective tissue. Hypointense zones contained mainly coagulation necrosis, calcifications, and scattered hemorrhages. Except for the presence of calcifications, the internal architecture of EHE was better defined by MR imaging than by CT.

MRI of complicated anal fistulae: comparison with digital examination.

Objectives The assessment of complicated anal fistulae is based on digital examination. Our objective was to study the potential role of MRI in this setting by comparing preoperative digital examination with MRI. Materials and Methods Digital examination and MRI were prospectively and blindly interpreted in 13 patients with complicated anal fistulae. Surgical findings were used as standard. Results Complete agreement between the results of digital examination and the surgical findings was observed in 8 patients and between the results of MRI and the surgical findings in 12 patients. Digital examination misclassified two low transsphincteric fistulae as high transsphincteric and missed high secondary extensions or abscesses in three other patients. Magnetic resonance misclassified only one suprasphincteric fistula as low transsphincteric and did not overlook high secondary extensions or abscesses. Conclusion These findings suggest that MRI may show abnormalities not detected by preoperative digital examination.

Dynamic CT in pancreatic lymphoma

We retrospectively reviewed the dynamic CT examinations of eight patients with pancreatic lymphoma. Four tumors were rounded masses with well-defined contours, four were more infiltrating lesions. The median cross-sectional diameter of the tumors was 6 cm (range 2.5–12 cm). At dynamic CT, the tumors were hypodense (n = 8) and somewhat heterogeneous (n = 6). Additional features were enlarged lymph nodes, 1–3 cm in diameter (n = 5), dilatation of the biliary tract and pancreatic duct (n = 5), abnormalities in the fat around the celiac trunk and/or the superior mesenteric artery (n = 4), and venous stenosis or occlusion (n = 7). The CT findings of pancreatic lymphoma are more various than has been previously reported. Findings such as small tumor size, well-defined contours, tumor heterogeneity, pancreatic duct dilatation, and venous invasion may be seen. Pancreatic lymphoma cannot be reliably distinguished from pancreatic carcinoma by CT findings alone.

Epiploic appendagitis: color Doppler sonographic findings.

Abstract The aim of this study was to analyze the color Doppler sonographic findings in primary epiploic appendagitis. Color Doppler sonographic findings of ten patients with primary epiploic appendagitis were reviewed. The following sonographic features were analyzed: identification of a mass adjacent to the colonic wall; identification of spotty color areas with arterial flow in this mass and detection of abnormalities of the colonic wall adjacent to the infiltrated fatty tissue. A well-delineated hyperechoic mass adjacent to the colonic wall was detected in each patient with sonography. No colonic wall abnormalities were observed nor color Doppler signal in and around the hyperechoic area. Absence of flow at color Doppler sonography is an additional feature of epiploic appendagitis.

Acute intestinal ischemia due to occlusion of the superior mesenteric artery: detection with Doppler sonography.

The aim of our study was to assess the feasibility of using Doppler sonography for the detection of acute intestinal ischemia due to occlusion of the superior mesenteric artery. Between September 1993 and March 1995, abdominal sonography with Doppler imaging of the mesenteric vessels was performed in 770 patients with emergency admissions for acute abdominal pain. In six cases, the diagnosis, based on surgery or arteriography and computed tomography, was acute intestinal ischemia due to complete or localized occlusion of the superior mesenteric artery. Five cases of occlusion of the superior mesenteric artery were correctly detected by Doppler sonography. These results suggest that Doppler sonography may be a feasible method for detecting acute intestinal ischemia due to proximal superior mesenteric artery occlusion.

Inflammatory pseudotumor of the liver: MRI with mangafodipir trisodium.

Inflammatory pseudotumor of the liver is a rare benign mass that is often misdiagnosed as hepatocellular carcinoma at preoperative imaging. We report a case of inflammatory pseudotumor of the liver with special emphasis on the appearance at MRI after administration of mangafodipir trisodium. On T1-weighted images the lesion was slightly hypointense relative to the liver, and on T2-weighted images it was isointense with a hyperintense capsule. No tumor enhancement was seen on T1-weighted images after administration of mangafodipir trisodium. MRI with mangafodipir trisodium might help to distinguish inflammatory pseudotumor of the liver from hepatocellular carcinoma.

Familial adenomatous polyposis associated with multiple adrenal adenomas in a patient with a rare 3' APC mutation.

Familial adenomatous polyposis (FAP) is characterised by hundreds of colorectal adenomas. Endocrine neoplasms have occasionally been reported, as have gastric polyps, which are usually hamartomatous in the fundus of the stomach and adenomatous in the antrum. A 57 year old man with colorectal, gastric, and periampullary adenomatous polyposis, in association with three bilateral adrenocortical adenomas, is presented. Mutation screening showed a 5960delA germline mutation in the adenomatous polyposis coli (APC) gene predicted to lead to a premature stop codon. This mutation was found in three of the four children of the patient. Western blot analysis of a lymphoblastoid cell line derived from the patient failed to detect any truncated APC polypeptide. This rare 3′ mutation is responsible for an unusually complex and late onset phenotype of FAP.