Visual outcomes and predictors in optic pathway glioma: a single centre study.

BACKGROUND/AIMS: Optic pathway gliomas (OPGs) may cause progressive visual loss despite chemotherapy. Newer, less toxic treatments might be given earlier, depending on visual prognosis. We aimed to investigate the prognostic value of visual evoked potentials (VEP) and optical coherence tomography (OCT). METHODS: A retrospective study of OPG patients (treated 2003-2017) was conducted. Primary outcome was PEDIG category visual acuity in better and worse eyes (good < = 0.2, moderate 0.3-0.6 and poor > = 0.7 logMAR). Binary logistic regression analysis was used to identify predictors of these outcomes. RESULTS: 60 patients (32 Neurofibromatosis type 1 [NF1] and 28 sporadic) had median presentation age 49 months (range 17-183) (NF1) and 27 months (range 4-92) (sporadic). Median follow up was 82 months (range 12-189 months). At follow up 24/32 (75%) of NF1 children and 14/28 (50%) of sporadic children had good better eye visual acuity and 11/32 (34%) of NF1 children and 15/28 (54%) of sporadics had poor worse eye acuity. Mean peripapillary retinal nerve fibre layer (RNFL) thickness predicted good better eye final acuity (OR 0.799, 95%CI 0.646-0.987, p = 0.038). Presenting with visual symptoms (OR 0.22 95% CI 0.001-0.508, p = 0.017) and poorer VEP scores (OR 2.35 95% CI 1.1-5.03, p = 0.027) predicted poor worse eye final acuity. 16 children had homonymous hemianopias at follow up, predicted by poor presenting binocular VEP score (OR 1.449 95%CI 1.052-1.995, p = 0.02). CONCLUSIONS: We found that both RNFL thickness on OCT and VEP were useful in predicting future visual acuity and vision and potentially in planning treatment. We had a high prevalence of homonymous hemianopia.

Unusual neuroimaging in a young boy with cerebral X-linked adrenoleukodystrophy.

We describe the case of a 7-year-old boy with cerebral X-linked adrenoleukodystrophy presenting with neurobehavioral symptoms. Attention deficit with hyperactive behavior was followed by progressive memory retrieval deficits and difficulty in writing. A detailed review of the clinical history, clinical examination and contrast-enhanced brain nuclear magnetic resonance imaging disclosed indeed an atypical cerebral form of X-linked adrenoleukodystrophy with diffuse anterior and posterior white matter involvement with a typical rim of contrast enhancement. A severe clinical deterioration occurred despite dietary treatment and hormonal replacement therapy. These rare but severe forms of atypical cerebral X-linked adrenoleukodystrophy can more often mimic attention deficit/hyperactivity disorder and should, therefore, be considered in young males with progressive neurobehavioral symptoms, particularly when possibly related to frontal white matter involvement. An accurate clinical history and examination should determine whenever a contrast-enhanced head MRI should be indicated.

Bariatric surgery in obesity: changes of glucose and lipid metabolism correlate with changes of fat mass.

BACKGROUND AND AIM: Bariatric surgery induces significant weight loss and improves glucose metabolism in obese patients (BMI>35 kg/m(2)). Our aim was to compare restrictive (LAGB, laparoscopic gastric banding) and malabsorptive approaches (BIBP, biliary-intestinal bypass) on the loss of fat-free mass (FFM), fat mass (FM), and on changes of glucose and lipid metabolism. METHODS AND RESULTS: Body composition (bio-impedance analysis, BIA), blood glucose (BG), insulin, triglycerides, total- and HDL-cholesterol, liver enzymes (AST and ALT) were measured at baseline and 1 year after surgery in patients undergoing LAGB, BIBP, and in diet-treated control patients. In the main study, with patients matched for initial BMI (43-55 kg/m(2), LAGB=24, BIBP=12, controls=6), decreases of BMI, FM, BG and cholesterol were greater in patients with BIBP than with LAGB (p<0.01), while decreases of FFM, insulin, HOMA-IR and triglycerides were similar. No effects on BMI, FM, FFM, BG, insulin, HOMA-IR or cholesterol were observed in the control patients. Decreases of BG, insulin, HOMA-IR, cholesterol and triglycerides correlated with FM but not with FFM decrease. Similar results were obtained in an additional study in patients with a different initial BMI (LAGB=25, BIBP=6, controls=24) and when considering all subjects together. A decrease of liver enzymes (ALT) was greater with LAGB than with BIBP, and HDL-cholesterol increased with LAGB and decreased with BIBP. CONCLUSION: BMI, FM, BG and cholesterol decrease more with malabsorptive than with restrictive surgery, while FFM, insulin, HOMA-IR and triglycerides decrease in a similar way. FFM loss is of low entity. Changes of glucose and lipid metabolism are proportional to a decrease of fat mass but not of fat-free mass.

Laparoscopic liver resections for hepatocellular carcinoma. Is it a feasible option for patients with liver cirrhosis?

BACKGROUND: Laparoscopic surgery has gained growing acceptance, but this does not hold for laparoscopic surgery of the liver, above all for patients with hepatocellular carcinoma (HCC) and cirrhosis. This approach mainly includes diagnostic procedures and interstitial therapies. However, we believe there is room for laparoscopic liver resections in well-selected cases. The aim of this study is to assess: (a) the risk of intraoperative bleeding and postoperative complications, (b) the safety and the respect of oncological criteria, and (c) the potential benefit of laparoscopic ultrasound in guiding liver resection. METHODS: A prospective study of laparoscopic liver resections for hepatocellular carcinoma was undertaken in patients with compensated cirrhosis. Hepatic involvement had to be limited and located in the left or peripheral right segments (segments 2-6), and the tumor had to be 5 cm or smaller. Tumor location and its transection margins were defined by laparoscopic ultrasound. RESULTS: From January 1997, 22 out of 250 patients with HCC (9%) underwent laparoscopic liver resections. The mean patient age was 61.4 years (range, 50-79 years). In three patients, conversion to laparotomy was necessary. The laparoscopic resections included five bisegmentectoies (2 and 3), nine segmentectomies, two subsegmentectomies and three nonanatomical resections for extrahepatic growing lesions. The mean operative time, including laparoscopic ultrasonography, was 199 +/- 69 min (median, 220; range, 80-300). Perioperative blood loss was 183 +/- 72 ml (median, 160; range, 80-400 ml). There was no mortality. Postoperative complications occurred in two out of 19 patients: an abdominal wall hematoma occurred in one patient and a bleeding from a trocar access in the other patient requiring a laparoscopic re-exploration. Mean hospital stay of the whole series was 6.5 +/- 4.3 days (median, 5; range, 4-25), while the mean hospital stay of the 19 laparoscopic patients was 5.4 +/- 1 (median, 5; range, 4-8). CONCLUSION: Laparoscopic treatment should be considered in selected patients with HCC and liver cirrhosis in the left lobe or segments 5 and 6 of the liver. It is clear that certain types of laparoscopic resection are feasible and safe when carried out by adequately skilled surgeons with appropriate instruments.

Laparoscopic radiofrequency of hepatocellular carcinoma using ultrasound-guided selective intrahepatic vascular occlusion.

BACKGROUND: The optimal treatment for hepatocellular carcinoma (HCC) is surgical resection. However, only a small percentage of patients are operative candidates. The authors aimed to assess a novel operative combination of laparoscopic radiofrequency (LRF) with a selective intrahepatic vascular occlusion (SIHVO) to obtain an increased rate of total necrosis and a reduced rate of local HCC recurrences. METHODS: For this study, 37 patients with HCC in liver cirrhosis were submitted to LRF with SIHVO. An LRF was indicated for patients not amenable to liver resection who evidenced at least one of the following criteria: severe impairment of the coagulation tests, large tumors (but <5 cm) or multiple lesions requiring repeated punctures, superficial lesions adjacent to visceral structures, deep-sited lesions with a very difficult or impossible percutaneous approach, and short-term recurrence of HCC after percutaneous loco-regional therapies. RESULTS: Laparoscopic ultrasound identified seven new malignant lesions (19%) undetected by preoperative imaging. There was no operative mortality. Of the 37 patients, 31 experienced no complications (84%). Computed tomography (CT) evaluation 1 month after treatment showed that a complete response with 100% necrosis had been achieved for all the patients (100%). During the follow-up period (mean, 11.8 +/- 8.2 months), new malignant nodules developed in 14 patients (42%), and 36% of these recurrences were located in the same treated segment of the HCC. CONCLUSIONS: The combined LRF and SIHVO procedure proved to be a safe and effective technique at least in the short and mid term. In fact, it permitted the treatment of lesions not treatable using the percutaneous approach with a complete clearance, and it had a low morbidity rate.

Impact of intraoperative ultrasonography in laparoscopic liver surgery.

BACKGROUND: Laparoscopic surgery has gained growing acceptance, but this does not hold for laparoscopic surgery of the liver. This mainly includes diagnostic procedures, interstitial therapies, and treatment of liver cysts. However, the authors believe there is room for a laparoscopic approach to the liver in selected cases. METHODS: A prospective study of laparoscopic liver resections was undertaken with patients who had preoperative diagnoses of benign lesion and hepatocellular carcinoma with compensated cirrhosis. The inclusion criteria required that hepatic involvement be limited and located in the left or peripheral right segments (segments 2-6), and that the tumor be 5 cm or smaller. The location of the tumor and its transection margin were defined by laparoscopic ultrasound (LUS). RESULTS: From December 1996, 17 (5%) of 313 liver resections were included in the study. There were 5 benign lesions and 12 hepatocellular carcinomas in cirrhotic patients. The mean age of the study patients was 59 years (range, 29-79 years). The LUS evaluation identified the presence of new hepatocellular carcinoma nodules in two patients (17%). The resections included 1 bisegmentectomy, 8 segmentectomies, 3 subsegmentectomies, and 3 nonanatomic resections. The mean operative time, including laparoscopic ultrasonography, was 156 +/- 50 min (median, 150 min; range, 60-250 min), and the perioperative blood loss was 190 +/- 97 ml. There was no mortality. Conversion to laparotomy was necessary for two patients. Postoperative complications were experienced by 3 of 15 patients, all of them cirrhotics. One of the patients had a wall hematoma, and the remaining two patients had bleeding from a trocar access requiring a laparoscopic reexploration. The mean hospital stay for the whole series was 6.9 +/- 4.9 days (median, 6 days; range, 2-25 days) and 5.6 +/-1.4 days (median, 6 days; range, 2-8 days) for the 15 laparoscopic patients. CONCLUSION: Laparoscopic treatment should be considered for selected patients with benign and malignant lesions in the left lobe or frontal segments of the liver. Evaluation by LUS is indispensable to guarantee precise determination of the segmental tumor location and the relationship of the tumor to adjacent vascular or biliary structures, excluding adjacent or adjunctive new lesions. The evolution of laparoscopic hepatectomies probably will depend on the development of new techniques and instrumentations.

Laparoscopic ultrasound-guided resection of gastric submucosal tumors.

BACKGROUND: The laparoscopic resection of gastric stromal tumors is being performed with increased frequency. Wedge resection of anterior wall lesions is generally performed. The treatment of posterior wall lesions is still controversial. METHODS: We report three cases of gastric submucosal tumors treated by a laparoscopic wedge resection of the stomach. All lesions were localized anterior gastric wall by intraoperative ultrasound on the. In the first patient the resection was performed with an endoscopic stapler; in the other patients, ultrasonic coagulation in association with an intracorporeal suture has been used. RESULTS: All patients were successfully treated laparoscopically; there were no conversions to open surgery. In all cases the operative course was uneventful. The postoperative hospital stay ranged from 6 to 8 days. CONCLUSIONS: The results suggest that laparoscopic surgery is an adequate strategy for gastric submucosal neoplasms including gastrointestinal stromal tumors (GIST). Intraoperative ultrasound is very useful in the selection of the technical approach with or without the endoscopic stapler.

Portacaval shunt in the treatment of primary Budd-Chiari syndrome.

We have performed a side-to-side portacaval shunt as the treatment of choice in six cases of primary Budd-Chiari syndrome. All the patients (three men and three women, mean age 33 years) had marked hepatomegaly and massive ascites. Four patients had endoscopically proved esophageal varices with no episodes of bleeding. Preoperative angiographic studies, caval pressure measurement, laparoscopy, liver biopsy specimens, liver scanning, and liver function tests confirmed the diagnosis. A possible etiologic factor was evident in only two cases. A plain side-to-side portacaval shunt was performed in four patients, while two required the interposition of a graft. One patient died after surgery of hepatorenal syndrome. Four of the surviving patients are free of ascites and doing well at 29, 27, 25, and 6 months. The remaining patient subsequently developed cirrhosis and died 76 months after surgery. None of the patients who survived developed encephalopathy. Shunt patency was confirmed endoscopically by variceal decompression in the four patients with esophageal varices. We believe the side-to-side portacaval shunt is a reliable and effective procedure for the definitive management of primary budd-Chiari syndrome.

Factors predicting chronic hepatic encephalopathy after distal splenorenal shunt: a multivariate analysis of clinical and hemodynamic variables.

BACKGROUND: This study was aimed at evaluating several factors that promote chronic hepatic encephalopathy by multivariate analysis of data for patients with cirrhosis with good or moderate liver function submitted to distal splenorenal shunts. METHODS: The study group comprised 131 patients: 55 had alcoholic and 76 nonalcoholic cirrhosis. Seventy patients were in Child's class A and 61 in class B. Cerebral function was assessed by a complete neurologic examination. Angiography with venous phase was performed before and within 1 month after the shunt operation. In 84 cases the original Warren technique was used and in 20 cases a Britton's modified procedure was used. Twenty-seven patients had distal splenorenal shunts with a splenopancreatic disconnection. Statistical analysis was performed by two multivariate analyses based on stepwise selection. RESULTS: Thirty-nine patients died during a follow-up period of 51 +/- 32 months. Chronic encephalopathy occurred in 18 patients (14%). According to the multivariate analysis of the preoperative prognostic factors, only age (p = 0.0001) and albumin values (p = 0.0002) were independent predictive risk factors for chronic encephalopathy. In the multivariate analysis concerning the hemodynamic consequences of the selective shunts, independent risk factors promoting chronic encephalopathy were postoperative portal perfusion (p = 0.0001), postshunt portal pressure (p = 0.001), and surgical disconnection (p = 0.0064). CONCLUSIONS: Our study has shown that chronic encephalopathy after selective shunt surgery is promoted by both clinical and hemodynamic factors. A better selection of the candidates for shunt surgery and prevention of the development of portal malcirculation by accurate surgical disconnection should further decrease the risk of chronic encephalopathy.

Common bile duct exploration and laparoscopic cholecystectomy: role of intraoperative ultrasonography.

BACKGROUND: In October 1993, to detect associated common bile duct (CBD) stones, we started an evaluation program of patients with symptomatic cholelithiasis who were candidates for laparoscopic cholecystectomy. STUDY DESIGN: We used a standard preoperative algorithm and a laparoscopic ultrasonographic (LUS) examination. Preoperative endoscopic retrograde cholangiopancreatography (ERCP) was reserved for high-risk patients for CBD stones. Laparoscopic ultrasonographic examination during cholecystectomy was routinely performed to identify stones unsuspected preoperatively. Two-hundred-sixteen patients with symptomatic cholelithiasis were included in the study; 177 patients (82%) were at low risk for choledocholithiasis and 39 patients (18%) were at high risk and had preoperative ERCP. In 17 patients (43.5%) CBD stones were found, and in 16 patients (41%) they were removed by endoscopic sphincterotomy. RESULTS: In all patients, the main intra- and extrahepatic ducts were well documented by LUS, but in eight cases the distal tract of the CBD was not well-visualized. In eight patients, small stones were found in the CBD. A subsequent peroperative cholangiography or CBD exploration confirmed the diagnosis. In one patient, both LUS and cholangiography suspected a small stone; the CBD exploration did not confirm it (false positive). In two patients a small stone in the CBD was found during the followup period (two false negatives). An endoscopic sphincterotomy solved the problem. CONCLUSIONS: Laparoscopic ultrasonographic examination may be a real alternative to cholangiography during laparoscopic cholecystectomy: this may be reserved for selected instances on the basis of LUS findings. On the other hand, considerable ultrasonographic experience is required for LUS to be performed successfully.

Laparoscopy with laparoscopic ultrasound for pretreatment staging of hepatocellular carcinoma: a prospective study.

Laparoscopy with laparoscopic ultrasound (L-LUS) has proved to be superior to conventional CT imaging in the staging of hepatocellular carcinoma (HCC). The aim of our prospective study was to evaluate the efficacy of L-LUS as compared with currently available imaging techniques (spiral CT or Lipiodol CT) in patients with HCC and liver cirrhosis. From January 1998 to May 2000, 70 consecutive patients (50 men and 20 women; mean age 67 +/- 7 years) were enrolled. Liver cirrhosis was related to chronic hepatitis C virus infection in 55, hepatitis B virus infection in seven, and alcohol abuse in eight patients. Preoperative diagnostic workup included the following: 70 ultrasound examinations of the liver, 23 CT scans after Lipiodol arteriography, and 53 spiral CT scans. A single lesion was found in 39 patients, two lesions in 20 patients, and three lesions in 11 patients. L-LUS was performed in all patients under general anesthesia using a two- to three-trocar technique. The examination was completed in 68 patients (97%); in two cases extensive adhesions prevented the L-LUS examination. L-LUS yielded additional information in 39 patients (57%). New histologically proved HCC lesions were detected in 14 patients (in the same liver segment in 4 cases and in different liver segments in 10 cases), and an adrenal metastasis was seen in one patient. In 23 patients, benign nodules were identified as regenerative macronodules, low-grade dysplastic nodules, or small hemangiomas. In 10 patients, correct localization of the primary lesion was detected by L-LUS in comparison with the preoperative liver location. In our experience, L-LUS is a safe and reliable procedure. It provides superior information (intraoperative histologic confirmation) for the diagnosis and pretreatment staging of HCC in patients with cirrhosis when compared with current radiologic imaging techniques.

Selective distal splenorenal shunt versus side-to-side portacaval shunt. Clinical results of a prospective, controlled study.

A prospective, controlled study comparing the clinical results of the selective distal splenorenal shunt procedure and the side-to-side portacaval shunt procedure was undertaken in 1980. Ninety-three cirrhotic patients with previous episodes of bleeding from esophageal varices underwent a distal splenorenal shunt procedure (47 patients). The operative mortality rate was 2 percent in both groups. The intraoperative decrease of portal hypertension after the portacaval shunt procedure was higher than after the distal splenorenal shunt procedure (p less than 0.05), and in those with patent shunts, there was a 0 percent incidence of early variceal rebleeding after the portacaval shunt procedure compared with a 9 percent incidence after the distal splenorenal shunt procedure (p less than 0.05). Both shunts, however, had similarly satisfactory results in preventing long-term variceal rebleeding (portacaval shunt 2 percent and distal splenorenal shunt 0 percent). Postoperative ascites was more common after the distal splenorenal shunt procedure (58 percent versus 24 percent; p less than 0.01). Analysis of actuarial survival curves showed no difference between the two procedures. The incidences of long-term episodes of chronic encephalopathy were not statistically different after both procedures. The only three instances of severe encephalopathy occurred in patients with the portacaval shunt (p less than 0.05). The distal splenorenal shunt also seemed to have a less negative effect on postoperative liver function than the portacaval shunt. These data suggest that the selective shunt should be viewed as a first choice strategy in the treatment of portal hypertension.

Isolated splenic metastasis from gastric carcinoma: value of CEA and CA 19-9 in early diagnosis: report of two cases.

We report two cases of an isolated splenic metastasis, occurring 5 and 3 years, respectively, after gastrectomy for gastric carcinoma. Serum carcinoembryonic antigen and CA 19-9 levels were evaluated preoperatively, postoperatively, and during the oncologic follow-up. The patients underwent splenectomy for solitary splenic metastasis. We conclude that the use of serum carcinoembryonic and CA 19-9 values may help in the early diagnosis of these recurrences and splenectomy allows radical treatment in patients with no evidence of disseminated disease.

Angioscintigraphic assessment of arterial and portal liver blood flow: comparison with splanchnic angiography.

In order to validate an angioscintigraphic method for the evaluation of the components of hepatic blood flow, the results of angioscintigraphy were compared with splanchnic angiography in 25 cirrhotics. Two indices of hepatic portal perfusion and a splenohepatic arterial index were calculated. These were correlated with portal diameter and hepatic artery diameter, respectively. No correlation was found between grades of portal perfusion according Nordlinger's criteria and these indices. The method is very simple and could be applied to evaluate the changes of liver hemodynamics induced by drugs or shunt surgery.

Laparoscopic radiofrequency of hepatocellular carcinoma (HCC) in liver cirrhosis.

BACKGROUND/AIMS: In this report, the feasibility and efficacy of laparoscopic radiofrequency interstitial thermal ablation of hepatocellular carcinoma has been evaluated in 20 patients. METHODOLOGY: The laparoscopic approach with the use of intraoperative ultrasonography allowed us to obtain additional information regarding liver nodules and a complete treatment of the liver lesions. RESULTS: The complication rate was low and there was no operative mortality. A complete necrosis has been obtained in 90% of the cases at 1 month dynamic computed tomography following the treatment. CONCLUSIONS: Laparoscopic radiofrequency thermal ablation of hepatocellular carcinoma proved to be a safe and effective technique; its use may be proposed in selected patients. Larger series are needed to accurately assess its role among the other ablative therapies of hepatocellular carcinoma.

Angioscintigraphic assessment of hemodynamic effects of penbutolol in cirrhotics with portal hypertension. A double-blind, randomized, controlled study.

This randomized double-blind controlled study analyzed the hemodynamic effects of penbutolol, a new levorotatory beta-blocker, using radionuclide angiography. Twenty cirrhotics with esophageal varices were randomized to two groups: 10 received 40 mg/day of penbutolol orally and the others placebo. Angioscintigraphy was performed before and after an 8-day treatment period. Three cases in the penbutolol group were lost due to software damage, hence the data of 17 patients were analyzed. The two groups were matched for age, sex, etiology of cirrhosis and hepatic function. The index of portal perfusion decreased significantly (-29%; p = 0.018) and the hepatic artery index increased significantly (+23%; p = 0.018), while no changes were observed after placebo. The heart rate decreased significantly after penbutolol (-9%; p = 0.028), while neither penbutolol nor placebo modified the ejection fraction. In conclusion, penbutolol decreased portal perfusion index (the compensatory increase in the hepatic artery index confirmed this change) without major modification of total hepatic blood flow or systemic hemodynamics. Angioscintigraphy is reasonably accurate, reproducible, safe and can be considered suitable for routine use in the assessment of liver hemodynamics.

Prevalence and laparoscopic ultrasound patterns of choledocholithiasis and biliary sludge during cholecystectomy.

A prospective study of laparoscopic ultrasound (LUS) for evaluation of the common bile duct during laparoscopic cholecystectomy was started in October 1993. LUS during cholecystectomy was performed routinely to preoperatively identify unsuspected stones. Three-hundred thirty patients with symptomatic cholelithiasis were included in the study. The preoperative work-up included endoscopic retrograde cholangiopancreatography in 49 patients. Common bile duct (CBD) stones were found in 22 cases; in 19 cases the stones were removed successfully by endoscopic sphincterotomy. LUS successfully visualized the CBD in all but 10 patients (3%). CBD stones were found in 17 patients and confirmed by preoperative cholangiography and/or CBD exploration. There were two false-negative and one false-positive result for LUS. In 47 patients (14.2%) LUS detected CBD sludge as low-amplitude echoes without acoustic shadowing. The presence of CBD sludge was correlated with some biochemical and clinical variables. A significant correlation was identified between the absence or presence of CBD sludge and endoscopic retrograde cholangiopancreatography, acute pancreatitis, gallbladder sludge, age, and the levels of serum bilirubin and alkaline phosphatase. A significant difference was recorded between CBD diameter and the presence or absence of stones or sludge (p = 0.00001). In our experience, LUS allowed good diagnosis of CBD stones during laparoscopic cholecystectomy. The clinical significance of CBD sludge remains to be elucidated.

[Diagnosis of secondary tumors of the pancreas. Analysis of 13 cases]. / La diagnosi dei tumori secondari del pancreas. Analisi di 13 casi.

The Authors report their experience of 13 cases of metastatic carcinoma of the pancreas, diagnosed over 350 pancreatic cancers examined by digestive angiography (4%). The rarity of this localization is emphasized and is affirmed that any organ may cause pancreatic metastasis (liver, bile ducts, oesofagus) although the kidney and the lung are more frequently the original sites. The pancreatic metastases were of different types but the multilocalized or the total forms don't prevail over the single ones. The angiographic modifications induced by these secondary lesions are evident but not characteristic and diagnosis only of pancreatic tumor can be evocated. Therefore digestive angiography is of high diagnostic value in this cases, although histologic examination is necessary to define the metastatic nature of these lesions.