Search

Cholangiocarcinoma is a rare disease, accounting for less than 2% of all human malignancies. 1 Although the entire biliary tree is potentially at risk, tumors involving the biliary confluence or the right or left hepatic ducts (hilar cholangiocarcinoma) are most common and account for 40% to 60% of all cases. 2–6 Meaningful clinical experience in managing hilar cholangiocarcinomas has been limited to a few referral centers because of the infrequency with which they are encountered. Although resection has long been recognized as the most effective therapy for hilar cholangiocarcinoma, 7 the importance of partial hepatectomy and the willingness of surgeons to use it routinely are relatively recent developments. 2,8–10 Many clinical series extend over a prolonged period, often greater than 20 years. 4,5,9,11,12 As a result, these reports lack a uniform approach to diagnosis, assessment of disease extent, and resection, and the results are therefore difficult to interpret. Further, most studies originate from surgical departments and tend to focus on surgical findings and results and often do not provide a full accounting of all patients seen. Long-term survival in patients with hilar cholangiocarcinoma depends critically on complete tumor resection. 2,10 In the absence of widespread disease, the likelihood of achieving a complete resection requires examination of all factors related to local tumor extent, which increasingly has become possible with noninvasive imaging studies. 13,14 Tumor location and extent within the biliary tree, as provided by the Bismuth-Corlette classification system, 15,16 is only one component. Additional factors that must be addressed relate to radial tumor growth and its impact on adjacent structures, specifically portal venous involvement and consequent hepatic lobar atrophy. Both the modified Bismuth-Corlette and the American Joint Committee on Cancer 17 staging systems fail to account for all of these local, tumor-related factors, which frequently influence therapy. We have shown previously that a preoperative staging system that accounts fully for local tumor-related factors accurately predicts resectability and correlates with survival. 2 The present study represents an analysis of all patients with hilar cholangiocarcinoma seen and treated at a single institution during a 9-year period. The organizational structure of the hepatobiliary program at Memorial Sloan-Kettering Cancer Center (MSKCC) allows a multidisciplinary review of all patients, regardless of disease stage. Thus, a full accounting of all patients, including those with unresectable tumors, is possible. The relatively short time interval ensures homogeneity with respect to disease assessment and surgical approach. This study also proposes a preoperative staging system, based on imaging data and modified from a previous report, 2 that stratifies patients into treatment groups with predictable resectability rates and survival.

Background: Palliating the effects of biliary obstruction is a major goal of therapy in patients with cancer at the hepatic duct confluence. This study was undertaken to evaluate the effectiveness of intrahepatic biliary-enteric bypass to either the segment III duct or the right sectoral hepatic ducts in patients with unresectable hilar cholangiocarcinoma or gallbladder carcinoma. Methods: From December 1991 to October 1996, 55 consecutive bypass procedures were prospectively evaluated in patients with unresectable hilar cholangiocarcinoma or gallbladder cancer. Patients were divided into three groups based on the primary tumor and the type of bypass performed: group 1A, cholangiocarcinoma/segment III bypass (n = 20); group 1B, cholangiocarcinoma/right sectoral hepatic duct bypass (n = 14); group 2, gallbladder cancer/segment III bypass (n = 21). Results: Mean hospital stay (14 ± 2 days) and mean blood loss (629 ± 84 mL) were similar among the three groups. Perioperative death occurred in 6 patients (11%): 0 in group 1A, 3 each in groups 1B and 2. All survivors had relief of jaundice and pruritis after bypass. Complications occurred in 25 patients (45%). Preoperative transhepatic biliary drainage, performed in 14 patients prior to referral, was associated with a higher incidence of contaminated bile, greater operative blood loss, and postoperative biliary leak that was less likely to resolve spontaneously. Median survival in patients with cholangiocarcinoma (groups 1A and 1B) was 52 weeks and was unaffected by the type of bypass performed. By contrast, median survival in patients with gallbladder cancer (group 3) was 20 weeks; all but 3 died within 32 weeks of surgery. In patients with cholangiocarcinoma, the 1-year bypass patency was 80% in group 1A (segment III bypass) and 60% in group 1B (right sectoral hepatic duct bypass). Overall, there were 9 late bypass failures (18%) requiring reintervention. Conclusions: Intrahepatic biliary-enteric bypass effectively relieves symptoms due to malignant hilar obstruction. In patients with cholangiocarcinoma, segment III bypass provides excellent palliation with relatively few late complications and can be performed with minimal morbidity and mortality. Bypass to the right sectoral hepatic ducts, on the other hand, is associated with significant procedure-related morbidity and mortality and more late complications. Patients with gallbladder cancer, because of their poor survival, are probably better palliated by percutaneous biliary stenting.

Background The indications for preoperative biliary stenting in patients with obstructive jaundice are controversial. We evaluated the effect of preoperative biliary stenting on bacterobilia and infectious complications following surgical treatment of proximal cholangiocarcinoma. Design A retrospective review was performed of all patients undergoing surgical treatment of proximal cholangiocarcinoma. Setting A metropolitan cancer surgery service. Patients and Methods Seventy-one patients underwent palliative biliary bypass or curative resection of proximal cholangiocarcinoma from March 1, 1991, to April 1, 1997, and were entered into a prospective database. Forty-one patients underwent preoperative biliary intubation and stent placement. We analyzed patient, nutritional, laboratory, and operating room factors. Statistical evaluation was performed using Studentttest and χ2analysis. Main Outcome Measure Data were recorded for a history of cholangitis, operative time, amount of blood loss, incidence of intraoperative bacterobilia, proportion of patients with postoperative infectious and noninfectious complications, and length of hospital stay. Results: All patients (n=14) with a history of preoperative cholangitis had been subjected to previous endoscopic retrograde cholangiopancreatography and/or percutaneous transhepatic biliary drainage. Groups were equivalent for risk for comorbidity, proportion undergoing curative vs palliative procedures, time spent in the operating room, and amount of blood loss. Patients with stents had a significantly lower bilirubin level (P=.005). Patients with stents had a significantly increased risk for bacterobilia (P=.001) and infectious complications (P=.03). Bacterobilia was present in 11 (100%) of 11 patients undergoing endoscopic stenting and in 15 (65%) of 23 patients undergoing percutaneous stenting. There was no increased risk for noninfectious complications, length of hospital stay, or mortality in patients with stents. In 10 (59%) of 17 patients with postoperative infectious complications and positive findings of intraoperative bile culture, the organism was synonymous. Conclusions Preoperative biliary stenting in proximal cholangiocarcinoma increases the incidence of contaminated bile and postoperative infectious complications. Endoscopic stents frequently do not relieve jaundice in high biliary obstruction and are rarely indicated, especially in light of their high contamination rate.

• Medical records of 52 human immunodeficiency virus (HIV)-infected patients who underwent a total of 80 anorectal operations from January 1985 to January 1990 were retrospectively reviewed to determined whether anorectal surgical wounds healed in HIV-infected patients and the mean survival time of these patients after surgery. Twenty-four operations were performed in asymptomatic HIV-infected patients, 19 in HIV-infected patients with persistent lymphadenopathy, and 37 in patients with acquired immunodeficiency syndrome. Wounds healed in 49 patients (94%). The mortality rate 30 days after surgery was 2%. There were no major complications. The mean survival time of HIV-infected patients after surgery was 15 months. We conclude that anorectal surgical wounds heal in most HIV-infected patients and that the survival time after surgery of HIV-infected patients with anorectal disease justifies appropriate surgical treatment. ( Arch Surg. 1991;126:1267-1271)

Five children in two families, three boys in one family and two girls in another, had the onset of nephrotic syndrome in early infancy. The proteinuria and edema formation were not altered by steroid therapy. Growth retardation, renal osteodystrophy, and tetany appeared at an early age. Glycosuria, aminoaciduria, and renal tubular acidosis developed during the course of illness, and nephrocalcinosis was noted. A renal biopsy specimen from a child in each family showed glomerular proliferative changes, glomerular scarring, interstitial inflammation, and microcalcinosis, together with extensive tubular atrophy. Three of the five children have died so far, all at about 8 years of age. The similarity of the clinical course suggests that the children in each of these families has a distinct inherited disease, a form of familial nephrotic syndrome.

Current information relating to the clinical significance of proteinuria in children through adolescence was reviewed. Proteinuria may be transient, orthostatic, or fixed. The data indicate that only fixed proteinuria—that is, proteinuria on multiple urine examinations without pos tural exaggeration—may indicate serious glomerular disease. The patient should be followed with periodic but infrequent examinations, avoiding unnecessary, complicated, and hazardous investigations.

Follow-up survival and health information were obtained, after a median of 27.5 years, from 132 patients who had been seen originally as children with nephrotic syndrome between 1951 and 1967. Ninety seven patients were alive. Recurring edema or proteinuria, or both, persisted in 15 percent of those still alive. Eight of 11 parous women reported relapses during pregnancy. There was no apparent increase in malignancies, atopic diseases, clinical defects in cell-mediated immunity, or cardiovascular diseases. Twenty two patients (17%) died of renal causes between 3 months and 8 years after the onset of nephrotic syndrome. Steroid resistance was the presenting feature universally predictive of a poor outcome; nine of the 11 such patients died and the other two are now receiving hemodialysis. Hematuria was present initially in 41 percent of the patients who died of renal causes, compared with 14 percent of those still alive. Hypertension was noted on the first examination in 22 percent of those who died of renal causes, compared with 10 percent of those alive.

Of six patients with diffuse glomerulonephritis and associated infected ventriculoatrial shunt, three had improved renal function after removal of the infected shunt, and three died from Central-nervous-system disease. The renal disease probably resulted from an immunologic response to the staphylococcal infection rather than from direct bacterial embolization of the kidney.

IN 1839 Frohlichfirst described the clinical entity now referred to as the prunebelly syndrome. At that time, although it was evident that the abdominal musculature was absent, the associated urinarytract anomalies were not recognized. The thin skin and omental covering of the abdomen appeared wrinkled and wizened like a dried prune, hence the appellation "prune-belly" (Fig 1). Parker, in 1895, called attention to the accompanying hydronephrosis, hydro-ureter, megalocystis, and undescended testes as comprising this syndrome. Although 146 cases of this syndrome have been reported in the literature,1-13including the more recent report of Williams and Burkholder7in August 1967, none has detailed the survival data of these children. We wish to add our 19 cases to this total, thus bringing the overall number of cases to 165, and to discuss the clinical findings and survival in thissyndrome. Our Series Since 1945, 19 patients with prune-belly syndrome have been seen at

GROSS HEMATURIA in a child is both unusual and alarming. In a previously healthy child without a history of trauma, the differential diagnosis in these cases includes glomerulonephritis, tumors of the urogenital system, hematologic diseases, inflammatory conditions, and reticuloendothelial diseases. Rarely is hydronephrosis considered a cause of gross hematuria. We recently saw an 8-year-old boy who had sudden onset of gross painless hematuria with the passage of clots. The anomaly and the hydronephrosis coexisting and the satisfactory surgical results were unusual enough to be reported. Report of a Case An 8-year-old boy was admitted to the hospital on April 8, 1966, because of painless gross hematuria of 12 hours' duration. At the onset of the hematuria, a few clots were present in the voided specimens. These were passed after fleeting and colicky pains in the right flank. The boy's past health had been good and there had been no previous

EPIPHYSEAL destruction caused by frostbite of the hands is apparently rare. We have found detailed reports of only five cases. 1-5 Bigelow and Ritchie 6 studied six children with frostbite injury of the hands, and reported some clinical details on three of these children. Since frostbite must be fairly common in some parts of the world, we are not certain whether this injury is really rare, or merely taken for granted. We are reporting a case in order to bring the entity to the attention of clinicians dealing with such injuries. Report of Case A 10-year-old boy complained of swollen finger joints. One year earlier, he had pushed his bicycle home through deep snow in a temperature of OF. He wore no gloves or mittens, and was outdoors for about 30 minutes. When he entered his home, the fingers of both hands were white and hard, distal to the proximal