Forme fruste choledochal cyst

Four patients had the characteristic features of choledochal cyst except for the cystic component. All patients had stenosis of the distal common bile duct, a "long common channel" secondary to a proximal junction of the common bile and pancreatic ducts, cholecystitis and the classic pathological microscopic features of choledochal cyst in the wall of the common bile duct. Three children had coexisting intrahepatic duct cysts and/or stenosis and one had intrahepatic choledocholithiasis. The clinical presentations were cholangitis (2), pancreatitis (1) and biliary obstruction (1). In all cases the common bile duct was resected and biliary reconstruction was carried out by choledochojejunostomy (Roux-en-Y). Morbidity was minor except in one patient with ductal disease extending far into the intrahepatic ducts. This child developed an anastomotic stricture requiring revision of the anastomosis and long-term "U" tube stenting. Forme fruste choledochal cyst appears to be another variation in the spectrum of pancreaticobiliary malformations of choledochal cyst. Treatment is identical, that is, excision of all malformed ductal tissue.     

Congenital choledochal dilatation: Classification, clinical features, and long-term results

Choledochal cyst is generally accompanied by pancreatobiliary malunion. Pancreatic juice usually refluxes into the bile duct via the malunion, and bile juice occasionally regurgitates into the pancreatic duct. As a result, various pathological conditions occur in the biliary tract, pancreas, and liver. We have revised our classification of choledochal cyst on the basis of malunion. Abdominal mass, pain, and jaundice are regarded as the triad of choledochal cysts, but symptoms are quite non-specific. In neonates and infants less than 12 months of age, choledochal cyst is usually huge and no cylindrical dilatation is observed. Vomiting and jaundice with acholic stool are often observed. No hyperamylasemia is seen, despite the presence of a malunion. Whereas children over 13 months of age tend to show diffuse dilatation, and an abdominal mass is rarely palpable. Epigastralgia with hyperamylasemia, fever, vomiting, and slight jaundice are often observed. Cyst excision is the treatment of choice to prevent the development of ascending cholangitis and cancer in the cyst. Late complications of excisional surgery include recurrent cholangitis, pancreatitis, and rarely, biliary malignancy. These complications usually develop 5 years or more after initial surgery and become the object of reoperation. Complete removal of the extrahepatic bile duct from the hepatic hilum to the intrapancreatic duct and a hilar hepaticoenterostomy with wide anastomosis after ductoplasty, including the coexisting primary stricture, are essential at the primary surgery. Provided a large anastomosis, favorable results can be obtained with either duodenostomy or Roux-Y jejunostomy. Surgical enlargement of the hilar ducts and careful lifelong follow-up should be routine in all patients who have undergone cyst excision. When ductal stricture with intrahepatic gallstones is confirmed, reoperation at an early stage is indicated to obtain a good quality of life with short hospitalization.     

Appraisal of surgical resection of gallbladder carcinoma with special reference to hepatic resection

Carcinoma of the gallbladder a gastrointestinal malignancy with an extraordinarily poor prognosis. However, aggressive surgery, with special reference to hepatic resection, may improve survival. To prove this, we performed a retrospective analysis over an 18-year period to investigate the experience of a center that began employing liver resection in patients with gallbladder cancer in 1978. The analysis was based on patients' documentation and regular follow-up to January 1996. The standard procedures were extended cholecystectomy (cholecystectomy with lymphadenectomy and wedge hepatic resection), anatomic segmentectomy of segments IVa and V, and extended hepatectomy. Significance was assessed by the log-rank test. Thirty-nine patients were resected, curatively in 41% (n = 22; group I) and palliatively in 31% (n = 17; group 2). In 28% (n = 15; group 3) a palliative or no operation was performed. Only curatively resected patients were analyzed and followed up to January 1996. No patients in group 1 died postoperatively. The actuarial 5-year survival rate of the patients with curative resection was 55%. Four patients had stage I, two had stage II, four had stage III, and two had stage IV disease according to TNM-classification. Six of the 16 patients without lymph node metastasis survived more than 5 years. A significant difference in long-term survival was recognised between stage II and stage IV patients and between stage (pT1a)- and (look table 1b) (pT1b)-patients (P < 0.01). Diagnostic efforts should focus on detecting early stages I and II gallbladder cancer. In advanced cases, aggressive surgery, particularly with hepatic resection, is the method of choice and is successful even in patients 70 years and older. Received for publication on July 31, 1997; accepted on April 1, 1998     

Histochemical and immunohistochemical studies on development of biliary carcinoma in forty-seven patients with choledochal cyst—Special reference to intestinal metaplasia in the biliary duct—

Histochemical and immunohistochemical studies on 47 consecutive specimens excised for choledochal cyst were performed to clarify possible metaplastic changes of the biliary duct in relation to carcinogenesis. An anomalous arrangement of the pancreaticobiliary ductal system was observed in all 39 cases examined. Among the 47 patients, 5 (10.6 per cent) had biliary carcinoma. 27.3 per cent mucous gland, 13.0 per cent goblet cell and 9.5 per cent argyrophil cell in 23 children. On the other hand, 81.8 per cent exhibited mucous gland, 41.7 per cent goblet cell and 27.3 per cent argyrophil cell in 24 adults. These metaplastic changes seemed to be an intestinal metaplasia and increased with age. Immunoreactive-gastrin or-somatostatin were evident immunohistochemically in 4 adults. These findings confirmed that intesinal metaplasia may develop in the biliary duct in cases of choledochal cyst. Although direct evidence between intestinal metaplasia and the development of biliary carcinoma was not found, reflux and stasis of pancreatic enzymes in the biliary duct may relate to the development of intestinal metaplasia and be an important factor related to the carcinogenesis of choledochal cyst.     

Intraoperative endoscopy during choledochal cyst excision: extended long-term follow-up compared with recent cases

Introduction

We routinely perform intraoperative endoscopy (IOE) of the intrahepatic bile duct (IHBD) and intrapancreatic bile duct (IPBD) with a pediatric cystoscope during excision of choledochal cyst (CC). Here we compare extended long-term follow-up patients with a series of recent cases.

Methods

We compared 25 CC patients treated over the past 5 years (group 1) with 69 CC patients treated between 1986 and 1998 and followed up for more than 10 years (group 2), focusing on stone formation.

Results

In group 1 (mean follow-up, 2.6 years) IOE identified IHBD debris in 7 (28%) of 25 and IPBD protein plugs in 9 (36%) of 25. In group 2 (mean follow-up, 14.6 years) IOE identified IHBD debris in 11 (16%) of 69 and IPBD protein plugs in 17 (25%) of 69. There was no relation between type of CC and incidence of IHBD debris or IPBD protein plugs. The incidence of postoperative stones to date is 0% in group 1 and 2.9% in group 2, rates far lower than those reported in the literature.

Conclusions

We attribute our lower incidence of stones directly to IOE and recommend that it be performed routinely during cyst excision. It is simple, is effective, and improves outcome.     

Initial experience with complex laparoscopic biliary surgery in children: biliary atresia and choledochal cyst

Background

Laparoscopic approaches are possible for many pediatric surgical diseases, including complex biliary disorders. The authors describe their early experience with laparoscopic procedures for biliary atresia and choledochal cysts.

Methods

A retrospective review was performed with Institutional Review Board (IRB) approval of children undergoing laparoscopic Kasai portoenterostomy (n = 2) or laparoscopic excision of a choledochal cyst with Roux-en-y biliary reconstruction (n = 3). The authors report the operative technique for these procedures and present their initial results.

Results

All procedures were performed with 4 to 5 trocars using 3- and 5-mm ports. Excision of the fibrous biliary remnant (biliary atresia) or of the cyst (choledochal cysts) was performed laparoscopically in all cases. The Roux-en-y limb was created through a 1-cm extension of the umbilical port site. Laparoscopic biliary reconstruction was performed successfully in 4 of 5 patients. In one child, the proximal extent of the choledochal cyst was at the bifurcation of the hepatic duct, and the biliary reconstruction was performed via an open incision with separate anastomoses of the right and left ducts. The gallbladder was used as a handle for retraction of the liver for portal visualization, as in a cholecystectomy, and was resected at the end of the procedure. All children with choledochal cysts have normal bilirubin values. One of 2 children who underwent laparoscopic Kasai has a normal postoperative bilirubin level, whereas the other child did not drain bile and underwent a successful liver transplantation. Mean length of surgery was 338 minutes; mean length of stay was 5.5 days. There were no significant postoperative complications. Cosmesis was excellent in all cases.

Conclusions

Laparoscopic approaches for children with biliary atresia and choledochal cysts are possible. Excellent visualization of the portal structures can be achieved laparoscopically with adequate retraction of the liver. Further follow-up is needed to determine broader application.     

Classification of congenital biliary cystic disease: special reference to type Ic and IVA cysts with primary ductal stricture

Congenital biliary cysts occur not only in the choledochus but also everywhere in the biliary tree, and are frequently accompanied by pancreatobiliary malunion. Alonso-Lej and colleagues first classified choledochal cysts into three types, in 1959. Due to the recognition of intrahepatic involvement, we refined their classification into six types, in 1977, and this has become the reference. However, this classification does not include the concept of pancreatobiliary malunion and has caused some confusion, especially in regard to types Ic and IVA. Type Ic, showing fusiform dilatation, has a pancreatobiliary malunion and often extends continuously to the intrahepatic duct. Type IVA, with intrahepatic involvement, shows primary ductal stricture, especially around the hepatic hilum and umbilicus. The incidence of the stricture is considerably greater than was previously assumed, and upstream intrahepatic ducts often join the dilated duct, with distinct caliber change. Intrahepatic dilatation may develop primarily, due to the stricture, combined with weakness of the duct wall. This stricture can be corrected by portal dissection in the liver to obtain a large anastomosis. In patients with intrahepatic involvement, we should carefully find the stricture by suitable cholangiograms, or by intraoperative cholangioscopy. Detecting the ductal stricture is obviously important, rather than classifying the cyst as type Ic or IVA.     

Surgical treatment of type IV-A choledochal cyst in a single institution: Children vs. adults

Background

The treatment of type IV-A choledochal cyst is particularly difficult and remains a challenge because of the rareness and the various presentations of the disease involving not only the extrahepatic but also the intrahepatic biliary tract. The purpose of this study is to analyze our clinical experience for surgical treatment of type IV-A choledochal cyst, and compare between children and adults.

Methods

During a 10-year period of time (2000–2010), clinical data of 81 consecutive patients with type IV-A choledochal cyst were retrospectively analyzed. We divided these patients into two groups, the child group (age ≤ 18 years) and the adult group (age > 18 years). According to whether the patient received additional liver resection, patients were divided into a extrahepatic cystectomy (EHC) group and an additional liver resection (LR) group. The long-term outcomes after surgery were evaluated in two groups.

Results

Of all 81 patients, there were 17 children and 64 adults; 16 children and 35 adults belonged to EHC group, one child and 29 adults belonged to LR group. The morbidity of biliary stricture and/or lithiasis in the adults was significantly higher than that in the children (p = 0.041 < 0.05). In the EHC group, the reoperation rate of adults was significantly higher than that of children (p = 0.019 < 0.05). For adult patients, the morbidity of biliary stricture and/or lithiasis and the reoperation rate in EHC group was significantly higher than that in LR group (p = 0.037 < 0.05 and p = 0.026 < 0.05 respectively). Five adults were found to have cholangiocarcinoma within a follow-up period, while no child was found to. However, for adult patients, no significant discrepancy was observed between EHC group and LR group (p = 0.366 > 0.05).

Conclusions

The present study suggests that the children have better outcomes than adults for patients with type IV-A choledochal cyst after EHC, while LR brings better outcomes than EHC for adult patients.     

Symptomatic liver cyst: Special reference to surgical management

We conducted a retrospective study of 14 patients with symptomatic liver cysts to evaluate current therapeutic interventions for this condition. Abdominal pain (n = 7) or abdominal mass (n = 5) were the most frequent presentations. Three patients also had renal cyst. Percutaneous aspiration with ethanol sclerotheraphy was carried out in 4 patients and all cysts so treated diminished in size, with relief of the symptoms. One patient was treated by aspiration only and re-retension occurred. Cystectomy was performed in 2 patients, unroofing in 5, and fenestration in 2 patients. All patients gained relief of symptoms, with no recurrence of symptoms. Computed tomography revealed that the cysts were diminished or were no longer observable after all the treatments. Our experience indicates that unroofing, fenestration, and cystectomy are safe and suitable procedures for treatment of the condition. Ethanol sclerotherapy may be a feasible alternative to surgical intervention in selected patients. Received for publication on July 23, 1997; accepted on Dec. 25, 1997     

Long-term outcomes after hepaticojejunostomy for choledochal cyst: a 10- to 27-year follow-up

Introduction

Choledochal cyst (CC) is closely associated with anomalous arrangement of the pancreaticobiliary duct, which is considered a high-risk factor for biliary tract malignancy. Early diagnosis and early treatment for CC could lead to a good prognosis. This study investigated late complications and long-term outcomes after surgery for CC.

Patients and Methods

Fifty-six patients with CC and over 10 years of postoperative follow-up were analyzed retrospectively. All patients had undergone total resection of the extrahepatic bile duct and hepaticojejunostomy.

Results

Six patients showed liver dysfunction manifested in the first 10 years after surgery, but all returned to normal thereafter. Dilatation of intrahepatic bile ducts persisted in 6 postoperatively, and in 3, this was still apparent more than 10 years after. Recurrent abdominal pain was encountered in 3, 1 had pancreas divisum with a pancreatic stone, and 1 had adhesive small bowel obstruction. Two patients developed biliary tract malignancy. A 14-year-old girl died of recurrent common bile duct cancer 2 years after the initial resection of CC with adenocarcinoma. A 26-year-old man with repeated cholangitis owing to multiple intrahepatic bile stones developed cholangiocarcinoma 26 years after the initial resection of CC. Event-free survival rate and overall survival rate were 89% (50/56) and 96% (54/56), respectively.

Conclusions

Choledochal cyst generally has an excellent prognosis with early total resection and reconstruction. Long-term surveillance for the development of malignancy is still essential, especially if there is ongoing dilatation of the intrahepatic bile duct or biliary stones.     

Determinants of long-term survival following hepatectomy for hepatocellular carcinoma, with special reference to patients surviving more than 10 years

To elucidate the determinants of survival and the clinicopathologic features of long-term survivors of resections for HCC, we reviewed 539 patients who had had hepatectomy alone or hepatectomy together with hepatic arterial ligation or ethanol injection for the treatment of hepatocellular carcinoma (HCC) at our department between 1973 and December 1992. Of these patients, 30% (79/264) survived for more than 5 years and 11% (10/87) for more than 10 years. All the long-term survivors had received curative resections. The 5- and 10-year survivors accounted for 58% (79/136) and 29% (10/35) of those with curative resections, respectively. Crucial determinants for long-term survival were the absence of portal invasion and satellite nodules, a diploid pattern of DNA content, and a curative resection. A curative resection for diploid HCCs led to much better survival rates (73%) at 5 years than such a resection for aneuploid HCCs (35%), and than for non-curative resections for aneuploid HCCs (0%). The history and tumor background of the ten patients who survived for more than 10 years were characterized by good reserve liver function, warranting a wider resection, predominance of female sex, single nodular growth, and the absence of poorly differentiated cells, in addition to the favorable conditions described above. Three of the ten patients developed a new lesion; these were successfully treated by re-resection or repeated arterial embolization, since they were confined to the remnant liver and showed single nodular growth without extranodular spread. This analysis indicates that hepatectomy is the option of first choice for HCC patients with the potential for cure and good reserve liver function.     

The effect of laparoscopic excision vs open excision in children with choledochal cyst: a midterm follow-up study

Purpose

Cyst excision with hepaticojejunostomy has been the classic procedure for treating choledochal cysts. Recently, laparoscopic treatment of the disease has gained popularity worldwide. The aim of this study is to evaluate whether laparoscopic management of choledochal cysts is as feasible and safe as conventional open surgery in children with this disease.

Methods

A retrospective study comparing the laparoscopic and the open procedures was performed in 77 consecutive patients with choledochal cyst in our hospital. Thirty-nine patients operated on between June 2001 and September 2003 were in the laparoscopic group, whereas 38 patients in the open group were operated on between February 1999 and May 2001.

Results

Patient demographics were similar between the 2 groups. The duration of operation was significantly longer in the laparoscopic group than in the open group (median, 230 vs 190 minutes; P < .001). In contrast, the durations of delayed oral feeding and hospital stay postoperatively were significantly shorter in the laparoscopic group (median, 4 vs 5 days [P < .01] and median, 5 vs 7 days [P < .01], respectively.) There were no differences in the early and late complication rates between the 2 groups.

Conclusions

Laparoscopic treatment of choledochal cyst in children is feasible and safe. For experienced centers, this procedure can be recommended.     

Carcinoma of the distal and middle bile duct: surgical results, prognostic factors, and long-term follow-up

BACKGROUND/PURPOSE: Carcinoma of the distal bile duct is associated with poor prognosis. Surgical resection remains the only potentially curative treatment. We conducted a retrospective study to identify prognostic factors determining longterm survival. METHODS: From 1990 to 2006, 95 patients with distal and/or middle bile duct carcinoma had resections. Fifty-four patients underwent pylorus-preserving pancreaticoduodenectomy (57%) and 41 patients underwent standard Kausch-Whipple pancreaticoduodenectomy (43%). Nine patients underwent pancreaticoduodenectomy including portal vein resection (9%). RESULTS: Overall 1-, 3-, and 5-year survival rates were 60%, 36%, and 29%, respectively. Five-year survival after R0 resection was 34%, and after R1 resection it was 0%. Four patients died during their hospital stay (4%). Multivariate analysis showed negative resection margins (P = 0.040), lymphatic vessel invasion (P = 0.036), and portal vein infiltration (P = 0.027) as strong predictors for survival, whereas the location of the tumor (distal bile duct vs middle bile duct) and lymph node status were not identified as independent prognostic factors. CONCLUSIONS: Five-year survival depends strongly on negative resection margins, independent of nodal status. Portal vein resections in patients with portal vein involvement fail to ameliorate long-term survival. Primary tumor site--middle bile duct or distal bile duct--did not determine prognosis.     

Transurethral approach to the distal ureter in nephroureterectomy: transurethral extraction vs. "pluck" technique with long-term follow-up

OBJECTIVES: We retrospectively compared two techniques of transurethral management of the lower ureter in nephroureterectomy. PATIENTS AND METHODS: From August 1992 to December 2003, 34 patients underwent either transurethral detachment of the intramural ureter and cephalad extraction ("pluck"; Group 1, N = 18) or transection of the ureter with subsequent transurethral extraction (Group 2, N = 16). Choice of technique was left to the operating surgeon. All patients with upper tract urothelial carcinoma (TCC) were regularly followed by cystoscopy and abdominal ultrasound. RESULTS: Of the 34 patients, 29 had upper tract TCC. Mean follow-up in these was 44 months (range: 1-129), with 24 (83.8%) over 24 months. On follow-up, 14 bladder tumors (all superficial) occurred in 7 patients (24.1%), but in no case on the scar of the excised ureteral orifice. No extravesical recurrences in the former ureteral bed were found. Of the 29 with upper tract TCC, 19 (65.5%) are alive without disease (median 45 months, range: 6-129), 5 (17.2%) have died with no evidence of disease (median 34 months, range: 20-58), and 4 (13.8%) have died from progressive disease (median 18 months, range: 1-33); 1 patient was lost to follow-up at 34 months with no evidence of disease. Differences between techniques with regard to blood loss, operative time, complications, and oncologic outcome were not significant. CONCLUSION: Both techniques proved technically and oncologically safe. Bladder tumor recurrence rate was in the range reported for classic nephroureterectomy. No extravesical tumor recurrence in the former ureteral bed or on the scar of the resected ureteral orifice occurred.     

Predicting relapse in ductal carcinoma in situ patients: an analysis of biologic markers with long-term follow-up

BACKGROUND: The incidence of ductal carcinoma in situ (DCIS) of the breast is increasing. Optimal treatment remains controversial and, because of a long natural history, may not be evident for many years. We undertook this study to identify markers of disease recurrence. METHODS: We studied 131 pure DCIS patients with a 100-month mean follow-up. We performed a complete histologic review, immunohistochemical staining for p53 and vascular endothelial growth factor expression, and enumerated microvessel density/mm2 using factor VIII-Ab. Statistical analysis was performed by using an SAS software package (Cary, NC). RESULTS: Eleven patients (8%) developed ipsilateral recurrence at a mean of 55 months (11-137 months) after initial treatment. Three were DCIS, and 8 were invasive cancer. Recurrence was significantly predicted by p53 overexpression (in 55% of tumors that recurred versus 22% of those that did not, P = .02) but not other factors. CONCLUSIONS: These data suggest that biologic factors may have an important role in predicting recurrence in DCIS patients.     

Traumatic ventral hernia: Report of a case, with special reference to surgical treatment

(Received for publication on May 8, 1998; accepted on Mar. 11, 1999)