Relief of intractable pruritis in Alagille syndrome by partial external biliary diversion

Background

Patients with Alagille syndrome (AGS) may develop pruritis, skin hypertrophy, and xanthomas because of chronic cholestasis and hypercholesterolemia. Partial external biliary diversion (PEBD) has been used successfully to treat chronic cholestasis in patients with progressive familial intrahepatic cholestasis (PFIC) and is a potentially useful treatment for patients with severe and intractable pruritis because of AGS.

Methods

Four children with chronic cholestasis and intractable pruritis were treated with PEBD, 1 by surgical cholecystostomy and 3 by cholecystojejunostomy.

Results

Three patients had a known diagnosis of AGS. The fourth was an 11-month-old infant boy with PFIC. The first patient, a 15-month-old boy with AGS, underwent surgical cholecystostomy, which has required frequent tube changes to maintain patency. Three patients underwent PEBD using a segment of jejunum as a conduit between the gallbladder and the skin, where bile is collected in a standard ostomy appliance. Mean follow-up is 15.5 months (range, 9-26 months).All patients experienced rapid and enduring relief of pruritis. Two adolescents with AGS had significant improvement of the hypertrophic skin of their hands. There was one significant complication: the infant with PFIC required reoperation for bleeding from the jejunal anastomosis 1 week after PEBD; he has subsequently done well. One adolescent girl with AGS initially had difficulty with her ostomy because of poor site placement and partial retraction, but nevertheless has managed quite well. The patient treated by cholecystostomy has had excellent relief of his pruritis and is being considered for conversion to cholecystojejunostomy.

Conclusions

Chronic cholestasis caused by AGS can cause debilitating symptoms that are resistant to medical therapy. Partial external biliary diversion is a safe and technically straightforward operation that may be effective for the relief of intractable pruritis and other symptoms in patients with AGS.     

Nuclear Imaging of the Liver: Is There a Diagnostic Role of HIDA in Posttransplantation?

Background and Aims

Biliary complications are common after liver transplantation. This study sought to assess the value of hepatic iminodiacetic acid (HIDA) scans (hepatobiliary iminodiacetic acid scan) to detect early posttransplantation biliary complications.

Methods

From April 2003 to June 2006, 34 liver transplantations (recipients of mean ± SD age of 43.0 ± 15.7 years) were performed in 25 (73.5%) males from 20 (58.8%) cadaveric donors and 14 (41.2%) living-related donors. The subjects underwent HIDA scans using a single head gamma camera Meridian (Philips) after intravenous (IV) administration of 185 MBq Tc-99m Disofenin. The mean time ± SD posttransplantation to HIDA scan was 14.6 ± 18.2 days (range, 0-74). The results were compared with endoscopic retrograde cholangio pancreatography, magnetic resonant cholangiopancreatography, percutaneous cholangiograhy, and/or liver biopsy.

Results

Twenty-four abnormalities were detected by HIDA scan in 16 patients (47.1%): 10 (29.4%) biliary leaks; 4 (11.4%) biliary obstruction or cholestasis; 1 (2.9%) delayed uptake; 5 (14.7%) delayed blood pool clearance; and 8 (23.5%) delayed transit to the bowel. The complications were more common among living-donor compared with deceased-donor graft recipients, albeit a not statistically significant difference (P = .066). Total and direct bilirubin levels were significantly higher in patients with abnormal than normal HIDA scans (P = .011 and P = .040, respectively). The sensitivity and specificity of HIDA scans to detect overall postoperative complications were 100% and 66.7%, respectively. Biliary leak results were false positives in 7/10 patients, and true in 3. Detection of obstruction was 75% sensitive by HIDA.

Conclusion

HIDA scans are a noninvasive, reliable modality for early exclusion of posttransplantation biliary complications. However, correlation with clinical status and imaging modalities is essential to confirm detected abnormalities.     

Hepatobiliary scintigraphy during cholestatic and noncholestatic periods in patients with progressive familial intrahepatic cholestasis after partial external biliary diversion

Background

The purpose of the study was to determine the distribution of excreted bile during cholestatic periods and in remission in patients with progressive familial intrahepatic cholestasis (PFIC) after surgery with partial external biliary diversion (PEBD), using hepatobiliary scintigraphy.

Methods

Using intravenously administered technetium Tc 99m-labeled mebrofenin, the distribution of bile during periods of biochemical cholestasis and in remission was investigated in patients with PFIC operated with PEBD. Stomal bile, urine, and feces from the patients were collected during 24 hours after administration of technetium Tc 99m-labeled mebrofenin; and the fractions of remaining radioactivity in the 3 compartments and the remaining isotopic activity in the body were quantified using scintigraphy.

Results

Nine patients (4 boys and 5 girls) were studied. The median age was 13 (range, 5-24) years, and they had been operated with PEBD at a median time of 10 (range, 4-14) years before entering the study. Thirteen scintigraphic examinations were analyzed: 8 during noncholestatic remission (n = 7 patients) and 5 during cholestasis (n = 3 patients). The patients studied during remission discharged a significantly larger fraction of isotopic activity through the stoma (median, 90% vs 22%; P < .05) and a significantly lower fraction through the urine (median, 2.5% vs 15%; P < .05) compared with the patients studied during cholestasis.

Conclusion

Hepatobiliary scintigraphy could detect substantial differences in the output of bile. Further studies are needed to determine whether these differences may explain the mechanism of the PEBD operation or merely are secondary to the degree of cholestasis.     

A contemporary analysis of parenteral nutrition-associated liver disease in surgical infants

Background/purpose

Despite advances in pediatric nutritional support and a renewed focus on management of intestinal failure, there are limited recent data regarding the risk of parenteral nutrition (PN)-associated liver disease in surgical infants. This study investigated the incidence of cholestasis from PN and risk factors for its development in this population.

Methods

A retrospective review was performed of all neonates in our institution who underwent abdominal surgery and required postoperative PN from 2001 to 2006. Cholestasis was defined as 2 conjugated bilirubin levels greater than 2 mg/dL over 14 days. Nonparametric univariate analyses and multivariate logistic regression were used to model the likelihood of developing cholestasis. Median values with range are presented.

Results

One hundred seventy-six infants met inclusion criteria, and patients received PN for 28 days (range, 2-256 days). The incidence of cholestasis was 24%. Cholestatic infants were born at an earlier gestational age (34 vs 36 weeks; P < .01), required a 3-fold longer PN duration (76 vs 21 days; P < .001), had longer inpatient stays (86 vs 29 days; P < .001), and were more likely to be discharged on PN. The median time to cholestasis was 23 days. Cholestasis was an early development; 77% of cholestatic infants developed cholestasis by 5 weeks of PN exposure. On multivariate regression, only prematurity was significantly associated with development of cholestasis (P < .05).

Conclusion

In this analysis, the development of PN-associated liver disease occurred early in the course of exposure to PN. These data help to define the time course and prognosis for PN-associated cholestasis in surgical infants.     

The spectrum of surgical jaundice in infancy

Background

Conjugated jaundice arising during infancy may be caused by a number of different surgical conditions. The aim of this study was to compare clinical features, management, and outcome of all types of surgical jaundice presenting in the first year of life.

Methods

A retrospective review was conducted of all infants born in the United Kingdom with jaundice caused by a surgical cause referred to the authors’ institution from January 1992 to December 1999.

Results

There were 171 infants who could be separated into 3 specific groups: biliary atresia (BA, n = 137), inspissated bile syndrome (IBS; n = 14), and choledochal malformation (CM; n = 12) together with a group containing various miscellaneous conditions (n = 8). Infants with BA had higher bilirubin (P < .01) and aspartate aminotransferase levels (P < .001) and came to surgery earlier (P < .01) than infants with either IBS or CM. Infants with IBS and CM were more likely to be premature and have other malformations, respectively. Ultrasound scan was the principal investigation in the differentiation of BA from other causes of jaundice. Accurate prelaparotomy diagnosis was made by percutaneous liver biopsy in 87% of cases later shown to be BA. Currently, 88 (64%) of children with BA are alive with their native liver postportoenterostomy, 4 have died, and 45 have undergone liver transplantation (with 1 death postoperatively). A policy of primary portoenterostomy for BA followed by transplantation, if necessary, resulted in a survival rate of over 95%. All children in the other diagnostic groups are alive and anicteric after appropriate surgical intervention.

Conclusions

Approximately 80% of infants presenting with surgical jaundice have biliary atresia, whereas those with inspissated bile syndrome and choledochal malformations make up most of the remainder. Mortality in this age-group is confined to infants with BA, but even on these infants an overall survival rate of greater than 95% is currently expected.     

Review of historical cohort: ursodeoxycholic acid in extrahepatic biliary atresia

Background

Ursodeoxycholic acid is a bile acid that was found to increase bile flow, protect hepatocytes, and dissolve gallstones.

Purpose

The objective of this study is to review ursodeoxycholic acid in infants and children with extrahepatic biliary atresia.

Methods

We used a statistical analysis of data of records of infants and children having extrahepatic biliary atresia who underwent Kasai portoenterostomy and attended Hepatology Clinic, New Children's Hospital, Cairo University, Egypt, from May 1985 until June 2005.

Results

Of 141 infants with extrahepatic biliary atresia, 108 received ursodeoxycholic acid for mean duration ± SD of 252.6 ± 544.9 days in a dosage of 20 mg/kg per day. The outcome of infants who did not receive ursodeoxycholic acid and those who did was the following: 8 (24.2%) and 11 (10.18%) had a successful outcome (P = .043), 0 (0%) and 7 (6.4%) improved (P = .148), 25 (75.7%) and 84 (77.7%) had a failed outcome (P = .489), and none vs 5 died (4.6%) (P = .135), respectively. The predictors of successful outcomes were age less than 65 days at portoenterostomy (P = .008) and absence of ursodeoxycholic acid intake (P = .04) with a likelihood of a successful outcome that was 2.8, that associated with ursodeoxycholic acid intake.

Conclusion

In this cohort of infants with extrahepatic biliary atresia, ursodeoxycholic acid was not shown to be effective, and its use was associated with a plethora of hepatic and extrahepatic complications.     

Preoperative observations and short-term outcome after partial external biliary diversion in 13 patients with progressive familial intrahepatic cholestasis

Background

Patients with progressive familial intrahepatic cholestasis (PFIC) often require liver transplantation to survive. An alternative approach is surgical diversion of bile, that is, partial external biliary diversion (PEBD). The aim of the study was to describe 13 patients with PFIC who have undergone PEBD.

Methods

Clinical and laboratory workups including growth data and histology specimens were analyzed to evaluate the short-term effects of PEBD. Follow-up, including liver biopsies, was performed 11 to 21 (median, 14) months post-PEBD.

Results

All patients showed typical features of PFIC. Eight out of 13 presented with variable signs of coagulopathy, and one patient presented with hypocalcemic seizures. The surgery was uneventful in all, but 4 patients were readmitted because of dehydration and electrolyte imbalance caused by excessive stomal losses. One month post-PEBD, 7 patients were apruritic. One patient had stomal dysfunction, showed no improvement on cholestasis after surgery, and had to undergo liver transplantation 2 months post-PEBD. At follow-up, significant biochemical improvement and gains in growth were seen in most of the patients.

Conclusions

Most of the patients with PFIC presented with signs of coagulopathy. Partial external biliary diversion had a dramatic effect on cholestasis and growth, although not all patients benefited from the surgery. Episodes of dehydration post-PEBD must be considered.     

Congenital biliary atresia: liver injury begins at birth

Background

The timing of onset of liver injury in biliary atresia (BA) is not known, although in approximately 10% of cases, biliary pathologic condition associated with the biliary atresia splenic malformation syndrome must begin well before birth.

Methods

The study involved retrospective case-note review for infants with definite BA who underwent laparotomy within first week of life.

Results

Three infants were identified who had occlusive BA evident on the first day of life. In all cases, their liver was grossly normal, and histologic changes were trivial.

Conclusion

This suggests that the detrimental cholestatic liver injury, later characteristic of BA, only begins from the time of birth despite a prenatal occlusive biliary pathology. It may be that tissue injury only occurs with the onset of the perinatal bile surge initiating periductal bile leakage and the triggering of an inflammatory and ultimately fibrotic response.     

Long-term outcome after partial external biliary diversion for progressive familial intrahepatic cholestasis

Background

Though patients with progressive familial intrahepatic cholestasis (PFIC) typically require liver transplantation, initial surgical treatment includes partial biliary diversion (PBD) to relieve jaundice-associated pruritus. This study was undertaken to describe long-term PFIC outcome data, which are currently sparsely reported.

Methods

Retrospective review of 7 patients diagnosed with PFIC who underwent PBD between 2004 and 2008 was directed toward long-term postoperative outcome including resolution of jaundice/pruritus, stoma complications, interval to transplantation, and death.

Results

Six patients who underwent PBD experienced short-term resolution of jaundice and pruritus. Four patients experienced persistent stoma-related complications requiring a total of 14 revisions. Three symptom-free patients have not yet required liver transplantation post-PBD (average, 70 months; range, 59-78 months). Two patients underwent orthotopic liver transplantation (average, 44 ± 18 months post-PBD). Two patients died at home because of gastroenteritis-associated dehydration before transplantation.

Conclusion

Partial biliary diversion for PFIC is effective as a bridge to liver transplantation in improving jaundice and pruritus but may be associated with a high incidence of stoma-related complications. Persistent or recurrent pruritus after PFIC is associated with an increased risk of stoma prolapse or reflux. Insufficiently replaced stomal losses over time may increase the risk of dehydration-related complications in association with gastroenteritis.     

Portoenterostomy for biliary atresia: Long-term survival and prognosis after esophageal variceal bleeding

Background/purpose

Portoenterostomy is the procedure of choice in patients with biliary atresia. The authors analyzed the long-term survival rate and complications of the procedure in children operated on at The University of Hong Kong Medical Centre between 1979 and 2000.

Methods

Seventy-seven consecutive patients were analyzed by retrospective chart review. The patients were divided into groups according to postoperative bile flow, decade of operation, and age at operation. The data were analyzed statistically.

Results

The overall actuarial transplant-free survival rate was 68% at 10 years after operation. For patients with poor, partial, and good postoperative bile flow, transplant-free survival rate was 0%, 22%, and 96%, respectively (P < .001). Age and decade of operation were not significant risk factors. Esophageal variceal bleeding occurred in 13 patients and was a prognostic indicator for end-stage liver failure (P = .044); the poor prognosis of patients with variceal bleeding, however, was related to poor or partial initial bile drainage.

Conclusions

The postoperative serum bilirubin level is the most important predictor of long-term survival in patients with portoenterostomy for biliary atresia. Esophageal hemorrhage is not an absolute indication for urgent liver transplantation in patients with good bile drainage.     

Cystic biliary atresia: an etiologic and prognostic subgroup

Introduction

Cystic biliary atresia (CBA) is an uncommon variant of biliary atresia (BA) in which prognosis may be relatively favorable but liable to misdiagnosis as choledochal cyst, and potentially offers insights into the etiology of BA. Because some cases can be detected antenatally, CBA in general may have its origins in utero life. We assessed our experience with CBA.

Methods

Single-center retrospective review of infants with CBA over a 13-year period (January 1994 to December 2006) was done. Data are given as medians (range).

Results

Of 270 infants with BA, 29 (9 male) were identified as CBA. Antenatal ultrasonography had detected an abnormality in 12 (41%) infants at a median of 22 weeks (17-34 weeks) of gestation. All infants underwent postnatal excision and Kasai portoenterostomy (KP). Those with antenatally detected CBA came to surgery younger (36 [14-67] vs 48 days [35-147 days], P = .004). Twenty cysts (69%) had a fibroinflammatory wall with no biliary epithelial lining and 6 (26%) contained bile. Age at KP was significantly and positively correlated (r = 0.46, P = .01) with liver fibrosis, as assessed in liver biopsy materials obtained at KP, but not with grade of “hepatocyte disarray” (P = .74). Twenty infants (69%) cleared their jaundice (bilirubin <20 μmol/L) within 6 months after KP. Age at KP markedly affected outcome.

Conclusion

Cystic BA is a clinically distinct variant of BA. Despite onset in prenatal life, earlier than presumed for isolated BA, it has a better prognosis, particularly with early surgery.     

Biliary complications and management in pediatric living donor liver transplantation for underlying biliary atresia

Background

Biliary complications are a major problem in pediatric liver transplantation. The aim of this study was to evaluate the management and outcomes of biliary complication after pediatric living donor liver transplantation (LDLT).

Methods

From 1994 to 2010, 157 pediatric LDLT due to biliary atresia were performed in our center. Doppler ultrasound was initially performed daily for 2 weeks postoperatively to evaluate biliary and vascular complications. Computed tomography and or magnetic resonance cholangiography were performed when complications were suspected. They were treated using radiological or surgical interventions.

Results

Among the 157 cases, we observed 10 (6.3%) biliary complications, which were divided into three groups: bile leakage (n = 3); biliary stricture without vascular complication (n = 4); and biliary stricture with vascular complication (n = 3). The three cases bile leakages recovered after interventional procedures. The seven biliary strictures underwent percutaneous transhepatic cholangial drainage (PTCD). All cases without vascular complications were completely cured after PTCD or a subsequent surgical re-anastomosis. In the vascular complication group, early recorrection of the HA occlusion with successful PTCD treatment were performed in two cases, but one other case with diffuse ischemic biliary destruction had a poor result.

Conclusion

Successful interventional radiographic approaches are effective for anastomotic biliary complications but with poor results in diffuse ischemic biliary destruction.     

Congenital biliary dilatation may consist of 2 disease entities

Background/Purpose

This study aims to establish the possible mechanisms of pathogenesis of congenital biliary dilatation and to classify the disease accordingly.

Methods

Radiologic features of congenital biliary dilatation and pancreaticobiliary malunion in 107 affected children were examined and correlated with laboratory results. Relative lengths/diameters were calculated to provide comparison between children of different ages. Intraluminal pressures of common bile duct (CBD) were measured intraoperatively.

Results

The minimal relative diameters of distal CBD negatively correlated with the maximal relative diameters/lengths of dilated CBD, the maximal relative diameters of common hepatic duct, and left/right hepatic ducts. The intraluminal pressure in patients with a stenotic distal CBD (stenotic group) was significantly higher than that in patients with a nonstenotic distal CBD (nonstenotic group). The narrower the distal CBD, the more deranged the liver function. Conversely, serum/bile amylase levels were more elevated in the nonstenotic group. Common channel protein plugs were only found in the nonstenotic group, whereas common hepatic duct strictures, intrahepatic duct dilatations, and calculi were detected more frequently in the stenotic group.

Conclusion

We propose to categorize congenital biliary dilatation into 2 subgroups: (1) cystic type with stenotic distal CBD associated with deranged liver function and common hepatic duct stricture and (2) fusiform type with nonstenotic distal CBD associated with pancreatitis and common channel protein plugs. Different underlying pathologies of each group require different operative strategies.     

Evaluation of the PELD risk score as a severity index of biliary atresia

Purpose

The authors evaluated the validity of the Pediatric End-Stage Liver Disease (PELD) Risk Scoring System as a severity index for patients with biliary atresia.

Methods

Individual hospital records of 104 patients with biliary atresia were reviewed at our institution and divided into 3 groups: nontransplant survivors (n = 61), nontransplant deaths (n = 17), and transplant patients (n = 26). PELD risk scores were calculated according to Wiesner et al, multiplied by 10, and rounded to the nearest integer, as is done in determining model of end-stage liver disease (MELD) scores.

Results

The PELD scores showed a significant difference between nontransplant survivors (range, −21 to 15) and dying nontransplant patients during their last few months of life (range, 2 to 40). No survivors except those below the age of one year recorded scores above 10. Transplant patients had higher scores (range, −5 to 37) before transplantation than nontransplant survivors. However, the scores were not elevated in elderly patients with intractable cholangitis, fulminant variceal rupture, and hepatopulmonary syndrome.

Conclusions

PELD profiling is a useful scoring system for selecting patients with the most severe liver dysfunction caused by biliary atresia. However, we advise caution in using this system for patients under the age of 1 year and for older patients with long-term complications.     

Urethral hydrodistension for management of urethral hypoplasia in prune belly syndrome: long-term results

Purpose

The aim of the study was to evaluate the efficacy and safety of urethral hydrodistension for management of urethral hypoplasia in prune belly syndrome (PBS).

Methods

During a 10-year period, 7 infants with PBS and urethral hypoplasia presented either with open urachus or surgically created urinary diversion referred to our hospital. Five milliliters of normal saline was pushed via a 22-gauge plastic angiocatheter into the urethra with simultaneous finger pressure on the perineum to occlude the proximal urethra that was repeated with higher volumes of the solution (up to 20 mL). The procedure was continued until a 6F or 8F feeding tube catheter confirmed the urethral patency. Hydrodistension was repeated in 3-month intervals till complete patency was confirmed by imaging.

Results

Median age of the infants was 6 (1-8) months. All urethral hydrodistension were successful after 1 to 3 sessions. Follow-up imaging studies showed significant improvement in all patients except one. Natural and surgically created urinary diversions were closed in 6 infants.

Conclusions

The hydrodistension create an equal and constant pressure into the urethral wall without any urethral damage. This technique can be considered along with the other available methods for management of urethral hypoplasia in selected cases of PBS.     

Biliary reconstruction in pediatric live donor liver transplantation: duct-to-duct or Roux-en-Y hepaticojejunostomy

Background

Duct-to-duct biliary reconstruction (DD) is currently a standard procedure in adult live donor liver transplantation (LDLT). Its pediatric feasibility, however, has rarely been reported. The goal of this study is to assess the incidence and treatment of biliary complication after pediatric LDLT with DD or Roux-en-Y hepaticojejunostomy (RY).

Method

Sixty children received LDLT between November 2005 and June 2008, and their database was reviewed.

Results

Biliary reconstruction was achieved with DD in 14 patients and with RY in 46 patients with mean follow-up period of 26.0 and 22.3 months, respectively. The incidence of biliary leakage in the DD and RY groups was 7.1% and 8.7%, respectively, and that of stricture was 28.6% and 10.9%, respectively; but the differences were not statistically significant. Biliary stricture in the DD group tended to require revision surgery with RY and longer treatment with percutaneous transhepatic cholangiodrainage compared with that in the RY group.

Conclusion

Theoretical advantages of DD over RY were not confirmed in this study. Duct-to-duct biliary reconstruction tended to encounter more biliary complications, especially stricture, with more difficulty in treating it than RY. Roux-en-Y hepaticojejunostomy seems preferable to DD in the setting of pediatric LDLT, but DD must be considered when making new Roux-en-Y limb seems impossible or troublesome owing to abdominal dense adhesion or short bowel syndrome.     

Does arterialisation time influence biliary tract complications after orthotopic liver transplantation?

Background

In the cardiac death donor era, many reports deal with biliary tract complications and concerns about ischemic reperfusion injury owing to the exclusive arterial vascularization of the biliary tree, the warm ischemia time has been implicated as responsible for biliary lesions during organ procurement. We defined the arterialization time as the second warm ischemia time. Our purpose was to study the correlation between the arterialization time during liver implantation and the appearance of biliary lesions.

Methods

We retrospectively collected data from the last 5-years of orthotopic liver transplantation: namely, indications, cold perfusion fluid, cold ischemia time, operative procedure times, and acute rejection events. We excluded split-liver transplantations, retransplantations, pediatric patients, transplantations for cholestatic disease, cases where hepatic artery thrombosis happened before biliary complications, or patients with posttransplant cytomegalovirus infection. We defined 2 groups: A) without biliary complications; and B) with biliary complications. We compared the mean arterialization time using Student t test to define whether the warm ischemic time during implantation was responsible for biliary tract complications. A P value of <.05 was considered to be significant.

Results

Between 2004 and the end of 2008, we grafted 402 patients among whom 243 met the inclusion criteria: 198 in group A and 45 in group B. Only the cold ischemia time was significantly different between the 2 groups (P = .039).

Conclusion

After the anhepatic time, the surgeon may take time for the arterial anastomosis without fearing increased biliary damage.     

Glucocorticoid treatment down-regulates chemokine expression of bacterial cholangitis in cholestatic rats

Background

Postoperative cholangitis is common after operation for biliary atresia. Empirical pulse therapy with glucocorticoid is effective in reversing some detrimental clinical manifestations, but the rationale for such a therapy still is not substantiated.

Methods

Adult male rats were divided into groups according to the treatment: sterile normal saline (NS) or Escherichia coli (EC, 1 mL containing 10⁸ cells of ATCC 25922 strain), 1 mL, were infused into the proximal choledochostomy (PC) tube 2 weeks after ligation of the PC tube (bile duct ligation, BDL), then immediate tube-tube choledocho-choledochostomy (biliary drainage, BD) was constructed. A high dose of dexamethasone (DEX, intraperitoneal injection; 2 mg/kg of body weight) was given after BD in treatment groups. Histopathology of the liver, as well as liver chemokine mRNA expression and serum chemokine levels, were studied 24 hours after treatment.

Results

Inflammatory cell infiltration to the liver was retarded with DEX treatment, which was correlated with a significantly lower expression of interleukin-8 (IL-8) and monocyte chemoattractant protein-1 (MCP-1) mRNA in the liver (P = .006). Serum IL-8 and MCP-1 levels were also significantly down-regulated with DEX treatment (P = 0.008).

Conclusions

Glucocorticoid treatment is effective in modulating IL-8 and MCP-1 expression and ameliorating inflammatory cell infiltration in rat liver with bacterial cholangitis and cholestasis.     

Button cholecystostomy for management of progressive familial intrahepatic cholestasis syndromes

Background/Purpose

Progressive familial intrahepatic cholestasis syndromes are characterized by impaired bile acid secretion resulting in pruritus, coagulopathy, diarrhea, and malnutrition leading to progressive liver failure and death in childhood. Partial internal or external biliary drainage can relieve symptoms and slow the progression of the disease. Objections to partial external biliary drainage include the need for a permanent biliary stoma with all the inherent complications of a stoma. We propose a novel approach to these diseases—placement of a “button” cholecystostomy tube.

Methods

Under general anesthesia and through a small right subcostal incision, a MIC-KEY button (Kimberly-Clark Worldwide, Inc, Draper, UT) is inserted into the mobilized fundus of the gallbladder and secured with 2 purse-string sutures. Time of drainage is adjusted to relieve pruritus.

Results

Three children with progressive familial intrahepatic cholestasis achieved adequate bile drainage via the cholecystostomy button to relieve pruritus for 1, 2, and 2 ½ years postoperatively, with drainage periods of 12 to 14 hours per day. There were no episodes of cholangitis. Dislodged tubes can be replaced, or stones can be retrieved via the tract that is formed. Patient (parent) acceptance has been excellent.

Conclusion

Button cholecystostomy is simple to perform, relieves pruritus with intermittent (nighttime) drainage, avoids complications of a permanent stoma, avoids an enteric anastomosis, and is accepted by parents.