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.     

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.     

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.     

Partial internal biliary diversion through a cholecystojejunocolonic anastomosis--a novel surgical approach for patients with progressive familial intrahepatic cholestasis: a preliminary report

Background/Purpose

The purpose of the study was to describe the initial experience with a novel approach to the surgical treatment of progressive familial intrahepatic cholestasis (PFIC), avoiding the creation of a permanent stoma.

Methods

Two teenaged patients, aged 15 and 17 years, underwent partial internal biliary diversion to treat incontrollable pruritus associated with PFIC. The surgical technique involved the creation of an isolated jejunal conduit, anastomosed proximally in a terminolateral fashion to the gallbladder and distally to the ascending colon. This operation combines the advantages of partially diverting the biliary flow from the enterohepatic cycle, avoiding an external biliary fistula. In one of the patients, this technique was used as a primary procedure, whereas in the other, a previous partial external diversion was converted to an internal diversion.

Results

Both patients had complete resolution of their pruritus and normalization of hepatic laboratory tests. One of the patients developed a mild choleretic diarrhea that can be controlled with eventual use of cholestyramine. No complications were observed related to this operation.

Conclusions

Biliary diversion appears to be a very attractive surgical option for the treatment of PFIC in children with a normal gallbladder. Long-term follow-up is necessary to evaluate late results and eventual complications of this approach.     

Cholestasis associated with small bowel atresia: do we always need to investigate?

Background/Purpose

Cholestasis occurs frequently in patients with small bowel atresia (SBA) and is often attributed to prolonged parental nutrition. When severe or prolonged, patients may undergo unnecessary intensive or invasive investigation. We characterized cholestasis and analyzed the pertinence of investigating this patient population.

Methods

With Research Ethics Board approval, patients with SBA between 1996 and 2005 were retrospectively reviewed. Demographics, location of atresia, operative findings, complications, investigations, resumption of feeding, duration of prolonged parental nutrition, and follow-up information were examined. Cholestasis was evaluated for incidence, severity, and evolution.

Results

Fifty-five patients (29 male, 26 female), with a median gestational age and birth weight of 36 weeks and 2025 g, respectively, were reviewed. Care was withdrawn for 2 patients before repair. For the remaining 53 patients, SBA were duodenal atresia in 18, jejunoileal atresia in 32, and multiple atresia in 3. Of 53, 24 (45%) patients developed cholestasis postoperatively (direct/total bilirubin >20%). All patients with short bowel (4) and 60% (6/10) of patients with a delay of enteral feeding more than 14 days postoperatively had cholestasis. Ten patients (36%) proceeded with in-depth evaluations for cholestasis, with 8 (28%) undergoing liver biopsy. No patient had biliary atresia. No deaths were related to isolated cholestasis/cirrhosis. Cholestasis resolved spontaneously in all the survivors.

Conclusions

Small bowel atresia is frequently associated with postoperative cholestasis that will resolve with time. We recommend a more selective and expectant approach to SBA-associated cholestasis to minimize unnecessary investigations.     

Nontransplant surgical interventions in progressive familial intrahepatic cholestasis

Background

Progressive familial intrahepatic cholestasis (PFIC) is a family of rare childhood diseases that was universally fatal until the development of liver transplant. In the last 20 years, the use of nontransplant surgery to treat PFIC has become the standard of care. There are various surgical techniques that have been performed. There are no reviews evaluating the outcome of these operations.

Methods

A systematic search of the literature for articles evaluating the outcome of nontransplant surgical interventions in PFIC patients was performed. Data from these studies was abstracted and summarized.

Results

No trials have been performed addressing nontransplant surgical interventions in PFIC patients. We analyzed 11 case series and case reports. Generally, patients had successful outcomes (81%) with cessation of progression of disease and resolution of symptoms. Treatment failures were often associated with more advanced disease.

Discussion

There is no evidence to demonstrate a superiority of one type of nontransplant surgical intervention in PFIC patients. We propose the development of a registry and standardization of outcomes measurements to allow improved comparison of results.     

The challenge of pediatric continent urinary diversion

Objective

Continent urinary diversion (CUD) may be required for refractory incontinence in children with various malformations. We review our experience with CUD to identify determinants of success and ongoing challenges.

Methods

Retrospective chart review of 43 consecutive patients undergoing CUD since 1991 at British Columbia Children's Hospital.

Results

Our preferred surgery was intestinal cystoplasty and either appendicovesicostomy (77%) or ileal-vesicostomy. Concomitant bladder neck surgery was performed in 67%. Mean follow-up was 2.5 years. There was a 16% revision rate for persistent leakage, the most common being cystoscopic injection of bulking agents. Continence was ultimately achieved in 88%. Early major and minor postoperative complications each occurred in 21% of cases. Delayed minor complications requiring surgical intervention occurred in 30% including stomal stenosis in 6 patients and stomal prolapse in 2. Urolithiasis required intervention in 5 patients.

Conclusions

Pediatric CUD is a challenging endeavor. Most patients achieve continence although many require additional minor procedures to do so. Early major and minor complications are common. A significant minority of patients require reoperation for delayed minor complications. Patients and families should be informed of these frustrations as part of their preoperative counseling. A high degree of family motivation and commitment is essential.     

Prenatal and postnatal ciliated hepatic foregut cysts in infants

Purpose

Ciliated Hepatic Foregut Cyst (CHFC) is a rare congenital lesion arising from the embryonic foregut. Since squamous cell carcinomas arising from CHFC have been reported in adults, complete resection should be considered. We report our experience with CHFC.

Methods

We reviewed the charts of 2 patients who had surgery after prenatal detection of a CHFC and 2 patients with postnatal diagnosis.

Results

Two patients had antenatally detected liver cyst. Postnatal ultrasonography showed a cyst in segment IV, with wall calcifications and sediments. Bile ducts were encased in the wall of the cyst. They underwent central hepatectomy with double biliary diversion and uneventful post operative course. The two other patients underwent non anatomical resection of a cyst on the left lobe and in segment IV, found prior or during liver surgery.Pathology examination showed cysts filled with mucinous fluid, surrounded by an epithelium composed of ciliated cells. One case had a squamous metaplasia.

Conclusion

In infants, CHFC are found antenatally or incidentally. A solitary uni or mutilocular cyst with wall calcifications, sediments, located in the central liver segments should raise the diagnosis. Resection of large cysts in the central segments of the liver is challenging and biliary diversion should be considered.     

Ileal exclusion for refractory symptomatic cholestasis in Alagille syndrome

Background

Alagille syndrome (AGS) can result in pruritic self-mutilation and disabling or disfiguring xanthomas. Though external biliary diversion and transplantation have been described for AGS, few data exist for the use of ileal exclusion (IE) in this setting.

Methods

Three patients with AGS with symptomatic cholestasis despite maximal medical management underwent IE. In each case, small bowel length was measured and the terminal 15% of ileum was excluded using stapled division and ileocecal anastomosis. Symptom scores were collected after institutional review board approval and are presented here as mean (range). Pruritus and xanthomas were graded as follows: 0 = none, 1 = mild scratching/minimal, 2 = active scratching/moderate, 3 = abrasions/disfiguring, 4 = mutilation/disabling.

Results

Mean follow-up was 30 months (4-45 months). Pruritus score decreased from 3.33 (3-4) to 0.33 (0-1). Xanthoma score decreased from 3.67 (3-4) to 1.67 (1-2). All patients were maintained on nutritional supplements pre- and postoperatively without a change in management. No patients experienced diarrhea or dehydration postoperatively. There were no complications.

Conclusions

Ileal exclusion effectively decreases refractory pruritus and xanthoma burden in AGS. This procedure offers the advantages of reversibility, avoidance of a stoma, and technical ease. Ileal exclusion should be considered for symptomatic AGS refractory to medical management as an alternative to external biliary diversion or liver transplantation.     

Functional liver recovery parallels autologous gut salvage in short bowel syndrome

Background/purpose

Parenteral nutrition (PN) is life saving in short bowel syndrome. However, long-term parenteral nutrition is frequently complicated by a syndrome of progressive cholestatic liver disease that is considered to be irreversible beyond the early stages of cholestasis, particularly in the presence of any degree of fibrosis in the liver. The purpose of this study was to examine apparent improvement in PN-associated liver dysfunction in a cohort of children with short bowel syndrome.

Methods

A retrospective case-record review of all patients managed within a dedicated Intestinal Rehabilitation Program (IRP) identified 13 patients with short bowel who had PN-associated liver dysfunction, defined for this purpose as hyperbilirubinemia or an abnormal liver biopsy.

Results

At referral, 12 of the 13 patients were exclusively on PN, and one was on 50% PN. At current follow-up, 3 patients have achieved complete enteral autonomy from PN, and 7 patients have had smaller decrements in PN requirements. Specific operative procedures to improve intestinal function were undertaken in 11 patients; 4 patients also underwent cholecystectomies with biliary irrigation at the time of intestinal reconstruction. The median highest bilirubin level in these 13 patients was 10.7 mg% (range, 3.2 to 24.5 mg%). Liver biopsy results indicated that 5 patients were cirrhotic, 3 had bridging fibrosis, and 4 had severe cholestasis or lesser degrees of fibrosis. Of 10 survivors in this series, 9 patients currently have a serum bilirubin less than 1 mg% with a median bilirubin in the group of 0.6 mg% (range, 0.3 to 6.4 mg%). Twelve of the 13 patients in this series were initially referred for liver-small bowel transplantation.

Conclusions

This preliminary experience suggests that PN-dependent patients with advanced liver dysfunction in the setting of the short bowel syndrome may, in some instances, experience functional and biochemical liver recovery. The latter appears to parallel autologous gut salvage in most cases. As a corollary, the authors believe that even advanced degrees of liver dysfunction should not preclude attempts at autologous gut salvage in very carefully selected patients. Such a policy of “aggressive conservatism” may help avoid the need for liver/intestinal transplantation in some patients who appear to be not responding to PN.     

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.     

Effects of bilirubin ditaurate overload on canalicular membrane function and ultrastructure of the pig liver

Aim

Bilirubin overload caused by haemolysis of transfused blood and breakdown of extravasated blood constitutes an important causative factor of jaundice in trauma patients. Intravenous infusions of large amounts of unconjugated bilirubin (UCB) block biliary phospholipid secretion and produce canalicular membrane lesions in pigs, putatively because of enhanced cytotoxicity of bile. Severe intrahepatic cholestasis is the functional end result. The aim of the study was to investigate whether bilirubin ditaurate (BDT) overload, which also inhibits biliary phospholipid secretion, also induces intrahepatic cholestasis.

Methods

Six pigs were infused with 2.8 g of BDT for 150 min. Six control pigs were infused with albumin sham solution for 150 min. Bile samples were analysed for bile acid- and phospholipid-secretion rates. Bile and serum samples were analysed for bilirubin concentration. Liver biopsies were obtained for scanning electron microscopic studies (SEM).

Results

Biliary bile acid secretion fell by 7.7% and biliary phospholipid secretion rate fell by 88% after BDT infusion. Thus, infusion of BDT for 150 min did not cause intrahepatic cholestasis. SEM showed some variability in the size of canalicular membrane microvilli, but no evidence of gross destruction.

Conclusion

BDT overload markedly lowers biliary phospholipid secretion. In contrast to UCB, BDT does not induce canalicular membrane damage nor cause intrahepatic cholestasis. Sustained, marked inhibition of phospholipid secretion does therefore not adequately explain UCB-induced cholestasis. Accumulation of UCB in the canalicular membrane may be the important factor in the pathogenesis of canalicular membrane lesions and intrahepatic cholestasis during UCB overload.     

Surgical intervention in the setting of parenteral nutrition-associated cholestasis may exacerbate liver injury

Purpose

This study compares postoperative markers of liver injury in patients receiving intravenous fish oil (IFO) with parenteral nutrition (PN)-associated cholestasis (PNAC) to patients with resolved PNAC.

Methods

A retrospective review of all cholestatic-IFO patients undergoing abdominal laparotomy between March 1, 2007, and July 1, 2009, led to inclusion of 23 patients who collectively underwent 27 abdominal operations (13 pre-PNAC resolution and 14 post-PNAC resolution). Direct bilirubin (DB), total bilirubin, and alanine aminotransferase levels were examined over time in relation to operations. The time to resume presurgical trend of decreasing DB was calculated.

Results

Sixty-nine percent (9/13) of pre-PNAC resolution procedures were associated with postoperative increase in DB compared with 7% (1/14) of post-PNAC resolution procedures associated with a recurrence of cholestasis (P = .02; odds ratio, 29.3; 95% confidence interval, 2.79-306.8). The median time to return to the preoperative downward trend of DB was 21 days.

Conclusions

Operations before PNAC resolution may be associated with an increased postoperative DB, possibly reflecting an exacerbation of liver injury. Operations post-PNAC resolution on IFO had a comparatively low incidence of postoperative cholestasis recurrence. Excepting clinical indication otherwise, it may be advisable to delay surgical intervention in the setting of PNAC in certain cases.     

The Malone antegrade continence enema procedure: the Amsterdam experience

Background

The Malone antegrade continence enema (MACE) procedure has been previously described as a safe and effective option for the treatment of children with chronic defecation disorders when maximal medical therapy and conventional treatment have failed.

Purpose

To evaluate clinical success, complications, and quality of life of children with chronic defecation disorders with a MACE stoma.

Methods

A retrospective analysis of 23 patients who underwent the construction of a MACE stoma was performed. Preoperative and postoperative data were evaluated. A specific questionnaire was used to assess patient satisfaction.

Results

A significant increase was found in defecation frequency (1.0 [range, 0-4] pretreatment vs 5.5 [range, 0-28] posttreatment per week; P < .006) and a significant decrease in fecal incontinence frequency (10 [range, 0-14] pretreatment vs 0 [range, 0-14] posttreatment per week; P < .034). Postoperative complications of the MACE procedure were fecal leakage (43%), wound infection (52%), and stomal stenosis (39%). A total of 86% of the patients were satisfied with the results of the Malone stoma (n = 21).

Conclusions

The MACE procedure is an effective treatment in children with intractable defecation disorders. Postoperative complications are, however, not uncommon. Further refinement of the technique focused to reduce the complication rate is necessary to expand the application of this approach.     

Conscious recovery response in post-hepatic transplant as a function of time-related acute hepatic encephalopathy

Background

H Coma due to acute hepatic failure produces a high mortality rate with rapid progression of cerebral edema and brain herniation. Early transplantation increases survival of patients with acute hepatic encephalopathy. In previous studies, scant attention has focused on the conscious recovery time after living donor liver transplantation (ldlt) and whether the conscious recovery time was directly proportional to the length of coma before transplantation.

Patients and methods

We have reported herein three adult patients with decompensated chronic end-stage liver disease who underwent right lobe LDLT. Their general conditions had markedly deteriorated; two patients displayed massive ascites. All three subjects displayed grade IV encephalopathy with endotracheal intubation and intensive care management. Their biochemical data revealed hyperammonemia, marked cholestasis, and coagulopathy.

Results

After LDLT the patients recovered from coma at a mean time similar to that in coma. Preoperatively the patients exhibited acute deep coma with respiratory failure on preoperative days 5, 3, and 1 with consciousness regained on postoperative day 5, 3 and 1, respectively.

Conclusion

We suggest that patients with acute deep coma (grade IV), who were formerly regarded as irreversible, benefit with LDLT, preventing worsening of complications, and that shows a time-dependent recovery the pretransplant comatose status.     

Intravenous landiolol, a novel β(1)-adrenergic blocker, reduces the minimum alveolar concentration of sevoflurane in women

Study Objective

To investigate the effect of intravenous (IV) landiolol, a novel β₁-adrenergic blocker, on the minimum alveolar concentration (MAC) of sevoflurane in adult women.

Design

Prospective, randomized study.

Setting

University hospital.

Patients

42 ASA physical status 1 and 2 women, aged 24-57 years, who were scheduled to undergo elective abdominal surgery.

Interventions

Anesthesia was induced in all patients by vital capacity rapid inhalation induction of sevoflurane. In the landiolol group, administration of landiolol began when patients took a vital-capacity breath: 0.125 mg/kg/min for one minute and then 0.04 mg/kg/min. Normal saline was administered in the control group.

Measurements

MAC was determined by a technique adapted from the conventional up-down method.

Main Results

The MAC of sevoflurane was 2.2% ± 0.2% in the control group and 1.7% ± 0.2% in the landiolol group, a statistically significant difference (P = 0.0005).

Conclusions

IV landiolol reduces the MAC of sevoflurane in women by approximately 20%.     

Betel leaf in stoma care

Background/Purpose

Construction of a stoma is a common procedure in pediatric surgical practice. For care of these stomas, commercially available devices such as ostomy bag, either disposable or of longer duration are usually used. These are expensive, particularly in countries like Bangladesh, and proper-sized ones are not always available. We have found an alternative for stoma care, betel leaf, which is suitable for Bangladeshis. We report the outcome of its use.

Methods

After construction of stoma, at first zinc oxide paste was applied on the peristomal skin. A betel leaf with shiny, smooth surface outwards and rough surface inwards was put over the stoma with a hole made in the center according to the size of stoma. Another intact leaf covers the stomal opening. When bowel movement occurs, the overlying intact leaf was removed and the fecal matter was washed away from both. The leaves were reused after cleaning. Leaves were changed every 2 to 3 days. From June 1998 to December 2005, in the department of pediatric surgery, Chittagong Medical College and Hospital, Chittagong, Bangladesh, a total of 623 patients had exteriorization of bowel. Of this total, 495 stomas were cared for with betel leaves and 128 with ostomy bags.

Results

Of 623 children, 287 had sigmoid colostomy, 211 had transverse colostomy, 105 had ileostomy, and 20 had jejunostomy. Of the 495 children under betel leaf stoma care, 13 patients (2.6%) developed skin excoriation. There were no allergic reactions. Of the 128 patients using ostomy bag, 52 (40.65%) had skin excoriation. Twenty-four (18.75%) children developed some allergic reactions to adhesive. Monthly costs for betel leaves were 15 cents (10 BDT), whereas ostomy bags cost about US$24.

Conclusion

In the care of stoma, betel leaves are cheap, easy to handle, nonirritant, and nonallergic.     

Treatment of early hepatic artery thrombosis after liver transplantation

Introduction

Early hepatic artery thrombosis remains one of the major causes of graft failure and mortality in liver transplant recipients. It is the most frequent severe vascular complication after orthotopic liver transplantation (OLT) accounting for >50% of all arterial complications. Most patients need to be considered for urgent liver retransplantation.

Materials and Methods

Among 911 OLTs in 862 from 1989 to 2011, we observed 23 cases (2.6%) of acute early hepatic artery thrombosis. Seventeen patients were qualified immediately for liver retransplantation, and 6 underwent endovascular therapies, including intra-arterial heparin infusion or percutaneous transluminal angioplasty with stent placement.

Results

Among patients who were assigned to early liver retransplantation, 11/17 survived with 3 succumbling due to postoperative complications, including 1 portal vein thrombosis, and 3 succumbling on the waiting list. All patients who underwent endovascular therapy survived with an excellent result obtained in 1 who underwent treatment <24 hours after arterial thrombosis. In 2 patients we achieved a satisfactory result not requiring retransplantation, but 3 patients assigned to endovascular treatment >24 hours after arterial thrombosis needed to be reassigned to liver retransplantation because of poor results of endovascular treatment.

Conclusions

Endovascular treatment efforts should be made to rescue liver grafts through urgent revascularization depending on the patient's condition and the interventional expertise at the transplant center, reserving the option of retransplantation for graft failure or severe dysfunction.