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.     

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.     

Protocolized management of infants with congenital diaphragmatic hernia: effect on survival

Background/Purpose

In 2006, we introduced a new protocol for congenital diaphragmatic hernia (CDH) management featuring nitric oxide in the delivery room, gentle ventilation, lower criteria for extracorporeal membrane oxygenation (ECMO), and appropriately timed operative repair on ECMO. Our goals were to assess outcomes after institution of this protocol and to compare results with historical controls.

Methods

Charts were reviewed of all newborns admitted to a large metropolitan children's hospital from 2002 to 2009 with a diagnosis of CDH. Data were recorded regarding delivery, ECMO, operative repair, length of stay, comorbidities/anomalies, complications, and survival. Postprotocol outcomes were compared to those from the preprotocol era and to data from the international CDH Registry.

Results

Comparison of the protocolized group (n = 43) to the historical group (n = 51) revealed no significant differences in gestational age, birth weight, Apgar scores, or comorbidities. New treatment strategies substantially improved survival to discharge (67% preprotocol, 88% postprotocol; P = .015). Among ECMO patients, survival increased to 82% (20% preprotocol; P = .002).

Conclusions

Our new protocol significantly improved survival to discharge for newborns with CDH. Institution of such a protocol is valuable in improving outcomes for patients with CDH and merits consideration for widespread adoption.     

Surgical treatment of biliary tract complications after liver transplantation

Objective

Biliary strictures are the most common biliary tract complication after liver transplantation. There are scarce data on the results of hepaticojejunostomy (HJ) in the management of biliary complications after orthotopic liver transplantation (OLT). Thus, the role of surgery in this setting remains to be established. The aim of this study was to evaluate the results of surgical treatment of patients with biliary complications at our institution.

Patients and Methods

We reviewed 1000 consecutive liver transplantations performed at our institution from 1984 to 2007. We used a prospectively recorded database to identify patients who underwent HJ to treat any biliary tract complication.

Results

Overall, 62 patients (6.2%) underwent HJ, 40 for an anastomotic and 7 for a non-anastomotic stricture as well as 15 for biliary leaks. Postoperative morbidity was 16%, and postoperative mortality 1.6%. There were 7 cases of anastomotic stenosis (11.3%). Four patients (5%) required retransplantation.

Conclusions

HJ is a safe procedure to manage biliary complications after OLT. It may be the first treatment choice especially for cases with anastomotic strictures.     

Treatment of toxic epidermal necrolysis in a pediatric patient with a nanocrystalline silver dressing

Background/Purpose

To report a case of toxic epidermal necrolysis (TEN) involving 90% body surface area, successfully treated with a nanocrystalline silver dressing (Acticoat, Smith & Nephew, Largo, FL).

Methods

A review of the hospital and acute wound center patient records and the recent English medical literature regarding TEN and nanocrystalline silver dressing.

Results

We found only 1 report of TEN treated with a nanocrystalline silver dressing. The nanocrystalline silver dressing was both effective in preventing wound infection and convenient for treating our patient with TEN.

Conclusion

The use of nanocrystalline silver dressing should be considered for the treatment of TEN.     

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.     

Malignancy development in lung transplant patients

Introduction

The most common neoplasias among transplant patients are skin cancers and lymphoproliferative disorders.

Objective

To characterize lung transplanted recipients who developed malignancies.

Methods

A retrospective analysis of clinical records of our patients.

Results

Seven patients developed malignancies: skin cancer (n = 5; 71%), and adenocarcinomas of prostatic, gastric, and lung (n = 1 each). One patient developed two hematologic malignancies: T-cell lymphoma and multiple myeloma. Among five patients who died (71%), 3 were due to advanced neoplasia. The mean presentation time was 4.3 years. Skin cancers were resected. The patient with lung adenocarcinoma developed pleural involvement and died. The patient with T-cell lymphoma was treated, but succumbed afterward due to multiple myeloma. The patient with gastric adenocarcinoma died at 3 months after the diagnosis, and the patient with prostate cancer underwent surgery without disease recurrence.

Conclusion

Malignancies are a late complication of transplant recipients that require a prompt diagnosis and treatment to improve outcomes.     

Endoscopic management of biliary complications after adult right-lobe living donor liver transplantation without initial biliary decompression

Objectives

We sought to examine biliary complications in adult right-lobe living donor liver transplantation (LDLT) with duct-to-duct anastomosis (RL-LDLT-DD), evaluating the efficacy of endoscopic retrograde cholangiography (ERC) in the diagnosis and management of biliary complications following LDLT.

Methods

Ninety adult RL-LDLT-DD were performed from June 2004 to August 2007, including 21 (23.3%) cases of biliary complications.

Results

The endoscopic retrograde cholangiopancreatiography (ERCP) findings were stricture only (n = 8), stricture plus leakage (n = 9), and leakage only (n = 4). In the overall 13 cases of leakage, nine patients recovered after treatment by stent or endoscopic nasobiliary drainage. The time to resolution was 3.0 ± 1.3 months with 2.2 ± 1.3 endoscopic examinations. All bile duct complications were treated by ERC first. Among 17 cases with stricture, seven cases were successfully treated by endoscopy and three cases by percutaneous transhepatic cholangiography plus stent (PTCS). In the other seven cases, the treatment was still ongoing in five cases and two subjects died during treatment. The mean time to stricture resolution 7.2 ± 3.3 months with 3.9 ± 1.4 endoscopic examinations. The results of 21 cases were 5/21 mortalities (23.8%), successful ERC treatment in 9/21; (42.9%), successful PTCS treatment in 3/21 (14.3%), and ongoing ERC treatment in 5/21, (23.8%), including one case with successful ERC treatment who died of lung infection postoperatively. During follow-up (13.1 ± 9.9 months), there was no recurrence in the stricture or leak.

Conclusions

When compared with the literature, RL-LDLT-DD without biliary drainage does not increase the incidence of biliary complications. From our study, ERC and PTC play a complementary roles in the treatment of bile duct complications.     

Histologic oddities at the porta hepatis in biliary atresia

Purpose

Highly unusual histologic findings at the porta hepatis in 3 infants who underwent Kasai portoenterostomy for biliary atresia are reported.

Methods

Portoenterostomy was performed using a standard operative technique. Serial transverse sections of the excised portal plate were examined by light microscopy along with sections from the distal extrahepatic biliary remnants, gallbladder, and liver biopsy.

Results

Of 61 consecutive infants who underwent Kasai portoenterostomy for biliary atresia, 3 were found to have highly unusual histologic features at the porta hepatis. All had type 3 biliary atresia. Two had hilar biliary ductules lined in part by squamous epithelium, and the third had a focus of mature hyaline cartilage surrounded by perichondrium adjacent to biliary ductules. In each case, these unusual histologic features were localized to the porta hepatis in the region of the transected portal plate.

Conclusions

The presence of hyaline cartilage at the portal plate is likely to be an expression of defective morphogenesis, thus supporting the concept of disordered embryogenesis in the etiology of biliary atresia. Squamous epithelium within biliary ductules might also reflect a similar mechanism but could alternatively be an unusual metaplastic response to inflammation at this site.     

Surgical outcomes in Alagille syndrome and PFIC: A single institution's 20-year experience

Background

Alagille Syndrome (AGS) and Progressive Familial Intrahepatic Cholestasis (PFIC) are rare pediatric biliary disorders that lead to progressive liver disease. This study reviews our experience with the surgical management of these disorders over the last 20 years.

Percutaneous Coronary Interventions in Cardiac Allograft Vasculopathy: A Single-Center Experience

Objecive

Cardiac allograft vasculopathy represents an accelerated form of obstructive coronary disease. It is the main cause of late death following heart transplantation. Percutaneous coronary intervention is considered a palliative procedure due to high restenosis rates. The aim of this study was to review our experience with percutaneous coronary interventions using stents in cardiac transplant recipients.

Methods

The present analysis included all primary adult heart transplanted patients who had been discharged from the hospital after transplantation, had a clinical follow-up of 12 months and underwent percutaneous coronary intervention (PCI).

Results

Seventy heart transplanted patients underwent percutaneous revascularization. Our analysis comprised 85 first-vessel procedures resulting in treatment of 135 lesions. The mean time from heart transplantation to first intervention was 9.3 ± 4.8 years. Primary success was obtained in 96% lesions; at least 1 recurrent stenosis event occurred in 16 patients with primarily successful PCI. Lesions treated with drug-eluting stents experienced recurrent stenosis in 16% of cases. During a mean follow-up after PCI of 45.2 ± 41.7 months, 27 deaths (19 cardiac) and 1 late re-transplantation occurred after PCI.

Conclusion

In cardiac transplant recipients, percutaneous coronary intervention with stents can be performed safely with high rates of primary success. Restenosis rates were higher compared with coronary interventions in native coronary arteries. Drug-eluting stents seemed to favorably impact restenosis compared with bare-metal stents. The clinical benefit from percutaneous coronary intervention may be reduced due to disease progression in untreated coronary segments.     

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.     

Thoracic spine pseudarthrosis treated by transpleural corporectomy and bone morphogenic protein

Background and purpose

Traumatic thoracic spine instability is a rare but difficult-to-treat entity, particularly above T5 where pedicle screwing is widely regarded as an unsatisfactory option and anterior surgery is considered difficult because of the anatomic relationship with the shoulder and large vessels.

Methods

A case of T4 and T5 pseudarthrosis after posterior fixation is reported. It was treated with transpleural corpectomy and fixation, with bone morphogenic protein used to obtain rapid arthrodesis between T3 and T6. No bracing or second posterior fixation was used.

Result

At 45 days of follow-up, the patient was without pain and the neurologic examination was normal. The CT scan showed bony bridges across all fracture lines. Complete cure of this pseudarthrosis was achieved with a single surgery. At the 12-month follow-up, the clinical status was normal.

Conclusions

The use of bone morphogenic proteins might be of great help in rare but challenging cases of spinal pseudarthrosis, with a quick return to normal activity after a single-stage surgery.     

Proteomics-based identification of spontaneous regression-associated proteins in neuroblastoma

Background

Spontaneous regression is usually found in stage 4s neuroblastoma, whereas the elucidation of the underlying molecular mechanism(s) is still limited.

Purpose

Our study aims to investigate the pathogenesis of spontaneous regression at the protein level.

Methods and materials

Differential expression of proteins in stage 4s neuroblastoma tissue, in stage 4 neuroblastoma tissue, and in normal adrenal tissue was investigated by use of 2-dimensional difference gel electrophoresis (2D-DIGE).

Results

Twenty-four protein spots were found to have significant changes among the different tissues, in which 16 proteins were identified using matrix-assisted laser desorption/ionization time-of-flight mass spectrometry (MALDI-TOF-MS). Among these proteins, 7 proteins (RhoGDP-dissociation inhibitor 1, phosphatidylethanolamine-binding protein, prohibitin, etc) were up-regulated and 2 proteins (F-actin capping protein 1 subunit and aldose reductase) were down-regulated in stage 4s neuroblastoma compared with stage 4 neuroblastoma. The differential expression of selected candidate protein (RhoGDP-dissociation inhibitor 1 and CAPZA1) was further validated by western blotting.

Conclusion

Some proteins are differentially expressed between stage 4s and stage 4 neuroblastoma tissue, including those associated with differentiation and proliferation as well as apoptosis. RhoGDP-dissociation inhibitor 1 is highly expressed in stage 4s neuroblastoma tissue, whereas CAPZA1 is down-regulated.     

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.     

Liver Transplantation Complications in the Intensive Care Unit and at 6 Months

Objective

This study sought to determine the factors that influence the 6-month outcomes of liver transplants.

Patients and Methods

One hundred ninety-six variables (donor, recipient, operation, intensive care unit [ICU], evolution at 3 and 6 months) were collected from the first 74 consecutive liver transplantation performed from 2002 to 2004. The primary endpoint was patient survival at 6 months. The statistical analysis included a screening univariate analysis followed by a stepwise logistic regression with forward inclusion to test independent associations and finally generation of receiver-operator characteristic (ROC) curves to evaluate predictive factors.

Results

Patient survival at 6 months was 86%, namely 10 deaths, including 4 intraoperatively and 6 postoperatively due to sepsis. Complications in the ICU were classified as reoperations due to biliary problems, vascular complications, and peritonitis. Late complications included 51% rejection episodes, 24% infections, 11% pleural effusions, and 16% diabetes mellitus. Logistic regression analysis showed independent negative predictors of survival were the number of packed red cells during transplantation, the number of fresh frozen plasma units administered in the ICU, blood urea nitrogen (BUN) concentration in the ICU, and graft complications. The odds ratios of these variables were 10.2, 5.2, 42.1, and 36.9, respectively. The area under the curve (AUC) of the ROC was 0.99; the sensitivity was 94%; and the specificity was 100%. The independent predictors of surgical complications were the length of the operation, the need for pressor support, and the number of fresh frozen plasma units administered in the operating room, with odds ratios of 1.0, 7.7, and 1.1, respectively.

Conclusion

This study revealed specific operative and ICU variables that correlated with the evolution of our patients.     

Biliary complications after orthotopic liver transplantation from donors after cardiac death: broad spectrum of disease

Background

Donation-after-death liver transplantation (DCD-LT) carries higher complication rates compared with donation-after-brain death liver transplantation (DBD-LT). In this report we describe our experience with biliary complications in DCD-LT with emphasis on anatomical patterns and outcomes.

Materials and Methods

We performed retrospective review of patients' medical records from August 2004 to December 2008, during which time total of 26 DCD-LTs were performed. Mean follow-up was 29 months (range 3 to 51 months).

Results

Biliary complications occurred in 12 patients (46%), of whom 9 were related to DCD (35%). Four patients had more than 1 biliary complication, and 4 had concomitant arterial problems (stricture/thrombosis). Treatment of complications included: ERCP (n = 5, 3 resolved), conversion to roux (n = 5, 2 resolved), revision of roux (n = 1), percutaneous transhepatic cholangiography (n = 1), artery revision (n = 3). Three patients with casts had operative extraction of casts depicting a mummified biliary tree; histology showed casts and fibrosis and anastomotic suture material. Six patients underwent retransplantation (23%). Among retransplanted patients, 2 deaths occurred (7.7%).

Conclusion

Our experience with DCD-LT reveals a high prevalence of biliary complications with a new and wide spectrum of clinicopathologic findings. Better strategies for prevention of these unique biliary complications are needed to better justify the added risks and costs for performance of DCD-LT.     

Ipsilateral talar and calcaneal fractures: A retrospective review of complications and sequelae

Background

Ipsilateral talar and calcaneal fractures represent a rare combination injury that has only recently been reported in the literature with small case series.

Objective

To identify the commonly observed fracture patterns, complications, and outcomes of a consecutive series of patients with ipsilateral talar and calcaneal fractures.

Methods

Forty-five cases of ipsilateral talus and calcanal fractures were identified from an orthopaedic trauma registry at a University-based, level I trauma center for retrospective review.

Main outcome measurements

Post-operative complications, the need for secondary surgery, and the visual analogus pain score.

Results

Five patients were treated with an early below knee amputation (BKA). Five patients were treated with a primary subtalar arthrodesis. Twenty-eight of the 35 patients who did not undergo early BKA or primary subtalar arthrodesis developed subtalar arthritis. Five patients had deep wound complications. Four patients had talar body collapse from avascular necrosis. There were 13 open fractures of which 8 resulted in an eventual BKA. The mean visual analogus pain score for the patient population was 4.0.

Conclusion

The combination of ipsilateral talar and calcaneal fractures represents a severe injury pattern that is associated with significant morbidity. Subtalar arthritis was a common finding regardless of treatment. Open fractures frequently resulted in a below knee amputation.