Non-operative management of biliary complications after Liver Transplantation in pediatric patients: A 30-year experience.

BACKGROUND: To evaluate the efficacy of percutaneous and endoscopic therapeutic interventions for biliary strictures and leaks following LT in children. METHODS: Retrospective analysis of 49 consecutive pediatric liver transplant recipients (27 girls, 22 boys, mean age at transplant 3.9 years) treated at our institution from 1989 to 2019 for biliary leak and/or biliary stricture was performed. Minimally invasive approach was considered clinically successful if it resulted in patency of the narrowed biliary segment and/or correction of the biliary leak. RESULTS: Forty-two patients had a stricture at the biliary anastomosis; seven had a biliary leak. After an average 13.8 years of follow-up, long-term clinical success with minimally invasive treatment (no surgery or re-transplant) was achieved for 24 children (57%) with biliary stricture and 4 (57%) with biliary leaks. Eight patients required re-transplant; however, only one was due to failure of both percutaneous and surgical management. For biliary strictures, failure of non-surgical management was associated with younger age at stricture diagnosis (p < .02). CONCLUSIONS: Percutaneous and endoscopic management of biliary strictures and leaks after LT in children is associated with a durable result in >50% of children.

Burden of Ionizing Radiation in the Diagnosis and Management of Necrotizing Pancreatitis.

INTRODUCTION: A step-up endoscopic or percutaneous approach improves outcomes in necrotizing pancreatitis (NP). However, these require multiple radiographic studies and fluoroscopic procedures, which use low-dose ionizing radiation. The cumulative radiation exposure for treatment of NP has not been well defined. METHODS: We conducted a retrospective study of consecutive patients with NP admitted to University of California San Francisco Medical Center from January 2011 to June 2019. We calculated effective doses for fluoroscopic procedures using the dose area product and used the National Cancer Institute tool for computed tomography studies. The primary outcome was the cumulative effective dose (CED). Multivariable logistic regression was used to evaluate risk factors of high exposure (CED > 500 mSv). RESULTS: One hundred seventy-one patients with NP (mean follow-up 40 ± 18 months) underwent a median of 7 (interquartile range [IQR] 5-11) computed tomography scans and 7 (IQR 5-12) fluoroscopic procedures. The median CED was 274 mSv (IQR 177-245) and 30% (51) of patients received high exposure. Risk factors of high exposure include multiorgan failure (aOR 3.47, 95%-CI: 1.53-9.88, P = 0.003), infected necrosis (adjusted odds ratio [aOR] 3.89 95%-CI:1.53-9.88, P = 0.005), and step-up endoscopic approach (aOR 1.86, 95%-CI: 1.41-1.84, P = 0.001) when compared with step-up percutaneous approach. DISCUSSION: Patients with NP were exposed to a substantial amount of ionizing radiation (257 mSv) as a part of their treatment, and 30% received more than 500 mSv, which corresponds with a 5% increase in lifetime cancer risk. Severity of NP and a step-up endoscopic approach were associated with CED > 500 mSv. Further studies are needed to help develop low-radiation treatment protocols for NP, particularly in patients receiving endoscopic therapy.

Endoscopy is of low yield in the identification of gastrointestinal neoplasia in patients with dermatomyositis: A cross-sectional study.

AIM: To determine the prevalence of gastrointestinal neoplasia among dermatomyositis patients who underwent an esophagogastroduodenoscopy and/or colonoscopy. METHODS: A cross-sectional study examining the results of upper endoscopy and colonoscopy in adults with dermatomyositis at an urban, university hospital over a ten year period was performed. Chart review was performed to confirm the diagnosis of dermatomyositis. Findings on endoscopy were collected and statistical analyses stratified by age and presence of symptoms were performed. RESULTS: Among 373 adult patients identified through a code based search strategy, only 163 patients had dermatomyositis confirmed by chart review. Of the 47 patients who underwent upper endoscopy, two cases of Barrett's esophagus without dysplasia were identified and there were no cases of malignancy. Of the 67 patients who underwent colonoscopy, no cases of malignancy were identified and an adenoma was identified in 15% of cases. No significant differences were identified in the yield of endoscopy when stratified by age or presence of symptoms. CONCLUSION: The yield of endoscopy is low in patients with dermatomyositis and is likely similar to the general population; we identified no cases of malignancy. A code based search strategy is inaccurate for the diagnosis of dermatomyositis, calling into question the results of prior population-based studies. Larger studies with rigorously validated search strategies are necessary to understand the risk of gastrointestinal malignancy in patients with dermatomyositis.

A 13 year-old boy with post-transplantation lymphoproliferative disorder presenting with obscure gastrointestinal bleeding: a case report.

One well recognized and potentially serious complication of chronic immunosuppression in organ transplant recipients is post-transplantation lymphoproliferative disorders (PTLD). This accounts for 20% of all malignancies in transplant recipients, which is four times higher than the general population (1,2). The diagnosis of PTLD is often difficult, due to various manifestations resulting in late diagnosis. We report an unusual presentation of PTLD in a pediatric patient where the diagnosis was achieved only after extensive investigation.

Inoperable pancreatic cancer patients who have prolonged survival exhibit an increased risk of cholangitis.

CONTEXT: Endoscopically placed metal stents, which are patent for 4-9 months, have been the favored decompressive strategy for biliary obstruction due to inoperable pancreatic cancer in order to minimize interventions. However, in the past decade chemotherapeutic options have improved survival. This raises the question of whether metal stents will continue to be the optimal method of decompression. OBJECTIVE: We performed a study to determine the outcome of patients with non-operatively managed pancreatic adenocarcinoma with regards to the development of cholangitis. DESIGN: We reviewed all ERCP performed for malignant distal biliary obstruction in between December 1999 and December 2005 at University of California, San Francisco (UCSF). PATIENTS: Only patients who received chemotherapy for pancreatic adenocarcinoma were included. Patients who underwent surgical biliary diversion procedures were excluded. PRIMARY OUTCOME MEASUREMENT: The primary outcome was the development of cholangitis requiring hospitalization. RESULTS: Among 200 patients with malignant distal biliary obstruction who underwent endoscopic biliary decompression procedures, 54 met study criterion. Metal stents were employed in 90.7% of these cases. The median survival of this population was 12.7 months (range: 2.6-34.6 months). Only 3 of 26 patients (11.5%) surviving one year or less developed cholangitis compared to 13 of 28 (46.5%) who survived more than one year. Thus patients surviving greater than one year had a five fold increase in the odds of developing cholangitis (odds ratio: 4.92; P=0.017). CONCLUSIONS: This cohort of inoperable pancreatic cancer patients undergoing chemotherapy survived longer than the expected patent period of metal stents employed for biliary decompression. The occurrence of cholangitis requiring hospitalization does increase markedly among long term survivors.

Predictors of endoscopic treatment outcomes in the management of biliary problems after liver transplantation at a high-volume academic center.

BACKGROUND: Biliary tract problems are the most common complications after liver transplantation. ERCP is increasingly being used to address posttransplantation biliary problems. OBJECTIVE: To identify predictors of endoscopic treatment outcomes in the management of post-liver transplantation complications. SETTING AND PATIENTS: All adult patients who underwent liver transplantation at the University of California, San Francisco between January 1999 and December 2008 were reviewed. DESIGN: A multivariate regression analysis. MAIN OUTCOME MEASUREMENTS: Identification of donor and recipient factors as well as technical considerations that predicted success or failure in the endoscopic management of posttransplantation biliary complications. RESULTS: In 1062 patients who underwent liver transplantation, there were 224 biliary complications. ERCP was the primary treatment modality and was successful in the majority of patients treated. Patients with biliary complications who had take-back surgery for a nonbiliary indication during the first month after liver transplantation (odds ratio [OR], 0.32; P = .03), particularly for bleeding (OR, 0.18; P = .02), were less likely to respond to endoscopic therapy. Those who received a graft from a donor after cardiac death (OR, 0.15; P = .02) or a living donor (OR, 0.11; P < .01) were also less likely to respond to endoscopic therapy. Take-back surgery for a nonbiliary indication in the first month after liver transplantation was also identified as a novel risk factor for the development of biliary complications (OR, 1.80; P = .02). LIMITATIONS: Retrospective design. CONCLUSIONS: ERCP can be used to treat the majority of posttransplantation biliary problems. However, endoscopic therapy is less efficacious in the treatment of complications associated with ischemia.

Management of biliary complications in the liver transplant patient.

Biliary complications after liver transplantation remain common. Resources for livers are limited, and these individuals are often ill, making nonoperative treatment and management attractive options. The endoscopic route for evaluation (endoscopic retrograde cholangiopancreatography) remains preferable, due to its safety profile, as opposed to the percutaneous route (percutaneous transhepatic cholangiography with percutaneous transhepatic biliary drainage), though the endoscopic route may not be possible in patients with a Roux-en-Y reconstruction. The two most common early complications include leaks from the anastomosis or cystic duct stump (of the donor or native duct) and obstruction at the surgical anastomosis. Nonoperative treatment is often successful in early complications. Late complications presenting with leaks and obstruction are often more difficult to treat nonoperatively and frequently require surgical treatment or retransplantation, though both endoscopic and percutaneous methods can be useful in the management of these complications or as a bridge to definitive surgical therapy.

The outcome of laparoscopic Heller myotomy for achalasia is not influenced by the degree of esophageal dilatation.

In the past, a Heller myotomy was considered to be ineffective in patients with achalasia and a markedly dilated or sigmoid-shaped esophagus. Esophagectomy was the standard treatment. The aims of this study were (a) to evaluate the results of laparoscopic Heller myotomy and Dor fundoplication in patients with achalasia and various degrees of esophageal dilatation; and (b) to assess the role of endoscopic dilatation in patients with postoperative dysphagia. One hundred and thirteen patients with esophageal achalasia were separated into four groups based on the maximal diameter of the esophageal lumen and the shape of the esophagus: group A, diameter<4.0 cm, 46 patients; group B, esophageal diameter 4.0-6.0 cm, 32 patients; group C, diameter>6.0 cm and straight axis, 23 patients; and group D, diameter>6.0 cm and sigmoid-shaped esophagus, 12 patients. All had a laparoscopic Heller myotomy and Dor fundoplication. The median length of follow-up was 45 months (range 7 months to 12.5 years). The postoperative recovery was similar among the four groups. Twenty-three patients (20%) had postoperative dilatations for dysphagia, and five patients (4%) required a second myotomy. Excellent or good results were obtained in 89% of group A and 91% of groups B, C, and D. None required an esophagectomy to maintain clinically adequate swallowing. These data show that (a) a laparoscopic Heller myotomy relieved dysphagia in most patients with achalasia, even when the esophagus was dilated; (b) about 20% of patients required additional treatment; (c) in the end, swallowing was good in 90%.

Magnetic resonance cholangiography of biliary strictures after liver transplantation: a prospective double-blind study.

PURPOSE: To compare magnetic resonance cholangiography (MRC) with endoscopic retrograde cholangiography (ERC) in quantitatively evaluating biliary strictures in liver transplant recipients. MATERIALS AND METHODS: Eight liver transplant recipients with suspected biliary complications were referred for ERC and also underwent MRC within 24 hours using a combination of single-shot rapid acquisition with relaxation enhancement (SS-RARE) and three-dimensional (3D)-RARE sequences. The studies were independently interpreted by two blinded radiologists and a single blinded endoscopist who recorded the presence of a stricture and/or upstream dilatation, the ratio of recipient-to-donor duct diameters at the anastomosis, as well as the proximal duct diameter, length, and percent stenosis of any stricture detected. RESULTS: Using ERC as the standard of reference, MRC had a sensitivity and negative predictive value of 100%, mean specificity of 83.3%, and mean positive predictive value of 92.9% in the detection of six strictures. Compared with ERC, MRC obtained accurate measurements of recipient-to-donor duct diameter ratios (r, 0.91; P < 0.01), proximal duct diameters (r, 0.83, P < 0.05), stricture lengths (r, 0.58; P = 0.06), and percent stenosis (r, 0.78; P = 0.06). CONCLUSION: MRC can provide equivalent imaging to ERC and can reliably identify and quantitatively evaluate biliary strictures in post-orthotopic liver transplantation (OLT) patients.

Spectrum of esophageal motility disorders: implications for diagnosis and treatment.

BACKGROUND: The named primary esophageal motility disorders (PEMDs) are achalasia, diffuse esophageal spasm (DES), nutcracker esophagus (NE), and hypertensive lower esophageal sphincter (HTN-LES). Although the diagnosis and treatment of achalasia are well defined, such is not the case with the other disorders. HYPOTHESIS: (1) Symptoms do not reliably distinguish PEMDs from gastroesophageal reflux disease; (2) esophageal function tests are essential to this distinction and to identifying the type of PEMD; (3) minimally invasive surgery is effective for each condition; and (4) the laparoscopic approach is better than the thoracoscopic approach. DESIGN: University hospital tertiary care center. SETTING: Retrospective review of a prospectively collected database. PATIENTS AND METHODS: A diagnosis of PEMD was established in 397 patients by esophagogram, endoscopy, manometry, and pH monitoring. There were 305 patients (77%) with achalasia, 49 patients (12%) with DES, 41 patients (10%) with NE, and 2 patients (1%) with HTN-LES. Two hundred eight patients (52%) underwent a myotomy by either a thoracoscopic or a laparoscopic approach. RESULTS: Ninety-nine patients (25%) had a diagnosis of gastroesophageal reflux disease at the time of referral and had been treated with acid-suppressing medications. In achalasia and DES, a thoracoscopic or laparoscopic myotomy relieved dysphagia and chest pain in more than 80% of the patients. In contrast, in NE the results were less predictable, and the operation most often failed to relieve symptoms. CONCLUSIONS: These results show that (1) symptoms were unreliable in distinguishing gastroesophageal reflux disease from PEMDs; (2) esophageal function tests were essential to diagnose PEMD and to define its type; (3) the laparoscopic approach was better than the thoracoscopic approach; (4) a laparoscopic Heller myotomy is the treatment of choice for achalasia, DES, and HTN-LES; and (5) a predictably good treatment for NE is still elusive, and the results of surgery were disappointing.

Early predictors of severe lower gastrointestinal bleeding and adverse outcomes: a prospective study.

BACKGROUND & AIMS: Unlike in upper tract bleeding, prognostic factors for ongoing or recurrent bleeding from the lower gastrointestinal tract have not been well-defined. The aim of this study was to identify risk factors for severe lower gastrointestinal bleeding and for significant adverse outcomes. METHODS: All patients seeking attention at a university emergency department for gastrointestinal bleeding were prospectively identified during a 3-year period. Ninety-four of 448 (21%) admitted patients had lower gastrointestinal bleeding. Clinical predictors available in the first hour of evaluation were recorded. The primary outcome, severe lower gastrointestinal bleeding, was defined as gross blood per rectum after leaving the emergency department associated with either abnormal vital signs (systolic blood pressure < 100 mm Hg or heart rate > 100/min) or more than a 2-unit blood transfusion during the hospitalization. Significant adverse outcomes, including death, were tabulated. RESULTS: Thirty-seven patients (39%) had severe lower gastrointestinal bleeding. Independent risk factors for severe lower gastrointestinal bleeding were initial hematocrit 100/min) 1 hour after initial medical evaluation (OR, 4.3; 95% CI, 1.4-12.5); and gross blood on initial rectal examination (OR, 3.9; 95% CI, 1.2-13.2). Nineteen patients (20%) experienced a significant adverse outcome, including 3 deaths. The main independent predictor of adverse outcomes was severe lower gastrointestinal bleeding (OR, 5.3; 95% CI, 1.7-16.5). CONCLUSIONS: Risk factors are available in the first hour of evaluation in the emergency department to identify patients at risk for severe lower gastrointestinal bleeding. Severe lower gastrointestinal bleeding is a significant risk factor for global adverse outcomes.

Endoscopic and radiologic management of pancreatic and biliary tract diseases.

Therapeutic biliary and pancreatic endoscopy has evolved over the last 30 years to a level where it represents the primarily mode of therapy for many frequently encountered diseases of the bile ducts liver and pancreas. The complication rates are expected to be low and the expectations of our colleagues are high. The endoscopist is expected to understand the origin and natural history of these diseases and the consequences of the various management options. The training of the endoscopist has taken on a very formal character, as has the emphasis on competency and quality improvement. The appearance of minimally invasive surgery and advanced imaging does not represent a threat to the biliary endoscopist but rather is complimentary and assists us so that unnecessary potentially morbid procedures are not done unnecessarily. The appearance of a new specialty: the minimally invasive biliary interventionalist, who would receive training by gastroenterologists, interventional radiologist and biliary-pancreatic surgeons, is the logical next step!