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We present a patient with COVID-19 pneumonia with severe multi-organ failure who developed a liver infarction in the setting of acute illness and following recovery, had persistent cholestatic liver injury. On further evaluation, he was found to have a severe destruction of the intrahepatic bile ducts and biliary cast syndrome and ultimately, was felt to have severe ischemic cholangiopathy in the setting of recent COVID-19 infection. Because of the extent of destruction and persistent cholestasis, he is at risk of secondary biliary cirrhosis and is therefore undergoing a liver transplant evaluation. This case highlights a rare entity of liver infarction and severe cholangiopathy with biliary cast syndrome in the setting of COVID-19 infection. It is the first case of this reported to date, and its recognition in the presence of the COVID-19 pandemic is important. Keywords: Biliary cast, Cirrhosis, Cholangiopathy , COVID-19, Liver infarction.

Background and Aim: The aim of this study is to describe the cholangiographic features and endoscopic management of biliary cast syndrome (BCS), a rare specific ischemic cholangiopathy following liver transplantation. Methods: Patients with biliary complications were identified from prospectively collected database records of patients who underwent liver transplantation at the Erasme Hospital from January 2005 to December 2014. After excluding patients with hepatico-jejunostomy or no suspicion of stricture, cholangiograms obtained during endoscopic retrograde cholangiopancreatography (ERCP) and magnetic resonance imaging were systematically reviewed. Biliary complications were categorized as anastomotic (AS) and non-AS strictures, and patients with BCS were identified. Clinical, radiological, and endoscopic data were reviewed. Results: Out of 311 liver transplantations, 14 cases were identified with BCS (4.5%) and treated with ERCP. Intraductal hyperintense signal on T1-weighted magnetic resonance and a “duct-in-a-duct” image were the most frequent features of BCS on magnetic resonance imaging. On initial ERCP, 57% of patients had no stricture. Complete cast extraction was achieved in 12/14, and one of these had cast recurrence. On follow-up, 85% of the patients developed biliary strictures that were treated with multiple plastic stents reaching 60% complete stricture resolution, but 40% of them had recurrence. After a median follow-up of 58 months, BCS patients had lower overall and graft survival (42.9% and 42.9%) compared with non-AS (68.8% and 56.3%) and AS (83.3% and 80.6%), respectively. Conclusions: Particular magnetic resonance-cholangiographic and ERCP-cholangiographic features of BCS have been identified. Outcomes for BCS are characterized by high complete cast extraction rates, high incidence of secondary strictures, and poorer prognosis., SCOPUS: ar.j, info:eu-repo/semantics/published

El síndrome de Wilkie o síndrome de la arteria mesentérica superior, es una entidad patológica poco frecuente. Descrito inicialmente por Von Rokitansky en 1861 y apoyado luego por Willet, en 1868. Se caracteriza por una compresión extrínseca de la tercera porción del duodeno por la arteria mesentérica superior y la aorta, ante la reducción del ángulo aortomesentérico. Se presenta el caso de un paciente masculino de 36 años de edad, sin patologías médicas previas, con un cuadro crónico de pérdida de peso, vómitos y sensación de plenitud gástrica. Consultó a valoración en el Servicio de Cirugía, tras realizarse gastroscopía que revelaba una estrechez extrínseca distal al píloro gástrico. Se efectuaron estudios complementarios radiográficos, los cuales revelaron la disminución del ángulo entre la arteria aorta y la arteria mesentérica superior. Posteriormente se realizó un abordaje laparoscópico con anastomosis duodeno yeyunal latero-lateral, con corrección del cuadro patológico y evolución favorable del paciente. Superior mesenteric artery (SMA) syndrome (Wilkie syndrome) is an uncommon condition. It was first described in 1861 by Von Rokitansky and then reviewed by Willet in 1868. The syndrome develops when the third part of the duodenum is obstructed by the angle formed between the Aortic Artery and the Superior Mesenteric Artery. A 36-year-old male, with no previous medical conditions, with chronic weight loss, vomiting and epigastric pain. The patient was assessed by the Surgery Department after a gastric endoscopy revealed an extrinsic obstruction of the duodenum. After several radiological studies, a reduction of the Aortic and Superior Mesenteric Artery angle was found. A laparoscopic duodenum-yeyunum anastomosis was performed, the medical condition was solved and the patient evolved positively.

Biliary Cast Syndrome A 62-year-old woman presented with a 2-month history of jaundice 3 months after undergoing orthotopic liver transplantation. Imaging revealed filling defects in the bile ducts...

We present a case report of a young man who developed cast syndrome in the course of endoscopic treatment for post liver transplantation anastomotic stricture, with emphasis on cholangioscopic aspects.

Objective: The aim of this study was to evaluate the most important factors which can cause “tight cast syndrome’’ (TCS) in pediatric patients with distal radius fractures. Methods: Patients, who were at or under 15 years old and treated conservatively with an diagnosis of distal radius fracture between August 2015 and August 2017 were included in to the study. Fifty four patients, who had been found to experience TCS were accepted as group 1 and sixty-two patients without TCS as group 2. Cast index, pre-/post reduction translation, pre-/post reduction angulation, localization and displacement of the fracture, need for re-manipulation, and presence of associated distal ulna fracture were evaluated for both groups. Statistical analysis was performed to evaluate cut off value for cast index values for both TCS and loss of reduction and logistic regression analysis of the other possible factors. Results: Pre-/post-reduction translation (over 50% and 10%, respectively) (odds ratios:0.167 and 0.524, respectively), a cast index value below than 0.775 (odds ratio:3.080), displaced type fracture (odds ratio:8.028), presence of re-manipulation (odds ratio:0.131) and associated distal ulna fracture (odds ratio:2.029) were found to be statistically significantly important for the occurrence of TCS. The most important factors were decreased cast index value and presence of initially displaced type fracture. Loss of reduction (LOR) risk was found to be increased in patients with a cast index value of greater than 0.875. Conclusion: One should be very careful when following a pediatric patient who have a displaced distal radius fracture which has initial/post reduction translation in AP plane, which is associated with distal ulna fracture, which required re-manipulation and most importantly which cast index is under than 0.775 in terms of occurrence of TCS. We recommend obtaining a cast index value between 0.775 and 0.875 to prevent both TCS and LOR. Level of evidence: Level III, Therapeutic study. Keywords: Cast index, Tight cast, Distal radius fracture, Pediatric, Loss of reduction

Biliary cast syndrome is an unusual complication in patients who have previously undergone liver transplantation. It occurs in approximately 5%–18% of such patients. Rare cases of biliary cast syndrome in patients without liver transplants have also been reported. The pathogenesis of biliary cast has not been clearly identified, although etiologic factors including post-transplant bile duct damage, ischemia, biliary infection, and presence of a post-operative biliary drainage tube have been proposed. Here we present a case of biliary cast that developed in a 49-year-old male who underwent a non-liver surgery after endoscopic and percutaneous management of common bile duct stone.

Biliary cast syndrome (BCS) is an unusual complication of liver transplantation (LT). The pathophysiology is not known, and it is thought to develop because of mucosal damage in the bile duct related to obstruction, ischemia, or bacterial infection. It occurs in 2.5–18% of LT patients and is associated with increased graft failure, need for retransplantation, and mortality. Here we report on a case of BCS of late appearance after LT who was successfully treated by endoscopic means.

Biliary cast syndrome (BCS) consists of mass-like hardened collections of inspissated bile and sloughed biliary mucosa filling the intrahepatic and/or extrahepatic bile ducts, which show an alternating pattern of dilatation and stricture. It is a rare but serious complication of liver transplantation frequently necessitating repeated percutaneous, endoscopic or surgical stent placements, and cast retrieval. Although not typically considered as the modality of choice for BCS compared with magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography, ultrasound can be quite useful for this diagnosis. This article reviews the sonographic appearance of BCS in postliver transplant patients and correlates with other imaging modalities including computed tomography, MRCP, and endoscopic retrograde cholangiopancreatography. Also reviewed are other intrahepatic pathologies that mimic the appearance of BCS on ultrasound.

Background: ‛Sclerosing cholangitis in critically ill patients' (SC-CIP) is a cholestatic liver disease of unknown etiology and represents the most prevalent form of secondary sclerosing cholangitis. Methods: This overview is based on a systematic review of the literature searching for ‘secondary sclerosing cholangitis', ‘SC-CIP', ‘cast syndrome', and ‘ischemic cholangitis' in the database PubMed. Results: SC-CIP can develop in patients with sepsis and acute respiratory distress syndrome during a long-term intensive care unit (ICU) treatment. It is a rare cholestatic liver disease with a rapid progression to liver cirrhosis and hepatic failure. SC-CIP is initiated by an ischemic injury to the biliary tree with subsequent stenoses of biliary ducts, biliary casts, and infections, often with multi-resistant bacteria. Mechanical ventilation with high positive end-expiratory pressure, prone positioning, and a higher volume of intraperitoneal fat have been proposed as risk factors for developing SC-CIP. Patients with SC-CIP have a poor prognosis, with liver transplantation (LT) being the only curative treatment option. Conclusion: In patients with sepsis, long-term ICU therapy and ongoing cholestasis SC-CIP must be excluded by endoscopic retrograde cholangiopancreatography. Due to the poor prognosis, the option of LT should be evaluated in all patients with SC-CIP.

It is also known as Wilkie's syndrome, cast syndrome, mesenteric root syndrome, chronic duodenal ileus and intermittent arterio-mesenteric occlusion. It is distinct from Nutcracker syndrome, which is the entrapment of the left renal vein between the AA and the SMA.

Our study shows that adding nonenhanced T1-weighted images to MR cholangiopancreatography significantly improves the diagnostic accuracy of MR imaging in the detection of biliary cast syndrome in liver transplant recipients.

Background The biliary cast syndrome (BCS) is a frequent problem following liver transplantation. The pathogenesis of this complication is not well understood. Previous research has demonstrated that the soluble form of MICA (sMICA) is significantly higher in patients with chronic liver disease and hepatocellular carcinoma (HCC) than in healthy volunteers. The aim of this study is to investigate the possible involvement of sMICA in the formation of BCS after liver transplantation. Methods Serum soluble MICA was retrospectively evaluated in pre- and post-transplant sera from 133 consecutive primary liver transplant patients and in sera from 88 healthy volunteers using sandwich ELISA. Normal distribution of serum sMICA was described by the data obtained from healthy population and sMICA concentration that was greater than the upper bound 95% normal range was considered as high levels of sMICA. Patient records were reviewed to identify patients who developed BCS. Results The results demonstrated that 37.6% of patients with end-stage liver diseases had significantly higher pre-transplant serum sMICA than in healthy population. 34.4% of recipients with post-transplant high levels of sMICA developed BCS. In contrast, 17.3% of patients with post-transplant normal levels of sMICA developed BCS. The risk of BCS development is significantly associated with the presence of post-transplant high levels of sMICA ( P = 0.0365). Further analysis disclosed that patients with decreased post-transplant sMICA following liver transplantation had a lower incidence rate of BCS than those with remained high levels of sMICA after transplantation (10.5% vs. 38.7%, P = 0.0302). Furthermore, log-rank test showed that BCS occurrence was significantly associated with dynamic changes of sMICA among different groups ( P = 0.0188). Conclusions Biliary cast syndrome is more likely to develop in recipients who have post-transplant high levels of sMICA. The data suggested that sMICA might have some immunologic effect on BCS development following liver transplant. Monitoring of serum sMICA could have a prognostic value in assessment of patients with liver transplantation.

Biliary cast syndrome (BCS), the presence of biliary casts and debris causing biliary obstruction, occurs in 4%-18% of orthotopic liver transplant (OLT) recipients. Potential consequences include cholangitis and graft damage or loss. Limited data exist regarding the etiology and outcomes of BCS. The purpose of this study was to evaluate donor and recipient risk factors and determine the impact of BCS. A retrospective review of 355 OLT cases identified 9 BCS patients (2.5%) diagnosed by cholangiography. Twenty-six matched controls were also identified. The warm ischemic time was significantly longer in BCS patients. Other recipient and donor preoperative and intraoperative characteristics, including the donor risk index, revealed no significant differences. Overall patient survival showed a trend toward worse outcomes at 6, 12, and 18 months and end of follow-up in the BCS group. Overall graft survival was also worse in the BCS group at all time periods, with statistical significance demonstrated at 18 months and end of follow-up. The number of therapeutic biliary procedures and hospital readmissions was significantly higher in the BCS group. Twenty-two percent of the BCS patients required repeat OLT versus none of the control patients. In conclusion, BCS is an uncommon complication of OLT. Except for a longer warm ischemic time, recipient and donor factors did not predict the occurrence of BCS. BCS patients showed a significantly worse graft survival, as well as a trend toward worse patient survival. Given the negative impact of BCS on liver transplant outcomes, further studies appear justified.

Biliary cast syndrome is an uncommon condition, almost always associated with liver transplantation. We describe 2 patients with biliary cast syndrome in a nonliver transplant setting. The casts were managed endoscopically.

ResumenAntecedentesEl síndrome de Wilkie o síndrome de la arteria mesentérica superior es una causa inusual de oclusión intestinal alta, se caracteriza por la compresión del duodeno en su tercera porción, es secundaria a un estrechamiento del espacio entre la arteria mesentérica superior y la aorta. El cuadro clínico suele estar caracterizado por síntomas de oclusión intestinal alta, la exploración física y los hallazgos de laboratorio suelen ser poco específicos. Diversos estudios de imagen son útiles para el diagnóstico. El tratamiento puede ir desde conservador, hasta la cirugía, que depende de cada caso.Caso clínicoVarón de 19 años, que cursó con dolor abdominal súbito, intenso, náuseas, vómitos y diarrea; motivo por el cual acudió al servicio de urgencias, donde a la exploración destacaron: rigidez abdominal con hiperestesia e hiperbaralgesia, y los estudios de imagen evidenciaron una disminución del ángulo de la arteria mesentérica superior y de la distancia aortomesentérica; además de disminución del calibre de la tercera porción del duodeno, hallazgos característicos del síndrome de Wilkie. Se dio tratamiento conservador, siendo dado de alta hospitalaria sin complicaciones.ConclusionesEl síndrome de Wilkie continúa siendo una entidad poco conocida por el médico general, el que poco se diagnostique o se piense en este síndrome, puede ocasionar complicaciones serias por lo que se requiere que el médico guarde un alto índice de sospecha diagnóstica ante esta patología. El tratamiento oportuno confiere un buen pronóstico la mayoría de las veces.AbstractBackgroundWilkie syndrome, also referred as superior mesenteric artery syndrome, is an unusual cause of a proximal small bowel obstruction. It is characterised by the compression of the duodenum in its third portion due to a narrowing of the space between the superior mesenteric artery and the aorta. Its presentation symptoms are consistent and include the obstruction of the proximal small bowel. However, the physical and laboratory findings are non-specific. Nevertheless, many imaging methods are useful for its diagnosis. The management of this condition varies between observation and surgery, depending on each particular case.Clinical caseThe case is presented of a 19 year-old male who began with acute, intense abdominal pain, nausea, vomiting, and diarrhoea. On examination, he had abdominal wall rigidity and hyperesthesia. Imaging studies were requested, revealing a decreased superior mesenteric artery angle, a shortening of the aortic mesenteric distance, and a decrease in the calibre of the third duodenal portion, all findings concomitant with Wilkie syndrome. Conservative treatment was applied and the patient was discharged without complications.ConclusionsWilkie syndrome continues to be an unknown condition to the general practitioner, and the underdiagnosis of this condition may put a patient at risk of serious complications. A high index of suspicion is required to reach a diagnosis. Early treatment should give a good outcome most of the time.

Despite numerous advancements in the management of patients who have undergone cadaveric liver transplantation, biliary complications continue to challenge clinicians. Biliary leaks in the early postoperative period and strictures in the late postoperative period represent the two major posttransplant biliary complications. Intrahepatic and hilar strictures are particularly difficult to manage and frequently require retransplantation, which should not be delayed. Choledocholithiasis and the biliary cast syndrome are frequently associated with underlying biliary strictures, and endoscopic attempts at removal should precede surgical interventions. Sphincter of Oddi dysfunction is increasingly recognized despite unclear pathophysiology. Hemobilia is most commonly iatrogenic and requires a high suspicion and prompt intervention. Although the number of diagnostic and therapeutic options have increased, there is no consensus as to which is superior. In recent years there has been a trend toward nonsurgical interventions, in particular endoscopic approaches.

Wilke’s syndrome is a rare cause of partial or complete duodenal obstruction due to exterior compression of the third part of the duodenum by the superior mesenteric artery. It is a true syndrome with characteristic clinical picture which should be included in the differential diagnosis of chronic duodenal ileus. It is initially treated conservatively and then operatively in case of failure of the conservative treatment or if the patient is not willing to follow the medical treatment. We present a case report of Wilke’s syndrome with a literature review on this pathologic entity.

Context : Superior mesenteric artery syndrome is a life- threatening upper gastrointestinal disorder due to compression of duodenum as it poses a difficult diagnostic dilemma. Third part of duodenum is in fixed compartment bounded anteriorly by the root of mesentery and superior mesentery artery and posteriorly by the aorta and lumbar spine. On barium contrast study and abdominal computerized tomography (CT) showed the dilatation of second part of duodenum and compression of the third part of duodenum between aorta and superior mesentery artery. Case Report : A 22 year young asthenic man admitted with the complaint of recurrent abdominal pain, epigastric fullness, and vomiting and weight loss. Abdominal examination revealed epigastric fullness and hyper peristaltic bowel sounds. Upper gastrointestinal barium study showed a dilated stomach with dilated second part of the duodenum and cut off at the third part of duodenum with no intrinsic mucosal abnormalities. There was no relief of obstruction in the left lateral decubitus or prone position. Conservative treatment was tried for one month but failed. Intra-operative findings confirmed the extrinsic obstruction of third part of duodenum with distension of 2 nd part. A retrocolic duodenojejunostomy, side to side anastomosis done. In post-operative follow up, patient was symptom free. Conclusion : Superior mesentery artery syndrome is a life threatening disease. It should be treated as soon as possible. Conservative trial can be given but Surgery is the treatment of the choice.