Wilkie's syndrome in an adolescent female patient

One of the rare cases of the upper gastrointestinal tract obstruction is superior mesenteric artery syndrome (SMAS) but potentially life-threatening if not recognized early. It is due to loss of fat pad between the aorta and SMA which will lead to a compression of the third portion of the duodenum. It has a different angle which will lead to different presentations as well as severity. We are reporting the 15 year-old (y/o) female who is medically free, presented to the emergency department (ED) complaining of severe colicky epigastric abdominal pain for the last 5 days in the epigastric area. The diagnosis of SMAS was made after clinical and radiological investigation. After proper supportive resuscitative measures, definitive management of the surgery was done by laparoscopic approach (duodenojejunostomy). The diagnosis of SMA syndrome is considered challenging due to many presentations and might be confused with other clinical conditions. Unless early diagnosed and treated, the outcome might be catastrophic. Medical treatment is attempted first in many cases depending on the severity and presentation but if failed, surgery will be the best option.

Síndrome de Wilkie / Wilkie Syndrome

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.

ERCP for diagnosis and management of biliary cast syndrome after liver transplantation / 中华消化内镜杂志

Objective To evaluate endoscopic retrograde cholangiopancreatography (ERCP) for diagnosis and management of biliary cast syndrome after orthotropic liver transplantation. Methods A total of 71 consecutive patients with abnormal liver function and MRCP findings after liver transplantation underwent ERCP for diagnosis and management. Their data were retrospectively reviewed. Results A total of 188 sessions of ERCP were carried out on the 71 patients, most of whom were found to have stenosis of anastomotic stoma and/or bile duct. Bile sludge was found and removed in all patients diagnosed within 3 months after liver transplantation, while pigmentoid stones were found and removed in patients diagnosed within 3-6 months and biliary casts in patients diagnosed at more than 6 months. Each patient underwent 2.6 sessions averagely. Biliary casts were formed at an average time of 22. 7 ± 15.6 months after transplantation. PostERCP complications included 2 cases of pancreatitis and 3 cholangitis, with an occurrence rate of 2. 6%(5/188), which were all controlled with conservative treatment. The follow-up data was available in 56 patients showing improvement in liver function after ERCP, among who 42 met the endoscopic criteria of cure,1 0 received second liver transplantation because of progressive sclerosing cholangitis and 4 died from diseases other than liver transplantation. Conclusion Therapeutic ERCP for the biliary cast syndrome after liver transplantation is feasible, safe and effective, and can be performed repeatedly with good short-term effect.

A Case of Biliary Cast Syndrome in a Non-liver Transplant Patient / 대한소화기내시경학회지

Biliary casts are uncommon but are typically seen in post-liver transplant patients, whereas very few cases have been reported in non-liver transplant patients. A 65-year-old man, who had not undergone a liver transplantation or other hepatobiliary surgery, presented with jaundice and fever. Radiological imaging studies showed diffuse thickening and enhancement of the intrahepatic duct with mild ductal dilatation and multiple linear filling defects. A percutaneous transhepatic cholangioscopy revealed severe bile duct inflammation and multiple biliary casts, which were removed with multiple percutaneous choledocoscopic procedures. The pathogenesis of the biliary casts in this patient was uncertain. However, we presumed that biliary tract infection with subsequent extensive cholangitis was an important predisposing factor in the cast formation. We report a case of biliary cast syndrome with no preexisting morbidity that was managed favorably with endoscopic removal.

A Case of Biliary Cast Syndrome in a Non-liver Transplatation Patients / 대한소화기내시경학회지

The development of total biliary casts is very unusual, and especially in patients who have not undergone liver transplantation. There are only a few reports of total biliary casts in non-liver transplantation patients who have antiphospholipid antibody syndrome, B-cell non-Hodgkin's lymphoma, cholecystectomy or allogenic hematopoietic stem cell transplantation. Here we present the case of a previously well 77-year-old man who developed a total biliary casts without any risk factors and there was no obvious liver insult. The casts were managed endoscopically.

A Case of Biliary Cast Syndrome with a Biliary Stricture and Suppurative Cholangitis after Liver Transplantation / 대한소화기내시경학회지

Biliary complications after liver transplantation occur in 13~35% of patients. Biliary cast syndrome, cast formation of biliary sludge along the bile duct, can develop in 4~18% of liver transplant recipients, although the incidence rate is significantly decreasing due to the improvement of graft harvesting and preservation. It is very important that early diagnosis and effective management of biliary cast syndrome be performed when there is a bile duct stricture or dilatation associated with jaundice and cholangitis in the recipient after liver transplantation, due to the possibility of retransplantation and death of the patient from graft loss. We report a case of a biliary cast formed with suppurative cholangitis and extracted incidentally with a plastic biliary stent during an endoscopic procedure after cadaveric liver transplantation, in which the ERCP findings revealed a stricture at the anastomosis site of the common bile duct and cholangitis.

A Case of Biliary Cast Syndrome after Cadaveric Liver Transplantation / 대한소화기학회지

We experienced one fatal case of biliary cast syndrome after cadaveric liver transplantation involving both intrahepatic ducts. A 58-year-old man underwent cadaveric liver transplantation because of hepatitis B virus related liver cirrhosis and concomitant hepatocellular carcinoma. Five weeks after the liver transplantation, postoperative course was complicated by development of acute cholangitis. Subsequent endoscopic retrograde cholangiography revealed diffuse intrahepatic bile duct strictures without filling defects. Percutaneous liver biopsy, which was done to exclude rejection, revealed biliary cast. Successful endoscopic removal was precluded due to its diffuse involvement. Because of the deterioration of patient's condition by refractory biliary obstruction and cholangitis, retransplantation from cadaveric donor was performed. Debridement of the biliary tree after graft removal yielded a near-complete cast of the intrahepatic ductal system. Biliary cast syndrome should be suspected when jaundice or cholangitis is associated with dilated ducts on abdominal imaging studies in cadaveric liver transplantation recipients. Initial therapeutic options include removal of biliary cast after endoscopic or percutaneous cholangiography. Although endoscopic retrieval of biliary cast by endoscopic retrograde cholangiopancreatography could be employed as a first-line management, other modalities such as endoscopic nasobiliary drainage, percutaneous transhepatic drainage, or retransplantation should be considered when complete removal is not feasible and the condition of the recipient deteriorates.

Biliary Cast Successfully Removed by Percutaneous Transhepatic Cholangioscopy, and This Developed in a Patient Who Received Orthotopic Liver Transplantation / 대한소화기내시경학회지

"Biliary cast syndrome" describes a cast formed from retained lithogenic material, and this cast is morphologically confined to the bile duct; this develops in 4~18% of liver transplant recipients. The pathogenesis of cast formation is not clearly understood. The proposing etiological factors for biliary cast syndrome include acute cellular rejection, a prolonged cold ischemic time, use of postoperative biliary drainage tubes and biliary infection. These casts are more likely to develop in the setting of hepatic ischemia and biliary stricture. Endoscopic and percutaneous cast extraction might achieve favorable results and this should be attempted before surgical therapy. We report here on a case of biliary cast syndrome that was secondary to orthotopic liver transplantation; this was successfully treated via percutaneous choledochoscopic removal. We also include a review of the literature.