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INTRODUCTION: Choledochal cysts (CC) are rare congenital biliary dilatations that are capable of presenting with a gamut of clinical findings. Perforation, a rather rare presentation, can account for 1.8-7% of cases. In an infant with non-specific abdominal complaints and a vaguely palpable upper abdominal mass, a perforated CC may be overlooked, leading to a delayed or misdiagnosis and a detrimental outcome. CASE PRESENTATION: We describe a 10-month-old girl who presented to the office with upper abdominal pain and associated fullness for two weeks. An abdominal ultrasonogram revealed perihepatic collections and an evident dilatation of the common bile duct. The bile aspirated from the collections prompted pigtail drainage as an emergency. Magnetic resonance cholangiopancreatography later revealed a spontaneously perforated CC with residual bilioma. Elective resection after six weeks, followed by bilio-enteric reconstruction, resulted in an uneventful recovery. CLINICAL DISCUSSION: Biliary peritonitis and localized biliomas are rare forms among the wide range of findings that can accompany CC. Satisfactory outcomes can be achieved with a two-stage procedure that entails biliary drainage with sepsis control, followed by resection of the CC and bilio-enteric reconstruction a few weeks later. CONCLUSION: It is important to be aware of the spectrum of findings that CC may present. We discussed our experience successfully treating a perforated type IVa CC.

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Bilomas are collections of bile outside the biliary tree. The most frequent etiologies are iatrogenic and trauma. Cases of spontaneous or atraumatic bilomas are rare. Management of bilomas depends on the size and location and may include monitoring only; if the size is < 4 cm, there may be percutaneous or endoscopic intervention. The use of antibiotics depends on the clinical status of the patient. We describe the case of a man who presented with a spontaneous biloma eight years after laparoscopic cholecystectomy and, in addition to signs of choledocholithiasis, a stricture of the common bile duct. In patients with symptoms of biliary pathology, the diagnosis of biloma should be considered even without a history of trauma or recent surgery to initiate appropriate treatment early. Many cases are asymptomatic and resolve spontaneously but occasionally require percutaneous or endoscopic management.

Los biliomas son colecciones de bilis fuera del árbol biliar. Las etiologías más frecuentes son la iatrogenia y el trauma. Los casos de biliomas espontáneos o atraumáticos son poco frecuentes. El manejo de los biliomas depende del tamaño y la localización y puede incluir vigilancia solamente, si el tamaño es < 4 cm, puede haber intervención percutánea o endoscópica. El uso de antibióticos depende del estado clínico del paciente. Presentamos el caso de un hombre que presentó un bilioma espontáneo 8 años después de una colecistectomía laparoscópica que, además de signos de coledocolitiasis, presentaba una estenosis del conducto biliar común. En los pacientes con clínica de patología biliar debe considerarse el diagnóstico de bilioma aun en los casos que no presenten antecedente de trauma o cirugía reciente con el fin de iniciar el tratamiento adecuado tempranamente. Muchos casos son asintomáticos y se resuelven espontáneamente, pero en ocasiones requieren manejo percutáneo o endoscópico.

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BACKGROUND: A variety of transhepatic percutaneous biliary procedures are appropriate for the treatment of pathologies of the biliary system. OBJECTIVES: The aim of this article is to describe best practices for performing percutaneous transhepatic cholangiography with placement of a biliary drain (PTCD), percutaneous transhepatic removal of bile duct stones, percutaneous stenting of the bile ducts, and percutaneous treatment of postoperative bilioma. MATERIALS AND METHODS: The authors reviewed existing literature on relevant current recommendations and presented them based on their own facility's approach. RESULTS: Biliary interventions are mostly aimed at treating some form of cholestasis of benign or malignant etiology. The technical success rate is up to 90%. CONCLUSION: Percutaneous biliary interventions are safe and effective procedures in the treatment of pathologies of the biliary system, preferably used when endoscopic access is not possible due to anatomical conditions.

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INTRODUCTION: Spontaneous perforation of the common bile duct is rare. The cause is usually unknown, although it is sometimes related to the malformation of the bile duct. CLINICAL CASE: Female of 1 year, with abdominal distention, hyporexia and fever, tomography with ascites, surgical findings included retroperitoneal bilioma, peritonitis and perforation of common bile duct. Block resection and primary hepato-duodenal anastomosis were performed. DISCUSSION: Spontaneous perforation of the common bile duct tend to evolve insidiously and delay in diagnosis is frequent. Surgical management is decisive, and there are different techniques described. CONCLUSION: A high index of suspicion is important, treatment must be individualized according to each patient.

INTRODUCCIÓN: La perforación espontánea del colédoco es rara. La causa generalmente se desconoce, aunque en ocasiones guarda relación con una malformación de la vía biliar. CASO CLÍNICO: Niña de 1 año, con distensión abdominal, hiporexia y fiebre, tomografía con ascitis, se interviene y se encuentra bilioma retroperitoneal, peritonitis y perforación de colédoco. Se realiza resección en bloque y anastomosis hepatoduodenal primaria. DISCUSIÓN: La perforación espontánea del colédoco tiende a evolucionar insidiosamente y el retraso en el diagnóstico es frecuente. El manejo quirúrgico es resolutivo; existen distintas técnicas descritas. CONCLUSIÓN: Es importante un alto índice de sospecha. El tratamiento se debe individualizar en cada paciente.

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BACKGROUND: Bilomas are defined collections of bile fluids mainly caused by iatrogenic injuries of the bile duct system. Owing to the infrequency of this disease, studies addressing bilomas are rare. METHODS: By using an endoscopic database, this retrospective study identified 32 patients with bilomas treated between 2004 to 2015, in order to analyse aetiology, clinical presentation, spectrum of pathogens, and resolution rate of bilomas. RESULTS: 65.6% of the study population (21/32) developed bilomas after surgery and 21.9% (7/32) after endoscopic retrograde cholangiography (ERC). Icterus, fever, and abdominal pain were the leading symptoms. 93.9% (46/49) of microbiological bile cultures revealed a positive microbiology. The predominant microorganisms were the group of Enterobacteriaceae (43.0%, 52/121), followed by Enterococcus spp. (32.2%, 39/121), and Candida spp. (9.1%, 11/121). Multiresistant bacteria like Enterobacteriaceae were isolated from one quarter of all patients. Single or multimodal treatment resulted in an overall complication rate of 4.8% (9/188). Clinical follow-up analysis showed a complete resolution rate of 78.3% for interventional therapy and 80% in the non-interventional group. CONCLUSIONS: Pathogen spectrum of bilomas mainly comprises the group of Enterobacteriacae and Enterococcus spp., with a high proportion of multiresistant bacteria. Different interventional approaches are available for biloma drainage, which seem to be safe and effective for most patients. TRIAL REGISTRATION: German Clinical Trials Register DRKS00015208 , retrospectively registered.

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BACKGROUND: Caval vein thrombosis after hepatectomy is rare, although it increases mortality and morbidity. The evolution of this thrombosis into a septic thrombophlebitis responsible for persistent septicaemia after a hepatectomy has not been reported to date in the literature. We here report the management of a 54-year-old woman operated for a peripheral cholangiocarcinoma who developed a suppurated thrombophlebitis of the vena cava following a hepatectomy. CASE SUMMARY: This patient was operated by left lobectomy extended to segment V with bile duct resection and Roux-en-Y hepaticojejunostomy. After the surgery, she developed Streptococcus anginosus, Escherichia coli, and Enterococcus faecium bacteraemias, as well as Candida albicans fungemia. A computed tomography scan revealed a bilioma which was percutaneously drained. Despite adequate antibiotic therapy, the patient's condition remained septic. A diagnosis of septic thrombophlebitis of the vena cava was made on post-operative day 25. The patient was then operated again for a surgical thrombectomy and complete caval reconstruction with a parietal peritoneum tube graft. Use of the peritoneum as a vascular graft is an inexpensive technique, it is readily and rapidly available, and it allows caval replacement in a septic area. Septic thrombophlebitis of the vena cava after hepatectomy has not been described previously and it warrants being added to the spectrum of potential complications of this procedure. CONCLUSION: Septic thrombophlebitis of the vena cava was successfully treated with antibiotic and anticoagulation treatments, prompt surgical thrombectomy and caval reconstruction.

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Objetivo. Presentar el segundo caso descrito en la bibliografía de bilioescroto secundario a bilioma retroperitoneal espontáneo y mostrar a su vez de manera escalonada el tratamiento que se le suministró hasta la resolución completa del cuadro. Caso clínico: Varón de 69 años que debutó con dolor escrotal derecho en relación con bilioescroto secundario a bilioma retroperitoneal espontáneo. Discusión: El bilioma retroperitoneal es un hecho poco habitual; generalmente se debe a complicaciones quirúrgicas o de procedimientos invasivos (intervencionismo radiológico, CPRE y esfinterotomía endoscópica), aunque puede ser consecuencia de roturas espontáneas de la vesícula o la vía biliar principal. La bilis en el retroperitoneo puede discurrir hasta el escroto, originando la rara entidad conocida como bilioescroto. Generalmente se presenta simulando una hernia inguinal incarcerada. No existe un manejo estandarizado de esta patología ya que está escasamente descrita.

Objective: To present the second case described in the literature of biliscrotum secondary to spontaneous retroperitoneal biloma and show a sequential treatment that we provided until complete resolution. Clinical case: We report the case of a man 69 years old, who debuted with right scrotal pain related to biliscrotum secondary to spontaneous retroperitoneal biloma. Discussion: The retroperitoneal biloma is a rare event, usually due to surgical or invasive procedures complications (radiological interventionism, ERCP and endoscopic sphincterotomy), although it may be the result of spontaneous rupture of the gallbladder or bile duct. Bile in the retroperitoneum may extend into the scrotum, causing the rare condition known as biliscrotum. Usually it occurs simulating an incarcerated inguinal hernia. Does not exist a standardized management of this condition because is poorly described.

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INTRODUCTION: Spontaneous rupture of the biliary duct, a rare condition in adults, is difficult to diagnose preoperatively and presents with acute abdominal symptoms. The treatment of this rare condition should be based on the individual's clinical status. We present peripheric biliary duct rupture (segment three) treated with external segment III drainage and postoperative endoscopic removal of the stones. PRESENTATION OF CASE: An 82-year-old male patient presented with abdominal pain and fever. An ultrasound (US) revealed a solid gall stone lesion, 3cm in diameter, in liver segments three and four with additional intra-abdominal fluid accumulation without coexisting free air. A diagnostic laparotomy was then performed because the patient had signs of peritonitis. Exploration revealed a biliary leakage from the posterior surface of segment three. An external biliary drainage catheter was inserted to the perforated segment III duct via a 6 French (6F) feeding catheter. He was discharged after 10 days and his intracholedocal stent was removed postoperative after three months. The patient continues to be monitored. DISCUSSION: Spontaneous rupture of the intrahepatic biliary duct is a rare condition. Although occurrence is frequently reported as spontaneous, the majority of cases are related to choledocholithiasis. The role of surgical treatment in cases of spontaneous bile duct rupture is unclear. When biliary peritonitis is present, drainage of contaminated biliary fluid, T-tube drainage, closure of the biliary duct, as well as primary disease conditions, should be reviewed prior to treatment. CONCLUSION: Surgical treatment of spontaneous biliary duct rupture should be indicated only after careful consideration of the patient's clinical and comorbidity status.

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Blunt injury abdomen causing biliary injury is rare in children. Collection of bile within or outside the liver after biliary injury is called bilioma. CT scan images of late presenting intra-hepatic bilioma are shown.

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Bilioma é qualquer coleção de bile fora das vias biliares. Geralmente, resulta de complicações cirúrgicas e trauma abdominal. A ocorrência espontânea é rara, ocasionalmente associada a coledocolitíase. Relata-se um caso de bilioma espontâneo, cujo diagnóstico foi confirmado radiologicamente. À laparotomia, observou-se bilioma retroperitoneal. A colangiografia transoperatória não evidenciou fístula. Após drenagem, o paciente teve boa evolução e alta hospitalar.

Biloma is defined as any collection of bile outside the biliary tree, usually resulting from surgery complications and abdominal trauma. Spontaneous occurrence of bilomas is rare, occasionally associated with choledocolithiasis. The present report describes a case of spontaneous biloma, whose diagnosis was radiologically confirmed. At laparotomy, the presence of a retroperitoneal biloma was observed. Intraoperative cholangiography has not demonstrated the presence of fistula. After drainage, the patient progressed well and was discharged.

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