Síndrome de Mirizzi, complicación inusual de la colelitiasis / Mirizzi Syndrome, a Rare Complication of Cholelithiasis

Introducción: El síndrome de Mirizzi es una entidad clínica rara y difícil de tratar; sin embargo, los avances tecnológicos recientes han brindado a los cirujanos nuevas opciones para un diagnóstico y tratamiento más efectivos de esta afección. Objetivo: Presentar el caso de síndrome de Mirizzi, complicación inusual de la colelitiasis. Presentación del caso: Se presenta una paciente femenina, de 56 años de edad, que acude a nuestra institución por presentar ictericia intermitente, dolor en el hipocondrio derecho, coluria y acolia. Conclusiones: El síndrome de Mirizzi, complicación de la colelitiasis, es una entidad poco frecuente, de difícil diagnóstico preoperatorio, por lo que en un gran porcentaje de los casos se diagnostica intraoperatoriamente. Su tratamiento es quirúrgico(AU)

Introduction: Mirizzi syndrome is a rare condition, difficult to treat; however, recent technological advances have provided surgeons with new options for diagnosing and treating this condition more effectively. Objective: To present a case of Mirizzi syndrome, an unusual complication of cholelithiasis. Case presentation: The case is presented of a 56-year-old female patient, who comes to our institution with intermittent jaundice, right hypochondrium pain, choluria and acholia. Conclusions: Mirizzi syndrome, a complication of cholelithiasis, is a rare entity, difficult to diagnose preoperatively; therefore, a large percentage of it's cases are diagnosed intraoperatively. Its treatment is surgical(AU)

A case of double gallbladder with cholelithiasis diagnosed with transabdominal ultrasound.

Gallbladder duplication is a rare biliary tract malformation. According to Boyden's classification, the double gallbladder is divided into the bilobed gallbladder and truly duplicated gallbladder, including the Y-shaped [cystic ducts uniting before entering the common bile duct (CBD)] and H-shaped (cystic ducts separately entering into the CBD) types. The case described here was the Boyden H-shaped type. Preoperative diagnosis of the disease is essential to rationalize surgical planning and avoid complications. Transabdominal ultrasound is the first imaging technique that can diagnose biliary tract abnormality at many institutions. The popularization of typical ultrasound images of the double gallbladder could aid in surgical planning and avoiding complications.

Targeted liver ultrasound performed by an expert is the pivotal imaging examination for low phospholipid-associated cholelithiasis.

OBJECTIVES: Low phospholipid-associatedcholelithiasis (LPAC) is a clinical syndrome that can be associated with variants in the adenosinetriphosphate-binding cassette subfamily B, member 4 (ABCB4) transporter gene, in a proportion of patients. The diagnosis of LPAC is defined by clinical as well as imaging criteria of intrahepatic hyperechoic foci or microlithiasis and biliary sludge on ultrasound. The aim of the study was to assess the role of imaging in investigating patients presenting with clinical features suggesting a diagnosis of LPAC. METHODS: Imaging findings in 51 patients with clinical LPAC were retrospectively reviewed. Most patients had been referred with difficult-to-manage biliary pain postcholecystectomy and some with intrahepatic dilated ducts and stones. The diagnosis of LPAC was made on clinical features. RESULTS: The patients were young with symptom onset at median age 24 years and were mainly female (75%). Ultrasound was performed by an expert in 48/51 and magnetic resonance cholangiopancreatography (MRCP) in 47/51 patients. Targeted liver ultrasound found small hyperechoic foci with comet tail artifacts or posterior acoustic shadowing typical of LPAC in 30/48 (63%) of examinations. However, ultrasound examinations performed before referral for investigation did not report these findings. Intrahepatic duct dilatation was seen in 26/51 (51%) of cases. MRCP did not reliably detect microlithiasis. CONCLUSIONS: Targeted liver ultrasound performed by an expert aware of the possible diagnosis is the pivotal investigation for patients with clinical features suggesting LPAC. The findings in ultrasound performed before referral suggest LPAC is under-recognized and under-diagnosed.

Duplicated gallbladder: an incidental anatomical variation in a patient with symptomatic cholelithiasis.

Congenital malformations of the biliary tract represent a relatively rare entity with which surgeons, radiologists and clinicians are not adequately familiarized. We present a rare case of gallbladder duplication in a 40-year-old female, with the accessory cystic duct entering the left hepatic duct, which depicts the fifth reported case in the international bibliography. Our case illustrates the importance of detailed knowledge of anatomical malformations of the biliary tree, serving the purpose of a preoperative diagnosis of symptomatic cholelithiasis. It is also of paramount importance to take under consideration biliary tract malformations to avoid inadvertent complications such as biliary duct injuries in case of laparoscopic cholecystectomy.

Clinical, biological, radiological, and genetic study of LPAC syndrome in Tunisian patients.

BACKGROUND AND STUDY AIMS: Low phospholipid-associated cholelithiasis (LPAC) syndrome is a form of cholelithiasis associated with the ABCB4 gene mutation. The defects of the protein ABCB4 encoded by this gene promote the formation of biliary cholesterol microcalculations. ABCB4 screening is negative in a significant proportion of patients. PATIENTS AND METHODS: An analytical study of the epidemiological, clinical, biological, and radiological characteristics of 19 patients was conducted, followed by Sanger-type sequencing of the 27 exons encoding the ABCB4 gene. RESULTS: Our results showed a female predominance, symptomatic vesicular lithiasis predominance, and a high frequency of biliary complications in patients carrying an ABCB4 mutation. Normal ​​ liver enzyme values were found in 84.2% of the cases. Intrahepatic hyperechoic foci were present in 68.4%. Molecular analysis detected a pathogenic mutation of the ABCB4 gene in 31.57% of patients. The mutations found were a nonsense mutation and three missense mutations, including two new mutations. CONCLUSION: Our epidemiological, clinical, and genetic results concord with previous studies of LPAC syndrome. Two of the mutations we found have never been detected in patients with LPAC. The low percentage of ABCB4 gene mutations can be explained by the absence of studies of other genes involved in bile acid homeostasis besides the ABCB4 gene and by the inclusion criteria used in this study.

Cystic duct stones in postcholecystectomy Mirizzi syndrome - A novel endoscopic treatment.

Mirizzi syndrome is a rare type of cholelithiasis, and the main treatment is still surgery. The development of endoscopic technology has made surgeons more active in the management of rare diseases of the biliary tract and pancreas. Here we report that our center applied the new endoscopic method to treat a Mirizzi patient with residual cystic neck duct stones after laparoscopic cholecystectomy.

Does "Sludge" Require Cholecystectomy?

Biliary sludge is a subjective, ill-defined term. Surgery is often consulted for laparoscopic cholecystectomy, regarded as a low risk procedure.After IRB approval, a word search was used to identify "sludge" in all ultrasounds performed in 2016. The number of patients undergoing cholecystectomy, complications, pathologic findings, and risk factors were identified. Non-operative patients were evaluated for subsequent symptoms and studies or procedures related to biliary pathology.2769 patients underwent RUQ US; 253 patients were found to have sludge. Of 48 (19%) cholecystectomy patients, 9 had cholelithiasis. No deaths occurred in the cholecystectomy group. Two surgical complications occurred. Fifty (24.4%) of the 205 non-operative patients underwent subsequent US imaging: 44% residual sludge, 28% normal, 18% stones, and 10% other.Sludge may resolve 28% of the time. Repeat ultrasound is prudent before proceeding with cholecystectomy. If an abnormality is seen on repeat imaging and risk factors persist, cholecystectomy may be reasonable.

CHOLELITHIASIS IN CAPTIVE MOUNTAIN CHICKEN FROGS (LEPTODACTYLUS FALLAX): DIAGNOSTIC IMAGING AND HISTOPATHOLOGICAL FEATURES.

The mountain chicken frog (Leptodactylus fallax) is a critically endangered species highly reliant on ex situ conservation programs. In captivity, cholelithiasis has been reported during clinical examinations using diagnostic imaging and observed during postmortem examinations. Some individuals have presented with nonspecific clinical signs potentially associated with this condition, but little is known about its clinical relevance. A retrospective evaluation of ultrasonographic (n = 139) and radiographic (n = 156) images and histopathology samples (n = 32) obtained between 2014 and 2020 from 133 individuals was performed. An ultrasound scoring system was developed to evaluate changes in the gallbladder, with 38% (53/139) of the images showing abnormal contents. Gallbladder distension appeared associated with these findings (generalized estimating equation model, P < 0.001). In contrast, radiography proved to have low overall sensitivity (48%) when compared to ultrasonography for identifying abnormal gallbladder contents. A weak level of agreement (Cohen's kappa = 0.535) was observed between the imaging modalities. Histologically, cholecystitis was present in 86% (12/14) of the individuals, thickening of the gallbladder wall in 71% (10/14), and intrahepatic cholangitis in 90% (18/20). There was no evidence of cholestasis in any of the affected individuals. A significant difference was observed in the hepatic melanomacrophage density between animals with and without cholelithiasis (Welch two-sample t test, P < 0.001). This study provides an overview on the use of diagnostic imaging modalities to evaluate cholelithiasis and proposes a convenient ultrasound score to standardize monitoring of the condition in L. fallax. It is also the first systematic review of histopathological changes associated with cholelithiasis in this species.

Clinical diagnosis of cholecystitis in emergency department patients with cholelithiasis is indication for urgent cholecystectomy: A comparison of clinical, ultrasound, and pathologic diagnosis.

BACKGROUND: Biliary pathology is a common reason for emergency department visits with discharge and outpatient follow up if sonographic evaluation does not reveal evidence of cholecystitis. This retrospective review was conducted to assess the sensitivity of clinical versus sonographic evaluation for indication of urgent cholecystectomy. METHODS: Retrospective chart review of 308 patients who presented to the emergency department (ED) with right upper quadrant (RUQ) pain and cholelithiasis whom underwent cholecystectomy. The history and physical exam, laboratory values, ultrasound (US), and final surgical pathology were compared for accuracy of clinical to pathologic diagnosis. RESULTS: 95.5% of our patients that presented to the ED secondary to RUQ pain with cholelithiasis had pathologic cholecystitis. Sensitivity of clinical diagnosis was superior to US findings as compared to pathologic diagnosis of cholecystitis at 96% vs 44% and 87% vs 18% for acute (AC) and chronic cholecystitis (CC) respectively. CONCLUSION: RUQ pain with known cholelithiasis lasting longer than 4 hours is sensitive for pathologic cholecystitis. This finding, even with the absence of sonographic evidence of cholecystitis, is indication for index encounter urgent cholecystectomy.

Evaluating the diagnostic accuracy of point-of-care ultrasound for cholelithiasis and cholecystitis in a canadian emergency department.

OBJECTIVES: Cholelithiasis and cholecystitis are common conditions that frequently require patients to come to the Emergency Department (ED) and undergo diagnostic imaging. The purpose of this study was to evaluate the test characteristics of emergency physician performed point-of-care ultrasound (POCUS) to diagnose cholelithiasis and cholecystitis in a Canadian ED. METHODS: A health records review was performed on all ED patients > 17 years of age for whom POCUS was performed to diagnose cholelithiasis and cholecystitis in a Canadian academic ED over a 5-year period. The sensitivity, specificity, predictive values, and likelihood ratios were calculated. The gold standard used for diagnosis was pathology, laparoscopy, radiology-performed comprehensive ultrasonography, followed by computed tomography scans. RESULTS: A total of 577 patients were included in the study. The sensitivity and specificity of POCUS to diagnose cholelithiasis was 95.2% (95% CI 91.1-97.8%) and 93.1% (95% CI 90.1-95.4%). The positive and negative likelihood ratios for POCUS to diagnose cholelithiasis were found to be 14 and 0.05; the negative predictive value was 97.6% (95% CI 95.5-98.7%). The sensitivity and specificity of POCUS to diagnose cholecystitis was 67.1% (95% CI 54.9-77.9%) and 97.6% (95% CI 95.9-98.8%). The positive and negative likelihood ratios for POCUS to diagnose cholecystitis were found to be 28 and 0.34; the negative predictive value was 95.6% (95% CI 93.9-96.8%). CONCLUSION: POCUS is reliable for the diagnosis of cholelithiasis and for ruling in cholecystitis. In cases where POCUS is negative or indeterminate for cholecystitis, further imaging should be obtained as clinical suspicion warrants.

RéSUMé: OBJECTIFS: La cholélithiase et la cholécystite sont des troubles médicaux courants qui obligent fréquemment les patients à se rendre aux urgences et subir une imagerie diagnostique. Le but de cette étude était d'évaluer les caractéristiques des tests de l'échographie au point d'intervention (POCUS) effectuée par des médecins urgentistes pour diagnostiquer la cholélithiase et la cholécystite dans une urgence canadienne. MéTHODES: Un examen des dossiers médicaux a été effectué sur tous les patients des services d'urgence, âgés de plus de 17 ans pour lesquels POCUS a été réalisée pour diagnostiquer la cholélithiase et la cholécystite dans un service d'urgence universitaire canadien sur une période de 5 ans. La sensibilité, la spécificité, les valeurs prédictives et les rapports de vraisemblance ont été calculés. L'étalon-or utilisé pour le diagnostic était la pathologie, la laparoscopie, l'échographie complète réalisée par radiologie, suivie de la tomodensitométrie. RéSULTATS: Au total, 577 patients ont été inclus dans l'étude. La sensibilité et la spécificité de POCUS pour diagnostiquer la cholélithiase étaient de 95.2% (IC 95% 91.1­97.8%) et 93.1% (IC 95% 90.1­95.4%). Les rapports de vraisemblance positifs et négatifs pour POCUS pour diagnostiquer la cholélithiase se sont révélés à 14 et 0.05; la valeur prédictive négative était de 97.6% (IC à 95% 95.5­98.7%). La sensibilité et la spécificité de POCUS pour diagnostiquer la cholécystite étaient de 67,1% (IC à 95% 54.9­77.9%) et de 97.6% (IC à 95% 95.9­98.8%). Les rapports de vraisemblance positifs et négatifs pour POCUS pour diagnostiquer la cholécystite se sont révélés à 28 et 0.34; la valeur prédictive négative était de 95.6% (IC à 9.5% 93.9­96.8%). CONCLUSION: POCUS est fiable pour le diagnostic de la cholélithiase et de la cholécystite. Dans les cas où le POCUS est négatif ou indéterminé pour la cholécystite, une imagerie supplémentaire doit être obtenue en cas de suspicion clinique.

SYMPTOMATIC CHOLELITHIASIS AS THE PRESENTATION OF PEDIATRIC PRIMARY SCLEROSING CHOLANGITIS - CASE SERIES AND LITERATURE REVIEW.

BACKGROUND: Primary sclerosing cholangitis (PSC) is a rare hepatobiliary disorder, whose etiology remains not fully elucidated. Given how rare PSC is in childhood, until the recent publication of a multicenter international collaboration, even data on its characteristics and natural history were scarce. Symptomatic cholelithiasis has not been previously reported as the presentation of PSC. OBJECTIVE: The aim of this study was the diagnosis of PSC following the initial unusual presentation with symptomatic cholelithiasis, that followed an atypical clinical course that could not be explained by cholelithiasis alone. A literature review was also conducted. METHODS: We conducted a retrospective chart review of three patients, who were diagnosed and/or followed at the Clinics Hospital, University of Campinas - Sao Paulo/ Brazil, between 2014 and 2020. Data analyzed included gender, age of presentation, past medical history, imaging findings, laboratory results, endoscopic evaluation, response to medical therapy and follow-up. RESULTS: Age at time of presentation with cholelithiasis varied from 10 to 12 years. In two of the cases reported, a more subacute onset of symptoms preceded the episode of cholelithiasis. Two patients were managed with cholecystectomy, not followed by any surgical complications, one patient was managed conservatively. Percutaneous liver biopsy was performed in all three cases, showing histological findings compatible with PSC. Associated inflammatory bowel disease (IBD) was not seen in any of the patients. The patients have been followed for a mean time of 3.4 years. CONCLUSION: PSC and cholelithiasis are both rare in the pediatric population. This study reports on symptomatic cholelithiasis as a presentation of PSC and raises the importance of suspecting an underlying hepatobiliary disorder in children with cholelithiasis without any known predisposing factors and/or that follow an atypical clinical course for cholelithiasis alone.

Colelitíase sintomática como apresentação de colangite esclerosante primária na infância - série de casos e revisão de literatura / Symptomatic cholelithiasis as the presentation of pediatric primary sclerosing cholangitis - case series and literature review

ABSTRACT BACKGROUND: Primary sclerosing cholangitis (PSC) is a rare hepatobiliary disorder, whose etiology remains not fully elucidated. Given how rare PSC is in childhood, until the recent publication of a multicenter international collaboration, even data on its characteristics and natural history were scarce. Symptomatic cholelithiasis has not been previously reported as the presentation of PSC. OBJECTIVE: The aim of this study was the diagnosis of PSC following the initial unusual presentation with symptomatic cholelithiasis, that followed an atypical clinical course that could not be explained by cholelithiasis alone. A literature review was also conducted. METHODS: We conducted a retrospective chart review of three patients, who were diagnosed and/or followed at the Clinics Hospital, University of Campinas - Sao Paulo/ Brazil, between 2014 and 2020. Data analyzed included gender, age of presentation, past medical history, imaging findings, laboratory results, endoscopic evaluation, response to medical therapy and follow-up. RESULTS: Age at time of presentation with cholelithiasis varied from 10 to 12 years. In two of the cases reported, a more subacute onset of symptoms preceded the episode of cholelithiasis. Two patients were managed with cholecystectomy, not followed by any surgical complications, one patient was managed conservatively. Percutaneous liver biopsy was performed in all three cases, showing histological findings compatible with PSC. Associated inflammatory bowel disease (IBD) was not seen in any of the patients. The patients have been followed for a mean time of 3.4 years. CONCLUSION: PSC and cholelithiasis are both rare in the pediatric population. This study reports on symptomatic cholelithiasis as a presentation of PSC and raises the importance of suspecting an underlying hepatobiliary disorder in children with cholelithiasis without any known predisposing factors and/or that follow an atypical clinical course for cholelithiasis alone.

RESUMO CONTEXTO: A colangite esclerosante primária (CEP) é uma doença hepatobiliar rara, cuja etiologia ainda não está totalmente elucidada. Dada a raridade do CEP na infância, até a recente publicação de uma colaboração multicêntrica internacional, mesmo dados sobre suas características e história natural eram escassos. A colelitíase sintomática não foi relatada anteriormente como a apresentação inicial de CEP na infância. OBJETIVO: O objetivo deste estudo foi o diagnóstico de CEP após a apresentação inicial incomum com colelitíase sintomática, que seguiu um curso clínico atípico que não poderia ser explicado apenas pela colelitíase. Também foi realizada uma revisão da literatura. MÉTODOS: Foi realizada uma revisão retrospectiva dos prontuários de três pacientes, que foram diagnosticados e/ou acompanhados no Hospital das Clínicas da Universidade Estadual de Campinas - São Paulo / Brasil, entre 2014 e 2020. Os dados analisados incluíram sexo, idade de apresentação, história médica pregressa, achados de imagem, resultados laboratoriais, avaliação endoscópica, resposta à terapia médica e acompanhamento. RESULTADOS: A idade no momento da apresentação da colelitíase variou de 10 a 12 anos. Em dois dos casos relatados, um início mais subagudo dos sintomas precedeu o episódio de colelitíase. Dois pacientes foram tratados com colecistectomia, não seguida de qualquer complicação cirúrgica, e um paciente foi tratado de forma conservadora. Biópsia hepática percutânea foi realizada em todos os três casos, mostrando achados histológicos compatíveis com CEP. Doença inflamatória intestinal associada não foi observada em nenhum dos pacientes. Os pacientes foram acompanhados por um tempo médio de 3,4 anos. CONCLUSÃO: CEP e colelitíase são raras na população pediátrica. Este estudo relata a colelitíase sintomática como uma apresentação de CEP e levanta a importância da suspeita de doença hepatobiliar subjacente em crianças com colelitíase sem quaisquer fatores predisponentes conhecidos e/ou que seguem um curso clínico atípico.

A new strategy of laparoscopic anatomical hemihepatectomy guided by the middle hepatic vein combined with transhepatic duct lithotomy for complex hemihepatolithiasis: A propensity score matching study.

BACKGROUND: There have been no studies on laparoscopic anatomical hemihepatectomy guided by the middle hepatic vein combined with transhepatic duct lithotripsy for the treatment of complex hemihepatolithiasis. This study aimed to investigate the safety and efficacy of laparoscopic anatomical hemihepatectomy guided by the middle hepatic vein combined with transhepatic duct lithotomy to treat complex hemihepatolithiasis. METHODS: The clinical data for patients who underwent laparoscopic anatomical hemihepatectomy for complex intrahepatic bile duct stones with or without common bile duct stones from January 2016 to June 2020 were prospectively collected. Patients were divided into 2 groups according to surgical approach: laparoscopic anatomical hemihepatectomy guided by the middle hepatic vein (middle hepatic vein group) or laparoscopic anatomical hemihepatectomy not guided by the middle hepatic vein (nonmiddle hepatic vein group). The safety and short-term and long-term efficacy outcomes of the 2 groups were compared with 1:1 propensity score matching. RESULTS: With only a slightly longer operative time (P = .006), the initial and final stone residual rates in the middle hepatic vein group (n = 70) were significantly lower than those in the nonmiddle hepatic vein group (n = 70) (P = .002, P = .009). The bile leakage rate and stone recurrence rate were also significantly lower (P = .001, P = .001). CONCLUSION: Laparoscopic anatomical hemihepatectomy guided by the middle hepatic vein is safe and effective for treating intrahepatic bile duct stones and can decrease the stone residual rate, reduce the bile leakage rate and stone recurrence rate, and accelerate early recovery. However, owing to the complicated technical requirements for surgeons and anesthesiologists, use of the procedure is limited to large and experienced medical centers.