RESUMEN
PURPOSE: We aimed to investigate whether prediction of liver fibrosis using two-dimensional shear wave elastography (2D-SWE) and vascular tree grading using superb microvascular imaging (SMI) are useful for postoperative follow-up in patients with biliary atresia (BA). METHODS: We retrospectively collected data from medical records of 134 patients who underwent ultrasound examination with 2D-SWE or SMI, including 13 postoperative patients with BA and 121 non-BA patients. We investigated the distribution of liver stiffness values with SWE and vascular tree grading with SMI and evaluated correlations between these findings and biochemical indices of liver fibrosis in postoperative BA patients. RESULTS: The SWE values of the BA group were not significantly different from that of any other disease groups in non-BA patients. In postoperative BA patients, SWE values correlated significantly with aspartate aminotransferase to platelet ratio index (Spearman rank correlation coefficient [rs] = 0.6380, p = 0.0256) and with the Fib-4 index (rs = 0.6526, p = 0.0214). SMI vascular tree grading of the BA group was significantly higher than that of the choledochal cyst group (p = 0.0008) and other hepatobiliary disorder group (p = 0.0030). In postoperative BA patients, SMI vascular tree grading was not positively correlated with any biochemical marker of fibrosis. CONCLUSION: 2D-SWE appears to be useful for follow-up in postoperative BA patients.
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Atresia Biliar , Diagnóstico por Imagen de Elasticidad , Cirrosis Hepática , Humanos , Atresia Biliar/cirugía , Atresia Biliar/diagnóstico por imagen , Diagnóstico por Imagen de Elasticidad/métodos , Masculino , Estudios Retrospectivos , Femenino , Cirrosis Hepática/diagnóstico por imagen , Lactante , Microvasos/diagnóstico por imagen , Hígado/diagnóstico por imagen , Hígado/irrigación sanguínea , Preescolar , Periodo Posoperatorio , Estudios de Seguimiento , Niño , Complicaciones Posoperatorias/diagnóstico por imagenRESUMEN
Biliary atresia (BA) is a congenital disease that occurs when extrahepatic bile ducts are either absent or deficient, resulting in liver fibrosis, portal hypertension, and eventually cirrhosis. It is the most common cause of persistent obstructive jaundice in newborns lasting more than two weeks is this condition. Abdominal ultrasound (US) is the primary imaging technique used to diagnose BA, while computed tomography (CT) is reserved for more complex cases. The gold standard for diagnosing BA is still intraoperative cholangiogram with liver biopsy. Treatment for BA usually involves Kasai hepatoportoenterostomy, but some patients still require liver transplantation due to diagnostic delays and advanced disease. In this study, the authors present the case of a 3-month-old infant with biliary atresia and its ultrasound characteristics, who underwent liver transplantation due to advanced disease. The primary objective of imaging is to provide a prompt diagnosis, given the crucial significance of timely surgical intervention.
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Atresia Biliar , Trasplante de Hígado , Ultrasonografía , Humanos , Atresia Biliar/diagnóstico por imagen , Atresia Biliar/cirugía , Atresia Biliar/complicaciones , Lactante , Ultrasonografía/métodos , Masculino , Hígado/diagnóstico por imagen , Hígado/patología , FemeninoRESUMEN
To evaluate the efficacy and safety of indocyanine green (ICG)-guided near-infrared fluorescence (NIRF) imaging during surgery to diagnose the cause of neonatal cholestasis (NC). Data on NC patients who underwent both NIRF with ICG and conventional laparoscopic bile duct exploration (the gold standard) at our institute from January 2022 to December 2022 were retrospectively analyzed. The patients' baseline characteristics and liver function outcomes were collected and analyzed, and the diagnostic consistency was compared between the 2 methods. In total, 16 NC patients were included in the study, comprising 8 (50%) male and 8 (50%) female patients, ranging in age from 42 to 93 days, with a median age of 54.4â ±â 21 days. During surgery, all the patients underwent NIRF with ICG, followed by conventional laparoscopic bile duct exploration. Finally, 15 of the patients were diagnosed with biliary atresia (BA) (1 with type-I BA, and 14 with type-II BA). The other patient was diagnosed with cholestasis. The diagnostic results from fluorescence imaging with ICG were consistent with those from conventional laparoscopic bile duct exploration. ICG-guided NIRF is associated with an easy operation, less trauma, and good safety. Also, its diagnostic accuracy is similar to conventional laparoscopic bile duct exploration.
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Colestasis , Verde de Indocianina , Imagen Óptica , Humanos , Verde de Indocianina/administración & dosificación , Femenino , Masculino , Estudios Retrospectivos , Colestasis/diagnóstico por imagen , Colestasis/etiología , Imagen Óptica/métodos , Lactante , Recién Nacido , Atresia Biliar/cirugía , Atresia Biliar/diagnóstico por imagen , Laparoscopía/métodos , Colorantes/administración & dosificación , Espectroscopía Infrarroja Corta/métodosRESUMEN
OBJECTIVE: Tc-99m N-pyridoxyl-5-methyl-tryptophan (PMT) hepatobiliary scintigraphy has high diagnostic performance for biliary atresia. Our hospital implements standard Tc-99m PMT administration followed by a 6 h static imaging review; booster doses are given in cases requiring 24 h delayed scans. This study aimed to evaluate the diagnostic performance of this method. METHODS: A total of 37 pediatric patients who underwent Tc-99m PMT biliary scintigraphy were classified into the surgically-diagnosed biliary atresia or non-biliary atresia groups. The absence of tracer accumulation in the small bowel was considered a hepatobiliary scintigraphic diagnosis of biliary atresia. The Clopper-Pearson method was used to calculate the 95% confidence intervals (CIs) for determining the diagnostic accuracy, negative predictive value, positive predictive value, sensitivity, and specificity of Tc-99m PMT biliary scintigraphy. RESULTS: Among the 37 patients, 12 were classified into the diagnosis of biliary atresia group. Regarding biliary scintigraphy findings, 16 of 37 patients demonstrated tracer accumulation in the small bowel within 6 h of testing. These cases were diagnosed as non-biliary atresia, requiring no further testing or booster administration. In contrast, 21 patients underwent delayed testing requiring booster administration, which revealed 13 without tracer excretion and 11 who were diagnosed with biliary atresia. Among the eight patients with tracer accumulation, only one was diagnosed with biliary atresia. Furthermore, two cases without tracer excretion and seven cases with tracer excretion were clinically diagnosed as non-biliary atresia. The diagnostic performance of our examination was as follows: a diagnostic accuracy of 91.9% (34/37; 95% CIs 78.0-98.3%), sensitivity of 91.6% (11/12; 95% CIs 61.5-99.8%), specificity of 92.0% (23/25; 95% CIs 74.0-99.0%), a positive predictive value of 84.6% (11/13; 95% CIs 54.6-98.0%), and a negative predictive value of 95.8% (23/24; 95% CIs 78.9-99.9%). CONCLUSIONS: Our protocol for Tc-99m PMT biliary scintigraphy using tracer booster administration demonstrated reliable diagnostic performance for biliary atresia. Notably, 43% of cases did not require booster administration, indicating that lesser radiation exposure may still yield comparable diagnostic accuracy.
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Atresia Biliar , Compuestos de Organotecnecio , Cintigrafía , Humanos , Atresia Biliar/diagnóstico por imagen , Masculino , Femenino , Cintigrafía/métodos , Lactante , Factores de Tiempo , Preescolar , Estudios Retrospectivos , Sistema Biliar/diagnóstico por imagen , NiñoRESUMEN
BACKGROUND: Biliary atresia is the most common cause of obstructive jaundice in infants and conventional cholangiography is the current diagnostic gold standard. Fluorescent cholangiography with indocyanine green can enhance biliary tree visualization during surgery because it is exclusively excreted into the bile ducts and eventually into the intestine. Therefore, we hypothesized that indocyanine green presence in stool could confirm bile duct patency in infants. METHODS: A prospective single center cohort study was performed on infants (age ≤ 12 months) with and without jaundice after obtaining IRB approval. Indocyanine green was administered intravenously (0.1 mg/kg). Soiled diapers collected post-injection were imaged for fluorescence. RESULTS: After indocyanine green administration, fluorescence was detected in soiled diapers for control patients (n = 4, x = 14 h22 m post-injection) and jaundiced patients without biliary atresia (n = 11, x = 13 h28 m post-injection). For biliary atresia patients (n = 7), post-injection soiled diapers before and after Kasai portoenterostomy were collected. Fluorescence was not detected in stool from 6 of 7 biliary atresia patients. As a test, indocyanine green detection in stool was 97% accurate for assessing biliary patency. CONCLUSION: Fluorescent Imaging for Indocyanine Green (FIInd Green) in stool is a fast and accurate approach to assess biliary patency non-invasively in infants. LEVEL OF EVIDENCE: Level III.
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Atresia Biliar , Colorantes , Heces , Verde de Indocianina , Humanos , Atresia Biliar/diagnóstico por imagen , Atresia Biliar/cirugía , Atresia Biliar/complicaciones , Proyectos Piloto , Lactante , Heces/química , Estudios Prospectivos , Masculino , Femenino , Colorantes/administración & dosificación , Colangiografía/métodos , Portoenterostomía Hepática , Imagen Óptica/métodos , Ictericia Obstructiva/etiología , Ictericia Obstructiva/diagnóstico por imagen , Recién NacidoRESUMEN
BACKGROUND: A previously trained deep learning-based smartphone app provides an artificial intelligence solution to help diagnose biliary atresia from sonographic gallbladder images, but it might be impractical to launch it in real clinical settings. This study aimed to redevelop a new model using original sonographic images and their derived smartphone photos and then test the new model's performance in assisting radiologists with different experiences to detect biliary atresia in real-world mimic settings. METHODS: A new model was first trained retrospectively using 3659 original sonographic gallbladder images and their derived 51,226 smartphone photos and tested on 11,410 external validation smartphone photos. Afterward, the new model was tested in 333 prospectively collected sonographic gallbladder videos from 207 infants by 14 inexperienced radiologists (9 juniors and 5 seniors) and 4 experienced pediatric radiologists in real-world mimic settings. Diagnostic performance was expressed as the area under the receiver operating characteristic curve (AUC). RESULTS: The new model outperformed the previously published model in diagnosing BA on the external validation set (AUC 0.924 vs 0.908, P = 0.004) with higher consistency (kappa value 0.708 vs 0.609). When tested in real-world mimic settings using 333 sonographic gallbladder videos, the new model performed comparable to experienced pediatric radiologists (average AUC 0.860 vs 0.876) and outperformed junior radiologists (average AUC 0.838 vs 0.773) and senior radiologists (average AUC 0.829 vs 0.749). Furthermore, the new model could aid both junior and senior radiologists to improve their diagnostic performances, with the average AUC increasing from 0.773 to 0.835 for junior radiologists and from 0.749 to 0.805 for senior radiologists. CONCLUSIONS: The interpretable app-based model showed robust and satisfactory performance in diagnosing biliary atresia, and it could aid radiologists with limited experiences to improve their diagnostic performances in real-world mimic settings.
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Atresia Biliar , Aplicaciones Móviles , Lactante , Niño , Humanos , Vesícula Biliar/diagnóstico por imagen , Inteligencia Artificial , Atresia Biliar/diagnóstico por imagen , Estudios Retrospectivos , RadiólogosRESUMEN
OBJECTIVES: To investigate the feasibility of using preoperative imaging indices to predict 2-year native liver survival after the Kasai procedure in patients with biliary atresia (BA). MATERIALS AND METHODS: The retrospective review included 190 BA patients who underwent the Kasai procedure between 2000 and 2020, with preoperative US and/or MRI, excluding cases with less than 2-year follow-up period. Multivariable logistic regression analysis was performed to identify imaging indices to predict 2-year native liver survival. Kasai failure was defined as the need for liver transplantation or death within 2 years of the Kasai procedure. RESULTS: Of the 90 patients included, all had preoperative US, and 61 also had MRI. Kasai failure occurred in 52% (47/90). Preoperative US identified gallbladder length (OR 0.40, 95% CI 0.17-0.95, p = 0.039; cutoff 1.6 cm, AUC 67.66) and biliary cysts (OR 24.64, 95% CI 1.97-308.08, p = 0.013) as significant Kasai failure predictors, with a combined accuracy of 73% (60/82). For patients having both preoperative US and MRI, significant predictors were hepatic artery diameter (OR 6.75, 95% CI 1.31-34.88, p = 0.023; cutoff 2 mm, AUC 73.83) and biliary cysts (OR 23.89, 95% CI 1.43-398.82, p = 0.027) on US, and gallbladder length (OR 0.25, 95% CI 0.08-0.76, p = 0.014; cutoff 1.2 cm, AUC 74.72) and spleen size (OR 2.53, 95% CI 1.02-6.29, p = 0.045; cutoff 6.9 cm, AUC 73.72) on MRI, with a combined accuracy of 85% (52/61). CONCLUSION: Preoperative US and/or MRI enhance the 2-year native liver survival prediction in BA patients after the Kasai procedure. CLINICAL RELEVANCE STATEMENT: BA patients with hepatic artery diameter > 2 mm (US), gallbladder length < 1.6 cm (US) or < 1.2 cm (MRI), spleen size > 6.9 cm (MRI), and absence of biliary cysts (US/MRI) have a decreased likelihood of 2-year native liver survival. KEY POINTS: ⢠Preoperative US and/or MRI can predict the probability of achieving 2-year native liver survival following the Kasai procedure. ⢠Combining US and MRI improved the accuracy to 85% for predicting 2-year native liver survival in BA patients. ⢠The hepatic artery diameter > 2 mm (US), gallbladder length < 1.6 cm (US) or < 1.2 cm (MRI), spleen size > 6.9 cm (MRI), and no biliary cysts (US/MRI) are significant predictors of Kasai failure in patients with biliary atresia.
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Enfermedades de los Conductos Biliares , Atresia Biliar , Quistes , Trasplante de Hígado , Humanos , Lactante , Atresia Biliar/diagnóstico por imagen , Atresia Biliar/cirugía , Portoenterostomía Hepática/métodos , Hígado/diagnóstico por imagen , Hígado/cirugía , Trasplante de Hígado/métodos , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
BACKGROUND: We investigated the utilities of the liver-to-psoas apparent diffusion coefficient ratios (LTPAR) yielded by diffusion-weighted magnetic resonance imaging (DWMRI) and the age-adjusted serum matrix metalloproteinase-7 (MMP-7) for the diagnosis of biliary atresia (BA) in cholestatic infants. METHODS: In total, 170 cholestatic infants were recruited, of whom 50 (29.41%) were diagnosed with BA after cholestatic workups. The LTPAR and MMP7 levels were assessed. RESULTS: The LTPAR was significantly lower in BA infants, and the age-adjusted MMP7 ratio was significantly higher, compared to other cholestatic infants (both p < 0.001). Receiver operating characteristic curve analysis yielded a cutoff > 0.1 ng/mL.day for the age-adjusted MMP-7 ratio, and an LTPAR < 1.01 for the optimal prediction of BA (both p < 0.001). Univariate logistic regression analysis revealed that both an age-adjusted MMP-7 ratio > 0.1 ng/mL.day and an LTPAR < 1.01 were significant predictors of BA among cholestatic infants (odds ratio = 30.98 and 13.28; p < 0.001 and < 0.001, respectively). The significance of the age-adjusted MMP-7 ratio and the LTPAR persisted on multivariate logistic regression analysis after adjusting for sex and the serum gamma-glutamyl transferase level (p < 0.001 and < 0.001, respectively). The negative predictive values (NPVs) for BA were 91.49% and 94.17%, respectively, for the LTPAR and age-adjusted MMP-7 ratio. CONCLUSION: The age-adjusted MMP-7 ratio and the LTPAR are both significant non-invasive predictors of BA. The consideration of both serum and imaging parameters may enhance BA diagnostic performance in cholestatic infants.
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Atresia Biliar , Colestasis , Metaloproteinasa 7 de la Matriz , Humanos , Lactante , Atresia Biliar/diagnóstico por imagen , Atresia Biliar/genética , Atresia Biliar/metabolismo , Hígado/diagnóstico por imagen , Hígado/patología , Imagen por Resonancia Magnética , Metaloproteinasa 7 de la Matriz/sangre , Metaloproteinasa 7 de la Matriz/químicaRESUMEN
BACKGROUND AND AIMS: Early diagnosis of biliary atresia (BA), particularly distinguishing it from other causes of neonatal cholestasis (NC), is challenging. This study aimed to design and validate a predictive model for BA by using the data available at the initial presentation. METHODS: Infants presenting with NC were retrospectively identified from tertiary referral hospitals and constituted the model design cohort (n = 148); others were enrolled in a prospective observational study and constituted the validation cohort (n = 21). Clinical, laboratory, and abdominal ultrasonographic features associated with BA were assessed. A prediction model was developed using logistic regression and decision tree (DT) analyses. RESULTS: Three predictors, namely, gamma glutamyl transpeptidase (γGT) level, triangular cord sign (TC sign), and gallbladder abnormalities, were identified as factors for diagnosing BA in multivariate logistic regression, which was used to develop the DT model. The area under the receiver operating characteristic (ROC) curve (AUC) value for the model was 0.905, which was greater than those for γGT level, TC sign, or gallbladder abnormalities alone in the prediction of BA. CONCLUSION: A simple prediction model combining liver function and abdominal ultrasonography findings can provide a moderate and early estimate of the risk of BA in patients with NC.
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Atresia Biliar , Colestasis , Enfermedades de la Vesícula Biliar , Lactante , Recién Nacido , Humanos , Atresia Biliar/diagnóstico por imagen , Atresia Biliar/complicaciones , Estudios Retrospectivos , Ultrasonografía , Colestasis/etiología , Diagnóstico Precoz , Diagnóstico DiferencialRESUMEN
BACKGROUND: Two-dimensional shear wave elastography (2D-SWE) has been proposed for detecting liver fibrosis in biliary atresia. OBJECTIVES: To assess the performance of 2D-SWE for detecting advanced liver fibrosis and cirrhosis in patients with biliary atresia. MATERIALS AND METHODS: Five electronic databases were searched to identify studies investigating the performance of 2D-SWE for diagnosing liver fibrosis in biliary atresia in children. We constructed the summary receiver operating characteristic (SROC) curves of 2D-SWE for detecting advanced liver fibrosis and cirrhosis, and then calculated the area under the SROC curves (AUROCs). RESULTS: Six studies with 470 patients (ages 55 days to 6.6 years) were included. The median correlation coefficient of 2D-SWE with pathological liver fibrosis stages was 0.779 (range: 0.443â0.813). The summary AUROCs for advanced liver fibrosis and cirrhosis were 0.929 and 0.883, respectively. The summary sensitivity and specificity of 2D-SWE for advanced liver fibrosis were 88% (95% confidence interval [CI]: 80â94%) and 85% (95% CI: 77â91%) with I values of 0% and 45.6%, respectively, and for cirrhosis were 80% (95% CI: 72â87%) and 82% (95% CI: 77â86%) with I values of 12.9% and 0%, respectively. The diagnostic odds ratio (DOR) of 2D-SWE for advanced liver fibrosis and cirrhosis were 40.3 (95% CI: 18.2â89.4) and 18.9 (95% CI: 11.2â31.7), respectively. For preoperative detection of cirrhosis, the pooled AUROC, sensitivity, specificity, and DOR based on the four 2D-SWE studies were 0.877, 79% (95% CI: 71â86%), 82% (95% CI: 77â86%), and 17.58 (95% CI: 10.35â29.85), respectively. CONCLUSIONS: Results show that 2D-SWE has potential as a non-invasive tool for detecting advanced liver fibrosis and cirrhosis in patients with biliary atresia.
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Atresia Biliar , Diagnóstico por Imagen de Elasticidad , Niño , Humanos , Atresia Biliar/complicaciones , Atresia Biliar/diagnóstico por imagen , Atresia Biliar/patología , Diagnóstico por Imagen de Elasticidad/métodos , Cirrosis Hepática/diagnóstico por imagen , Cirrosis Hepática/patología , Fibrosis , Hígado/diagnóstico por imagenRESUMEN
ABSTRACT: The application of intracavity contrast-enhanced ultrasound in the evaluation of biliary disease has been confirmed valuable among pediatric population. This pictorial essay aims to demonstrate the role of percutaneous ultrasound cholangiography (PUSC) with microbubbles in the diagnosis of different pediatric biliary diseases in our center. The biliary system's morphologic characteristics in PUSC mode of neonatal hepatitis, biliary atresia, choledochal cysts, and biliary complications of hepatobiliary surgery are presented.
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Atresia Biliar , Sistema Biliar , Recién Nacido , Niño , Humanos , Lactante , Microburbujas , Colangiografía , Sistema Biliar/diagnóstico por imagen , Atresia Biliar/diagnóstico por imagen , Atresia Biliar/complicaciones , UltrasonografíaRESUMEN
In recent years, it has been reported that indocyanine green can be used for intraoperative navigation in Kasai surgery. However, there are no reports of its application in surgery for rare type II cystic biliary atresia. We report a girl presented with jaundice and light-colored stools. Laboratory tests showed impaired liver function with elevated serum bilirubin and bile acid levels. The abdominal ultrasound and MRCP suggested a common hepatic duct cyst. A diagnosis of choledochal cyst was suspected and biliary atresia could not be excluded. Conservative treatment was unsatisfactory. Laparoscopic exploration with indocyanine green fluorescence was performed on the 38th day of her life, and intraoperative diagnosis of type II CBA was made because the common hepatic duct cyst and its downstream anatomical structures did not show fluorescence. The postoperative bilirubin and bile acid levels decreased significantly and she was discharged two weeks after surgery. This result suggests that indocyanine green can be safely used in laparoscopic surgery for type II CBA, which not only helps in the differential diagnosis of CBA and choledochal cyst, but also confirms bile flow in real time.
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Atresia Biliar , Quiste del Colédoco , Laparoscopía , Fotoquimioterapia , Humanos , Femenino , Atresia Biliar/diagnóstico por imagen , Atresia Biliar/cirugía , Verde de Indocianina , Quiste del Colédoco/diagnóstico por imagen , Quiste del Colédoco/cirugía , Fotoquimioterapia/métodos , Fármacos Fotosensibilizantes , Laparoscopía/métodos , Imagen Óptica , Bilirrubina , Ácidos y Sales BiliaresRESUMEN
OBJECTIVE: To evaluate the feasibility of ultrasound shear wave elastography (SWE) for predicting hepatic fibrosis and native liver outcomes in patients with biliary atresia. MATERIALS AND METHODS: This prospective study included 33 consecutive patients with biliary atresia (median age, 8 weeks [interquartile range, 6-10 weeks]; male:female ratio, 15:18) from Severance Children's Hospital between May 2019 and February 2022. Preoperative (within 1 week from surgery) and immediate postoperative (on postoperative days [PODs] 3, 5, and 7) ultrasonographic findings were obtained and analyzed, including the SWE of the liver and spleen. Hepatic fibrosis, according to the METAVIR score at the time of Kasai portoenterostomy and native liver outcomes during postsurgical follow-up, were compared and correlated with imaging and laboratory findings. Poor outcomes were defined as intractable cholangitis or liver transplantation. The diagnostic performance of SWE in predicting METAVIR F3-F4 and poor hepatic outcomes was analyzed using receiver operating characteristic (ROC) analyses. RESULTS: All patients were analyzed without exclusion. Perioperative advanced hepatic fibrosis (F3-F4) was associated with older age and higher preoperative direct bilirubin and SWE values in the liver and spleen. Preoperative liver SWE showed a ROC area of 0.806 and 63.6% (7/11) sensitivity and 86.4% (19/22) specificity at a cutoff of 17.5 kPa for diagnosing F3-F4. The poor outcome group included five patients with intractable cholangitis and three undergoing liver transplantation who showed high postoperative liver SWE values. Liver SWE on PODs 3-7 showed ROC areas of 0.783-0.891 for predicting poor outcomes, and a cutoff value of 10.3 kPa for SWE on POD 3 had 100% (8/8) sensitivity and 73.9% (17/23) specificity. CONCLUSION: Preoperative liver SWE can predict advanced hepatic fibrosis, and immediate postoperative liver SWE can predict poor native liver outcomes in patients with biliary atresia.
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Atresia Biliar , Colangitis , Diagnóstico por Imagen de Elasticidad , Niño , Humanos , Masculino , Femenino , Lactante , Atresia Biliar/diagnóstico por imagen , Atresia Biliar/cirugía , Diagnóstico por Imagen de Elasticidad/métodos , Estudios Prospectivos , Cirrosis Hepática/diagnóstico por imagenRESUMEN
BACKGROUND: Biliary Atresia (BA), an obstructive cholangiopathy, is the most common cause of end-stage liver disease and liver transplantation in children. Timely differentiation of BA from other causes of neonatal jaundice remains a challenge, yet is critical to improving outcomes. METHODS: Clinical characteristics including demographics, age at jaundice presentation, age at hepatobiliary scintigraphy, age at surgery, severity of liver fibrosis, and native-liver survival were reviewed in infants with hyperbilirubinemia and suspected BA for this single center retrospective cohort study. We investigated the accuracy of hepatobiliary scintigraphy as well as elapsed time from jaundice presentation to diagnostic intervention. RESULTS: BA was suspected in 234 infants. BA was identified in 17% of infants with hepatobiliary scintigraphy and 72% of infants who underwent operative exploration without hepatobiliary scintigraphy. Elapsed time from jaundice presentation to Kasai Portoenterostomy (KPE) for BA patients was 2.1x longer if hepatobiliary scintigraphy was obtained (p = 0.084). The mean age at KPE for this cohort was 66.8 days (n = 54), with a significantly higher mean age at KPE (75.2 days) for infants who were later listed or underwent liver transplantation (p = 0.038). Histologically, the lowest liver fibrosis scores were seen in infants undergoing KPE <30 days old and worsened significantly with increased age (p < 0.001). CONCLUSION: Hepatobiliary scintigraphy compared to operative exploration for the diagnostic evaluation of infants with suspected BA introduces significant time delays to KPE but enables avoidance of surgery in some infants. The temporal pattern of worsening cholestatic liver injury from BA with each day of increased age highlights the importance of intervening as early as possible for the best prognosis. TYPE OF STUDY: Retrospective study, Level of evidence: III.
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Atresia Biliar , Ictericia , Recién Nacido , Lactante , Niño , Humanos , Portoenterostomía Hepática , Estudios Retrospectivos , Atresia Biliar/diagnóstico por imagen , Atresia Biliar/cirugía , Cintigrafía , Ictericia/cirugía , Cirrosis Hepática/cirugíaRESUMEN
BACKGROUND: Biliary atresia (BA) is one of the causes of conjugated hyperbilirubinemia in infants which if untreated leads to end-stage liver disease and death. Percutaneous Trans-hepatic Cholecysto-Cholangiography (PTCC) is a minimally invasive study which can be utilized in the diagnostic work-up of these patients. This study's purpose is to describe the experience with PTCC in neonates, the imaging findings encountered, and the abnormal patterns which warrant further investigation. METHODS: A 16-year single-center retrospective study of patients with persistent neonatal cholestasis (suspected BA) undergoing PTCC. Patient demographics, laboratory values, PTCC images, pathology and surgical reports were reviewed. RESULTS: 73 patients underwent PTCC (68% male, mean age 8.7 weeks, mean weight 4.0 Kg). The majority of studies were normal (55%). Abnormal patterns were identified in 33 cases, 79% were diagnosed with BA and 12% with Alagille syndrome. Non-opacification of the common hepatic duct with a narrowed common bile duct (42%) and isolated small gallbladder (38%) were the most common patterns in BA. CONCLUSION: PTCC is a minimally invasive study in the diagnostic work-up of infants presenting with conjugated hyperbilirubinemia (suspected BA). Further invasive investigations or surgery can be avoided when results are normal.
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Atresia Biliar , Colestasis , Recién Nacido , Lactante , Humanos , Masculino , Femenino , Vesícula Biliar/diagnóstico por imagen , Diagnóstico Diferencial , Estudios Retrospectivos , Colangiografía/métodos , Colestasis/diagnóstico por imagen , Colestasis/etiología , Atresia Biliar/diagnóstico , Atresia Biliar/diagnóstico por imagen , Hiperbilirrubinemia/etiologíaRESUMEN
OBJECTIVE: To explore the diagnostic performance of prenatal ultrasound in the prediction of biliary atresia (BA). METHODS: We prospectively collected cases of suspected biliary abnormalities in the 2nd trimester of pregnancy and performed a series (at least 3) of prenatal ultrasound examinations in the 2nd and 3rd trimester. The presence of the gallbladder was examined each time, and its size and shape were assessed if the gallbladder was visible. The existence of other abnormalities was carefully evaluated. Neonatal ultrasound examination was conducted within 1 month after birth, and clinical data were followed-up for 6 months after birth. RESULTS: Among the 41â895 patients, 298 were suspected to have biliary abnormalities, while 82 patients were excluded due to loss to follow-up or induced labor caused by other abnormalities. A total of 216 patients were included in this study, and 15 were diagnosed with BA. We summarized the ultrasound findings of the gallbladders and defined a high-risk gallbladder for the prenatal diagnosis of BA. This was demonstrated to have the best diagnostic performance as a single parameter, with an area under the curve of 0.914 (95â%CI: 0.869-0.948). In addition, higher incidences of biliary cysts, right hepatic artery dilation, echogenic bowel, and ascites were observed in BA fetuses. Logistic regression analysis showed that the combination of 5 parameters had better diagnostic performance, with an area under the curve of 0.995 (95â%CI: 0.973-0.999). CONCLUSION: The fetal gallbladder was found to be a critical feature for the identification of BA. Concomitant abnormalities could be helpful to improve the accuracy of the diagnosis.
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Atresia Biliar , Recién Nacido , Embarazo , Femenino , Humanos , Atresia Biliar/diagnóstico por imagen , Tercer Trimestre del Embarazo , Ultrasonografía , Diagnóstico Prenatal , Vesícula Biliar/diagnóstico por imagen , Feto , Ultrasonografía PrenatalRESUMEN
BACKGROUND: Magnetic resonance cholangiopancreatography (MRCP) is a useful and non-invasive method to diagnose biliary atresia (BA) in term infants, however few studies have investigated its use in preterm infants. This study aimed to evaluate the accuracy of MRCP in the diagnosis of BA in preterm infants with cholestasis. METHODS: Infants aged less than 6 months who received MRCP for cholestasis at a tertiary medical center were enrolled from 2011 to 2020. Demographic and laboratory data were retrospectively obtained. One pediatric radiologist reviewed the MRCP images. The sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and accuracy of MRCP to diagnose BA based on surgical proof or at least 6 months of follow-up were assessed. RESULTS: A total of 80 infants (36 preterm and 44 term) were analyzed. The mean post-chronological age was 1.8 months, and the female-to-male ratio was 0.78. Six (16.7%) preterm and 16 (36.4%) term infants were confirmed to have BA. BA was obscured by a choledochal cyst preoperatively in two term infants. In the preterm infants, the sensitivity, specificity, PPV, NPV, and accuracy of MRCP to diagnose BA were 100%, 77%, 46%, 100%, and 81%, respectively, compared to 81%, 86%, 76%, 89%, and 84% in the term infants. Using MRCP to differentiate BA from other cholestasis in the preterm infants had superior sensitivity (100% vs. 81%) and NPV (100% vs. 89%), and lower specificity (77% vs. 86%) and PPV (46% vs. 76%) than in the term infants. CONCLUSIONS: Negative MRCP findings can be used to exclude BA in preterm infants with cholestasis based on a favorable NPV.
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Atresia Biliar , Colestasis , Lactante , Niño , Recién Nacido , Masculino , Humanos , Femenino , Atresia Biliar/diagnóstico por imagen , Atresia Biliar/patología , Pancreatocolangiografía por Resonancia Magnética/métodos , Estudios Retrospectivos , Recien Nacido Prematuro , Sensibilidad y Especificidad , Colestasis/diagnóstico por imagen , Colestasis/etiologíaRESUMEN
The objective is to explore the correlation between ultrasonic gallbladder length-width ratio (LTWR) and age, and the value of differential diagnosis between biliary atresia (BA) and other hepatic cholestasis. From January 2016 to June 2022, the data of 183 patients with jaundice who underwent abdominal ultrasound and surgical exploration in the Affiliated Hospital of Zunyi Medical University were analyzed retrospectively. The demographic data, liver function, and ultrasonic parameters were recorded and analyzed. There were statistically significant differences between BA group and non-BA group in maximum length, maximum width and LTWR of gallbladder (P < 0.001). In all age groups (I: ≤ 30 days; II: 31-60 days; III: 61-90 days; IV: 91-120 days; V: ≥ 121 days), in which group III (61-90 days) had the highest area under the curve (AUC) of 0.843, and group V (≥121 days) had the lowest AUC of 0.548. The sensitivity, specificity, positive predictive value, negative predictive value and accuracy of gallbladder LTWR > 3.26 for BA in group II (31-60 days) were 78.9%, 75.0%, 75.0%, 78.9% and 76.9%, respectively. The sensitivity, specificity, positive predictive value, negative predictive value and accuracy of gallbladder LTWR > 3.69 for BA in group III (61-90 days) were 76.6%, 84.6%, 92.5%, 59.5% and 78.9%, respectively. Ultrasonography LTWR of gallbladder has certain value in the diagnosis of BA. The diagnostic value of gallbladder LTWR in infants with different ages was quite different, and it was relatively high in infants with 31-90 days.
Asunto(s)
Atresia Biliar , Colestasis , Lactante , Humanos , Atresia Biliar/diagnóstico por imagen , Vesícula Biliar/diagnóstico por imagen , Estudios Retrospectivos , Sensibilidad y Especificidad , Colestasis/diagnóstico , Ultrasonografía , Diagnóstico DiferencialRESUMEN
Biliary atresia (BA) is a rare but devastating cholangiopathy. We report a case series of dysmorphic gallbladders detected during prenatal ultrasound, which were confirmed as BA after birth. We present the prenatal ultrasound findings as well as integral follow-up, with an aim to raise awareness regarding the association between BA and dysmorphic gallbladder. Although this dysmorphic gallbladder is a strong hint for BA, it is also important to search for other related sonographic features, such as the presence of microcysts at the hepatic hilum, dilated right hepatic artery, and seroperitoneum, which may provide more evidence for the diagnosis of BA.
Asunto(s)
Atresia Biliar , Embarazo , Femenino , Humanos , Lactante , Atresia Biliar/diagnóstico por imagen , Vesícula Biliar/diagnóstico por imagen , Abdomen , Hígado , Ultrasonografía PrenatalRESUMEN
OBJECTIVES: To investigate microvascular alterations in the Glisson system of biliary atresia (BA) patients after Kasai portoenterostomy (KP) using three-dimensional (3D) virtual histopathology based on X-ray phase-contrast CT (PCCT). METHODS: Liver explants from BA patients were imaged using PCCT, and 32 subjects were included and divided into two groups: KP (n = 16) and non-KP (n = 16). Combined with histological analysis and 3D visualization technology, 3D virtual histopathological assessment of the biliary, arterial, and portal venous systems was performed. According to loop volume ratio, 3D spatial density, relative surface area, tortuosity, and other parameters, pathological changes of microvasculature in the Glisson system were investigated. RESULTS: In the non-KP group, bile ducts mostly manifested as radial multifurcated hyperplasia and twisted into loops. In the KP group, the bile duct hyperplasia was less, and the loop volume ratio of bile ducts decreased by 13.89%. Simultaneously, the arterial and portal venous systems presented adaptive alterations in response to degrees of bile duct hyperplasia. Compared with the non-KP group, the 3D spatial density of arteries in the KP group decreased by 3.53%, and the relative surface area decreased from 0.088 ± 0.035 to 0.039 ± 0.015 (p < .01). Deformed portal branches gradually recovered after KP, with a 2.93% increase in 3D spatial density and a decrease in tortuosity from 1.17 ± 0.06 to 1.14 ± 0.04 (p < .01) compared to the non-KP group. CONCLUSION: 3D virtual histopathology via PCCT clearly reveals the microvascular structures in the Glisson system of BA patients and provides key insights into the morphological mechanism of microvascular adaptation induced by biliary tract dredging after KP in BA disease. KEY POINTS: ⢠3D virtual histopathology via X-ray phase-contrast computed tomography clearly presented the morphological structures and pathological changes of microvasculature in the Glisson system of biliary atresia patients. ⢠The morphological alterations of microvasculature in the Glisson system followed the competitive occupancy mechanism in the process of biliary atresia.