Caracterización clínica-epidemiológica de quiste de colédoco en la población infantil atendida en el Hospital Materno Infantil

Objetivo: Realizar caracterización clínica-epidemiológica de quiste de colédoco en la población infantil atendida en el Hospital Materno Infantil. Material y Método: Estudio transversal, retrospectivo, descriptivo. La población del estudio consistió en los expedientes con diagnóstico de quiste de colédoco (QC) en el servicio de cirugía pediátrica. Criterios de inclusión: desde el nacimiento hasta los 18 años, operados en este hospital, diagnóstico clínico e imagenológico. Análisis estadístico: base de datos Epi Info.12.0, usando medidas de frecuencia, como la media y varianza. Resultados: Un total de 12 pacientes, la relación entre mujer hombre fue de 3:1, con predominio en mujeres. El grupo etario con mayor frecuencia fue el preescolar con 41,7%. El síntoma más frecuente fue el dolor abdominal con 83,3%; el tipo de QC más frecuente fue el tipo I con 75%, todos fueron resecados y la reconstitución de tránsito más utilizada fue la hepaticoyeyuno anastomosis en Y de Roux (HYYR) con 83,3%. Discusión: El grupo etario, el sexo y el tipo de QC en este estudio, coincide con la literatura citada. El ultrasonido puede ser suficiente para el diagnóstico de QC, la colangiopancreatografia por resonancia magnética permite identificar el subtipo de quiste y planificar la intervención quirúrgica. Conclusión: en nuestro hospital hay poca experiencia en la realización de hepaticoduodeno anastomosis (HD) y ninguna experiencia en la realización de HD y HYYR laparoscópicas, por lo cual, se plantea la necesidad de mejorar las opciones terapéuticas según estándares internaciones. Además, tomando este estudio como base, se deben realizar investigaciones posteriores con mayor complejidad metodológica.

Objective: To perform a clinical-epidemiological characterization of choledochal cyst in the pediatric population attended at the Hospital Materno Infantil. Material and Methods: Cross-sectional, retrospective, descriptive study. The study population consisted of records with a diagnosis of choledochal cyst (CC) in the pediatric surgery service. Inclusion criteria: from birth to 18 years of age, operated in this hospital, clinical and imaging diagnosis. Statistical analysis: Epi Info.12.0 database, using frequency measures, such as mean and variance. Results: A total of 12 patients, female to male ratio was 3:1, with female predominance. The age group with the highest frequency was preschool with 41.7%. The most frequent symptom was abdominal pain with 83.3%, the most frequent type of CC was type I with 75%, all of them were resected and the most frequent reconstruction was Roux-en-Y hepaticojejunostomy (HJ) with 83.3%. Discussion: The age group, sex and type of CC in this study coincides with the literature cited. Ultrasound may be sufficient for the diagnosis of CC, magnetic resonance cholangiopancreatography allows identification of the subtype of cyst and planning of surgical intervention. Conclusion: In our hospital there is little experience in performing hepaticoduodenostomy (HD) and no experience in performing laparoscopic HD and HJ, therefore, there is a need to improve the therapeutic options according to international standards. Furthermore, taking this study as a basis, further research with greater methodological complexity should be carried out.

A Rare Case of Cystic Biliary Atresia

@#Biliary atresia is one of the commonest causes of prolonged jaundice in neonate, whereas cystic biliary atresia is a rare form of biliary atresia with similar presentation, but a slightly different sonographic finding. Being able to differentiate them radiologically is important as the surgical management is different for these two diagnoses. An ultrasound examination was done for a 2-month-old baby girl with prolonged jaundice with the finding of a cyst along the portal triad, which raised the suspicion for cystic biliary atresia. The diagnosis was confirmed with intraoperative cholangiography (IOC) and Kasai procedure was done. The patient was well after the surgery with resolution of jaundice.

Dilataciones quísticas congénitas de las vías biliares (segunda parte). Vías biliares extrahepáticas / Congenital bile duct cystic dilatations (part 2). Extrahepatic bile ducts

RESUMEN Las dilataciones quísticas congénitas de la vía biliar extrahepática son infrecuentes, principalmente en Occidente, y afectan sobre todo a niños pero son poco comunes en adultos. El diagnóstico requiere un alto índice de sospecha y suele llevarse a cabo con estudios colangiográficos de los cuales la CRMN es, en la actualidad, el de mayor utilidad. Es conocido el aumento de malignización de estas dilataciones, por lo que el tratamiento completo de la bolsa quística es obligatorio.

ABSTRACT Congenital dilatation of extrahepatic bile ducts is rare in the Western countries and is more common in children than in adults. The diagnosis requires high level of suspicion and is made by cholangiography tests, among which MRCP is the most useful nowadays. Malignant transformation of these cystic dilatations is well-known; therefore, complete resection of the cystic pouch is mandatory.

Efficacy of percutaneous jejunal drainage in treatment of hepatolithiasis after choledochojejunostomy / 中华肝胆外科杂志

Objective:To study the feasibility and safety of percutaneous jejunal drainage in treatment of hepatolithiasis after choledochojejunostomy.Methods:The clinical data of 24 patients with hepatolithiasis after choledochojejunostomy treated by percutaneous jejunal drainage at the Cholelithiasis Center, Shanghai East Hospital Affiliated to Tongji University from May 2021 to May 2022 were retrospectively analyzed. There were 16 males and 8 females, aged (50.46±10.89) years old. Ultrasound and X ray guided percutaneous jejunography was performed under local anesthesia for patients with hepatolithiasis after choledochojejunostomy. Then the fistula was directly dilated to 16.0Fr, and percutaneous jejunal choledochoscopy was performed 3 days later. The success rate, complication rate and stone removal rate were analysed.Results:Twenty-four patients were treated with percutaneous jejunography, with a success rate of 79.2%(19/24), including 19 patients after anterior colonic cholangiojejunostomy with a success rate of 94.7%(18/19), and 5 patients after retrocolonic cholangiojejunostomy with a success rate of 20.0%(1/5). There was no complication including bleeding, intestinal leakage and bile leakage. In 19 patients with successful percutaneous jejunography, the success rate of fistula dilation was 100%(19/19), and there was no complication. Five patients with failed percutaneous jejunography underwent open choledocholithotomy through the jejunal output-loop, and bile leakage occurred in one patient. Thirteen patients with anastomotic stenosis, 5 with intrahepatic biliary strictures, and 6 with anastomotic and intrahepatic biliary strictures were diagnosed by choledochoscopy and selective cholangiography in these 24 patients. After choledochoscopic electrotomy, cylindrical balloon dilation, stone removal, stenting and other treatments, the stenosis relief rate was 100%(24/24), and the clearance rate of intrahepatic bile duct stones was 91.7%(22/24).Conclusion:Percutaneous jejunal drainage was a feasible, safe and minimally invasive method for treatment of hepatolithiasis after choledochojejunostomy. The procedure was especially suitable for patients with anterior colonic cholangiojejunostomy.

Experience in using needle-grasper assisted single-port laparoscopic hepaticojejunostomy to treat choledochal cysts in children / 中华肝胆外科杂志

Objective:To study the treatment efficacy and safety in using needle-grasper assisted single-port laparoscopic hepaticojejunostomy to treat choledochal cysts in children.Methods:The data of 41 patients with choledochal cysts treated at the Department of Pediatric Surgery, the First Affiliated Hospital of Xiamen University and the Second Hospital of Hebei Medical University from January 2018 to December 2021 were reviewed. There were 8 males and 33 females, aged (2.5±1.9) years. These patients were divided into the needle-grasper assisted single-port laparoscopic hepaticojejunostomy group (needle-grasper group, n=21) and the single-port laparoscopic hepaticojejunostomy group (control group, n=20). Operation time, intestinal function recovery time, gastric tube retention time, abdominal drain indwelling time, postoperative hospital stay, and perioperative complications were compared between the two groups. Results:All 21 children in the needle-grasper group underwent successful surgery without any need to convert to conventional laparoscopic or open surgery. The operation time (156.4±21.2) min was significantly shorter than the control group (218.3±28.6) min ( t=2.95, P=0.017). There were no significant differences in intestinal function recovery time, gastric tube retention time, abdominal drain indwelling time, postoperative hospital stay and perioperative complications between the two groups (all P>0.05). Parents were very satisfied with the cosmetic effect of the invisible scar after surgery. Conclusion:Needle-grasper assisted single-port laparoscopic hepaticojejunostomy was safe, reliable and the operation time was shorter than using a single-port to achieve minimally invasive and scarless surgery.

Efficacy of robot-assisted laparoscopic resection for choledochal cysts / 中华肝胆外科杂志

Objective:To study the clinical results of robot-assisted laparoscopic resection for choledochal cysts.Methods:From October 2020 to June 2022, clinical data on 20 patients who underwent choledochal da Vinci robot-assisted laparoscopic choledochal cyst resection at Fujian Provincial Hospital were retrospectively analyzed. Forty-two children with choledochal cysts were included in this study. There were 16 males and 26 females, aged 25 (range 15.0 to 45.0) months. The operations were carried out by the same team of surgeons. These patients were divided into two groups based on the surgical methods: children who underwent da Vinci robot-assisted laparoscopic surgery were in the robotic group ( n=20) and children who underwent single-port (transumbilical) laparoscopic surgery were in the laparoscopic group ( n=22). The general clinical data, operation time, intraoperative blood loss, fasting time, hospital stay, total medical costs and complications were collectd and analysed. Follow-up was performed in outpatient visits. Results:Both the 2 groups underwent surgery successfully, and no patient was converted to laparotomy. When compared with the laparoscopic group, intraoperative blood loss [(10.65±3.75) vs. (14.86±3.75) ml], indwelling time of abdominal drainage tube [(5.45±2.21) vs. (7.05±1.94) d] and fasting time [(4.00±1.49) vs. (6.05±1.43) d] in the robotic group were significantly less, while the operation time [394(299, 422) vs. 351(269, 380) min] and hospitalization cost [(79 200±4 000) vs. (31 100±3 000) yuan] were significantly increased (all P<0.05). The differences in postoperative complication rates and pain score between the two groups showed no significant differences (all P>0.05). On follow-up, no patients developed complications such as cholangitis, jaundice, intestinal obstruction and cholangiocarcinoma, except for one patient in the laparoscopic group who developed cholangitis after surgery. Conclusion:Robot-assisted laparoscopic choledochal cystectomy was effective and safe in treatment of children with choledochal cysts.

Tratamiento laparoscópico de un quiste de colédoco gigante en un paciente pediátrico / Laparoscopic treatment of a giant choledochal cyst in a pediatric patient

Resumen Introducción: Se define como quiste de colédoco gigante aquel con un diámetro ≥ 10 cm. A pesar de que el abordaje laparoscópico ha sido contraindicado, se presenta el caso de un adolescente con un quiste de colédoco gigante resuelto por laparoscopía. Caso clínico: Paciente de sexo masculino de 14 años con un quiste de colédoco gigante tratado con anastomosis hepático-duodenal laparoscópica. Conclusiones: El tamaño promedio de los quistes de colédoco tratados por laparoscopía es de 40 mm. No se recomienda la resección de quistes gigantes por mínima invasión debido a adherencias y restricción del campo visual. En este caso se realizó un tratamiento laparoscópico de manera exitosa.

Abstract Background: The giant choledochal cyst has a diameter ≥ 10 cm. Although laparoscopy has been contraindicated, we present the case of a teenager with a giant choledochal cyst resolved by laparoscopy. Case report: A 14-year-old male patient with a giant choledochal cyst treated with hepatic-duodenum laparoscopic anastomosis. Conclusions: The average size of bile duct cysts treated by laparoscopy is 40 mm. Giant cysts should not be resected through minimal invasion due to adhesions and a restricted visual field. We report a case of a giant cyst successfully treated by laparoscopy.

Laparoscopic Choledochal Cyst Excision with Hepaticoduo-denostomy: An Experience

Background: Standard treatment for choledochal cyst (CDC) is excision of cyst with biloenteric reconstruction. Most common methods of reconstruction following CDC excision are Roux en y hepaticojejunostomy (RYHJ) and Hepaticoduodenostomy (HD). Although HD has been employed in paediatric population, its use in adult patients has been limited. Aim: The aim of the study was to analyse our experience of patients who underwent laparoscopic excision of CDC and to compare the short term and long term outcomes following HD versus RYHJ as a method of reconstruction in adults. Methods: This is a retrospective analysis of prospectively collected data of 65 patients who underwent laparoscopic cyst excision from January 2016 to March 2021in a single surgical unit at GB Pant Institute of Post graduate Medical Education and Research, New Delhi. Following CDC excision HD was our preferred as method of biliary reconstruction. Patients with restricted duodenal mobility and thin friable duct underwent RYHJ.Short-term outcomes included operative time,blood loss, length of hospital stay and complications such as anastomotic leakage and post operative bleeding.Long-term outcomes included anastomotic stricture formation and need for redo biliary reconstruction. Results: Total 65 patient underwent laparoscopic CDC excision. Mean age was 31.66 ± 12.77years and male: female ratio was 1:5.4. Type I cyst was the most common (60/65=92.3%) with mean size of 2.59 ±0.78cm. Laparoscopic HD was feasible in 87% (57/65) of patients. RYHJ was done in 8 patients. Outcome in laparoscopic HD vs RYHJ group: Mean blood loss was 54.22±8.9ml vs 92±16 mL, mean operative time was 182±41.6 vs 240±52.2 mL, mean hospital stay was 4±2.1 vs 5±3.2 days. There was no difference in anastomotic leak rate (3 vs 1, p=0.42) or post-operative bleeding rate between the two groups.There was no perioperative mortality. Conclusion: Laparoscopic HD was feasible in most of the adult CDC patients with better short term and comparable long term results.It may be used as the preferred biliary reconstruction method during laparoscopic CDC excision in adults as it is technically easier, requires single anastomosis and also accessible for future endoscopic intervention.

Quiste de colédoco Todani IVa. Reporte de caso y revision de la literatura / Choledochal Cyst Todani Iva. Case Report and Literature Review

Resumen: Las dilataciones biliares son malformaciones congénitas raras que pueden involucrar la totalidad de la vía biliar, más comunes durante la primera década de la vida. La mayoría de los casos se presentan con dolor abdominal crónico localizado en cuadrante superior derecho e ictericia. La alta sospecha clínica acompañada de estudios de imagen son indispensables para el diagnóstico. El tratamiento ha evolucionado hasta que en la actualidad la resección completa de los quistes se ha convertido en el manejo de elección, ya que presenta buena respuesta, aunque sin quedar exentos de complicaciones a largo plazo. Presentamos el caso de un paciente del sexo masculino, de 27 años de edad, con historia de dolor abdominal epigástrico de 3 meses de evolución, diagnosticado como quiste de colédoco Todani IVa por resonancia magnética. Se realizó manejo quirúrgico abierto con resección total del quiste y derivación biliodigestiva en Y de Roux sin complicaciones. Fue egresado a los 7 días postoperatorios sin eventualidades. Se realizó revisión de la literatura enfocada en el abordaje y manejo quirúrgico de casos similares.

Abstract: Biliary tree dilations are unusual congenital malformations that may involve the entire bile circulation and they are common during the first life decade. Most cases initiate with jaundice and chronic abdominal pain which localizes in right upper quadrant. High clinical suspicion accompanied by imaging studies are essential for diagnosis. Treatment has evolved until today complete cyst resection has become the treatment of choice, since it presents a good response, although without being free from long-term complications. We present the case of a 27-year-old male patient with a history of 3 months onset epigastric abdominal pain diagnosed as Todani IVa common bile duct choledochal cyst by magnetic resonance imaging. Open surgical management was performed with total resection of the cyst and biliodigestive Roux-en-Y diversion without any complications. He was discharged 7 days post-op. A review of the literature focused on the surgical approach and management of similar cases was carried out.

Guía de práctica clínica en dilatación congénita de las vías biliares en la edad pediátrica / Clinical practice guidelines in congenital dilation of biliary tract in pediatric ages

Las dilataciones congénitas de los conductos biliares, son poco frecuentes y se pueden descubrir en cualquier etapa de la vida, incluido el período prenatal. Es una afección que puede ser asintomáticas, ocasionar síntomas escasos o producir graves complicaciones, razones por las cuales, la actitud actual del especialista se enfoca hacia el diagnóstico temprano y el tratamiento correcto de las diferentes variantes de dilatación congénita de la vía biliar antes que aparezcan las complicaciones mencionadas. El objetivo de publicar esta guía responde a la necesidad de contar con herramientas actualizadas en el manejo de estas afecciones que permitan sistematizar la conducta y mejorar la calidad de la asistencia médica, la docencia y las investigaciones y está aprobada por la Sociedad Cubana de Cirugía Pediátrica. Se realizó una extensa revisión de la literatura científica basada en la mejor evidencia publicada mundialmente sobre el tema. Se presentan el concepto de esta afección, su epidemiología, clasificación, etiología, cuadro clínico, formas de presentación, diagnóstico y conducta a seguir con los diferentes tipos, así como las medidas posoperatorias y de seguimiento del paciente. Si todos los procedimientos se realizan de forma correcta y en el momento oportuno, el pronóstico es favorable en la mayoría de los casos(AU)

Congenital dilation in the biliary duct is not common and can be found in any stage of life, including the prenatal period. It is a condition that can be asymptomatic, causing scarse symptoms or producing severe complications; therefore, the current performance of specialists must be focused towards the early diagnosis and the accurate treatment of the different types of congenital dilation of the biliary tract before the above mentioned complications appear. The aim of publishing this guidelines has to do with the need of having updated tools for the management of these conditions which allow to systematize the behaviours and to improve the quality of medical care, teaching and researches, and also because it is approved by the Cuban Society of Pediatric Surgery. It was made an extensive review of the scientific literature based in the best evidences published worldwide on the topic. It is presented the concept of this condition, its epidemiology, classification, etiology, clinical picture, ways of presentation, diagnosis and behaviour to follow with the different types of it, as well as the patient's post-operative and follow-up measures. If all the procedures are made in a proper way and in the opportune moment, the prognosis is favorable in most of the cases(AU)

Experience of management of abdominal cysts

Benign intra-abdominal cystic masses in children are rare and they have diverse etiopathogenesis, clinical presentation. The present study highlights the experience in the management of benign intra-abdominal cysts pertaining to the diverse etiologies associated with these lesions. The medical records of our hospital between November 2016 to November 2019 were retrospectively reviewed. Patients with cystic abdominal masses were studied with respect to less different clinical presentations, localization of masses, diagnostic tests, surgical aapproaches, histopathological examinations and outcome. Out of the 55 cases, most common lesion was a choledochal cyst. Miscellaneous diagnosis includes an omental cyst, urachal cyst and a pedunculated bile duct cyst. All the cystic lesions of the abdomen need to be considered as close differentials in clinical practice due to the common presentations and similar symptoms produced by these lesions. All the lesions were managed by exploratory laparotomy except two ovarian cysts which were managed with laparoscopic approach.

Coledococele: caso diagnosticado por resonancia magnética / Choledococele: case diagnosed by MRI

Resumen: El coledococele es una dilatación quística del segmento distal del conducto biliar común que sobresale en el lumen duodenal. Estos comprenden menos del 2% de todos los casos reportados de quistes de colédoco (2). Los hallazgos imagenológicos del coledococele y de los quistes de duplicación duodenal son muy similares, ambos presentan además clínica variada e inespecífica, por lo que el diagnóstico definitivo suele ser durante la cirugía. Presentamos un caso de una paciente de 10 años que consulta por dolor abdominal con elevación de transaminasas que, tras estudio con resonancia magnética con colangioresonancia y EDA se diagnostica coledococele.

Abstract: Choledochocele is a cystic dilation of the distal segment of the common bile duct protruding into the duodenal lumen. Is also known as type III choledochal cyst according to Todani's classification. It is usually misdiagnosed as duodenal duplication cyst. Multislice spiral computed tomography and magnetic resonance cholangiopancreatography may be comparable to endoscopic retrograde cholangiography for diagnosis of choledochocele. We present a case of a 10-years-old girl presented with abdominal pain, elevation of transaminases and a magnetic resonance cholangiopancreatography (MRCP) scan that revealed a choledochocele.

Choledochal cysts in children: How to Diagnose and Operate on

OBJECTIVE: To identify the best mode for diagnosing and treating the patients with choledochal cysts. METHODS: A retrospective study was performed with medical records of patients diagnosed with choledochal cysts from January 1994 to December 2017. In all cases, the diagnosis was based on ultrasound examination. All the patients underwent cyst resection and were divided in two groups: bile enteric anastomosis in the high portion of the common hepatic duct or in the dilated lower portion. RESULTS: Eighty-one cases were studied. The age of presentation was 4 y 2 mo ± 4 y 1 mo, and the age for the surgical treatment was 5 y 5 mo ± 4 y 6 mo. In 61 cases, US was the only image examination performed. There were 67 cases of Todani type I (82.7%), 13 cases of type IV (16.0%) and one case of type III (1.2%). Nine patients (29.0%) in the first period and 2 patients (4.0%) in the second period presented with postoperative complications (p=0.016). CONCLUSION: In patients with choledochal cysts, US is the only necessary diagnostic imaging. Performing the bile enteric anastomosis in the lower portion of the common hepatic duct is safer and has a lower risk of complications.

Dynamic contrast enhanced MRI quantitative parameters for evaluation of liver fibrosis in children with congenital choledochal cyst / 中国医学影像技术

Objective: To investigate the value of dynamic contrast enhanced MRI (DCE-MRI) quantitative parameters for evaluation of liver fibrosis caused by pediatric congenital choledochal cyst (CCC). Methods: Totally 33 CCC children with liver fibrosis were included into case group, while 14 children were selected as control group. All children underwent liver DCE-MRI. Quantitative parameters were acquired with Tofts model,including volume transfer constant from blood plasma to extravascular extracellular space (Ktrans), transfer rate constant between extravascular extracellular space and blood plasma (Kep) and volume of extravascular extracellular space (Ve). The differences and correlations of the above parameters among different liver fibrosis stages were analyzed, and AUC of each parameter in diagnosing different grades of liver fibrosis were drawn. Results: Ktrans and Kep were significantly different among fibrosis subgroups (all P0.05) and negative correlation with liver fibrosis staging (r=-0.249, P>0.05). AUC for identifying normal vs fibrosis and mild vs advanced fibrosis by Ktrans and Kep were 0.949, 0.748 and 0.933, 0.731, and the cutoff values were 0.239,.0.186 and 1.814, 1.663, respectively. Conclusion: DCE-MRI perfusion parameters such as Ktrans and Kep are valuable for diagnosing and staging of liver fibrosis caused by CCC in children.

Characteristics of Choledocal Cyst Patients in Bandung, Indonesia : Single Centre Experience

@#Introduction: Choledochal cyst (CC) is a congenital cystic dilation of bile duct. Although considered a benign disorder, but CC has various complications like cholangitis, cholelithiasis, pancreatitis and malignant degeneration. The characteristic of this malformation are still not well documented in our region. We aimed to describe the characteristic of CC patients in Hasan Sadikin Hospital Bandung, Indonesia. Method: The medical records of patients that were diagnosed with CC between 2014 and 2017 were reviewed. We retrospectively collected demographic data, clinical symptoms, diagnostic imaging and surgical data. Results: Fifteen patients were diagnosed as CC, predominantly were girls (12 patients). The mean age was 63,47 months old (range from 7 to 144 months). Jaundic was found in all patients, followed by abdominal pain in 12 patients (80%) and abdominal mass in 9 patients (60%). The laboratory results showed signs of bile obstruction in 5 patients (33,3%). Diagnostic imaging using ultrasonography revealed 5 patients (33,3%) with CC type IV, whereas type I in 6 patients (40%). MRI was performed in 8 patients (53,3%) and CT Scan was performed in 5 patient (33,3%). Eleven patients (73,3%) underwent cyst excision and Roux-en-Y hepaticojejunostomy with liver biopsy. Conclusion: Our data suggested that CC predominantly found in girls, and abdominal pain was the most common symptom. Ultrasonography, MRI, and CT are imaging modalities use to diagnose CC. Surgical treatment should be done includes total cyst excision dan biliodigestive reconstruction.

Clinical characteristics and risk factors of congenital choledochal cysts / 中华围产医学杂志

Objective@#To investigate the clinical characteristics and risk factors of congenital choledochal cysts (CCC).@*Methods@#This retrospective study recruited 52 cases who were antenatally diagnosed with CCC and underwent surgical treatment after birth in Guangdong Women and Children Hospital from January 2013 to August 2018, with complete clinical data. According to the enlargement of cysts during pregnancy, they were divided into two groups: progressive group (≥15 mm, 22) and stable group (<15 mm, 30). Antenatal and postpartum ultrasound and MRI features of the two groups were analyzed. Clinical manifestations and biochemical examination results before and after operation were compared between the two groups. Other data, including amylase level in cyst fluid during operation, cholangiography findings, liver biopsy results, and post-operation follow-up, were also analyzed. Chi-square test, t (t') test, and Pearson correlations tests were performed for data analysis.@*Results@#(1) The average age of the 52 patients at operation was 46(7-822) d. The cysts of all cases were first detected during 19-21 weeks of gestation. The maximum diameter of the cyst in the progressive group was larger than that in the stable group after 34 weeks of pregnancy [31-34 weeks: (31.1±8.4) vs (23.1±6.6) mm, t=3.911; >34 weeks: (36.1±6.8) vs (27.1±7.3) mm, t=4.557; pre-operation: (51.8±18.0) vs (34.0±15.6) mm, t=3.809; all P<0.01]. (2) In the progressive group, the cysts were irregular in shape and enlarged after birth. The common hepatic duct and intrahepatic bile duct were dilated and gradually distended after birth, while the distal end of the common bile duct was narrowed, thus to form a cone-like duct. Deposits could be seen inside the cysts after delivery. Irregular cysts were also presented in the stable group, and five of them had dilatation of common hepatic duct and intrahepatic bile duct after birth. However, no cone-like formation was seen, the distal end of the common bile duct was visible, and deposits in cysts were occasionally found. (3) Twenty-five patients underwent laparotomy, and seven of them showed increased amylase level in cyst fluid including four with 2-5 times above the upper limit of normal value (one in the progressive group and three in the stable group). The other three cases were all in the stable group and their amylase levels in cyst fluid were more than ten times of the upper limit. The level of direct bilirubin in the progressive group was higher than that in the stable group before the operation [18.40(2.50-113.30) vs 8.70(0.00-16.80) μmol/L, u=2.400, P<0.05]. (4) Among the 52 cases, patients with type Ⅰ, Ⅳ and Ⅴ cyst accounted for 71.1% (37/52), 26.9% (14/52) and 2.0% (1/52), respectively. All cases were followed up regularly six months to one year after the operation. Liver function and bilirubin became normal and the growth and development of the babies were similar to those of the same age. (5) Different degrees of liver fibrosis and inflammation were shown in 46(88.5%) cases and more severe in older babies among those who underwent surgery in the progressive group. The time at operation was not associated with the severity of liver fibrosis and inflammation in the stable group. Hepatic fibrosis and inflammation were more serious in the progressive group than in the stable group (fibrosis grading: χ2=14.260, P=0.006; inflammatory activity grading: χ2=9.904, P=0.019).@*Conclusions@#Larger diameter (≥30 mm) in the initial prenatal examination or a significant increase in cystic diameter (≥15 mm) during pregnancy are risk factors for early stenosis or occlusion in the distal end of common bile duct requiring close follow-up after birth. When jaundice or abnormal liver function occur and stool color becomes light, early surgical treatment (one to two months after birth, generally within three months) for CCC is recommended to rule out the anomalous union of the pancreaticobiliary duct and hepatic disorders, as well as the cystic biliary atresia.

Clinical characteristics and risk factors of congenital choledochal cysts / 中华围产医学杂志

Objective To investigate the clinical characteristics and risk factors of congenital choledochal cysts (CCC).Methods This retrospective study recruited 52 cases who were antenatally diagnosed with CCC and underwent surgical treatment after birth in Guangdong Women and Children Hospital from January 2013 to August 2018,with complete clinical data.According to the enlargement of cysts during pregnancy,they were divided into two groups:progressive group (≥ 15 mm,22) and stable group (＜15 mm,30).Antenatal and postpartum ultrasound and MRI features of the two groups were analyzed.Clinical manifestations and biochemical examination results before and after operation were compared between the two groups.Other data,including amylase level in cyst fluid during operation,cholangiography findings,liver biopsy results,and post-operation follow-up,were also analyzed.Chi-square test,t (t1) test,and Pearson correlations tests were performed for data analysis.Results (1) The average age of the 52 patients at operation was 46(7-822) d.The cysts of all cases were first detected during 19-21 weeks of gestation.The maximum diameter of the cyst in the progressive group was larger than that in the stable group after 34 weeks of pregnancy [31-34 weeks:(31.1 ±8.4) vs (23.1 ± 6.6) mm,t=3.911;＞34 weeks:(36.1 ± 6.8) vs (27.1 ± 7.3) mm,t=4.557;pre-operation:(51.8± 18.0) vs (34.0± 15.6) mm,t=3.809;all P＜0.01].(2) In the progressive group,the cysts were irregular in shape and enlarged after birth.The common hepatic duct and intrahepatic bile duct were dilated and gradually distended after birth,while the distal end of the common bile duct was narrowed,thus to form a cone-like duct.Deposits could be seen inside the cysts after delivery.Irregular cysts were also presented in the stable group,and five of them had dilatation of common hepatic duct and intrahepatic bile duct after birth.However,no cone-like formation was seen,the distal end of the common bile duct was visible,and deposits in cysts were occasionally found.(3) Twenty-five patients underwent laparotomy,and seven of them showed increased amylase level in cyst fluid including four with 2-5 times above the upper limit of normal value (one in the progressive group and three in the stable group).The other three cases were all in the stable group and their amylase levels in cyst fluid were more than ten times of the upper limit.The level of direct bilirubin in the progressive group was higher than that in the stable group before the operation [18.40(2.50-113.30) vs 8.70(0.00-16.80) μmol/L,u=2.400,P＜0.05].(4) Among the 52 cases,patients with type Ⅰ,Ⅳ and Ⅴ cyst accounted for 71.1％ (37/52),26.9％ (14/52) and 2.0％ (1/52),respectively.All cases were followed up regularly six months to one year after the operation.Liver function and bilirubin became normal and the growth and development of the babies were similar to those of the same age.(5) Different degrees of liver fibrosis and inflammation were shown in 46(88.5％) cases and more severe in older babies among those who underwent surgery in the progressive group.The time at operation was not associated with the severity of liver fibrosis and inflammation in the stable group.Hepatic fibrosis and inflammation were more serious in the progressive group than in the stable group (fibrosis grading:x2=14.260,P=0.006;inflammatory activity grading:x2=9.904,P=0.019).Conclusions Larger diameter (≥ 30 mm) in the initial prenatal examination or a significant increase in cystic diameter (≥ 15 mm) during pregnancy are risk factors for early stenosis or occlusion in the distal end of common bile duct requiring close follow-up after birth.When jaundice or abnormal liver function occur and stool color becomes light,early surgical treatment (one to two months after birth,generally within three months) for CCC is recommended to rule out the anomalous union of the pancreaticobiliary duct and hepatic disorders,as well as the cystic biliary atresia.

Spontaneus Perforation of the Common Bile Duct in a 3 Years Old Boy

@#Peritonitis caused by biliary tract perforation is unsual. After other causes, such as trauma, biliary tract stone, cyst of choledocal duct, can be rule out, we should reconsider leakage or rupture of biliary tract. We report a 3 years old boy was administered to emergency room with abdominal distended, vomiting and diarrhea, low-grade fever, and diffuse abdominal pain. There’s no history of jaundice and abdominal pain before, neither trauma. Sign of peritonitis were found. The patient underwent laparotomy, perforation at common bile duct was found without any other disease. Intra abdominal drain was placed near the leak and primary repair was done with tube drain inside the duct. The pastient discharged after 8 days hospitalized uneventful.