Association of TP53 germline variant and choledochal cyst among clinically diagnosed Filipino pediatric patients

@#Background and Objective. Choledochal cysts (CC) are rare congenital, cystic dilations of the biliary tree occurring predominantly in Asian populations and in females. Patients are usually children presenting with any of the following: abdominal pain, palpable abdominal mass, and jaundice. Its congenital nature hints at a potential genetic cause. A possible causal gene is TP53, a tumor suppressor with a germline variant called rs201753350 (c.91G>A) that changed from a G allele to an A allele, decreasing the cell proliferation suppressing activity of its functional protein. Currently, there is no information on the TP53 rs201753350 germline variant available for the Filipino population. This study determined the prevalence of rs201753350 and the association between the functional G allele, the rs201753350 germline variant A allele, and the occurrence of CCs in Filipino pediatric patients in a tertiary government hospital. Methods. Genomic DNA was extracted from blood samples of pediatric patients clinically diagnosed with CC. Controls were DNA samples collected from a previous study. The samples underwent PCR, electrophoresis, and sequencing. Results. A total of 109 participants (22 cases and 87 controls) were included in the study. The A allele (22.94%) occurs at a lower frequency than the G allele (77.06%) among both cases and controls. More individuals have a homozygous G/G genotype (54.13%) than a heterozygous A/G genotype (45.87%) while the homozygous A/A genotype was not observed. The estimated risk of choledochal cyst occurrence is significantly lower in individuals with the A allele (PR: 0.08, 95% CI: 0.01 – 0.55) and the A/G genotype (PR: 0.06, 95% CI: 0.01 – 0.40). Conclusion. There is no significant evidence to suggest an association between the TP53 rs201753350 germline variant and the occurrence of choledochal cysts in Filipinos. It is recommended that other mutations within and beyond the TP53 gene be investigated for possible associations with choledochal cyst occurrence.

Tratamiento quirúrgico inmediato en niños con perforación espontánea de la vía biliar. Experiencias de 16 años

Introducción: La perforación espontánea de los conductos biliares es una rara enfermedad caracterizada por una disrupción no traumática de la vía biliar en pacientes aparentemente sanos. Se trata de una grave situación potencialmente letal, pero diagnosticada y tratada correctamente tiene un pronóstico excelente. Objetivo: Caracterizar los principales elementos clínico-quirúrgicos expresados en una serie de 5 pacientes operados en un servicio de referencia nacional. Presentación de casos: Se presenta la experiencia con una serie de casos en 16 años en una sola institución. La afección se observó en niñas recién nacidas y lactantes con una edad media de 4 meses, y se presentó desde la clínica como una colestasis acompañada de distensión abdominal, ascitis biliar, acolia, y signos de irritación peritoneal. El 80 % de los casos se intervinieron en el hospital "William Soler", y en un caso se ejecutó el procedimiento después de una laparotomía por una posible apendicitis aguda, en otro hospital. El diagnóstico se basó en el cuadro clínico descrito, la ecografía abdominal, la paracentesis con medición del índice bilirrubina líquido ascítico/bilirrubina sérica, y la colangiografía intraoperatoria. La cirugía definitiva se realizó inmediatamente, y consistió en: lavado peritoneal, colangiografía diagnóstica, reparación hepaticoyeyunostomía en Y de Roux y colocación de drenaje. Conclusiones: El tratamiento realizado resulta eficaz y seguro en todos los casos, con una excelente evolución, sin complicaciones importantes y con una total supervivencia posoperatoria. La colangiografía intraoperatoria permitió identificar el sitio de la perforación y diagnosticar malformaciones asociadas como dilataciones biliares congénitas y anomalías de la unión bilio-pancreática.

Introduction: Spontaneous bile duct perforation is a rare condition characterized by non-traumatic disruption of the bile duct in apparently healthy patients. It is a serious potentially lethal situation, but correctly diagnosed and treated its prognosis is excellent. Objetive: To characterize the main clinical-surgical elements expressed in a series of 5 patients operated in a national referral service. Case presentation: The experience with a series of cases during a period of 16 years in a single institution is presented. The condition was observed mainly in newborn girls and infants with an average age of 4 months, and presented clinically as cholestasis accompanied by abdominal distension, biliary ascites, acholia, and signs of peritoneal irritation. 80% of the cases were operated primarily in the "William Soler" hospital, and in one case the procedure was performed after a laparotomy for a possible acute appendicitis, in another hospital. The diagnosis was based on the clinical picture described, abdominal ultrasound, paracentesis with measurement of the ascitic liquid bilirubin/serum bilirubin index, and intraoperative cholangiography. Definitive surgery was performed immediately and consisted of: peritoneal lavage, diagnostic cholangiography, Roux-en-Y liver and jejunostomy repair and drainage placement. Conclusions: The treatment performed was effective and safe in all cases, with an excellent evolution, no major complications and total postoperative survival. Intraoperative cholangiography made it possible to identify the site of perforation and to diagnose associated malformations such as congenital biliary dilatations and anomalies of the biliary-pancreatic junction.

A case of X-linked hyper IgM syndrome with a novel CD 40LG mutation / 中华实用儿科临床杂志

A retrospective analysis was performed on the clinical data of a child with X-linked hyper IgM syndrome (XHIGM) with cholangiectasis as a major manifestation in Children′s Hospital of Fudan University in March 2017.The patient was a 4-year-old boy who was admitted to the hospital due to repeated diarrhea for half a year and yellow skin for 5 days.No abnormalities were found in his fetal period and birth history; The patient had 2 severe pneumonias and suppurative infection of the left axillary lymph node in infancy.Physical examination revealed delayed physical development, severe malnutrition, moderately stained yellow, lymphadenopathy and hepatomegaly.Laboratory examinations showed elevated leukocyte, eosinophils and C-reactive protein, low hemoglobin and albumin, high gamma-glutamyl transpeptidase (GGT), low IgG and normal IgM.Imaging examination revealed diffuse expansion of intrahepatic and extrahepatic bile ducts.Hepatic pathology showed hyperplasia in the bile canaliculus and some fibrous tissues around the large bile ducts.High-throughput sequencing identified a pathogenic mutation in the XHIGM gene CD 40LG (exon5 c. 506A>G, p.Y169C), with his mother as a carrier.After admission, the patient was given anti-infection, diet adjustment, albumin, intravenous immunoglobulin and ursodeoxycholic acid.The patient was discharged after the improvement in his condition.This case suggested that in addition to the common infection characteristics, XHIGM can also be manifested as diffuse intrahepatic, extrahepatic cholangiectasis and significantly elevated eosinophil.c.506A>G mutation in CD 40LG was the pathogenic mutation of this disease.

Pancreatic cyst in a cat / Cisto pancreático em um gato

ABSTRACT: Pancreatic cysts are rare in both humans and animals. They are defined as an enclosed structure externally surrounded by a capsule, internally coated with a cuboidal epithelium and filled with liquid or semi-solid content. This case described the clinical and pathological characteristics of a pancreatic cyst in a feline. A mixed breed cat with a history of recurrent vomiting was attended. Physical examination revealed pain on abdominal palpation. Abdominal ultrasonography showed a cystic, anechoic structure with well-defined edges located in the left cranial abdomen and in close contact with the duodenum and pancreas. Partial pancreatectomy was performed. Microscopically, the structure was surrounded by fibrous material, coated with cuboidal to columnar epithelium, and containing eosinophilic material. Although, pancreatic cyst is rare in animals, they should be included in the differential diagnosis of causes of vomiting in young cats.

RESUMO: Os cistos epiteliais pancreáticos são raros em humanos e animais, e são definidos como estruturas encapsuladas rodeadas por epitélio cuboidal, preenchido por substância líquida. O objetivo desse estudo foi descrever as características clínico-patológicas de um cisto pancreático verdadeiro em um felino. Foi atendido uma gata sem raça definida com histórico de vômitos recorrentes. No exame físico o animal apresentou dor à palpação abdominal. Na ultrassonografia abdominal, evidenciou-se a presença de uma estrutura cística, anecóica e com bordos bem definidos localizado no abdome cranial esquerdo, em contato com o duodeno e o pâncreas. Foi realizada pancreatectomia parcial. No exame microscópico observou-se estrutura encapsulada, circundado por material fibroso, revestido por epitélio cuboidal a colunar, contendo material eosinofílico. Embora os cistos pancreáticos verdadeiros sejam raros nos animais, eles devem ser incluídos no diagnóstico diferencial de doenças que cursam com vômitos nos gatos.

Diagnosis and treatment of biliary pancreatic duct dilatation / 中华消化外科杂志

Objective@#To summarize the diagnosis and treatment of biliary pancreatic duct dilatation.@*Methods@#The retrospective and descriptive study was conducted. The clinical data of 22 patients with biliary pancreatic duct dilatation who were admitted to Renji Hospital of Shanghai Jiaotong University School of Medicine between October 2013 to September 2017 were collected. There were 6 males and 16 females, aged from 33 to 82 years, with an average age of 66 years. Surgical exploration was decided according to clinical symptoms, results of laboratory test and imaging examinations. For patients with space occupying lesions, surgical procedure was selected based on results of pathological examination. Patients without surgical exploration or space occupying lesions were allocated into follow-up. Observation indicators: (1) surgical exploration; (2) relationship of clinical symptoms and preoperative examinations with surgical exploration positive for space occupying lesions; (3) surgical treatment; (4) follow-up. Follow-up using outpatient examination was performed on patients up to October 2018. Follow-up was performed on patients with positive surgical exploration to detect postoperative complications.For patients with positive results of imaging examinations, no jaundice, normal laboratory indicators or mild abnormality, liver function, tumor markers and B-ultrasound were re-examined each month, and computed tomography (CT) and magnetic resonance imaging (MRI) was performed once every 3 months. Surgical exploration was performed when total bilirubin (TBil) or tumor markers showed a progressive increase. Follow-up was performed on patients with negative results of imaging examination, jaundice, and mildly elevated CA19-9. TBil and CA19-9 were re-examined monthly, and if they were progressively elevated, patients were transferred to surgical exploration. For patients with negative results of imaging examination, no symptoms, and negative laboratory test, liver function, tumor markers, and B-ultrasound were re-examined once every 3 months, and enhanced CT and MRI were re-examined once every 6 months within one year. Follow-up was performed once every 6 months during the second year, and once a year after two years. Measurement data with normal distribution were represented as Mean±SD, and comparison between groups was analyzed using the t test. Count data were descibed as absolute numbers, and they were analyzed using the chi-square test under R×C chart or Fisher exact probability.@*Results@#(1) Surgical exploration: of 22 patients, 11 underwent surgical exploration, and 11 underwent follow-up. Of the 11 patients with surgical exploration, 4 were positive for space occupying lesions including 1 of false negative, and 7 were negative for space occupying lesions. (2) Relationship of clinical symptoms and preoperative examinations with surgical exploration positive for space occupying lesions. ① Relationship of clinical symptoms and laboratory test with surgical exploration positive for space occupying lesions: juandice was significantly associated with surgical exploration positive for space occupying lesions (P<0.05), and elevated TBil and DBil were significantly associated with surgical exploration positive for space occupying lesions (χ2=0, 0, P<0.05), with a sensitivity of 75.0% and specificity of 100.0%. ② Relationship between imaging examination and surgical exploration positive for space occupying lesions: results of CT, MRI, endoscopic retrograde cholangio-pancreatography, endoscopic ultrasonography, PET-CT, and combined imaging examinations had no significant association with surgical exploration positive for space occupying lesions (χ2=0, 0.77, 0, 0, 1.00, 0, 0, 0, 0, P>0.05). PET-CT had no significant association with surgical exploration positive for space occupying lesions (P>0.05). ③ Relationship of imaging examination and laboratory test with surgical exploration positive for space occupying lesions: positive imaging examination combined with elevated TBil and CA19-9 was significantly associated with surgical exploration positive for space occupying lesions (P<0.05), with a sensitivity of 50.0% and specificity of 100.0%. ④ Relationship of preoperative diameters of biliary ducts and pancreatic ducts with surgical exploration positive for space occupying lesions: of 22 patients, the diameters of biliary ducts and pancreatic ducts were (13.8±4.3)mm and (4.6±1.5)mm for patients with positive surgical exploration, (13.0±2.8)mm and (3.5±0.5)mm for patients with negative surgical exploration, (11.6±2.4)mm and (3.2±0.4)mm for patients with follow-up, respectively, showing no significant difference between them (t=0.22, 0.36, P>0.05). (3) Surgical treatment: 9 of 11 patients with surgical exploration followed the standard procedure. Of the 9 patients, 4 were found space-occupying lesions at the choledocho-pancreatico-duodenal junction (3 undergoing pancreaticoduodenectomy and 1 undergoing duodenal papilla partial resection), 5 with negative exploration underwent common bile duct incision and T-tube drainage (one patient was unable to pinch the T-tube one month after operation and detected obstruction at the lower end of the bile duct by radiography, and was confirmed pancreatic head cancer by reoperation 3 months after the first operation). Two patients didn′t follow the exploratory procedure, and underwent the child operation only based on the preoperative imaging findings, without intraoperative pathological examination. Postoperative pathological examination showed chronic ampulla and chronic pancreatitis, respectively. (4) Follow-up: 22 patients were followed up for 12-60 months, with a median follow-up time of 36 months. Two of 11 patients with surgical exploration had postoperative gastroplegia, 1 had bile leakage, 1 had incisional infection, and they were improved after symptomatic treatment. Four patients undergoing surgeries for positive exploration had no recurrence during follow-up. Of 5 patients with negative exploration undergoing common bile duct incision and T-tube drainage, 1 was confirmed pancreatic head cancer and underwent pancreaticoduodenectomy, 4 were removed T-tube after by T-tube cholangiography at 2 months after surgery. During the follow-up, no positive signs showed in laboratory test or imaging examination. No recurrence occurred in the two patients undergoing pancreaticoduodenectomy. Of 11 patients with follow-up, 10 had abdominal pain before surgery, including 3 with pain during follow-up and 7 with symptoms disappeared. There was no abnormalities in the laboratory test.@*Conclusions@#The positive imaging examinations combined with jaundice and elevated CA19-9 is an absolute indication for surgical exploration in patients with biliary duct dilatation. Those patients who do not meet this criteria should be distributed into the follow-up. If no positive pathological results were obtained during the operation, the surgery should be terminated and the patients should be transferred into follow-up. The reckless biliary anastomosis or biliary stents placement is opposed.

Diagnosis and treatment of biliary pancreatic duct dilatation / 中华消化外科杂志

Objective To summarize the diagnosis and treatment of biliary pancreatic duct dilatation.Methods The retrospective and descriptive study was conducted.The clinical data of 22 patients with biliary pancreatic duct dilatation who were admitted to Renji Hospital of Shanghai Jiaotong University School of Medicine between October 2013 to September 2017 were collected.There were 6 males and 16 females,aged from 33 to 82years,with an average age of 66 years.Surgical exploration was decided according to clinical symptoms,results of laboratory test and imaging examinations.For patients with space occupying lesions,surgical procedure was selected based on results of pathological examination.Patients without surgical exploration or space occupying lesions were allocated into follow-up.Observation indicators:(1) surgical exploration;(2) relationship of clinical symptoms and preoperative examinations with surgical exploration positive for space occupying lesions;(3) surgical treatment;(4) follow-up.Follow-up using outpatient examination was performed on patients up to October 2018.Follow-up was performed on patients with positive surgical exploration to detect postoperative complications.For patients with positive results of imaging examinations,no jaundice,normal laboratory indicators or mild abnormality,liver function,tumor markers and B-ultrasound were re-examined each month,and computed tomography (CT) and magnetic resonance imaging (MRI) was performed once every 3 months.Surgical exploration was performed when total bilirubin (TBil) or tumor markers showed a progressive increase.Follow-up was performed on patients with negative results of imaging examination,jaundice,and mildly elevated CA19-9.TBil and CA19-9 were re-examined monthly,and if they were progressively elevated,patients were transferred to surgical exploration.For patients with negative results of imaging examination,no symptoms,and negative laboratory test,liver function,tumor markers,and B-ultrasound were re-examined once every 3 months,and enhanced CT and MRI were re-examined once every 6 months within one year.Follow-up was performed once every 6 months during the second year,and once a year after two years.Measurement data with normal distribution were represented as Mean±SD,and comparison between groups was analyzed using the t test.Count data were descibed as absolute numbers,and they were analyzed using the chi-square test under R×C chart or Fisher exact probability.Results (1) Surgical exploration:of 22 patients,11 underwent surgical exploration,and 11 underwent followup.Of the 11 patients with surgical exploration,4 were positive for space occupying lesions including 1 of false negative,and 7 were negative for space occupying lesions.(2) Relationship of clinical symptoms and preoperative examinations with surgical exploration positive for space occupying lesions.① Relationship of clinical symptoms and laboratory test with surgical exploration positive for space occupying lesions:juandice was significantly associated with surgical exploration positive for space occupying lesions (P＜0.05),and elevated TBil and DBil were significantly associated with surgical exploration positive for space occupying lesions (x2 =0,0,P＜0.05),with a sensitivity of 75.0％ and specificity of 100.0％.(② Relationship between imaging examination and surgical exploration positive for space occupying lesions:results of CT,MRI,endoscopic retrograde cholangiopancreatography,endoscopic ultrasonography,PET-CT,and combined imaging examinations had no significant association with surgical exploration positive for space occupying lesions (x2 =0,0.77,0,0,1.00,0,0,0,0,P＞0.05).PET-CT had no significant association with surgical exploration positive for space occupying lesions (P＞0.05).③ Relationship of imaging examination and laboratory test with surgical exploration positive for space occupying lesions:positive imaging examination combined with elevated TBil and CA19-9 was significantly associated with surgical exploration positive for space occupying lesions (P＜0.05),with a sensitivity of 50.0％ and specificity of 100.0％.④ Relationship of preoperative diameters of biliary ducts and pancreatic ducts with surgical exploration positive for space occupying lesions:of 22 patients,the diameters of biliary ducts and pancreatic ducts were (13.8±4.3)mm and (4.6±1.5)mm for patients with positive surgical exploration,(13.0±2.8)mm and (3.5±0.5) mm for patients with negative surgical exploration,(11.6±2.4) mm and (3.2±0.4) mm for patients with follow-up,respectively,showing no significant difference between them (t =0.22,0.36,P＞0.05).(3) Surgical treatment:9 of 11 patients with surgical exploration followed the standard procedure.Of the 9 patients,4 were found space-occupying lesions at the choledocho-pancreatico-duodenal junction (3 undergoing pancreaticoduodenectomy and 1 undergoing duodenal papilla partial resection),5 with negative exploration underwent common bile duct incision and T-tube drainage (one patient was unable to pinch the T-tube one month after operation and detected obstruction at the lower end of the bile duct by radiography,and was confirmed pancreatic head cancer by reoperation 3 months after the first operation).Two patients didn't follow the exploratory procedure,and underwent the child operation only based on the preoperative imaging findings,without intraoperative pathological examination.Postoperative pathological examination showed chronic ampulla and chronic pancreatitis,respectively.(4) Follow-up:22 patients were followed up for 12-60 months,with a median followup time of 36 months.Two of 11 patients with surgical exploration had postoperative gastroplegia,1 had bile leakage,1 had incisional infection,and they were improved after symptomatic treatment.Four patients undergoing surgeries for positive exploration had no recurrence during follow-up.Of 5 patients with negative exploration undergoing common bile duct incision and T-tube drainage,1 was confirmed pancreatic head cancer and underwent pancreaticoduodenectomy,4 were removed T-tube after by T-tube cholangiography at 2 months after surgery.During the follow-up,no positive signs showed in laboratory test or imaging examination.No recurrence occurred in the two patients undergoing pancreaticoduodenectomy.Of 11 patients with follow-up,10 had abdominal pain before surgery,including 3 with pain during follow-up and 7 with symptoms disappeared.There was no abnormalities in the laboratory test.Conclusions The positive imaging examinations combined with jaundice and elevated CA19-9 is an absolute indication for surgical exploration in patients with biliary duct dilatation.Those patients who do not meet this criteria should be distributed into the follow-up.If no positive pathological results were obtained during the operation,the surgery should be terminated and the patients should be transferred into follow-up.The reckless biliary anastomosis or biliary stents placement is opposed.

Diagnosis and treatment of congenital submandibular duct dilatation / 华西口腔医学杂志

OBJECTIVE@#To study the clinical features and treatments of congenital submandibular duct dilatation.@*METHODS@#Seven children with congenital submandibular duct dilatation from January 2008 to March 2018 were included in this study, whose average age was 5 months and 22 days. The clinical manifestations are unilateral swelling of the mouth floor. All seven children underwent sublingual gland resection, submandibular gland dilatation catheter resection, and catheter reroute under general anesthesia. Intraoperatively, the orifice of the submandibular gland was constricted and part of the catheter was dilated.@*RESULTS@#All seven patients had good healing without swelling or cyst formation.@*CONCLUSIONS@#Congenital submandibular duct dilatation occurs at a young age. Early diagnosis and treatment can help prevent further expansion of the catheter and avoid gland atrophy, feeding difficulty, and breathing obstruction. Simultaneous excision of the sublingual gland can avoid the formation of postoperative sublingual cyst.

Longitudinal pancreaticojejunostomy for paediatric chronic pancreatitis with dilated pancreatic ducts / 中华肝胆外科杂志

Objective To analyze the results of longitudinal pancreaticojejunostomy in treatment of paediatric chronic pancreatitis with dilated pancreatic ducts.Methods A retrospective study was carried out on 13 patients with paediatric chronic pancreatitis complicated with dilated pancreatic ducts treated with longitudinal pancreaticojejunostomy in Children's Hospital of Chongqing Medical University from December 2011 to January 2017.The perioperative morbidity and mortality rates,long-term treatment results and postoperative growth of these children were analyzed.Results The 13 patients all underwent successful surgery.In 11 patients,the serum and urine amylase levels returned to normal after 8 days of operation,and the abdominal pain disappeared completely.In 1 patient,the abdominal pain gradually disappeared in 1 year,and the serum and urine amylase levels gradually returned to normal.This patient gained weight well.In the remaining patient who had severe pancreatic atrophy,the patient took high-fat diets before and after surgery,and drank alcohol occasionally.The patient developed repeated attacks of abdominal pain with occasional increase in serum and urine amylase levels and had poor weight gain.There were no complications such as postoperative bleeding,pancreatic leakage and intestinal obstruction in this study.The body weight and growth rates of the whole group of patients before and 1 year after surgery were different.Conclusion Longitudinal pancreaticojejunostomy for paediatric chronic pancreatitis complicated with dilated pancreatic ducts was safe and effective in alleviating symptoms,improving quality of life,and resulted in normal growth of these children.

High-frequency ultrasound imaging-guided wedge resection for the treatment of paraurethral duct dilatation following gonococcal paraurethral duct infection in 11 male patients / 中华皮肤科杂志

Objective To evaluate therapeutic effects of wedge resection on male paraurethral duct dilatation following gonococcal paraurethral duct infection. Methods With the aid of high-frequency ultrasound images, 11 male patients with paraurethral duct dilatation following gonococcal paraurethral duct infection were treated with wedge resection. The data were collected, including the surgical duration, amount of bleeding during the surgery, period of wound healing and complications. If the ostium beside the external urethral orifice disappeared within 4 weeks after the surgery, there was no discharge from the ostium with pressure, and no tubular echoic area was observed by high-frequency ultrasound imaging, the patient was considered to be recovered. If none of the above three conditions could be met, the surgical treatment was considered to be ineffective. Results The average surgical duration was 19.19 ± 2.71 minutes(range, 14-23 minutes), the average amount of bleeding during the surgery was 11.09 ± 2.07 ml (range, 8-14 ml), and the average period of wound healing was 14.91 ± 1.45 days(range, 13-17 days). Of the 11 patients, 10 were cured, and 1 showed no response. No complications were observed in any of the 11 patients, and no defects formed in the glans penis. Conclusion Wedge resection is a kind of effective therapy for paraurethral duct dilatation following gonococcal paraurethral duct infection in males.

Clinical value of endoscopic ultrasonography in diagnosis and treatment of common bile duct dilatation / 中华消化内镜杂志

Objective To investigate the value of endoscopic ultrasonography(EUS)in the diagnosis and treatment of undetermined etiology of common bile duct (CBD) dilatation. Methods Patients, who were referred for dilated CBD but unable to identify the cause by imaging, underwent EUS for the diagnosis of etiology in Endoscopy Center of Zhongshan Hospital Affiliated to Fudan University from December 2015 to December 2016. The therapy was on the basis of diagnosis of EUS. Final diagnoses were determined by surgical pathology or follow-up for at least 3 months. Results A total of 76 patients were included in the study. The sensitivity, specificity and accuracy of EUS for patients with choledocholithiasis,patients with ampullary tumor, and patients with inflammatory stenosis were 100.0%(7/7), 100.0%(69/69), 100.0%(76/76), and 88.0%(22/25), 92.2%(47/51), 90.8%(69/76),and 90.9%(40/44), 93.8%(29/32), 90.8%(69/76), respectively. Accuracy of EUS for etiological diagnosis of CBD dilatation was 90.8%(69/76). The sensitivity,specificity and accuracy of EUS combined with tumor markers for patients with malignant CBD dilatation were 96.0%(24/25), 96.1%(49/51),and 96.1%(73/76), respectively. Conclusion EUS is an effective method for the etiological diagnosis of CBD dilatation and has guiding significance for the treatment. EUS combined with tumor markers may benefit differential diagnosis of benign and malignant CBD dilatation.

Analysis on the incidence of bile duct dilatation after open and laparoscopic cholecystectomy / 中国现代普通外科进展

Objective:To investigate the open method(OC) and laparoscopic cholecystectomy (LC) after bile duct dilation incidence of contrast.Methods:collected from 2006 December to 2014 December in the department of hepatobiliary surgery requires 412 patients underwent cholecystectomypatients hospitalized with cholecystolithiasis,chronic cholecystitis,gallbladderpolyps,were randomly divided into LC group and OC group,LC group of 207 cases,205 cases in OC group,and were respectively treated with LC and OC.Start regular follow-up after a month,the comparison of 2 surgical operation time,blood loss and postoperative application of antibiotics time,anus exhaust time,eating time and hospitalization time.And to observe the two groups the incidence of patients after bile duct dilatation in follow-up after operation.Results:207 cases of LC patients were 89 patients had bile duct dilation,205 cases of OC patients were 41 patients had bile ductdilation,statistically significant differences between the two groups (P＜0.05).In group LC,the diameter of common bile duct in operation half months began to increase,the increase continued until 3 months after operation.After 3 months of little change in the diameter of common bile duct.The averagepreoperative bile duct diameter is 5.3 mm,after 6.1 mm,there was significant difference(P＜0.01).Conclusion:LC is a safe and reliable operation method,Identifying cause dilatation of common bile duct after LC operation and attention as soon as possible to give the corresponding prevention and treatment,further can reduce complications occur.

Diagnosis and treatment of type Ⅰ congenital biliary dilatation / 中华肝胆外科杂志

Type Ⅰ congenital biliary dilatation is a congenital biliary duct defect disease.It is also called congenital choledochocele cyst (CCC).Endoscopic ultrasonography (EUS),magnetic resonance cholangiopacreatography (MRCP) and endoscopic retrograde cholangiopancreatography (ERCP) are very important for the diagnosis.Operation is generally regaded as the best choose.It is recommended that cholecystectomy + choledochal cyst excision + hepatic duct jejunum Roux-Y anastomosis is the chief therapeutic method.This article reviews the pathogenesis,classifications,diagnosis and treatment of the disease.

A Case of Pancreatic Neuroendocrine Tumor Mimicked Intraductal Papillary Mucinous Neoplasm / 대한췌담도학회지

Neuroendocrine tumors of the pancreas are exremely rare tumors, but recent imaging examination advances, diagnostic frequency is also increasing. However, there is difficulty of diagnosis of pancreatic serotonin producing neuroendocrine tumors, because tumors grow slowly and clinical symptoms are not significant. A 60-year-old male patient with pancreatic duct dilatation progresses gradually during the seven years without obstructing lesion in imaging studies, we suspected the mass as intraductal papillary mucinous neoplasm. However, we diagnosed his case as neuroendocrine tumor after surgery and report here with literature review.

A Small Pancreatic Neuroendocrine Tumor with Marked Pancreatic Duct Dilatation and Parenchymal Atrophy / 대한내과학회지

Pancreatic ductal adenocarcinomas often cause marked pancreatic duct dilatation and associated parenchymal atrophy. We present the case of a small pancreatic neuroendocrine tumor with upstream pancreatic duct dilatation and severe parenchymal atrophy. A small enhancing tumor was observed at the head of the pancreas on computed tomography (CT). Endoscopic ultrasonography-guided fine-needle aspiration was negative for malignancy. We performed a pylorus-preserving pancreatoduodenectomy since we could not exclude the presence of pancreatic ductal adenocarcinoma. The pathological and immunohistochemical examination revealed a serotonin-positive neuroendocrine tumor, measured 1.0 x 0.5 cm. The pathological specimen was remarkable for the marked stromal fibrosis in the area of the tumor, which resulted in narrowing of the main pancreatic duct. Here, we report a rare small pancreatic neuroendocrine tumor, the CT image of which resembled that of pancreatic ductal adenocarcinoma, in which the expression of serotonin and associated fibrosis might be a possible mechanism for the marked main pancreatic duct dilatation.

MRI Findings of Long-term Survivals after Kasai Portoenterostomy

The purpose of this study is to analyse clinical impact of specific MRI findings in liver in patients of long-term survivors after Kasai portoenterostomy (KPE). Twenty-eight patients who were underwent KPE were followed up more than 5 years. Macro-regenerative nodule (MRN) and beaded-duct dilatation (BDD) were considered as important findings in liver MRI. The association between these findings in MRI and clinical indicator, serum bilirubin level and history of cholangitis were evaluated. Sixteen patients (57.1%) were shown MRN in liver MRI. There were 14 patients(50%) whose MRI showed BDD. Serum total and direct bilirubin were 3.6mg/dL and 1.8mg/dL respectively in positive MRN group whereas 1.4mg/dL and 0.7mg/dL in negative MRN group (p 0.427). Serum total and direct bilirubin level were 4.2mg/dL and 2.1mg/dL in patients with BDD negative group compare to 1.1mg/dL and 0.5mg/dL in BDD positive group (p 0.281). The odds ratio to have cholangitis in the patient with MRN was 2.3 and 0.53 in patient with BDD in their MRI findings. MRN in liver MRI may suggest high bilirubin level and more chance to have cholangitis, but the findings of BDD may related to low bilirubin level and less change to have cholangitis.

A Case of Ampullary Adenomyoma Associated with Dilatations of Pancreatic and Biliary Ducts / 대한소화기내시경학회지

Adenomyoma is a nonneoplastic lesion that can be found anywhere in the gastrointestinal tract, but it's rarely found in the ampulla of Vater. To the best of our knowledge, it is a benign lesion, but most cases are misdiagnosed as carcinoma or adenoma by a preoperative endoscopic or radiologic procedure, and this leads to unnecessarily extensive surgical resection. We report here on a case of ampulla of Vater adenomyoma that resulted in biliary and pancreatic duct dilatation. The tumor was diagnosed by endoscopic papillectomy.

Postoperative Change of Intrahepatic Bile Duct Dilatation in Choledochal Cyst

Choledochal cyst is a congenital dilatation of the bile duct. Intrahepatic bile duct dilatation of type IVa by Todani's classification at the time of diagnosis resolved spontaneously after cyst excision and hepaticojejunostomy in many cases. It should be distinguished from the true cystic dilatation of the intrahepatic ducts, which tends to persist, albeit after some regression. We therefore studied postoperative intrahepatic duct dilatation changes in choledochal cyst. A total of seventy-six choledochal cysts were managed at the Division of Pediatric Surgery, Department of Surgery, Samsung Medical Center from May 1995 to December 2005. The ratio of males to females was 1:2.8. Preoperative radiologic diagnosis by Todani's classification was Type I (n=52, 68.4 %), II (n=1, 1.3 %), IVa (n=23, 30.3 %). Among fifty-five patients with intrahepatic bile duct dilatation we were able to follow up forty-eight by ultrasonography. Twenty-two patients were type IVa, and twenty-six patients were type I and showed intrahepatic duct dilatation. Mean follow-up duration was 35.3 months (9~105 months). Complete regression of dilated intrahepatic duct was observed in fifteen patients of type IVa and twenty-four patients of type I. Incomplete regression of dilated intrahepatic duct was observed in six patients in type IVa and two patients in type I. Only one patient in type IVa showed no change in ductal dilatation during a follow-up period of 15 months. We conclude that true type IVa is much less frequent than what was diagnosed preoperatively by imaging study. Therefore in type IVa patients who are diagnosed preoperatively the decision to perform liver resection should be carefully considered. Postoperative long term follow up of choledochal cyst with intrahepatic bile duct dilation is needed.

Management Strategy for Congenital Choledochal Cyst with Co-existing Intrahepatic Dilation and Aberrant Bile Duct As Well As Other Complicated Biliary Anomalies

The purpose of this study was to investigate and discuss imaging methods and management strategies for congenital choledochal cyst with co-existing intrahepatic dilation and aberrant bile duct as well as other complicated biliary anomalies. In this study we reviewed and analyzed 72 patients with congenital choledochal cyst, ranging in age from 15 days to 12 years old and who were seen at our hospital during the past 12 years, from January 1993 to October 2005. The image manifestation and clinical significance of patients with co- xisting intrahepatic biliary dilation and aberrant bile duct were carefully examined during operation via MRCP, cholangiography and choledochoscope. Twenty-two cases (30.1%) presented with intrahepatic bile duct dilation and 12 of these were of the cystic type. That is, the orifice of the dilated intrahepatic tract that converged into the common hepatic duct showed membrane or septum-like stenosis. In 10 cases the dilation tapered off from the porta hepatis to the initiating terminals of the intra-hepatic bile ducts and was not accompanied by stenosis. An aberrant bile duct was observed in 2 of the cases. In 3 cases, the right and left hepatic ducts converged at the choledochal cyst. In conclusion, the imaging methods for intrahepatic bile duct dilation possess important clinical significance. Further, for hepatojejunostomy with radical excision of a choledochal cyst, additional operative procedures for intrahepatic stenosis, possible bile duct malformation and pancreaticobiliary common duct calculi can potentially reduce postoperative complications.

Management Strategy for Congenital Choledochal Cyst with Co-existing Intrahepatic Dilation and Aberrant Bile Duct As Well As Other Complicated Biliary Anomalies

The purpose of this study was to investigate and discuss imaging methods and management strategies for congenital choledochal cyst with co-existing intrahepatic dilation and aberrant bile duct as well as other complicated biliary anomalies. In this study we reviewed and analyzed 72 patients with congenital choledochal cyst, ranging in age from 15 days to 12 years old and who were seen at our hospital during the past 12 years, from January 1993 to October 2005. The image manifestation and clinical significance of patients with co- xisting intrahepatic biliary dilation and aberrant bile duct were carefully examined during operation via MRCP, cholangiography and choledochoscope. Twenty-two cases (30.1%) presented with intrahepatic bile duct dilation and 12 of these were of the cystic type. That is, the orifice of the dilated intrahepatic tract that converged into the common hepatic duct showed membrane or septum-like stenosis. In 10 cases the dilation tapered off from the porta hepatis to the initiating terminals of the intra-hepatic bile ducts and was not accompanied by stenosis. An aberrant bile duct was observed in 2 of the cases. In 3 cases, the right and left hepatic ducts converged at the choledochal cyst. In conclusion, the imaging methods for intrahepatic bile duct dilation possess important clinical significance. Further, for hepatojejunostomy with radical excision of a choledochal cyst, additional operative procedures for intrahepatic stenosis, possible bile duct malformation and pancreaticobiliary common duct calculi can potentially reduce postoperative complications.