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.

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.

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).

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.

Humans ; Infant ; Biliary Atresia/diagnosis* ; Choledochal Cyst/diagnosis* ; RNA, Circular ; Diagnosis, Differential

The diagnosis of cystic liver disease has made great progress with the advent of enhanced imaging techniques. At the same time, its management has gradually improved over the past few decades, providing the basis for the development of appropriate diagnostic and treatment guidelines. To this end, the European Association for the Study of the Liver has developed clinical guidelines for the diagnosis and treatment of non-infectious cystic liver disease. This guideline put forward recommendations based on an in-depth review of the relevant literature for addressing clinical issues, including the diagnosis and treament of hepatic cysts, hepatic mucocystic tumors, biliary hamartomas, polycystic liver disease, Caroli disease or Caroli syndrome, biliary hamartomas, and peribiliary cyst.
Humans ; Liver Diseases/pathology* ; Cysts/pathology* ; Caroli Disease/diagnosis* ; Liver Neoplasms/therapy* ; Hamartoma

Caroli Disease ; Humans

Extrahepatic biliary tract tumors are rare and among them rhabdomyosarcoma is most common. Rhabdomyosarcoma is a soft tissue malignant musculoskeletal tumor and is a very rare malignancy of the common bile duct in children. It usually presents as obstructive jaundice and/or pruritus. If there is no local invasion to the adjacent tissues, the radiological appearance of the tumor lesion is like a choledochal cyst. So the diagnosis is usually made at surgery or by preoperative biopsy. It is important to diagnose early and differentiate it from choledochal cyst and start treatment as early as possible for long time survival of the patient. This case report presented a case of a 10-year-old boy with recurrent onset of obstructive jaundice and fever preoperatively who was diagnosed as choledochal cyst and postoperatively as embryonal rhabdomyosarcoma of the common bile duct. After surgical resection and postoperative chemotherapy, the child had a good prognosis. So it is crucial to know that this rare tumor can mimic congenital choledochal cyst and it should be considered in the differential diagnosis of obstructive jaundice in children.
Child ; Choledochal Cyst ; Common Bile Duct/pathology* ; Diagnosis, Differential ; Humans ; Jaundice, Obstructive/etiology* ; Male ; Rhabdomyosarcoma, Embryonal/diagnosis*

OBJECTIVE: To explore the feasibility of enhanced recovery after surgery (ERAS) in treatment of children with congenital choledochal cyst. METHODS: One hundred and thirty children with congenital choledochal cysts admitted in the Children's Hospital of Zhejiang University from June 2017 to June 2019 were divided into ERAS group (=65) and control group (=65) according to admission order. The intestinal tract condition during operation, time of operation, surgical results, time for eating after operation, abdominal drainage after operation, length of hospital stay after operation, total hospital expenses and complications were compared between two groups. RESULTS: Compared with the control group, the satisfaction of intestinal operation field, recovery of gastrointestinal function after operation,time required for the volume of peritoneal drainage fluid to be less than 50 mL,time of abdominal drainage tube removal, and length of hospital stay were all improved in ERAS group (<0.05 or <0.01).ERAS group had more peritoneal effusion after removal of abdominal drainage tube (<0.01), but the incidence of edema after operation was lower (<0.05). The satisfaction of parents in the two groups was similar, but the cooperation of parents in the ERAS group was improved (<0.05) and the total cost of hospitalization was reduced (<0.01). CONCLUSIONS ERAS has advantages over the traditional scheme and can be used in the clinical treatment of children with congenital choledochal cyst.
Case-Control Studies ; Child ; Choledochal Cyst ; economics ; surgery ; Enhanced Recovery After Surgery ; standards ; Humans ; Length of Stay ; Postoperative Complications ; prevention & control

PURPOSE: Improvements in surgical techniques and a better understanding of the unique anesthetic requirements in neonates undergoing laparoscopy have suggested that laparoscopic surgery may be effective in newborns. This study therefore evaluated the safety and feasibility of laparoscopic excision of the cyst (LEC) in neonates. METHODS: This retrospective study included 43 neonates who underwent excision of choledochal cysts between November, 2001, and January, 2018, including 21 who underwent open excision and 22 who underwent LEC. Their perioperative and surgical outcomes were reviewed. The patients were followed up for a median 37 months (range, 3–141 months). RESULTS: Baseline characteristics did not differ significantly in the open and LEC groups. Mean intraoperative peak partial pressure of arterial CO2 (PaCO2) (45.5 mmHg vs. 48.0 mmHg) and total operation time (208.3 ± 71.0 minutes vs. 235.0 ± 47.2 minutes) were similar in both groups. Parents of the patients in the LEC group provided a more positive evaluation of scar scale and greater satisfaction with wound. No patient in either group experienced any critical complications. Three patients in the open excision group required readmission for cholangitis and 2 patients had ileus. No patient in the laparoscopic excision group experienced any postoperative complications during follow-up. CONCLUSION: Despite difficulties performing laparoscopic surgery in neonates, LEC was safe and feasible when intraperitoneal peak pressure was maintained under 10 mmHg and PaCO₂ was closely monitored by a pediatric anesthesiologist. Compared with open excision, LEC provided improved cosmetic outcomes without severe complications. Prospective randomized studies with large numbers of patients are warranted.
Cholangitis ; Choledochal Cyst ; Cicatrix ; Follow-Up Studies ; Humans ; Ileus ; Infant ; Infant, Newborn ; Laparoscopy ; Minimally Invasive Surgical Procedures ; Parents ; Partial Pressure ; Postoperative Complications ; Prospective Studies ; Retrospective Studies ; Wounds and Injuries

Hepatic duct diverticulum is a rare form of choledochal cyst that does not fit into the most widely used Todani classification system. Because of its rarity, it may be difficult for clinicians to diagnose and treat it. Here, we present a case of left hepatic diverticulum in a 57-year-old woman with epigastric pain. At presentation, there were mild elevations in the liver function tests. Computed tomography and magnetic resonance cholangiopancreatography showed diverticulum-like cystic lesion with sludge ball near the confluence portion of both intrahepatic bile duct, but the origin of the lesion could not be identified. The clinical impression was type II choledochal cyst. Surgical excision was planned due to recurrent abdominal pain. The operative findings revealed diverticulum arising from left hepatic duct. Histopathology confirmed the lesion to be diverticulum lined by biliary epithelium. The patient had no postoperative complication and no further symptoms since the operation.
Abdominal Pain ; Bile Ducts ; Bile Ducts, Intrahepatic ; Cholangiopancreatography, Magnetic Resonance ; Choledochal Cyst ; Classification ; Diverticulum ; Epithelium ; Female ; Hepatic Duct, Common ; Humans ; Liver Function Tests ; Middle Aged ; Postoperative Complications ; Sewage

Caroli disease is a rare congenital disorder characterized by nonobstructive dilatation of intrahepatic ducts. In cases with symptomatic intrahepatic manifestations, treatment should correspond to the type with hepatic resection for localized disease and transplantation for diffuse forms. If possible, complete resection of the cysts can cure the symptoms and avoid the risk of malignancy. A 66-year-old woman presented to Wuxi Xishan People's Hospital with recurrent intermittent upper quadrant abdominal pain. Further examinations suggested the diagnosis of Caroli disease limited to the left hepatic lobe. She underwent laparoscopic hepatectomy. Pathological examination confirmed the diagnosis of Caroli disease, and no malignancy was found. There were no immediate complications and no long-term complications after one and one-half years of follow-up. Laparoscopic hepatectomy could be a feasible, safe treatment option for localized Caroli disease.
Abdominal Pain ; Aged ; Caroli Disease* ; Cholecystectomy ; Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Diagnosis ; Dilatation ; Female ; Follow-Up Studies ; Hepatectomy* ; Humans ; Laparoscopy

PURPOSE: Increasing surgical expertise in minimally invasive surgery has allowed laparoscopic surgery to be performed in many abdominal surgeries. Laparoscopic choledochal cyst excision and Roux-en-Y hepaticojejunostomy are challenging and sophisticated surgeries because of the difficult anastomosis. Recent advances in robotic surgery have enabled more delicate and precise movements, and Endowrist instruments allow for securing sutures during anastomosis. This study aimed to compare surgical outcomes of laparoscopic and robotic hepaticojejunostomy in choledochal cyst excision. METHODS: Sixty-seven patients who underwent laparoscopic or robotic-hybrid choledochal cyst excision from 2004 to 2016 were retrospectively analyzed and compared. In robotic surgery, dissection was performed laparoscopically, and hepaticojejunostomy was performed using a robotic platform. RESULTS: The mean operative time was significantly longer in robotic surgery than in laparoscopic surgery (247.94 ± 54.14 minutes vs. 181.31 ± 43.06 minutes, P < 0.05). The mean estimated blood loss (108.71 ± 15.53 mL vs. 172.78 ± 117.46 mL, respectively, P = 0.097) and postoperative hospital stay (7.33 ± 2.96 days vs. 6.22 ± 1.06 days, P = 0.128) were comparable between procedures. Compared to the laparoscopic approaches, robotic surgery had significantly less short-term complications (22.4% vs. 0%, P = 0.029). There were more biliary leakage (n = 7, 14.3%) observed during the first 30 days after surgery in laparoscopy while none were observed in the robotic method. CONCLUSION: Robotic surgery allow for more precise and secure sutures during anastomosis thereby reducing biliary complications. With expanding knowledge and expertise, robotic surgery may offer more advantages over laparoscopy in the era of minimally invasive surgery.
Anastomosis, Roux-en-Y ; Choledochal Cyst* ; Humans ; Laparoscopy ; Length of Stay ; Methods ; Minimally Invasive Surgical Procedures ; Operative Time ; Retrospective Studies ; Robotic Surgical Procedures ; Sutures