Clinical implications of pediatric biliary intraepithelial neoplasia diagnosed from a choledochal cyst specimen.

BACKGROUND: Biliary intraepithelial neoplasia (BilIN), a noninvasive precursor of cholangiocarcinoma, can manifest malignant transformation. Since cholangiocarcinoma (CCA) may progress due to chronic inflammation in the bile ducts and gallbladder, choledochal cysts are considered a precursor to CCA. However, BilIN has rarely been reported in children, to date. METHODS: We reviewed medical records of patients (< 18 years of age, n = 329) who underwent choledochal cyst excision at Asan Medical Center from 2008 to 2022. BilIN was diagnosed in 15 patients. Subsequent analyses were performed of the demographics, surgical procedures, clinical course, and outcomes in these patients. Subgroup analysis and multivariate logistic regression test were performed to identify factors influencing BilIN occurrence. RESULTS: The mean age of the patients included in our study was 40.1 ± 47.6 months. In 15 patients, BilIN of various grades was diagnosed. Todani type I was prevalent in 80% of the patients. The median age at surgery was 17 months. During a mean follow-up of 63.3 ± 94.0 months, no adverse events such as stone formation in the remnant intrapancreatic common bile duct and intrahepatic duct or cholangiocarcinoma were observed, indicating a favorable outcome until now. CONCLUSIONS: The potential progression of choledochal cysts to BilIN in children was demonstrated. These results could underscore the importance of early and comprehensive excision of choledochal cysts, including resection margins for associated lesions and more thorough postoperative surveillance in patients with or at risk of BilIN.

The outcome of hiatal hernia repair and factors associated with surgical outcome in the pediatric population: a single-center experience.

Purpose: Hiatal hernia in children is a rare condition, and there is limited knowledge available about the disease itself. There is currently no agreement or consensus on the treatment of hiatal hernia in the pediatric population due to lack of evidence. In this study, we were to assess our experience with hiatal hernia, including the characteristics of our patients, surgical outcomes, and factors that influence the outcomes. Methods: We retrospectively reviewed the medical records of 49 patients below the age of 18 years who underwent hiatal hernia repair at the Asan Medical Center between 2006 and 2021. We analyzed and compared the general characteristics and surgical outcomes based on the presence of recurrence and coexisting congenital diseases. Results: Hiatal hernia progression was found to be associated with various medical conditions; however, no significant differences in patient characteristics or surgical outcomes between those with and without comorbidities were observed. There were no significant differences in patient characteristics or outcomes between the initial and redo operations. Fundoplication was performed in 19 patients (36.7%) during the initial operation and in 7 patients (87.5%) during repeat hiatal hernia repair. Conclusion: The presence of an underlying disease didn't influence the treatment and progression of hiatal hernia. Furthermore, there were no significant differences in the clinical course between patients with recurrent hiatal hernia and those experiencing it for the first time. Additionally, the impact of fundoplication on the recurrence of hiatal hernia in pediatric patients was found to be minimal.

Modified patch-conduit venoplasty for portal vein hypoplasia in pediatric liver transplantation.

Background: Portal vein (PV) interposition can induce various PV-related complications, making more reliable techniques necessary. The present study describes the development of a modified patch venoplasty technique, combining the native PV wall and a vein homograft conduit, called modified patch-conduit venoplasty (MPCV). Methods: The surgical technique for MPCV was optimized by simulation and applied to seven pediatric patients undergoing liver transplantation (LT) for biliary atresia combined with PV hypoplasia. Results: The simulation study revealed that inserting the whole-length native PV wall as a longitudinal rectangular patch was more effective in preventing PV conduit stenosis than the conventional technique using triangular partial insertion. These findings were used to develop the MPCV technique, in which the native PV wall was converted into a long rectangular patch, acting as a backbone for PV reconstruction. A longitudinal incision on the vein conduit converted the cylindrical vein into a large vein patch. The wall of the native PV was fully preserved as the posterior wall of the PV conduit, thus preventing longitudinal redundancy and unwanted rotation of the reconstructed PV. This technique was applied to seven patients with biliary atresia undergoing living-donor and deceased-donor split LT. None of these patients has experienced PV complications for up to 12 months after transplantation. Conclusions: This newly devised MCPV technique can replace conventional PV interposition. MCPV may be a surgical option for reliable PV reconstruction using fresh or cryopreserved vein homografts during pediatric LT.

Long-term outcomes of liver transplantation using grafts from donors with active hepatitis B virus replication: a multicenter cohort study.

Purpose: Liver grafts from donors with HBV infection contributed to expanding the donor pool under the hepatitis B immunoglobulin and antiviral agents (nucleos(t)ide analogues) in the HBV-endemic area. We report long-term outcomes of liver transplantations (LTs) using grafts from donors with active or chronic HBV infection. Methods: Overall, 2,260 LTs performed in 3 major hospitals in Seoul from January 2000 to April 2019 were assessed for inclusion. Twenty-six grafts (1.2%) were obtained from HBsAg (+), HBeAb (+), or HBcAb (+) donors, and recipient outcomes were retrospectively reviewed. Donor and recipient demographics and transplantation outcomes were analyzed. Results: Sixteen deceased donor LTs were performed using active HBsAg (+) grafts. Ten other LTs were sourced from 10 living donors. There was no significant difference in survival in patients who received deceased donor LTs compared with that in those who underwent LT with non-hepatitis virus-infected grafts. Fourteen patients who were followed up for >5 years were stable, and no difference in hepatocellular carcinoma recurrence rate was observed 5 years after transplantation between transplants from donors with and those without HBV. Conclusion: Considering long-term outcomes, liver grafts from donors with active HBV replication can be safely used for LT.

Progression of a persisting mesenchymal hamartoma to intrahepatic cholangiocarcinoma 24 years after the initial diagnosis: A case report.

Mesenchymal hamartoma of the liver (MHL) is a rare benign tumor that often presents in early childhood, and it rarely occurs in adulthood. Aberrant development of the portal tract is a known cause of MHL. Although limited information is available on the natural course of MHL, malignant transformation has been reported in a few cases. Here, we report a case of a 26-year-old female with intrahepatic cholangiocarcinoma secondary to unresected MHL. The patient underwent resection of the hepatic mass, which was diagnosed as MHL at 2 years of age, due to an increase in mass size and a suspicion of malignant transformation during work-up. Histopathology confirmed intrahepatic adenosquamous carcinoma in the background of MHL, with a T2N0M0 pathological stage (stage II). The surgical margin was free from tumor cells. The patient fully recovered postoperatively and started receiving adjuvant chemotherapy. Previous case reports have only reported about the development of undifferentiated embryonal sarcoma or angiosarcoma as malignant transformation of MHL. Cases of other malignancies have not been published; however, it is difficult to rule out the occurrence of various malignancies related to the portal tract when considering the pathogenesis of the disease. To the best of our knowledge, this is the first case report of adenocarcinoma of bile duct origin secondary to MHL. This case report suggests that aggressive surgical management should be considered after the initial diagnosis of MHL.

A 17-year experience on bilaterality of ovarian germ cell tumors in pediatric population and its clinical implications: A single-center study.

BACKGROUND: Since the incidence of ovarian germ cell tumor (GCT) in pediatric population is low, there is little information regarding this tumor. A synchronous and metachronous bilateral mass is not infrequently observed in GCT patients. It is important to prevent early ovarian failure and preserve fertility. METHODS: We retrospectively reviewed the medical records of 112 patients less than 18 years surgically treated for ovarian GCT between 2008 and 2021 at the Department of Pediatric surgery of the Seoul National University Hospital (SNUH). Among these, 13 patients (11.6%) had bilateral masses. In metachronous masses, the recurrence period and therapeutic approach were evaluated. When ovarian failure was a concern, fertility preservation was attempted. RESULTS: Mean age was 8.1 ± 4.0 years. Patients with a bilateral mass were older (p = 0.004). Follow-up was 39.6 ± 40.6 months. Five patients had a synchronous mass, and 8 patients experienced metachronous recurrence. Two patients with a synchronous mass had a metachronous neoplasm (n = 1) and recurrence (n = 1). Patients with metachronous recurrence started menstruation except for two patients who had not reached puberty. The mean recurrence interval was 45.9 ± 38.6 months. Ovum banking was requested for 6 patients and performed in 2. CONCLUSIONS: During our 17 years of experience, bilaterality was observed in 11.6% of GCT patients. Metachronous mass occurs after 5 years. A longer follow-up plan is needed for early detection. Our multidisciplinary approach to preserve fertility seemed to be successful with close cooperation with gynecologists.

Laparoscopic removal of mesh migrating into the sigmoid colon after totally extraperitoneal (TEP) laparoscopic inguinal hernia repair with positive faecal occult blood test.

A 76-year-old man was referred to our clinic after a foreign body seen in his sigmoid colon during a colonoscopy. He had undergone three operations for a left inguinal hernia within the previous 8 years, and the first procedure was a laparoscopic totally extraperitoneal approach. Four years later, removal of migrated and infected mesh was conducted by open approach. He then had a positive stool occult blood test for routine check-up 4 years after the remnant mesh removal. An ill-defined lesion was identified on colonoscopy. CT revealed a 2.7 cm diameter enhancing lesion in the sigmoid colon. Laparoscopic sigmoidectomy was performed, and remnant mesh fragment was found in the sigmoid colon and removed. The migrated mesh could not be wholly removed by open abdominal approach and the remnant mesh fragment migrated to sigmoid colon. It suggests the importance of a laparoscopic approach to remove the entire mesh.

Recurrent parvovirus B19 infection-associated pure red cell aplasia in a kidney transplant patient.

Posttransplant anemia is a common complication after kidney transplantation. Parvovirus B19 (PVB19) infection can induce pure red cell aplasia (PRCA) in immunosuppressed transplant patients. We herein report a case of recurrent PVB19-associated PRCA in a kidney transplant patient. A 49-year-old woman presented with anemia and normal renal function 1 year after a deceased-donor kidney transplantation for immunoglobulin A nephropathy-related end-stage renal disease. She received desensitization therapy, and 2 years later, she underwent transplantation with thymoglobulin induction. Despite repeated red cell transfusion and erythropoietin therapy, her anemia aggravated progressively. Bone marrow biopsy revealed normocytic normochromic PRCA. Real-time polymerase chain reaction detected a high plasma load of PVB19. Administration of intravenous immunoglobulin (IVIG) at 2 g/kg with adjuvant reduction of tacrolimus and discontinuation of myfortic acid effectively treated the anemia. However, the PVB19 load remained high, and PRCA recurred 7 months after the initial IVIG treatment. Tacrolimus was switched to cyclosporine in the second IVIG treatment, which successfully improved PRCA and reduced the PVB19 load. Our case suggested that PVB19-associated PRCA should be suspected when persistent anemia is observed in kidney transplant patients with heavy immunosuppression and that PVB19-associated PRCA can recur in the presence of persistent PVB19 viremia.

Successful treatment of early acute antibody-mediated rejection in an human leukocyte antigen-incompatible and ABO-incompatible living-donor kidney transplant patient.

For successful human leukocyte antigen-incompatible (HLAi) or ABO-incompatible (ABOi) living-donor kidney transplantations (LDKTs), pretransplant desensitization is essential; however, early antibody-mediated rejection (ABMR) remains the most important complication after HLAi or ABOi transplantation. Here, we report a case of early acute ABMR in simultaneous HLAi and ABOi LDKT with preformed donor-specific antibody (DSA), despite desensitization. Dialysis-dependent, severe ABMR occurred with a rebound of pre-existing DSA and appearance of de novo DSA after initial normalization of renal function, 8 days postoperatively. However, a low anti-ABO antibody titer (1:8) was maintained after transplantation. Combination therapy of plasmapheresis, high-dose intravenous immunoglobulin, and bortezomib improved both ABMR and renal functions. Thus, an appropriate preventive and therapeutic management for early ABMR is important among high-risk LDKT patients. Furthermore, early AMBR can occur despite pretransplant desensitization as seen in this case, and close monitoring of the patient and prompt management are considered vital for better therapeutic outcomes.

Sedative and analgesic effects of intravenous xylazine and tramadol on horses.

This study was performed to evaluate the sedative and analgesic effects of xylazine (X) and tramadol (T) intravenously (IV) administered to horses. Six thoroughbred saddle horses each received X (1.0 mg/kg), T (2.0 mg/kg), and a combination of XT (1.0 and 2.0 mg/kg, respectively) IV. Heart rate (HR), respiratory rate (RR), rectal temperature (RT), indirect arterial pressure (IAP), capillary refill time (CRT), sedation, and analgesia (using electrical stimulation and pinprick) were measured before and after drug administration. HR and RR significantly decreased from basal values with X and XT treatments, and significantly increased with T treatment (p < 0.05). RT and IAP also significantly increased with T treatment (p < 0.05). CRT did not change significantly with any treatments. The onset of sedation and analgesia were approximately 5 min after both X and XT treatments; however, the XT combination produced a longer duration of sedation and analgesia than X alone. Two horses in the XT treatment group displayed excited transient behavior within 5 min of drug administration. The results suggest that the XT combination is useful for sedation and analgesia in horses. However, careful monitoring for excited behavior shortly after administration is recommended.