Characteristics and outcomes of minimally invasive surgery for congenital biliary dilatation in children aged <6 years: Comparison between children and adults.

BACKGROUND/PURPOSE: We evaluated the minimally invasive surgery for congenital biliary dilatation (CBD) in adults and children and analyzed the surgical outcomes, especially in children aged <6 years. METHODS: Characteristics and surgical outcomes of patients with CBD who underwent minimally invasive surgery at our hospital between 2013 and 2023 were retrospectively reviewed. RESULTS: Overall, 129 patients (89 children aged <6 years, 9 children aged between 6 and 18 years, and 21 adults) were included in this study. Children exhibited more protein plug presence and abnormal biochemical data than adults. Incidence of postoperative pancreatic fistulas was highest in the adult group (3.4%, 11%, and 33%, respectively, p < .01). In children aged <6 years, postoperative bile leakage and pancreatic fistulas occurred in 9.0% and 3.4% of patients, respectively. Multivariate logistic regression analysis revealed that the Todani IVA was associated with a decrease in postoperative bile leakage (odds ratio: -1.7; 95% confidence interval: (-3.3)-(-0.22), p = .03). CONCLUSION: Adults with CBD required prolonged operative times and had more short-term complications than children with CBD. In children aged <6 years, minimally invasive surgery for CBD can be safely performed; however, a small diameter of the bile duct may be associated with bile leakage.

First pediatric pelvic surgery with the Senhance® robotic surgical system: A case series.

The Senhance® robotic system (Senhance [Asensus Surgical Inc., Naderhan, NC, USA]) is a new surgical assistive robot following the da Vinci Surgical System that has been demonstrated to be safe and efficacious. Herein, we report the first case series of pediatric pelvic surgery using Senhance. Two anorectoplasties and one rectal pull-through coloanal anastomosis for rectal stenosis were performed in three children (5-9 months, 7-9 kg) using a 10-mm three-dimensional (3D) 4K camera and 3 and 5 mm forceps operated with Senhance. None of the patients had intraoperative complications or a good postoperative course. Pediatric pelvic surgery with Senhance could be performed precisely and safely with a small body cavity. With its beautiful 3D images, motion of forceps with reduced tremor, and availability of 3-mm forceps, Senhance may be better suited for children compared with other models.

Bile lakes in patients with biliary atresia who presented with jaundice-free native liver survival indicating the risk of subsequent liver transplantation due to various factors.

PURPOSE: The prognostic factors of subsequent liver transplantation (LT) in patients with biliary atresia (BA) who presented with jaundice-free native liver survival were investigated. METHODS: This study retrospectively reviewed patients who underwent portoenterostomy (PE) for BA. Patients with jaundice-free native liver survival at 1 year postoperatively were divided into the autologous liver survivor and liver transplant recipient groups. Peri- and postoperative data were compared between the two groups. RESULTS: Among 97 patients with BA, 29 who received LT within 1 year after PE were excluded from the analysis. Further, 48 patients currently living with native liver and 20 who received LT after 1 year postoperatively were compared. Bile lake (BL) was the strongest risk factor of LT. The risk score was 2.38 ∗ B L s c o r e + 0.00466 ∗ T B A , and the area under the receiver operating characteristic curve was 0.83. Patients with BL and those without significantly differed in terms of the native liver survival rate. Patients with BL who presented with not only cholangitis but also gastrointestinal hemorrhage and hepatopulmonary syndrome received LT. CONCLUSION: BL can cause different pathologies. Moreover, it is an evident risk factor of subsequent LT in patients with BA who are living with native liver at 1 year after PE.

Thoracoscopic primary repair is useful for esophageal atresia with tracheoesophageal fistula in neonates with low body weight.

PURPOSE: The surgical indication of thoracoscopic primary repair for esophageal atresia with tracheoesophageal fistula is under debate. The current study aimed to investigate the outcome of thoracoscopic primary repair for esophageal atresia with tracheoesophageal fistula in patients weighing < 2000 g and those who underwent emergency surgery at the age of 0 day. METHODS: The surgical outcomes were compared between patients weighing < 2000 g and those weighing > 2000 g at surgery and between patients who underwent surgery at the age of 0 day and those who underwent surgery at age ≥ 1 day. RESULTS: In total, 43 patients underwent thoracoscopic primary repair for esophageal atresia with tracheoesophageal fistula. The surgical outcomes according to body weight were similar. Patients who underwent surgery at the age of 0 day were more likely to develop anastomotic leakage than those who underwent surgery at the age of ≥ 1 day (2 vs. 0 case, p = 0.02). Anastomotic leakage was treated with conservative therapy. CONCLUSION: Thoracoscopic primary repair is safe and useful for esophageal atresia with tracheoesophageal fistula even in newborns weighing < 2000 g. However, emergency surgery at the age of 0 day should be cautiously performed due to the risk of anastomotic leakage.

Surgical approach for fecal incontinence with a patulous anus after transanal pull-through for Hirschsprung disease.

BACKGROUND: We have performed transanal pull-through (TAPT) for Hirschsprung disease since 1998. Some of our patients after TAPT showed a patulous anus and suffered from severe true fecal incontinence. We performed anal canal plasty for these patients and evaluated its efficacy in restoring anorectal function. METHODS: Thirty-one patients who were ≥5years old were previously operated on for Hirschsprung disease, and seven (22.5%) of these were indicated for this procedure. Anorectal function was evaluated using the Japanese Study Group of Anorectal Anomalies (JSGA) clinical assessment of defecation function score. For surgery, the patients were positioned in the prone jackknife posture. The posterior half of the anal canal was exposed and folded inward until the anal canal lumen was as narrow as the surgeon's index finger. External anal sphincter muscles were repaired, and the wound was closed vertically. RESULTS: The mean preoperative JSGA score was 1.42±0.4. The mean JSGA scores at 2-6months and 2years after this procedure were 5±2.1 and 5.8±2.1, respectively. Postoperatively, the JSGA score significantly improved at both times (p<0.05). CONCLUSIONS: Anal canal plasty may be effective for true fecal incontinence and a patulous anus after TAPT. This surgical approach may be useful for these conditions. LEVEL OF EVIDENCE: Type of study: Treatment study, Level IV.

Cartilage-hair hypoplasia associated with isolated hypoganglionosis: A case report.

Cartilage-hair hypoplasia is a rare metaphyseal chondrodysplasia characterized by diverse clinical manifestations and a high incidence of Hirschsprung disease. We present a male patient with cartilage-hair hypoplasia associated with severe intestinal obstruction. Genetic analysis of ribonuclease mitochondrial RNA-processing complex gene identified compound heterozygous mutations consisted with previously reported mutations: n.-14_3dupGAAGCTGAGGACGTGGT and n.183G > T. First, we considered that intestinal obstruction was due to an extensive type of Hirschsprung disease, but it was later confirmed as isolated hypoganglionosis. Isolated hypoganglionosis is rare and its therapeutic strategies are not well established. In cases of cartilage-hair hypoplasia associated with severe intestinal obstruction, the differential diagnosis of not only Hirschsprung disease, but also isolated hypoganglionosis, should be considered.

Kinetics of Alloantigen-Specific Regulatory CD4 T Cell Development and Tissue Distribution After Donor-Specific Transfusion and Costimulatory Blockade.

BACKGROUND: The influence of donor-side regulation toward recipient antigens on graft outcome is poorly understood. METHODS: Because this influence might be due in part to the accumulation of tissue-resident memory T cells in the donor organ, we used a standard murine tolerization model (donor-specific transfusion plus CD40L blockade) to determine the kinetics of development and peripheralization of allospecific regulatory T cell in lymphoid tissues and liver, a secondary lymphoid organ used in transplantation. RESULTS: We found that donor-specific transfusion and CD40L blockade leads to a progressive and sustained T regulatory allospecific response. The cytokines IL10, TGFß, and IL35 all contributed to the regulatory phenomenon as determined by trans vivo delayed hypersensitivity assay. Unexpectedly, an early and transient self-specific regulatory response was found as well. Using double reporter mice (forkhead box p 3 [Foxp3]-yellow fluorescent protein, Epstein-Barr virus-induced gene 3 [Ebi3]-TdTomRed), we found an increase in Foxp3+CD25+ regulatory T (Treg) cells paralleling the regulatory response. The Ebi3+ CD4 T cells (IL35-producing) were mainly classic Treg cells (Foxp3+CD25+), whereas TGFß+ CD4 T cells are mostly Foxp3-negative, suggesting 2 different CD4 Treg cell subsets. Liver-resident TGFß+ CD4 T cells appeared more rapidly than Ebi3-producing T cells, whereas at later timepoints, the Ebi3 response predominated both in lymphoid tissues and liver. CONCLUSIONS: The timing of appearance of donor organ resident Treg cell subsets should be considered in experiments testing the role of bidirectional regulation in transplant tolerance.

Effective Surveillance to Identify the Surgical Patients Carrying Methicillin-resistant Staphylococcus aureus on Admission in a Pediatric Ward.

Background To effectively detect surgical patients carrying methicillin-resistant Staphylococcus aureus (MRSA) in a pediatric ward, we investigated who should be tested through bacteriological surveillance on admission. Methods In 1124 patients, bacteriological examination of throat swabs and stool samples was performed within 48 hours after admission. The relations between carrier rate (CR) of MRSA and a history of hospitalization, neurological disorder (ND), and age group were retrospectively investigated. Results The CR of all patients was 7.8%. The CRs of the patients with no history of hospitalization, a history of hospitalization before the previous year, and a history of hospitalization within the past year (HWPY) were 2.3%, 3.4%, and 14.5%, respectively; the CR of those with HWPY was significantly higher than the CRs of the other 2 groups (p<0.0001). The CR of the patients with an ND (19.2%) was significantly higher than that of the patients without an ND (6.1%) (p<0.0001). The CRs of the patients <3 (11.7%) and 15 (11.9%) years old were higher than that of other patients (3.8%) (p< 0.05). With multivariate logistic regression analysis, HWPY, an ND and age groups <3 and k15 were independent risk factors. Conclusions - HWPY, an ND, and being <3 or k 15 years old were found to be risk fqctors for carrying MRSA on admission. Active bacteriological surveillance on admission should be performed for patients with these risk factors.