World Journal of Pediatric Surgery最新文献

Background: Sedation is often required for infant and preschool children to obtain clear magnetic resonance imaging (MRI). This study was designed to determine the 95% effective dose (ED₉₅) of oral midazolam (MID) and intranasal dexmedetomidine (DEX) in combination for sedation in pediatric MRI.

Methods: We have used a biased coin design up-and-down sequential methodology. Initially, 144 patients were split into two groups. A total of 72 patients were randomly assigned to determine the ED₉₅ of DEX in combination with a fixed dose of 0.5 mg/kg MID, and 72 were given various doses of MID combined with a fixed dose of DEX at 1 µg/kg to determine the ED₉₅ of MID. ED₉₅ was calculated using isotonic regression. At last, the plan was to include 225 cases to test the sedation success rate of DEX combined with MID ED₉₅ dose. Adverse events were recorded.

Results: The ED₉₅ of DEX was 0.89 µg/kg (95% confidence interval (CI) 0.68 to 0.95) combined with a fixed dose of MID at 0.5 mg/kg. The ED₉₅ of MID was 0.47 mg/kg (95% CI 0.30 to 0.50) combined with a fixed intranasal dose of 1 µg/kg DEX. Using 1 µg/kg DEX combined with 0.5 mg/kg MID, the sedation success rate was 95.1% in a verification group of 225 children.

Conclusions: This study reports relatively low ED₉₅ doses of intranasal DEX and oral MID when DEX is determined with a fixed dose of MID and MID determined with a fixed dose of DEX. The combination provides efficient and safe sedation for young children for MRI scanning. Further validation is required.

Trial registration number: ChiCTR2300068611.

The Japanese Biliary Atresia Registry (JBAR) was launched in 1989 by the Japanese Biliary Atresia Society (JBAS) to investigate the epidemiology and etiology of biliary atresia and to improve surgical outcomes. The JBAR collects data through initial, liver transplantation, and follow-up questionnaires. Pediatric surgeons from JBAS member institutions and hospitals affiliated with the Japanese Society of Pediatric Surgeons are responsible for registering patients and submitting data through an online system. Each patient is to be followed up for 40 years. As of 2023, 3951 patients had been registered, with 1688 undergoing liver transplantation. The native liver survival rates in the 10th, 20th, and 30th year surveys were 50.5%, 44.4%, and 40.9%, respectively. The overall survival rates in the 10th, 20th, and 30th year surveys were 88.9%, 87.6%, and 85.7%, respectively. The surgical outcome of biliary atresia has markedly improved owing to the cooperation between Kasai portoenterostomy and liver transplantation. A comprehensive Japanese database of patients with biliary atresia (https://jbas.net/en/national-registration/) is now available.

Background: This study aimed to investigate if mashed ripe bananas (bananagram) could be used to assess the integrity of the gut before transverse colostomy closure in patients with Hirschsprung disease (HSCR) in a low- and middle-income country tertiary-level hospital.

Methods: This is a prospective, single-center, hospital-based cohort study. A feeding tube tip was inserted about 5-8 cm through the distal loop, and 15 mL of mashed banana was introduced. The amount introduced and the spontaneous expulsion of mashed banana were measured. We also measured colonic transit time (CTT), input and expulsion ratio, color of the expulsed banana stream, size of the banana stream passed through the anus and clinical signs of bowel obstruction or perforation after the procedure.

Results: From January 2018 to June 2023, 266 HSCR patients with a transverse colostomy were included. The mean±standard deviation (SD) age was 26.45±6.41 months. The median CTT of mashed banana was 8 min (interquartile range (IQR): 4-13 min). The mean amount of mashed banana spontaneously expelled through the anus was 13.25±0.95 mL. More than 90% of patients expelled unchanged mashed bananas, and 9.4% expelled stool-banana mixtures. Banana stream was 0.3-1.5 cm in 257 patients and <0.3 cm in nine patients. These nine patients needed revision pull-through.

Conclusion: Bananagram can be performed at the bedside. It is easy to perform, cost-effective, available throughout the year and does not pose radiation hazards.

Organoids are three-dimensional and self-organizing cell cultures of various lineages that resemble structures and functions of an organ in many ways, and they are versatile tools in disease modeling and patho-mechanistic study of human diseases affecting their tissues of origin. Biliary atresia (BA), a cholangiopathy affecting the bile ducts of the liver, is a heterogeneous and multifaceted liver disease of complex pathogenesis. Cholangiopathies refer to a category of liver diseases that affect the cholangiocytes, the epithelial cells lining the lumen of the biliary trees. Biliary organoids consist of cholangiocytes in a spherical monolayer epithelium, which favorably resembles the structures and functional properties of the bile duct cholangiocytes. Biliary tissue-derived cells, pluripotent stem cells or embryonic stem cells, and hepatic progenitor cells are capable of generating biliary organoids. In the last decade, a considerable advancement has been made in the generation of biliary organoids for modeling liver physiology and pathophysiology. Using biliary organoids, scientists have advanced our knowledge underlying the pathogenic roles of genetic susceptibility, dysregulated hepatobiliary development/structure, environmental factors, and dysregulated immune-inflammatory responses to an injury in BA. This review will summarize and discuss the derivation and the use of biliary organoids in the disease modeling and patho-mechanistic study of BA.

Background: Multisystem inflammatory syndrome in children (MIS-C) is a disease that emerged during the COVID-19 pandemic. Patients exhibit symptoms mimicking the clinical presentation of an acute abdomen, representing a novel differential diagnosis, particularly in the young generation. This study aims to investigate the current level of awareness of MIS-C among surgeons and medical students.

Methods: We conducted an anonymous online questionnaire among members of the Swiss Surgical Society and Swiss medical students. The questionnaires collected participants' baseline demographics and their awareness regarding MIS-C.

Results: Both students and surgeons obtained very low scores in the self-assessment and had a low score in the section with awareness questions (true score). In medical students, we observed a positive correlation between self-assessment and true scores (ρ=0.422, p=0.001), while surgeons had a negative correlation (ρ=-0.243, p<0.001). Furthermore, there was a positive correlation between gender and self-assessment (ρ=0.245, p<0.001), depicting a higher self-assessed score in female surgeons (median female self-assesment=5, IQR: 2-7). Likewise, board-certified pediatric surgeons and surgeons treating both children and adults had a higher self-assessment compared with non-pediatric surgeons. In both populations (surgeons and students), the true scores were similar in all the variables analyzed. Two-thirds of surgeons stated that MIS-C should be considered a differential diagnosis, and about half of surgeons indicated MIS-C being part of their differential diagnosis already.

Conclusion: The results show an insufficient level of awareness concerning MIS-C among students and surgeons, warranting implementation in students' curriculum and surgeons' continuous training.

To analyze surgical challenges and outcomes in patients who completed the whole journey of vertical expandable prosthetic titanium rib (VEPTR) treatment for congenital early-onset scoliosis (C-EOS), given the limited evidence available on VEPTR graduates. A retrospective review was conducted on nine consecutive patients with C-EOS and thoracic hypoplasia treated at a single tertiary care center, with assessment of clinical and radiological outcomes. At mean duration of 7.4 (range 4.3-10.5) years of VEPTR treatment, the mean coronal deformity angle measured 65° preoperatively, 50° postoperatively, and 58° at final follow-up. Mean T1-S1 length (pre-op 252 mm, final follow-up 333 mm) and T1-T12 length (preop 128 mm, final follow-up 196 mm) improved by 32% at final follow-up. Mean space available for lung was 86% (range 79%-93%) preoperatively, increasing to 90% (range 85%-95%) postoperatively and 97% (range 87%-107%) at final follow-up. Nine children had a cumulative 17 (188%) complications comprising wound problems, infection, and device migration or prominence. In five patients who underwent definitive fusion, mean coronal deformity angle and T1-S1 length improved by 17% and 11%, respectively. VEPTR is valuable in managing EOS, particularly in patients with thoracic insufficiency syndrome. However, the need for multiple surgeries, limited correction potential, and risk of partial loss of correction make it less suitable for other cases.

Background: Biliary atresia (BA) is a rare condition that meets the criteria for neonatal screening. Taiwan province of China led the way in BA screening during the 1990s by introducing a neonatal stool color card (SCC), which proved effective in facilitating early BA diagnosis and improving outcomes. Another commonly studied BA screening approach is serum bilirubin measurement. Several European countries have also begun implementing BA screening initiatives, although slowly. In this study, we evaluate BA screening strategies across Europe.

Methods: Published data, after having performed a scoping review, as well as internet searches were analyzed. Screening approaches proposed in Europe are described, including SCC, serum bilirubin measurements, and other biochemical markers such as bile acids or amino acid profiles.

Results: In Europe, national BA screening programs have been established solely in Switzerland, France, and Germany, all using the SCC. Other European countries, such as the Netherlands, Portugal, and Italy, have made efforts, but have yet to achieve broad implementation beyond localized initiatives. Skepticism among healthcare professionals and logistical challenges seem to hinder broader adoption. Emerging technologies, such as artificial intelligence-enhanced SCC applications, may show promise in overcoming these barriers. Serum bilirubin measurement is another widely deliberated method, particularly in the UK, where it has been shown to be sensitive and specific for BA detection. However, logistical and financial limitations remain key obstacles to its widespread use. Other biochemical methods, such as bile acid and amino acid profiling, have shown potential in research settings, but lack clinical translation in Europe.

Conclusions: This review highlights Europe's limited role in global BA screening efforts and emphasizes the need for advocacy, collaboration, and integration of screening strategies tailored to regional healthcare systems. Combining the SCC with bilirubin measurements could optimize cost-effectiveness and efficiency. Expanding BA screening programs requires strengthening advocacy efforts to improve outcomes for affected infants.

Background: The discrepancy in size between donor and recipient presents a complex challenge in pediatric liver transplantation (PLT), often necessitating secondary abdominal closure to prevent abdominal compartment syndrome. The aim of this study is to determine the variables associated with an increased risk of requiring secondary closure in PLT.

Methods: The retrospective study analyzed all primary liver transplantations performed in patients under 18 years of age from January 2014 to July 2022. The primary endpoint was the risk of secondary abdominal closure. Variables analyzed included pretransplant status, perioperative and postoperative data.

Results: A total of 664 PLT recipients were identified, of which 58 required secondary abdominal closure (8.7%). Most patients had biliary atresia (n=412, 62.0%), followed by metabolic diseases (n=78, 11.7%). Statistical difference were found in donor gender (p=0.020) and the recipient-to-donor body weight ratio (RDBW), which was lower in the secondary closure group (0.1±0.1 vs. 0.2±0.27; p=0.001), lower in secondary closure. The mean hospital and intensive care unit (ICU) stay after PLT was significantly longer in the intervention group compared to those with primary abdominal closure (24.4±20.4 days vs. 12.5±13.1 days, p<0.001). Multivariable Cox regression analysis identified male donor as an independent risk factor for secondary abdominal closure (hazard ratio 1.9, p=0.030).

Conclusions: Patients requiring secondary closure were smaller, had a lower RDBW, and received grafts with a higher graft-to-recipient weight ratio (GRWR), Graft size modulation and secondary abdominal closure are currently the techniques used to prevent compartment syndrome in PLT, particularly for children with low body weight.