Inhibition of Notch3/Hey1 ameliorates peribiliary hypoxia by preventing hypertrophic hepatic arteriopathy in biliary atresia progression.

Emerging evidence indicates the presence of vascular abnormalities and ischemia in biliary atresia (BA), although specific mechanisms remain undefined. This study examined both human and experimental BA. Structural and hemodynamic features of hepatic arteries were investigated by Doppler ultrasound, indocyanine green angiography, microscopic histology, and invasive arterial pressure measurement. Opal multiplex immunohistochemistry, western blot, and RT-PCR were applied to assess Notch3 expression and the phenotype of hepatic arterial smooth muscle cells (HASMCs). We established animal models of Notch3 inhibition, overexpression, and knockout to evaluate the differences in overall survival, hepatic artery morphology, peribiliary hypoxia, and HASMC phenotype. Hypertrophic hepatic arteriopathy was evidenced by an increased wall-to-lumen ratio and clinically manifested as hepatic arterial hypertension, decreased hepatic artery perfusion, and formation of hepatic subcapsular vascular plexuses (HSVPs). We observed a correlation between overactivation of Notch3 and phenotypic disruption of HASMCs with the exacerbation of peribiliary hypoxia. Notch3 signaling mediated the phenotype alteration of HASMCs, resulting in arterial wall thickening and impaired oxygen supply in the portal microenvironment. Inhibition of Notch3/Hey1 ameliorates portal hypoxia by restoring the balance of contractile/synthetic HASMCs, thereby preventing hypertrophic arteriopathy in BA.

Current trends and prospects of surgical techniques for hepatoblastoma.

Hepatoblastoma, a common extracranial malignant solid tumor in childhood, is often detected at an advanced stage and is difficult to treat surgically. Despite the availability of multiple comprehensive treatments that can be combined with surgery, hepatoblastoma treatment outcomes remain poor. Surgery is the main treatment strategy for hepatoblastoma, but it faces many challenges, including tumor attachment to surrounding tissues, tumor wrapping or invading of vital organs and tissues, the presence of giant or multiple tumors, distant metastasis, the formation of a tumor thrombus, and significant surgical trauma. In this review, we discuss recent research advances and propose potential strategies for overcoming these challenges. Such strategies may improve the rate of hepatoblastoma resection and local control in children, as well as reduce complications and trauma.

Comparison of robotic versus thoracoscopic repair for congenital esophageal atresia: a propensity score matching analysis.

BACKGROUND: Despite the rapid advancement of robotic surgery across various surgical domains, only cases of robotic repair (RR) for neonates with esophageal atresia (EA) have been reported. Comprehensive studies comparing RR and thoracoscopic repair (TR) are lacking. The authors aimed to compare the safety and efficacy of RR and TR for EA. METHODS: A retrospective multicenter study was conducted on 155 EA neonates undergoing RR (79 patients) or TR (76 patients) between August 2020 and February 2023 using propensity score matching. Asymmetric port distribution and step-trocar insertion techniques were applied during RR. Demographics and surgical outcomes were compared. RESULTS: After matching, 63 patients (out of 79) in RR group and 63 patients (out of 76) in TR group were included. There were no significant differences in short-term outcomes between two groups, except for longer total operative time (173.81 vs. 160.54 min; P <0.001) and shorter anastomotic time (29.52 vs. 40.21 min; P <0.001) in RR group. Compared with TR group, the RR group had older age at surgery (8.00 vs. 3.00 days; P <0.001), but a comparable pneumonia rate. More importantly, the incidence of anastomotic leakage (4.76 vs. 19.05%, P =0.013), anastomotic stricture (15.87 vs. 31.74%, P =0.036) within 1 year postoperatively, and unplanned readmission (32.26 vs. 60.00%, P =0.030) within 2 years postoperatively were lower in RR group than in TR group. CONCLUSIONS: RR is a technically safe and effective option for EA patients. This approach delays the age of surgery without increasing respiratory complication rates while reducing the incidence of postoperative anastomotic complications and unplanned readmission.

Robotic-Assisted Proctosigmoidectomy vs. Laparoscopic-Assisted Soave Pull-Through for Hirschsprung's Disease: Medium-Term Outcomes From a Prospective Multicenter Study.

OBJECTIVE: To compare the surgical outcomes of robotic-assisted proctosigmoidectomy (RAP) and laparoscopic-assisted Soave pull-through (LAP) for children with Hirschsprung's disease (HD). SUMMARY BACKGROUND DATA: LAP and RAP have been developed for minimally invasive pull-through of HD, but the clinical benefits of robotic-assisted versus laparoscopic-assisted approaches have yet to be proven in a multicenter prospective study. METHODS: This study was a prospective multicenter clinical trial conducted on children with rectosigmoid/descending HD from July 2015 to June 2022, with registration in the Chinese Clinical Trial Registry (ChiCTR2000035220). The primary outcome was the medium-term functional outcomes in children aged ≥4 years based on bowel functional scores, which were assessed and compared between LAP and RAP. RESULTS: A total of 328 consecutive patients (RAP=165, LAP=163) were approached who were considered eligible for elective minimally invasive endorectal pull-through, and 219 patients aged ≥4 years of age completed follow-up (RAP=109, LAP=110). The transanal dissection length and anal traction time were significantly shorter in RAP than those in LAP (0.30 cm vs. 3.70 cm, P <0.001; 45 min vs. 62 min, P <0.001). The RAP group had significantly lower urinary retention rate (0% vs. 5.52%, P=0.006), while other short-term results between two groups were not significantly different. The medium-term overall BFS scores were comparable between two groups; however, among the subgroup of children aged ≤ 3 months at surgery, the RAP group had better anal canal resting pressure at one year postoperatively and amounted to better annual POFC scores at 4-7 years old postoperatively (all P <0.05). CONCLUSIONS: RAP and LAP should have similar medium-term bowel functional outcomes in HD children, but RAP may be associated with a slight functional benefit in infants operated on below age 3 months, requiring further investigation in larger case cohorts.

Laparoscopic lateral duodenojejunostomy for pediatric superior mesenteric artery compression syndrome: a cohort retrospective study.

PURPOSE: There are only a few case reports of laparoscopic lateral duodenojejunostomy (LLDJ) in children with Wilkie's syndrome, also known as superior mesenteric artery compression syndrome (SMAS). We aimed to describe our laparoscopic technique and evaluate its outcomes for SMAS in children. METHODS: From January 2013 to May 2021, SMAS children who received LLDJ were included. The procedure was carried out utilizing the four-trocar technique. The elevation of the transverse colon allows good exposure of the dilated and bulging second and third sections of the duodenum. Using a linear stapler, we established a lateral anastomosis connecting the proximal jejunum with the third part of the duodenum. Following that, a running suture was used to intracorporeally close the common enterotomy. Clinical data on patients was collected for analysis. The demographics, diagnostic findings, and postoperative outcomes were analyzed retrospectively. RESULTS: We retrospectively analyzed 9 SMAS patients (6 females and 3 males) who underwent LLDJ, aged between 7 and 17 years old. The mean operative time was 118.4 ± 16.5 min and the mean estimated blood loss was 5.6 ± 1.4 ml. There were no conversion, intraoperative complications or immediate postoperative complications. The mean postoperative hospital stay was 6.8 ± 1.9 days and the mean follow-up time was 5.4 ± 3.0 years. During follow-up, seven patients (77.8%) experienced complete recovery of symptoms prior to surgery. One patient (11.1%) still had mild vomiting, which resolved with medication. Another patient (11.1%) developed psychological-induced nausea, which significantly improved after treatment with education, training and diet management. CONCLUSIONS: LLDJ represents a feasible and safe treatment option for SMAS in well-selected children. Further evaluation with more cases and case-control studies is required for the real benefits.

Cost-effectiveness analysis of robot-assisted laparoscopic surgery for complex pediatric surgical conditions.

BACKGROUND: Robotics has been used safely and successfully in a variety of adult surgeries and is gradually gaining ground in pediatrics. While the benefits of robotic-assisted surgery in disease treatment are well recognized, its high cost has led to questions. To investigate whether robotic-assisted laparoscopic surgery (RALS) is cost-effective compared to conventional laparoscopic surgery (LS) in pediatric surgery, we attempted to construct a model to perform an analysis of these two surgical approaches using Python statistical analysis software. METHODS: We selected four common complex pediatric surgical conditions (choledochal cyst, Hirschsprung's disease, vesicoureteral reflux, and congenital hydronephrosis) from three systems (pediatric hepatobiliary, gastroenterology, and urology). Models were constructed using Python statistical software to compare hospital costs and surgical outcomes for RALS and LS. In addition, we performed a preferred strategy analysis for both surgical modalities while assessing model uncertainty using one-way sensitivity analysis. RESULTS: For the four diseases, the operative time decreased sequentially. The total inpatient costs of RALS were 10,816.72, 9145.44, 8414.29, 7973.58 dollars, respectively, yielding 1.789, 1.712, 1.749, 1.792 quality adjustment life years (QALYs) over two years post-operatively. The incremental cost of RALS relative to LS for each disease was 3523.44, 3200.20, 3049.79, 3043.66 dollars, respectively, with an incremental utility of 0.060, 0.054, 0.051, 0.050 QALYs. The incremental cost-effectiveness ratios (ICERs) for RALS for each of the four diseases were 58,724.01, 59,262.95, 59,799.79, 60,873.20 dollars/QALY, all less than 100,000 dollars/QALY. The cost of robot consumables was the main incremental cost of RALS and had the most significant impact on the model. CONCLUSION: For the four pediatric surgical conditions described above, RALS has higher inpatient costs than LS, but it has better postoperative outcomes, and all four RALS treatments are cost-effective. Children with complex diseases and long operative times appear to benefit more from RALS.

Robotic-assisted proctosigmoidectomy for Hirschsprung's disease: A multicenter prospective study.

BACKGROUND: Robotic surgery is a cutting-edge minimally invasive technique that overcomes many shortcomings of laparoscopic techniques, yet few studies have evaluated the use of robotic surgery to treat Hirschsprung's disease (HSCR). AIM: To analyze the feasibility and medium-term outcomes of robotic-assisted proctosigmoidectomy (RAPS) with sphincter- and nerve-sparing surgery in HSCR patients. METHODS: From July 2015 to January 2022, 156 rectosigmoid HSCR patients were enrolled in this multicenter prospective study. Their sphincters and nerves were spared by dissecting the rectum completely from the pelvic cavity outside the longitudinal muscle of the rectum and then performing transanal Soave pull-through procedures. Surgical outcomes and continence function were analyzed. RESULTS: No conversions or intraoperative complications occurred. The median age at surgery was 9.50 months, and the length of the removed bowel was 15.50 ± 5.23 cm. The total operation time, console time, and anal traction time were 155.22 ± 16.77, 58.01 ± 7.71, and 45.28 ± 8.15 min. There were 25 complications within 30 d and 48 post-30-d complications. For children aged ≥ 4 years, the bowel function score (BFS) was 17.32 ± 2.63, and 90.91% of patients showed moderate-to-good bowel function. The postoperative fecal continence (POFC) score was 10.95 ± 1.04 at 4 years of age, 11.48 ± 0.72 at 5 years of age, and 11.94 ± 0.81 at 6 years of age, showing a promising annual trend. There were no significant differences in postoperative complications, BFS, and POFC scores related to age at surgery being ≤ 3 mo or > 3 mo. CONCLUSION: RAPS is a safe and effective alternative for treating HSCR in children of all ages; it offers the advantage of further minimizing damage to sphincters and perirectal nerves and thus providing better continence function.

The interplay of common genetic variants NRG1 rs2439302 and RET rs2435357 increases the risk of developing Hirschsprung's disease.

Introduction: As a congenital and genetically related disease, many single nucleotide polymorphisms (SNPs) have been reported to be associated with the risk of HSCR. Our previous research showed that SNP rs2439302 (NRG1) interacted with rs2435357 (RET) to increase the risk of HSCR development. However, the underlying molecular mechanism is still not well understood. Methods: SNP rs2439302 (NRG1) and rs2435357 (RET) were genotyped in 470 HSCR cases. The expression of NRG1 and RET was investigated in the colon of HSCR patients. Knockdown of the NRG1 and RET homologs was performed in zebrafish to investigate their synergistic effect on ENS development. The effect of SNP rs2439302 and rs2435357 polymorphism on neuron proliferation, migration, and differentiation were investigated in SHSY-5Y cells and IPSCs. Results: Significant downregulation of NRG1 and RET expression was noticed in the aganglionic segment of HSCR patients and SHSY-5Y cells with rs2439302 GG/rs2435357 TT genotype. NRG1 and RET double mutants caused the most severe reduction in enteric neuron numbers than NRG1 single mutant or RET single mutant in the hindgut of zebrafish. SHSY-5Y cells and IPSCs with rs2439302 GG/rs2435357 TT genotype exhibited a decreased proliferative, migration, and differentiative capacity. CTCF showed a considerably higher binding ability to SNP rs2439302 CC than GG. NRG1 reduction caused a further decrease in SOX10 expression via the PI3K/Akt pathway, which regulates RET expression by directly binding to rs2435357. Discussion: SNP rs2439302 (NRG1) GG increases the risk of developing HSCR by affecting the binding of transcription factor CTCF and interacting with rs2435357 (RET) to regulate RET expression via the PI3K/Akt/SOX10 pathway.

Robotic-assisted surgery in the pediatric surgeons' world: Current situation and future prospectives.

Robotic-assisted surgery has been fully embraced by surgeons for the adult population; however, its acceptance is too slow in the world of pediatric surgeons. It is largely due to the technical limitations and the inherent high cost associated with it. In the past two decades, indeed, there has been considerable advancement in pediatric robotic surgery. A large number of surgical procedures were performed on children with the assistance of robots, even with comparative success rates to standard laparoscopy. As a newly developing field, it still has many challenges and obstacles. This work is centered on the current status and progression of pediatric robotic surgery as well as the future perspectives in the field of pediatric surgery.

Update on the Pathogenesis of the Hirschsprung-Associated Enterocolitis.

Despite the significant progress that has been made in terms of understanding the pathophysiology and risk factors of Hirschsprung-associated enterocolitis (HAEC), the morbidity rate has remained unsatisfactorily stable, and clinical management of the condition continues to be challenging. Therefore, in the present literature review, we summarized the up-to-date advances that have been made regarding basic research on the pathogenesis of HAEC. Original articles published between August 2013 and October 2022 were searched in a number of databases, including PubMed, Web of Science, and Scopus. The keywords "Hirschsprung enterocolitis", "Hirschsprung's enterocolitis", "Hirschsprung's-associated enterocolitis", and "Hirschsprung-associated enterocolitis" were selected and reviewed. A total of 50 eligible articles were obtained. The latest findings of these research articles were grouped into gene, microbiome, barrier function, enteric nervous system, and immune state categories. The present review concludes that HAEC is shown to be a multifactorial clinical syndrome. Only deep insights into this syndrome, with an accrual of knowledge in terms of understanding its pathogenesis, will elicit the necessary changes that are required for managing this disease.

Validation and effect of the NASA-TLX score on the assessment of the workload of pediatric robotic operations.

OBJECTIVE: The objective of the study was to assess the validity of the NASA-TLX score in rating the workload of pediatric robotic operations. METHODS: The workload of 230 pediatric gastrointestinal and thoracic robotic operations was rated using the NASA-TLX score. The difference between the high workload group and the low workload group in each subscale of the NASA-TLX score was analyzed. The correlation of each subscale with the total workload score in the high workload group and low workload group was also analyzed. A logistic regression analysis was subsequently conducted to assess the effects of different factors (sex, age, weight, procedure duration, procedure specialties, combined malformation and blood loss) on the workload. RESULTS: The average NASA-TLX score was 56.5 ± 5.1 for the total group, 56.9 ± 5.0 for the gastrointestinal group and 54.6 ± 4.8 for the thoracic group, p = 0.007. The score of the high workload group was 62.7 ± 3.2, while it was 50.6 ± 2.7 for the low workload group (p < 0.001). The score on each subscale was also significantly different between the high and low workload groups. In the high workload group, a stronger correlation was observed between the total score and TD and Fr and a lower correlation with MD and Pe. In the low workload group, all six subscales showed a moderate correlation with the total score. A multivariate logistic regression analysis revealed that the procedure duration was an independent influencing factor for a higher workload score. CONCLUSIONS: NASA-TLX is a valid tool to rate the surgeon's workload in pediatric robotic surgery. A longer operative time contributes to a higher workload.

Robotic-assisted Kasai portoenterostomy for biliary atresia.

BACKGROUND: Robotic-assisted Kasai portoenterostomy (RAKPE) has been utilized to treat biliary atresia (BA). However, RAKPE is not widely performed and its efficacy remains unknown. We summarized the experience of RAKPE for BA and determined its efficacy. MATERIALS AND METHODS: We retrospectively analyzed 25 consecutive infants with non-syndromic type III BA who received RAKPE in our center from January 2020 to July 2021. RAKPE is a three-arm setup and four-trocar operation. Bipolar coagulation was used to dissect the small blood vessels at the hepatic portal. The fibrous cone was shallowly transected with bending electric scissors, followed by gelatin sponge compression to staunch the hemorrhage. Finally, a wide anastomosis was accurately constructed. Demographics and outcomes were recorded. RESULTS: The mean operative time was 211.64 ± 18.93 min. No conversion to laparotomy or intraoperative complications occurred. The mean estimated blood loss was 7.64 ± 2.43 mL. Enteral feeding was resumed after 3.44 ± 1.23 days. All patients achieved bile excretion postoperatively, and dark green bile-stained stools were passed 1.50 days (range 1.00-3.00 days) after surgery. The average postoperative length of hospital stay was 10.32 ± 2.59 days. The jaundice clearance (JC) rate was 76.00% within 6 months after surgery and the incidence of cholangitis was 48.00% within 1 year following surgery. The survival with native liver (SNL) rate was 80.00% at 1 year and 66.67% at 2 years. CONCLUSION: RAKPE can be regarded as a treatment option for patients with BA due to the good outcomes reported. However, long-term studies comparing open or laparoscopic approaches are needed.

Robotic resection of paediatric solid tumours at a single institution.

BACKGROUND: To explore the clinical application of robotic-assisted surgery in paediatric solid tumours and to explore its feasibility. METHODS: From 2015 to 2022, 53 children with solid tumours underwent robotic-assisted surgery in our centre were retrospectively analysed. RESULTS: The mean weight of the patients was 27.7 kg, and the mean age was 6.7 years. The average tumour volume was 5.5*4.6*3.7 cm. Two procedures (3.8%) were converted. The mean total operative time was 198.5 min. The mean estimated blood loss was 27.1 ml, and no intraoperative complications occurred. Two (3.8%) patients had postoperative complications. At a median follow-up of 21.2 months, one (1.9%) patient with malignant tumours stopped treatment, and two (3.8%) patients developed tumour recurrence. CONCLUSIONS: Robotic-assisted tumour resection is feasible in highly selected cases of young age, light weight, huge tumour or malignant tumour.

Comparison of efficacy and safety of robotic surgery and laparoscopic surgery for choledochal cyst in children: a systematic review and proportional meta-analysis.

BACKGROUND: Most commonly, cyst excision and Roux-en-Y hepaticojejunostomy reconstruction are the optimal treatment for choledochal cysts (CC). Robotic surgery (RS) is being conducted with increasing frequency to treat CC. It is unclear whether RS can overcome the limitations of laparoscopic surgery (LS) and improve the prognosis of patients. In terms of efficacy, evidence concerning which minimally invasive surgery is preferred is, however, sparse. Our objective is to further compare the efficacy of RS and LS in children with CC and draw a useful clinical conclusion. METHODS: Studies meeting inclusion criteria were identified from a series of databases, consisting of PubMed, Embase, Scopus, Web of Science, the Cochrane Library and their reference list of articles up to May 2022. Eligible articles comprised at least five objects that were younger than 18 years of age and the language was limited to English. Two authors independently evaluated selected studies and extracted data for analysis. RESULTS: Forty studies were selected for analysis, with thirty-six reporting data on LS and eight containing data on RS. The pooled conversion rate and pooled postoperative complication rate of RS were lower than those of LS, but none of them was statistically significant. Moreover, comparisons of the following detailed postoperative complication rates were not statistically significant, such as intestinal obstruction or ileus, anastomotic bleeding, anastomotic or bile leakage, and anastomotic stenosis. However, the intraoperative blood loss and the postoperative hospital stay in RS group were significantly lower than those in LS group. CONCLUSIONS: RS is a safe and feasible option for children with CC. Further studies with more cases, long-term efficacy and health economics analysis are needed to confirm whether RS is more advantageous.

Robotic-assisted choledochal cyst excision with Roux-en-Y hepaticojejunostomy in children: does age matter?

BACKGROUND: Robotic-assisted surgery (RAS) is being increasingly used in pediatric choledochal cysts (CCs), but is most commonly performed in older children and adolescents. The outcomes in young infants remain to be explored. The purpose of this study is to compare outcomes in infants aged ≤ 1 year with an older cohort. METHODS: From July 2015 to January 2020, a retrospective study was conducted to evaluate the RAS in patients with CCs at our institution. Patients were divided into two groups (group A ≤ 1 year old and group B > 1 year old). Demographics, intraoperative details, complications, and outcomes were analyzed. RESULTS: A total of 79 patients were included in the study (28 patients in group A and 51patients in group B). The median age of patients at the surgery in group A was 4.9 months (IQR: 3.1-9.1), compared with 46.8 months (IQR: 28.5-86.5) in group B. Three patients in group A were neonates. No conversion to open surgery was required. No significant differences were found between the two groups including sex, Todani type, or diameter of the cysts. The diameter of the common hepatic duct was smaller in group A (6.0 ± 1.7 vs. 9.0 ± 3.0 mm; p < 0.001). Group A had the longer hepaticojejunostomy time [51(44-58) vs. 42(38-53) min; p = 0.013], while Group B had the longer cyst excision time [43(41-59) vs. 50(43-60) min; p = 0.005]. However, their total operative time and console time were similar. There were no statistical differences in length of hospital stay and complications between the two groups. CONCLUSIONS: Robot-assisted cyst resection and hepaticojejunostomy are feasible and safe in infants ≤ 1 year old. Age cannot be considered an absolute contraindication for robotic surgery in patients with CCs.

Robotic approach for pediatric pulmonary resection: preliminary investigation and comparative study with thoracoscopic approach.

Background: Minimal invasive pulmonary resection in children is challenging, irrespective of whether it is conducted using a robot or a thoracoscope. This study presents the preliminary results of pediatric robotic pulmonary resection (RPR) and comparison outcomes with conventional thoracoscopic pulmonary resection (TPR). Methods: This is a retrospective study conducted in patients underwent RPR (RPR group; n=30) and TPR (TPR group; n=44). The clinical data, including operative time, post-operative body temperature, surgical complications, surgeon's workload (by NASA-TLX), postoperative hospital stay, and scar score (using the SCAR scale), of both the RPR and TPR groups were collected and compared. Results: Both groups had similar age and weight. The youngest patient belonged to the RPR group and was 6 months old and weighed 8 kg. One case in the RPR group and two in the TPR group were converted to thoracotomy. RPR had a longer total operative time (148.3±36.8 min), but a shorter pure operative time (103.9±28.5 min) than those of the TPR group [118.3±22.5 (P<0.001) and 111.4±18.3 min (P=0.045), respectively]. Compared to the TPR group, fewer patients in the RPR group reported fever postoperatively (2/29 vs. 11/42, P=0.039). The workload of the surgeons was also lower in the RPR group (55.2±4.7 vs. 62.9±6.0, P<0.01). No significant difference was observed in perioperative complications, drainage length, postoperative hospital stays, and scar score of the two groups. Conclusions: The safety and effectiveness of the robotic approach are similar to those of the thoracoscopic surgery for pediatric pulmonary resection in children heavier than 8 kg. In addition, the robotic approach shows improved operative dissection efficiency and accuracy for patients and reduced workload for surgeons. Hence, it is beneficial to both surgeons and patients.

Sternum length norms in Han youngsters in central China.

The sternum is one of the most important components of the chest wall. However, to the best of our knowledge, at present there is no reference value of the sternum length in normal Han Chinese children that has been published in the literature. The aim of the present study was therefore to establish the reference value of sternum length in a normal Han Chinese population of different ages and sex groups. Chest computed tomography scans and three-dimensional reconstruction images of 1,080 individuals who were younger than 18 years old and without congenital or acquired structural anomalies or congenital metabolic diseases were retrospectively reviewed. The length of the sternum was measured for each individual and comparisons of the sternum length according to sex were conducted using the Mann-Whitney test. Age or region group comparisons were performed using the ANOVA test, and the association between the length of the sternum and age was assessed by regression analysis. A significant association between the age (x) of the individual and the length of the sternum (y) was confirmed, although different regression patterns were identified for the sexes (the regression equation for males was y=5.616x+60.408; P<0.001; R2=0.890, whereas that for females was y=-0.134x2+6.543x+56.805; P<0.001, R2=0.890). No significant differences in sternum length were identified between the sexes for subjects aged 1-13 years old, whereas significant differences were observed comparing the sexes of the subjects aged 14-18 years old. In conclusion, the present study revealed that the length of the sternum in normal Han Chinese children can be precisely estimated by the age of the child using our formulae for the different sexes (where the most-fit formulae for the two sexes are different). This knowledge can be applied clinically in chest wall deformity assessment, and in surgery planning.

Dynamic analysis of serum MMP-7 and its relationship with disease progression in biliary atresia: a multicenter prospective study.

PURPOSE: We aimed to assess the dynamic changing trend of serum matrix metalloproteinase-7 (MMP-7) in biliary atresia (BA) patients from diagnosis to LTx to further elucidate its clinical value in diagnosis and prognoses and its relationship with disease progression. METHODS: In this multicentre prospective study, 440 cholestasis patients (direct bilirubin level of > 17 µmol/L) were enrolled. Serum MMP-7 levels were measured using an enzyme-linked immunosorbent assay at diagnosis, 1 week, 2 weeks, 1 month, 6 weeks, 2 months, 3 months, 6 months and then every 6 months post-KPE. The medical record at each follow-up visit for post-Kasai portoenterostomy patient was collected and analyzed. RESULTS: Using a cut-off value of > 26.73 ng/mL, serum MMP-7 had an AUC of 0.954 in BA neonates and 0.983 in BA infants. A genetic mutation (G137D) was associated with low MMP-7 levels in serum of BA patients. MMP-7 showed a mediation effect on the association between inflammation and liver fibrosis in BA patients. Four dynamic patterns of serum MMP-7 post-KPE were associated with prognosis. Serum MMP-7 was the only significant predictor at 6 weeks post-KPE and the most accurate predictor at 3 months post-KPE of survival with the native liver in 2 years. CONCLUSION: As one of the critical factors associated with BA occurrence and progression, serum MMP-7 can be used for early diagnosis of BA and post-KPE MMP-7 level is the earliest prognostic biomarker so far.

Re-evaluation of Laparoscopic Hepatic Subcapsular Spider-Like Telangiectasis Sign: A Highly Accurate Method to Diagnose Biliary Atresia in Infants.

Objective: Operative cholangiography, the gold standard for the diagnosis of biliary atresia (BA), is being challenged due to an increase in the studies of misdiagnosis. A previous study has shown that the laparoscopic hepatic subcapsular spider-like telangiectasis (HSST) sign was accurate for diagnosing BA. This study aims to compare the performance of the HSST sign with cholangiography in the identification of BA. Methods: We prospectively screened consecutive infants with cholestasis who underwent laparoscopic exploration in this multicenter study. Demographics, intraoperative findings (videos and images), and outcomes were retrospectively analyzed. The data of the HSST sign and cholangiography were compared according to the final diagnosis. Then, the diagnostic accuracy of the BA using the HSST sign and cholangiography was validated in other independent cohorts. Results: A total of 2,216 patients were enrolled in this study. The sensitivity and negative predictive values were both 100% for diagnosing BA based on the HSST sign and cholangiography. The specificity, negative predictive value, and accuracy of the HSST sign (97.2, 99.2, 99.3%) in discriminating BA were significantly higher than operative cholangiography (81.6, 94.9, 95.8; p < 0.001). Moreover, to realize the early diagnosis of BA, the accuracy of the HSST sign in identifying BA was better than cholangiography in the subgroup of neonates (98.7% vs. 95.0%; p = 0.032). Interestingly, 92 non-BA patients without the HSST sign had positive cholangiography. Among them, 28 infants had negative cholangiography when the common bile duct was compressed and 39 patients displayed visible bile ducts due to repeated postoperative biliary irrigation. The other 25 patients (18 with the Alagille syndrome, 5 with progressive familial intrahepatic cholestasis, and 2 with the neonatal hepatitis syndrome) had consistently positive cholangiography. In the independent validation cohort, the diagnostic accuracy of the HSST sign (99.2%) was higher than cholangiography (95.0%, p = 0.012). Conclusion: The laparoscopic HSST sign is superior to cholangiography in the diagnosis of BA in the infants with cholestasis and has advantages in early diagnosis. This method is expected to become a novel shift for diagnosing BA during ongoing laparoscopy.

Laparoscopic simultaneous inguinal hernia repair and appendectomy in children: A multicenter study.

BACKGROUND: Inguinal hernias (IHs) are sometimes encountered incidentally in children during laparoscopic appendectomy. This study aims to evaluate the efficacy and outcomes of laparoscopic simultaneous inguinal hernia repair and appendectomy in children. METHODS: A multicentric study was performed in patients with AA and concurrent IH who received laparoscopic simultaneous inguinal hernia repair and appendectomy (study group), compared with patients who underwent two-stage laparoscopic procedures (control group) between September 2012 and January 2020. Intraoperative data, postoperative complications, and clinical outcomes were prospectively collected and retrospectively analyzed. RESULTS: 189 patients with AA and concurrent IH (117 children in the study group, and 72 children in the control group) were enrolled. No significant differences in preoperative characteristics were identified between the two groups. Patients in the study group had a shorter total operative time and hospital stay than those in the control group (43.2 ±â€¯8.1 vs 53.9 ±â€¯7.3 min, p < 0.001; 1.5 ±â€¯0.8 vs 2.2 ±â€¯0.9 days, p = 0.023). The study group incurred lower costs than the control group (9198.7 ±â€¯587.6 vs 14,392.5 ±â€¯628.6 RMB, p < 0.001). During follow-up (range 1.5-6.0 years), three children in the study group and two children in the control group experienced wound infection. One child in the study group had recurrent IH. CONCLUSIONS: Laparoscopic simultaneous procedures do not increase the incidence of wound infection or recurrent IH. Moreover, they avoid repeat anesthesia and hospitalization. Therefore, this approach is safe, feasible and cost-effective for children with AA and concurrent IH. LEVEL OF EVIDENCE: Level III.