Relationships of serum MMP-7 and clinical characteristics in choledochal cyst children.

BACKGROUND: Matrix metalloproteinase-7 (MMP-7) is associated with biliary injury. This study aimed to evaluate the relationships of serum MMP-7 with clinical characteristics in choledochal cysts (CDC) children. METHODS: Between June 2020 and July 2022, we conducted a prospective study of CDCs who underwent one-stage definitive operation at our center. Serum MMP-7 was measured using an enzyme-linked immunosorbent assay. We evaluated the relationships between serum MMP-7 and age, laboratory tests, imaging examinations, liver fibrosis, MMP-7 expression, and perforation. RESULTS: A total of 328 CDCs were enrolled in the study, with a median serum MMP-7 of 7.67 ng/mL. Higher serum MMP-7 was correlated with younger age at diagnosis (p < 0.001), larger cyst sizes (p < 0.001), higher liver fibrosis stages (p < 0.001), and higher incidence of perforation (p < 0.01). Liver MMP-7 was mainly expressed in intrahepatic and extrahepatic biliary epithelial cells. The area under the receiver operating characteristic curve (AUROC) was 0.630 (p < 0.001) for serum MMP-7 in predicting perforation. When serum MMP-7 was combined with γ-glutamyl transferase (GGT), the AUROC increased to 0.706 (p < 0.001). CONCLUSIONS: Serum MMP-7 was associated with biliary obstruction in CDCs. Patients with high serum MMP-7 were more likely to have severe liver damage and biliary injury, with higher incidences of liver fibrosis and perforation.

The critical roles of m6A RNA methylation in lung cancer: from mechanism to prognosis and therapy.

Lung cancer, a highly malignant disease, greatly affects patients' quality of life. N6-methyladenosine (m6A) is one of the most common posttranscriptional modifications of various RNAs, including mRNAs and ncRNAs. Emerging studies have demonstrated that m6A participates in normal physiological processes and that its dysregulation is involved in many diseases, especially pulmonary tumorigenesis and progression. Among these, regulators including m6A writers, readers and erasers mediate m6A modification of lung cancer-related molecular RNAs to regulate their expression. Furthermore, the imbalance of this regulatory effect adversely affects signalling pathways related to lung cancer cell proliferation, invasion, metastasis and other biological behaviours. Based on the close association between m6A and lung cancer, various prognostic risk models have been established and novel drugs have been developed. Overall, this review comprehensively elaborates the mechanism of m6A regulation in the development of lung cancer, suggesting its potential for clinical application in the therapy and prognostic assessment of lung cancer.

Quinpirole ameliorates nigral dopaminergic neuron damage in Parkinson's disease mouse model through activating GHS-R1a/D2R heterodimers.

Growth hormone secretagogue receptor 1a (GHS-R1a) is an important G protein-coupled receptor (GPCR) that regulates a variety of functions by binding to ghrelin. It has been shown that the dimerization of GHS-R1a with other receptors also affects ingestion, energy metabolism, learning and memory. Dopamine type 2 receptor (D2R) is a GPCR mainly distributed in the ventral tegmental area (VTA), substantia nigra (SN), striatum and other brain regions. In this study we investigated the existence and function of GHS-R1a/D2R heterodimers in nigral dopaminergic neurons in Parkinson's disease (PD) models in vitro and in vivo. By conducting immunofluorescence staining, FRET and BRET analyses, we confirmed that GHS-R1a and D2R could form heterodimers in PC-12 cells and in the nigral dopaminergic neurons of wild-type mice. This process was inhibited by MPP+ or MPTP treatment. Application of QNP (10 µM) alone significantly increased the viability of MPP+-treated PC-12 cells, and administration of quinpirole (QNP, 1 mg/kg, i.p. once before and twice after MPTP injection) significantly alleviated motor deficits in MPTP-induced PD mice model; the beneficial effects of QNP were abolished by GHS-R1a knockdown. We revealed that the GHS-R1a/D2R heterodimers could increase the protein levels of tyrosine hydroxylase in the SN of MPTP-induced PD mice model through the cAMP response element binding protein (CREB) signaling pathway, ultimately promoting dopamine synthesis and release. These results demonstrate a protective role for GHS-R1a/D2R heterodimers in dopaminergic neurons, providing evidence for the involvement of GHS-R1a in PD pathogenesis independent of ghrelin.

Risk factors analysis for clinical symptoms of prenatally diagnosed choledochal cysts: a retrospective study.

BACKGROUND: This study aimed to screen the impact factors for clinical symptoms of prenatally diagnosed choledochal cysts (CDCs), to warn about the occurrence of clinical symptoms and the timing of surgery. METHODS: Medical records of patients with prenatally diagnosed CDCs admitted to our hospital from April 2013 to April 2018 were retrospectively reviewed. Fetal hilar or abdominal cysts were found by prenatal ultrasonogram. All patients underwent laparoscopic cyst excision and hepaticojejunostomy in our center. Univariate analysis and multivariate logistic regression analysis were performed to screen the factors related to clinical symptoms intimately. RESULTS: Two hundred eighteen cases were included. One hundred thirty-four patients (134/218, 61.5%) presented clinical symptoms before surgery. The results of univariate analysis showed that patients with clinical symptoms had earlier time of prenatal diagnosis (P = 0.002), higher values of GGT, TBIL, DBIL (P < 0.001, P < 0.001, P < 0.001, respectively) and larger maximum diameter of cyst before surgery (P = 0.012). Multivariate logistic regression analysis suggested that the time of prenatal diagnosis (P = 0.001, OR = 0.898, 95% CI: 0.845 ~ 0.955) and the GGT value within one week of life (P = 0.028, OR = 1.002, 95% CI: 1.000 ~ 1.003) were independent influencing factors for symptoms. CONCLUSIONS: For children with prenatally diagnosed CDCs, approximately 2/3 patients presented noticeable clinical symptoms before surgery. The time of prenatal diagnosis and the GGT value within 1 week of life were independent impact factors for the occurrence of clinical symptoms.

Development and validation of non-invasive models in predicting advanced fibrosis of choledochal cyst.

PURPOSE: Patients with choledochal cyst (CDC) develop liver fibrosis, especially advanced fibrosis without prompt surgery. This study validated the aspartate aminotransferase (AST)-to-platelet ratio index (APRI) and Fibrosis-4 index (FIB-4) and constructed a model for predicting advanced fibrosis in pediatric CDCs. METHODS: Between January 2020 and March 2022, 330 CDCs (advanced fibrosis: 34, Ludwig staging 3-4; non-advanced fibrosis: 296, Ludwig staging 0-2) were reviewed. APRI and FIB-4 were validated. The area under the receiver operating characteristic (AUROC) curve was used to assess discrimination. Relevant variables were analyzed by backward stepwise logistic regression. Enhanced bootstrap method was used for internal verification with 1000 samples. RESULTS: The AUROCs of APRI and FIB-4 were 0.761 (0.673-0.850) and 0.561 (0.455-0.667). AST to prealbumin ratio (APAR), was constructed with an AUROC of 0.776 (0.693-0.860). The AUROCs of APAR + APRI and APAR + FIB-4 were 0.791 (0.713-0.869) and 0.782 (0.699-0.865). No significant differences were noted in the AUROCs of the indices or their combinations. APAR and APRI could be used together to reduce missed diagnosis rate. The risk of advanced fibrosis varied from different APAR and APRI scores. CONCLUSION: Both APAR and APRI were indispensable to identify CDC patients at high risk of advanced fibrosis.

Correlation of ectopic distal location of papilla of Vater and clinical characteristics in pediatric choledochal cysts.

PURPOSE: Ectopic distal location of papilla of Vater (EDLPV) is an obvious pathological feature of choledochal cyst (CDC). This study aimed to investigate the correlation between EDLPV and clinical characteristics of CDCs. METHODS: Three groups were studied: Group 1 (G1), papilla in the middle third of second part of duodenum (n = 38); Group 2 (G2), papilla from the distal third of second part to the beginning of third part of duodenum (n = 168); Group 3 (G3), papilla from the middle of third part to fourth part of duodenum (n = 121). Relative variables among three groups were compared. RESULTS: Compared with G1 and G2, G3 patients had the largest cysts (relative diameter: 1.18 vs. 1.60 vs. 2.62, p < 0.001), the youngest age (20.52 vs. 19.47 vs. -3.40 months, p < 0.001), the highest rate of prenatal diagnosis (26.32% vs. 36.31% vs. 62.81%, p < 0.001), the lowest occurrence of protein plugs in common channel (44.74% vs. 38.69% vs. 16.53%, p < 0.001), and the most elevated total bilirubin level (7.35 vs. 9.95 vs. 28.70 µmol/L, p < 0.001). Prenatally diagnosed G3 patients had heavier liver fibrosis than G2 (13.16% vs. 1.67%, p = 0.015). CONCLUSION: The more distal papilla location, the more severe clinical characteristics of CDCs, suggesting a crucial role in its pathogenesis.

Prenatal diagnosis at different gestational times and clinical features of choledochal cysts: a single tertiary center report.

PURPOSE: This study aimed to evaluate the effect of prenatal diagnosis at different gestational times on the clinical features of patients with choledochal cysts (CDCs). METHODS: Medical records of patients with prenatally diagnosed CDCs admitted to our hospital (April 2013-April 2018) were retrospectively reviewed. The clinical characteristics and pathological CDC features were analyzed. RESULTS: Two hundred eighteen cases were included. Patients were divided into two groups. Group 1 and group 2 had a prenatal diagnosis at ≤ 27 weeks of gestation (second trimester of gestation, n = 157) and > 27 weeks (third trimester of gestation, n = 61), respectively. The incidence of jaundice and the TBIL, IBIL and GGT levels were higher in Group 1 (P = 0.021, P = 0.029, P = 0.042, P = 0.007, respectively). The maximum cyst diameter at the time of surgery was larger in Group 1 (P = 0.015). An association study showed that the time of prenatal diagnosis was negatively correlated with the maximum cyst diameter both postnatally (r = - 0.223, P = 0.001) and at the time of surgery (r = - 0.268, P < 0.001). CONCLUSION: Unlike patients diagnosed at a late prenatal age, patients diagnosed at an early prenatal age tend to present clinical symptoms (jaundice, manifested as high indirect bilirubin), hepatic function damage, and large cysts at the time of surgery.

A review of the occurrence, metabolites and health risks of butylated hydroxyanisole (BHA).

Butylated hydroxyanisole (BHA) is mainly used as a food additive due to its antioxidant properties, which prevent or delay oxidation reactions and extend the storage life of products. The widespread use of BHA has led to its extensive presence in various environmental matrices and human tissues. Food intake is the main route of human exposure to BHA. Under different conditions, BHA can produce different metabolites, with tert-butyl hydroquinone (TBHQ) being one of the major products. Several studies have shown that BHA could cause thyroid system damage, metabolic and growth disorders, neurotoxicity, and carcinogenesis. Mechanisms such as endocrine disruption, genotoxicity, disturbances of energy metabolism, reactive oxygen species (ROS) production, signaling pathways, and imbalances in calcium homeostasis appear to be associated with the toxic effects of BHA. Avoiding the toxic effects of BHA to the maximum extent possible is a top priority. Finding safe, non-toxic and environmentally friendly alternatives to BHA should be the focus of subsequent research. In all, this review summarized the current situation related to BHA and might make recommendations for future research directions. © 2023 Society of Chemical Industry.

A nomogram model to predict prognosis of patients with hepatoblastoma.

BACKGROUND: Hepatoblastomas (HBs) are malignant liver tumors that most commonly develop in pediatric patients. Microvascular invasion may be a prognosis factor for patients with HBs. This study aimed to construct a model to predict the survival outcome in HBs. METHODS: We retrospectively analyzed the clinical data of 311 patients with HBs who underwent surgical resection at our institution between June 2014 and August 2021. First, patients were divided into two groups: those who had pathologic microvascular invasion (n = 146) and those who did not (n = 165). Propensity score-matched (PSM) analysis was carried out between the two groups. The preoperative parameters and overall survival (OS) rate were compared between the two groups. Second, all 311 patients were randomly divided into the training and validation cohort in a ratio of 4:1. A nomogram was created in the training cohort to visualize the prediction of OS. Moreover, the validation cohort was used for validation. RESULTS: Multivariate analysis suggested that age, histology type, microvascular invasion, multifocality, distant metastasis, and macrovascular involvement are independent prognostic factors for HBs. The nomogram showed good predictive ability in the training and validation cohorts with a C-index of 0.878 (95% CI, 0.831-0.925) and 0.847 (95% CI, 0.757-0.937), respectively. The calibration curve indicated good agreement between the prediction and observation for one-, two-, and three-year OS probabilities. CONCLUSION: By combining preoperative imaging results and other clinical data, we established a nomogram to predict OS probability for patients with HB, which could be a potential tool to guide personalized treatment.

One- versus two-stage single-incision laparoscopic cyst excision and hepaticojejunostomy in patients with completely perforated choledochal cysts and good medical conditions.

BACKGROUND: The aim of the current study was to evaluate the efficacy of one- and two-stage single-incision laparoscopic hepaticojejunostomy (SILH) for perforated CDCs with good medical conditions. METHODS: Between June 2015 and December 2020, 57 patients were reviewed: Group 1: patients who underwent one-stage SILH (n = 16); Group 2: patients who underwent two-stage SILH (n = 41). The demographic characteristics, operational details, postoperative outcomes and postoperative complications were evaluated. RESULTS: The mean follow-up durations of group 1 and 2 were 39.3 and 38.6 months, respectively. One patient (6.3%) in group 1, and 4 patients (9.8%) in group 2 were converted to laparotomy (p = 0.67). No statistical significance was found in operative time, blood transfusion, time to resume full diet, duration of drainage after definitive surgery and postoperative hospital stays between the two groups. Four patients in group 2 developed bile leakage, which was higher than that in group 1 (9.8% vs 0, p = 0.20). None suffered incidental injury, bleeding, anastomotic stenosis, cholangitis, cholelithiasis, pancreatic leakage, pancreatitis, Roux-loop obstruction, adhesive intestinal obstruction or wound infection. Liver function normalized within 1 year postoperatively in both groups. CONCLUSIONS: In experienced hands, one-stage single-incision laparoscopic hepaticojejunostomy is safe and effective for patients with complete perforations and good medical conditions.

Pattern of anatomic disorder and surgical management of anorectal prolapse in anorectal malformation.

AIM: Due to the paucity of data and controversy regarding the etiology and surgical approach for managing anorectal prolapse (ARP) after anorectoplasty, we sought to investigate the underlying anatomic disorder and the surgical outcome in managing this challenging complication. METHODS: We performed a retrospective study on 83 patients with ARP related to anorectal malformations (ARM). Logistic regression analyses were performed to detect the risk factors for the ARP severity. Surgical procedures were stratified according to identified anatomical abnormalities and surgical outcomes were analyzed. RESULTS: 50 patients (62.7%) had high-type ARM. The original anorectoplasty had a higher rate of ARP in laparoscopic-assisted anorectoplasty (n = 49, 59.0%) versus posterior sagittal anorectoplasty (n = 11, 13.3%). ARP was associated with rectal fat hyperplasia (67.5%), dilated muscular tunnel (79.5%), longitudinal muscle (LM) discontinuity (16.9%), rectal dilation (22.9%), mislocated anus (7.2%), and excessive mobile mesorectum (3.6%). Based on the ARP severity, the patients were divided into a severe group (Group 1, n = 38) and a moderate group (Group 2, n = 45). Binary logistic regression analysis showed that hyperplasia rectal fat (OR 4.55, 95% CI 1.16-17.84), rectal dilation (OR 4.21, 95% CI 1.05-16.94), and high-type ARM (OR 2.90, 95% CI 1.14-7.39) were independent risk factors for the development of severe ARP. Complications after stratified surgical repair included wound infection in six patients (7.2%), anal stenosis in one patient (1.2%), and ARP recurrence in two patients (2.4%). Twenty-six patients without colostomy before prolapse repair were followed up for 2 to 12 years. All the patients maintained voluntary bowel movements. Following ARP repair, there was an overall higher rate of no soiling or grade 1 soiling (88.5 vs. 65.4%), but 3 of 12 patients with grade 2 constipation were upgraded to grade 3. CONCLUSION: Our study shows that ARM-related anorectal prolapse is associated with excessive rectum, hyperplasia of rectal fat, mobile mesorectum, loose muscular tunnel, LM discontinuity, and anal mislocation. Surgical repair with techniques stratified according to the patients' underlying risk factors is effective to prevent recurrence and improve the soiling continence.

A novel combined enteroscopy and laparoscopy approach to prevent urethral complications in management of rectourethral fistula.

BACKGROUND: For the last 20 years, laparoscopy management of anorectal malformations (ARM) has been challenged due to the development of postoperative urethral diverticulum or injury caused by the imprecise transection of rectourethral fistulae, particularly rectobulbar fistulae situated deep in the pelvis. We have developed a combined approach of enteroscopy and laparoscopy for intraluminal incision of a rectourethral fistula. METHODS: We retrospectively reviewed 47 ARM patients who underwent surgical corrections using the combined approach between January 2019 and June 2020. Early postoperative and subsequent follow-up results were evaluated. RESULTS: The median follow-up period was 12 months. The average age at surgery was 3.18 ± 0.64 months. The mean operative time of a single-incision laparoscopic-assisted anorectoplasty (SILAARP) was 1.19 ± 0.29 h. The time for intraluminal incision of the fistula was shortened from 14 to 2 min. No patients underwent a conversion. The average postoperative hospital stay, time to full feeds and placement of an anal tube were 10 days, 1 day, and 5 days, respectively. No urethral diverticulum, urinary injury, wound infection, rectal retraction, anal stenosis or rectal prolapse was encountered in the cohort. CONCLUSIONS: The combined enteroscopy and laparoscopy approach offers precise management of rectourethral fistulae. It could effectively obviate urethral complications, eliminating the obstacles of laparoscopy application in the management of ARMs.

Long-term outcome following cholecystocolostomy in 41 patients with progressive familial intrahepatic cholestasis.

PURPOSE: Progressive familial intrahepatic cholestasis (PFIC) is a cohort of autosomal recessive syndromes which presents with jaundice, severe pruritus and liver derangement. Without treatments, patients progress to liver failure in early childhood. Biliary diversion strategies have been deployed to interrupt enterohepatic circulation to alleviate symptoms and delay progression to cirrhosis. Cholecystocolostomy has been the diversion method of choice at our institution and we aim to evaluate its long-term outcome. METHODS: All patients with PFIC who underwent cholecystocolostomy between August 2003 to May 2019 were included. PFIC diagnosed by clinical course, serum liver biochemistry and genotyping excluding other causes of cholestasis. All patients received ursodeoxycholic acid prior to biliary diversion. Those without long-term follow-up were excluded. Long-term follow-up conducted with physical examination, abdominal ultrasonography, liver function tests, contrast enema studies and colonoscopies. Outcome analysis was performed with patients divided into three groups according to their postoperative responses. RESULTS: 58 children underwent cholecystocolostomy, 41 were included in the study. Overall survival rate was 73.2% without a liver transplant. Survival improved to 81.1% in those without cirrhosis. 83.3% of those without a transplant was to no longer need any medication after their cholecystocolostomy. Recurrent cholestasis was seen in those with constipation (n = 8), ascending cholangitis (n = 10), intrahepatic reflux from Y-loop (n = 3) and cystic duct stenosis (n = 4). CONCLUSION: Cholecystocolostomy is a safe and effective technique for treatment of cholestasis in PFIC patients without cirrhosis. Careful monitoring and proactive management of postoperative constipation and ascending cholangitis is required to prevent stenosis of the cystic duct leading to recurrent cholestasis.

Refinements in surgical techniques for visualized tunnel formation in laparoscopic-assisted anorectoplasty.

PURPOSE: Laparoscopic-assisted anorectoplasty (LAARP) is becoming a more popular procedure for anorectal malformation (ARM) repair. However, the conventional technique for creating pull-through tunnel between the perineal skin and the pelvic floor has been criticized as being semi-blind. This study aims to present a refined version of our previously reported clamp introduction technique for visualized tunnel formation in the center of the sphincter muscle complex (SMC) for rectal pull- through for ARMs. METHODS: A retrospective review was performed for ARM patients who underwent LAARP from Jan 2019 to Jun 2020. Longitudinal muscle tube (LMT) tunnel was created using the clamp-dilator introduction technique: a laparoscopic dilator was used to create a pelvic tunnel within LMT in high ARM, and the clamp introduction under the direct vision technique was performed for creating the perineal tunnel of LMT for both high and intermediate ARMs. RESULTS: Seventy patients (1-198 days) with high-type (27cases) and intermediate-type (43 cases) ARM underwent LAARP using clamp-dilation introduction technique. No patients suffered from urinary tract injury, recurrent rectourethral fistula, urethral diverticulum and urinary incontinence. One patient suffered from wound infection and rectal retraction which required a redo pull-through on postoperative day 7. Rectal prolapse requiring surgical intervention developed in one patient. Postoperative MRI examination confirmed central placement of the rectum within the LMT in all cases. CONCLUSION: Our experience demonstrates that a visualized tunnel formation in the LMT center can be achieved by the clamp-dilator introduction technique in LAARP for both high and intermediate ARMs.

Impact of microscopically margin-positive resection on survival in children with hepatoblastoma after hepatectomy: a retrospective cohort study.

BACKGROUND: Impact of R1 (microscopically margin-positive) resection on survival of patients with hepatoblastoma (HB) remains debatable. This study aimed to compare the long-term outcomes of R0 (microscopically margin-negative) and R1 resection for HB in children after hepatectomy. METHODS: We retrospectively reviewed files of children with HB who underwent resection at our institution between September 1, 2005, and November 30, 2017. Survival analyses and prognostic factors were evaluated using Kaplan-Meier curves and Cox regression models. RESULTS: Of 259 patients, 218 (84.2%) underwent R0 and 41 (15.8%) R1 resection. After adjusting for confounding factors, R1 resection demonstrated non-significantly lower overall survival (OS: hazard ratio [HR] = 0.75; 95% CI 0.34-1.64) and shorter event-free survival (EFS: HR = 0.97; 95% CI 0.53-1.78) rates than R0 resection. However, stratified analysis showed significantly increased risk of poor OS and EFS in patients with metastasis and mixed epithelial/mesenchymal pathologic subtype in R1 compared with R0 resection (P values for interactions < 0.05). There was no significant difference between R0 resection with metastasis and R1 resection with metastasis in the incidence of local recurrence (P = 0.494); however, a significant difference in the incidence of local recurrence was seen between R0 and R1 resection for subgroups with mixed pathologic subtypes (P = 0.035). CONCLUSIONS: With effective chemotherapy, microscopic margin status may not be associated with survival outcome in children with HB undergoing hepatectomy. However, stratified analysis showed that R1 resection might be associated with decreased survival in children with mixed epithelial/mesenchymal HB, compared with R0 resection, and not affect survival outcomes in those with an epithelial subtype and without metastasis.

Repeatability and Reliability of Home-Based Stool Color Card Screening for Biliary Atresia Based on Results in China and Japan.

Biliary atresia (BA) is the most frequent hepatic cause of death in early childhood. Early referral and timely Kasai portoenterostomy are essential for the improvement of long-term native liver survival rate of BA patients. Screening with stool color card (SCC) has been implemented in Japan since 1994. Recently current digital edition of SCC consisted of seven digitally created images was introduced to China. Our study aimed to evaluate the repeatability and reliability of same edition of SCC used in Beijing, China and Sapporo, Japan. In Beijing from 2013 to 2014, SCCs were distributed to infants' guardians by trained nurses in maternal facilities during information sessions on neonatal screening programs. SCC was used at three checkpoints for each infant after birth for screening. The SCC data were collected from 27,561 infants (92.5%) in Beijing by 42-day health checkup, mobile phone and social network services. In Sapporo from 2012 to 2015, the SCCs with a postcard and guardian instructions were inserted into Maternal and Child Health Handbook and distributed to all pregnant women. The data were collected from a total of 37,478 (94.3%) infants in Sapporo via the postcard during the 1st month infant health checkup. We thus identified two BA patients in Sapporo and two BA patients in Beijing. High rates of sensitivity and specificity in both cities were observed. The frequency distribution of color images on SCC reported in both cities was similar. This study shows excellent repeatability and reliability of the current digital edition of SCC.

Laparoscopic-assisted anorectoplasty for intermediate type rectovestibular fistula: a preliminary report.

PURPOSE: Laparoscopic-assisted anorectoplasty (LAARP) is considered to benefit the male patients with anorectal malformation (ARM). This study evaluates LAARP management for intermediate type rectovestibular fistula (IRVF) in the female patient with ARM. METHODS: Twelve patients with IRVF (aged 3-5 months) underwent LAARP from 2017 to 2019 in our institute. LAARP was performed for mobilization of the rectum, visualization and enlargement of the center of the sphincter muscle complex (SMC) from pelvic and perineal aspects, intra-fistula mucosectomy and rectal pull-through in the SMC with the fourchette and the perineal body unattached. RESULTS: LARRP was performed in all patients without conversion to open procedure. No patient suffered from wound infection, vaginal injury, recurrent fistula and anal stenosis. The parents were satisfied with the appearance of the wound. Rectal prolapse developed in one patient and needed surgical correction. The patients were followed up for a mean of 19.7 months (ranged from 12 to 35 months). CONCLUSION: Our preliminary experience shows that LAARP offers an alternative method of correction for the IRVF with good visualization of the SMC and may diminish the risks of wound dehiscence and vaginal injury.

Normal anorectal musculatures and changes in anorectal malformation.

PURPOSE: We investigated the anorectal musclulature in normal children and anorectal malformations (ARM) to evaluate its role in bowel control mechanism. METHODS: Pelves of 50 neonates died of ARM-unrelated diseases and 16 patients with anorectal malformations (8 high, 5 intermediate, and 3 low ARMs) were dissected and analyzed. RESULTS: Normal anorectal musculature was divided into three muscular tubes: the internal sphincter tube (IAST), longitudinal muscle tube (LMT) and transverse muscle tube (TMT). The LMT came from the outer longitudinal smooth muscle fiber of the rectum and the striated muscle fiber of the levator ani, and the TMT composed of the puborectalis and the external anal sphincter. However, in ARM, the IAST was absent and the LMT, the center of the sphincter muscle complex, was only from the levator ani and could be divided into the pelvic portion and the perineal portion. The former, from the upper rim of the puborectalis to the bulbar urethral, became narrowed and dislocated anteriorly near to the posterior urethra in high ARM and rectal pouch in intermediate ARM. The latter, below the bulbar urethra to the anal dimple, was fused to a column both in high and intermediate ARM. The columnar perineal LMT run downwards and then split, penetrated the superficial part of EAS and terminated at the deep aspect of the skin, to form the anal dimple, which represents the center of the perineal LMT from the perineal aspect. The length of the LMT was longer in high and intermediate ARM than the normal neonate. The columnar perineal LMT and narrowed pelvic LMT could be possibly identified by laparoscopic and perineal approaches retrospectively and widened to allow the passage of the rectum through. CONCLUSIONS: The anorectal musculature in ARM is composed of agenesic LMT and TMT and the narrowed LMT gives anatomical evidence of the center, where the neorectum should pull through.

Laparoscopic surgical technique to enhance the management of anorectal malformations: 330 cases' experience in a single center.

PURPOSE: Laparoscopic-assisted anorectoplasty (LAARP) is considered to benefit the patients with vesico-prostatic fistula. The aim of this study is to present the details of our LAARP technique for improving the short- and long-term outcomes in the patients with high and intermediate types of anorectal malformations (ARMs). METHODS: 330 patients with high-type (174 cases) and intermediate-type (156 cases) anorectal malformation (aged 8 days to 15 years) underwent LAARP from 2001 to 2019. LAARP was performed for full mobilization and resection of the dilated rectum, intra-rectal closure of the fistula, visualization, and enlargement of the center of the longitudinal muscle tube (LMT) from pelvic and perineal aspects. RESULTS: LAARP was performed in all patients and no patient was converted to open procedure. The urethral diverticulum was found in three patients (1.02%, 3/294) according to postoperative protocol voiding cystourethrogram but was not associated with any symptoms such as urinary tract infection and dysuria. Rectal prolapse requiring surgical intervention developed in 25 (7.6%) of 330 patients. Anal stricture occurred in three patients and re-do anoplasty was performed 5 months after LAARP. Anal retraction occurred in two patients and re-pull-through was conducted at 5 and 6 days, respectively, after LAARP. 228 patients who were older than 3 years were followed up. The median follow-up period was 5.8 years (range 3-15 years). 217 patients (95.2%) had voluntary bowel movements; 202 patients (88.6%) were free from soiling or with grade 1 soiling; 30 patients (13.6%) and 25 patients (11.3%) suffered from grade 1 and grade 2 constipation, respectively, while no patient had grade 3 constipation. CONCLUSION: Our experience demonstrates that the LAARP has advantages on rectal mobilization and resection, intra-rectal fistula closure and accurate tunnel formation in the LMT with minimal trauma. The improvement of the short-term and long-term outcomes after LAARP has been shown not only for high-type ARM but also for intermediate-type ARM.

Results of surgical resections with positive margins for children with hepatoblastoma: Case series from a single Asian center.

The influence of margin status on the survival of patients with hepatoblastoma (HB) remains controversial. Here, we report long-term follow-up outcomes of 26 patients with HB who underwent hepatectomy with positive microscopic margins. Although these patients had microscopic residuals, the 5-year overall survival and event-free survival rates of those who had no metastases or macrovascular involvement (MVI) were 86.7% and 80.8%, respectively. This may support the hypothesis that patients with HB who undergo hepatectomy with positive microscopic residuals but without MVI or metastases can also achieve satisfactory survival rate. Further studies in this field are required.