Normal anorectal musculatures and changes in anorectal malformation Abstract 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.

Commentary to: Close relationship between the short round ligament and the ovarian prolapsed inguinal hernia in female infants—H Kuyama, S Uemura, A Yoshida, M Yamamoto, PSI (2019) 35:625–629

Reply to the letter to the editor concerning “Close relationship between the short round ligament and the ovarian prolapsed inguinal hernia in female infants”

Risk factors for the recurrence of perineal canal Abstract Purpose The aim of this study was to investigate risk factors for recurrence in the perineal canal (PC). Methods Patients with PC who underwent operations were enrolled in this study and were divided into recurrence and non-recurrence groups. Preoperative infection, the age at the operation, the presence of colostomy and the treatment procedure for fistula were retrospectively investigated. Regarding the treatment procedure for fistula, either closure of the rectal wall with stitches or ligation of fistula in the rectum was performed. These factors were compared between the two groups. Results Six of 17 patients with PC who underwent surgical treatment had recurrence. There were no significant differences in the incidence of preoperative infection, age at operation or presence of colostomy (p = 0.60, 0.38, 1.00, respectively). In the recurrence group, all patients were treated by closure of the rectal wall. In the non-recurrence group, five were treated by the closure of the rectal wall with stitches and six by ligation of the fistula. There was a significant association between recurrence and the treatment procedure for fistula (p = 0.04). Conclusion Closure of the rectal wall with stitches is a risk factor for the recurrence of PC.

Efficacy of and prognosis after steroid pulse therapy in patients with poor reduction of jaundice after laparoscopic Kasai portoenterostomy Abstract Purpose High-dose postoperative steroid therapy after Kasai portoenterostomy is reported to improve jaundice clearance and a strong anti-inflammatory activity might prevent fibrous tissue formation which is often observed at the porta hepatis in revision surgery. We started steroid pulse therapy for the patients with cessation of decrease in jaundice and aimed to evaluate the efficacy in this study. Methods The demographics and outcomes of patients who underwent laparoscopic Kasai portoenterostomy and received steroid pulse therapy within 2 months postoperatively between September 2014 and December 2018 were retrospectively reviewed; the therapy was determined successful when the serum total bilirubin level decreased to or below two-thirds of the pre-therapy level after 2 weeks. Patient data in the successful group were compared with those in the unsuccessful group. Results Steroid pulse therapy was successful in seven of 16 patients (43.8%). The percentage of patients whose serum total bilirubin level decreased to normal was significantly higher in the successful group at 3 months (85.7% vs. 11.1%, P = 0.0028) and after all (100% vs. 33.3%, P = 0.011). Conclusions Steroid pulse therapy was effective for some patients. Unsuccessful cases may have little chances of jaundice clearance; revision Kasai portoenterostomy would be a good option.

Impact of congenital heart disease on outcomes after primary repair of esophageal atresia: a retrospective observational study using a nationwide database in Japan Abstract Purpose To verify the association between congenital heart disease (CHD) and postoperative complications after primary repair of esophageal atresia in patients from a Japanese nationwide database. Methods We identified babies in the Diagnosis Procedure Combination database who underwent radical surgery for esophageal atresia from 2010 to 2016. We used multivariable logistic regression analyses to evaluate the occurrence of anastomotic leakage and anastomotic stricture. Results Among 431 patients who underwent primary anastomosis, 114 patients (27%) had CHD. Anastomotic leakage occurred in 77 patients (17.9%) and stricture in 154 (35.7%). Compared with patients whose anesthetic duration was less than 240 min, those with anesthesia lasting from 240 to 360 min (odds ratio 2.49; 95% confidence interval (CI) 1.17–5.27; p = 0.02) or more than 360 min (odds ratio 4.10; 95% CI 1.69–9.96; p = 0.002) were more likely to experience anastomotic leakage. Male patients had a lower risk of anastomotic stricture than female patients (odds ratio 0.65; 95% CI 0.43–0.9; p = 0.04). Conclusions CHD was not associated with anastomotic leakage or stricture. The only significant predictor of anastomotic leakage was duration of anesthesia.

Probiotic strategies to prevent necrotizing enterocolitis in preterm infants: a meta-analysis Abstract Purpose We aimed to compare probiotics with placebo for necrotizing enterocolitis in preterm infants and to evaluate the safety and effect and strict effect of specific probiotic genera. Methods Data recorded until January 2019 were searched, and relevant academic articles from PubMed, MEDLINE, EMBASE, and the Cochrane Central Register of Controlled Trials were selected by two independent reviewers. Two reviewers independently included randomized controlled trials that compared probiotics and placebo in preterm infants. The outcomes included more than one of the following outcomes: incidence of necrotizing enterocolitis, necrotizing enterocolitis-related mortality, incidence of sepsis, and all-cause mortality. Two reviewers independently extracted data and assessed the risk of bias and quality of evidence. Results We identified 34 eligible studies of 9161 participants. This meta-analysis showed an overall advantage of probiotics to prevent the incidence of necrotizing enterocolitis (3.54%) and gut-associated sepsis (15.59%), and decrease mortality (5.23%) in preterm infants. A probiotic mixture showed a huge advantage and vitality in preventing necrotizing enterocolitis (2.48%) and gut-associated sepsis (18.39%), and in reducing mortality (5.57%) in preterm infants. Conclusion The probiotic mixture showed advantages over the single strains to decrease the incidences of necrotizing enterocolitis and gut-associated sepsis, and mortality in preterm infants.

Application of indocyanine green in pediatric surgery Abstract The use of near-infrared (NIR) fluorescence imaging with indocyanine green (ICG) has gained popularity in many fields in adult surgery, such as sentinel lymph node mapping, intra-operative solid tumor identification, and organ perfusion assessment. However, the clinical application of ICG in pediatric surgery is just at the beginning. This review paper presents the advantages, current applications and potential developments of NIR fluorescence imaging with ICG in our field.

Serum Mac-2-binding protein (M2BPGi) as a marker of chronological liver fibrosis in biliary atresia patients with cirrhosis Abstract Purpose Biliary atresia (BA) is characterized by progressive liver fibrosis, but it is difficult to assess the progression after the patient develops cirrhosis. Mac-2-binding protein glycosylation isomer (M2BPGi) is a new marker for hepatic fibrosis. We examined the chronological changes in M2BPGi levels in BA patients with cirrhosis. Methods Patients with cirrhosis were selected from among pediatric BA patients who had their native livers. Serum M2BPGi levels and Child–Pugh classification were evaluated. A total of 11 pediatric BA patients with cirrhosis were recruited. Results Initial M2BPGi level after diagnosis of liver cirrhosis based on liver biopsy was on average 3.4, and the most recent M2BPGi level under observation was on average 4.3. The follow-up period from the initial M2BPGi measurement averaged 22.6 months. The ratio of the initial and most recent values (M2BPGi ratio) was on average 1.3 (0.5–2.4). Three cases with improved fibrosis (M2BPGi ratio < 1.0) remained in Child A, as did six cases (1.0 ≤ M2BPGi ratio < 2.0), but two cases with marked fibrosis progression (2.0 ≤ M2BPGi ratio) advanced to decompensated cirrhosis Child B. Conclusion M2BPGi is useful as a prognostic factor for BA patients with liver cirrhosis. In addition, fibrosis improved even after the development of cirrhosis.

Bio-engineering of fetal cartilage for in utero spina bifida repair Abstract Purpose During in utero surgical spina bifida repair, a multi-layer closure is used to cover the defect. These soft tissues, however, might be not sufficient to protect the spinal cord during the future life. Our goal is to develop a more rigid protective tissue construct consisting of bioengineered cartilage and skin. Methods Ovine fetal chondrocytes were tested for their in vitro chondrogenic potential in three-dimensional cultures. Scaffolds based on natural biopolymers (collagen I, fibrin glue) were loaded with varying amounts of fetal chondrocytes and assessed for their ability to support cartilage formation in vitro. The bioengineered constructs were analyzed using cartilage-specific histology stainings and compared to native fetal cartilage. Results Fetal chondrocytes actively produced cartilage extracellular matrix in three-dimensional cultures, even at high passages. Among all bioengineered scaffolds, only the collagen I-based hydrogels loaded with high densities of fetal chondrocytes showed cartilage-like structure in vitro but also extensive shrinking. Conclusion Fetal chondrocytes represent a good cell source for cartilage bioengineering. Collagen I scaffolds support cartilage formation in vitro, but the construct shrinking constitutes a major limitation. Future steps include the identification of suitable bioprintable materials which maintain their shape and size, as well as the analysis of the interphase between bioengineered cartilage and skin.