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Some neuroblastoma (NB) cases are suitable for minimally invasive surgery (MIS), but indication and technical issue are unclear. We assessed the current status of MIS for abdominal NB after mass screening period in Japan. Preliminary questionnaires requesting the numbers of NB cases that underwent MIS from 2004 to 2016 were sent to 159 Japanese institutes of pediatric surgery. The secondary questionnaires were then sent to the institutions that reported MIS cases of NB in order to collect detailed data. One hundred and thirty-four (84.2%) institutions responded to the preliminary questionnaires, and 83 (52.2%) reported managing operative cases. The total number of operative cases was 1496. MIS was performed for 175 (11.6%) cases, of which the completed forms of 140 patients were returned, including 100 abdominal NB cases. The male/female ratio was 51/49. Forty-seven cases underwent a laparoscopic biopsy, and 2 (4.3%) cases were converted to laparotomy due to bleeding. Sixty-five cases underwent MIS for radical resection, and 7 (10.8%) were converted to laparotomy. The reasons for open conversion were bleeding and severe adhesion. Regarding open conversion, there were no significant relationships between conversion and neo-adjuvant chemotherapy, biopsies, stage, size, or MYCN amplification. We found no relationship between resectability and vascular encasement in this study. There was relationship between the resected tumor size and the patients’ height, which was expressed using the following formula: $$y = 0.0316x + 1.4812$$ (x, patients height, y, tumor size; p = 0.004219, SE: 1.55566). Postoperative complications after radical resection were recognized in 7 (10.8%) cases. MIS was performed in limited cases of abdominal NB. A laparoscopic biopsy with careful attention to bleeding is feasible. The resected tumor size was shown to correlate with the patients’ height. Tumor size within 6 cm of maximum diameter can be resected safely.

Background An ectopic gallbladder is a rare anomaly and can result in the misinterpretation of imaging findings and clinical confusion. Knowledge of such anomalies facilitates accurate diagnoses and prompt management. We report a pediatric case of an ectopic gallbladder concomitant with congenital biliary dilatation (CBD). Case presentation A 9-year-old girl was referred to our hospital for elevated liver enzyme levels. Following physical examination and a review of medical imaging findings, she was diagnosed with Todani type IV-A CBD. We could not visualize the gallbladder by abdominal ultrasonography, CT, and MRI scans; therefore, we suspected gallbladder agenesis. A laparoscopic excision of the extrahepatic bile duct was performed to treat the CBD. Neither a gallbladder nor a cystic duct were revealed on the liver undersurface. Therefore, gallbladder agenesis was considered as a diagnosis based on preoperative imaging and intraoperative findings. However, during dissection of the hepatic hilum, a cyst-like structure was found on the ventral side of the common hepatic duct, slightly to the right, and a small additional duct that originated from the cystic structure was found. Upon incision, a small amount of bile was drained from the small duct. Thus, the cystic structure was diagnosed as an ectopic gallbladder with hypoplasia. Following the removal of the ectopic gallbladder, the extrahepatic bile duct was excised. Subsequently, laparoscopic Roux-en-Y hepaticojejunostomy was performed without any complications. Postoperative histopathological evaluations of the resected specimen revealed Rokitansky–Aschoff sinuses in the resected cystic lesion. The pathological investigations confirmed the diagnosis of an ectopic gallbladder. Following an uneventful postoperative course, the patient was discharged on day nine. Conclusions To our knowledge, this is the first pediatric case report describing an ectopic gallbladder concomitant with CBD. If the gallbladder cannot be detected in a preoperative imaging examination, it is important to consider the possibility of an ectopic gallbladder.

Background This study aimed to describe our experience with three-dimensional endoanal ultrasonography (3D-EAUS) in patients who underwent surgery for Hirschsprung disease and to summarize the relationship between postoperative anal function and the distance between the anastomosis and intersphincteric groove (DBAI) measured via 3D-EAUS. Results We retrospectively reviewed patients with a history of undergoing surgery for Hirschsprung disease who visited our outpatient clinic between December 2018 and December 2019. All patients underwent 3D-EAUS for DBAI measurement. We used the Krickenbeck classification to evaluate postoperative anorectal function. Eleven patients (all males aged 3–14 years) were evaluated. Four (36.4%), four (36.4%), and three (27.3%) patients had no soiling, grade 1 soiling, and grade 3 soiling, respectively. Four (36.4%) and seven (63.6%) patients had no constipation and grade 3 constipation, respectively. The median DBAI values were 7.0 mm, 8.4 mm, and 5.6 mm (p = 0.14) in the no soiling, grade 1, and grade 3 soiling groups, respectively. Conclusions 3D-EAUS enabled precise visualization of the anal anatomy and evaluation of the anastomosis. The DBAI was relatively short in patients with grade 3 soiling, although not significantly so. Further evaluation is warranted.

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. 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. 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). CHD was not associated with anastomotic leakage or stricture. The only significant predictor of anastomotic leakage was duration of anesthesia.

Laparoscopic-assisted anorectoplasty (LAARP) is a minimally invasive procedure in which the levator ani muscle is left to potentially improve postoperative fecal continence. This study aimed to retrospectively evaluate postoperative complications and long-term outcomes of fecal continence after LAARP and compare them to those after posterior sagittal anorectoplasty (PSARP). Forty-five male patients with high and intermediate anorectal malformation (aged ≥ 4 years) who underwent LAARP or PSARP (LAARP 29, PSARP 16) from 1999 to 2013 were included. Postoperative complications and postoperative fecal continence were retrospectively evaluated and compared. Postoperative complications were also compared before and after introducing a urethroscope during fistula resection. Complications after LAARP and PSARP were seen in 12 vs. 2 cases (p = 0.09) of mucosal prolapse and in 9 vs. 1 case (p = 0.07) of posterior urethral diverticulum (PUD), respectively. The incidence of PUD decreased after introduction of a urethroscopy from 40% in 8/20 cases to 11% in 1/9 cases. No significant difference was found in terms of fecal continence in both groups. Through our study, greater improvement of postoperative fecal continence after LAARP has not been shown. LAARP was at higher risk for mucosal prolapse and PUD. However, precise dissection of the urethral fistula could be performed after the introduction of urethroscopy.

Purpose: Anovestibular fistula (AVF) is the most common type of anorectal malformation in females. Delayed anorectoplasty with fistula dilatation is commonly performed during infancy; however, we have been actively performing anorectoplasty in neonates. We report the surgical complications and postoperative defecation function associated with single-stage anorectoplasty performed in neonates. Methods: Patients who underwent surgery for AVF between 2007 and 2017 at two institutions were retrospectively studied. The operation time, amount of bleeding, time to start oral intake, perioperative complications, and Kelly’s score were compared among patients who underwent surgery as neonates and those who underwent surgery as infants. Results: Eighteen neonates and 17 infants underwent anterior sagittal anorectoplasty. The median operation time and time to start oral intake were significantly shorter in the neonatal group (72 min; 3 days, respectively) than in the infant group (110 min, p = 0.0002; 5 days, p = 0.0024, respectively). Postoperative wound disruption was significantly more frequent in the infant group. Of the ten patients each in the neonatal and infant groups, there was no significant difference in Kelly’s score at age ≥ 4 years. Conclusion: Single-stage anorectoplasty in neonates with AVF can be feasibly performed and does not impair postoperative defecation function., ファイル公開：2019/10/01

Purpose: The Soave procedure (SO) is performed most commonly for Hirschsprung disease. SO reduces the risk of injury to the pelvic structures; however, a residual aganglionic muscle cuff could interfere with bowel movement and lead to obstructive enterocolitis. The Swenson procedure is considered ideal in terms of peristalsis. Currently, laparoscopic surgery provides better visualization and facilitates precise dissection, possibly leading to feasible performance of the laparoscopic modified Swenson procedure (SW). We present our operative technique and the efficacy of the SW compared with that of SO. Methods: We retrospectively reviewed the records of 16 and 27 patients who underwent SW and SO, respectively, between 2012 and 2017. Results: Operative time, blood loss, length of stay, and frequency of bowel movements showed no significant difference between the two groups. In the SW group, temporary dysuria occurred in one patient, postoperative enterocolitis in two, wound infection in one, and severe perianal excoriation in four, whereas in the SO group, obstructive symptoms occurred in three patients, small-bowel obstruction in one, and severe perianal excoriation in three. The complications and outcomes were comparable between both groups. Conclusion: Laparoscopic SW was safe and feasible for the short-term follow-up outcomes., ファイル公開：2019/10/01

Backgroud Recently, laparoscopic percutaneous extraperitoneal closure (LPEC) has gained increased popularity for pediatric inguinal hernia repair. To improve cosmesis, we developed single incision LPEC (SILPEC). The aim of this study was to assess the safety and feasibility of SILPEC compared with traditional open repair (OR). Methods This was a single-center retrospective cohort study of 2028 children who underwent inguinal hernia repair between April 2005 and August 2014. Nine hundred and ninety-five patients underwent OR and 1033 patients underwent SILPEC. Medical records were reviewed with respect to operative time, recurrence, incidence of contralateral metachronous inguinal hernia (CMIH), and complications. Patient satisfaction with cosmetic result was also investigated using questionnaires sent by mail. Results All SILPEC procedures were completed without conversion. Operative time was longer in the SILPEC group than in the OR group for both unilateral and bilateral surgery regardless of sex (unilateral male: p = 0.0006, unilateral female: p

Owing to recent advances in minimally invasive surgery (MIS), laparoscopic and thoracoscopic surgery have been gradually introduced for use in neonates and infants. This review focuses on two popular MIS procedures for diaphragmatic diseases in neonates and infants: congenital diaphragmatic hernia (CHD) repair and plication for diaphragmatic eventration. While several advantages of MIS are proposed for CDH repair in neonates, there are also some concerns, namely intraoperative hypercapnia and acidosis and a higher recurrence rate than open techniques. Thus, neonates with severe CDH, along with an unstable circulatory and respiratory status, may be unsuitable for MIS repair, and the use of selection criteria is, therefore, important in these patients. It is generally believed that a learning curve is associated with the higher recurrence rate. Contrary to CDH repair, no major disadvantages associated with the use of MIS for diaphragmatic eventration have been reported in the literature, other than technical difficulty. Thus, if technically feasible, all pediatric patients with diaphragmatic eventration requiring surgical treatment are potential candidates for MIS. Due to a shortage of studies on this procedure, the potential advantages of MIS compared to open techniques for diaphragmatic eventration, such as early recovery and more rapid extubation, need to be confirmed by further studies.

Tracheobronchomalacia is a respiratory disorder that results from abnormal cartilage development. Since 2003, we have performed external stabilization for severe tracheobronchomalacia using separated ring-reinforced expanded polytetrafluoroethylene (ePTFE) grafts. The aim of this study was to analyze the short- and long-term outcomes of our procedure. We reviewed the charts of patients who underwent external tracheobronchial stabilization from January 2003 through December 2012. Separated ring-reinforced ePTFE grafts were fixed to the anterior and posterior lesions of malacia independently, with confirmation of a well-opened lumen by bronchoscopy. Five children with severe tracheobronchomalacia underwent six operations. The median follow-up period was 43 months (range 11–109 months). There was no operative mortality associated with the procedure. One patient required removal of a section of the graft owing to the development of bronchial granulation tissue at the site of fixation. All patients showed improved respiratory function, and tracheal growth was confirmed by follow-up computed tomography. External stabilization with separated ring-reinforced ePTFE grafts for severe tracheobronchomalacia is effective, less invasive than alternatives and preserves the growth potential of the affected airway segment.

Single-incision laparoscopic appendectomy was recently introduced as a less-invasive surgical technique than existing methods, and is increasingly being performed worldwide. We have started to perform transumbilical laparoscopic-assisted appendectomy (TULAA) in children. In this study, we assessed its feasibility, efficacy, and cost-effectiveness compared with conventional multiport laparoscopic appendectomy (MLA). We retrospectively reviewed all of the patients who underwent MLA or TULAA between August 2009 and December 2012. Patient characteristics, operative time, length of hospital stay, postoperative complications, cost-effectiveness, and cosmetic results were analyzed. A total of 88 patients underwent laparoscopic appendectomy, with MLA in 48 patients and TULAA in 40 patients. TULAA was successful in 36/40 patients. There were no significant differences in mean operative time or mean length of stay between the two groups of patients. There were no postoperative complications in the TULAA group. The cost of TULAA was much lower than that of MLA. TULAA also provided nearly scarless surgery. TULAA, in which the appendix is exteriorized via a combination of open and laparoscopic techniques, is a safe and effective procedure for treating appendicitis in children. TULAA is a cost-effective treatment that provides excellent cosmetic results.

The optimal method of treating long-gap esophageal atresia has not been established; however, the native esophagus is recognized as one of the best materials to use for bridging a long gap. Several case reports describe long-gap esophageal atresia being treated successfully via thoracotomy, by applying external traction sutures to the proximal and distal ends of the esophagus, thereby gradually elongating the esophagus prior to delayed primary anastomosis. However, this method carries a risk of infection and disruption of the esophageal ends. We devised a simple method of internal traction for esophageal elongation, which we performed as a two-step thoracoscopic procedure to treat a neonate with long-gap esophageal atresia.

In 2006, The Japanese Society of Pediatric Endoscopic Surgeons devised a plan to develop a pediatric endoscopic surgical skill qualification (ESSQ) system. This system is controlled by The Japan Society for Endoscopic Surgery. The standard requirement for skills qualification is the ability of each applicant to complete common types of laparoscopic surgery. The main goal of the system is to decrease complications of laparoscopic surgery by evaluating the surgical skills of each applicant and subsequently certify surgeons with adequate skills to perform laparoscopic operations safely. A committee of pediatric ESSQ created a checklist to assess the applicant’s laparoscopic surgical skills. Skills are assessed in a double-blinded fashion by evaluating an unedited video recording of a fundoplication for pediatric gastroesophageal reflux disease. The initial pediatric ESSQ system was started in 2008. In 2008 and 2009, respectively, 9 out of 17 (53%) and 6 out of 12 (50%) applicants were certified as expert pediatric laparoscopic surgeons. Our ultimate goal is to provide safe and appropriate pediatric minimally invasive procedures and to avoid severe complications. To prove the predictive validity of this system, a survey of the outcomes of operations performed by certified pediatric surgeons is required.

Oil-producing fungus Mortierella alpina 1S-4 is an industrial strain. To determine its physiological properties and to clarify the biosynthetic pathways for polyunsaturated fatty acids, a transformation system for this fungus was established using a derivative of it, i.e., a ura5- mutant lacking orotate phosphoribosyl transferase (OPRTase, EC.2.4.2.10) activity. Transformation with a vector containing the homologous ura5 gene as a marker was successfully performed using microprojectile bombardment, other methods frequently used for transformation, such as the protoplasting, lithium acetate, or electroporation methods, not giving satisfactory results. As a result, two types of transformants were obtained: a few stable transformants overexpressing the ura5 gene, and many unstable transformants showing OPRTase activity comparable to that of the wild-type strain. The results of quantitative PCR indicated that the stable transformants could retain the ura5 genes originating from the transformation vector regardless of the culture conditions. On the other hand, unstable transformants easily lost the marker gene under uracil-containing conditions, as expected. In this paper, we report that an overall transformation system for this fungus was successfully established, and propose how to select useful transformants as experimental and industrial strains.

Background: Laparoscopic findings of levator muscle and the efficacy of laparoscopic muscle stimulator (LMS) in infants with high imperforate anus have not been reported. Methods: Twelve patients underwent laparoscopically assisted anorectoplasty for high imperforate anus. Following laparoscopic dissection of the distal rectum and division of the fistula, levator muscles in the pelvic floor were stimulated with a 5-mm-diameter LMS. Dilatation was done by inserting a guidewire and balloon catheter through the center of the levator muscle sling and muscle complex. Rectal pull-through and anastomosis between the rectum and anus were successfully completed. Results: LMS showed good contraction of levator muscles and enhanced accurate midline placement of pull-through rectum. LMS was particularly useful in observing weak muscles in infants with rectovesical fistula. Conclusions: Laparoscopy and LMS offer excellent visualization of the pelvic musculature and precise tract of rectal pull-through. Fecal continence will be assessed by long-term follow-up.

Enhanced production of 5,8,11-eicosatrienoic acid (Mead acid, 20:3omega9) was attained with a mutant fungus, Mortierella alpina JT-180, derived from delta12 desaturation activity-defective and delta6 desaturation activity-enhanced M. alpina M209-7. Production of 20:3omega9 by JT-180 was 1.4 times greater than that of the parent strain M209-7. This is thought to be due to its enhanced Delta5 desaturation activity, which was 3.3 times higher than that of M209-7. In both strains, 78.5-80.4% of the total lipids comprised triacylglycerol (TG), and 76.6-79.0% of 20:3omega9 was present in TG. Comparing the fatty acid compositions among various lipid species, the highest percentages (24.1-37.6%) of 20:3omega9 in total lipids were found in phosphatidylcholine. For optimization of 20:3omega9 production by JT-180, a glucose concentration of 4% in the culture medium and shifting of the growth temperature from 28 degrees C to 20 degrees C on the 2nd day were shown to be effective. Under optimal conditions, 20:3omega9 production by JT-180 reached 1.92 g/l culture medium in a 10-l jar fermentor (corresponding to 81.5 mg/g dry mycelia and 18.3% of total fatty acids), which is greater than that reported previously from M209-7 (1.65 g/l).

Background Arachidonic acid (ARA) is an essential fatty acid and a major constituent of biomembranes. It is converted into various lipid mediators, such as prostaglandin E2 (PGE2) and lipoxin A4 (LXA4). The effects of dietary ARA on colon maintenance are unclear because PGE2 has both mucosal protective and proinflammatory effects, and LXA4 has an anti-inflammatory role. Our objective is to clarify the effects of dietary ARA on an experimental murine colitis model.

The laparoscopic procedure involving total resection of abdominal neuroblastoma combined with lymphadenectomy has not been reviewed in English literature. The aim of this study was to evaluate the significance and accuracy of laparoscopic resection of abdominal neuroblastoma.Since July 1997, five patients with abdominal neuroblastoma underwent laparoscopic resection combined with lymphadenectomy or sampling of the lymph nodes. The length of operation, intraoperative blood loss, resectability, and complications were retrospectively reviewed and evaluated.Four cases were managed laparoscopically, but one case was converted to open procedure because of poor visualization around large vessels. The mean operation time was 135 min and the intraoperative blood loss 52 ml.Good visualization of the primary tumor and large vessels is, arguably, the most important factor for successful completion of this procedure laparoscopically. Precise indicators for laparoscopic resection of abdominal neuroblastoma provide a better prognosis and a good quality of life for children with neuroblastoma.

A 2-month-old boy with biliary atresia (BA) with an extremely rare prenatal history is reported. A cystic lesion in the hepatic hilum was diagnosed during prenatal ultrasonography at 20 weeks' gestation. However, it disappeared during later prenatal observation. He was diagnosed with an uncorrectable type of BA after a massive subdural hemorrhage at the age of 66 days. A fetus suspected of having a cyst of the biliary tree should be followed even after the cyst has disappeared spontaneously.

We report an unusual case of anorectal agenesis with a rectourethral fistula diagnosed in a 48-year-old man. The patient presented after noticing hematuria, although he had been aware of urinary leakage from his colostomy with occasional fecal urine for about 4 years. He had had a double-barrel colostomy created soon after birth for an imperforate anus, with revision at the age of 4 years to correct a prolapse of the stoma, but his malformation had never been repaired. We performed a physical examination, which did not reveal a perineal fistula, but urethrocystography demonstrated high anorectal agenesis with a rectourethral fistula. Thus, we resected the rectourethral fistula and created an end-colostomy. The patient had an uneventful postoperative course, and was discharged in good health on postoperative day 19. To our knowledge, this is the oldest patient to be diagnosed with anorectal agenesis and undergo resection of a rectourethral fistula.

An arachidonic-producing fungus, Mortierella alpina 1S-4, was found to accumulate ω-unsaturated fatty acids of C-20 chain length together with ω1-hexadecenoic acid, ω1-octadecenoic acid and so on, when grown on 1-alkenes, i.e., 1-hexadecene and 1-octadecene. The results of mass spectroscopy and proton NMR showed that a C20 polyunsaturated fatty acid (PUFA) is a novel cis-5,8,11,14,19-eicosapentaenoic acid (20:5ω1). This PUFA was obtained at a yield of 0.13 mg/ml culture broth (2.8% of the fungal total fatty acid content) on cultivation of the fungus in a medium containing 4% (v/v) 1-hexadecene and 1% yeast extract at 28°C for 1 week. Investigation of the distribution of fatty acids showed that about 90% (by mol.) of the PUFA was present in the triglycerides and 10% was in the phospholipid fraction. About 70% of that found in the phospholipids was phosphatidylcholine (PC) and the value accounted for ca. 10% of the total fatty acid content. The formation of these ω-unsaturated fatty acids was presumed to occur through the arachidonic acid biosynthetic pathway (n-6 route).

The intracellular concentration of 5,8,11,14,17-cis-eicosapentaenoic acid (EPA) and 4,7,10,13,16,19-cis-docosahexaenoic acid (DHA) was carried out by Mortierella alpina 1S-4 in a medium containing fish oil as the main carbon source. The EPA and DHA contents reached 29.2% and 20.0% of total fatty acids, respectively, when the fungus was grown in a medium containing salmon oil (EPA and DHA contents, 14.0% and 17.3%, respectively) as the main carbon source in a 5-1 bench-scale fermentor. EPA and DHA in the added fish oil were incorporated into both the mycelial polar lipids and triglycerides.

Various Mortierella fungi were assayed for their productivity of arachidonic acid (ARA). Only strains belonging to the subgenus Mortierella accumulated detectable amounts of ARA together with dihomo-γ-linolenic acid. None of the strains belonging to the subgenus Micromucor tested accumulated these C-20 fatty acids, although they produced a C-18 fatty acid, γ-linolenic enic acid. A soil isolate, M. alpina 1S-4, was found to grow well in a liquid medium containing glucose and yeast extract as carbon and nitrogen sources, respectively. Addition of several natural oils such as olive and soybean oils to the medium increased the accumulation of ARA. Under optimal culture conditions in a 5-1 bench-scale fermentor, the fungus produced 3.6 g/l of ARA in 7 days. On cultivation for 10 days at 28°C in a 2000-1 fermentor, the same fungus produced 22.5 kg/kl mycelia (dry weight) containing 9.9 kg lipids, in which ARA comprised 31.0% of the total fatty acids. On standing the harvested mycelia for a further 6 days, major mycelial fatty acids (i.e. palmitic acid, oleic acid, linoleic acid, etc.) other than ARA rapidly decomposed and the ARA content of the total fatty acids reached nearly 70%.

The production of 5,8,11,14,17-cis-eicosapentaenoic acid (EPA) by Mortierella fungi was hgghly stimulated when they were grown in a medium containing linseed oil at low temperature. This phenomenon was suggested to be mainly due to low temperature-dependent production of EPA from arachidonic acid formed through the n-6 route, although conversion of α-linolenic acid in the added linseed oil to EPA through the n-3 route took place at the same time. The amount of EPA accumulated reached 1.88 mg/ml of culture broth (66.6 mg/g dry mycelia) on cultivation of M. alpina 1S-4 with linseed oil at 12° C.