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Pyriform sinus cyst (PSC) and pyriform sinus fistula (PSF) is a rare congenital malformation that arises from the third or fourth branchial structure. In our study, we describe the safety and the utility of endoscopic electrocauterization against PSC/PSF.We retrospectively reviewed the records of patients who underwent endoscopic electrocauterization for PSC/PSF at our hospital. The internal opening of the fistula was identified under general anesthesia using a flexible endoscope (XQ-260 or H-290; Olympus, Tokyo, Japan), and the DualKnifeJ (KD-655L; Olympus) was used to ablate the internal opening.We experienced three PSF and three PSC patients. The postoperative course was uneventful in all cases. The patients declared no pain in the neck, and there were no cases showing recurrent nerve paralysis. Five in six cases (83%), the closure of fistula was archived in the first cauterization. One case (16.6%) required repeated cauterization. No recurrence was found during the follow-up period ( median: 1 year) in any cases.Owing to its rarity in neonates, the diagnosis and treatment of PSC remains complicated and not clearly described. Complete removal of the fistula and the cyst with or without affected thyroid tissue was previously the most commonly used treatment. From our experience, we believe that endoscopic electrocauterization can be the first choice not only for PSF but also for neonatal PSC. In conclusion, endoscopic electrocauterization is feasible even for neonatal PSC. Further investigations including multicenter analyses are needed.

To evaluate the status of surgical training in Japan through a national-level needs assessment. A survey was sent to all 909 graduating residents (GRs) and their 611 program directors (PDs) for the year 2016. A working group of surgical educators from around the country was formed under the education committee of the Japan Surgical Society. The survey items were developed by consensus of this working group. The survey investigated the knowledge and problems of the current curriculum, and the status of the current residency training. The response rates were 56.3% of the GRs and 76.8% of the PDs. Among the participants, 47.6% of the GRs and 29.4% of the PDs believed that the residency curriculum did not match the clinical experience. Over 80% of the GRs and PDs agreed on the importance of training outside of the OR, whereas only 13% of the GRs had received such training regularly. Trainees also reported a lower satisfaction rate about the opportunity to train outside of the OR. This national-level needs assessment of surgical training in Japan identified several gaps in the curriculum. These results provide valuable data to assist the ongoing efforts for surgical residency curriculum improvement.

This study sought to assess the disparity between regions and facilities in surgical resident training in Japan via a national level needs-assessment. A survey was sent to all 909 graduating residents of 2016. Residents trained in the six prefectures with a population of 7 million or more were included in the large prefecture (LP) group. Residents trained in the other 41 prefectures were included in the small prefecture (SP) group. Each group was further divided into a university hospital (UH) group and a non-university hospital (NUH) group. The response rate was 56.3% (n = 512). Excluding nine residents who did not report their prefectures and facilities, surveys from 503 residents were analyzed. The UH group received significantly more years of training. In the SP and UH groups, there were significantly fewer residents who had performed 150 procedures or more under general anesthesia in comparison to the LP and NUH groups, respectively. Self-assessed competencies for several procedures were significantly lower in the SP and UH groups. Disparity in surgical resident training was found between regions and facilities in Japan. The surgical residency curriculum in Japan could be improved to address this problem.

Here, the authors present an extremely rare case of an extensive spinal epidural teratoma (SET) in an infant and provide a review of the cases in the literature. In this report, the authors focused on the clinical manifestation and management of extensive SET. A 64-day-old girl presented with severe dyspnea and paraparesis caused by a large thoracic mass. Imaging studies revealed that the mass originated from the epidural space of the thoracic spine and extended from C7 to L1. The tumor extended bilaterally through the intervertebral foramina and formed a large posterior mediastinal mass. The tumor was partially resected via laminotomy after an emergency thoracotomy. The remnant grade I immature teratoma grew rapidly. After a re-laminotomy and bilateral thoracotomy, the residual tumor stopped growing. However, the patient’s paraparesis improved very little, and her scoliosis progressed gradually. Therefore, SET should be included in the differential diagnosis when an infant patient with paraparesis of the lower extremities is encountered. Timely diagnosis, aggressive treatment, and close monitoring are of critical importance to successful recovery in such patients.

Purpose To evaluate the self-assessed competency of graduating residents (GRs) in Japan upon completion of their residency and to identify the gap between their competency and the competency expected by their program directors (PDs). Method A list of 31 essential surgical procedures was compiled according to the consensus of surgical educators from around the country. A survey with this list was sent to all 909 GRs and their 611 PDs in 2016. The GRs rated their competency to perform these procedures and the PDs were asked to evaluate the expected competency of their GRs using the Zwisch Scale. Result The response rate was 56.3% for the GRs and 76.8% for the PDs. Fewer than half of the GRs who responded felt confident performing ten (32%) of the surgical procedures evaluated. For most procedures, the GRs' self-reported competency was lower than the expectation reported by their PDs. This gap was more than 10% for 13 of the procedures. Conclusion More than half of the GRs in Japan lacked the confidence in their skill to perform one-third of the surgical procedures selected for evaluation in this study. These findings should be used to update the surgical education curriculum in Japan.

Background Indocyanine green (ICG) fluorescence imaging is a promising tool for intraoperative decision-making. The aim of this study was to evaluate the utility of near-infrared fluorescence cholangiography (NIR-FCG) with ICG in primary surgery for biliary atresia (BA). Methods We performed NIR-FCG with ICG in 10 BA patients and observed the fluorescence of their hilar micro-bile ducts and hilar exudate in order to assess the appropriate level at which to dissect the hilar fibrous corn. We compared the jaundice outcome of 10 patients using NIR-FCG (Group A) to that of 35 historical patients in whom NIR-FCG had not been used (Group B). Results The mean age of patients was 74.8 days. The classification of BA was type I in two cases and type-III in eight cases. NIR-FCG visualized the hilar micro-bile ducts, and the incidence of positive fluorescence was 80%. The ratio of postoperative normalization of hyperbilirubinemia in Group A was significantly higher than that in Group B (1.0 vs. 0.65, p Conclusion NIR-FCG provided important objectifiable information about the biliary structures in surgery of BA. Although the number of cases was small, our results suggest that NIR-FCG may be useful for improving the outcome of primary surgery for BA. Type of Study Study of Diagnostic Test. Level of Evidence Level III.

For 30 years, we have consecutively performed rectal mucosal punch biopsy to diagnose Hirschsprung’s disease. The aim of this study was to evaluate the safety of our technique. Patients with suspected Hirschsprung’s disease who underwent punch biopsy, including our original “K-PUNCH” method using an S-moid forceps and non-specific blood-collecting tube at our department and branch hospital between April 1986 and March 2016 were included in the present study. Our punch biopsy technique is characterized by excellent visibility and a direct grasping sensation. The backgrounds and complications of the patients were retrospectively investigated. During this period, 954 patients (median age 4 months; range 1 day–73 years) underwent punch biopsy. Although there were no cases of severe complications (i.e., rectal perforation, infection or full-thickness biopsy), one (0.1%) of the 954 cases in the early period showed liver dysfunction and required transfusion due to bleeding. In addition, inappropriate specimens were obtained in 37 patients (3.9%). Punch biopsy including the “K-PUNCH” method is considered safe and feasible and is associated with a low rate of complications and inappropriate specimen harvesting among patients of all ages. Comorbidities, including the potential for hemorrhage, should always be considered.

Acquired isolated hypoganglionosis (A-IH) is a late-onset intestinal pseudo-obstruction disorder and shows different pathophysiological findings from congenital isolated hypoganglionosis (C-IH). In this study, we retrospectively examined five cases of A-IH and investigated the features of A-IH. Five cases of A-IH were extracted from a nationwide retrospective cohort study in 10 years, from which totally 355 cases of Allied Disorders of Hirschsprung’s Disease (ADHD) were collected. Ages of onset were between 13 and 17 years in three cases, and 4 years and 4 months in ones. Initial symptoms were abdominal distension and/or chronic constipation in 4 cases, whereas one exhibited intestinal perforation. Affected lesions varied from case to case, extending various length of intestinal tracts. All cases underwent multiple operations (average: 4.6 times), such as enterostomy, resection of dilated intestines, and/or pull-through. Pathological findings showed the decreased numbers of ganglion cells and degeneration of ganglion cells, whereas the size of the plexus was normal. Currently, all cases were alive and almost all eat regular food without requiring parenteral feeding. A-IH is rare, but distinct entity characterized by different clinical courses and pathological findings from those of C-IH. The outcome is considered to be favorable after a resection of affected intestine.

UNLABELLED Cornelia de Lange syndrome is known to be occasionally associated with gastrointestinal malformation. However, the occurrence of cecal volvulus in such anomalies is very rare. We report a 15-y-old Japanese boy with Cornelia de Lange syndrome associated with a cecal volvulus secondary to non-fixation of the cecum and ascending colon. The patient was admitted to our hospital because of abdominal pain and bilious vomiting. At emergent laparotomy, cecal volvulus with non-fixation of the ascending colon and cecum was diagnosed. Release of the cecal volvulus and the fixation of the cecum and ascending colon to the right retroperitonium were performed. The postoperative course was uneventful, except for sepsis caused by aspiration pneumonia soon after the operation. CONCLUSION Clinicians treating patients with this syndrome should be aware that such patients may be at risk for developing severe gastrointestinal anomalies, including cecal volvulus.