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Abstract Background Duodenal duplication cysts (DDC) are rare duplications of the alimentary tract. Their treatment depends on their size and location. A radical treatment is total resection, if possible. However, partial excision, puncture, and marsupialization can be selected to prevent surgical injury to the pancreaticobiliary tract despite the risk of recurrence. There are some reports of pancreaticoduodenectomy for DDC because of the risk of recurrent symptoms and malignancy. However, this is considered excessively invasive for DDC, particularly in pediatric cases, because of its extremely low rate of malignancy and high morbidity and mortality rates. We encountered a case of DDC with a congenital duodenal position anomaly occurring in the second part of the duodenum. Taking advantage of the congenital duodenal position anomaly, the DDC was completely resected without injuring the pancreaticobiliary duct. Case presentation A 6-year-old boy was diagnosed with a duodenal duplication cyst with obstruction. There was a congenital duodenal position anomaly. The distal second part of the duodenum was the dorsal side of the proximal second part of the duodenum and ascended upward from the proximal second part of the duodenum. The third and fourth parts of the duodenum ran downward to the left and posterior parts of the portal vein, forming the ligament of Treitz. Complete laparoscopic resection of the duodenal duplication cyst and the second to fourth parts of the duodenum, and duodenojejunostomy with retrocolic reconstruction was performed because the duodenum was easily mobilized to the ligament of Treitz owing to the duodenal position anomaly. The duodenojejunostomy with retrocolic reconstruction achieved a more physiologically normal appearance compared to what would have been achieved with a Roux-en-Y reconstruction. The patient was discharged on postoperative day 12 without any complications. Conclusions The procedure used in this case might not be easily applied in all laparoscopy cases. However, it could be an option for duodenal duplication cysts with congenital duodenal position anomalies.

Abstract Background Preoperative management of Hirschsprung’s disease (HD) is currently being conducted with the goal of performing single-stage radical surgery without ileostomy. Methods We retrospectively reviewed HD cases between 2013 and 2022, as well as their outcomes related to preoperative management. Results Thirty-nine patients with HD were included in this study, including short-segment HD (30 cases), long-segment HD (4 cases), and total colonic aganglionosis (5 cases). Among these 39 patients, 95% (37 of 39 patients) underwent single-stage radical surgery after management with glycerin enema use (n = 13), irrigation with tube insertion each time irrigation was performed (n = 13), and irrigation using a tube placed in the bowel (n = 11). Conclusions Preoperative management of patients with HD allowed for single-stage surgery of long-segment HD and total colonic aganglionosis. Cases that could be managed without performing an emergency enterostomy during the neonatal period were managed with irrigation until radical surgery was performed.

Abstract Background An esophageal anastomotic stricture (EAS) after an esophageal atresia surgery occurs in approximately 4–60% of the cases, and its first-line therapy includes balloon dilatation. Oral balloon dilatation cannot be performed in some EAS cases; conversely, even if dilatation is possible, these strictures recur in some cases, necessitating a surgical procedure for repairing the stenosis. However, these procedures are invasive and have short- and long-term complications. If an EAS recurs repeatedly after multiple balloon dilations, gastroesophageal reflux disease (GERD) may be the underlying cause. A fundoplication procedure may be effective for treating a refractory EAS, as in the present case. Case presentation A neonatal patient with type D esophageal atresia underwent thoracoscopic esophago-esophageal anastomosis at the age of 1 day, and her postoperative course was uneventful. Thereafter, the patient underwent gastrostomy for poor oral intake at the age of 3 months. After gastrostomy, the patient presented with a complete obstructive EAS. Balloon dilatation via the oral route was attempted; however, a guidewire could not be inserted into the EAS site. Hence, retrograde balloon dilatation via gastrostomy was performed successfully. However, the EAS recurred easily thereafter, and laparoscopic anti-reflux surgery was performed to prevent GERD. The anti-reflux surgery cured the otherwise refractory EAS and prevented its recurrence. Conclusions Retrograde balloon dilatation is another treatment option for an EAS. When an EAS recurs soon after dilatation, the patient must be evaluated for GERD; if severe GERD is observed, an appropriate anti-reflux surgery is required before dilating the EAS.

Abstract Background Immunoglobulin G4-related disease (IgG4-RD) is a systemic inflammatory disease and affected individuals typically present with an increased infiltration of IgG4-positive plasma cells in the pancreas, hepatobiliary tract, and liver but rarely in the gastrointestinal tract. Case presentation A 12-year-old girl presented with vomiting and poor weight gain. Gastroscopy revealed duodenal stenosis and ulceration. Computed tomography revealed edematous duodenal wall thickening and air-fluid levels on the right side of the duodenum, which suggested duodenal perforation or penetration. She underwent pancreaticoduodenectomy, and IgG4-RD was diagnosed via histopathology. Conclusions This is the first pediatric case of isolated duodenal IgG4-RD resulting in duodenal obstruction after multiple ulcers. Gastrointestinal IgG4-RD should be among the differential diagnoses of unexplained gastrointestinal obstruction or ulceration even in children.

Background: Pediatric patients often exhibit congenital disorders and rare diseases. Consequently, pediatric surgeons must frequently perform atypical surgeries, dealing with a great deal of stress. Regarding atypical surgeries, navigation is required to ensure safety and surgical precision. In this study, we report our experience with 213 indocyanine green (ICG) fluorescence navigation (ICG-nav) surgeries and introduce our newly developed protocols of ICG-nav for pediatric surgery. Methods: The route and method of ICG administration, surgery, complications, and efficacy of ICG-nav surgery were retrospectively evaluated. Thereafter, the protocols were established. Results: Stable navigation was possible for chylothorax, cholelithiasis, primary hepatoblastoma lesions, hepatoblastoma lung metastases, nephroblastoma liver metastases, organ blood flow, the thoracic duct, lymphatic malformation, and lung segmentectomy. New protocols were established for these cases. Conclusions: ICG-nav could be performed safely in children given that the total amount of ICG administered was carefully controlled. Furthermore, ICG-nav was a highly applicable navigation surgical technique that could be used for treating a wide variety of diseases. To our knowledge, this is the first report of successful ICG-nav for liver metastases from nephroblastoma presented in this series.

Abstract Background We encountered two cases of a new type of retroperitoneal hernia. We herein report the unique features of these cases. Case presentation Case 1: A Japanese girl was born at a gestational age of 37 weeks, weighing 2550 g. She underwent laparotomic left diaphragmatic hernia repair for a left Bochdalek hernia at the age of one day. The postoperative course was uneventful; however, chest radiography at the age of 35 days revealed bowel gas in the mediastinum, while computed tomography exhibited intestinal prolapses from the medial side of the mesh into the thoracic cavity. Reoperation was performed at the age of 77 days, showing that the defect hole was not at the diaphragm but in the absence of retropancreatic fascia, which was connected to the posterior mediastinum from the supramesocolic space. The mediastinum space was closed with a suturing spine and artificial mesh, and the defect hole in the pancreatic body was sutured. Case 2: A Japanese boy was born at a gestational age of 40 weeks, weighing 3502 g. He was diagnosed with a left diaphragmatic hernia at birth and underwent laparotomy at the age of two days. Operative findings showed no defect hole in the diaphragm, and no intestine was observed in the abdominal cavity. After close observation of the abdominal cavity, the intestine was found around the pancreatic body, and manual reduction of the intestine was performed. The defect hole existed in the absence of the retropancreatic fascia, which was connected to the extra-pleural space. The defect hole in the pancreatic body was sutured and closed with a non-absorbable thread. Conclusions We assumed that our cases were a new type of retroperitoneal hernia, which we named “retropancreatic fascia hernia”.

Abstract Background The urachus is an embryonic structure that connects the bladder to the allantois during early embryonic development. Occasionally, it fails to disappear at birth, leading to a case of urachal remnant (UR). This study aimed to determine whether our policy for selecting an appropriate UR resection approach is valid. We performed preoperative imaging to examine whether UR continued toward the bladder apex. If so, the UR and bladder apex were excised using the trans-umbilical approach, in addition to laparoscopy, if necessary. If preoperative imaging indicated that the UR ended near the umbilicus, the UR from the umbilicus to the duct end was resected. Pathological evaluations were performed to determine the appropriateness of the surgical approach indicated by preoperative imaging. Methods We retrospectively reviewed pediatric patients with UR who underwent surgery between 2015 and 2021. Their background characteristics and surgical outcomes were evaluated. Results Twenty patients with UR were included (median age, 7 [interquartile range, 2–10.25] years). UR continued toward the bladder apex in 10 patients and ended near the umbilicus in 10 patients. Urachus tissue at the bladder site was observed when the UR and bladder apex were excised. When UR was resected from the umbilicus to the duct end, urachus tissue was not pathologically detected at the resection margin. Conclusion Our policy results in complete resection without excessive surgical invasion.

Abstract Background Omphalocele is a congenital abdominal wall defect of the umbilical cord insertion site. A giant omphalocele, with a fascial defect > 5 cm in diameter and/or containing > 50% of the liver within the hernia sac, can be challenging for pediatric surgeons. Recently, negative pressure wound therapy has been reported as an effective management for giant omphaloceles; however, it is not recommended for an infected wound with necrotic tissue as it may exacerbate infection. We adopted negative pressure wound therapy with irrigation and dwell time (NPWTi-d) for a case of a ruptured giant omphalocele. Artificial membranes, followed by artificial dermis, were used to promote fibrous capsule formation, and then NPWTi-d was used to promote granulation while controlling infection. However, studies have not been conducted regarding NPWTi-d for ruptured giant omphaloceles; hence, we present our treatment experience with NPWTi-d for a giant omphalocele. Case presentation The patient was a boy born at 38 weeks and 3 days of gestation, weighing 1896 g. He was diagnosed with a ruptured giant omphalocele with a total liver and intestine defect hole of 10 cm × 10 cm. The patient underwent silo placement using an artificial mesh, followed by plicating the artificial mesh at 4 days of age. The herniated viscera were gradually reduced into the abdominal cavity; however, the defect size was still large. Hence, a collagen-based artificial dermis was patched on the defect hole. After creating a fresh and smooth granulated tissue, NPWTi-d was applied at 33 days of age to promote granulation and control infection. We used the 3 M™ V.A.C.® Ulta Therapy Unit with 3 M™ VeraFlo™ therapy. NPWTi-d was stopped at 60 days of age when the granulation tissue was well formed including at the artificial dermis site. The wound was managed with prostandin ointment and appropriate debridement, resulting in complete epithelialization at 5 months of age. Conclusions Artificial membranes followed by artificial dermis were used to promote a fibrous capsule and artificial dermis granulation, which protects against organ damage. NPWTi-d achieved better control of infection and promoted wound healing. NPWTi-d combined with artificial dermis can effectively treat ruptured giant omphaloceles.

Congenital duodenal atresia with situs inversus is occasionally accompanied by a preduodenal portal vein (PDPV), which is incidentally diagnosed during surgery. Duodenoduodenostomy is the most common and effective treatment. However, some patients require other anastomoses. Here, we present two cases of laparoscopic gastrojejunostomy for congenital duodenal atresia with situs inversus and PDPV and describe the reason for selecting gastrojejunostomy. The optimal surgical strategy is patient specific and should be determined based on the patient's general and physical condition.

Retropancreatic fascia hernia is a novel internal hernia originating from the retropancreatic fascial defect, which subsequently expands toward the dorsal aspect of the pancreatic body and migrates into the retroperitoneal space. We encountered a rare case of concomitant retropancreatic fascia and Bochdalek hernias. Here, we describe the imaging characteristics of this hernia type and its surgical strategies.

Abstract Background Esophageal atresia (EA) is often associated with tracheomalacia (TM). The severity of TM symptoms varies widely, with serious cases requiring prolonged respiratory support and surgical treatment. Although we performed thoracoscopic posterior tracheopexy (TPT) during primary EA repair to prevent or reduce the symptoms of TM, few studies have investigated the safety and effectiveness of TPT during primary EA repair. Therefore, this study aimed to evaluate the safety and efficacy of TPT in neonates. Methods We retrospectively reviewed the records of all patients diagnosed with TM who underwent primary thoracoscopic EA repair between 2013 and 2020 at the Nagoya University Hospital. Patients were divided into two groups: TPT (TPT group) and without TPT (control group). TPT has been performed in all patients with EA complicated by TM since 2020. We compared patient backgrounds, surgical outcomes, postoperative complications, and treatment efficacy. Results Of the 22 patients reviewed, eight were in the TPT group and 14 were in the control group. There were no statistically significant differences in the surgical outcomes between the groups (operation time: p = 0.31; blood loss: p = 0.83; time to extubation: p = 0.30; time to start enteral feeding: p = 0.19; time to start oral feeding: p = 0.43). Conversion to open thoracotomy was not performed in any case. The median operative time required for posterior tracheopexy was 10 (8–15) min. There were no statistically significant differences in postoperative complications between the groups (chylothorax: p = 0.36; leakage: p = 1.00; stricture: p = 0.53). The respiratory dependence rate 30 days postoperative (2 [25%] vs. 11 [79%], p = 0.03) and the ratio of the lateral and anterior–posterior diameter of the trachea (LAR) were significantly lower in the TPT group (1.83 [1.66–2.78] vs. 3.59 [1.80–7.70], p = 0.01). Conclusions TPT during primary EA repair for treatment of TM significantly lowered respiratory dependence rate at 30 days postoperative without increasing the risk of postoperative complications. This study suggested that TPT could improve TM associated with EA.

Abstract Background The purpose of this study was to evaluate the utility of endoscopic retrograde cholangiopancreatography (ERCP) in pediatric patients with pancreaticobiliary diseases. Methods A retrospective review was performed on patients who underwent ERCP for the treatment of biliary tract disease and detailed examination of pancreatitis at our institution from January 1999 to December 2020. Results ERCP was performed for congenital biliary dilatation (CBD) (n = 42), choledocholithiasis (n = 9), common bile duct stenosis (n = 1), and several types of pancreatitis (n = 13). The only severe complication of ERCP was common bile duct injury. Three (5.8%) of 52 biliary diseases failed to be treated by ERCP. All patients with pancreatic disease were correctly diagnosed and treated. Conclusions Endoscopic biliary drainage with a temporary stent was adequate for symptomatic relief in CBD. Stenting of the pancreatic duct was useful for improving the angulation and drainage of the pancreatic duct. ERCP was useful for understanding the anatomy of the pancreatic duct and revealing potential treatments. Therefore, ERCP and transendoscopic therapy are sufficiently feasible in pediatric patients and should be actively introduced for the investigation and treatment of pancreaticobiliary diseases.

Background: Laparoscopic definitive surgery for choledochal cyst (CC) in infants requires advanced skills because of their small size. If patients with a prenatal diagnosis of CC have any biliary symptoms, they need semi-emergency definitive surgery. This study aimed to estimate whether laparoscopic definitive surgery for CC can be performed safely and effectively in infants, especially when emergency surgery is required. Patients and Methods: From January 2006 to December 2019, 21 patients under 1 year of age underwent laparoscopically or open definitive surgery, and 16 patients aged 3–5 years underwent laparoscopic surgery for CC at our institution. In cases of prenatal diagnosis, elective surgery (EL) was performed at about 6 months of age for patients with no biliary symptoms; the semi-emergency surgery (EM) was performed when patients had any biliary symptoms. Surgical outcomes were retrospectively compared between the Lap

BackgroundWe present the surgical technique and outcomes of reduced-port laparoscopic restorative proctocolectomy with ileal-J-pouch anal canal anastomosis (IPACA) without diverting ileostomy for total colonic and extensive aganglionosis (TCA+).MethodsWe retrospectively reviewed TCA+ cases between 2014 and 2022. Preoperative ileostomy was performed when transanal bowel irrigation was ineffective. Radical surgery for TCA+ was performed at approximately 6 kg. The surgery was performed using laparoscopy through a multi-channel trocar with or without an additional 3-mm trocar and IPACA reconstruction with indocyanine green fluorescence angiography (ICG) to assess anastomotic perfusion and Lugol's iodine staining to visualize the surgical anal canal.ResultsTen patients with TCA+ were included. Ileostomy was performed in seven cases. The median operation time and blood loss were 274.5 min and 20 ml, respectively. No significant postoperative complications were found. All patients experienced frequent liquid stools and perianal excoriation in the early postoperative period, requiring anti-flatulence or codeine. The median follow-up period was 3.5 years. Three patients required irrigation management 1 year postoperatively, and the others defecated a median of 3.5 times per day. The median Kelly's clinical score was 5 in 5 patients aged >4 years.ConclusionReduced-port surgery, combined with Lugol's iodine staining and ICG, was safe, feasible, and had cosmetically and clinically acceptable mid-term outcomes.

A tracheoesophageal fistula (TEF) recurs in approximately 2%–13% of cases of esophageal atresia with TEF that are treated surgically. Currently, there is no consensus on the most effective treatment to prevent recurrent TEF (RTEF). Herein, we present a patient with type C esophageal atresia who underwent thoracoscopic esophago-esophageal anastomosis and TEF repair at 2 days old. However, RTEFs were observed at ages 3, 6, and 11 months, and thoracoscopic TEF repairs using a pleural patch, fascia lata graft, and pectoralis major myocutaneous (PMMC) flap were performed, respectively. A fourth recurrence led to mediastinitis, shock liver, disseminated intravascular coagulopathy, and a compromised respiratory status. Hence, laparoscopic esophageal transection was first performed to improve the respiratory condition by preventing the regurgitation of gastric contents. Once the patient was stable, a subtotal esophageal resection with TEF closure followed by gastric tube reconstruction was performed. In conclusion, we encountered a case of refractory RTEF that was repaired four times using various techniques, including a fascia lata graft and PMMC flap. However, TEF still recurred after these four operations. The final surgical strategy involved an esophageal transection as a palliative therapy, which improved the respiratory condition, followed by closure of the TEF and subtotal esophageal resection. Finally, esophageal reconstruction using a gastric tube after the complete remission of inflammation was effective. This multi-stage surgery was considered the only choice to rescue the patient and effectively prevent another recurrence.

Hernioplasty for giant inguinal hernias can cause abdominal compartment syndrome (ACS) in adults but rarely does in infants. We encountered a case of a giant bilateral inguinal hernia in infancy complicated by ACS after hernioplasty. Silo placement via a skin incision effectively treated ACS, after which the abdominal wall was safely closed. Hernioplasty performed early in the clinical course can help expand the abdominal cavity and avoid ACS. Thus, hernioplasty should be performed earlier if the hernia size in the flank space gradually increases.

BackgroundThe prognosis of BA is known to be poor if definitive surgery is performed too late. Therefore, excluding BA as a diagnosis at an early stage is crucial. Conventional cholangiography requiring cannulation through the gallbladder may be unnecessarily invasive for patients, especially when ruling out BA. Therefore, a less invasive alternative such as indocyanine green (ICG) cholangiography, which does not require cannulation, should be established. In this study, we focused on excluding BA and confirmed the usefulness of intravenous ICG fluorescence cholangiography. To the best of our knowledge, this is the first preliminary study to report the use of intravenous ICG cholangiography for BA exclusion.MethodsThe study participants were patients who underwent liver biopsy and intraoperative cholangiography after they were suspected to have BA, between 2013 and 2022. ICG fluorescence cholangiography was performed on all patients who provided informed consent.ResultsDuring the study period, 88 patients underwent a laparoscopic liver biopsy and cholangiography. Among them, 65 (74%) were diagnosed with BA and underwent a subsequent laparoscopic Kasai portoenterostomy. BA was ruled out intraoperatively in 23 patients. Of the 23 patients in whom BA was ruled out, 14 underwent ICG cholangiography, 11 had gallbladder (GB) fluorescence, and 9 had both GB and common bile duct (CBD) fluorescence. Conventional cholangiography was very difficult in 2 of 23 cases: in 1 case, cannulation of the atrophic gallbladder was impossible, and cholecystectomy was indicated after multiple attempts; in 1 case, upstream cholangiography was not possible. In both cases, ICG fluorescence cholangiography successfully imaged the CBD and the GB.ConclusionsIn conclusion, intravenous ICG fluorescence cholangiography might be a useful and less invasive diagnostic procedure that can rule out BA in infants.

Abstract Bile duct and anastomotic strictures and intrahepatic stones are common postoperative complications of congenital biliary dilatation (CBD). We performed double-balloon endoscopic retrograde cholangiography (DBERC) for diagnostic and therapeutic purposes after radical surgery. We focused on the effectiveness of DBERC for the treatment of postoperative complications of CBD patients. Bile duct and anastomotic strictures and intrahepatic stones are common postoperative complications of congenital biliary dilatation (CBD). We performed double-balloon endoscopic retrograde cholangiography (DBERC) for diagnostic and therapeutic purposes after radical surgery. We focused on the effectiveness of DBERC for the treatment of postoperative complications of CBD patients. This retrospective study included 28 patients who underwent DBERC (44 procedures) after radical surgery for CBD between January 2011 and December 2019. Strictures were diagnosed as “bile duct strictures” if endoscopy confirmed the presence of bile duct mucosa between the stenotic and anastomotic regions, and as “anastomotic strictures” if the mucosa was absent. The median patient age was 4 (range 0–67) years at the time of primary surgery for CBD and 27.5 (range 8–76) years at the time of DBERC. All anastomotic strictures could be treated with only by 1–2 courses of balloon dilatation of DBERC, while many bile duct strictures (41.2%) needed ≥ 3 treatments, especially those who underwent operative bile duct plasty as the first treatment (83.3%). Although the study was limited by the short follow-up period after DBERC treatment, DBERC is recommended as the first-line treatment for hepatolithiasis associated with biliary and anastomotic strictures in CBD patients, and it can be safely performed multiple times.

Abstract Urine is a complex liquid containing numerous small molecular metabolites. The ability to non-invasively test for cancer biomarkers in urine is especially beneficial for screening child patients. This study attempted to identify neuroblastoma biomarkers by comprehensively analysing urinary metabolite samples from children. A total of 87 urine samples were collected from 54 participants (15 children with neuroblastoma and 39 without cancer) and used to perform a comprehensive analysis. Urine metabolites were extracted using liquid chromatography/mass spectrometry and analysed by Metabolon, Inc. Biomarker candidates were extracted using the Wilcoxon rank sum test, random forest method (RF), and orthogonal partial least squares discriminant analysis (OPLS-DA). RF identified three important metabolic pathways in 15 samples from children with neuroblastoma. One metabolite was selected from each of the three identified pathways and combined to create a biomarker candidate (3-MTS, CTN, and COR) that represented each of the three pathways; using this candidate, all 15 cases were accurately distinguishable from the control group. Two cases in which known biomarkers were negative tested positive using this new biomarker. Furthermore, the predictive value did not decrease in cases with a low therapeutic effect. This approach could be effectively applied to identify biomarkers for other cancer types.

Abstract Background In cases of Hirschsprung disease, complete and reproducible resection of the aganglionic bowel is ideal to achieve good postoperative bowel function. Reliable identification of the upper margin of the surgical anal canal, which is the squamous-columnar junction, is necessary during transanal pull-through. Here, we describe a novel staining technique using Lugol’s iodine stain to visualize the upper margin of the surgical anal canal. Methods Lugol’s iodine staining was performed in five patients with Hirschsprung disease treated using a single-stage laparoscopic transanal pull-through modified Swenson procedure. In two of these patients, endocytoscopic observation with ultra-high magnification was performed using methylene blue and crystal violet to mark the border of the squamous epithelium at 1 week before surgery. The alignment between the incisional line, which was revealed using Lugol’s iodine staining and endocytoscopic marking, was evaluated. Complications, including postoperative bowel dysfunction, were evaluated. Results In all cases, Lugol’s iodine staining produced a well-demarcated line. The endocytoscopic marking of the upper margin of the surgical anal canal was aligned with the line revealed by Lugol’s iodine staining. There were no complications associated with the transanal pull-through procedure, including postoperative bowel dysfunction. Conclusions Lugol’s iodine staining could be a safe and practical method to visualize the upper margin of the surgical anal canal intraoperatively. This finding may be useful for surgeons to make a consistent removal of the aganglionic bowel during surgery for Hirschsprung disease.

Abstract Background The fundamental treatment for patients with pediatric malignant mediastinal tumors is chemotherapy. Therefore, accurate diagnosis is essential for selecting the appropriate chemotherapeutic regimen. However, malignant mediastinal tumors occasionally cause respiratory distress, and biopsies under general anesthesia are dangerous for such patients as invasive mechanical ventilation can aggravate airway obstruction caused by mass effect. In this study, we reviewed our 10-year diagnostic experience to evaluate the efficacy of our practices and confirm a safe diagnostic protocol for future patients. Methods We retrospectively reviewed medical records of children with malignant mediastinal tumors diagnosed at Nagoya University Hospital from 2007 to 2018 who demonstrated respiratory distress. Respiratory distress included dyspnea, massive pleural effusion, wheezing, and hypoxemia owing to tumors. Data on sex, age at onset, primary symptoms, location of tumor, management strategy (especially the method of diagnosis and definitive diagnosis), clinical course, prognosis during the acute phase (within 3 months from the onset of respiratory symptoms), and long-term outcome were collected. Results Twelve pediatric patients met the review criteria. There were seven anterior mediastinal tumors and five posterior mediastinal tumors. All anterior mediastinal tumors were diagnosed via bone marrow smear, thoracentesis, or core needle biopsy while maintaining spontaneous breathing. Regarding posterior tumors, two patients were diagnosed via a core needle biopsy and lymph node excisional biopsy under spontaneous breathing. Two cases were initially diagnosed solely using tumor markers. One patient with severe tracheal compression underwent tumor resection with extracorporeal membrane oxygenation stand-by. No patient died of diagnostic procedure-related complications. Conclusions In 11 of the 12 cases reviewed, safe and accurate tumor diagnosis was accomplished without general anesthesia. A diagnostic strategy without general anesthesia considering the tumor location proved to be useful.

Purpose: Experimental models of laparoscopic surgery generally use large animals owing to a sufficient abdominal working space. We developed a novel laparoscopic surgery model in rats. We performed intestinal anastomosis to demonstrate the feasibility and reliability of this model. Materials and Methods: We designed a device for rats that expanded the abdominal working space and allowed us to manipulate the intraperitoneal organs by hand under direct vision with pneumoperitoneum. We performed small bowel resection and intestinal anastomosis in rats using this model. To elucidate the effects of pneumoperitoneum and skin incision length, rats were randomly divided into four groups with differing surgical techniques: small incision group, large incision group, small incision + pneumoperitoneum group, and large incision + pneumoperitoneum group. Intraoperative abdominal pressure and postoperative cytokines were measured. Results: One experimenter completed small bowel resection and hand-sewn anastomosis under direct vision without any difficulties or assistance. Carbon dioxide pneumoperitoneum was maintained at 8–10 mmHg during surgery in both pneumoperitoneum groups. Necropsies revealed no evidence of anastomotic leakage at 24 h after surgery. The interleukin-6 and C-reactive protein concentrations were significantly greater in large incision group than in small incision group, but were not significantly different between small incision + pneumoperitoneum group and small incision group. These cytokines concentrations were the greatest in large incision + pneumoperitoneum group. Conclusions: Our laparoscopic surgery model in rats is a simple and reliable experimental model. The length of skin incision might be a more influential determinant of surgical invasiveness than pneumoperitoneum.

Abstract Background Emergency rescue lung resection is rarely performed to treat congenital lung cysts (CLCs) in neonates. Many reports have described fetal CLC treatment; however, prenatal predictors for postnatal respiratory failure have not been characterized. We hypothesized that fetal imaging findings are useful predictors of emergency surgery. Methods We retrospectively studied patients with CLC who underwent lung surgery during the neonatal period in our hospital between January 2001 and December 2015. The demographic data, fetal imaging findings, and intra- and postoperative courses of patients who underwent emergency surgery (Em group) were compared with those of patients who received elective surgery, i.e., non-emergency surgery (Ne group). Results The Em group and Ne group included 7 and 11 patients, respectively. No significant difference was noted in gestational age, time at prenatal diagnosis, birth weight, and body weight at surgery. The volumes of contralateral lung per thoracic volume were significantly smaller in the Em group than in the Ne group (p = 0.0188). Mediastinal compression was more common in the Em group (7/7) than in the Ne group (4/11) (p = 0.0128). Conclusions This is the report describing neonatal emergency lobectomy in patients with CLC evaluated by fetal MRI using the lung volume ratio and mediastinal shift. In patients with CLC, mediastinal shift and significant decreases in contralateral lung volumes during the fetal stages are good prenatal predictors of postnatal emergency lung resection.

A right aortic arch (RAA) is found in 5% of neonates with tracheoesophageal fistulae (TEF) and may be associated with vascular rings. Oesophageal repairs for TEF with an RAA via the right chest often pose surgical difficulties. We report for the first time in the world a successful two-stage repair by left-sided thoracoscope for TEF with an RAA and a vascular ring. We switched from right to left thoracoscopy after finding an RAA. A proximal oesophageal pouch was hemmed into the vascular ring; therefore, we selected a two-stage repair. The TEF was resected and simple internal traction was placed into the oesophagus at the first stage. Detailed examination showed the patent ductus arteriosus (PDA) completing a vascular ring. The subsequent primary oesophago-oesophagostomy and dissection of PDA was performed by left-sided thoracoscope. Therefore, left thoracoscopic repair is safe and feasible for treating TEF with an RAA and a vascular ring.

Patients with hepatic arteriovenous malformations rarely present with pulmonary hypertension. We report the case of a 3-month-old boy who developed severe pulmonary hypertension due to a hepatic arteriovenous malformation. The use of pulmonary vasodilators to treat the patient's pulmonary hypertension worsened his high-output heart failure. This is the first case in which emergency hepatectomy rescued a patient with hepatic arteriovenous malformations who developed pulmonary hypertension.