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Purpose Tissue engineering of esophagus is required for management of long-gap esophageal atresia (LGEA). Collagenous connective tissue membranes fabricated by in-body tissue architecture (iBTA), called biosheets, can repair esophageal defects and generate tissues similar to native esophagus. However, iBTA requires second-stage surgery because of heterotopic preparation of biosheets. Our aim was to develop orthotopic iBTA for primary engineering of the esophagus by interposing a tubular mold to the esophageal defect. Method The cervical esophagus of six rats was transected. An acrylic tube (internal diameter 2.6 mm, length 7.0 mm) was inserted and fixed between the ends of the upper and lower esophagus, and a 3 mm-long esophageal defect was created. Four weeks later, the rats were sacrificed for histological analysis. Results Postoperatively the rats could intake liquid food. After four weeks, the esophageal defects were filled with regenerated tissues. Histologically the new esophageal walls stained positive for collagen type I. The inner surfaces were covered with stratified squamous epithelium that expressed pan-cytokeratin. In only one of six rats, regeneration of muscular-like tissue was suggested by positive immunohistochemical staining for desmin. Conclusion Orthotopic iBTA can regenerate a substitute esophagus with esophageal epithelium and collagenous wall. This technique may be a novel treatment for esophageal atresia with gaps of various lengths including LGEA.

Introduction: Comprehensive genomic profiling (CGP) was widely adopted in Japan after its coverage by national healthcare insurance began in June 2019. We investigated the clinical utility of CGP in pediatric and adolescent young adults (AYA) solid tumor patients. Materials and Methods: Between November 2017 and December 2019, 13 patients who progressed with or who were likely to progress with standard therapies were recruited to the PROFILE-F study to undergo CGP using either FoundationOneⓇ CDx or FoundationOneⓇ Heme. Results: The median age was 28 years old. Tumor types were as follows: neuroblastoma (n = 1), Wilms’ tumor (n = 1), rhabdomyosarcoma (n = 2), Ewing sarcoma (n = 1), gastric cancer (n = 1), rectal cancer (n = 1), osteosarcoma (n = 1), neuroendocrine tumor (n = 2), salivary gland carcinoma (n = 1), tracheal adenoid cystic carcinoma (n = 1), and thymic cancer (n = 1). In 92% of cases, at least one genomic alteration was identified, including CDKN2A (four cases), TP53 (three cases), and MYC (two cases). Actionable aberrations were found in 10 cases (77%), and a clinical trial candidate was found in seven cases (54%). However, no patients were able to receive biomarker-matched therapy according to their genomic alterations. Conclusions: Further efforts to increase basket trials and collection of clinical genomic data to predict response are necessary to advance precision cancer medicine and surgical management in pediatric and AYA populations.

Purpose Pneumothorax often develops in patients with Marfan syndrome (MFS). Here, we examined the effects of conservative and surgical pneumothorax treatments in children with MFS. Methods In this study, 23 patients, less than 20 years old, diagnosed with both MFS and pneumothorax between 1999 and 2019 were included. All data were collected retrospectively from patients’ medical records. Results In total, 18 of 23 patients (78%) had relapsed pneumothorax either on the ipsilateral or contralateral side. Among these 18 patients, 6 (26%) patients had multiple relapses. Conservative and surgical treatments of pneumothorax were attempted in 33 and 29 lungs, respectively. The conservative treatment was attempted as a definitive therapy in 21 lungs. Twelve conservative treatments (57%) failed, which required surgical intervention. In 9 lungs (43%) with successful conservative treatment, 6 (67%) had ipsilateral relapses. In contrast to the above findings, only 4 (13%) ipsilateral relapses were observed in 29 surgical treatments. Conclusions Our study revealed a low response and high relapse rate when MFS adolescents who diagnosed pneumothorax were subjected to the conservative treatment modality. Thus, we recommend surgical intervention as the first line of therapy to treat pneumothorax in adolescents diagnosed with MFS. Level of Evidence Ⅲ (Treatment Study)

Pulmonary hypoplasia is a rare entity in a fetus with imperforate anus. The fetus was diagnosed with high-type imperforate anus with rectourethral fistula based on the dilated fetal bowel and the presence of bowel calcification at 19 weeks of gestation. As gestation advanced, fetal ultrasonography demonstrated development of pulmonary hypoplasia, progressive bowel dilation, and persistent oligohydramnios from 28 weeks of gestation despite a fluid-filled bladder without hydroureter or hydronephrosis. To prevent further worsening of pulmonary hypoplasia caused by thoracic compression due to bowel dilation and oligohydramnios, a male neonate was delivered by cesarean section at 32 weeks of gestation. The neonate showed respiratory failure requiring full respiratory support. Although a catheter did not pass through the urethra into the bladder at birth, cystourethrography revealed the patency of fistula and stenosed lower urinary tract. Prenatal and postnatal findings strongly suggested that the meconium in the colon might have passed into the urethra in the penis, resulting in the physical blockage of urine outflow to the amniotic space which leads urine flow from the bladder to the colon through the fistula, which resulted in subsequent oligohydramnios and bowel dilation. To the best of our knowledge, this is the first case report of a fetus with imperforate anus developing pulmonary hypoplasia possibly due to urethral obstruction.

Chizue Ichijo, Shohei Takami, Kan Suzuki, Jun Fujishiro, Miho Watanabe, Retroperitoneal lymphatic malformation causing scrotal swelling– a useful diagnostic work-up with two-phase MRI to differentiate from scrotal lymphatic malformation or abdomino-scrotal hydrocele, Journal of Pediatric Surgery Case Reports, Volume 64, 2021, 101701, ISSN 2213-5766, https://doi.org/10.1016/j.epsc.2020.101701., Background: Lymphatic malformation (LM) is an abnormal collection of lymphatic fluid within cysts or channels. LM can occur in any part of the body, but LM leading to scrotal swelling is very rare, and this unusual location often leads to diagnostic errors because the most common cause of a scrotal cystic lesion is a hydrocele. In the case presented here, a previously healthy 3-year-old boy recently developed a left scrotal swelling clinically mimicking a communicating hydrocele. However, a diagnostic laparoscopy showed a cystic lesion at the left internal inguinal ring with a closed internal inguinal ring, which is not an expected finding of communicating hydrocele. Differential diagnoses at surgery were scrotal LM, retroperitoneal LM, or abdomino-scrotal hydrocele (ASH). Two phase MRI performed both at the time of scrotal swelling and scrotal non-swelling showed a retroperitoneal LM bulging into the scrotum via the inguinal canal. Therefore, the retroperitoneal LM was completely resected using the inguinal approach. Conclusion: LM causing scrotal cystic lesion is rare, and it requires a high index of suspicion to make the correct diagnosis. Laparoscopy was needed to rule out the communicating hydrocele, and two-phase MRI was very useful to differentiate retroperitoneal LM causing scrotal swelling from scrotal LM or ASH. Both examinations helped with diagnosis and treatment planning.

Infants with Alagille syndrome (AGS) frequently develop neonatal cholestasis, and some AGS infants who suspected of biliary atresia subsequently undergo the Kasai operation with the diagnosis of biliary atresia. The aim of this study was to investigate the effect of the Kasai operation on liver and patient outcomes among AGS patients, using a meta-analysis. A systematic review and meta-analysis of studies describing the outcomes of AGS patients with/without the Kasai operation were conducted. The analyzed outcomes were liver transplantation, not living with the native liver, and mortality for any reason. We identified 6 studies (394 AGS patients). All studies were retrospective cohort or case-control studies. The incidences of liver transplantation, not living with the native liver, and mortality were significantly higher in AGS patients who underwent the Kasai operation than in those who did not undergo the Kasai operation (odds ratio: 6.46, 95% CI 3.23–12.89, p

Biliary atresia (BA) is a destructive inflammatory obliterative cholangiopathy of the neonate that affects various parts of the bile duct. If early diagnosis followed by Kasai portoenterostomy is not performed, progressive liver cirrhosis frequently leads to liver transplantation in the early stage of life. Therefore, prompt diagnosis is necessary for the rescue of BA patients. However, the prompt diagnosis of BA remains challenging because specific and reliable biomarkers for BA are currently unavailable. In this study, we discovered potential biomarkers for BA using deep proteome analysis by data-independent acquisition mass spectrometry (DIA–MS). Four patients with BA and three patients with neonatal cholestasis of other etiologies (non-BA) were recruited for stool proteome analysis. Among the 2110 host-derived proteins detected in their stools, 49 proteins were significantly higher in patients with BA and 54 proteins were significantly lower. These varying stool protein levels in infants with BA can provide potential biomarkers for BA. As demonstrated in this study, the deep proteome analysis of stools has great potential not only in detecting new stool biomarkers for BA but also in elucidating the pathophysiology of BA and other pediatric diseases, especially in the field of pediatric gastroenterology.

Tracheomalacia is a major cause of morbidity in conditions such as oesophageal atresia. However, symptoms usually improve with age. A more rapid growth of tracheal cartilage can be induced by basic-Fibroblast Growth Factor (b-FGF). This study aimed to investigate whether slow-release b-FGF could act as a novel treatment for tracheomalacia.Biodegradable gelatin hydrogel sheets incorporating 0.5, 5, or 50 μg/20 μl of b-FGF solution were inserted between the cervical trachea and esophagus of rats. No intervention was performed in rats in a control group. All animals were sacrificed 4 weeks later, and the luminal area of the cervical trachea and the thickness of the cartilage were measured.The mean luminal areas in the control group and in the b-FGF groups were 3.1, 3.2, 3.8, and 2.6mm(2), respectively, and showed a peak area at 5 μg of b-FGF. A significant difference was seen only between the control group and the b-FGF 5 μg group (p0.05). The mean thickness of the tracheal cartilage was 0.12, 0.13, 0.19, and 0.32 mm in the control and the b-FGF groups, respectively, and showed a dose-dependent increase, which was statistically significant between the b-FGF 5 μg or 50 μg groups and the control group (p0.01).This study showed that slow-release b-FGF enlarges the tracheal lumen and thickens the cartilage in a dose-dependent fashion.

We report here on a case of condyloma acuminatum in the neourethra after urethroplasty for hypospadias. A 37-year-old male visited an other clinic with the chief complaint of a meatal tumor. He had undergone a urethroplasty for hypospadias in his childhood. He was diagnosed as having condyloma acuminatum of the urethral meatus, and was referred to our hospital. Some other lesions in the neourethra were recognized under urethroscopy. We firstly resected the meatal tumor, and diagnosed it as condyloma acuminatum, pathologically. Thereafter, we performed transurethral resection of the urethral condyloma acuminatum. Recurrence of the condyloma acuminatum was noted after 6 months, postoperatively, so we performed TUR again. No recurrence has been seen to date.

This study aimed to develop a novel procedure for treating long-gap pure esophageal atresia. This procedure, which entails the combined use of laparoscopy and natural orifice translumenal endoscopic surgery (NOTES), would enable primary repair without cervical and thoracic incisions and prevent postoperative gastroesophageal reflux disease (GERD).Nonsurvival experiments were conducted in 9 pigs to study the technical feasibility. The procedure comprised the following: (1) creation of the disease model by laparoscopic resection of the lower esophagus; (2) laparoscopic fundoplication, complete mobilization of the stomach, and enlargement of the esophageal hiatus; (3) formation of a peroral transesophageal entry site into either the postmediastinum or the right thoracic cavity followed by fashioning a tunnel to the peritoneal cavity; (4) gastric pull-up by using both laparoscopy and NOTES; (5) esophagoesophageal anastomosis using BraceBar™, a prototype of the double T-bar suturing device (Olympus Medical Systems Co., Tokyo, Japan).Laparoscopic procedures were performed without complications. The postmediastinal tunnel was successfully created three times with a complication of pleural injury. However, gastric pull-up via this route could not be completed due to porcine anatomical reasons. Gastric pull-up through the right thoracic route was achieved five times in six attempts. Two disorientations and a hemorrhagic death occurred during the procedures.This study showed that combined use of laparoscopy and NOTES enabled gastric pull-up without cervical and thoracic incisions. Our method has the potential of lowering the incidence of GERD and enabling primary repair of this disease.

A 41-day-old female infant with VACTERL association was transferred to the pediatric intensive care unit of our hospital. She had been delivered at 36 weeks gestation by spontaneous vaginal delivery and weighed 2340 g. Esophageal atresia type A with long gap, anal atresia, cardiac anomaly (atrial septal defect and patent ductus arteriosus), thoracic vertebral dysplasia, left renal agenesis, and minor anomalies (left-side facial nerve palsy, left-side difficulty in hearing, and the absence of the right thenar) had been diagnosed by various examinations. She was transferred to our hospital to receive treatment for heart failure due to a cardiac anomaly. We recognized vaginal atresia during a radical operation for anal atresia (rectovestibular fistula) at 8 months of age. Furthermore, magnetic resonance imaging (MRI) revealed agenesis of the uterus. MURCS association includes Mullerian duct aplasia or hypoplasia, renal aplasia, and cervicothoracic somite dysplasia. This is the first case of complete VACTERL association combined with MURCS association.

Body stalk anomaly is characterized by severe scoliosis, severe pulmonary hypoplasia, and giant omphalocele. The prognosis of the disease is poor and most obstetricians consider it fatal. Very few patients with body stalk anomaly survive. We report the case of a baby diagnosed with body stalk anomaly in fetal life, who was saved by intensive care after birth. We closed the giant omphalocele successfully by placing karaya gum sheets over it, which created a humidified environment and promoted natural skin epithelization over the skin defect.

Open surgery is the standard therapeutic approach for renal malignant tumor in children; while in adults, laparoscopic radical nephrectomy (LRN) is used. Only few reports on LRN in children have been published because the most common form of pediatric renal malignancy, Wilms tumor, is well known for presenting with large palpable masses. Here, we report the case of a 13-year-old girl with small chromophobe renal cell carcinoma (RCC) who was managed by LRN. RCC is a rare condition, with an incidence rate of only 2–6% of all renal tumors in children. We found LRN for small RCC to be feasible and safe.