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Background/Objectives: Transurethral incision (TUI) is a common procedure for posterior urethral valves (PUV). However, no standardized method has been established to assess its efficacy intraoperatively. In this study, we aimed to develop and evaluate a real-time ultrasound-guided TUI (RUG-TUI) technique. Methods: A single-center, retrospective feasibility study with a cohort design was conducted using historical controls to compare RUG-TUI with standard TUI in children with PUV. Data from patients who underwent RUG-TUI for PUV between April 2021 and July 2022 or TUI without real-time ultrasound guidance between August 2020 and March 2021 (control group) were retrospectively reviewed. A transperineal linear probe provided longitudinal imaging. The diameters of the constricted (C) and expanded (E) portions of the urethra before and after the procedure were measured, and the E/C ratio was calculated. The primary outcome was the duration of postoperative gross hematuria, and the secondary outcomes included changes in the urethral diameter ratio (E/C ratio), intraoperative complications, and residual obstruction on VCUG. Results: The mean duration of post-procedure macroscopic hematuria was significantly shorter in the RUG-TUI group than in the control group (p = 0.049). No massive intraoperative bleeding or sphincter damage occurred. In the RUG-TUI group, the mean diameters of the constricted segment before and after the procedure were 3.0 (±1.0) and 5.7 (±1.2) mm, respectively, while the pre- and postoperative E/C ratios were 1.8 (±0.5) and 0.9 (±0.1), respectively (p < 0.0001). Conclusions: RUG-TUI for PUV enabled visualization of the longitudinal axis of the urethra, allowing assessment of the anatomical relationship between the stenosis and external urethral sphincter. In this retrospective feasibility cohort, RUG-TUI was associated with a shorter duration of postoperative gross hematuria. These exploratory findings suggest that RUG-TUI may support intraoperative evaluation of procedural adequacy.

PurposeTo determine the features which predict torsion and the pre-operative indicators of malignancy in cases of ovarian torsion in ovarian tumors (OTs) in children.MethodsThe medical records of 35 pediatric patients who underwent surgery for OT, except for neonate cases, from 1997 to 2018 at our institution were reviewed retrospectively.ResultsThe pathological diagnosis was mature teratoma in 17, immature teratoma in 9, yolk sac tumor in 3, and others in 6. The preoperative diagnosis, which was made based on the imaging findings and the serum tumor marker values, matched with the pathological diagnosis in 29/35 (83%). Ovarian torsion occurred in 14/35 (40%). All but one case that presented with torsion had intermittent abdominal pain as the primary symptom. The preoperative white blood cell count was significantly higher in cases where ovary preservation was impossible than where it was possible (p = 0.01) among the cases presenting with torsion.ConclusionPreoperative imaging findings and the serum tumor marker values enabled us to make an accurate preoperative diagnosis. Patients with intermittent abdominal primary symptoms were more likely to have ovarian torsion than those without such symptoms, and leukocytosis may indicate irreversible ischemic changes in the affected ovary.

ObjectiveTo determine the appropriate initial ventilatory mode for neonatal congenital diaphragmatic hernia (CDH) by comparing patient prognosis following conventional mechanical ventilation (CMV) versus high-frequency oscillatory ventilation (HFO).Study designThis multicenter retrospective cohort study was performed at 15 participating hospitals in Japan between 2011 and 2016. The 328 eligible CDH infants were classified into CMV (n = 78) and HFO groups (n = 250) to compare mortality and incidence of bronchopulmonary dysplasia (BPD). Propensity score matching was applied to reduce confounding by indication.ResultWhile crude mortality was significantly higher in the HFO than the CMV group, adjusted odds ratio (OR) did not show significant difference in mortality between groups (OR of HFO group: 0.98, 95% confidence interval (CI): 0.57–1.67). Adjusted OR of BPD incidence showed no significant difference between groups (OR of HFO group: 1.66, 95%CI: 0.50–5.49).ConclusionInitial ventilatory mode in CDH patients, whether CMV or HFO, does not affect prognosis.

Patch closure is necessary to achieve tension-free repair in large congenital diaphragmatic hernia. However, the use of prosthetic material may lead to granulation, allergic reaction, infection, recurrence of hernia, and thoracic deformity. Tissue engineering may become an alternative treatment strategy for diaphragmatic hernia repair, since the regenerated autologous tissue is expected to grow potentially without rejection or infection. We evaluated the efficacy of diaphragmatic hernia repair in a rat model using a poly-lactic-co-glycolic acid (PLGA) mesh–collagen sponge hybrid scaffold, designed for in situ tissue engineering. Twenty-four F344 female rats were used. Oval-shaped defects were surgically created in the left diaphragm and repaired with three different grafts, including PLGA mesh in group 1 (n = 7), PLGA mesh–collagen sponge hybrid scaffold in group 2 (n = 7), and PLGA mesh–collagen sponge hybrid scaffold seeded with bone marrow-derived mesenchymal stem cells (MSCs) in group 3 (n = 10). The animals were killed at 1, 2, and 3 months after operation. The specimens were examined macroscopically and microscopically. No recurrence or eventration was observed. In all animals, autologous fibrous tissue with vascularization was generated at the graft site. Although no muscular tissue was detected, scattered desmin-positive cells were observed in groups 2 and 3. The 'neodiaphragm' in groups 2 and 3 was significantly thicker compared with that in group 1. There was no significant difference in the 'neodiaphragm' between groups 2 and 3. The PLGA mesh–collagen sponge hybrid scaffold provided better promotion of autologous in situ tissue regeneration in the diaphragm, suggesting its potential application to diaphragmatic repair in place of other prosthetic patches. application/pdf

Recently, tissue engineering of the autologous esophagus has been thought to provide a promising strategy for esophageal substitution. In this study, gastric acellular matrix (GAM) was used as a scaffold for regeneration of the esophagus in a rat model. Usage of GAM has an advantage that naturally derived extracellular matrix autograft can be prepared less invasively in a clinical setting. Twenty-seven F344 female rats were used as recipients. Patch defects created in the abdominal esophagus were replaced by GAM patch grafts. The rats were sacrificed 1 week to 18 months after implantation. The specimen was examined macroscopically as well as microscopically. 5'-Bromo-2'-deoxyuridine (BrdU) proliferation assay was performed in six rats that were sacrificed 1, 2, and 4 weeks after implantation. Twenty-four rats survived without complications. The graft site did not show esophageal stenosis or dilatation in any rat. Keratinized stratified squamous esophageal mucosa was regenerated in the entire graft 2 weeks after implantation. Regeneration of the muscle layer or lamina muscularis mucosae in the graft site was not observed even 18 months after implantation. Marked incorporation of BrdU was observed only in the mucosal layer but not in the muscle layer. GAM patch graft provided satisfactory mucosal regeneration of the esophagus without stenosis or dilatation, although muscle regeneration was still a future challenge.

Purpose Infants with higher anorectal anomalies often develop fecal incontinence after surgical reconstruction mainly due to the incomplete development of defecation muscles. We investigated the possibility of defecation muscle regeneration by myoblast transplantation to improve fecal continence. Methods Myoblasts from F344 female rats at ages of 1 day, 1, 2, 3, 4, 8, and 12 weeks were prepared by a preplating method. In vivo muscle differentiation of myoblasts was evaluated using immunofluorescence after transplantation of GFP-positive myoblasts into nude mice, the damaged thigh muscles, and the levator ani muscle of GFP-negative rats. Results The ratios of myoblasts obtained from 1 day, 1, 2, 3, 4, 8, and 12-week-old rats were 35, 71, 65, 61, 52, 44, and 23%, respectively. Myotube formation by transplanted myoblasts was observed in the back of nude mice. Myoblasts transplanted into damaged thigh muscles were integrated into recipient muscles with myofiber formation. Transferred myoblasts formed myotubes surrounding the levator ani muscle, although myofiber formation was not observed. Conclusion Myoblasts were most efficiently obtained from juvenile rats. Myoblast transplantation may provide a novel treatment strategy for improving fecal continence after repair of anorectal anomalies in infants.

Currently, magnetic resonance cholangiography (MRC) is used for the differentiation of biliary atresia (BA) from other causes of infantile cholestasis. The authors present a case of type I BA without an extrahepatic biliary cyst in a 2-month-old girl. MRC clearly visualized the patency of the gallbladder, cystic duct, and hepatic ducts with disappearance of the common bile duct. Intraoperative cholangiography demonstrated a cloudy appearance of the intrahepatic bile ducts, confirming the diagnosis of type I BA. We believe that this is the first reported case of type I BA without an extrahepatic biliary cyst diagnosed by MRC.

Pancreatitis, a late complication of an annular pancreas (AP), results from coexisting pancreaticobiliary malformations including pancreas divisum (PD), and pancreaticobiliary maljunction (PBM). The authors report the case of a 3-year-old boy with an unusual type of AP in which the dorsal anlage encircled the duodenum. The patient developed duodenal obstruction as well as duodenopancreatic reflux with resulting hyperamylasemia and hyperlipasemia. This type of AP associated with duodenopancreatic reflux in AP has not been reported previously. The patient was successfully treated by duodenoduodenostomy, which, by correcting the duodenopancreatic reflux, prevented the later development of pancreatitis.

Nevoid basal cell carcinoma syndrome (NBCCS) is characterized by basal cell carcinoma, skeletal abnormalities, benign tumors including ovarian fibroma, and various other phenotypic expressions. Ovarian fibromas in NBCCS before puberty are very rare. We report a 6-year-old prepubescent girl with NBCCS showing skeletal abnormalities, medulloblastoma, and ovarian fibromas. The patient was referred to our hospital owing to abdominal distension. On admission, a huge elastic hard tumor was palpable and computed tomography showed a huge tumor of the left ovary. We performed a left salpingo-oophorectomy and diagnosed the tumor as a benign fibroma. Further examination of the computed tomography images showed skeletal abnormalities. In addition, the patient had a history of medulloblastoma at the age of 4 years. Therefore, we diagnosed NBCCS. A genetic examination indicated a novel 1 bp deletion in exon 18 (c.3055delG). Sequence analysis of exon 18 using DNA from the ovarian tumor revealed a mutant allele (c.3055delG) dominant to the wild-type allele, thus suggesting loss of heterozygosity in the PTCH1 gene, which is known to be associated with NBCCS. Conclusion On the basis of our experience, physicians treating pediatric ovarian tumors should be aware that such huge benign ovarian tumors may be a phenotype of NBCCS, as shown in our patient. In addition, genetic examination focusing on the PTCH1 gene might be important for diagnosis of NBCCS in pediatric patients.

Cloacal dysgenesis sequence (CDS) is a rare and lethal malformation. We report such a case of long-term survival, currently to 12 years of age. In the fetal period, she received a timely placement of vesico-amniotic shunt for a megabladder due to a severe urethral obstruction. Postnatally, cystostomy and colostomy were created because of no perineal opening of urethra, vagina, and anus. Anorectoplasty, construction of efferent conduit, and colostomy closure were performed at 4 years of age. Ileovaginoplasty and ileovesicostomy which was a Mitrofanoff-type of conduit, and labioplasty were performed at the age of 11 years. To the best of our knowledge, only five survivors with CDS over 1 year of age have been reported.