Pediatric Surgery International

A large, nonspecific, chronic ulcer was found in the sigmoid colon of a 13-year-old child with neglected, undiagnosed Hirschsprung’s disease (HD). There is no known association between HD and colonic ulcers, suggesting that the ulcer was a true stercoraceous ulcer of the colon and not an intrinsic defect of the aganglionotic bowel.

In a current publications, it has been suggested that hypoxia-triggered "good angiogenesis" involving hypoxic up-regulation of vascular endothelial growth factor (VEGF) molecules, genes, and receptors is likely responsible in a major way for the remarkable gut and patient salvage experience associated with use of the "patch, drain, and wait" (PD&W) surgical approach to perforated necrotizing enterocolitis and midgut volvulus (MGV) with extensive ischemia/necrosis. We report a case in which extensive ischemia/necrosis in a newborn with gastroschisis (likely MGV-induced) was managed successfully by PD&W with an associated marked (24-fold) elevation of VEGF in drainage fluid at 7 days post-initiation of PD&W.

Severe chordee with hypospadias may require repair by ventral corporoplasty with a free graft before urethroplasty. We report an 11 year experience with one-stage and two-stage hypospadias repair using dermal grafts. From 12/1997 to 12/2008, 47 hypospadias with severe chordee were repaired using a ventral dermal graft to correct the chordee. Forty cases were repaired in two stages; the remaining seven patients had adequate preputial skin and were repaired in one stage. In 28 cases the neourethra was covered with both tunica vaginalis and penile dartos flap while 12 were covered only with penile dartos flap. Patients ranged from 12 to 152 months of age. Follow-up ranged from 6 months to 4 years. 28 patients with both tunica vaginalis and dartos flap had 7 urethral fistulas and 2 developed meatal stenosis. 12 patients with only penile dartos flap had 3 fistulas and 2 had meatal regression. The seven patients who underwent a one-stage repair had good results with a straight penis and no evidence of urethral fistula or urethral stenosis. Dermal graft can be performed in one or two stage hypospadias repair. Complication rates are similar to other standard repairs for proximal hypospadias.

Neonates with gastroschisis make up an increasing proportion of prolonged surgical NICU admissions. While infectious complications are known to increase patient morbidity, it is unclear whether they vary according to abdominal closure method, or can be predicted by initial patient assessment. A national, prospective, disease-specific database was evaluated for episodes of wound infection (WI) and catheter-related infection (CRI). Antibiotic use and timing, as well as method and location of abdominal closure were studied. The gastroschisis prognostic score (GPS) was calculated and evaluated as a predictor of infectious complications. Of 395 patients, 48 (12.6%) had a documented abdominal WI, and 59 patients (14.9%) had at least one episode of CRI-most commonly coagulase negative staphylococcus. Most abdominal closures took place within 6 h of admission (194 = 51.3%), while 132 (34.9%) were delayed greater than 24 h. The WI rate was greater in the delayed group (21.2 vs. 8.2%, p = 0.0006). The GPS was found to predict development of an infectious complication (WI + CRI, p = 0.04). Infectious complications remain an important consideration in the management of gastroschisis. GPS correlates with the development of infectious complications. Prophylaxis for skin flora and early closure, when feasible, may reduce WI rates.

Female urethral injuries may cause serious morbidity with loss of the entire urethra in some cases. We review the results of construction of a neourethra by the flipped anterior bladder-tube technique in five girls aged 3–18 years (mean 8 years). All had pelvic fractures and had their initial management elsewhere; four were run over by motor vehicles and one suffered a pelvic gunshot injury. Three patients came with a suprapubic cystostomy, one was totally incontinent, and another had a urethrovaginal fistula. Three had an associated vaginal injury. All five patients underwent construction of a neourethra using a flipped anterior bladder-wall tube (FABWT) and have been followed for 18–80 months (mean 44 months). All patients were continent in the daytime and three passed urine spontaneously. Two were managed by clean intermittent catheterization (CIC), one per urethram and other via a continent abdominal stoma (Mitrofanoff). The FABWT technique should be considered as an option in the management of females with severe urethral injury.

The biological heterogeneity of neuroblastoma results in a varied outcome ranging from spontaneous regression to fatal tumor progression. Microarray expression profiling and genetic polymorphism arrays may help identify key genes that differ in aggressive neuroblastomas from those observed in tumors associated with a favorable outcome. Total RNA was extracted from 16 neuroblastomas obtained from patients who subsequently died of the disease and from 16 favorable neuroblastomas and analyzed using a human whole genome oligomicroarray (55K CodeLink). Genes overexpressed in favorable tumors were subsequently analyzed in 121 neuroblastoma tumors obtained before chemotherapy using real-time RT-PCR. And among these cases, expression levels of these genes were also analyzed in 20 tumors obtained after chemotherapy. Oligomicroarray analysis revealed the overexpression of 283 genes in favorable tumors that were associated with either regressing or maturing tumors. Three candidate genes, including DHRS3, NROB1, and CYP26A1, were selected that were significantly overexpressed in favorable tumors by quantitative real-time RT-PCR (P < 0.01). No cases with overexpression of all three genes showed poor outcomes. In 20 post-chemotherapeutic tumors, the expression levels of these genes increased in the cases where patients survived but decreased in the fatal cases. Using microarray expression profiling, we identified genes that exhibit altered gene expression in neuroblastoma tumors associated with a favorable outcome. These candidates warrant further study for use in risk assessment and/or as therapeutic targets in neuroblastoma.

The Treves’ field congenital hernias (TFCH) are an unusual cause of bowel obstruction that may result in irreversible damage of the small bowel or a fatal outcome, unless treated timely and properly. We analyzed retrospectively the medical records of four patients who underwent surgery for TFCHs over a period of 9 years. The types of TFCHs, only diagnosed at surgery, were identified as transmesenteric through a defect in Treves’ field in three patients, and as hernia into Treves’ field pouch in one patient. Patients had non-specific clinical manifestations, and imaging findings demonstrated only small bowel obstruction. Delay in accurate preoperative diagnosis, and especially patient-related delay, led to a primary enterectomy–anastomosis for necrotic small bowel in the patients with transmesenteric hernias. One infant with necrotic bowel died postoperatively. It may be difficult to diagnose TFCHs in children preoperatively. Misdiagnosis of bowel obstruction caused by this type of internal hernia might lead to small bowel necrosis or death. The risk of this complication seems to be more influenced by pre-hospital delay. Awareness of the severe consequences of delay in diagnosis, high clinical suspicion, early recognition, and proper surgical intervention are essential in the successful management of TFCHs.

The purpose of our study is to identify potential biomarkers of hepatoblastoma (HB) and further explore the pathogenesis of it. Differentially expressed genes (DEGs) were incorporated into the combined random forest and artificial neural network diagnosis model to screen candidate genes for HB. Gene set enrichment analysis (GSEA) was used to analyze the ARHGEF2. Student’s t test was performed to evaluate the difference of tumor-infiltrating immune cells (TIICs) between normal and HB samples. Spearson correlation analysis was used to calculate the correlation between ARHGEF2 and TIICs. ARHGEF2, TCF3, TMED3, STMN1 and RAVER2 were screened by the new model. The GSEA of ARHGEF2 included cell cycle pathway and antigen processing presenting pathway. There were significant differences in the composition of partial TIICs between HB and normal samples (p < 0.05). ARHGEF2 was significantly correlated with memory B cells (Cor = 0.509, p < 0.05). These 5 candidate genes contribute to the molecular diagnosis and targeted therapy of HB. And we found “ARHGEF2–RhoA–Cyclin D1/CDK4/CDK6–EF2” is a key mechanism regulating cell cycle pathway in HB. This will be helpful in the treatment of HB. The occurrence of HB is related to abnormal TIICs. We speculated that memory B cells play an important role in HB.