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The management of hepatic hemangioendothelioma (HHE) may be challenging. We aimed to review a large cohort of children who presented to our centers with symptomatic HHE in the last 16 years. We collected age at presentation, clinical features, histology, diagnostic process, management and outcome. Twenty seven patients (male/female 5/22), median age 13 days (1–1530) presented with hepatomegaly (24/27), cardiac failure (10/27), cutaneous hemangiomas (8/27), fever and anemia (6/27 each), vomiting (5/27), splenomegaly (4/27). The lesion was focal, multifocal, or diffuse in 9 patients of each group. The management included medical treatment (8/27), embolization (8/27), resection (3/27), observation (6/27), transplantation (2/27). After 16 months’ follow-up (30 days–11 years), 23/27 (85%) were alive. Diffuse lesions (4/4), cardiac failure (4/4), type II histology (4/4), age older than 6 months at diagnosis (3/4) predicted mortality (all p

Colostomy is considered by most authors the first step of treatment for neonates affected by high or intermediate anorectal malformations (ARMs). However, for this subset of patients, the debate between total diverting colostomy (TDC) and loop colostomy (LC) is still ongoing among the paediatric surgeons community. The aim of this paper is to present our experience consisting in applying the Brooke technique, until now used only for ileostomy, in order to perform a functionally diverting loop colostomy (FDLC) in patients with high or intermediate ARMs. At our institution, from January 2014 to December 2018, 12 patients (6 males and 6 females) with high or intermediate ARMs underwent colostomy according to the Brooke ileostomy technique. The only complication observed was mild proximal stoma prolapse in 1 patient. Creation and closure of the stomas were easy, and the final cosmetic result was very satisfying in all patients. We believe that this is a promising technique since it might overcome the drawbacks of a TDC as it is less invasive, easier, and quicker to perform. Furthermore, it guarantees final better cosmetic results, albeit functioning as a TDC.

S. typhi infection rarely involves the genitourinary system. We report the first described case of acute epididymo-orchitis due to S. typhi in a 14-year-old boy from Bangladesh. A high index of suspicion should be maintained when evaluating patients coming from endemic countries also in case of unusual sites of infection.

We report a child with post-surgical short bowel state who underwent bowel expansion followed by spiral intestinal lengthening and tailoring (SILT) at 10 months of age. Growth at 1-year follow-up is along the 15-25th centile on 82 % oral calories as normal diet and 18 % as parenteral nutrition, and he is passing 2-3 semisolid motions daily. SILT is a versatile technique for reconstructing dilated bowel towards improved propulsion and absorption, and has a role in the management of the short bowel state.

The original version of this article unfortunately contained a mistake. Arnaldo Caruso was not listed among the authors.

We report an extremely rare case of extrarenal testicular Wilms' tumor in a 3-year-old boy with intrabdominal undescended left testis. The patient was admitted because of pain and vomiting, with evidence of a huge abdominal mass. At surgery a large tumor arising from the intrabdominal testis was found. Histology showed the classical triphasic Wilms' tumor elements: epithelial, mesenchymal and blastemal areas. Extrarenal Wilms' tumors account for only 3% of all Wilms' tumors and just -100 cases have been reported in literature. Testicular origin is anecdotic. We present histomorphological, histogenetic, clinical, diagnostic, prognostic and therapeutic features of this rare tumor.

To identify risk factors that can predict prevalence of anastomotic strictures (AS) following esophageal atresia (EA) repair.Of 46 consecutive patients with EA managed at our institution between 2004 and 2012, 35 underwent esophageal anastomosis and were included in this retrospective longitudinal study. Routine endoscopy was performed 1 month after surgical repair. According to stricture index (SI), endoscopically calculated as SI = (D - d)/D, where D is the diameter of the esophageal pouch and d the stricture diameter, population was divided into Group 1, SI ≤ 0.1 (no evidence of stricture); Group 2, 0.3 SI 0.1 (mild stricture); Group 3, SI ≥ 0.3 (high-grade stricture). Trends of subsequent endoscopic esophageal dilatations were compared between the groups using Wilcoxon-Mann-Whitney or Pearson's tests. Cox regression analysis was performed to estimate the hazard ratio.Gastro-esophageal reflux disease (P = 0.04), tension on the anastomosis (P = 0.02) and long-gap form (P = 0.008) have an increased risk of developing AS. SI at 1 month after surgery correlates with the average number of future dilatations: Group 2 and 3 compared to Group 1 required more dilatations (hazard ratio 2.291 and 12.765).AS remain frequent complications of esophageal surgery, especially in specific subgroups of patients. SI at 1 month after surgery could already predict the severity of the stricture and the need for subsequent endoscopic esophageal dilatations.