Delayed diagnosis in Hirschsprung disease.

Introduction: Diagnostic delay in Hirschsprung disease is uncommon. Different definitions have been proposed but that of a diagnosis achieved after 12 months of age seems to be the most reliable and resorted to. Some authors reported a worse outcome in case of delay.

A unicentric cross-sectional observational study on chronic intestinal inflammation in total colonic aganglionosis: beware of an underestimated condition.

Background: Inflammatory Bowel Diseases (IBD) are known to occur in association with Hirschsprung disease (HSCR). Most of cases are represented by Crohn Disease (CD) occurring in patients with Total Colonic Aganglionosis (TCSA) with an estimated prevalence of around 2%. Based on these considerations and on a number of provisional data belonging to our Center for Digestive Diseases, we developed a unicentric cross-sectional observational study aimed at describing phenotype, genotype, pathology and metagenomics of all patients with TCSA and Crohn-like lesions.

White matter and cerebellar involvement in alternating hemiplegia of childhood.

Objective: To determine whether brain volumetric and white matter microstructural changes are present and correlate with neurological impairment in subjects with alternating hemiplegia of childhood (AHC).

Methods: In this prospective single-center study, 12 AHC subjects (mean age 22.9 years) and 24 controls were studied with 3DT1-weighted MR imaging and high angular resolution diffusion imaging at 3T.

Complications of Minimally Invasive Surgery in Children: A Prospective Morbidity and Mortality Analysis Using the Clavien-Dindo Classification.

There is a lack of information about evaluation of pediatric minimal access surgery complications; the Clavien-Dindo classification was never used for a large series of laparoscopic and thoracoscopic pediatric procedures. With a prospective Morbidity and Mortality database, all the minimal access surgical procedures carried out between 2012 and 2016 were included in this study. Statistical analyses were used to valuate modification of surgical techniques in to 2 periods (period 1: January 2012 to February 2014; period 2: February 2014 to February 2016).

A Provisional Experience with Robot-Assisted Soave Procedure for Older Children with Hirschsprung Disease: Back to the Future?

Hirschsprung disease is a congenital disease characterized by intestinal aganglionosis of various extents. Most patients are younger than 1 year of age. Though, a minority of cases can be older or even adult.

Prenatal Echocardiographic Assessment of Foramen Ovale Appearance in Fetuses with D-Transposition of the Great Arteries and Impact on Neonatal Outcome.

Introduction: Neonates with D-transposition of the great arteries (dTGA) may die at birth because of the inadequate intracardiac mixing due to a misdiagnosed restrictive foramen ovale. We reviewed our experience in echocardiographic assessment and perinatal management of fetuses with dTGA searching for new features that may predict the need for urgent balloon atrial septostomy (BAS) immediately after birth.

Patients And Methods: We included fetuses diagnosed with dTGA between January 2000 and December 2014.

Brachiocephalic vein for percutaneous ultrasound-guided central line positioning in children: A 20-month preliminary experience with 109 procedures.

Background: Ultrasound-guided (USG) cannulation of the brachiocephalic vein (BCV) is gaining worldwide consensus for central venous access in children. This study reports a 20-month experience with this approach in children.

Methods: All patients who underwent percutaneous USG central venous catheter (CVC) positioning in the BCV between August 2013 and March 2015 have been included.

Congenital Lung Malformations: Shifting from Open to Thoracoscopic Surgery.

Background: Over the years the need for surgical treatment, timing of intervention, and the type of surgical approach have been discussed, but the treatment of congenital lung malformations remains controversial. The aim of this study was to compare the thoracotomy approach with the thoracoscopic technique by evaluating different surgical outcomes (duration of surgery, postoperative hospital stay, and complications).

Methods: All patients operated from January 2011 to March 2015 for suspected congenital cystic lung were included in the study.

Video-Assisted Needle Core Biopsy in Children Affected by Neuroblastoma: A Novel Combined Technique.

 This study aims to evaluate the experience gained with video-assisted needle-core biopsy in patients affected by neuroblastoma (NB).  We retrospectively reviewed all the patients presenting at our center with a thoracic, abdominal, and/or pelvic NB who underwent biopsy between 2007 and 2014. Data on demographics, localization, and size of the tumor, image-defined risk factors involved in each case, technical details about biopsies performed, qualitative and quantitative adequacy of tumor sampling and histological diagnosis, postoperative details, and complications were recorded and analyzed.

Preliminary results of video-assisted anal fistula treatment (VAAFT) in children.

Background: Anal fistula is a common acquired anorectal disorder in children. Treatment methods that have been used are associated with inconsistent results and possible serious complications. In 2011 a minimally invasive approach, video-assisted anal fistula treatment (VAAFT) was described for adult patients.

Long-term outcome and need of re-operation in gastro-esophageal reflux surgery in children.

Background: Fundoplication is considered a mainstay in the treatment of gastro-esophageal reflux. However, the literature reports significant recurrences and limited data on long-term outcome.

Aims: To evaluate our long-term outcomes of antireflux surgery in children and to assess the results of redo surgery.

Minimally invasive surgery for paediatric inflammatory bowel disease: Personal experience and literature review.

The incidence of paediatric inflammatory bowel disease (PIBD) has dramatically increased in the last 20 years. Although first reported in mid 1970s', diagnostic laparoscopy has started to be routinely adopted in paediatric surgical practice since late 1990s'. Minimally invasive surgery was first limited to diagnostic purposes.

Transperitoneal Laparoscopic Approach to the Perinephric Area in Children: Technical Report and Lessons Learned.

Purpose: This study presents a technical report of a standardized approach to the perinephric area in a series of pediatric patients, demonstrating that whatever renal or suprarenal surgery is planned, this can be approached and accomplished laparoscopically with an identical or very similar port triangulation, thus facilitating the learning curve within the same surgical team.

Patients And Methods: All patients undergoing renal and adrenal gland surgery with a minimally invasive approach in the period from October 2008 to November 2013 were retrospectively reviewed and included in the study. Technical details and clinical outcomes are described.

Cone-like resection, fistulectomy and mucosal rectal sleeve partial endorectal pull-through in paediatric Crohn's disease with perianal complex fistula.

Background: Perianal abscesses and fistulae have been reported in approximately 15% of patients with paediatric Crohn's disease and they are associated with poor quality of life. Several surgical techniques were proposed for the treatment of perianal Crohn's disease, characterized by an elevated incidence of failure, incontinence, and relapse. Aim of our study was to present the technical details and results of our surgical technique in case of recurrent, persistent, complex perianal ano-rectal destroying Crohn's disease not responding to medical treatment.

Inguinal hernia in neonates and ex-preterm: complications, timing and need for routine contralateral exploration.

Background/purpose: Aim of this study was to present a series of neonates and ex-preterm babies who underwent inguinal hernia repair focusing on complications and possible indication to perform routine contralateral groin exploration.

Methods: This is a retrospective study of a series of consecutive patients weighing less than 5 kg who underwent inguinal hernia repair between January 2007 and December 2012. Only the affected side was treated.

[Role in mini-invasive surgery in children].

Aim: This paper describes our experience with minimally invasive surgery and underlines its increasingly important role in the treatment of pediatric patients.

Methods: The study included all those patients undergoing a minimally invasive surgical procedure between January 2006 and March 2012. Patient demographics, type of operation (classified according to disease and anatomic site), complications, and length of hospital stay (LOS) were recorded.

A novel balanced isotonic sodium solution vs normal saline during major surgery in children up to 36 months: a multicenter RCT.

Background: The use of isotonic electrolytic solutions for the intraoperative fluid management in children is largely recognized, but the exact composition still needs to be defined.

Objectives: The primary objective of this randomized controlled open trial was to compare the changes in chloride plasma concentration using two intraoperative isotonic fluid regimens (Sterofundin vs. normal saline, both added with 1% of glucose) in children undergoing major surgery.

Beneficial effect of fenoldopam mesylate in preventing peak blood lactate level during cardiopulmonary bypass for paediatric cardiac surgery.

Objectives: To evaluate the efficacy of fenoldopam mesylate (dose 0.2 µg/kg/min) in reducing the occurrence of hyperlactataemia (i.e.

Laparoscopic resection of adrenal neuroblastoma without image-defined risk factors: a prospective study on 21 consecutive pediatric patients.

Background: Over the last 20 years MIS has progressively gained popularity in children with cancer. We therefore aimed at evaluating the safety of Minimally Invasive Surgery (MIS) resection in a series of children affected by adrenal neuroblastoma (NB) presenting without Image-Defined Risk Factors (IDRFs).

Methods: An Institutional protocol for MIS resection of adrenal NB in pediatric patients without IDRFs has been applied since 2008.

Preoperative evaluation in infants and children: recommendations of the Italian Society of Pediatric and Neonatal Anesthesia and Intensive Care (SARNePI).

Background: The preoperative assessment involves the process of evaluating the patient's clinical condition, which is intended to define the physical status classification, eligibility for anesthesia and the risks associated with it, thus providing elements to select the most appropriate and individualized anesthetic plan. The aim of this recommendation was provide a framework reference for the preoperative evaluation assessment of pediatric patients undergoing elective surgery or diagnostic/therapeutic procedures.

Methods: We obtained evidence concerning pediatric preoperative evaluation from a systematic search of the electronic databases MEDLINE and Embase between January 1998 and February 2012.

Magnetic resonance urography and laparoscopy in paediatric urology: a case series.

Paediatric urology often presents challenging scenarios. Magnetic resonance urography (MRU) and laparoscopy are increasingly used. We retrospectively studied children affected by a disease of the upper urinary tract who after MRU were elected for laparoscopic treatment.

Abdominal wall defects: a 33-year unicentric experience.

Aim: The aim of this paper was to provide the main clinical features, surgical details, and long term outcome of patients with gastroschisis and omphalocele operated on at Giannina Gaslini Institute between 1976 and 2009.

Methods: All patients who were operated on between 1976 and 2009 for omphalocele or gastroschisis were included. Detailed informations regarding demographics, maternal history, type of delivery, associated anomalies, surgical details, complications, morbidity and mortality were collected.

Sirolimus as maintenance treatment in an infant with life-threatening multiresistant pure red cell anemia/autoimmune hemolytic anemia.

A 9-month-old boy with life-threatening multiresistant pure red cell anemia/autoimmune hemolytic anemia within the frame of a possible, undiagnosed immune-mediated disease was initially treated with prednisone. Further-line therapies of the following 7 relapses included immunoglobulins, rituximab, cyclophosphamide, and alentuzumab followed by other maintenance treatments as cyclosporine, methotrexate, and mycophenolate. After all the administered therapies failed, the patient was successfully treated by splenectomy followed by fludarabine and then sirolimus as maintenance treatment.

Early diverting colostomy for perianal sepsis in children with acute leukemia.

Perineal sepsis is a life-threatening complication of acute leukemia. Although conservative management (antibiotics, incision, and drainage, alone or in combination) is considered the criterion standard, it provides an outcome that is not fully satisfactory, with an overall mortality of roughly 30%. This report presents a case series of 4 children who underwent early defunctioning colostomy for the treatment of perineal sepsis during leukemia.