Overcoming Underpowering in the Outcome Analysis of Repaired-Tetralogy of Fallot: A Multicenter Database from the CMR/CT Working Group of the Italian Pediatric Cardiology Society (SICPed).

Managing repaired tetralogy of Fallot (TOF) patients is still challenging despite the fact that published studies identified prognostic clinical or imaging data with rather good negative predictive accuracy but weak positive predictive accuracy. Heterogeneity of the initial anatomy, the surgical approach, and the complexity of the mechanism leading to dilation and ventricular dysfunction explain the challenge of predicting the adverse event in this population. Therefore, risk stratification and management of this population remain poorly standardized.

Outcomes and costs analysis of Externalized PyeloUreteral versus internal Double-J ureteral stents after paediatric laparoscopic Anderson-Hynes pyeloplasty.

Background: The gold standard treatment for Uretero-Pelvic Junction Obstruction (UPJO) is laparoscopic dismembered pyeloplasty according to the Anderson-Hynes technique. The internal Double-J ureteral (DJ) and the Externalized PyeloUreteral (EPU) stents are usually the drainage of choice. Only a few articles have compared the clinical impact of the different drainage techniques on the perioperative morbidity and none presented a cost analysis of the incurred hospital stay.

Extended clinical and immunological phenotype and transplant outcome in CD27 and CD70 deficiency.

Biallelic mutations in the genes encoding CD27 or its ligand CD70 underlie inborn errors of immunity (IEIs) characterized predominantly by Epstein-Barr virus (EBV)-associated immune dysregulation, such as chronic viremia, severe infectious mononucleosis, hemophagocytic lymphohistiocytosis (HLH), lymphoproliferation, and malignancy. A comprehensive understanding of the natural history, immune characteristics, and transplant outcomes has remained elusive. Here, in a multi-institutional global collaboration, we collected the clinical information of 49 patients from 29 families (CD27, n = 33; CD70, n = 16), including 24 previously unreported individuals and identified a total of 16 distinct mutations in CD27, and 8 in CD70, respectively.

Colectomy and health-related Quality of Life in children with ulcerative colitis.

Background: Restorative proctocolectomy and ileal pouch-anal anastomosis (IPAA) is the recommended elective surgery for children with ulcerative colitis (UC). The aim of this study was to evaluate functional and long-term health-related Quality of Life (HRQoL) outcomes of surgery in pediatric patients with UC.

Methods: We reviewed the hospital records of all pediatric patients who had undergone surgery for UC between January 2009 and December 2016 in the Units of Pediatric Gastroenterology and Surgery, of both the University Hospital of Messina, and the Gaslini Children's Hospital of Genoa.

Ectopic Thymus: An Unusual Case of Subglottic Mass.

Objectives: The aim of our study is to report a case of a child with subglottic thymus that was suspected during diagnostic work-up for severe airway obstruction, excised surgically and confirmed with final histopathological examination. Moreover, we performed a narrative literature review to outline clinical and diagnostic features of this rare condition and to report suggestions for the management of subglottic masses.

Methods: We report the case of a 7-month-old boy who was admitted to our Pediatric Airway Team Unit due to a history of worsening biphasic stridor and recurrent episodes of upper airway obstruction.

"Atypical steroid response" in a pulmonary inflammatory myofibroblastic tumor.

A 6-year-old girl was evaluated for the presence of a paratracheal mass with right upper lobe atelectasis due to an endobronchial mass. Bronchoscopic biopsy established a diagnosis of inflammatory myofibroblastic tumor (IMT) and prednisone initially led to a significant reduction of the endobronchial lesion. However, 8 weeks later, when still on prednisone, the mediastinal mass enlarged dramatically.

Intermittent gaseous bowel distention: atypical sign of congenital tracheoesophageal fistula.

Three girls, 5-, 9-, and 15-year-old, were evaluated for recurrent airway infections and pneumonia. Chest X-rays, which included the upper portion of the abdomen, showed marked gaseous bowels distention, while computed tomography scans of the chest demonstrated the presence of tracheoesophageal fistula (TEF), confirmed by fiberoptic bronchoscopy. Abdominal gaseous distension, a known possible clinical manifestation of TEF in the neonatal period generated by airflow through the fistula into the oesophagus, has not been reported as a clue to the diagnosis in older children.

Recurrent severe lower respiratory tract infections in a child with abnormal tracheal morphology.

Localized recurrent respiratory infections, leading to severe hypoxia in young children without immunological abnormalities or other risk factors, should raise the suspicion of airway structural abnormalities. In a 24-month-old boy, with recurrent severe post-viral wheezing and a history of RSV-induced bronchiolitis and gastro-esophageal reflux, fiberoptic bronchoscopy demonstrated an abnormal morphology of the distal portion of the trachea, ending in four openings. Computed tomography (CT) scans demonstrated the presence of a right tracheal bronchus and an anomalous upper lobar bronchus, originating at the level of the major carina.

Outcome of primary endorectal pull-through for the treatment of classic Hirschsprung disease.

Introduction: Endorectal pull-through (ERPT) is a widely accepted procedure for the treatment of Hirschsprung's disease (HSCR). This study was aimed at presenting the long-term results of patients with classic HSCR who were operated on with a laparoscopic-assisted Georgeson procedure and to compare them to patients treated with a Soave-Boley procedure.

Patients And Methods: Patients treated for Hirschsprung disease in the period 1997-2006 with a minimum follow-up of 6 months were prospectively included in this study.

Multiple forearm diaphyseal fracture: reduction and plaster cast control at the end of growth.

The authors followed up 20 patients with multiple diaphyseal fractures of the radius and ulna who were treated nonoperatively and who healed with axial deviation >5 degrees in at least one plane 20.4+/-6.7 years after radiographic evidence of fracture union.

Effectiveness of esophageal manometry in predicting the outcome of children with primary GER after floppy Nissen-Rossetti wrap.

Background: Surgical treatment of gastroesophageal reflux (GER) can result in many postoperative problems because of an incorrect indication or an unsuitable fundoplication. Many preoperative tests have been suggested to perform a "tailored fundoplication," but there is no clear evidence as to which is the best. The aim of our study was to define the effectiveness of esophageal manometry in predicting the outcome of children who need fundoplication because of refractory primary gastroesophageal reflux.

Results of a mechanical Duhamel pull-through for the treatment of Hirschsprung's disease and intestinal neuronal dysplasia.

Background/purpose: The authors analyzed the results of a modified entirely mechanical Duhamel pull-through for the treatment of Hirschsprung' disease or type B intestinal neuronal dysplasia. The aim of the follow-up was to evaluate results of a Duhamel procedure entirely performed with the use of staplers.

Methods: Fifty-six patients were followed up to detect complications and outcome.

Necessity for surgery in children with gastrooesophageal reflux and supraoesophageal symptoms.

Background/purpose: The majority of gastrooesophageal reflux (GER) manifestations in children are supraoesophageal, and "spitting/posseting" is "the tip of the iceberg" because most reflux episodes are not regurgitated. Aim of the present study was to prospectively evaluate the incidence of gastrooesophageal reflux and the incidence of antireflux surgery in patients with difficult-to-treat respiratory symptoms.

Patients And Methods: Five hundred and ninety-five children with difficult-to-treat respiratory symptoms were prospectively enrolled in a blind study looking for the correlation between clinical presentation (asthma or non-asthma), oesophageal pH monitoring, X-ray barium meal, broncho-alveolar lavage, necessity for surgery, and outcome.

Outcome of laparoscopic Nissen-Rossetti fundoplication in children with gastroesophageal reflux disease and supraesophageal symptoms.

Background: The role of surgery is debated for children with gastroesophageal reflux disease (GERD), particularly when they show atypical symptoms. This study was designed to evaluate the safety and outcome of laparoscopic Nissen-Rossetti fundoplication performed in a selected population of children with gastroesophageal reflux and atypical supraesophageal symptoms.

Methods: This prospective study included 595 patients younger than 14 years with GERD who reported recurrent respiratory symptoms and had no benefit from standard medical treatment.

Pinning in slipped capital femoral epiphysis: long-term follow-up study.

This long-term follow-up study was aimed at evaluating patients with hip epiphysiolysis treated by pinning and previously controlled by short-term follow-up, in order to evaluate both clinical and radiographic evolution. The case series included 36 patients (17 female and 19 male) for overall 48 hips (21 right, 27 left). The epiphysiolysis was bilateral in 12 patients.

Complications of mechanical suturing in pediatric patients.

Background/purpose: Although it is widely accepted that staplers are effective in reducing operating time, potential complications related to their use in the pediatric population are not well defined yet. The aim of this study is to evaluate the safety of mechanical suturing.

Methods: Between 1996 and 2001, 174 procedures were performed using circular staplers in gastrointestinal anastomosis, whereas linear staplers were used in gastrointestinal and lung resection.

Laparoscopic vs open approach for the treatment of gastroesophageal reflux in children.

Background: The laparoscopic approach has become increasingly popular for fundoplication over the last few years; however many surgeons are skeptical about its real advantages.

Methods: We conducted a prospective comparative study of children operated on for gastroesophageal reflux (GER). Exclusion criteria included age <1 YEAR AND >14 years, previous surgery on the esophagus or stomach, and neurologic impairment.

Medium-term results after cholecystectomy in patients younger than 10 years.

Background: Gallbladder stones are a well-known and widely studied problem in children. Hematological disorders are the most common diseases that can cause cholelithiasis. However, in the last few years, the proportion of children with idiopathic cholelithiasis has increased 50%.