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Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) represents the most severe entity among the foregut malformations, with patients at risk of chronic morbidity. This study aims to investigate on health-related quality of life (QoL) and long-term results in patients with EA.50 patients (30M, 20F, mean age: 14-year-old), among 82 patients operated from January 1995 to December 2005, answered the questionnaire. Two groups where compared: 8-12 years (A), 12-18 years (B). The survey investigated on 5 itmes: dysphagia; GERD; pulmonary affections (asthma, bronchitis, episodes of chronic cough during a year); growth and nutritional status (BMI); QoL scores. A control group of 50 healthy children was used.42 type III AE (3 long-gap and 1 VACTER association). Median BMI was 18,72 (SD 3,38) (range: 30,52-12,80). In 22 pts BMI18,5. 19 pts were positive for dysphagia (EAT-10 score= 3). A correlation was found between dysphagia and low BMI (Pearson 0,37). Dysphagia and low BMI were more diffuse in the younger population, while decreased in group B. 15 pts treated in their life for GERD (8 with medical therapy; 7 fundoplication). GerdQ test data showed only 4 pts with score6. GERD and dysphagia: 11 pts with GERD presented EAT-10 test3 (Pearson value confirmed this correlation: 0,59). Respiratory pathologies: 26 pts with positive anamnesis for chronic pulmonary affections (recurrent bronchitis in 21 pts; chronic cough in 15; the association of both in 12; 11 pts with asthma). PedsQL 4.0 median value was 15,4 (SD 10,1), control group value was 15,6 (SD 7,6); difference between the 2 groups was not statistically significant (p-value: 0,11). Correlation test for comparing high value of PedsQL and long-term complication in AE: the strongest association was with the dysphagia (Pearson: 0,55).Dysphagia resulted the most disabling symptom in group A but swallowing function slightly improved with the growth. Generally, AE seems not heavily influence patients' QoL.

Background: Gastrostomy tube placement (G-Tube) is a frequently offered procedure in children with feeding difficulties. Various procedures exist for G-Tube, with the pull technique more commonly u...

Patient: Female, 1-month-old Final Diagnosis: Intussusception Symptoms: Rectal bleeding Medication: — Clinical Procedure: Ultrasonography Specialty: Pediatrics and Neonatology Objective: Challenging differential diagnosis Background: Intussusception is the most common cause of intestinal obstruction in children, with a peak incidence usually before the second year of age, while in neonates it is a rare entity. We describe a delayed and incidental diagnosis of neonatal intussusception secondary to Meckel’s diverticulum in a neonate with shaken baby syndrome (SBS). This is, to the best of our knowledge, the first reported case of a neonatal intussusception with a Meckel’s diverticulum as a lead point in a neurologically impaired child. Case Report: A term baby presented at 22 days of age at our Emergency Department in severe conditions due to a suspected SBS. Eight days following hospitalization in the Intensive Care Unit, an isolated episode of rectal bleeding occurred, without any worsening of general conditions or abdominal distension. The ultrasonography showed a “doughnut sign” with high suspicion of ileocecal intussusception. A rectal barium contrast enema was performed but was not resolutive. At exploratory laparotomy an ileocecal intussusception with Meckel’s diverticulum acting as a lead point was identified and an intestinal resection was needed due to the ischemic condition of the ileum. The post-operative course was uneventful and the baby recovered well; the residual neurological impairment needed long-term follow-up. Conclusions: Intussusception is a rare entity in neonates and, when severe neurological impairment is present, the diagnosis can be missed because of the compromised condition of the baby and the paucity of gastrointestinal manifestations. In addition, due to the high incidence of lead point in neonatal cases, we recommend reserving non-operative treatment only for selected cases.

Background Ingestion of metallic foreign bodies (MFBs) is a frequent occurrence in children and is commonly diagnosed via X-rays. In recent years, the handheld metal detector (HMD) has been increasingly adopted by several paediatric hospitals as it is considered an effective and accurate diagnostic tool that avoids exposure to ionizing radiations. Sensitivity of HMD has been reported high (99.4%) in case of coin ingestion, but significantly lower (46%) when considering the ingestion of other types of MFBs. Methods We tested the effectiveness of the HMD in diagnosing ingested MFBs in children less than 14 years of age, in our Paediatric Emergency Department (PED). We prospectively evaluated all cases of MFBs ingestion that presented at the PED of our hospital from March 2015 to July 2017. Results 98 patients were included. The overall sensitivity was 63.2% (79.5% for coins, 25.5% for batteries and 56% for other objects) while the specificity was 95%. The HMD could have replaced the X-Ray examination only if a MFB was detected below the xyphoid process. Conclusions Based on our findings, a negative result of HMD is not sufficient to exclude an ingestion of MFBs. Therefore, in case of an evocative history and depending on type and size of the foreign body, a radiological investigation is still necessary.

A nine-year-old boy came to our clinic for the appearance of a voluminous swelling at the base of the neck in the jugular area after coughing. He underwent fibroscopy and a contrast-enhanced chest computed tomography (CT) scan, which did not indicate pathological findings even during the Valsalva maneuvre. After a color-Doppler ultrasound of the epiaortic vessels was obtained, a diagnosis of idiopathic phlebectasia of the internal jugular veins was made. The cause of the jugular phlebectasia remains unclear, and no treatment is indicated for this rare, benign, and self-limiting condition.

Acquired haemophilia A (AHA) is a rare autoimmune disease caused by IgG1-4 autoantibodies directed against coagulation factor VIII (FVIII), which causes the sudden onset of severe bleeding in patients with negative family and personal histories. Here, we describe the unusual case of a mother who developed AHA postpartum and had major bleeding. The patient showed isolated aPTT elongation (max. 75 s), a decreased activity of FVIII (min. 2,3%), and the presence of inhibitors [max 1.823 Bethesda Unit (BE)]. On the 14th day of life, her newborn showed several episodes of rectal bleeding without abdominal pain. It was thought that the cause of the bleeding was the same as his mother, so coagulation tests were done, and the results were normal. For feeding, a typical cow’s milk was not used because of protein intolerance. Instead, oral feeding with hydrolysed milk was started, which offered prompt resolution of symptoms.

A 2870 g male infant was born at 36+1 weeks’ gestation by cesarean section due to mild polyhydramnios and a non-reassuring cardiotocography. An ultrasound at 31 weeks demonstrated transient hyperechogenic fetal bowel (HFB). At birth, the Apgar scores were 9 and 10. The abdominal examination was unremarkable. He spontaneously passed meconium. After 20 hours, he developed left hemiabdominal distension with visible dilated bowel loop sign (figure 1) and bile-stained vomiting. Figure 1 ‘Bowel loop sign’ on abdominal wall due to a segmental intestinal dilatation. Abdominal radiography showed …

Severe combined immunodeficiency (SCID) is a life-threatening syndrome of recurrent infections and gastrointestinal alterations due to severe compromise of T cells and B cells. Clinically, most patients present symptoms before the age of 3 months and without intervention SCID usually results in severe infections and death by the age of 2 years. Its association with intestinal anomalies as multiple intestinal atresias (MIA) is rare and worsens the prognosis, resulting lethal. We describe the case of a four year-old boy with SCID-MIA. He presented at birth with meconium peritonitis, multiple ileal atresias and underwent several intestinal resections. A targeted Sanger sequencing revealed a homozygous 4-bp deletion (c.313ΔTATC; p.Y105fs) in tetratricopeptide repeat domain 7A (TTC7A). He experienced surgical procedures including resection and stricturoplasty. Despite parenteral nutrition-associated liver disease, the patient is surviving at the time of writing the report. Precocious immune system assessment, scrutiny of TTC7A mutations and prompt surgical procedures are crucial in the management.

Introduction Advantages in 3-dimensional (3D) laparoscopy are mostly described in adults for better depth perception, precise visualization of anatomical structures, as well as for complex surgical maneuvers in small spaces. Using Visionsense III stereoscopic endoscopy system (Neuromed Spa), we performed a comparative study between surgical skills achievements using 2-dimensional (2D) and 3D laparoscopic equipment in a pediatric laparoscopic surgery simulator model. Materials and Methods Three skills were evaluated both in 2D and 3D modalities. Pediatric residents ( n = 20) without any previous laparoscopic experience were randomly divided in 2 groups and evaluated doing the established tasks in a laparoscopic simulator validated for pediatric surgery. Switching the type of vision from 2D to 3D or vice versa, we evaluated bimanual dexterity, efficiency, and efficacy. Three tasks were proposed—task 1: transfer of objects (6 pegs transferred one-by-one on a pegboard); task 2: pattern cutting (cutting a paper, following a circular dotted line); and task 3: threading eyelet (transfer, twisting and passing through a eyelet-shaped support, a specific 3D object). Performance was measured using a scoring system rewarding precision and speed. Any physical discomfort related to the 3D vision was recorded. Results Of the 20 participants included, 10 began the skills in the 2D modality and then performed them in 3D, and the other 10 began in 3D and ended in 2D. Overall task 1 performance (time and number of errors) was significantly better using stereoscopic compared with monoscopic visualization. Both groups experienced a 35.6% decrease in the time needed to complete the peg transfer using 3D instead of 2D. In task 2, the 3D performance was superior (less time to correctly cut the paper along the dotted line), but did not reach statistical significance. In task 3, the residents experienced with 3D a 31.7% decrease in the time necessary to complete the passage of the object trough the eyelet. Most participants (65%) "subjectively" defined 3D laparoscopy easier overall; 6 participants (30%) did not experience any issue related to the use of 3D technology; and 1 person (5%) of group 1 found more difficulties using 3D compared with 2D. Headache (25%), nausea (20%), and visual disturbance (1%) were the most common issues reported by the students during 3D procedures. Finally, the results show that residents achieved significantly better results working with 3D vision rather than with 2D vision. Discussion As other studies have demonstrated, there was improvement in the overall performance using the 3D laparoscope. This was the first attempt to verify 3D skills in naive subjects, directly on a simulator conceived exclusively for pediatric surgery; therefore, bias was limited by using a population without surgical experience. Conclusions 3D laparoscopic surgical skills showed superior to 2D, with higher percentages of tasks completion, less time in performing them, and a shorter learning curve. Our results indicate that 3D was subjectively easier than 2D in performing complex tasks in the skills laboratory setting.

Introduction. Acute appendicitis is the most common surgical emergency in the pediatric population. The peak incidence occurs in the first decade of life, while it is uncommon to face appendicitis in children younger than 5 years of age. Laparoscopy is now demonstrated to be the optimal approach also to treat complicated appendicitis, but in very young children this standardized operation is not always easy to perform.Material and Methods. From January 2009 to December 2015 we operated on 525 acute appendicitis, with 120 patients less than 5 years of age.Results. 90 children had a complicated appendicitis (localized or diffuse peritonitis): 43 (48%) were operated on by open approach and 47 (52%) by laparoscopy. The overall incidence of postoperative complications was greater in the open appendectomy group (63% versus 26%) and all severe complications requiring reintervention (6% of cases: 3 postoperative abscesses resolved with ultrasound guided percutaneous abscess drainage; 1 tubal surgery for salpingitis; 1 adhesion-related ileus requiring relaparotomy) were mostly associated with open surgery.Conclusions. Laparoscopic surgery resulted as the best approach for treating complicated appendicitis also in younger children, with minor and less severe postoperative complications compared to open surgery.

Summary Objective Many authors consider surgical therapy of pediatric ranula and intraoral mucocele as the election treatment. Recently, an intracystic sclerosing injection with OK-432 has been proposed as a ranula primary treatment. This preliminary study evaluates the effectiveness of the use of Nickel Gluconate–Mercurius Heel–Potentised Swine Organ Preparations as the primary treatment of pediatric ranula and intraoral mucocele. Methods Eighteen children (9 ranulas, 9 labial mucoceles, 2 lingual mucoceles) were treated with oral administration of Nickel Gluconate–Mercurius Heel–Potentised Swine Organ Preparations D10/D30/D200. Results Eighty-nine percent ranulas (8 out of 9), 67% labial mucoceles (6 out of 9) completely responded to the therapy. One ranula, that interrupted therapy after only 4 weeks, was subjected to marsupialization in another hospital. A double mucocele case partially responded (one of the two was extinguished), another case incompletely responded, decreasing the size beyond 50%, and just one case, changing volume, resisted the therapy. Lingual mucocele healed at once. Blandin–Nuhn polypoid congenital mucocele responded to the treatment with gradual reabsorption, permitting surgical excision of the atrophic polypoid remnant, without removing glands of origin. No solved case showed recurrence (follow up range: 4–32 months). Conclusion Homotoxicological therapy with Nickel Gluconate–Mercurius Heel–Potentised Swine Organ Preparations D10/D30/D200 is an effective primary treatment of pediatric ranula and intraoral mucocele.

Objective: A Tracheomalacia complicates 11–33% of cases of Oesophageal Atresia with distal Tracheo-Oesophageal Fistula. The lesion generally involves only the thoracic segment of the trachea, and it has close anatomical relationships with the mediastinal structures, specially with the aortic arch. We therefore tried to define the most important morphotypes of tracheobronchial malacia by using dynamic fiberoptic bronchoscopy (DFB) and spiral multilayer computed tomography (CT). Methods: Between 1999 and 2003 we studied 40 children from two different institutions who had been operated on at birth for oesophageal atresia. All patients were been submitted to DFB, and the positive cases underwent examination by CT with an iodinated contrast medium. CT angiographic images of great vessels and multiplanar and threedimensional images of the airways (virtual broncoscopy and broncography) were obtained for morphological evaluation. Results: Twenty-five patients (62%) tested positive for malacia using DBF and all were also confirmed by CT study. In 11 cases (46%), the malacia was located at the thoracic section of the trachea, which was occluded by compression of the aorto-innominate complex. A simple intrinsic tracheomalacia without any vascular compression was present in eight cases (33%), while in five cases (21%), the malacia was complex. Conclusions: A correct morphological analysis of the malformed segment permitted ‘tailored surgery’ for each individual patient, allowing us to take account of the type of malacia, its length, and the compressive action exercised by the mediastinal great vessels. Q 2005 Elsevier B.V. All rights reserved.

An emphysema in a lower limb is usually a clinical sign of a severe and life-threatening infection. We report a rare case of subcutaneous emphysema of the left lower limb associated with a massive retro-pneumoperitoneum and pneumatosis intestinalis after cardiac transplantation in a 4-year-old girl. The child was nearly asymptomatic beside an abdominal distension. A benign pneumoperitoneum associated with an extensive pneumatosis intestinalis is a rare complication after organ transplantation and should be treated conservatively. The association with an emphysema in a lower limb in a child has not been previously reported to our knowledge in the literature.

Objectives We compare the outcome of medical treatment for ranula versus surgery in children. Study Design Multicentric cohort study. Methods The case series includes 37 children with ranulas (diameter 2.7 ± 1.5 cm). Eighteen patients, including 3 who had relapsed after either simple marsupialization or ranula removal alone, received oral nickel gluconate, mercurius heel, and glandula submandibularis suis D10/D30/D200. Fifteen cases underwent marsupialization with packing, and 2 underwent sublingual gland and ranula en-bloc excision. Two patients who recovered spontaneously shortly after diagnosis were excluded. Results No recurrences occurred among medical patients. Of the 17 surgical patients, 3 treated with marsupialization with packing relapsed. With the 3 surgical failures from other centers a total of 6 of 20 relapses were considered. Swelling or tension was common in surgical cases but unusual in medical patients. Conclusions In this case series oral medical treatment for ranula was very effective and more effective than marsupialization with packing.

Aims The aim of our study was to evaluate the effect of cardiac resyncronization therapy on QT dispersion (QTd), JT dispersion (JTd), and transmural dispersion of repolarization (TDR), markers of heterogeneity of ventricular repolarization in a study population with severe heart failure. Methods and results Fifty patients (43 male, 7 female, age 60.2+3.1 years) suffering from congestive heart failure (n ¼ 39 NYHA class III; n ¼ 11 NYHA class IV) as a result of coronary artery disease (n ¼ 19)or of dilated cardiomyopathy (n ¼ 31), with sinus rhythm (SR), QRS duration .120 ms (mean QRS duration ¼ 156+21 ms), an ejection fraction ,35%, left ventricular end-diastolic diameter .55 mm, presence of atrioventricular asynchrony, intra- and inter-ventricular asynchrony, underwent permanent biventricular pacemaker implantation. A 12-lead standard electrocardiogram was performed at baseline, during right-, left-, and biventricular pacing (BiVP) and QTd, JTd, and TDR were assessed. BiVP significantly reduced QTd (73.93+19.4 ms during BiVP vs. 91+6.7 ms in SR, P ¼ 0.004), JTd (73.18+17.16 ms during BiVP vs. 100.72+39.04 at baseline, P ¼ 0.003), TDR (93.16+15.60 vs.101.55+19.08 at baseline, P , 0.004), compared with SR. Right ventricular endocardial pacing and left ventricular epicardial pacing both increased QTd (RVendoP 94+51 ms, P , 0.03; LVepiP 116+71 ms, P , 0.02), and TDR (RVendoP 108.13+19.94 ms, P , 0.002; LVepiP 114.71+26.1, P , 0.05). There was no effect on JTd during right and left ventricular stimulation. Conclusions BiVP causes a statistically significant reduction of ventricular heterogeneity of repolarization and has an electrophysiological anti-arrhythmic influence on the arrhythmogenic substrate of dilated cardiomyopathy.

Objective: The aim of our study was to evaluate the effect of cardiac resyncronization therapy (CRT) on QT dispersion (QTd), JT dispersion (JTd) and transmural dispersion of repolarization (TDR), markers of heterogeneity of ventricular repolarization in a study population with severe heart failure. Methods and Results: Fifty patients (43 male, 7 female, aged 60.2 ± 3.1 years) suffering from congestive heart failure (N = 39 NYHA class III; N = 11 NYHA class IV) as a result of coronary artery disease (N = 19) or of dilated cardiomyopathy (N = 31), sinus rhythm, QRS duration >130 ms (mean QRS duration >156 ± 21 ms), an ejection fraction < 35%, left ventricular end-diastolic diameter >55 mm, underwent permanent biventricular DDDR pacemaker implantation. A 12-lead standard electrocardiogram was performed at baseline, during right-, left-, and biventricular pacing and QTd, JTd and TDR were assessed. Biventricular pacing significantly reduced QTd (73.93 ± 19.4 ms during BiVP vs 91 ± 6.7 ms at sinus rhythm, p = 0.004), JTd (73.18 ± 17.16 ms during BiVP vs 100.72 ± 39.04 at baseline p = 0.003), TDR (93.16 ± 15.60 vs 101.55 ± 19.08 at baseline; p

Starting from the observation that a number of consecutive patients with non-Hodgkin's lymphoma (NHL) resulted positive for hepatitis C virus (HCV) antibodies on routine testing, we set up a survey for HCV contact prevalence in all patients with lymphoproliferative disorders (LPD) followed in our institution. We searched for HCV antibodies by a third-generation ELISA technique, followed by a confirmation test (RIBA III); serum viral RNA and HCV genotype were investigated by a RT-PCR technique. We screened a total of 315 patients suffering from B-NHL (91), multiple myeloma (56), MGUS (48), chronic lymphocytic leukemia (57), Waldentrom's macroglobulinemia (13), Hodgkin's disease (HD)(43), and T-NHL (9). While only 1 of 52 patients with a non-B-LPD (HD or T-NHL) had signs of HCV contact (i.e., 1.9%, which is in the range of the normal population in the South of Italy), 59 of 263 patients with a B-LPD (22.4%) had HCV antibodies or RNA, or both, with no major differences among the various types of disorders, except for WM, in which the rate was higher (61.5%). The same prevalence was found for patients tested at diagnosis or during the follow-up, and in transfused or never-transfused patients. Only a few patients were aware of having a liver disease; one-half of HCV-positive patients never had transaminase increase. A review of data from Central and Northern Italy is included, showing similar findings; a report from Japan has confirmed such an association, while limited surveys in England have not revealed any correlation. These findings may have important biological and clinical implications.

We thank Dr Swenne and his colleagues for their interest in our manuscript.1 Transmural dispersion of repolarization (TDR) in the heart has been linked to a variety of arrhythmic manifestations.2 Three electrophysiologically distinct cell types have been identified in the ventricular myocardium: endocardial, epicardial, and M cells. Differences in the time course of repolarization of these three ventricular myocardial cell types contribute prominently to inscription of the electrocardiographic T-wave.3 In isolated ventricular wedge preparations, the …