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Background In Italy, as in many European countries, Pediatric Emergency Medicine is not formally recognized as a pediatric subspecialty, hindering nation-wide adoption of standards of care, especially in the field of procedural sedation and analgesia (PSA) in the Emergency Department (ED). For this reason PSA in Italy is mostly neglected or performed very heterogeneously and by different providers, with no reference standard. We aimed to describe the procedures and results of the first multidisciplinary and multi-professional Consensus Conference in Italy on safe and effective pediatric PSA in Italian EDs. Methods The preparation, organization and conduct of the Consensus Conference, held in Florence in 2017, followed the recommended National methodological standards. Professionals from different specialties across the country were invited to participate. Results Overall 86 recommendations covering 8 themes (pre-sedation evaluation, pharmacologic agents, monitoring, equipment and discharge checklists, training, non-pharmacologic techniques, the adult ED setting, impact on hospitalizations) were developed, taking into account the Italian training system and healthcare organization characteristics. Conclusion The results of the first multidisciplinary and multi-professional Consensus Conference in Italy are meant to provide up-to-date national guidance to improve the standard of care of children undergoing painful and stressful procedures in the ED. The recommendations will be periodically updated as new relevant evidence is published.

Il Neuroblastoma (NB) con sintomatologia ipertensiva riveste un’esigua percentuale dei tumori neuroblastici trattati ogni anno, attualmente in letteratura esistono unicamente due studi (1,2), che riportano un’incidenza di sintomatologia ipertensiva del 10-19% dei casi. Il follow-up dei pazienti studiati risulta breve, inoltre il meccanismo fisiopatologico non è stato tuttora dimostrato, ipotizzando una combinazione tra la compressione del peduncolo vascolare renale e la secrezione di catecolamine urinarie. Per quanto riguarda il management intra-operatorio, che può risultare difficoltoso a causa di picchi iper\ipotensivi durante la manipolazione della massa, esistono unicamente case reports (3,4). Lo scopo dello studio è valutare l’approccio diagnostico e la gestione perioperatoria del bambino affetto da NB con sintomatologia ipertensiva

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Lymphangiomas are rare benign congenital tumours, involving both the head and the neck and causing obstructing symptoms in the upper airways as well as aesthetic anomalies. In recent years, sclerosing therapy with OK-432 has become the treatment of choice in the management of these lymphatic malformations. Nonetheless, surgery still seems to be the therapy advocated for resolution of symptoms. Herein, three cases of lymphangiomas involving the head and the neck are described and a review of the English scientific literature is outlined.

Introduction. Stoma formation in neonates is often a life-saving procedure across a variety of conditions but is still associated with significant morbidity. Tube stoma technique was originally described for short bowel patients, but in selected cases of neonates this approach could prevent the incidence of stoma-related complications. The aim of the study was to evaluate the safety and utility of tube stomas as an alternative to conventional enterostomy in the neonatal population. Material and Methods. A retrospective multicentre analysis of neonates undergoing emergency laparotomy and tube stoma formation between 2005 and 2017 was performed. Tube stoma complications were analysed. The investigation focused on stricture, skin lesion, enteric fistula and prolapse. Results. Thirty-seven neonates underwent tube stoma fashioning during the study period. Tube-stoma complications were limited to three patients (8.1%), with two children (5.4%) requiring additional stoma surgery during the first 30 days because of an enterocutaneous fistula, and one child (2.7%) for bowel stenosis. Conclusions. In select neonates, such as those with proximal enteric stomas, the tube stoma avoids some of the commonly encountered complications (prolapse, skin excoriation). Further prospective studies are needed to validate these findings in order for us to recommend this technique as superior.

Early bowel lengthening procedure (EBLP) has been defined as any bowel lengthening procedure performed before six months of age. The purpose of this paper is to compare our experience with literature on this subject to identify common indications. A bi-institutional retrospective analysis was performed. Diagnosis, type of surgery, age at procedure and outcomes were analysed. Eleven EBLP were performed in Manchester and Florence from 2006 to 2021. The median age at surgery was 126 days (102–180), pre-operative median short bowel (SB) length was 28 cm (17–49) with a post-operative median increase of 81%. Furthermore, a PubMed/Embase search was undertaken regarding bowel lengthening procedures performed in the last 40 years. Sixty-one EBLP were identified. The median age was 60 days (1–90). Serial transverse enteroplasty (STEP) was the most frequent procedure used, with a median increased bowel length of 57%. This study confirms that no clear consensus on indication or timing to perform early SB lengthening is reported. According to the gathered data, EBLP should be considered only in cases of actual necessity and performed in a qualified intestinal failure centre.

BACKGROUND: To prevent malnutrition and food aspiration in children with chronic neuromuscular problems, enteral nutrition provided by gastrostomy is recommended. Long-term follow-up data about surgical and medical complications of PEG are available, but few papers have addressed all of the issues in the same series. METHODS: This retrospective study enrolled patients under 18 years who had a gastrostomy tube placed at our institution between 2003 and 2017. The aim is to evaluate outcomes after gastrostomy placement, focusing both on surgical complications (early and late), and its effect on their nutritional status, on the prevention of pulmonary infections, and their parents&rsquo, opinion. RESULTS: Eighty-four gastrostomies were placed in total (35 F, 49 M). Seventy-seven patients had a severe neurocognitive impairment (GMFCS 5). The principal indication for gastrostomy was severe dysphagia (53.3%). No gastrostomy-related death was observed. Early surgical complications were observed in five of 84 (5,9%) patients, late complications were observed in 15 of 84 (17.8%) patients. Twenty-two patients were diagnosed with subsequent gastroesophageal reflux, five patients developed dumping syndrome (6%). Complete medical follow-up data were available for 45 patients. A progressive improvement of nutritional status was observed in 29 patients, and 11 maintained the same percentile, the occurrence of respiratory infections and need for hospitalization decreased. In 90% of cases, parents were fully satisfied with the g-tube. CONCLUSION: This study confirms the positive nutritional outcomes of gastrostomy-tube with an associated small risk of surgical complications and a reduction in the number of respiratory infections, with most parents scoring their experience as positive.

Hepatic pulmonary fusion is a rare malformation associated with right congenital diaphragmatic hernia (CDH), often only discovered during surgical repair of the defect. Fourteen previous cases have been reported in the literature. We describe a case of a full term male newborn with prenatal ultrasound diagnosis of right CDH who underwent a thoracoscopy converted to a thoracotomy, due to this rare aforementioned intraoperative incidental finding. We reviewed the previous reported literature, especially focusing on the chosen surgical approach, concluding that an early and appropriate preoperative imaging investigation may be crucial for the best management of these kinds of patients.

Purpose Congenital cystic malformations of the lung are more frequently diagnosed before birth, but guidelines for surgical management of asymptomatic cases are lacking. The aim of this article is to review our 10-year results with antenatally diagnosed congenital cystic adenomatoid malformations (CCAMs) to debate indications for early postnatal surgical management in asymptomatic patients. Method Twenty-four cases were reviewed; of these, 18 were operated on before 15 days of life for respiratory distress or mediastinal shift, whereas 6 were submitted to elective surgery at 3 months of age. Results Twenty lobectomies and 4 atypical resections were performed. Two of the latter required a second surgery for incomplete primary perinatal resection. No postsurgical complications were reported. Nineteen (19/24) of the resected specimens showed signs of chronic inflammation. In the perinatal period, 100% (8 cases) of CCAM type II and 50% (8 cases) of CCAM type I resulted to be inflamed. Of the asymptomatic cases, 50% (3/6) were also found to be affected. No infections were detected at bacteriologic culture and bacterial debris was stained in 3 specimens. Conclusion In this series, a 79% incidence of pulmonary inflammation was detected. The CCAM type II resulted to be always involved in this process of inflammation. This was an unexpected finding, particularly in cases without mediastinal shift or respiratory distress. In light of these results, early postnatal treatment, at around 3 to 6 months of age, could be considered even in asymptomatic patients.

Tuberculosis (TB) has been described in association with different malignancies including Hodgkin's disease. However, the association with primary pulmonary Hodgkin's disease (PPHD) is hardly reported in literature and in teenage is quite exceptional. We report a case of an 11 years old boy in whom the diagnosis of tuberculosis preceded and delayed the diagnosis of PPHL.

Background Our study aims at disclosing epidemiology and most relevant clinical features of esophageal atresia (EA) pointing to a model of multicentre collaboration. Methods A detailed questionnaire was sent to all Italian Units of pediatric surgery in order to collect data of patients born with EA between January and December 2012. The results were crosschecked by matching date and place of birth of the patients with those of diagnosis-related group provided by the Italian Ministry of Health (MOH). Results A total of 146 questionnaires were returned plus a further 32 patients reported in the MOH database. Basing on a total of 178 patients with EA born in Italy in 2012, the incidence of EA was calculated in 3.33 per 10,000 live births. Antenatal diagnosis was suspected in 29.5% patients. 55.5% showed associated anomalies. The most common type of EA was Gross type C (89%). Postoperative complications occurred in 37% of type C EA and 100% of type A EA. A 9.5% mortality rate was reported. Conclusions This is the first Italian cross-sectional nationwide survey on EA. We can now develop shared guidelines and provide more reliable prognostic expectations for our patients.

Purpose The study's aim was to evaluate how information related to a prenatal diagnosis of fetal malformation could modify parenthood experience descriptions during pregnancy and after the child's birth. Methods A longitudinal case–control clinical study was conducted. Data on parenthood experience descriptions collected using a validated semantic differential technique during pregnancy and after the child's birth were compared between seven couples of parents receiving a prenatal diagnosis of fetal malformation and seven couples without any fetal diagnosis. Results Our results show that during pregnancy parents in the clinical group describe themselves as more fragile, passive, and timid [p=0.007] than those in the control group. On the other hand, after the child's birth, there are no significant differences between groups. Conclusions Data are discussed with reference to better knowledge of the psychological dynamics involved in becoming a parent and to rational planning of support for parents receiving a diagnosis of fetal malformation.

Meckel’s diverticulum (MD) is the most frequent congenital abnormality of the small bowel and it is often difficult to diagnose. It is usually asymptomatic but approximately 4% are symptomatic with complications such as bleeding, intestinal obstruction, and inflammation. The authors report a case of a 7-year-old boy with a one-year history of recurrent periumbilical colicky pain with associated alimentary vomiting, symptoms erroneously related to a cyclic vomiting syndrome but not to MD. The clinical features and the differential diagnostic methods employed for diagnosis of MD are discussed.