La Pediatria Medica e Chirurgica <p><strong>La Pediatria Medica e Chirurgica&nbsp;</strong>(Medical and Surgical Pediatrics) publishes original papers in the field of basic science, clinical and laboratory research pertinent to medical and surgical pediatrics.</p> <p>In addition to Original Articles, <strong>La Pediatria Medica e Chirurgica&nbsp;</strong>(Medical and Surgical Pediatrics) welcomes Editorials (on invitation), Brief Reports, Letters to the Editor and Book Reviews as well. All manuscripts are critically assessed by external and/or in-house experts in accordance with the principles of peer review. Manuscripts must be written in English or Italian.</p> PAGEPress Scientific Publications, Pavia, Italy en-US La Pediatria Medica e Chirurgica 0391-5387 <p><strong>PAGEPress</strong> has chosen to apply the&nbsp;<a href="" target="_blank" rel="noopener"><strong>Creative Commons Attribution NonCommercial 4.0 International License</strong></a>&nbsp;(CC BY-NC 4.0) to all manuscripts to be published.<br><br> An Open Access Publication is one that meets the following two conditions:</p> <ol> <li>the author(s) and copyright holder(s) grant(s) to all users a free, irrevocable, worldwide, perpetual right of access to, and a license to copy, use, distribute, transmit and display the work publicly and to make and distribute derivative works, in any digital medium for any responsible purpose, subject to proper attribution of authorship, as well as the right to make small numbers of printed copies for their personal use.</li> <li>a complete version of the work and all supplemental materials, including a copy of the permission as stated above, in a suitable standard electronic format is deposited immediately upon initial publication in at least one online repository that is supported by an academic institution, scholarly society, government agency, or other well-established organization that seeks to enable open access, unrestricted distribution, interoperability, and long-term archiving.</li> </ol> <p>Authors who publish with this journal agree to the following terms:</p> <ol> <li>Authors retain copyright and grant the journal right of first publication with the work simultaneously licensed under a Creative Commons Attribution License that allows others to share the work with an acknowledgement of the work's authorship and initial publication in this journal.</li> <li>Authors are able to enter into separate, additional contractual arrangements for the non-exclusive distribution of the journal's published version of the work (e.g., post it to an institutional repository or publish it in a book), with an acknowledgement of its initial publication in this journal.</li> <li>Authors are permitted and encouraged to post their work online (e.g., in institutional repositories or on their website) prior to and during the submission process, as it can lead to productive exchanges, as well as earlier and greater citation of published work.</li> </ol> Robotic removal of Müllerian duct remnants in pediatric patients: our experience and a review of the literature <p>Persistent Müllerian duct syndrome is a disorder of sexual development, which features a failure of involution of Müllerian structures. An enlarged prostatic utricle is a kind of Müllerian duct remnant (MDR) with a tubular shaped structure communicating with the prostatic urethra. Treatment is aimed at relieving symptoms when present, preserve fertility and prevent neoplastic degeneration. We describe 3 cases of successful robot assisted-removal of symptomatic MDRs. The first case came to our attention for pseudo-incontinence; the other two for recurrent urinary tract infections. The patients have not presented such symptoms anymore on follow-up. We then reviewed existent literature on authors who have recently investigated the main issues concerning MDRs and have attempted a roboticassisted approach on them. Robot-assisted laparoscopy can be considered a valid, safe and effective minimally-invasive technique for the primary treatment of prostatic utricle.</p> Mario Lima Michela Maffi Niel Di Salvo Giovanni Ruggeri Michele Libri Tommaso Gargano Hubert Lardy ##submission.copyrightStatement## 2018-05-30 2018-05-30 10.4081/pmc.2018.182 Kearns-Sayre syndrome is genetically and phenotypically heterogeneous <p>Not available.</p> Josef Finsterer Sinda Zarrouk-Mahjoub ##submission.copyrightStatement## 2018-05-29 2018-05-29 10.4081/pmc.2018.193 Torsion of huge epididymal cyst in a 16-year-old boy: case report and review of the literature <p>Epididymal cysts (ECs) are relatively common in adults, rare in children. Normally their treatment is conservative. They may be situated anywhere in the organ, frequently in the region of the head. Torsion of these cysts is extremely rare in both children and adults, causing acute scrotal swelling. The diagnosis is intraoperative. A 16-year-old boy was referred to our Divisional Clinic by the treating physician for scrotal swelling appeared 4 months earlier. Absence of a history of minor scrotal trauma. Ultrasonography showed a 40×50 mm fluid-filled right para-testicular mass. We performed surgery finding a large black cyst connected to the head of the epididymis with 720°-degrees rotation. Histology revealed an acquired EC. The particularity of our case is due to the absence of symptoms in association with a big EC twisted of 720° degrees. This is the only case reported in literature. All patients with EC torsion reported presented symptoms related to acute scrotum.</p> Cosimo Bleve Maria Luisa Conighi Valeria Bucci Lorenzo Costa Salvatore Fabio Chiarenza ##submission.copyrightStatement## 2018-05-29 2018-05-29 10.4081/pmc.2018.162 Robot-assisted thoracoscopic repair of a late-onset Bochdalek hernia: a case report Bochdalek hernia (BH) is a major malformation that consists of a postero-lateral diaphragmatic defect through which abdominal contents can migrate into the thorax. It is most commonly a neonatal pathology associated with significant morbidity and mortality. Rarely, this type of congenital diaphragmatic hernia (CDH) presents later in life, some even in adulthood. Indications for surgery are not clear even though the majority of authors, being aware of the possible severe complications, recommend surgical correction in all cases. Many surgical approaches have been tried for this pathology, both open (laparotomic or thoracotomic) and minimally invasive (laparoscopic or thoracoscopic). We report a case of a late-onset BH corrected with a robot-assisted thoracoscopic procedure. The use of robotic technology for CDH has never been described to date. Mario Lima Niel Di Salvo Sara Ugolini Michele Libri Giovanni Ruggeri ##submission.copyrightStatement## 2018-05-25 2018-05-25 10.4081/pmc.2018.173 Laparoscopic treatment of symptomatic simple renal cysts in children: single-center experience Simple renal cysts, although common in adults, are rare in children. They are usually discovered incidentally in the course of the study of other urinary tract symptoms, although they are not always asymptomatic. Renal cysts can be classified as being either simple or complex. The purpose of this review is to present our case series of simple symptomatic renal cysts treated with laparoscopy. Nineteen patients with symptomatic renal cysts (6 to 13.5 cm) were referred to our institution between January 2006 and January 2017. They comprised 12 (40.5%) females and seven (59.5%) males, aged 8 to 15, with a mean age of 12.2 years. Of these patients, nine had previously been treated unsuccessfully by ultrasound-guided aspiration/alcoholization with 95%-ethanol, between 9 and 13 months prior to the laparoscopy. Five patients had undergone one treatment and four had undergone two treatments. All of the patients were treated by laparoscopic threetrocar deroofing. The cysts were opened and the wall excised using scissors and a monopolar hook. In most cases, to better handle the edges of the cyst and obtain a better grip, a needle was used to aspirate a small amount of fluid (used for cytological examination). The wall of the cyst was excised, the cyst edges were sealed, and the perirenal fat was placed on the bottom of the cyst (wadding technique). The mean operating time was 95 minutes (range 50- 150). The postoperative course was uneventful for all of the patients. The hospital stay ranged from one to three days. All of the patients were asymptomatic following the treatment. At a mean follow-up time of 3.6 years, none of the patients had experienced a recurrence. Renal function, as assessed by a MAG3 renal scintigraphy scan, was well-preserved in all of the patients, and all of them undergo an annual ultrasound scan. Antonio Marte Lucia Pintozzi ##submission.copyrightStatement## 2018-05-24 2018-05-24 10.4081/pmc.2018.168 Functional gastrointestinal disorders in newborns: nutritional perspectives Functional gastrointestinal disorders (FGIDs) definition in children has changed over the years trying to facilitate clinicians, because the diagnostic process is complicated by the interpretation variability of symptoms described by children or by their caregivers for newborns and toddlers. This review refers to the Rome IV classification system, drafted in 2016. FGIDs pathophysiology is multifactorial and still poor understood, with limitations for the therapeutic process, which results often in unnecessary and alternative treatments trying to reduce the relevant caregiver distress, but with increased costs for families and for the National Health Service. This study reports the most recent evidence-based treatments for FGIDs in newborns: though in most cases the first action is an educational and behavioral intervention, reassuring caregivers about the transient and self-limiting natural history of FGIDs, there is now more evidence to recommend probiotics in some infant FGIDs. Roberto Bellù Manuela Condò ##submission.copyrightStatement## 2018-05-23 2018-05-23 10.4081/pmc.2018.198 Nutritional management of surgical newborns and infants in the acute phase and during follow up The neonatal period represents a crucial stage of life since it is characterized by the development of organ structure and function. Early life is particularly susceptible to environmental factors through a programming effect. As a result, inadequate nutrition in early life leads to long-term mental and physical developmental alterations and increased mortality and morbidity. In this review, we will summarize available evidence with a particular focus on metabolic responses to surgical trauma, nutritional strategies that can be implemented in surgical infants and specific barriers to nutritional support after surgery. Fabio Mosca ##submission.copyrightStatement## 2018-05-23 2018-05-23 10.4081/pmc.2018.197 Feeding dysfunctions and failure to thrive in neonates with congenital heart diseases Congenital heart disease (CHD) is the most common neonatal congenital malformation. The variety and severity of clinical presentation depend on the cardiac structures involved and their functional impact. The management of newborns with CHD requires a multidisciplinary approach, in which the nutritional aspect plays an important role. An adequate caloric intake during either preand post-surgical period, in fact, improves the outcome of these patients. In addition, the failure to thrive of these children in childhood has been related to long-term cognitive delay (attention deficit disorders, aggressive behaviour and poor social and emotional development). To date, there is a lack of standardized feeding protocols and caloric goals about how to feed neonates with CHD, and current practice varies widely between centres. The latest American Society for Parenteral and Enteral Nutrition guidelines reiterate the importance of proteins, and recommend early start of enteral nutrition, also in the most severe heart diseases, such as univentricular forms. Necrotizing enterocolitis (NEC), the most frequent and feared complication of early feeding of these newborns, often represents an obstacle in spreading this practice. Furthermore, as demonstrated in premature infants, breastfeeding seems to reduce the incidence of NEC. That is why breastfeeding must be encouraged, even if it can be difficult for these mothers due to delivery complications, associated with infant disease. In addition, eating difficulties may persist even after discharge, because these patients require nutritional support through nasogastric tubes or percutaneous endoscopic gastrostomies. Giovanna Mangili Elena Garzoli Youcef Sadou ##submission.copyrightStatement## 2018-05-23 2018-05-23 10.4081/pmc.2018.196 Introducing complementary foods in the first year of life Introduction of solid foods is a fundamental step in the development of an individual. There are many implications that weaning contains not only on a nutritional plan, but also on the contingent and long-term health of an individual. Over time this nutritional passage has evolved through the acquisition of new knowledge about maturation of anatomical and neurosensory structures involved in all the phases of such a complex process. The understanding of a maturing taste of infant and cultural changes is another key to understand the evolution of introduction of solid foods in infants. What is contained in this text encapsulates thus the evolutionary path of weaning in recent years, showing current trends in the light of cultural changes and new scientific acquisitions. Francesco Tandoi Laura Morlacchi Angela Bossi Massimo Agosti ##submission.copyrightStatement## 2017-12-22 2017-12-22 10.4081/pmc.2017.186 Functional nutrients in infants born by vaginal delivery or Cesarean section The development of a proper neonatal microbiota is of great importance, especially for the effects that dysbiosis has in acute and chronic diseases’ onset. The microbiota, particularly the intestinal one, plays a crucial role in maintaining the health of the host, preventing colonization by pathogenic bacteria and significantly influencing the development and maturation of a normal gastrointestinal mucosal immunity. Several factors may interfere with the physiological development of microbiota, such as diseases during pregnancy, type of delivery, maternal nutrition, type of neonatal feeding, use of antibiotics, exposition to hospital environment (<em>e.g.</em>, neonatal intensive care unit) and genetic factors. Thanks to a proper maternal and neonatal supplementation with specific functional nutrients, it is now possible to correct dysbiosis, thus reducing the risks for the newborn’s health. In this review of the literature, we give an overview of the studies highlighting the composition of the maternal, fetal and neonatal microbiota, the factors potentially responsible for dysbiosis and the use of functional nutrients to prevent diseases’ onset. Gianluca Lista Fabio Meneghin Ilia Bresesti Francesca Castoldi ##submission.copyrightStatement## 2017-12-22 2017-12-22 10.4081/pmc.2017.184