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    Frailty in Children From the Perioperative Management to the Multidisciplinary Approach

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    The Oxford Dictionary defines the noun “frailty” as “weakness or poor health” and the adjective “frail” as “physically weak and thin” or “easily damaged or broken.” Surgeons deal every day with frail individuals either because the surgical disease itself is endangering their health status and/or (more often) because, in addition to the current disease, they may have comorbidities. An aging patient with intestinal obstruction has a serious problem, but if this happens in association with diabetes, coronary insufficiency, and emphysema, the problem becomes very serious. Except in emergency situations, elective surgery is delayed until the comorbidities are more or less under control, and this is why pre-operative tests and anesthetic consultations are mandatory. Pediatric surgeons are not different from adult surgeons in this respect, and they have also to deal with similar situations although the age and the nature of comorbidities are usually different. In the core of the specialty the complexity of the situation may be extreme while performing surgery for newborns: A 900 g baby with respiratory distress syndrome, an open ductus, and stage 3 necrotizing enterocolitis is a very “frail” patient. The skin barrier is weak, immunity is developing, the g.i. tract is in the process of establishing a symbiotic flora, the lung is immature, and so are the clotting mechanisms and the brain. But prematurity is not the only dangerous comorbidity. A 3000 g term baby with left CDH, associated congenital heart disease, pulmonary insufficiency, and hypertension requiring ECMO may have more mature functions than a premature but shares the same extreme frailty. This could be extended to other surgical situations beyond the neonatal period. In addition, in a number of these clinical conditions there is no chance for “elective” surgery. If I look back at my career as a pediatric surgeon extended along more than 50 years, I realize that these issues have become more and more complicated with the passage of time. When I was a trainee, almost half the patients with esophageal atresia died and so did the majority of babies with congenital diaphragmatic hernia or Wilms’ tumor. Ventilation in newborns was at its beginnings, total parenteral nutrition was not yet available, and many of the supporting drugs currently used were not in the market. Survival of newborns below 1000 or even 1500 g was rare and our “frail” patients were the tip of the iceberg. Rapid progress made the iceberg emerge and it grew more and more until producing many of these cases that nowadays constitute a large results but, frankly, at the expense of a much more complex specialty. This is why this book on frailty is timely and appealing. Moreover, the editors, Mario Lima and Maria Cristina Mondardini, authors of a number of acknowledged books on various aspects of our specialty, planned a comprehensive coverage of the subject and recruited a set of first-class authors for different chapters. The book is structured in five parts: After an introduction, the frailty of the newborn is covered extensively. The next parts analyze the multiple comorbidities that may cause additional frailty, the various actions pointed to improving therapeutic actions in this context, and the need for social support, continuity of care, and transition to adult care of these individuals that may require follow-up and support for life. I predict that this volume will have great success and that many of us will be illuminated by this comprehensive view of the growing intricacies of our surgical activity

    criptorchidismo

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    diagnosi e trattamento del criptorchidism

    Neonatal Surgery

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    In 1989, the British National Confidential Enquiry into Perioperative Deaths (NCEPOD) ruled “that pediatricians and general surgeons must recognize that small babies differ from other patients not only in size and stated that they pose quite separate problems of pathology and management” [1]. As pediatric surgeons, we are convinced that children are not just small adults. This is all the more true for neonates. Neonates have some unique problems that require very special knowledge, special surgical managements, and facilities specifically designed for them. Pediatric surgeons must understand their special needs and that of their relatives. They must learn team working with other specialists. They have to create the conditions to follow their patients from birth into adulthood as the treatments do not end with the healing of the problem but once the child has become an adult. With the rapid advances in fetal diagnosis, babies are no longer referred at the time of birth, but when prenatal diagnosis is made even if termination of pregnancy is planned because of an expected poor prognosis. Direct contacts between the prenatal team, the neonatologists, and the pediatric surgeons are also highly recommended to ensure continuity in the messages delivered to the parents. We live now in the era of evidence-based medicine (EBM), and best evidences are generated from prospective trials. Unfortunately, when compared with adult general surgeons who may operate hundreds of similar cases, pediatric surgeons perform a great variety of different procedures but few of each. Consequently, the indications for surgery and the type of procedure performed in neonates are rarely supported by randomized controlled trials, the majority being supported by retrospective studies and surgeon’s preferences. Hall and Pierro have tried to summarize what was the EBM randomized controlled trial (RCT) (level I evidence) of some of the most common neonatal procedures (esophageal atresia, congenital diaphragmatic hernia (CDH), bowel atresia, anorectal malformations, anterior abdominal wall defects, congenital lung lesions, Hirschsprung’s disease, inguinal hernia, necrotizing enterocolitis, pyloric stenosis). Their review highlights the fact that a quality evidence base supporting many of these interventions is lacking. Only a few randomized controlled trials have been done in neonatal diseases such as congenital diaphragmatic hernia, necrotizing enterocolitis, pyloric stenosis, and inguinal hernia. All of these trials have been based on collaboration between pediatric surgical units convinced by the importance of networks to promote multicenter prospective studies [2]. In 1999, Hardin and Stylianos undertake to study the current state of the pediatric surgery literature and its value in determining best clinical practice. As of March 1, 1998, they found 9373 references provided through Medline. After review, only 34 studies (0.3%) were classified as prospective, randomized, controlled studies [3]. Twelve years later, Ostlie and St Peter have done a similar study in 2010, collecting all randomized controlled trials from January 1999 through December 2009 published in the English literature excluding transplant, oncology, and the other non-general subspecialties, to conclude that randomized controlled trials represent less than 0.05% of all publications involving pediatric surgery in the 26 journals with at least one trial (<1 trial for every 200 articles) [4]. It is concerning that they document a similar lack in the twenty-first century, despite the increased educational and public expectations placed on EBS. In a recent lecture, Juan Tovar advocated to which extent pediatric surgery needs to base its therapeutic attitudes and operations on a solid research background [5]. This is particularly difficult on the field of clinical research because of the low prevalence of many of the conditions involved and also because of the fact that patients are minors that are not entitled to give informed consent by themselves for randomized studies. As regards laboratory research, this specialty is scarcely interesting for basic scientists. This situation can only be improved by prospective randomized studies performed in network collaboration with other hospitals/countries and by basic research conducted by pediatric surgeons and/or in association with other scientists [5]. Among the three particularly relevant recommendations that NCEPOD made in the report on perioperative pediatric deaths [1], the first one was: “surgeons and anesthetists should not undertake occasional pediatric practice”. This was also a statement of the European Union of Medical Specialists (EUMS) in 1995: “Surgeons taking care of children should have adequate training in a pediatric surgical unit. They should also continue to have regular exposure to this type of patients.” Neonatal surgery should only be carried out by surgeons and anesthetists whose pediatric workload is of adequate volume to maintain a high level of surgical competence and to allow the training of the residents. Congenital birth defects complicate 3–6% of pregnancies leading to live birth. As for example of the structural birth defects associated with significant mortality/morbidity, CDH is among one of the most common anomalies, occurring in about one per 2000–3000 live births. Consequently, the opportunity of training—and to keep his expertise—on a 120 CDH is low. Added to these facts, the combination of a shortened training period and the “new deal” on junior doctors’ about the number of hours has serious implications for training. This means that neonatal malformations need to be concentrated in some centers to allow sufficient case load. There are arguments for and against such large regional specialist pediatric centers. The benefits of centralization include concentration of expertise, more appropriate consultants on call, development of support services, and training. The disadvantages include children and their families far from their homes and the loss of expertise at a local level. The benefits of centralization far outweigh the adverse effects of having to take children to a regional pediatric intensive care center [6]. Unfortunately, in many places, politicians favor the multiplication of small regional centers to satisfy their voters who are poorly informed of the cold hard facts. Nowadays, it is unacceptable to train on real patients. The new technologies, namely, minimal invasive surgery and simulators, have been of great help using simulation technology to reduce risks to both students and patients by allowing training, practice, and testing in a safe environment prior to real-world exposure. This is supported by interest in quality of care, restrictions on the use of animal models, limited number of cases, medicolegal pressures, and cost-effective performance. Many models are available. The usefulness of mechanical simulators with faithful models have been proven efficient: hypertrophic pyloric stenosis (Plymale, 2010), closure of patent peritoneo-vaginal tract (Breaud, 2014), pyeloplasty (Breaud, 2014), esophageal atresia (Maricic and Bailez, 2012; Barsness, 2014), and CDH (Barsness, 2013). They have shift to realistic interactive models. Computerized modern technology with electronically assisted devices and virtual reality environment has provided new tools to the mechanical simulators. We have now the tools to evaluate cognitive/clinical skills, technical skills, and social/interactive skills as we have seen how important this could be in neonatal surgery. Surgical simulators (mechanical, computerized, virtual) and models (animals and interactive) are the appropriate tools to learn, to train, to assess surgical skills, and to keep his expertise, in spite of the small number of cases. Becoming a pediatric surgeon requires completion of one of the longest training programs among the medical systems and probably the widest as they have to learn a great variety of procedures but few of each. While specialization among adult surgeons usually focuses on a particular organ or region of the body, pediatric surgery deals with a defined age group. Pediatric surgeons are trained to operate anywhere on the body, and thus they appear to be probably the last general surgeons. They must ask their authorities to provide them modern tools to avoid training on real babies. Undoubtedly, this is expensive, but as said by Bok Derek at Harvard Law School, “If you think education is expensive, try ignorance!” They have to learn teamwork and multicenter collaboration. This will be the challenge of the new generation of pediatric surgeons to promote collaboration between pediatric surgical units and to create networks as to publish multicenter prospective studies with adequate sample sizes. In spite of these daunting challenges, they remain some courageous volunteers as you probably are, you reader of this book. We need neonatal surgeons, motivated, well trained, wishing to transmit their skills and their knowledge to the future one

    Ovarian Cryopreservation

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    Ovarian tissue collection for cryopreservation is a technique for fertility preservation. Gonadal tissue is collected laparoscopically, then is processed and cryopreserved and it can be reimplanted to restore endocrine and reproductive function. Patients eligible for this procedure are all those females, especially prepubertal, who are exposed to a risk of premature ovarian failure. Premature ovarian failure is associated to exposure to chemiotherapics and radiation [1, 2] (Tab 1). Ovarian tissue cryopreservation was firstly described in 1996 by Hovatta et al. [3] Even if this technique is still to be considered experimental, it is currently the only option applicable in prepubertal girls. The feasible techniques for tissue collection are mainly 3: - Ovarian cortical tissue biopsy: it consists of obtaining several specimen of cortical tissue away from the hilum using a laparoscopic biopter. - Partial oophorectomy: it consists of partial excision of ovarian cortical tissue. Different authors report from 1/4 to 2/3 of ovarian tissue to be removed leaving the hilar part intact - Unilateral ovariectomy: it consists of the collection of the whole ovary including vascular pedicle that will be used during the reimplantation. The collected tissue is then harvested and prepared for cryopreservation. The most used method is slow freezing, which represents the standard of care, but also vitrification is an available method [4] Vitrification difers from slow freezing for concentration of cryoprotectant (higher in vitrification and rate of cooling (faster in vitrification) [5]. The tissue can be cryopreserved for about 7 years [5]. When the fertility has to be restored, after the end of therapies, the harvested tissue can be reimplanted in an orthotopic or heterotopic position. The rest of the native ovary or a peritoneal fold near the fimbriae are considered orthotopic positions as they allow spontaneous pregnancies. Forearm or abdominal wall are the most used heterotopic sites. The reactivation of the tissue appears 4 to 9 months after the reimplantation [5].Ovarian tissue collection for cryopreservation is a technique for fertility preservation. Gonadal tissue is collected laparoscopically and then is processed and cryopreserved, and it can be reimplanted to restore endocrine and reproductive function

    ano-rectal endosonography and manometry in paediatrics

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    The application of endoanal ultrasonography in children, as the use of highresolution anorectal manometry, is not recent at all. Nevertheless, differently from what happened in adults medicine, these two sophisticated investigations are prerogative of only few centers in Europe. Furthermore, there is an increased interest in both pediatricians and pediatric surgeons on these diagnostic technologies. This is the reasons why we felt the need to organize and lead, from Bologna, two internal workshops, respectively, in June and October 2020, with a surprising successful attendance. These two events involved some of the most important experts on congenital colorectal anomalies in Europe. The idea to write this monograph was the outcome of this successful experience; our wish was to create a starting point for future studies, research, and technical developments, with the aim of offering both young and experienced pediatric surgeons a comprehensive textbook and an updated review on this rapidly changing field. We hope that result will achieve such high goals. The text is divided into 16 chapters. The first chapters (Chaps. 1–3) were written in order to recall some basics of anorectal anatomy and physiology, necessary to understand and interpret these sophisticated endoanal investigations. The central chapters (Chaps. 4–13 and Chap. 16) explore the present main indications of endoanal ultrasonography and manometry. The last chapters (Chaps. 14, 15) instead are meant to show some innovative applications of these technologies, opening the reader’s mind on the future possibilities on this field. I wish to thank all those who have actively collaborated on the creation of this book, and all the authors, who for friendship and desire to pass on their knowledge, agreed to provide their contribution
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