Seminars in Pediatric Surgery最新文献

The lymphatic system is one of the most essential and complex systems in the human body. Disorders that affect the development or function of the lymphatic system can lead to multi-system complications and life-long morbidity. The past two decades have seen remarkable progress in our knowledge of the basic biology and function of the lymphatic system, the molecular regulators of lymphatic development, and description of disorders associated with disrupted lymphangiogensis. In this chapter we will touch on the clinical features of complex lymphatic anomalies, new molecular knowledge of the drivers of these disorders, and novel developmental therapeutics for lymphatic disease.

Congenital heart disease affects 1/100 live births and is one of the most common congenital abnormalities. The relationship between congenital heart disease and lymphatic abnormalities and/or dysfunction is well documented and can be grossly divided into syndromic and non-syndromic etiologies. In patients with genetic syndromes (as examples listed above), there are known primary abnormal lymphatic development leading to a large pleiotropic manifestation of lymphatic dysfunction. Non-syndromic patients, or those without clear genetic etiologies for their lymphatic dysfunction, are often thought to be secondary to physiologic abnormalities as sequelae of congenital heart disease and palliative surgeries. Patients with congenital heart disease and lymphatic dysfunction have a wide variety of clinical manifestations for which there were not many therapeutic interventions available. The development of new imaging techniques allows us to understand better the pathophysiology of these problems and to develop different percutaneous interventions aiming to restore normal lymphatic function.

Patients with central lymphatic conduction disorders commonly have recalcitrant pleural effusions and or ascites. These conditions cause a profound deterioration in the patient's quality of life. Support measures such as low-fat diet and diuretics alone hardly ever provide meaningful improvement. New understanding of the pathophysiology of these disorders has opened the door in recent years to the development of several surgical procedures that have remarkable success rates. However, these patients must be managed by expert multidisciplinary teams.

Lymphatic dysfunction in critical illness is complex. Primary complex lymphatic anomalies can lead to profound organ dysfunction, particularly respiratory failure and shock. Critical illness, the complications of critical illness, and the procedures and therapies used to treat critical illness, can lead to secondary lymphatic dysfunction. This is most often seen with congenital and acquired cardiovascular disease and respiratory disease. The critical care management of these patients requires an expert multidisciplinary team.

The development of new imaging techniques for the study of the central lymphatic system allows us to understand the anatomy and pathophysiology of all the disorders of the thoracic duct. With the help of catheters placed percutaneously in the thoracic duct, we can do now complex operations on the thoracic duct to restore its functionality. Advance imaging, expert percutaneous skills, and expert microsurgical skills are critical to the success of these interventions.

Perianal complications are common and morbid in children with Crohn’s disease. In this review, we describe the epidemiology, the presentation and diagnosis, evaluation and management. We focus on updates such as the increasing frequency of biologic medications and MRI for evaluation. We also highlight controversies on the timing and approaches to surgical techniques. Finally, perianal disease requires the coordination of multidisciplinary care with nursing, radiology, gastroenterology, and surgery to optimize outcomes – both medical and patient-centered.

Management of inflammatory bowel disease, both Crohn's disease (CD) and ulcerative colitis (UC), has seen a seismic shift over the past decade. Over the past five years, there has been the introduction of many new therapies with differing mechanisms of action and a goal of achieving mucosal healing, as well as clinical and biochemical remission (1,2). In addition, management is aimed at restoring normal growth and normalizing quality of life. The ultimate goal is to individualize medical management and determine the right drug for the right patient by identifying which inflammatory pathway is predominant and avoiding unwarranted lack of efficacy or side effects through biomarkers and risk prognostication. Patient's age, location of disease, behavior (inflammatory vs. penetrating/structuring), severity and growth delay all play into deciding on the best treatment approach. Ultimately, early intervention is key in preventing complications. The therapeutic approaches to management can be broken down to nutritional therapy, biologic agents, immunomodulators (including corticosteroids), aminosalicylates and antibiotics. There are numerous other therapies, such as small molecule agents recently approved in adults, which are garnering a great deal of interest.

Enhanced recovery protocols (ERP) have been widely adopted in adult populations, with over 30 years of experience demonstrating the effectiveness of these protocols in patients undergoing gastrointestinal (GI) surgery. In the last decade, ERPs have been applied to pediatric populations across multiple subspecialties. The objective of this manuscript is to explore the evolution of how ERPs have been implemented and adapted specifically for pediatric populations undergoing GI surgery, predominantly for inflammatory bowel disease. The reported findings reflect a thorough exploration of the literature, including initial surveys of practice/readiness assessments, consensus recommendations of expert panels, and data from a rapidly growing number of single center studies. These efforts have culminated in a national prospective, multicenter trial evaluating clinical and implementation outcomes for enhanced recovery in children undergoing GI surgery. In short, this historical and clinical review reflects on the evolution of ERPs in pediatric surgery and expounds upon the next steps needed to apply ERPs to future pediatric populations.