World Journal of Pediatric Surgery最新文献

Hirschsprung-associated enterocolitis (HAEC) is an important cause of morbidity and the leading cause of mortality in patients with Hirschsprung disease. The pathophysiology of disease includes dysmotility of the enteric nervous system, dysbiosis of the microbiota, failure of the intestinal barrier, and impaired immunity. Common manifestations include fever, abdominal distension, lethargy, vomiting, and diarrhea. Given the non-specific signs and symptoms of HAEC, high clinical suspicion is warranted, especially in patients with risk factors. Diagnosis and management of HAEC depend on the severity of disease presentation. Several preoperative and postoperative modalities have been explored to prevent HAEC. The current review elaborates on the risk factors, pathogenesis, diagnosis, treatment, and prevention of HAEC.

Objective: To develop a model using patient-specific computational fluid dynamics (CFD) to predict the required anastomotic size for total anomalous pulmonary venous connection (TAPVC) surgery and to forecast surgical outcomes.

Methods: Based on clinical data from patients, a CFD model was used to simulate the anastomosis between pulmonary venous confluence and the left atrium. Blood flow velocity, wall shear stress, power loss, and pressure were calculated using numerical algorithms within the model. Various sizes of anastomosis were applied during the simulation. The energy dissipation at the anastomosis was computed from the results and compared with real-world data.

Results: As the simulated anastomotic size increased, blood flow velocity, pulmonary venous pressure, and energy loss decreased. However, when the anastomotic size exceeded 18 mm, the efficiency of energy conversion no longer improved. The realistic and simulated velocities matched well for anastomosis sizes ranging from 15 to 20 mm.

Conclusion: The model can assist surgeons in preoperative planning for determining the anastomotic size in TAPVC surgical treatment.

Although patients with Hirschsprung disease are mostly affected by imperfect bowel function and fecal control in the long term, they are also predisposed to lower urinary tract symptoms, impaired sexual functions, infertility, psychosocial issues, and decreased quality of life. Rare, but notable comorbidities, which may manifest after childhood, include inflammatory bowel disease and familial medullary thyroid cancer. Despite frequent occurrence of fecal incontinence and constipation, the overall long-term outlook is quite optimistic as social continence with a good quality of life can be achieved by efficient bowel management also in those affected patients, whose self-coping strategies and medical treatment remain insufficient. Bowel dysfunction and other potential long-term complications are best managed by an interdisciplinary specialized approach not overlooking psychosocial issues, which also helps to correctly identify areas requiring continuing input by adult healthcare for young adult patients after transition. Additional research is needed to unravel the pathophysiological mechanisms of the long-term bowel dysfunction to identify novel therapeutic targets for development of more efficient innovative management strategies and thereby improvement of quality of life.

The treatment of Hirschsprung disease (HSCR) is surgical resection of aganglionic bowel and subsequent pull-through of ganglionated bowel. Despite many advances since the initial description of the disease and its surgical management more than half a century ago, there remain considerable controversies regarding the history of the surgical technique, the optimal timing of the primary and multistage pull-through, the best treatment for patients with a delayed diagnosis of HSCR, and the management of post pull-through complications such as soiling due to sphincter incompetence, the presence of a transition zone, and the prevention of enterocolitis. The following review will explore each of these controversies.

Pediatric anesthesia presents greater challenges than does adult anesthesia. This bibliometric analysis aimed to analyze the top 100 most cited articles to be better understand the hot spots and prospects in pediatric anesthesia. Articles and reviews related to pediatric anesthesia were retrieved from the Web of Science Core Collection from 1990 to 2023. A bibliometric analysis of the top 100 most cited articles was also performed using information such as topics, author names, countries, institutions, publication years, and journals. A total of 32 831 articles were identified, with a total of 32 230 citations for the top 100 articles. The peak period for pediatric anesthesia research was from 2005 to 2009. The USA has emerged as the most active country in pediatric anesthesia research. Major journals published included Anesthesia and Analgesia, Anesthesiology, and Pediatrics, underscoring their authority in the field. Clinical studies on the top 100 most cited articles have focused on different stages of the perioperative period, the use of different anesthetic agents, and adverse outcomes in pediatric patients. The current study conducted a bibliometric analysis of the top 100 most cited articles in the field of pediatric anesthesia. Such insights are valuable for identifying research hot spots, assessing academic impact and collaboration in pediatric anesthesia, and guiding future research directions.

Worldwide, 150 children are born each day with congenital diaphragmatic hernia (CDH), a diaphragmatic defect with concomitant abnormal lung development. Patients with CDH with large defects are particularly challenging to treat, have the highest mortality, and are at significant risk of long-term complications. Advances in prenatal and neonatal treatments have improved survival in high-risk patients with CDH, but surgical treatment of large defects lacks standardization. Open repair by an abdominal approach has long been considered the traditional procedure, but the type of defect repair (patch or muscle flap) and patch material (non-absorbable, synthetic or absorbable, biological) remain subjects of debate. Increased experience and improved techniques in minimally invasive surgery (MIS) have expanded selection criteria for thoracoscopic defect repair in cardiopulmonary stable patients with small defects. However, the application of MIS to repair large defects remains controversial due to increased recurrence rates and unknown long-term effects of perioperative hypercapnia and acidosis resulting from capnothorax and reduced ventilation. Current recommendations on the surgical management rely on cohort studies of varying patient numbers and data on the long-term outcomes are sparse. Here, we discuss surgical approaches for diaphragmatic defect repair highlighting advancements, and knowledge gaps in surgical techniques (open surgery and MIS), patch materials and muscle flaps for large defects, as well as procedural adjuncts and management of CDH variants.

Congenital diaphragmatic hernia (CDH) is a congenital malformation characterized by failure of diaphragm closure during embryonic development, leading to pulmonary hypoplasia and pulmonary hypertension, which contribute significantly to morbidity and mortality. The occurrence of CDH and pulmonary hypoplasia is theorized to result from both abnormalities in signaling pathways of smooth muscle cells in pleuroperitoneal folds and mechanical compression by abdominal organs within the chest cavity on the developing lungs. Although, the precise etiology of diaphragm maldevelopment in CDH is not fully understood, it is believed that interplay between genes and the environment contributes to its onset. Approximately 30% of patients with CDH possess chromosomal or single gene defects and these patients tend to have inferior outcomes compared with those without genetic associations. At present, approximately 150 gene variants have been linked to the occurrence of CDH. The variable expression of the CDH phenotype in the presence of a recognized genetic predisposition can be explained by an environmental effect on gene penetrance and expression. The retinoic acid pathway is thought to play an essential role in the interactions of genes and environment in CDH. However, apart from the gradually maturing retinol hypothesis, there is limited evidence implicating other environmental factors in CDH occurrence. This review aims to describe the pathogenesis of CDH by summarizing the genetic defects and potential environmental influences on CDH development.

Prioritizing lung-protective ventilation has produced a clear mortality benefit in neonates with congenital diaphragmatic hernia (CDH). While there is a paucity of CDH-specific evidence to support any particular approach to lung-protective ventilation, a growing body of data in adults is beginning to clarify the mechanisms behind ventilator-induced lung injury and inform safer management of mechanical ventilation in general. This review summarizes the adult data and attempts to relate the findings, conceptually, to the CDH population. Critical lessons from the adult studies are that much of the damage done during conventional mechanical ventilation affects normal lung tissue and that most of this damage occurs at the low-volume and high-volume extremes of the respiratory cycle. Consequently, it is important to prevent atelectasis by using sufficient positive end-expiratory pressure while also avoiding overdistention by scaling tidal volume to the amount of functional lung tissue rather than body weight. Paralysis early in acute respiratory distress syndrome improves outcomes, possibly because consistent respiratory mechanics facilitate avoidance of both atelectasis and overdistention-a mechanism that may also apply to the CDH population. Volume-targeted conventional modes may be advantageous in CDH, but determining optimal tidal volume is challenging. Both high-frequency oscillatory ventilation and high-frequency jet ventilation have been used successfully as 'rescue modes' to avoid extracorporeal membrane oxygenation, and a prospective trial comparing the two high-frequency modalities as the primary ventilation strategy for CDH is underway.