World Journal of Pediatric Surgery最新文献

Hirschsprung disease (HSCR) is the most common congenital motility disorder of the intestine, characterized by the absence of ganglion cells in the myenteric and submucosal plexuses, leading to functional bowel obstruction. Short-segment Hirschsprung disease (SS-HSCR) accounts for the majority of cases, with surgical resection being the cornerstone of treatment. Despite advances in surgical techniques, considerable variability exists in practice regarding the timing of surgery, the choice of technique, and the length of aganglionic rectal cuff to resect. This review synthesizes the current evidence surrounding surgical management of SS-HSCR, discussing technique-specific outcomes and areas for future research, with a focus on optimizing patient care and functional outcomes.

Objective: We aimed to evaluate the characteristics, complications and outcomes of necrotizing pneumonia (NP) requiring surgical intervention.

Methods: We conducted a retrospective study of all children who underwent surgical therapy for NP from January 2010 to December 2023. Patients were analyzed based on two surgical approaches: anatomic resection (AR) or non-AR (NAR).

Results: A total of 66 patients (median age: 36 months) required a surgical intervention for NP. A total of 37 patients received AR, 29 received NAR. The AR procedures were segmentectomy (n=29), lobectomy (n=8), bilobectomy (n=1) whereas NAR included wedge resection (n=13) and necrosectomy (n=16). The most common reasons for surgery were failure to respond to treatment (43.9%) and sepsis/septic shock (42.4%). A significantly greater proportion of patients in the AR group underwent surgery due to sepsis (p=0.023). There was no difference in the proportion of patients experiencing complications between the AR group (40.5%) and the NAR group (27.5%) (p=0.266). The majority of complications in both groups (68.0%) were categorized as minor, with 59.0% of cases occurring in patients who underwent AR. Prolonged air leak was the most frequent complication in both groups. There was no difference in the postoperative hospital stay, or duration of mechanical ventilation between the groups. There were no deaths.

Conclusions: Surgical intervention for NP may result in complications in one-third of patients, mostly minor and unlikely to significantly impact outcomes. Surgery should be tailored to the extent of parenchymal involvement.

Background: In Uganda, only two public hospitals provide pediatric surgery services. With less than 10 pediatric surgeons serving approximately 20 million children in Uganda, most patients with anorectal malformations (ARMs) must make several trips to the hospital before undergoing surgery. As a result, households borrow money, sell assets, or solicit contributions from friends and relatives to meet healthcare expenses. We used a cross-sectional study to examine methods families use to raise funds for the treatment of ARMs at a single institution in Southwestern Uganda.

Methods: This cross-sectional study was conducted in the pediatric surgery unit at a Regional Referral Hospital/University Teaching Hospital in Southwestern Uganda from June 2021 to July 2023. Participants included caretakers of children presenting with ARMs for treatment at our referral hospital.

Results: A total of 157 participants were enrolled. Mothers were the main caregivers (77.9%) present at the hospital. Out of a median monthly household income of UGX200 000 (US$51.68), families spent a median of UGX50 000 (US$12.92) to travel to the hospital. To raise funds for healthcare expenses, 68% of households reported selling assets.

Conclusion: Families sell household assets to afford ARMs treatment in Southwestern Uganda. Financial protection by the government through a national child health insurance policy would shield families from substantial health-related expenditures and decrease this burden. In addition, targeted policy to strengthen pediatric surgical capacity through workforce expansion and skills training such as the Pediatric Emergency Surgery Course, may minimize costs, improve timeliness of care, and prevent case cancellations.

Background: This study aims to address the timing of repair for severe congenital diaphragmatic hernia (CDH) without the use of extracorporeal membrane oxygenation (ECMO) and to determine the feasibility of an earlier intervention to avoid deaths associated with non-repair in patients who are more challenging to stabilize without ECMO.

Methods: This single-center retrospective study was conducted on neonates with CDH from 2013 to 2023. Based on the timing of surgery, the patients were classified into three groups: <24 hours (group A), 24-48 hours (group B) and ≥48 hours (group C). The 90-day survival rates were analyzed using Kaplan-Meier curves and compared among groups via log-rank tests. The independent factors related to survival assessed using the multivariate Cox regression model.

Results: Of 132 CDH infants, the overall 90-day survival rate was 81.8% (108/132), with a median operative time of 26.00 (24.00, 38.50) hours. A significant difference was observed in the 90-day survival rate among the three groups: 60.5% (23/38) in group A vs. 91.3% (74/81) in group B vs. 84.6% (11/13) in group C (log-rank p<0.001). In mild and severe cases and those with an oxygen index ≥7.5, group A resulted in significantly reduced survival rates. Multivariate Cox regression analysis indicated that surgical timing <24 hours remained an independent mortality-related risk factor in infants with CDH.

Conclusions: Repair surgery should be performed at least 24 hours after birth. The optimal timing for CDH neonates in non-ECMO centers appears to be 24-48 hours after birth, which can prevent the loss of treatment opportunities for severe cases.

Background: In approaching surgical correction of Hirschsprung disease (HSCR), laparoscopic Swenson endorectal pull-through (Lap-S-ERPT) requires less transanal dissection and sphincter stretch. This may lead to more immediate postoperative obstructive symptoms. While antibiotics and rectal irrigations are mainstays of treatment, there is concern about rectal instrumentation in the setting of recent low anal anastomosis. The purpose of this study was to assess the incidence and safety of early rectal irrigations following Lap-S-ERPT.

Methods: This is a single-center, retrospective review of all pediatric patients who underwent Lap-S-ERPT for HSCR from January 2018 to October 2023. Irrigations were performed if patients had obstructive symptoms including emesis, obstipation, and dilated colonic loops on radiographs. The primary outcome was need for postoperative rectal irrigation. Secondary outcomes included time from surgery to irrigation, duration of irrigation, and incidence of anastomotic leak.

Results: A total of 37 patients (62% male) underwent a Lap-S-ERPT at a median age of 3 months (interquartile range (IQR): 0.5, 5.0). Rectosigmoid disease was the most common diagnosis (n=29, 78.4%). There were 11 patients underwent rectal irrigations with a median time to initiation of irrigation of 46 hours (IQR: 32.0, 114.0) postoperatively and a median duration of irrigations of 3 days (IQR: 2.0, 4.5). There was no difference in anastomotic leak rate between patients who received irrigations and those who did not (9.1% v.s. 7.7%, p=0.887).

Conclusion: Following Lap-S-ERPT, nearly 30% of patients underwent rectal irrigation for postoperative obstructive symptoms. Despite concerns about instrumentation with a recent low anastomosis, there was no increase in leak rate in patients who received rectal irrigations.

Hirschsprung disease (HSCR) is a congenital enteric neuropathy in which the enteric nervous system (ENS) fails to develop along variable lengths of the distal gastrointestinal (GI) tract. This aganglionosis results in a functional bowel obstruction and requires surgical resection of the aganglionic segment. Despite surgery, however, long-term bowel dysfunction affects many patients. Understanding the embryologic causes and pathophysiologic consequences of HSCR is critical to improving its diagnosis and treatment. During normal gut development, the ENS arises from neural crest cells (NCCs) that delaminate from the neural tube to populate the entire GI tract with enteric neurons and glia. This process requires NCCs to undergo proliferation, migration and differentiation to form the complex neuroglial network that regulates gut motility and other intestinal functions. This review discusses the cellular and molecular processes that control normal ENS formation and what goes awry to give rise to HSCR. The complex pathophysiologic consequences of aganglionosis are discussed, including recent observations that describe novel aspects of HSCR beyond the absence of ganglion cells. This review aims to expand the understanding of HSCR and to stimulate new ideas on how to improve current management of the disease.

Congenital diaphragmatic hernia (CDH) is a rare heterogenous disorder with varying degrees of severity. Infant survival rates in high-income countries are approaching 80% in isolated CDH; however, over 50% will have long-term morbidities. Advanced antenatal imaging, including ultrasound and magnetic resonance imaging, has made it possible to prognosticate severity of CDH and to stratify risk when counseling expectant parents. Risk stratification can also better prepare healthcare teams to enable optimal neonatal management, and provide options for fetal intervention or, where legally permitted, pregnancy termination. Factors that may affect the immediate and long-term prognosis for CDH include prenatal diagnosis, gestational age at detection and delivery, side of the defect, presence of additional structural or genetic abnormalities, defect size, estimation of fetal lung volume, the extent of visceral herniation, and the delivery center's experience in caring for neonates with CDH. Optimizing the outcome for families and infants begins with an early prenatal diagnosis followed by referral to a diverse and inclusive multidisciplinary center with CDH expertise. Prediction of disease severity is supported by accurate fetal imaging and comprehensive genetic testing, and allows the care team to provide realistic outcome expectations during the counseling of expectant parents of all racial and ethnic backgrounds.

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.