World Journal of Pediatric Surgery最新文献

Focal nodular hyperplasia (FNH)-like lesions of the liver are rare in the pediatric population and are seldom reported as congenital lesions. The differential diagnosis between these lesions and pure-fetal hepatoblastoma (HBL) is challenging. We present a case of a congenital FNH-like hepatic lesion, managed with a right hepatectomy due to suspected fetal HBL. Additionally, a review of all published cases of congenital FNH-like lesions of the liver was carried out.MRI.

Background: Anorectal malformation (ARM) have a high association with spinal cord anomaly (SCA) impacting bladder and bowel function. This study aims to report the diagnostic accuracy of ultrasound (US) and MRI to detect SCA in children with ARM.

Method: A systematic review was performed as per PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidance. Search terms used were broadened in three consecutive searches to find papers investigating outcomes from spinal imaging in ARM, with four author search validation. Study quality was assessed as per Quadas 2 score. Meta-analysis comparing US diagnosis to MRI findings was performed using random effects model, including only clinically relevant SCA and considering children who did not develop negative outcomes as a true negative.

Results: Eight studies were included, six reporting US outcomes, and two MRI. All studies but one were retrospective studies. Overall, the included studies were applicable, but all suffered risk of bias with incomplete and non-protocolized follow-up. Pooled analysis for MRI compared with surgery found a sensitivity of 97% and a specificity of 94%, with one false positive and one false negative in an 8-month-old baby. When clinically relevant diagnoses were extracted and meta-analysis performed, a sensitivity of 33% and a specificity of 87% were found for infant US diagnosis of SCA.

Conclusion: The normal US spine in early infancy does not have diagnostic weight. A positive early scan will allow you to stream into active surveillance. Mandated MRI of infants will depend on the local approach to prophylactic de-tethering surgery.

The enteric nervous system (ENS) consists of a network of neurons and glia that control numerous complex functions of the gastrointestinal tract. Hirschsprung disease (HSCR) is a congenital disorder characterized by the absence of ENS along variable lengths of distal intestine due to failure of neural crest-derived cells to colonize the distal intestine during embryonic development. A patient with HSCR usually presents with severe constipation in the neonatal period and is diagnosed by rectal suction biopsy, followed by pull-through procedure to surgically remove the affected segment and reconnect the proximal ganglionated intestine to the anus. Outcomes after pull-through surgery are suboptimal and many patients suffer from ongoing issues of dysmotility and bowel dysfunction, suggesting there is room for optimizing the management of this disease. This review focuses on discussing the recent advances to better understand HSCR and leverage them for more accurate and potentially less invasive diagnosis. We also discuss the potential future management of HSCR, particularly cell-based approaches for the treatment of HSCR.

Objective: To explore the value of preoperative Aspartate Aminotransferase-to-Platelet Ratio Index (APRI) and Fibrosis-4 score (FIB-4) in predicting short-term prognosis of children with biliary atresia (BA) undergoing Kasai portoenterostomy (KPE).

Methods: Clinical data from children who underwent KPE were analyzed. Patients were divided into two groups based on their 2-year native liver survival after KPE. General information and laboratory findings were collected before KPE. The difference in liver fibrosis between the two groups was analyzed. The predictive efficacy of each index for short-term prognosis of children with BA was evaluated using the receiver operating characteristic curve.

Results: The APRI and FIB-4 in the good prognosis group were lower than those in the poor prognosis group (p=0.008 and 0.023, respectively), and postoperative jaundice clearance rate was higher (p=0.002). In the poor prognosis group, gamma-glutamyl transpeptidase levels in the F3+F4 fibrosis subgroup were significantly higher than those in the F1 subgroup (p=0.038). The area under the curve (AUC) for preoperative APRI in predicting short-term prognosis was the highest at 0.667, with a cut-off value of 1.190. The AUC for preoperative FIB-4 was predicted to be 0.642. The combination of preoperative APRI and alanine aminotransferase showed a higher AUC for prognosis prediction compared with either marker alone.

Conclusions: Preoperative APRI and FIB-4 may havepredictive values for short-term prognosis. The predictive value of APRI and FIB-4 combined with liver function indicators for the short-term prognosis of children is superior to that of a single indicator, but the results are not satisfactory.

Introduction: While large and symptomatic urachal anomalies (UAs) often lead to surgical excision, urachal malignancy is rare, rendering prophylactic excision unwarranted. We hypothesize that in the pediatric population, the presentation of an infected UA is the predominant etiology leading to surgical intervention.

Methods: We retrospectively identified patients with UA from July 2012 to December 2021 evaluated in our urology outpatient. Inclusion criteria included patients ≤18 years old and confirmation of UA on ultrasound (US). Exclusion criteria were patients diagnosed with clinical mimickers on US, excision by general pediatric surgery service, or excision done concomitantly as part of another urological procedure.

Results: We identified a total of 78 patients with UA. Of those, 35 (44.9%) underwent excision. The observation cohort was younger (5 months vs. 73 months, p=0.002), more likely to be asymptomatic (65.1% vs. 85.7%, p=0.038), and more likely to have UA characterized as a 'remnant' on US (72.1% vs. 48.6%, p=0.034). Univariate analysis showed that infected or symptomatic UA, or those characterized as hyperemic, cystic, or as a diverticulum on US were more likely to be excised. Multivariable analysis shows that patients with a 'urachal cyst' classification (p=0.008) and infectious presentation (p=0.046) were more likely to undergo surgical intervention. Excision was accomplished laparoscopically (80.0%) or robotically (11.4%). No excised UA was suspicious for malignancy.

Conclusion: We present a large pediatric cohort with UA and found that infectious symptoms at presentation and those classified as 'urachal cyst' based on US were more likely to prompt surgical excision as compared with other factors.

Background: Open-heart surgeries carry significantly greater risks in neonates than in older children. The current study aimed to analyze the short-term outcomes and risk factors for mortality in neonates undergoing cardiac surgeries.

Methods: This retrospective analysis included neonates who underwent cardiac surgeries with cardiopulmonary bypass (CPB) at our center between 2019 and 2023. Perioperative and operative factors were collected, and in-hospital outcomes and risk factors for mortality were identified.

Results: We analyzed the medical records of 190 neonates, including 111 males and 79 females. The median age at the time of surgery was 15 days, with a median weight of 3.2 kg. The overall mortality rate was 11.6%. Major postoperative complications included infection (48.9%), low cardiac output (48.4%), hepatic insufficiency (24.9%), acute kidney injury (22.1%), unplanned reintervention (13.1%), intracranial hemorrhage (3.7%), and cerebral infarction (0.5%). Multivariable analysis identified prolonged CPB time (odds ratio (OR)=1.018, p=0.014), Risk Stratification for Congenital Heart Surgery (RASCH-2) categories of ≥4 (OR=11.927, p=0.026), extracorporeal membrane oxygenation (ECMO) duration (OR=1.606, p=0.036), and peritoneal hemodialysis (OR=9.252, p=0.014) as significant predictors for mortality.

Conclusions: Despite advancements in recent decades, continued vigilance is required to further reduce mortality rates among neonates undergoing cardiac surgeries.

Hirschsprung's disease (HSCR) is widely recognized in pediatric surgery. This condition has been elucidated, and therapeutic approaches have been developed. However, even when ganglion cells are present in the rectum, some patients still experience symptoms such as bowel obstruction, intestinal dilatation, and chronic constipation, which are similar to those observed in HSCR. A consensus regarding the terminology for these diseases is yet to be established. This group of diseases was defined as 'allied disorders of Hirschsprung's disease' (ADHD). They are classified into two categories based on pathology: (1) Abnormal ganglia, including immaturity of ganglia, hypoganglionosis, and intestinal neuronal dysplasia; and (2) Normal ganglia, including megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS), segmental dilatation, internal anal sphincter achalasia, and chronic idiopathic intestinal pseudo-obstruction (CIIP). Hypoganglionosis, MMIHS, and CIIP are particularly severe and not curable by surgery. Guidelines were compiled to facilitate an accurate clinical diagnosis and provide appropriate treatment strategies for each disease. A full-thickness rectal biopsy with H&E and acetylcholinesterase staining is often required for a differential diagnosis. Patients are now able to survive longer with enteral nutrition combined with long-term intravenous nutrition and decompression of the gastrointestinal tract. However, all treatment strategies are symptomatic. It is necessary to improve the results of small intestine transplantation and to develop new therapies using regenerative medicine.

Objective: This study aimed to evaluate the medium and long-term outcomes of tunneled labial mucosa tube graft urethroplasty in the treatment of primary severe hypospadias and failed hypospadias.

Methods: We retrospectively reviewed the medical records of the patients who underwent tunneled labial mucosa tube graft urethroplasty at a single center between 2007 and 2020. Data collected included patient demographic, operative and post-operative characteristics. The surgical procedure was performed in two stages. Binary logistic regression model was used to identify risk factors for postoperative complication.

Results: A total of 82 patients who were followed for at least 12 months were included in this study. Among these, 56 patients received primary repair, while 26 had previously undergone failed reconstruction. The mean age at surgery was 5.0 years. Postoperative urinary fistula occurred in 36 patients, with 24 requiring surgical repair and 12 resolving spontaneously. Urethral stricture developed in 26 patients, with 20 undergoing surgical intervention and 6 managed with urethral dilation. Preoperative surgical history was identified as an independent risk factor for postoperative complications (p=0.015).

Conclusions: Although the complication rate associated with tunneled labial mucosa tube graft urethroplasty is relatively high compared with other techniques, it remains a viable option for patients with insufficient replacement materials, particularly for primary severe hypospadias. For cases of failed hypospadias repair, this procedure should be considered selectively based on patient-specific factors.

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.