This study aimed to evaluate discrepancies in prognosis after the Kasai portoenterostomy (KPE) procedure between neonatal and non-neonatal periods among patients undergoing the KPE procedure less than 60 days after birth (≤ 60 days). All type III BA patients who performed KPE less than 60 days from June 2020 to May 2024 in the Department of Neonatal Surgery of Beijing Children’s Hospital were retrospectively reviewed. They were divided into two groups according to the age at Kasai: the early KPE group (EK group, or neonatal group ≤ 30 days after birth) and the late KPE group (LK group, or non-neonatal group 31–60 days after birth), to evaluate the effect of surgical age on the postoperative efficacy following Kasai. 61 BA patients were included in our study. The median age at KPE in the EK group was 14.0 [10.0, 20.0] days and 47.0 [36.8, 51.1] days in the LK group. Regarding postoperative complications, Patients in the EK group tended to have a higher incidence of postoperative intestinal obstruction (10.5

BACKGROUND:Bowel perforation is a severe complication in neonates with Hirschsprung disease (HD), particularly during the neonatal period. Identifying risk factors may aid in early detection and intervention. METHODS:We retrospectively analyzed the clinical data of 300 neonates diagnosed with HD at our hospital from 2007 to 2024. The patients were divided into bowel perforation and non-perforation groups based on whether they developed bowel perforation during the neonatal period. The clinical characteristics of both groups were compared. Univariate and multivariate logistic regression analyses were performed to identify the associated risk factors, including general information, clinical symptoms, examination results, and birth history. RESULTS:A total of 300 neonates with HD were included in this study, with a bowel perforation rate of 6.0% (18/300). The sites of perforation were the sigmoid colon (44.4%, 8/18), descending colon (27.8%, 5/18), ascending colon (11.1%, 2/18), terminal ileum (11.1%, 2/18), and transverse colon (5.6%, 1/18). The results of univariate and multivariate logistic regression analyses revealed that age at admission < 5 days, CRP > 20.5 mg/L, and radiographic findings of intestinal wall pneumatosis were independent risk factors for bowel perforation in neonates with HD, with OR (95% CI) of 4.709 (1.187, 18.684), 35.185 (9.394, 131.782), and 9.667 (2.264, 41.278), respectively. CONCLUSION:The incidence of bowel perforation in neonates with Hirschsprung disease is 6.0%. Bowel perforation in neonates with HD is associated with early age at admission, elevated CRP, and intestinal wall pneumatosis.

Neonatal neuroblastoma is defined as neuroblastoma diagnosed via prenatal screening or within the first 28 days of life, comprising approximately 5

Background:Neonatal intestinal malrotation complicated by midgut volvulus is a serious and common life-threatening complication. When the midgut volvulus is prolonged or severe, it can lead to secondary necrosis of the entire midgut, with high mortality rates. Therefore, improving understanding the clinical characteristics of this condition is necessary to facilitate early diagnosis and treatment. Herein, we report a case of congenital intestinal malrotation and necrosis of the midgut volvulus in a newborn. Case Description:The patient was admitted to the hospital with vomiting, abdominal distension, and bloody stools. An emergency laparotomy revealed a large-area necrosis of the small intestine and torsion of the mesenteric root. External intestinal placement was performed initially, followed by a secondary laparotomy 72 hours later, which showed complete necrosis of the jejunum but recovery of the ileum. Duodenal and ileal ultra-high position fistulas were created, which were managed by intestinal fluid return and milk micropump feeding. Following that, fistula formation was performed 84 days after the second operation, and full enteral feeding was quickly achieved. The growth and development of the child were good at later follow-ups. Conclusions:The condition of the intestine in severe cases of neonatal intestinal malrotation combined with necrosis of midgut volvulus is unpredictable. The comprehensive management of operation timing, operation method, and close cooperation between neonatal physicians, surgeons, and nursing teams can greatly improve the success rate of treatment.

Bowel perforation is a severe complication of Hirschsprung disease (HD) that necessitates prompt diagnosis and aggressive management to reduce mortality and morbidity. This study aimed to provide insights into the clinical management of neonatal HD cases complicated by bowel perforation. A retrospective review was conducted on neonates diagnosed with HD and bowel perforation at Beijing Children’s Hospital from January 2007 to January 2024. Inclusion criteria included neonates ≤ 28 days old with confirmed HD diagnosis based on postoperative histology. Data collected included perinatal history, patient characteristics, HD classification, clinical presentations, surgical interventions, and outcomes. Statistical analysis was performed using SPSS 26.0. Among 300 neonates diagnosed with HD, 18 (6.0

Background Bowel perforation is a severe complication of Hirschsprung's disease (HD) that necessitates prompt diagnosis and aggressive management to reduce mortality and morbidity. This study aimed to provide insights into the clinical management of neonatal HD cases complicated by bowel perforation. Methods A retrospective review was conducted on neonates diagnosed with HD and bowel perforation at Beijing Children’s Hospital from January 2007 to January 2024. Inclusion criteria included neonates ≤ 28 days old with confirmed HD diagnosis based on postoperative histology. Data collected included perinatal history, patient characteristics, HD classification, clinical presentations, surgical interventions, and outcomes. Statistical analysis was performed using SPSS 26.0. Results Among 300 neonates diagnosed with HD, 18 (6.0%) developed preoperative bowel perforation. Most perforations were in the proximal ganglionic bowel, with short-segment HD (7/18, 38.9%) and long-segment HD (9/18, 50.0%) being most common. All patients underwent surgery within 24 hours of perforation detection, with 94.4% (17/18) requiring stoma creation. Postoperative complications included stoma retraction, Hirschsprung disease-associated enterocolitis, and adhesive bowel obstruction, but no mortality was reported. Conclusion The incidence of preoperative bowel perforation in neonates with HD was 6.0%, primarily in cases of short-segment and long-segment HD. Perforations were mainly located in the proximal ganglionic segments. Prompt surgical intervention, particularly stoma creation, resulted in favorable outcomes for most patients.

An otherwise healthy 4-month-old girl presented to the community health service center because her abdomen was distended. Over the next 2 months, the girl's abdomen gradually became more distended. Her examination was notable for abdominal distention with a large, mobile, non-tender abdominal mass. Abdominal ultrasound images and subsequently obtained CT images showed a large, circumscribed cystic and solid mass. This led to the presumptive diagnosis of teratoma of the mesentery. The mass was completely resected during a laparotomy. The pathology, along with the surgical findings and imaging, led to the final diagnosis.

Background:We aimed to evaluate the function of the reconstructed anal canal in postoperative anorectal malformations (ARMs) patients through three dimension (3D) high-definition anorectal manometry. Methods:From January 2015 to December 2019, 3D manometry was performed as a postoperative functional assessment of patients with ARMs divided into age subgroups based on the time of manometry. Manometric parameters, such as the length of the anorectal high-pressure zone (HPZ-length), the mean resting and squeeze pressure of HPZ (HPZ-rest and HPZ-sqze), recto-anal inhibitory reflex (RAIR), and strength distribution of the anal canal, were collected and compared with age-matched controls. Their functional outcomes were analyzed with SPSS 23.0 software for statistical analysis. Results:171 manometric measurements were performed on 142 postoperative patients (3 months∼15 years). The HPZ-rest in all patients was significantly lower than in age-matched controls (p < 0.05). HPZ-sqze was notably decreased in patients older than 4 years, whereas other age groups were comparable to controls (p < 0.05). The proportions of asymmetric strength distribution and negative RAIR were higher in ARMs patients. The type of anorectal malformations and lower HPZ-rest were the impact factors affecting postoperative functional outcomes. Conclusions:The majority of the ARMs patients had acceptable functional outcomes. 3D manometry can objectively assess the reconstructed anal canal function. The patients with fecal incontinence had a high proportion of extremely low HPZ-rest and HPZ-sqze, negative RAIR, and asymmetric strength distribution. The manometric details will help the clinicians explore the causes of defecation complications and guide further management.

ImportanceNeonatal appendicitis (NA) is a rare and potentially fatal neonatal disease. However, misdiagnosis is common owing to atypical clinical manifestations and non-specific laboratory tests. ObjectiveThe aim of this study was to summarize the clinical characteristics, treatments, and prognoses of infants with NA. MethodsThis retrospective analysis included 69 patients diagnosed with NA admitted to Beijing Children's Hospital between 1980 and 2019. The patients were divided into surgical and non-surgical groups based on whether surgery was performed. Their clinical characteristics were analyzed using the chi-square test, t-test, or the Mann-Whitney U test. ResultsThe study included 47 males and 22 females with NA. The primary symptoms were abdominal distension (n = 36, 52.2%), fever (n = 19, 27.5%), refusal to feed or decreased feeding (n = 16, 23.2%), and vomiting (n = 15, 21.7%). Sixty-five patients underwent abdominal ultrasound examinations; 43 had definite appendiceal abnormalities, 10 had right lower abdominal adhesive masses, and 14 had neonatal enterocolitis manifestations. Twenty-nine and 40 patients were in the surgical and non-surgical groups, respectively. No statistically significant differences were observed between the groups regarding sex, age at onset, birth weight, admission weight, or hospitalization time. However, parenteral nutrition was prolonged in the surgical group (P = 0.001). Additionally, two patients (2.9%) died. InterpretationNA is a rare neonatal disease with atypical clinical manifestations. Abdominal ultrasonography may aid in the diagnosis. Similarly, appropriate treatment can improve the prognosis.

OBJECTIVES:This study aimed to explore the clinical characteristics of septic shock in pediatric patients caused by acute appendicitis.METHODS:This case series included patients with septic shock caused by acute appendicitis in Beijing Children's Hospital between January 2015 and December 2020.RESULTS:Six patients with septic shock caused by acute appendicitis were enrolled. One patient was an infant with extremely low weight; 2 patients were obese. The diagnosis was delayed in 4 patients (the time from onset to diagnosis was 5 days in 3 children and 4 days in 1 child). All patients had abnormally raised inflammatory markers (C-reactive protein 119.17 ± 48.36 mg/L, procalcitonin 129.95 ± 86.09 ng/mL). Severe abdominal infection was found in all patients. There was appendix perforation in 4 patients and diffused peritonitis in 3 patients. Two patients had metabolic diseases (Wilson disease and decreased biotinase activity, respectively). Five patients had an appendectomy and 1 patient received conservative treatment. Five patients were discharged in stable condition, while 1 patient died.CONCLUSIONS:Children with delayed diagnosis, abnormal body weight, significant elevation in inflammatory markers, and underlying metabolic disease may be at greater risk of complicated appendicitis and septic shock.EVIDENCEBASED MEDICINE:Level of Evidence: IV.

Background: Intraoperative cholangiography (IOC) has been the gold standard for diagnosing biliary atresia (BA). Our study attempted to diagnose BA using laparoscopic fluorescein cholangiography (LFC).Methods: We retrospectively included 18 patients with preoperative suspected BA as the case group and 4 without extrahepatic biliary obstruction requiring laparoscopic surgery as the control group. All patients received indocyanine green (ICG) intravenously at 0.05 mg/Kg. The first 6 patients in the case group underwent IOC and LFC simultaneously, and the control group completed LFC. The imaging characteristics of LFC were recorded and summarized by the conventional and fluorescence mode of the endoscopic fluorescence imaging system (DPM-ENDOCAM-03). On this basis, 12 patients in the case group were diagnosed as BA according to LFC without IOC, and all 18 patients completed open Kasai surgery to confirm the diagnosis.Results: Laparoscopic fluorescence mode in BA detected liver fluorescence but no visualization of the extrahepatic bile ducts. However, the extrahepatic bile ducts in the control group were visible. Based on the imaging char-acteristics summarized from the LFC of the first 6 cases with BA in the case group, the remaining 12 cases who only underwent LFC were also successfully diagnosed with BA. Furthermore, the formation of hepatic hilar fibrous mass was found in all the patients during the open Kasai procedure, which confirmed the BA diagnosis.Conclusions: LFC appears as a specific pattern in BA and may be used for intraoperative diagnosis of BA. It has the advantages of simplicity, short time-consuming, and no radiation damage.

Anorectal malformations are common congenital digestive malformations requiring surgical interventions for reconstructing anal canal during an early life period. Defecation dysfunctions, such as fecal incontinence, soiling or constipation, are the most common postoperative long- term complications affecting the quality- of- life of children. Therefore proper postoperative assessment of anorectal function is vital. Anorectal manometry is currently the most prevalent method for examining functional anorectal disorders of reconstructed anal canal in postoperative patients. Real- time three - dimensional information of anorectal canal is quantitative and comparable. It provides an important guide for prognosis and rehabilitation.

To evaluate the safety and efficacy of transanal endorectal pull-through (TEPT) and the long-term outcomes in newborns with Hirschsprung disease (HD). A total of 229 newborns with HD underwent one-stage TEPT between 2007 and 2020, and the diagnoses were confirmed by rectal biopsy. The perioperative clinical course for all patients was reviewed, and the postoperative short- and long-term outcomes were assessed. A total of 229 neonates (187 male and 42 female) had a median age at TEPT of 17 days (range 6–28 days). Sixty-eight patients (29.7%) underwent TEPT combined with an abdominal approach or laparoscopy. Early postoperative complications (using the Clavien–Dindo grading system) were documented in 36 patients (15.7%), and late postoperative complications were noted in 9 patients (3.9%). The follow-up period in the remaining 165 children ranged from 1.2 to 14.0 years (median 5.0 years). A total of 106 of the patients older than four years old took part in an interview about bowel function, and 85 patients (80.2%) had bowel function scores (BFS) ≥ 18. TEPT is effective and safe for HD in the neonatal period and presents with a low rate of complications and an acceptable outcome.

BACKGROUND:We aimed to evaluate anorectal function of normal infants and children through three dimension high-definition anorectal manometry (3D ARM) to fill the research blank in this area.METHODS:From November 2014 to January 2019, 104 normal infants and children among patients who underwent 3D ARM were divided into four groups according to age (≤1 month; >1 month to ≤1 year; >1 year to ≤4 years and >4 years) and reviewed. The following parameters were performed: the length of anorectal high-pressure zone (HPZ), the resting pressure of HPZ (HPZ-rest), the squeezing pressure of HPZ (HPZ-sqze), recto-anal inhibitory reflex (RAIR), and pressure distribution of the anal canal. Graphpad Prism 7.0 software was used for statistical analysis.RESULTS:HPZ, HPZ-rest, and HPZ-sqze increased with age. There were differences in HPZ-rest and HPZ-sqze between the groups (p < 0.05). Sex had a significant effect on HPZ, but not on HPZ-rest and HPZ-sqze. The HPZ of males was higher than that of females among those ≤1 month old (p < 0.05). RAIR (-) was found in 17 patients (16%) for whom Hirschsprung's disease were excluded by biopsy, and these patients had regular defecation during follow-up. Most of the patients (85%) older than 1 year had a symmetric anal pressure distribution during the contracting period (p < 0.05).CONCLUSIONS:Anal canal function parameters gradually increased with age in normal infants and children which demonstrated the important role of age in evaluation, and these parameters can provide a reference for postoperative evaluation of anal canal function.

Purpose This study was aiming to explore the risk factors contributing to enterostomy in neonates with Hirschsprung disease (HD) to provide a reference for clinicians to make treatment decisions. Methods Medical records of 284 patients diagnosed with HD during the neonatal period were retrospectively analyzed. The patients were divided into 2 groups based on operative intervention (one stage transanal pull-through, versus enterotomy and staged transanal pull-through). Univariate and multivariable logistic regression analysis was performed to identify risk factors contributing to enterostomy. Results The incidence of enterostomy was 12.0% (34/284) in neonates with HD. Univariate and multivariate logistic regression analysis showed that serum albumin < 25.4 g/L, radiographic results as subphrenic free air, and level of aganglionosis with long-segment or total colonic aganglionosis (TCA) were independent risk factors of enterostomy in neonates, with OR of 42.045 (6.131, 288.319), 285.558 (26.651, 3059.694) and 15.573 (4.319, 56.157), respectively. Conclusions The low serum albumin level, bowel perforation, and level of aganglionosis with long-segment or TCA could influence the occurrence of enterostomy in neonates with HD.

BACKGROUND:Indocyanine green (ICG) is known to facilitate real-time imaging of the bile outflow during the Kasai procedure. This study explored more possibilities for applying ICG quantification in biliary atresia (BA). METHODS:We enrolled nine BA patients in this study. All patients received intravenous ICG injections before surgery and underwent Kasai operation. With a fluorescence imaging system (Nanjing Nuoyuan, REAL-IGS FLI-108) to observe the ICG and quantify the ICG fluorescence intensity (ICG-FI) changes of the hepatic portal fibrous tissue and the liver during the operation. As a short-term prognosis assessment, we monitored the postoperative ICG metabolism time in stool and used the jaundice-free (TBIL < 2 mg/dl) at 1-3 months after the operation. RESULTS:The average age of the patients at the time of surgery was 73 days. There were no adverse reactions after ICG injection. All patients were observed ICG-FI increased after the dissection of hepatic portal fibrous tissue, and the ICG-FI of the liver decreased during the operation. They all received standardized treatment after surgery. Four patients completely metabolized ICG within about two weeks (no fluorescence detected in stool), and the other five were longer than two weeks. Five patients achieved a jaundice-free outcome in the short-term postoperatively, and the other four did not. CONCLUSIONS:It is feasible to quantify ICG-FI in real-time to evaluate the anatomical degree of hepatic portal fibrous tissue in BA. The variations of ICG-FI in the liver and postoperative ICG metabolism time may be related to prognosis.

Purpose Alimentary tract duplication involving the rectum (ATD-R) is rare. The purpose of the study was to describe the features of pediatric ATD-R patients and propose a surgical management strategy.Methods Nine consecutive children operated on for ATD-R at a tertiary center for pediatrics from January 2010 to June 2021 were retrospectively reviewed and followed up. Eighty-six children with the same diagnosis from the literature were reviewed to assist the investigation. Classifications of ATD-R consisted of cystic, tubular, and diverticular.Results Surgical treatment and histopathological examination identified six females and three males with ATD-R. Initial clinical symptoms included perianal lesions, abnormal discharge, and anorectal malformation (ARM). Apart from one tubular ATD-R patient with cloaca malformation, the other eight patients had normal-developed anorectum. Complete or partial lesion resection maintaining the integrity of the proper colorectum was a principle of surgery. Six patients were followed up for a median time of 71 (range 12-121) months with good prognoses. A surgical management strategy of ATD-R in children was proposed.Conclusions ATD-R commonly occurred concurrently with normal-developed anorectum, seldom combined with ARM. ATD-R should be considered as a differential diagnosis in anorectal symptoms. The timely and appropriate operation was curative.

Background To compare the clinical outcomes between thoracoscopic approach and thoracotomy surgery in patients with Gross type C Esophageal atresia (EA) and tracheoesophageal fistula (TEF). Methods Patients with Gross type C EA/TEF who underwent surgery from January 2007 to January 2020 at Beijing Children's Hospital were retrospectively analyzed. The patients were divided into two groups according to surgical approaches. The perioperative factors and postoperative complications were compared among the two groups. Results One hundred and ninety patients (132 boys and 58 girls) with a median birth weight of 2975 (2600, 3200) g were included. The primary operations were performed via thoracoscopic (n = 62) and thoracotomy (n = 128) approach. After comparison of clinical characteristics between the two groups, we found that there were statistically significant differences in associated anomalies, method of fistula closure, duration of mechanical ventilation after surgery, feeding option before discharge, management of pneumothorax, and prognosis (all P < 0.05). To a certain extent, thoracoscopic surgery reduced the incidence of anastomotic leakage and increased the incidence of anastomotic stricture in this study. However, there were no statistically significant differences between the two groups in terms of operative time, postoperative pneumothorax, anastomotic leakage, anastomotic stricture, and recurrent tracheoesophageal fistula (all P > 0.05). Conclusions Thoracoscopy surgery for Gross type C EA/TEF is a safe and effective, minimally invasive technique with comparable operative time and incidence of postoperative complications.

Purpose: Most pediatric surgeons give little attention to the diagnosis of Hirschsprung disease (HD) in preterm infants. We aimed to explore the safety and accuracy of suction rectal biopsy (SRB) for diagnosing HD in preterm infants. Methods: A retrospective review was conducted of 45 preterm patients who underwent SRB from 2015 to 2019 in our hospital. We collected the clinical characteristics and pathology results of the patients and information on follow-up. The sensitivity and specificity of SRB for HD diagnosis were calculated. Results: The median gestational age of the patients was 35 weeks (range: 28.9–36.9 weeks), the median gestational age at biopsy was 38.6 weeks (range: 33.4–60.0 weeks), and the median weight was 2,790 g (range: 1,580–4,100 g). Fifteen patients (33.3%) were positive for HD, which was confirmed after pull-through surgery. Ganglion cells were present in 30 patients. The diagnosis of HD was excluded in 29 patients after discharge follow-up. The sensitivity of SRB ranged from 93.7 to 100%, and the specificity was 100%. No complications occurred after SRB among the patients whose biopsy age was <37 weeks (10 patients) or biopsy weight was <2,000 g (five patients). Conclusion: SRB is accurate and safe for diagnosing HD in late preterm infants.

Background To analyze the possible causes, treatment and outcomes of postoperative pneumothorax in patients with Gross type C esophageal atresia/tracheoesophageal fistula (EA/TEF). Methods Medical records of patients with Gross type C EA/TEF who were diagnosed and treated in Beijing Children’s Hospital from January 2007 to January 2020 were retrospectively collected. They were divided into 2 groups according to whether postoperative pneumothorax occurred. Univariate and multivariate logistic regression analysis were performed to identify risk factors for pneumothorax. Results A total of 188 patients were included, including 85 (45 %) in the pneumothorax group and 103 (55 %) in the non-pneumothorax group. Multivariate logistic regression analysis showed that postoperative anastomotic leakage [ P < 0.001, OR 3.516 (1.859, 6.648)] and mechanical ventilation [ P = 0.012, OR 2.399 (1.210, 4.758)] were independent risk factors for pneumothorax after EA/TEF repair. Further analysis of main parameters of mechanical ventilation after surgery showed that none of them were clearly related to the occurrence of pneumothorax. Among the 85 patients with pneumothorax, 33 gave up after surgery and 52 received further treatment [conservative observation (n = 20), pleural puncture (n = 11), pleural closed drainage (n = 9), both pleural puncture and closed drainage (n = 12)]. All of the 52 patients were cured of pneumothorax at discharge. Conclusions Anastomotic leakage and postoperative mechanical ventilation were risk factors for pneumothorax after repair of Gross type C EA/TEF, but the main parameters of mechanical ventilation had no clear correlation with pneumothorax. After symptomatic treatment, the prognosis of pneumothorax was good.