Diagnose and treatment for Type D congenital esophageal atresia with tracheoesophageal fistula.

Importance: Type D esophageal atresia (EA) with tracheoesophageal fistula (TEF) is characterized by EA with both proximal and distal TEFs. It is a rare congenital anomaly with a very low incidence. Objective: To investigate diagnostic and treatment strategies for this rare condition. Methods: We retrospectively reviewed the clinicopathological features of patients with EA/TEF treated at our institution between January 2007 and September 2021. Results: Among 386 patients with EA/TEF, 14 (3.6%) had type D EA/TEF. Only two patients were diagnosed with proximal TEF preoperatively. Seven patients were diagnosed intraoperatively. Five patients were missed for diagnosis during the initial surgery but was later confirmed by bronchoscopy. During the neonatal period, seven patients underwent a one-stage repair of proximal and distal TEF via thoracoscopy or thoracotomy. Due to missed diagnosis and other reasons, the other 7 patients underwent two-stage surgery for repair of the proximal TEF, including cervical incision and thoracoscopy. Ten of the 14 patients experienced postoperative complications including anastomotic leakage, pneumothorax, esophageal stricture, and recurrence. Patients who underwent one-stage repair of distal and proximal TEF during the neonatal period showed a higher incidence of anastomotic leak (4/7). In contrast, only one of seven patients with two-stage repair of the proximal TEF developed an anastomotic leak. Interpretation: Type D EA/TEF is a rare condition, and proximal TEFs are easily missed. Bronchoscopy may aim to diagnose and determine the correct surgical approach. A cervical approach may be more suitable for repairing the proximal TEF.

Comparison of robotic versus thoracoscopic repair for congenital esophageal atresia: a propensity score matching analysis.

BACKGROUND: Despite the rapid advancement of robotic surgery across various surgical domains, only cases of robotic repair (RR) for neonates with esophageal atresia (EA) have been reported. Comprehensive studies comparing RR and thoracoscopic repair (TR) are lacking. The authors aimed to compare the safety and efficacy of RR and TR for EA. METHODS: A retrospective multicenter study was conducted on 155 EA neonates undergoing RR (79 patients) or TR (76 patients) between August 2020 and February 2023 using propensity score matching. Asymmetric port distribution and step-trocar insertion techniques were applied during RR. Demographics and surgical outcomes were compared. RESULTS: After matching, 63 patients (out of 79) in RR group and 63 patients (out of 76) in TR group were included. There were no significant differences in short-term outcomes between two groups, except for longer total operative time (173.81 vs. 160.54 min; P <0.001) and shorter anastomotic time (29.52 vs. 40.21 min; P <0.001) in RR group. Compared with TR group, the RR group had older age at surgery (8.00 vs. 3.00 days; P <0.001), but a comparable pneumonia rate. More importantly, the incidence of anastomotic leakage (4.76 vs. 19.05%, P =0.013), anastomotic stricture (15.87 vs. 31.74%, P =0.036) within 1 year postoperatively, and unplanned readmission (32.26 vs. 60.00%, P =0.030) within 2 years postoperatively were lower in RR group than in TR group. CONCLUSIONS: RR is a technically safe and effective option for EA patients. This approach delays the age of surgery without increasing respiratory complication rates while reducing the incidence of postoperative anastomotic complications and unplanned readmission.

Diagnosis and management of congenital type D esophageal atresia.

This study was performed to describe the current clinical practice and outcomes of type D esophageal atresia. We retrospectively analyzed 10 patients who were diagnosed with type D esophageal atresia and underwent esophageal atresia and tracheoesophageal fistula repair in the Capital Institute of Pediatrics and Beijing Children's Hospital from January 2017 to May 2022. Ten patients include three newborns and seven non-newborns. Seven (70%) cases were misdiagnosed as type C esophageal atresia before the first operation. Three neonatal children underwent thoracoscopic distal tracheoesophageal fistula ligation and esophageal anastomosis: the proximal tracheoesophageal fistula was simultaneously repaired with thoracoscopy in one of these children, and the proximal tracheoesophageal fistula was not detected under thoracoscopy in the other two children. Among the seven non-neonatal children, one underwent repair of the proximal tracheoesophageal fistula through the chest and the other six underwent repair through the neck. Nine patients were cured, and one died of complications of severe congenital heart disease. Type D esophageal atresia lacks specific clinical manifestations. Misdiagnosis as type C esophageal atresia is the main cause of an unplanned reoperation. Patients without severe malformations have a good prognosis.

Laparoscopic indocyanine green fluorescence imaging technique in rare type II cystic biliary atresia.

In recent years, it has been reported that indocyanine green can be used for intraoperative navigation in Kasai surgery. However, there are no reports of its application in surgery for rare type II cystic biliary atresia. We report a girl presented with jaundice and light-colored stools. Laboratory tests showed impaired liver function with elevated serum bilirubin and bile acid levels. The abdominal ultrasound and MRCP suggested a common hepatic duct cyst. A diagnosis of choledochal cyst was suspected and biliary atresia could not be excluded. Conservative treatment was unsatisfactory. Laparoscopic exploration with indocyanine green fluorescence was performed on the 38th day of her life, and intraoperative diagnosis of type II CBA was made because the common hepatic duct cyst and its downstream anatomical structures did not show fluorescence. The postoperative bilirubin and bile acid levels decreased significantly and she was discharged two weeks after surgery. This result suggests that indocyanine green can be safely used in laparoscopic surgery for type II CBA, which not only helps in the differential diagnosis of CBA and choledochal cyst, but also confirms bile flow in real time.

Long-term bowel function after single-stage transanal endorectal pull-through in neonatal patients with Hirschsprung disease.

BACKGROUND: The aim of this study was to assess long-term outcomes of neonatal patients with Hirschsprung disease (HD) after single-stage transanal endorectal pull-through (TEPT) and to explore the predictive factors contributing to subnormal bowel function. METHODS: Patients aged > 3 years operated for HD with TEPT during neonatal period between 2007 and 2019 answered the bowel function score (BFS) questionnaire. The patients were retrospectively divided into two groups according to whether they had normal bowel function. The clinical variables were compared between the subnormal and normal BFS groups. Univariate and multivariable logistic regression analysis were performed to identify the predictive factors contributing to subnormal bowel function. RESULTS: A total of 160 children (71.7%) were included in this study, with mean follow-up time of 7.3 years (range 3.0-15.1 years). The level of aganglionosis were determined to be the short-segment (124/160, 77.5%), long-segment (33/160, 20.6%), and TCA (3/160, 1.9%). One hundred and thirty-four patients (83.8%) had a BFS ≥ 17, and 26 patients (16.2%) with subnormal bowel function (BFS < 17). Univariate and multivariate logistic regression analysis showed that level of aganglionosis with long-segment or TCA and postoperative hospital stay > 8.5 days were independent risk factors with OR of 3.213 (1.252, 8.246) and 3.813 (1.371, 10.606) for subnormal BFS, respectively. CONCLUSION: Most HD patients who underwent one-stage TEPT in the neonatal period have favorable long-term results, and the level of aganglionosis with long-segment or TCA and long postoperative hospital stay may be closely related to subnormal bowel function.

Three dimension high definition manometry evaluated postoperative anal canal functions in children with congenital anorectal malformations.

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.

Clinical characteristics and prognosis of 69 cases of neonatal appendicitis.

Importance: Neonatal appendicitis (NA) is a rare and potentially fatal neonatal disease. However, misdiagnosis is common owing to atypical clinical manifestations and non-specific laboratory tests. Objective: The aim of this study was to summarize the clinical characteristics, treatments, and prognoses of infants with NA. Methods: This 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. Results: The 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. Interpretation: NA is a rare neonatal disease with atypical clinical manifestations. Abdominal ultrasonography may aid in the diagnosis. Similarly, appropriate treatment can improve the prognosis.

Development of a Modified Duodenum Traction Technique in Laparoscopic Duodenal Web Excision.

Introduction: Laparoscopic duodenal web resection surgery remains safe in neonates. The pearls in laparoscopic duodenal web excision are a proper and stable duodenal exposure. Herein, we present a modified duodenal traction technique, which can improve operative field exposure in laparoscopic surgery. Material and Methods: This modified technique was performed in 54 patients during laparoscopic duodenal web resection surgery at our center. It was performed using a 5-0 PDS-II suture, which was introduced percutaneously at 1-2 cm under either side of the costal margin at the anterior axillary line, respectively, to retract the duodenum. Results: Perioperative data of these patients and short-term follow-up data of duodenal web patients were retrospectively reviewed. All 54 procedures were completed without conversion to open surgery or requiring additional ports. Patients' mean age at surgery was 5 days (range 2-30 days), and the median weight at the time of surgery was 3.25 kg (range 2.52-3.88 kg). Eight patients (14.8%) had complete membranes, whereas 46 (85.2%) had a membrane with a hole. The mean time required for this technique was 336 (range 216-416) seconds and the mean duration of the entire surgery was 77 (range 65-89) minutes. The mean postoperative hospital stay was 16 (range 9-90) days and no postoperative complication related to the suspension procedure occurred. Conclusion: Our outcomes demonstrated the modified duodenal traction technique is a feasible and ideal method during laparoscopic duodenal web resection surgery.

A 6-month-old girl with mesenteric teratoma: A Case report and literature review.

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.

Necroptosis of macrophage is a key pathological feature in biliary atresia via GDCA/S1PR2/ZBP1/p-MLKL axis.

Biliary atresia (BA) is a severe inflammatory and fibrosing neonatal cholangiopathy disease characterized by progressive obstruction of extrahepatic bile ducts, resulting in cholestasis and progressive hepatic failure. Cholestasis may play an important role in the inflammatory and fibrotic pathological processes, but its specific mechanism is still unclear. Necroptosis mediated by Z-DNA-binding protein 1 (ZBP1)/phosphorylated-mixed lineage kinase domain-like pseudokinase (p-MLKL) is a prominent pathogenic factor in inflammatory and fibrotic diseases, but its function in BA remains unclear. Here, we aim to determine the effect of macrophage necroptosis in the BA pathology, and to explore the specific molecular mechanism. We found that necroptosis existed in BA livers, which was occurred in liver macrophages. Furthermore, this process was mediated by ZBP1/p-MLKL, and the upregulated expression of ZBP1 in BA livers was correlated with liver fibrosis and prognosis. Similarly, in the bile duct ligation (BDL) induced mouse cholestatic liver injury model, macrophage necroptosis mediated by ZBP1/p-MLKL was also observed. In vitro, conjugated bile acid-glycodeoxycholate (GDCA) upregulated ZBP1 expression in mouse bone marrow-derived monocyte/macrophages (BMDMs) through sphingosine 1-phosphate receptor 2 (S1PR2), and the induction of ZBP1 was a prerequisite for the enhanced necroptosis. Finally, after selectively knocking down of macrophage S1pr2 in vivo, ZBP1/p-MLKL-mediated necroptosis was decreased, and further collagen deposition was markedly attenuated in BDL mice. Furthermore, macrophage Zbp1 or Mlkl specific knockdown also alleviated BDL-induced liver injury/fibrosis. In conclusion, GDCA/S1PR2/ZBP1/p-MLKL mediated macrophage necroptosis plays vital role in the pathogenesis of BA liver fibrosis, and targeting this process may represent a potential therapeutic strategy for BA.

Indocyanine green fluorescence imaging localization: A helpful addition to laparoscopic dissection and division of rectourethral fistulae.

BACKGROUND: Rectourethral fistulae (RUF) are the most prevalent type of anorectal malformations in boys, with various surgical treatment methods investigated in recent years. Currently, research is focused on preventing urethral damage or urethral diverticulum formation caused by imprecise dissection during the laparoscopically assisted anorectal pull-through (LAARP) technique. This study aimed to determine the efficacy of indocyanine green (ICG) fluorescence imaging to improve the localization and separation of the RUF during laparoscopic surgery. METHODS: ICG was intrarectally injected through a pre-inserted gastric tube at the distal enterostomy port to locate the fistula. This retrospective analysis included children with RUF who were treated surgically with ICG fluorescence imaging localization-assisted LAARP between January and June 2022. We investigated the patient demographics, perioperative conditions, and subsequent follow-up results. RESULTS: Four patients underwent ICG-assisted LAARP. Their median age was 80 days (range, 63-120) and the median duration of each procedure was 145 min (range, 120-165). Postoperatively, the duration of catheter retention and hospital stay was eight days. The children's prognosis was based on the follow-up outcomes of gastrointestinal, urinary tract function, and imaging examination. None of the included patients was diagnosed with urinary diverticulum, urinary tract injury, anal stricture, or rectal prolapse. CONCLUSIONS: Injection of ICG at the end of the rectum during LAARP surgery is a feasible adjunct for locating the RUF, providing a greater degree of accuracy for laparoscopic separation and resection of fistulae, thereby decreasing the risk of urological complications.

Computed tomography findings of communicating bronchopulmonary foregut malformation: a retrospective study of 18 patients.

BACKGROUND: Communicating bronchopulmonary foregut malformation is a rare anomaly characterized by a patent congenital communication between the esophagus or stomach and an isolated portion of the respiratory system. An esophagogram is taken as the gold standard for diagnosis. Compared with esophagography, computed tomography (CT) is more widely used and easily obtained, but CT findings have been described as nonspecific. PURPOSE: To describe CT findings in 18 patients with communicating bronchopulmonary foregut malformation to assist with early diagnosis. MATERIAL AND METHODS: A retrospective review of 18 patients who had proven communicating bronchopulmonary foregut malformation between January 2006 and December 2021 was conducted. For each patient, the medical records, including demographics, clinical manifestations, upper gastrointestinal radiography, magnetic resonance imaging and CT findings, were reviewed. RESULTS: Among the 18 patients, there were 8 males. The right to left ratio was 3.5:1. An entire lung was involved in 10 patients, a lobe or a segment was involved in 7 patients and an ectopic lesion was located in the right neck in 1 patient. The isolated lung may arise from the upper esophagus, mid-esophagus, lower esophagus or stomach, which were detected in 1, 3, 13, and 1 patient, respectively. On chest CT, an extra bronchus which did not arise from the trachea was detected in 14 patients. Contrast-enhanced chest CT was performed in 17 patients, the isolated lung receiving its blood supply from the pulmonary artery in 13 patients, the systemic artery in 11 patients and both pulmonary and systemic arteries in 7 patients. CONCLUSIONS: The presence of an extra bronchus, which does not arise from the trachea, highly suggests the diagnosis of communicating bronchopulmonary foregut malformation. Contrast-enhanced chest CT can provide accurate information regarding the airways, lung parenchyma and vascular structures that is useful to plan surgery.

Preliminary exploration of the efficacy of laparoscopic fluorescence cholangiography (LFC) in the diagnosis of biliary atresia compared with intraoperative cholangiography (IOC).

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 characteristics 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.

The Chinese Mandarin Version of the Esophageal-Atresia-Quality-of-Life Questionnaires for Children and Adolescents: Evaluation of Linguistic and Content Validity.

BACKGROUND: After repair of esophageal atresia (EA), children risk digestive and respiratory morbidity, but knowledge of their health-related quality of life (HRQOL) in China is lacking. The EA-QOL questionnaires were developed in Sweden and Germany to evaluate condition-specific HRQOL in children with EA aged 2-7 and 8-17. This study aimed to evaluate the linguistic and content validity of the Chinese Mandarin version of the EA-QOL questionnaires. METHODS: The procedure was conducted in compliance with international standards, including a forward-backward translation procedure, expert reviews, and cognitive debriefing interviews with 14 Chinese families of children with EA (parents of 8 children aged 2-7/6 children aged 8-17 and their parents). RESULTS: Following forward-backward translation, minor issues were identified and solved. In interviews, all participants rated all EA-QOL items easy to understand, none expressed negative emotions about them and most described them comprehensive and relevant for EA. Leading from cognitive debriefing, three EA-QOL items in the questionnaire version for children aged 2-7 and three EA-QOL items in the questionnaire version for children aged 8-17 were modified in the Chinese language to improve cultural appropriateness and/or clarity. CONCLUSION: The Chinese Mandarin version of the EA-QOL questionnaires achieved satisfactory linguistic and content validity. This can help increase focus of HRQOL in research and clinical practice of children with EA in China.

One-stage transanal endorectal pull-through for Hirschsprung disease: experience with 229 neonates.

OBJECTIVE: To evaluate the safety and efficacy of transanal endorectal pull-through (TEPT) and the long-term outcomes in newborns with Hirschsprung disease (HD). METHODS: 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. RESULTS: 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. CONCLUSION: TEPT is effective and safe for HD in the neonatal period and presents with a low rate of complications and an acceptable outcome.

Indocyanine green fluorescence imaging localization-assisted thoracoscopy revision surgery after repair of esophageal atresia.

Revision surgery for the complications after repair of esophageal atresia is often complex because of previous surgeries and chest infections and thus requires surgical expertise. This study describes surgical experiences with the use of indocyanine green (ICG) fluorescence imaging localization-assisted thoracoscopy during revision surgery, including recurrent tracheoesophageal fistula (rTEF) (8 cases, one of which was esophageal-pulmonary fistula) and delayed esophageal closure (1 case). We performed fistula repair and esophageal reconstruction according to the indications of ICG. The application of this method avoids the excessive trauma caused by freeing the trachea and esophagus. Contrast imaging taken one week and one month after surgery indicated no spillover of the contrast agent from the esophagus, except in 1 case. Indocyanine green fluorescence imaging localization-assisted thoracoscopy is worth promoting for revision surgery after esophageal atresia repair.

Comprehensive Analysis of Gut Microbiota and Fecal Bile Acid Profiles in Children With Biliary Atresia.

Background: Biliary atresia (BA) is the most common cholestatic liver disease in neonates. Herein, we aimed at characterizing the gut microbiota and fecal bile acid profiles of BA patients, defining the correlations between them, and evaluating the relationship between the clinical pathogenesis and changes in the gut microbiota and bile acid profiles. Methods: A total of 84 fecal samples from BA patients (n = 46) and matched healthy controls (HCs, n = 38) were subjected to sequencing by 16S rRNA gene amplification, and fecal bile acid were analyzed by targeted metabolomics. Findings: Compared with the controls, a structural separation of the intestinal flora of BA patients was uncovered, which was accompanied by changes in the composition of fecal bile acids. In the BA group, Actinobacillus, Monoglobus, and Agathobacter were enriched in patients without cholangitis (p < 0.05). Selenomonadaceae and Megamonas were more abundant in patients without recurrent cholangitis episodes (p < 0.05), while Lachnospiraceae and Ruminococcaceae were enriched in patients with multiple recurrences of cholangitis (p < 0.05). Postoperative jaundice clearance was associated with Campylobacter and Rikenellaceae (p < 0.05), and tauroursodeoxycholic acid was associated with jaundice clearance (p < 0.001). Conclusion: BA patients are characterized by different compositions of gut microbiota and bile acids, and their interaction is involved in the process of liver damage in BA, which may be closely related to the occurrence of postoperative cholangitis and jaundice clearance.

Exploratory application of indocyanine green quantification in biliary atresia observational study.

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.

Growth assessments for children with recurrent tracheoesophageal fistulas.

PURPOSE: To assess the growth status of children with recurrent tracheoesophageal fistula (rTEF), and determine the possible risk factors of growth retardation (GR). METHODS: The medical records of 83 patients with rTEF who underwent surgical repair were retrospectively analyzed. The patients were retrospectively divided into two groups according to whether they had GR. The clinical variables were compared between the GR and non-GR groups. Univariate and multivariable logistic regression analysis were performed to identify the risk factors for GR. RESULTS: Eighty-three children diagnosed with rTEF were included in this study. After a median follow-up of 31.4 (19.8, 48.7) months, GR occurred in 28 patients (33.7%). Among them, six patients with only weight for age Z score (WAZ) < -2SD, five patients with only height for age Z score (HAZ) < -2SD, and six patients with only BMI for age Z score (BAZ) < -2SD, while seven patients with both WAZ and HAZ < -2SD and four patients with both WAZ, HAZ and BAZ < -2SD. Multivariate logistic regression analysis showed that birth weight, anastomotic stricture and dysphagia after rTEF repair were independent risk factors with OR of 0.325 (0.119, 0.891), 4.396 (1.451, 13.324) and 5.341 (1.153, 24.752) for GR, respectively. CONCLUSIONS: GR is a common complication after rTEF repair. Birth weight, anastomotic stricture and dysphagia after rTEF repair are independent risk factors affecting growth.

Esophageal elongation by bougienage and delayed primary thoracoscopic anastomosis for pure esophageal atresia without tracheoesophageal fistula.

PURPOSE: We aim to share our experience of esophageal elongation by bougienage and delayed primary thoracoscopic anastomosis for pure esophageal atresia (EA) without tracheoesophageal fistula (TEF). METHODS: Fifteen patients with pure EA treated with delayed primary thoracoscopic anastomosis combined with or without esophageal elongation by bougienage were retrospectively analyzed. RESULTS: Four patients were managed without bougienage, and their surgical repair was performed thoracoscopically after natural esophageal growth. Among the remaining 11 patients, the average tension-free distance before elongation was 5 (4.5-6) vertebral bodies, and the mean age at the start and end of the bougienage period was 123 (63-280) days and 173 (106-350) days, respectively, with an average duration of 50 (29-82) days. The average age at the definitive operation in this series was 184 (107-385) days, with a mean operative duration of 186 (95-300) min. Neither anastomotic leakage nor TEF occurred, and oral feeding was partially or completely established in 13 patients during hospitalization. Among all patients, one was lost to follow-up, and others were followed up with an average duration of 47.7 (9.8-97.1) months. All patients had different degrees of anastomosis stricture, and 8 patients had gastroesophageal reflux. Oral feeding was completely established in 12 patients; however, tube feeding was required in 2 patients. CONCLUSIONS: The management of pure EA is complicated and inconclusive. Esophageal elongation by bougienage and delayed primary thoracoscopic anastomosis for long-gap pure EA without TEF is safe and effective.