Enhanced lymphangiogenesis in the left lateral segment of a biopsied liver during portoenterostomy for biliary atresia.

PURPOSE: We investigate the histopathology of the portal vein branches and lymphatic vessels to elucidate the mechanism of atrophy of the left lateral segment (LLS) of the liver in biliary atresia (BA). METHODS: LLS and right anterior segment (RAS) liver biopsy samples obtained during Kasai portoenterostomy (KPE) from ten consecutive patients with BA underwent histopathological investigation of the portal vein and lymphatic vessels using double chromogenic immunostaining for CD31/D2-40 and the hepatitis-like findings (HLF) score. Each parameter and clinical data were compared between prognostic groups. RESULTS: HLF scores in the LLS were always higher than those in the RAS. There was no difference in portal vein and lymphatic vascular morphology, whereas the number of lymphatic vessels was correlated with the fibrotic area of all specimen areas. Left-to-right ratio of the number of lymphatic vessels was correlated with the age at KPE (r = 0.784, p = 0.007) and the pre-KPE CRP value (r = 0.723, p = 0.018). CONCLUSIONS: Lymphangiogenesis on the LLS compared to the RAS was significantly correlated with the degree of fibrosis and the age at KPE. Further investigation is warranted to clarify the causes of LLS atrophy and lymphangiogenesis relevant to immune dysregulation.

Endoscopic vacuum assisted closure therapy for esophagopericardial fistula in a 16-year-old male: A case report.

BACKGROUND: Esophagopericardial fistula (EPF) is a rare, life-threatening condition with limited scientific literature and no established management guidelines. This case report highlights a successful multidisciplinary approach and the innovative use of endoscopic vacuum assisted closure (endoVAC) therapy in treating this complex condition. CASE SUMMARY: A 16-year-old male with a history of esophageal atresia and colon interposition presented with progressive chest pain, fever, and dyspnea. Imaging revealed an EPF with associated pleural and pericardial effusions. Initial management with an esophageal stent failed, prompting the use of an endoVAC system. The patient underwent multiple endoVAC device changes and received broad-spectrum antibiotics and nutritional support. The fistula successfully closed, and the patient recovered, demonstrating no new symptoms at a 6-month follow-up. CONCLUSION: EndoVAC therapy can effectively manage EPF, providing a minimally invasive treatment option.

Newborn screening for biliary atresia using direct bilirubin: An implementation science study.

OBJECTIVE: Biliary atresia (BA) is a liver disease of infancy characterized by obstruction of the biliary tree. Infants with BA have the best outcomes when identified early and the Kasai portoenterostomy is performed before 45 days of life (DoL). In our hospital system, the average age at Kasai was 60 DoL. To address the problem of late presentation, we implemented a two-stage BA screening strategy utilizing direct bilirubin (DB). METHODS: New institutional policies were established that all newborns were tested at 24-48 h of life, and those with levels ≥0.5 mg/dL were followed further. The infant's primary care provider was contacted to recommend a repeat DB at 2 weeks of life. If the repeat DB was ≥1.0 mg/dL, the patient was evaluated by gastroenterology. RESULTS: Over the 16 months, 3880 infants were born and 3861 (99.5%) were screened; 53 infants (1.3%) had DB levels ≥0.5 mg/dL initially. Upon repeat testing at 2 weeks, there were three groups of infants: not retested (n = 1), retested <1.0 mg/dL (n = 40), and retested ≥1.0 mg/dL (n = 12). The average time to be seen by gastroenterology was 4.3 days or 18.3 DoL. DISCUSSION: The screening included a series of steps that needed to be implemented effectively. Screening had a net false positive rate of 0.3% (12 out of 3861) and identified causes of cholestasis other than BA. BA was excluded by 28 DoL on average. Our results can provide a template for other institutions interested in implementing a BA screening protocol in their practice.

Anchoring guide catheter to facilitate recanalising occluded pulmonary vein in small children: a novel technique.

Transcatheter pulmonary vein recanalisation is challenging. Herein, we described a novel technique facilitating the recanalisation of occluded three pulmonary veins in two small children. This technique enhances the catheter's alignment and stability during the recanalisation process. Referred to as the "anchoring guide catheter" technique, it can be applied only when the ipsilateral vein is patent.

Our experience in repairs using the native esophagus such as the Foker and Gazi methods in the management of patients with long-gap esophageal atresia.

This study aimed to share our experience with infants with repaired long-gap esophageal atresia (LGEA) using the native esophagus and Foker and Gazi methods. We retrospectively analyzed the medical records of 10 patients with LGEA (six with pure esophageal atresia [EA], and four with distal trachea-esophageal fistula [TEF] + EA). The mean length between the esophageal pouches was 5.9 cm (4-9 cm). Five Foker methods, three Gazi methods, and two delayed primary repairs after a daily bougie were performed an average of 19.3 days after the first surgery and 26.4 days after the final esophageal anastomosis. For the Foker technique, it was 36.1 days. Their first oral intake was 10.2 days, and their transition to full enteral food was 26.2 days. An esophageal leak was detected in six patients. Fundoplication and dilatations were performed for three and four patients, respectively. For good results, LGEA patients should be operated on at least under the supervision of an experienced surgeon in specialized centers, and the team should be familiar with the techniques for using the native esophagus.

Polymeric immunoglobulin receptor promotes Th2 immune response in the liver by increasing cholangiocytes derived IL-33: a diagnostic and therapeutic biomarker of biliary atresia.

BACKGROUND: Biliary atresia (BA) is a devastating neonatal cholangiopathy with an unclear pathogenesis, and prompt diagnosis of BA is currently challenging. METHODS: Proteomic and immunoassay analyses were performed with serum samples from 250 patients to find potential BA biomarkers. The expression features of polymeric immunoglobulin receptor (PIGR) were investigated using human biopsy samples, three different experimental mouse models, and cultured human biliary epithelial cells (BECs). Chemically modified small interfering RNA and adenovirus expression vector were applied for in vivo silencing and overexpressing PIGR in a rotavirus-induced BA mouse model. Luminex-based multiplex cytokine assays and RNA sequencing were used to explore the molecular mechanism of PIGR involvement in the BA pathogenesis. FINDINGS: Serum levels of PIGR, poliovirus receptor (PVR), and aldolase B (ALDOB) were increased in BA patients and accurately distinguished BA from infantile hepatitis syndrome (IHS). Combined PIGR and PVR analysis distinguished BA from IHS with an area under the receiver operating characteristic curve of 0.968 and an accuracy of 0.935. PIGR expression was upregulated in the biliary epithelium of BA patients; Th1 cytokines TNF-α and IFN-γ induced PIGR expression in BECs via activating NF-κB pathway. Silencing PIGR alleviated symptoms, reduced IL-33 expression, and restrained hepatic Th2 inflammation in BA mouse model; while overexpressing PIGR increased liver fibrosis and IL-33 expression, and boosted hepatic Th2 inflammation in BA mouse model. PIGR expression promotes the proliferation and epithelial-mesenchymal transition, and reduced the apoptosis of BECs. INTERPRETATION: PIGR participated in BA pathogenesis by promoting hepatic Th2 inflammation via increasing cholangiocytes derived IL-33; PIGR has the value as a diagnostic and therapeutic biomarker of BA. FUNDING: This study was financially supported by the National Natural Science Foundation of China (82170529), the National Key R&D Program (2021YFC2701003), and the National Natural Science Foundation of China (82272022).

Parents caring and sham-feeding their child born with Esophageal atresia at home while waiting for reconstructive surgery.

PURPOSE: For children with Esophageal atresia who have to wait for reconstructive surgery, long hospital stay, delayed introduction of oral feeds and hampered oro-motor function has traditionally been draw-backs for this treatment as the patients have minimal training of oro-motor function while waiting for surgery. In this paper, we present the concept of sham-feed at home awaiting reconstructive surgery with the aim to obliviate these problems. The aim was to describe the characteristics of patients with Esophageal atresia waiting for reconstructive surgery sham-feeding at home by their parents and further describe adverse events that arose. METHODS: The study is a retrospective descriptive single center study on all children with a delayed reconstruction of Esophageal atresia who was sham-fed by their parents at home before reconstructive surgery between January 2010 and January 2023 at the Karolinska University Hospital, Stockholm. RESULTS: Nine patients where home waiting for reconstructive surgery for a median of 72 days. No adverse events were reported related to the sham-feed procedure at home. The children had reconstructive surgery at a median 120 days of age. Five of the children ate full meals orally day 8-27 after surgery. Two children ate fully before 1 year after surgery. Two children had surgery less than 1 year ago and were not eating fully orally at the time of data collection. CONCLUSION: Sham-feeding at home by the parents was safe and feasible with the benefits of a prolonged time out of hospital awaiting reconstructive surgery.

Infracoccygeal/transperineal window: new method to prenatally diagnose and classify level of anal atresia.

OBJECTIVES: To introduce a two-dimensional sonographic method to assess the fetal anus, and to evaluate the feasibility of this method to diagnose anal atresia prenatally and identify the presence or absence of anoperineal fistula (in males) and anovestibular fistula (in females). METHODS: This was an observational study of suspected cases of anal atresia referred to a single center in Israel between August 2018 and October 2023. In addition to conventional evaluation of the perineum in the axial plane, fetuses referred to our center for suspected malformation were scanned with a new method termed the 'infracoccygeal/transperineal window'. This window consisted of a midsagittal view of the fetal pelvis, including the distal rectum and the anal canal. Normal anatomy was confirmed when the anal canal was continuous with the rectum and terminated at the expected location on the perineum. In female fetuses, the normal anal canal runs parallel to the vaginal canal and diverges posteriorly, terminating at the perineal skin, distant from the vestibule. In male fetuses, the normal anal canal diverges posteriorly in relation to the corpora cavernosa, terminating at the perineal skin, distant from the scrotum. High anal atresia was identified when a blind-ending rectal pouch was demonstrated in the pelvis without a fistula to the perineum or vestibule. Low anal atresia was determined when a rectal pouch was continuous with an anteriorly deflected fistula. In females, the fistula converges with the vaginal canal, terminating at the vestibule; in males, the fistula deflects anteriorly, terminating at the base of the scrotum. Postnatally, the diagnosis and type of anal atresia were confirmed through physical examination with direct visualization of the fistula, radiographic studies, surgical examination and/or postmortem autopsy. RESULTS: Of the 16 fetuses diagnosed prenatally with anal atresia, eight were suspected to have low anal atresia and eight were suspected to have high anal atresia. The median gestational age at diagnosis was 23 (range, 14-37) weeks. All cases showed additional structural malformation. Eleven patients opted for termination of pregnancy, of which four had low anal atresia and seven had high anal atresia. Postnatal confirmation was not available in four cases due to curettage-induced mutilation or in-utero degradation following selective termination of the affected twin, leaving 12 cases for analysis, of which seven were diagnosed with low anal atresia and five with high anal atresia. In these 12 cases, all prenatal diagnoses were confirmed as correct, rendering 100% sensitivity and 100% specificity in this high-risk fetal population. CONCLUSIONS: The infracoccygeal/transperineal window is an effective method to detect and classify the level of anal atresia prenatally. This may improve prediction of postnatal fetal continence and optimize prenatal counseling. © 2024 International Society of Ultrasound in Obstetrics and Gynecology.

Long-gap esophageal atresia: is native esophagus preservation always possible?

Introduction: Esophageal atresia (EA) is a congenital defect that causes esophageal discontinuity, often with an associated tracheo-esophageal fistula (TEF) in 70%-90% of cases. When the distance between esophageal ends precludes primary anastomosis, it results in long gap esophageal atresia (LGEA), complicating the surgical management. This study retrospectively reviewed LGEA cases from the past decade, treated with the goal of preserving the native esophagus, comparing surgical techniques and outcomes with current literature. Materials and methods: The data of patients treated for LGEA between 2013 and 2024 were collected from medical charts, focusing on patients treated with the preservation of their native esophagus. Results: Ten patients were enrolled for this study. All of them had a gap between the esophageal ends equal to or greater than three vertebral bodies. Four patients (40%) underwent a delayed primary anastomosis (DPA) procedure, while the remaining six (60%) underwent a traction staged repair. All patients were treated with open surgery. The follow-up period extended from 3 months to 10 years. Conclusion: Preserving the native esophagus in patients with LGEA is a challenging but feasible goal, with delayed primary anastomosis and traction techniques playing key roles. We advocate for the preservation of the native esophagus as the preferred approach for ensuring a high quality of life for patients, as it helps to avoid severe long-term complications associated with esophageal substitution.