Background: Congenital biliary atresia is a type of obstruction of the bile ducts inside and outside the liver, which can lead to cholestatic liver cirrhosis and eventually liver failure. The preduodenal portal vein (PD-PV) is a rare developmental malformation of the PV. The PV courses in front of the duodenum. However, very few cases of neonatal biliary atresia combined with PD-PV have been reported in the scientific literature.
Case Summary: A 1-mo-and-4-d-old child was admitted to the hospital in January because of yellowish skin. After surgical consultation, surgical intervention was recommended. The child underwent Hilar-jejunal anastomosis, duodenal rhomboid anastomosis, and abdominal drainage under general anesthesia. During the operation, the PV was located at the anterior edge of the duodenum.
Conclusion: Diagnoses: (1) Congenital biliary atresia; (2) PD-PV; and (3) Congenital cardiovascular malformations. Outcomes: Recommendation for liver transplantation. Lessons: The choice of treatment options for neonatal biliary atresia combined with PD-PV.
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http://dx.doi.org/10.12998/wjcc.v9.i25.7542 | DOI Listing |
Pediatr Surg Int
December 2024
Department of Pediatric Surgery, Oslo University Hospital, Nydalen, P. O. Box 4950, N-0424, Oslo, Norway.
Background: The experience with Enhanced Recovery After Surgery (ERAS) protocols in neonatal intestinal surgery is very limited. We present the development and implementation of an Enhanced Recovery Protocol (ERP) designed specifically for neonates treated for congenital duodenal obstruction (CDO), and early outcome after implementation.
Methods: An ERP for CDO was developed and implemented.
Preduodenal portal vein (PDPV) is a rare congenital vascular malformation, which was first described by Knight in 1921 as an anomalous vein that lies in front of the duodenum, common bile duct, and hepatic artery instead of beneath them. This abnormal position may result in congenital duodenal obstruction and puts it in danger during operations around this region. PDPV is typically associated with other congenital anomalies, mainly intraabdominal and cardiac ones.
View Article and Find Full Text PDFEur J Pediatr Surg
December 2024
Pediatric Surgery, UKE, Hamburg, Germany.
Background Kasai procedure (KPE) is a palliative intervention in infants with biliary atresia (BA) aiming to restore biliary drainage. While the measure of success in BA is the post-Kasai native liver survival, BA remains the most frequent indication for liver transplantation in children. While a considerable amount of children fail to clear their jaundice following KPE, resulting in early liver failure and transplantation, some children become jaundice-free after "successful" KPE.
View Article and Find Full Text PDFJHEP Rep
January 2025
Division of Gastroenterology and Hepatology, Department of Medicine, University of Pennsylvania, Philadelphia, PA, USA.
Background & Aims: EGF-containing fibulin extracellular matrix protein 1 (EFEMP1, also called fibulin-3) is an extracellular matrix protein linked in a genome-wide association study to biliary atresia, a fibrotic disease of the neonatal extrahepatic bile duct. Fibulin-3 is deposited in most tissues and null mice have decreased elastic fibers in visceral fascia; however, fibulin-3 does not have a role in the development of large elastic fibers and its overall function in the extrahepatic bile ducts remains unclear.
Methods: We used staining and histology to define the amount and organization of key extracellular matrix components in the extrahepatic bile ducts.
Front Pediatr
December 2024
Department of General Surgery, Children's Hospital of Soochow University, Suzhou, China.
Background: The pivotal importance of surgical treatment for pediatric biliary atresia is well-established. This systematic review and meta-analysis was designed to assess the comparative efficacy and safety of open portoenterostomy (OPE) and laparoscopic portoenterostomy (LPE) in managing this condition, providing valuable guidance for clinical decision-making.
Methods: A comprehensive literature review was conducted by two researchers in databases such as PubMed, up to July 10, 2024, focusing on studies that evaluated the role of LPE vs.
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