Liver transplant in syndromatic biliary atresia
Introduction and Objectives: Biliary atresia (BA) is an obliterative cholangiopathy, there are at least two phenotypes, one of them is the syndromic form, which occurs in 10-15% of cases, it is associated with polysplenia, heart disease, heterotaxy and malrotation intestinal. The objective of this r...
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| Main Authors: | , , |
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| Format: | Article |
| Language: | English |
| Published: |
Elsevier
2025-04-01
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| Series: | Annals of Hepatology |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S1665268125001164 |
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| Summary: | Introduction and Objectives: Biliary atresia (BA) is an obliterative cholangiopathy, there are at least two phenotypes, one of them is the syndromic form, which occurs in 10-15% of cases, it is associated with polysplenia, heart disease, heterotaxy and malrotation intestinal. The objective of this review is to present the experience when performing liver transplantation Materials and Patients: Female patient daughter of a diabetic mother, carrier of BA type III who underwent Kasai surgery at 89 days of life, carrier of intestinal malrotation, preduodenal portal vein, intraventricular communication of 1.7 mm without hemodynamic repercussion and dyslipidemia, without biliary clearance and pondostatural arrest, worthy of performing a liver transplant from an unrelated living donor, due to complications of cirrhosis such as ascites, malnutrition and cholesasis, at the time of surgery, annular pancreas, were found as additional findings to those described agenesis of cava, agenesis of the celiac trunk, presented early partial thrombosis of the portal vein, meriting anticoagulant and antithrombotic treatment, with resolution of the condition, without requiring surgical intervention. Currently, after one year of follow-up with adequate evolution, without cholestasis or transaminasemia, adequate growth, immunosuppression with a calcineurin inhibitor, the dyslipidemia resolved. Our patient does not have polysplenia. Results: The clinical case of syndromic BA is presented, although BA is rare, the syndromic presentation is even more, so we present a successful case, with complex vascular malformations combined with extrahepatic malformations, mainly cardiac, with which mortality at time of performing the transplant is high, greater than 90%. Our patient had a satisfactory surgical and clinical evolution. Conclusions: BA is the main cause of liver transplantation in pediatrics; reported cases of the syndromatic type are rare. The complete evaluation and planning of possible vascular malformations associated at the time of transplantation should alert the medical and surgical team. |
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| ISSN: | 1665-2681 |