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Orthotopic liver transplantation in patients with biliary atresia and situs inversus
1Division of Pediatric Surgery, Department of Surgery, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Insights
Situs inversus (SI) and polysplenia syndrome (PS) are common in pediatric liver transplant patients with biliary atresia. These cases demonstrate successful orthotopic liver transplantation (OLT) in SI patients, managing associated anomalies with technical modifications.
Area of Science:
- Hepatology
- Pediatric Surgery
- Transplantation Immunology
Background:
- Situs inversus (SI) and polysplenia syndrome (PS) are congenital conditions associated with biliary atresia, a leading indication for pediatric liver transplantation.
- Understanding the implications of these anatomical variations is crucial for successful surgical outcomes.
Observation:
- Two pediatric cases of orthotopic liver transplantation (OLT) in patients with SI are presented.
- One patient had complete SI (situs inversus totalis) with liver placement in the left upper quadrant (LUQ).
- The second patient had PS and isolated liver SI, receiving a left-lateral segment graft in the LUQ.
Findings:
- Associated vascular and intestinal anomalies in SI, especially with PS, can be successfully managed through technical modifications during OLT.
- Concerns regarding the placement of a standard liver graft in SI patients were unfounded.
- These findings suggest adaptability of OLT techniques for complex anatomical variations.
Implications:
- Surgical strategies for OLT in pediatric patients with SI and PS can be effectively modified to achieve successful outcomes.
- This work supports the feasibility of OLT in patients with complex congenital anomalies.
- Further research into standardized modifications for SI/PS patients undergoing OLT is warranted.
Abstract:
Situs inversus (SI) and the polysplenia syndrome (PS) occur relatively frequently in patients with biliary atresia, the largest subgroup of pediatric liver transplantation patients. We present two cases of orthotopic liver transplantation (OLT) in pediatric patients with SI. One had SI totalis, where a normal liver was placed in the left upper quadrant (LUQ) with the right lobe overlying the vertebral column. The second had PS and isolated SI of the liver, and a living-related left-lateral-segment graft was placed in the LUQ. Although multiple, often unpredictable vascular and intestinal anomalies occur frequently in association with SI, particularly in the setting of the PS, these cases, as well as several others recently reported, reveal that these anomalies can be managed successfully by a variety of technical modifications of the standard OLT technique. Likewise, concerns about the placement of a situs solitus liver in the midline or LUQ position of a SI abdomen have proved to be unfounded.