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Combined general and epidural anesthesia for a child with alagille syndrome: a case report
Kathirvel Subramaniam1, Laura B Myers
1Department of Anesthesiology and Critical Care Medicine, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA 02115, USA.
Insights
Alagille syndrome, a rare genetic disorder, requires careful perioperative planning for surgical procedures. This case highlights the management of a pediatric patient with Alagille syndrome undergoing ileal exclusion and epidural anesthesia.
Area of Science:
- Pediatric Surgery
- Anesthesiology
- Gastroenterology
Background:
- Alagille syndrome is the most common inherited cause of intrahepatic cholestasis.
- It presents with a range of clinical manifestations affecting multiple organ systems.
Observation:
- This report details the perioperative management of a pediatric patient diagnosed with Alagille syndrome.
- The patient underwent an ileal exclusion procedure.
Findings:
- Specific challenges in managing Alagille syndrome during surgery were identified.
- The use of epidural anesthesia in these patients presents unique considerations.
Implications:
- This case provides valuable insights for anesthesiologists and surgeons managing pediatric patients with Alagille syndrome.
- Optimized perioperative strategies are crucial for improving outcomes in patients with this complex condition.
Abstract:
Alagille syndrome (syndromic paucity of interlobular bile ducts) is the most common form of familial intrahepatic cholestasis. We describe the perioperative management of a pediatric patient with Alagille syndrome undergoing ileal exclusion and the specific issues associated with epidural anesthesia with this syndrome.
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