Anesthetic Nuances Of A Patient With Acyanotic Congenital Heart Disease And Smith-Lemli-Opitz Syndrome Scheduled For Hypospadias Repair: A Case Report
Year: 2025
Type: article
Study design: Case Report
Abstract: Introduction: Smith-Lemli-Opitz Syndrome (SLOS) is a rare autosomal recessive disorder characterized by defective cholesterol biosynthesis leading to multiple congenital anomalies including central nervous, cardiovascular, and genitourinary malformations. Patients with SLOS pose unique anesthetic challenges including susceptibility to neuromuscular hyperexcitability and malignant hyperthermia-like events. Main symptoms: A 16-year-old male with SLOS and acyanotic congenital heart disease presented with dribbling of urine diagnosed as hypospadias requiring surgical repair. Diagnosis, intervention, outcome: He had a small patent ductus arteriosus (PDA) with a left-to-right shunt and stable biventricular function. Anesthesia was successfully managed with total intravenous anesthesia (TIVA) using target-controlled infusion (TCI) of propofol, vecuronium muscle relaxation, and caudal block without complications. The perioperative course was uneventful, and the patient was discharged on post-operative day one. Conclusion: This case highlights the importance of individualized anesthetic management in SLOS patients with congenital heart disease, avoiding volatile anesthetics and depolarizing muscle relaxants while maintaining cardiovascular stability.
DOI: 10.64252/rm1v3997
Authors: Roshni Raman, Mahesh Vakamudi, Somaganesh Neethirajan, Kishore Manivannan
Language: English
Subfield: Anesthesiology and Pain Medicine
Field: Medicine
Domain: Health Sciences
Open Access status: hybrid
OpenAlex ID: W4413743623