Anesthetic Management of Cesarean Section in a Patient With Mucopolysaccharidosis VI (MPS VI): A Case Report.

OBJECTIVE

Mucopolysaccharidosis VI (MPS VI), a rare lysosomal storage disorder, leads to multisystem involvement (airway, skeletal, cardiac, and spinal pathologies) that significantly elevates anesthetic risks during cesarean delivery.

CASE REPORT: A 29-year-old primigravida with MPS VI underwent clinically indicated elective cesarean section at 26 weeks' gestation due to progressive lower limb weakness that made her unable to tolerate continued pregnancy, following multidisciplinary obstetric and anesthesiology evaluation. Critical clinical features included extreme short stature, wheelchair dependence, macroglossia, severely restricted mouth opening (Mallampati class IV), and concomitant aortic valve disease. Ultrasound-guided continuous epidural anesthesia was administered through incremental injection of lidocaine carbonate, achieving adequate surgical anesthesia (T6 sensory level). The procedure was completed uneventfully, with hemodynamic stability and successful delivery of a viable neonate.

CONCLUSION

This case demonstrates that continuous epidural anesthesia, when supported by comprehensive preoperative evaluation, ultrasound guidance, and contingency general anesthesia preparedness, represents a viable option for cesarean delivery in MPS VI patients.