Anesthetic management in pregnancy with osteogenesis imperfecta type XI: A comprehensive case report.

INTRODUCTION

Osteogenesis imperfecta (OI) type XI or Bruck syndrome is an extremely rare genetic disorder characterized by congenital joint contractures and bone fragility. OI presents unique and considerable challenges in the perioperative and anesthetic management of affected patients.

PRESENTATION OF CASE: A 29-year-old primigravida with OI type XI (100 cm, 26 kg) and severe kyphoscoliosis underwent urgent Caesarean delivery at 32 weeks under general anesthesia (sevoflurane/nitrous oxide). A female infant (1470 g) required resuscitation. Postoperative recovery was uneventful.

DISCUSSION: The rarity of this syndrome, along with the physiological changes associated with pregnancy, creates an unprecedented clinical scenario that demands a thorough and cautious approach to patient care.

CONCLUSION

Osteogenesis imperfecta (OI) type XI necessitates careful anesthetic management in pregnancy. This case highlights the anesthetic management challenges and the use of a multidisciplinary approach to enhance clinical understanding and improve patient outcomes.