ABSTRACT
Hutchinson-Gilford Progeria Syndrome (HGPS) is a rare genetic disorder causing accelerated aging and age-related pathologies. Weighing benefits and risks on doing surgical versus conservative pain management require multidisciplinary planning and consideration in HGPS patients. This presents a case of a 15-year-old patient with HGPS with severe pain from bilateral hip dislocation managed with peripheral nerve block and steroid injection. This afforded her immediate pain relief allowing her to undergo physical rehabilitation comfortably.
ABSTRACT
Osteogenesis imperfecta is a rare inherited connective tissue disorder that presents with skeletal deformities and extraskeletal abnormalities. Pregnancy superimposed on existing osteogenesis imperfecta has a significantly increased maternal and fetal morbidity, hence presents multiple unique anesthetic challenges. A 25- year old term primigravid previously diagnosed with osteogenesis imperfecta and muscular dysthrophy presents for cesarean section. Important considerations in the management of this patient included anesthetic choice with their corresponding advantages and possible complications, patient positioning, intraoperative monitoring and possibility of difficult airway. Knowledge of the physiologic and anatomic abnormalities of the individual patients as well as understanding the advantages and complications associated with both regional and general anesthesia are thus crucial in formulating the appropriate anesthetic management plan that would ensure safety of both mother and child.