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Management of a Parturient with Osteogenesis Imperfecta
Abstract Number: T4C-1
Abstract Type: Case Report/Case Series
This is a case report of a 31 year old female, G4P2022, with history of Osteogensis Imperfecta Type I (OI), who underwent repeat elective cesarean section at 39 weeks of gestation. It is estimated that the incidence of OI is about 1:30,000, which is an autosomal dominant collagen disorder.
Multidisciplinary team discussion was conducted to plan an optimal obstetric and anesthetic plan of care. Our patient’s history was significant for femoral fracture at one year of age but no other significant history. She was 4' 7” tall and had abnormal facial deformities and previous cesarean section x2 under neuraxial anesthesia. Echocardiogram revealed good EF and no other abnormalities. The fetus was found to have OI Type I and already showing bowing of extremities with possible fractures on imaging.
In the operating room standard ASA monitors were placed. Combined spinal epidural was placed uneventfully. Patient was positioned cautiously, taking extra care to pad pressure points. After hemodynamic stability was achieved, blood pressure cuff was set to cycle at every 5 minutes. Larger skin incision was made to facilitate easy delivery and avoid fundal pressure. Good uterine tone was ascertained prior to closure. Patient was carefully transferred to stretcher and brought to PACU. Patient was discharged on POD #3.
OI involves abnormal endochondral ossification with increased bone fragility and risk of multiple, spontaneous fractures. Different types with varying severity exist. Classic clinical findings are bluish sclera, hyperhidrosis, conduction deafness, hyperthermia, teeth deformities, platelet dysfunction, cardiac valvular disease and cardiomyopathy.
Management includes multidisciplinary discussion between obstetrician, anesthesiologist and pediatrician. Planning included careful positioning of patient with avoidance of excessive pressure to patient or fetus. Because this disease is transmitted in an autosomal dominant fashion, risk to the fetus must also be considered.
It has not been found that cesarean section provides any benefit to reduce injury to the mother or fetus. However due to maternal pelvic deformities and associated short stature there is an increased incidence of cephalopelvic disproportion leading to cesarean section.
Anesthetic considerations include careful assessment for musculoskeletal abnormalities. If neuraxial anesthesia is planned, gentle placement of spinal/epidural to avoid trauma or fracture must be taken. If general anesthesia is required, gentle laryngoscopy and intubation is key to avoiding injury to structures of neck, mandible and cervical spine. Multidisciplinary team approach is essential to coordinate care for successful outcomes.
Sharma A, George L, Erskin K - Osteogenesis Imperfecta in pregnancy: two case reports and review of literature. Obstet Gynecol Surv, 2001;56:563-566.