///2019 Abstract Details
2019 Abstract Details2019-07-13T07:45:15-05:00

Anesthetic Considerations for Osteogenesis Imperfecta in Pregnancy

Abstract Number: FCI-537
Abstract Type: Case Report Case Series

Neil Kalariya MD1 ; Jessica Ansari MD2

Osteogenesis imperfecta (OI) or “brittle bone disease” encompasses a wide spectrum of features related to genetic malformation of type 1 collagen. This collagen plays a pivotal role in the formation of bones, dentin, sclera, ligaments, and tendons. Here we describe a case of maternal and fetal OI and considerations when assessing and treating these patients.

A 29 year old G1P000 at 33w1d with history of mild intermittent asthma and osteogenesis imperfecta that presented for primary cesarean section for preeclampsia with severe features and non-reassuring fetal heart rate tracing. Her pregnancy has additionally been complicated by high suspicion for fetal osteogenesis imperfecta, IUGR, and preeclampsia.

Complications of her OI include poor dentition and severe scoliosis requiring C7 to S1 complete spinal fusion with Harrington rods with subsequent severe kyphosis and lordosis. She has been primarily wheelchair bound since childhood, but is able to transfer independently. She is 3’9” and on day of delivery had a BMI of 59. Although she had not undergone genetic testing, from her clinical exam, she had been previously been diagnosed with type 3/4.

Preoperatively, large bore intravenous access was established as well as arterial line. Intraoperatively there was an unsuccessful attempt at neuraxial block, followed by uncomplicated general anesthetic for delivery. Special attention was made to padding and positioning. The fetus was delivered en caul and amniotic sac was ruptured with NICU on the surgical field. Postoperative pain was managed with bilateral TAP blocks and PCA.

Osteogenesiis imperfecta

Incidence

-1 ini 20,000 live births

Concerns with Pregnancy

-Gravid uterus can cause further reduction on FRC from expected as there is often a “bell shaped” chest and restrictive lung physiology

-Higher risk of postpartum hemorrhage from platelet adhesion abnormalities

-Often associated with preterm delivery, preeclampsia, and gestational diabetes.

Anesthetic Management

A primary concern includes preventing additional fractures. This can be accomplished by:

-Avoiding succinylcholine as these patients often have prolonged states of immobility and fasciculations could result in fractures:

-Meticulous attention to padding and positioning

-Utilizing invasive arterial blood pressure monitoring to avoid regular blood pressure cuff cycling.

-Planned cesarean delivery

Additional Considerations include:

-No link between OI and malignant hyperthermia, but can have a hypermetabolic state under general anesthesia causing hyperthermia

-Short stature makes appropriate dosing for neuraxial blocks challenging, recommend a catheter technique

-Often have a history of scoliosis and multiple vertebral body compressions making neuraxial block difficult

Osteogenesis Imperfecta. In: Mankowitz S. (eds) Consults in Obstetric Anesthesiology. Springer, Cham

Osteogenesis imperfecta. Lancet. 2016; 387:1657-71.

SOAP 2019