///2009 Abstract Details
2009 Abstract Details2018-05-01T17:45:11+00:00

Anesthetic Considerations in a Parturient with Osteogenesis Imperfecta

Abstract Number: 250
Abstract Type: Case Report/Case Series

Brenda J. Christopher BS, MD1 ; James I, Thomas MD2; Lindsey Nelson MD3

Anesthetic Considerations in a Parturient with Osteogenesis Imperfecta

Christopher BJ, MD, James TI, MD, Nelson L, MD

Department of Anesthesiology, University of Cincinnati College of Medicine

Introduction

Osteogenesis imperfecta is an autosomal dominant connective tissue disorder resulting from a defect in type I collagen formation that leads to multisystem involvement which further complicates perioperative anesthetic management in pregnancy. Although the primary manifestation involves increased susceptibility to fractures, patients may have problems with airway anomalies, cardiovascular abnormalities, ENT malformations, perioperative bleeding, intraoperative hyperthermia and hyperthyroidism. The progressive deforming type, type III, is the most severe non-lethal form that involves multiple fractures, kyphoscoliosis, blue sclera and dentinogenesis imperfecta.

Case presentation

A 33 year old G4P1 with type III osteogenesis imperfecta presented for anesthesia evaluation at 24 weeks. The patient had preeclampsia with her first child requiring cesarean section at 32 weeks. The patient had short stature and a short neck, severe kyphoscoliosis, a history of neck fractures, limited cervical spine mobility, baseline shortness of breath due to decreased functional residual capacity, and gastroesophageal reflux. An awake fiberoptic intubation was discussed with the patient.

The day of the cesarean section, oral bicitra and nebulized lidocaine were administered, and the patient then positioned with left uterine displacement. After preoxygenation and sedation with versed and remifentanil, titrated to maintain spontaneous respirations, direct laryngoscopy with a Miller 2 blade revealed a grade I view of the cords enabling intubation with a 7.0 ETT tube. Anesthesia was maintained with sevoflurance and hydromorphone and the patient was extubated successfully without postoperative events.

Discussion

Osteogenesis imperfecta coexisting with pregnancy can present unique anesthetic challenges. The increased susceptibility to fractures and the thoracic skeletal deformities in type III can compromise respiratory function (1). These bony abnormalities in addition to the metabolic, pulmonary and cardiac disorders can significantly increase maternal morbidity and mortality (2). Endotracheal intubation can be accomplished with awake fiberoptic or direct laryngoscopy while titrating sedatives to minimize respiratory depression during the procedure.

References

1. Stynowick GA, Tobias JD. Perioperative Care of the Patient with Osteogenesis Imperfecta. Orthopedics 2007;30:1043.

2. Vogel TM, et al. Pregnancy Complicated by Severe Osteogenesis Imperfecta: A Report of Two Cases. Anesthesia Analgesia 2002;94:1315-1317.

SOAP 2009