Join now to get access to this content and more.
Become a SOAP member and have access to our benefits.
- For Review: SOAP Consensus Statement on Neuraxial Procedures in Thrombocytopenic Parturients
- Sample Centers of Excellence Applications
- ASA Corner
- SOAP Policy and Procedure Manual (P&P Manual)
- SOAP Expert Opinions
- SOAP's Learning Modules
- 2019 Annual Meeting Lecture Videos
- December 2018 - SOAP Unofficial Guide to ASA Committees Webinar
- Submit a Position
- View Job Postings
- Previous Meeting Archives
- Previous Meeting Abstract Search
- CMS Guidelines
- Member Benefits
- Newsletter Clinical Articles
- ACOG Documents
- Search our Patient Safety Archive
- Ask SOAP a Question
- Global Health Opportunities
- And more…
Cesarean Delivery of 2 Parturients with Fibrodysplasia Ossificans Progressiva: A Case Series
Abstract Number: F5C-3
Abstract Type: Case Report/Case Series
Fibrodysplasia Ossificans Progressiva (FOP) is a rare autosomal dominant disease characterized by progressive ossification of muscles, tendons, ligaments, aponeurosis and fascia. Ossification can occur sporadically or as a result of tissue trauma beginning in early childhood, leading to cumulative disability and death by age 40. (1) Knowledge of the management of pregnant FOP patients is limited due to only five reported childbirths in medical literature (excluding the 2 in this report). (2) Significant limb contractures predispose the FOP parturient to complex cesarean delivery as most of these patients are wheel-chair bound by their late twenties. FOP patients present a unique anesthetic challenge due to ossification of their temporomandibular joint (TMJ) and spine resulting in jaw immobility, severe kyphoscoliosis and restrictive lung disease. In addition, neuraxial anesthesia must be avoided as needle puncture causes painful inflammatory swelling with new bone growth at the injection site. (3) Our first FOP patient is a 24 y.o. G1P0, 58 kg female who delivered at 25 3/7 weeks, due to preterm labor and vaginal bleeding. Three years later, her sister also diagnosed with FOP was admitted for management of pre-eclampsia without severe features and IUGR. She is a 27 y.o. G1P0, 43 kg female who delivered urgently at 34 weeks due to preterm labor with rupture of membranes. Both patients underwent cesarean delivery with bilateral tubal ligation under general anesthesia with awake nasal fiberoptic intubation. ENT was present at bedside for possible emergent trach. Successful management of the FOP parturient requires multidisciplinary care with maternal fetal medicine (MFM), obstetric anesthesia, rheumatology and ENT. Both patients were initially evaluated in late second trimester, giving the team sufficient time to coordinate a delivery plan.
1. Fibrodysplasia Ossificans Progressiva. Atlas of Genetic Diagnosis and Counseling. Humana Press. 2006 pp 410-414.
2. Muglu JA, Garg A, Pandiarajan T, Shore EM, Kaplan FS. Pregnancy in Fibrodysplasia Ossificans Progressiva. Obstetric Medicine. 2012 Mar; 5(1): 35–38
3. Pignolo RJ, Shore EM, Kaplan FS. Fibrodysplasia Ossificans Progressiva: Diagnosis, Management and Therapeutic Horizons. Pediatr Endocrinol Rev. 2013 Jun; 10(0 2): 437–448