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Profound Weakness in Labor: Labor Analgesia Management for Patients With Fascioscapulohumeral Muscular Dystrophy
Abstract Number: T2D-2
Abstract Type: Case Report/Case Series
Fascioscapulohumeral dystrophy (FSHD) is an autosomal dominant disorder with a prevalence around 1:20,000, making it the second most common adult muscular dystrophy in the United States.
This disorder is characterized by facial, shoulder, and arm weakness, but other muscle groups may also be affected. Severity varies amongst those with the disorder. Though affected patients typically have a normal life expectancy, 20% end up wheelchair bound by the 4th or 5th decade of life.
During pregnancy, 25% of patients with a diagnosis of FSHD experience a worsening of symptoms which fails to improve after childbirth. Although rare, respiratory and or cardiovascular muscles may also become compromised.
Due to the rarity of this disorder, there are no clear guidelines to guide the care of the affected parturient.
A 27-year-old woman at 39.6 weeks gestation and a known history of fascioscapulohumeral dystrophy presented to the L&D suite in active labor with no prior anesthesia consult. Uneventful prenatal course. The patient had received adequate prenatal care throughout her pregnancy with close neurological care.
Patient diagnosed at five years old due to weak smile, and inability to play wood instruments.
Progressive left> right scapular winging and abdominal weakness
Father with known diagnosis, who is wheelchair bound
Progressive right-hand weakness throughout pregnancy
Hypermetabolic; ht 5’7’’ and wt 110lbs on admission
PFTs completed during pregnancy showed normal spirometry; normal FVC, FEV1, and FEV1/FVC ratio.
The case was discussed extensively with neurology, obstetrical and anesthesia teams. Per patient’s request, the decision was made for an early labor epidural placement for pain management. During the second stage of labor maternal exhaustion was noted. Vacuum-assisted vaginal delivery was performed to expedite delivery. Baby girl. APGARS 7/8. The patient remained clinically stable and was discharged home on post-partum day #3.
Two retrospective cohort studies have shown favorable birth outcomes for women with FSHD, though the disorder is associated with prolonged second stage of labor and increased risk of operative vaginal delivery and cesarean section.
Increased pulmonary complications associated with general anesthesia
Patients may have weakened oropharyngeal muscles placing them at increased risk of aspiration, especially with emergence
If general anesthesia used, avoidance of succinylcholine due to increased risk of hyperkalemia
Care must be used with TOF lead placement to avoid atrophied muscles.
Patient at baseline may not be able to lift head, so head lift may not be the best measure of strength for extubation.
Slowly loaded epidural may be preferred over spinal or CSE to avoid respiratory muscle paralysis
To be added on poster