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Intrathecal anesthesia for emergency cesarean delivery in a parturient with extreme short stature due to Noonan Syndrome.
Abstract Number: S3D-6
Abstract Type: Case Report/Case Series
A 33-year-old G3P1 with history of Noonan Syndrome presented for emergency cesarean at 32/5 due to nonreassuring fetal status. History and physical were notable for short stature (132 cm or 4’3.9”), kyphoscoliosis with lumbar spine rod placement, and facial dysmorphism with Mallampati III/IV airway. Torso length appeared unaffected by short stature, whereas limb length was obviously shortened. Two prior non-emergent cesarean deliveries were performed under epidural anesthesia at another institution.
Neuraxial anesthesia was preferred to general because of concern for difficult airway. Due to the urgent nature of the procedure, single-shot intrathecal dosing was chosen rather than an epidural technique with titration. 1.6mL of hyperbaric bupivacaine (0.75%), 150 mcg morphine and 15 mcg fentanyl were given with successful T4 blockade achieved. Estimated blood loss was 1L.
Postoperative course was complicated by hemoperitoneum requiring surgical exploration and transfusion. The patient developed SVT and stress cardiomyopathy with a decrease in EF from normal to 25-30% during ICU convalescence; SVT resolved with beta blockade and EF returned to normal without further treatment. Pseudomonas bacteremia was detected and treated with levofloxacin and metronidazole; the patient was discharged home on postoperative day 13.
Noonan Syndrome is an autosomal dominant disorder marked by multiple system involvement, many of the hallmarks of which are of concern to the anesthesiologist. Short stature, cardiac abnormalities, coagulopathies, lung dysfunction, kyphoscoliosis and difficult airway complicate anesthetic choice, especially in cases of emergent obstetric procedures. Literature on intrathecal dosing in extreme short stature for cesarean procedures is mostly limited to the achondroplastic dwarf population. The small number of case reports involving Noonan Syndrome is skewed towards epidural anesthesia, whereas one report of intrathecal anesthetic for emergency cesarean reports inadequate blockade requiring intravenous supplementation. We present here a case in which full-dose spinal anesthesia achieved the desired level of sensory blockade for surgery with no directly-related adverse outcomes.
The risks and benefits of neuraxial versus general anesthesia must be weighed carefully in cases of extreme short stature given that guidelines for intrathecal dosing have not been established in this population. In cases of syndromic facial dysmorphism neuraxial anesthesia may be preferred for emergency cesarean due to safety concerns; this case illustrates that it is possible to deliver safe and efficacious intrathecal anesthesia to a patient with Noonan Syndrome.
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