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Anesthetic Management of Primigravidad with Arthrogryposis Congenita (AMC) for Caesarian Section
Abstract Number: SA-63
Abstract Type: Case Report/Case Series
Arthrogryposis Multiplex Congenita (AMC) is a multifactorial genetic condition resulting in multiple joint contractures evident from birth. Our patient is a 34 yr old G1P0 female (152cm, 63.6kg) with AMC and severe scoliosis presenting with contractions at 38 wks gestation, one week prior to a planed caesarian section for fetal macrosomia and unfavorable pelvic anatomy. Her medical history includes multiple orthopedic procedures and extensive spinal fusion from T3-S2 for severe scoliosis as a child. Patient has been wheelchair bound since age of two. She had been on lovenox for thromboembolic prophylaxis during pregnancy and was bridged to subcutaneous heparin (5000 units) BID two weeks prior to presentation. She also has a history of hypertension with normal pressures during pregnancy and T wave abnormalities seen on EKG with normal follow-up echocardiogram.
Patient was previously seen in anesthesia consult clinic. Her airway exam showed a Mallampati class 2 airway with full range of cervical motion, full dentition, adequate mouth opening and >3 FB thyromental distance. Due to patient's discomfort, spinal anesthesia was attempted with patient in lateral decubitus position. Tactile feedback from dural puncture was not obtained. After multiple attempts, decision was made to proceed with general anesthesia. Glidescope 3 was used with grade 1 view obtained. Initially we had trouble passing a size 6 ETT until cricoid pressure was briefly released. Patient's pharyngeal anatomy appears slightly distorted: her epiglottis appeared to be more omega shaped and tissues around arytenoids appeared contracted. Patient tolerated the procedure without any issues maintained on 0.5 MAC volatile agents and 50% NO. She was extubated without any issues.
There are several perioperative and intraoperative concerns for this patient including mechanical respiratory compromise, difficult intubation, inability to obtain intravenous access, coagulopathy due to immobility and challenge of spinal/neuraxial technique. Respiratory function is affected due to advancing gestation in normal women due to increased intraabdominal girth, leading to a reduction in functional residual capacity which may be more pronounced in women in AMC due to contractures and spinal deformity. The risk of DVT in this patient was greater given her poor mobility in the setting of hypercoagulable state of pregnancy. Most importantly neuraxial techniques in such a patient would naturally be challenging given her underlying contractures, spinal deformities and surgical adhesions. The possibility of a failed spinal due to inability to access intrathecal space and the possibility of a patchy epidural due to inadequate spread from scarring were discussed with patients in preop clinic.