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OUR OBSTETRIC ANESTHETIC EXPERIENCE IN A PARTURIENT WITH ELLIS VAN CREVELD SYNDROME
Abstract Number: F-69
Abstract Type: Case Report/Case Series
INTRODUCTION: Ellis-van Creveld syndrome (EVC) is an inherited disorder of bone growth that occurs in 1 in 60,000 to 1 in 200,000 newborns, resulting in dwarfism. Individuals with this syndrome have short forearms and legs, polydactyly, a narrow chest with short ribs and associated cardiac defects. They are not known to have the airway or spine abnormalities seen in achondroplasia. We describe the anesthetic management of a parturient with this syndrome with an original plan for vaginal delivery with neuraxial labor analgesia but later changed to C-section under GA.
CASE: A 32 year-old patient G1P0 with EVC and with an IVF pregnancy was admitted for induction of labor at 36 weeks gestation. She had been diagnosed with preeclampsia at 33 weeks gestation and now complained of a headache of 2 days duration. Her current medical history was significant for moderate mitral regurgitation and mild pulmonary stenosis. She was 4’9” tall and weighed 158 lb. She had the facial features of dwarfism with shortened extremities. Her airway and lumbar spine exam were unremarkable.
Induction of labor was initiated with vaginal misoprostol administration with subsequent request for neuraxial labor analgesia. The lumbar epidural space was easily identified; however we experienced difficulty in threading the epidural catheter. We were able to insert the catheter at another level after two attempts using CSE. A 0.2% ropivacaine infusion was started at a low dose due to initial hypotension, with relief of pain. The patient had infrequent contractions thereafter and reported mild discomfort during her contractions, which was attributed to the low dose of infusion. The infusion rate was increased minimally with no further increase due to concern for hypotension. After being in labor for more than 24 hours, she was started on oxytocin with subsequent arrest of descent. A decision was made to perform a C- section. Local anesthetic was given epidurally. She experienced a one-sided dense anesthetic block discovered on exam. Surgery was performed under GA uneventfully.
DISCUSSION: EVC and achondroplasia are varieties of dwarfism. Failure to establish sensory blockade after spinal anesthesia, in a pregnant patient with EVC has been described (1).We had unexplained unilateral blockade even though the catheter location appeared optimal with about 4 cm in the epidural space. The unpredictable behavior of local anesthetics with neuraxial blocks has been described with achondroplasia (2). We experienced difficulty in threading the epidural catheter, which might be explained by an altered anatomy secondary to her narrow chest. Our patient was scheduled for vaginal delivery, as she was perhaps not thought to have significant cephalopelvic disproportion. We recommend that an elective C-section might be preferable in these patients.
1. Rev Esp Anestesiol Reanim. 2010 Oct; 57(8):528-31
2. Br J Anaesth 1998 Oct; 81(4):619-621