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Successful Spinal Anesthesia for Cesarean Section in a Parturient with Spondyloepiphyseal Dysplasia Congenita
Abstract Number: RF7B10-217
Abstract Type: Case Report Case Series
Introduction:Patients with short limb dwarfism are known to be at a higher risk for anesthetic complications.We report the successful use of spinal anesthesia for cesarean section in a patient with Spondyloepiphyseal Dysplasia Congenita (SEDc),a rare type of dwarfism caused by a mutation in the COL2A1 gene.
Case Report: A 31yo G3P0110 at 31+3 weeks presented with palpitations, SOB, vomiting and diarrhea.History remarkable for SEDc and anxiety, treated with venlafaxine. Surgical history: neck fusion, ankle surgery, R hip replacement, cleft palate repair,10 skeletal surgeries for bone lengthening. Two prior pregnancies had resulted in terminations due to lethal congenital anomalies. EKG showed sinus tachycardia. Lab tests, including thyroid studies, PFTS and Echo were unremarkable. CTA was negative for PE. The tachycardia was attributed to dehydration, anxiety, and SNRI use. The decision was made for cesarean section due to the HR and concern for maternal compromise. PE:Ht 132cm,Wt 65 kg, BMI 37.6, HR 97-152,BP 142/95. She had typical features of disproportionate dwarfism with short limbs, lumbar lordosis, short trunk,and thoracic kyphoscoliosis; limited neck ROM, Mallampati IV. After preload with 1000 ml. LR, spinal anesthesia was easily administered in sitting position at L3-4 using 25G Pencan needle,8.25 mg of 0.75% hyperbaric bupivacaine with 10ug fentanyl and 0.15 mg morphine. T4 level achieved. Male child was delivered, Apgars 7/8.Surgery proceeded uneventfully. EBL 800ml. Patient continued to have tachycardia post-op in the 120s and was started on metoprolol prior to discharge.
Discussion: SEDc is a rare genetic entity characterized by a disruption in Type II collagen production and delayed ossification of the epiphyses resulting in disproportionate dwarfism. It may be inherited as an autosomal dominant or resulting from a de novo mutation; incidence:1/100,000 births. Findings include kyphoscoliosis with possible cord compression, increased lumbar lordosis, narrowed intervertebral disc spaces, platyspondyly with posterior wedging of vertebral bodies, and occasionally, laryngotracheal stenosis. Due to a hypoplastic odontoid, patients may develop atlantoaxial instability and cervical vertebral body changes. Restrictive lung disease may be present due to kyphoscoliosis and may be associated with chronic hypoxia and pulmonary HTN. C/section is indicated due to a narrow pelvis. Given these features, both GA and neuraxial techniques pose unique challenges. GA,spinal,CSE and epidural anesthesia have all been described(1,2). An SED patient information site specifically contraindicates spinal anesthesia and recommends GA(3).Despite this contention,we achieved successful, uncomplicated spinal anesthesia with a bupivacaine dose based on a recommendation of 0.06 mg/cm(4). A continuous technique might be advantageous due to unpredictable spread.
1. IJOA 2002;11;185-9
2. BJA 2001;86:133-4
4. Minerva Anestesiol 67:573-7