Abstract
We report a patient with Pena-Shokeir syndrome with severe scoliosis corrected with spinal rod placement from T1-L5, with a known history of malignant hyperthermia presenting for cesarean section. A 21 year old female, 132 cm tall and 35.5 Kg presenting for a primary C-section at 38 weeks with sudden onset of dyspnea, tachypnea, palpitation, and chest pain. A spiral CT demonstrated no pulmonary embolus or any other pathology and clinical symptoms resolved with oxygen and albuterol therapy. The patient had a history of Pena-Shokeir syndrome which included severe myopathy and extreme lower extremity weakness since birth. The patient had contractures of all her major joints, involving both the upper and lower extremities. Additionally she also had cleft palate and severe congenital scoliosis significantly limiting her mobility. Past surgical history included cleft palate repair in childhood, and correction of scoliosis with spinal rod placement between T1- L5 vertebral levels, 7 years earlier, with a very difficult peri-operative course complicated by malignant hyperthermia intra-operatively and severe respiratory failure requiring prolonged mechanical ventilation and tracheostomy formation. A spinal anesthetic was administered (7.5mg hyperbaric bupivacaine only) at the L5-S1 level on the first attempt with positive CSF. A surgical anesthetic level (T6) was obtained and an uncomplicated C-section was performed. This is the first case report of a parturient with Pena- Shokeir syndrome surviving to 21 years of age and then delivering a normal baby by Caesarean section. Despite numerous anesthetic and medical concerns, she received a successful spinal anesthetic and had an uneventful delivery. Early consultation with obstetric and anesthesia team during the antepartum period aided in developing an appropriate clinical plan for this patient.
Highlights
Regional anesthesia is the preferred method for operative delivery in parturients, certain conditions can make its’ utilization challenging
Regional anesthesia is difficult in patients with surgically corrected scoliosis with spinal rod placement, usually having an increased risk for both complications as well as failure to obtain satisfactory analgesia [1]
We report a patient with Pena-Shokeir syndrome [5,6] with severe scoliosis corrected with spinal rod placement from T1-L5, with a known history of malignant hyperthermia presenting for elective C-section, who had an uncomplicated C-section under spinal anesthesia
Summary
Regional anesthesia is the preferred method for operative delivery in parturients, certain conditions can make its’ utilization challenging. We report a patient with Pena-Shokeir syndrome [5,6] with severe scoliosis corrected with spinal rod placement from T1-L5, with a known history of malignant hyperthermia presenting for elective C-section, who had an uncomplicated C-section under spinal anesthesia. Muscle biopsy had been performed which had ruled out other forms of congenital myotonic muscular dystrophy and spinal muscular atrophy Her past surgical history consisted of a previous cleft palate repair in childhood, and correction of scoliosis with spinal rod placement between T1-L5 vertebral levels, 7 year earlier, this latter surgery being associated with malignant hyperthermia and prolonged mechanical ventilation postoperatively requiring tracheostomy. A number of clinical problems relative to anesthetic management were apparent following this preoperative evaluation These included a positive history of malignant hyperthermia, the presence of a congenital myopathy, a potentially difficult airway and a. The postoperative period was uneventful, a TEE being performed given clinical history suggestive of pulmonary embolus at the time of presentation, which demonstrated nil of note and the patient was discharged home on the 4th post-operative day
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