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Anesthesia for a cesarean section for a diastrophic dwarf
Abstract Number: T-72
Abstract Type: Case Report/Case Series
Introduction: Diastrophic Dysplasia (DTD) is a rare autosomal recessive disorder characterized by dwarfism, limb deformities, cervical kyphosis, and scoliosis.1 Pregnancy in patients with DTD is not well described; only 3 case reports have been published in the literature.1-3 Anesthetic challenges include respiratory compromise during pregnancy, difficult IV access, difficult airway, and challenges with placement and dosing of neuraxial anesthesia (NA).
Case: A 34 year old 145 cm G2P1 with DTD presented for anesthetic consultation at 30 weeks gestation. Her past medical history included concern for malignant hyperthermia (MH) and failed intubation at the time of her prior cesarean delivery (CD). At the time of consultation, the patient refused NA. Her airway exam revealed an immobile cervical spine and mallampati 4 view with a large tongue and small mouth opening. The plan was made for an elective CD at >37 weeks under general anesthesia (GA) with an awake fiberoptic intubation (FOI). However, she presented with labile blood pressures at 37 weeks and the decision was made to proceed with CD prior to the scheduled date. The patient was admitted in the evening hours and an 18 gauge IV was placed. ENT was consulted and performed a bedside nasal flexible laryngoscopy revealing normal laryngeal anatomy. The next morning she was premedicated with midazolam and glycopyrrolate. She was taken to the OR where the OB team as well as the ENT team (with a rigid bronchoscope and a tracheostomy kit) were present. She was topicalized with 4% nebulized lidocaine and a remifentanyl infusion was begun. A Williams airway, lubricated with 2% lidocaine, was inserted and a flexible pediatric bronchoscope was used to secure a glottic view. She was intubated with a 6.0 ETT and GA was induced. Anesthesia was maintained with a non-triggering anesthetic. A male fetus was delivered with Apgars of 2,1, 7. At the conclusion of surgery, she was extubated. Her postoperative course was uncomplicated and she was discharged on postpartum day 4.
Discussion: A patient with DTD presents complex anesthetic challenges for both GA and neuraxial techniques. Prior case reports of CD in patients with DTD reveal two cases utilizing a similar anesthetic technique described above with FOI and GA as well as a single case report under MRI guided epidural anesthesia.1-3 Optimal care requires careful consideration of the patient’s specific risk factors and a multidisciplinary approach. Obtaining an early anesthesia and ENT consult allowed us to formulate a safe anesthetic plan. In our case, the patient refused NA and with a history of difficult airway, an awake FOI was deemed to be appropriate. Regardless of the anesthetic technique selected, a thorough preoperative assessment, multidisciplinary team approach, and ongoing communication is crucial to safe and appropriate peripartum management.
1. Int J Obstet Anesth 2007;16:145-8
2. Lancet 2001;358:1778
3. A A Case Rep 2015;5(1):6-8