P019: COOLEY'S ANEMIA IN CAUDA EQUINA - PERIOPERATIVE CONSIDERATIONS OF BETA THALASSEMIA MAJOR
Harry E Kranichfeld, DO, MSc; Marc Levine, MD; Gerald Rosen, MD
Mount Sinai Medical Center
Introduction / Background: Beta Thalassemia Major (Cooley's Anemia) is a hereditary hemoglobinopathy characterized by severe and chronic anemia. Patients require serial blood transfusions and other systemic therapies which can precipitate iron overload and subsequent organ dysfunction including cardiomyopathies, hepatic derangements, endocrinopathies. Anesthesiologists may be tasked with managing the thalassemic patient who present for elective or emergent surgeries such as correction of facial deformities, therapeutic splenectomy, or traumatic bony fractures.
Here, we describe the anesthetic and peri-operative considerations as they pertain to a 35-year old Pakistani male with history of Cooley's Anemia, who sustained an acute L3 burst fracture and resultant cauda equina syndrome following a tonic-clonic seizure. Interestingly, this patient had been participating in hyperbaric oxygen therapy for treatment of bilateral lower extremity leg ulcerations at the time of his witnessed seizure. On presentation, he was in excrutiating low back pain, with progressively worsening weakness and paresthesias of the lower extremities and concomitant urine retention raising concern for acute cauda equina syndrome. These concerns were confirmed upon CT imaging which demonstrated severe thecal sac stenosis at the levels of the L2-L3 disc space. He was emergently taken to the neurosurgical suite for stabilization, which demanded careful blood component optimizations, positioning strategies, and hemodynamic precautions to ensure a reassuring clinical outcome.
Methods: Preoperative evaluation included focused review of patient's primary hematology records. This review revealed a complex daily medication regimen including;
- synthetic androgenic steroid
- corticosteroid
- Factor Xa inhibitor
- histamine blockers
- iron-chelaters
- Immunomodulators (hydroxyurea, B-cell therapy)
- Folic acid
- Recently being started on tramadol and antibiotic therapy for treatment of worsening lower extremity venous insufficiency wounds.
Bedside cardiac assesment with 2D echocardiography showed no significant cardiomyopathy or pulmonary hypertension. Airway exam revealed no overt maxillary hypertrophy or other indications for difficult airway access.
Results: General anesthesia was rapidly induced with lidocaine 100mg, propofol 100mg, fentanyl 100mcg, and succinylcholine 100mg. Manual in-line stabilization of the cervical spine was provided, and Grade 1 view of the vocal cords was obtained via GlideScope. The left radial artery was catheterized for beat-to-beat hemodynamic monitoring. Two large bore (16G) peripheral IVs were obtained. Careful attention was payed to the pretibial lower extremities bilaterally, where acutely friable on chronic-appearing lesions were scattered. Prone positioning involved close attention to padding of bony prominences and pressure points, with focus on further stabilization of thoracolumbosacral spine. To facilitate SSEP and MEP monitoring, general anesthesia was maintened with total intravenous anesthesia (TIVA); propofol 100-120mcg/kg/min and remifentantil 0.15-0.25mcg/kg/min. A loading dose of levetiracetam was administered at the request of neurosurgical team.
Despite reversal with 4-factor PCC in the emergency department, significant bleeding was noted intraoperatively. Aminocaproic acid drip was initiated at 1g/hour.
Conclusion: This case highlights the sucecssful anaesthetic management of emergent spine surgery in a patient with Cooley's Anemia. Key considerations included peri-operative management of hemoglobin and coagulation factors, vigilant airway and hemodynamic monitoring, prone positioning precautions, and surgical ICU care. Prompt surgical decompression facilitated neurologic recovery.