Intraoperative Hemodynamic Instability in Concomitant Pectus Excavatum and Scoliosis

Patients with pectus excavatum and scoliosis can present a unique clinical challenge to operative correction. In patients with severe deformities, vascular structures in between the spine and sternum are at risk of compression, leading to hemodynamic collapse during correction of a spine deformity in the setting of unrepaired pectus excavatum. Careful consideration and multidisciplinary coordination should be used to determine the optimal timing, sequence, and operative approach in repair of the anterior and posterior deformities. Patients with pectus excavatum and scoliosis can present a unique clinical challenge to operative correction. In patients with severe deformities, vascular structures in between the spine and sternum are at risk of compression, leading to hemodynamic collapse during correction of a spine deformity in the setting of unrepaired pectus excavatum. Careful consideration and multidisciplinary coordination should be used to determine the optimal timing, sequence, and operative approach in repair of the anterior and posterior deformities. Pectus excavatum develops from abnormal inward growth of costochondral cartilage that leads to a concave chest wall deformity.1Jaroszewski D. Notrica D. McMahon L. Steidley D.E. Deschamps C. Current management of pectus excavatum: a review and update of therapy and treatment recommendations.J Am Board Fam Med. 2010; 23: 230-239https://doi.org/10.3122/jabfm.2010.02.090234Crossref PubMed Scopus (168) Google Scholar The severity of pectus excavatum is commonly quantified by the pectus or Haller index, a ratio between the transverse diameter and the anterior-posterior diameter of the chest wall at the deepest point of the sternum.2Haller J.A. Kramer S.S. Lietman S.A. Use of CT scans in selection of patients for pectus excavatum surgery: a preliminary report.J Pediatr Surg. 1987; 22: 904-906https://doi.org/10.1016/s0022-3468(87)80585-7Abstract Full Text PDF PubMed Scopus (0) Google Scholar A high Haller index has been associated with increased cardiopulmonary dysfunction.3Swanson J.W. Avansino J.R. Phillips G.S. et al.Correlating Haller Index and cardiopulmonary disease in pectus excavatum.Am J Surg. 2012; 203: 660-664https://doi.org/10.1016/j.amjsurg.2011.12.013Abstract Full Text Full Text PDF PubMed Scopus (33) Google Scholar Nearly 20% of all patients with pectus excavatum have scoliosis, and both conditions are associated with Marfan syndrome.4Johnson W.R. Fedor D. Singhal S. Systematic review of surgical treatment techniques for adult and pediatric patients with pectus excavatum.J Cardiothorac Surg. 2014; 9: 25https://doi.org/10.1186/1749-8090-9-25Crossref PubMed Scopus (63) Google Scholar Patients with both of these deformities can present with clinical challenges when the timing and sequence of correction of these conditions are being decided. There have been prior reports of hypotension during scoliosis and pectus excavatum repair; however, these cases described hypotension due to patient position or anesthesia before operative intervention.5Bafus B.T. Chiravuri D. van der Velde M.E. Chu B.I. Hirshl R. Farley F.A. Severe hypotension associated with the prone position in a child with scoliosis and pectus excavatum undergoing posterior spinal fusion.J Spinal Disord Tech. 2008; 21: 451-454https://doi.org/10.1097/BSD.0b013e31815725f2Crossref PubMed Scopus (26) Google Scholar Herein, we report the case of a 15-year-old boy with Marfan syndrome in whom acute hypotension developed during spinal deformity correction. The hypotension immediately resolved with reversal of the spinal correction, suggesting that the severe chest wall deformity contributed to hemodynamic instability. The cause of hypotension was presumed to be compression of the inferior vena cava (IVC), leading to decreased venous return and cardiac preload. A 15-year-old boy initially presented for evaluation of progressive scoliosis associated with Marfan syndrome. Past surgical history was remarkable for a valve-sparing aortic root replacement for aortic root dilation and an implantable cardioverter. The patient had a marfanoid habitus with joint hypermobility, significant kyphoscoliosis, and profound pectus excavatum deformity with a Haller index of 19.2. Clinical photographs illustrate the extent of the deformity (Figure 1). Given the magnitude and progression of the spinal deformity, the patient was scheduled for posterior segmental instrumentation and fusion of the spine from the T2 to L4 vertebral bodies with periapical posterior column osteotomies. Preoperative cardiac evaluation was obtained, and the decision was made to proceed with elective correction. The patient was situated in prone position; exposure of the spinal column was performed without complication, and a contoured rod was positioned in the left-sided pedicle screws that were placed from the T2 to L4 vertebral bodies bilaterally. Shortly after placement of the left-sided rod, there was an acute drop in the systolic blood pressure from 140 mm Hg to 50 mm Hg. The rod was immediately removed, and the patient’s blood pressure normalized. The correction maneuver was reattempted in a slower manner, but acute hypotension occurred again. A third attempt was performed with a more flexible rod, but once again, severe hypotension occurred with resolution after rod removal. The procedure was aborted, and a pediatric surgeon was consulted intraoperatively to ascertain possible anatomic causes of the hemodynamic instability. It was hypothesized that during the spinal correction maneuver, the IVC was compressed between the sternum and the spine. After the patient was medically stabilized, a plan was developed for surgical correction of the pectus deformity in an attempt to prevent compression of the IVC during subsequent spinal deformity correction. Although the patient had a relatively symmetric chest wall deformity, he had a severe Haller index with significant leftward displacement of the heart and a previous thoracotomy with presumed scar tissue in the mediastinal plane. Taking these factors into account, an open Ravitch procedure was favored to the minimally invasive Nuss technique. Preoperative computed tomography of the chest was performed, which demonstrated compression of the IVC between the sternum and T10 vertebral body (Figure 2). The repair was performed 3 weeks from the initial aborted spine surgery. A symmetric chevron incision was made over the depth of the defect at the lower level of the breast tissue. Tissue flaps were elevated to the start of the pectus defect superiorly and to the costal margin inferiorly. Pectoralis muscles were elevated from the chest wall with use of cautery. The cartilaginous portions of the ribs from the top of the sternal depression to the costal margin were resected. The Rultract sternal retractor (Rultract Incorporated) was then attached to the lower sternum. The rectus muscle attachments were then detached, and the retrosternal space was entered below the xiphoid. Special care was taken during this step because the IVC was directly posterior to the sternum on imaging. Several sternal wires placed at the time of sternotomy from the previous aortic surgery were removed. The pleura was swept away, and the sternum was mobilized. An osteotomy was created at the apex of the sternal deformity. The sternum was then flexed forward and stabilized across the osteotomy with polyester-coated multistrand high-molecular-weight polyethylene sutures. The defect appeared to be corrected and remained so in the resting position. The rectus fascia and perichondrium were attached to the sternum with polyglactin sutures, and the rest of the tissues were closed in layers with similar suture material. Intraoperative monitoring showed no signs of neuronal compromise, and the patient was hemodynamically stable throughout the duration of the operation. The postoperative Haller index improved from 19.3 to 4.7 (Figure 3). Two weeks after pectus excavatum repair, correction of the spinal deformity was completed. During placement of the rods and correction of the scoliosis, the patient’s blood pressure remained stable. The patient was extubated immediately after the procedure, and postoperative examination revealed no neurologic deficit. Severe cases of pectus excavatum have led to significant cardiac sequelae.3Swanson J.W. Avansino J.R. Phillips G.S. et al.Correlating Haller Index and cardiopulmonary disease in pectus excavatum.Am J Surg. 2012; 203: 660-664https://doi.org/10.1016/j.amjsurg.2011.12.013Abstract Full Text Full Text PDF PubMed Scopus (33) Google Scholar Although there have been reports of pectus excavatum causing hemodynamic changes during cardiac procedures, few have described pectus deformity producing significant hemodynamic instability during spine deformity correction. In our patient, the anterior-posterior diameter of the chest wall at the deepest portion of the sternum was approximately 1.3 cm, with a Haller index of 19.2. We believe that the severe depression of the sternum caused occlusion of the IVC when the spinal correction maneuver was performed. The temporal relationship between corrective maneuver and hypotension that was replicated 3 different times and reversed with the undoing of the spinal corrective maneuver supports an anatomic cause of the hypotension. This case illustrates the benefit of perioperative multidisciplinary management of patients with both pectus excavatum and significant spinal deformity to anticipate potential hemodynamic complications during manipulation. The authors have no funding sources to disclose.