JBJS | Respiratory Arrest After Anterior Cervical Discectomy and Arthrodesis in a Patient with Down Syndrome. A Case Report and Review of the Literature*

The Journal of Bone & Joint Surgery, Volume 81, Issue 12

Articles | December 01, 1999

Respiratory Arrest After Anterior Cervical Discectomy and Arthrodesis in a Patient with Down Syndrome. A Case Report and Review of the Literature*

SEAN E. MCCANCE, M.D.†; MICHAEL D. SMITH, M.D.‡, MINNEAPOLIS, MINNESOTA

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Investigation performed at Twin Cities Spine Center, Minneapolis

The Journal of Bone & Joint Surgery. 1999; 81:1741-3

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Introduction

Introduction | Case Report | Discussion | References

Down syndrome, or trisomy 21, is a genetic disease that has been associated with many orthopaedic conditions^1,3, including progressive dysplasia of the hip, slipped capital femoral epiphysis, genu valgum, dislocated patellae, planovalgus deformity, polyarticular arthropathy, and clinodactyly. Scoliosis occurs in approximately 50 percent of patients who have Down syndrome³. Abnormalties of the cervical spine have included an increased atlanto-odontoid interval (in approximately 10 percent of patients), odontoid hypoplasia, instability of the occiput and laminar defects at the first cervical level, and precocious arthritis of the midcervical level³.

Patients who have Down syndrome are at increased risk for complications after operations involving the cervical spine, as noted in several reports on posterior cervical arthrodesis for atlantoaxial instability^6,10,11. Reported complications have included an increased risk of infection, wound dehiscence, resorption of bone graft, neurological injury, respiratory problems, and death. The inherent ligamentous laxity and collagen defects in patients who have Down syndrome are thought to contribute to these problems¹⁰. Death secondary to respiratory failure has been reported in association with posterior cervical arthrodesis^9-11 but not, to our knowledge, in association with anterior cervical discectomy and arthrodesis.

We report the occurrence of postoperative pneumomediastinum and bilateral pneumothorax with respiratory arrest in a patient with Down syndrome who had had an anterior cervical discectomy and arthrodesis for the treatment of a large herniated disc at the level between the sixth and seventh cervical vertebrae. This complication is potentially fatal, and both intraoperative and postoperative precautions should be taken in order to minimize any potential sequelae. The susceptibility of these patients to intrinsic pulmonary damage (barotrauma) and respiratory arrest should be recognized in order to avoid a fatal outcome.

*No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article. No funds were received in support of this study.

†Department of Orthopaedic Surgery, Lenox Hill Hospital, 130 East 77th Street, 11th Floor, New York, N.Y. 10021.

‡Twin Cities Spine Center, 913 East 26th Street, Suite 600, Minneapolis, Minnesota 55404-4515. Please address requests for reprints to Dr. Smith.

*No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article. No funds were received in support of this study.

†Department of Orthopaedic Surgery, Lenox Hill Hospital, 130 East 77th Street, 11th Floor, New York, N.Y. 10021.

‡Twin Cities Spine Center, 913 East 26th Street, Suite 600, Minneapolis, Minnesota 55404-4515. Please address requests for reprints to Dr. Smith.

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Fig. 1 Sagittal magnetic resonance image revealing a large disc herniation with narrowing of the canal and displacement of the spinal cord at the level between the sixth and seventh cervical vertebrae.

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Fig. 2 Computed tomography scan of the chest, showing air surrounding the trachea circumferentially (arrow) and extending down into the mediastinum.

Case Report

Introduction | Case Report | Discussion | References

A forty-eight-year-old woman with Down syndrome had an uncomplicated arthrodesis of the first and second cervical vertebrae with transarticular screw fixation for the treatment of atlantoaxial instability in 1993. She did well after the procedure and had an uncomplicated postoperative course. In December 1996, the patient was seen because of the onset of incontinence of the bladder, and catheterization revealed a large postvoid residual. The patient was able to walk with a broad-based gait. Neurological examination revealed brisk reflexes but no localizing findings. Radiographs showed degenerative changes at the fifth and sixth cervical levels, and magnetic resonance imaging scans revealed a large disc herniation with spinal cord compression at the level between the sixth and seventh cervical vertebrae (Fig. 1). The patient had no history of problems related to respiration or aspiration. An anterior cervical discectomy and arthrodesis was performed at the sixth and seventh cervical levels with use of an autogenous tricortical graft from the iliac crest and a plate (Synthes Spine, Paoli, Pennsylvania). Anterior fixation was used because the patient had micrognathia and a history of combativeness, and it was thought that these two factors would make it more difficult for her to comply with the conventional postoperative bracing regimen. During the operation, there were no major difficulties with intubation or ventilation. A cervical collar was applied immediately after the operation.

The patient was extubated in the recovery room and did well until the morning of the first postoperative day, when a partial obstruction of the upper airway developed. Treatment with nebulized epinephrine and intravenous administration of steroids were initiated, and the patient was transferred to the intensive-care unit. On the second postoperative day, she had respiratory arrest and was resuscitated and intubated. Laryngoscopy revealed no substantial edema of the upper airway. A radiograph of the chest showed a pneumothorax on the left side and a widened mediastinum. Computed tomography revealed a bilateral pneumothorax, with the defect on the left being larger than the one on the right, and pneumomediastinum (Fig. 2). On the seventh postoperative day, a chest tube was placed and a tracheostomy was performed. Serial radiographs of the chest revealed gradual resolution of the pneumothoraces and a decrease in the widening of the mediastinum. The chest tube was removed on the ninth postoperative day. On the seventeenth postoperative day, the patient, wearing a cervical collar and with a tracheostomy tube in place, was discharged to a skilled-nursing facility.

Follow-up radiographs, made six weeks after the operation, revealed early incorporation of the bone graft at the levels of the sixth and seventh cervical vertebrae. Physical examination showed no neurological abnormalities. The patient died, four and one-half months after the operation, secondary to another respiratory arrest of unknown cause. An autopsy was not performed.

Discussion

Introduction | Case Report | Discussion | References

Individuals who have Down syndrome are at increased risk for respiratory disease, especially pneumonia^4,8. Respiratory diseases are among the most common causes of the increased mortality seen in this population of patients¹². Mechanical disorders of the pulmonary system, such as tracheal stenosis¹³ and bronchopulmonary dysplasia⁴, also have been reported.

Patients who have Down syndrome are at increased risk for pulmonary complications in the perioperative period^2,9-11,14 and have a tendency toward apnea postoperatively². Previous reports have discussed the occurrence of respiratory complications in these patients after treatment with posterior cervical arthrodesis^5,6,9-11. Patients who have Down syndrome are known to have hypoplastic alveoli with deficient elastic fibers in the alveolar wall, a condition that is thought to increase the risk of intrinsic pulmonary damage following the use of mechanical ventilation¹⁴. Yamaki et al., in an autopsy study of patients with Down syndrome who had had postoperative respiratory failure, found interstitial emphysema and overdistension of peripheral air spaces¹⁴. Those authors concluded that the hypoplastic lung tissue of patients who have Down syndrome is more susceptible to damage resulting from the stress of mechanical ventilation and that interstitial emphysema is a cause of respiratory arrest.

To the best of our knowledge, we are the first to describe the complication of pneumomediastinum and bilateral pneumothorax with respiratory arrest after anterior cervical discectomy and arthrodesis in a patient with Down syndrome. In our patient, respiratory arrest occurred approximately thirty-six hours after a technically uncomplicated procedure. The development of pneumomediastinum and bilateral pneumothorax most likely was related to the increased susceptibility to interstitial lung damage and alveolar rupture that has been noted in association with the use of mechanical ventilation in patients who have Down syndrome¹⁴. The issue regarding the use of mechanical ventilation in this susceptible population is crucial, and special vigilance by the anesthesia team may help to reduce the risk of this complication. Our patient needed an extended hospital stay after the procedure and ultimately died as the result of a second respiratory arrest that occurred after she had been discharged.

In summary, the present report corroborates the well known risks of operations on the cervical spine in patients who have Down syndrome and describes what we believe to be a previously unreported complication of anterior cervical discectomy and arthrodesis. Patients with Down syndrome must be carefully monitored intraoperatively and postoperatively in order to avoid a potentially fatal outcome. As the longevity of these patients increases, involvement of the subaxial spine secondary to Down syndrome is becoming increasingly recognized as a clinically important entity⁷. The treatment of operative complications will continue to pose an important challenge.

References

Introduction | Case Report | Discussion | References

Diamond, L. S.; Lynne, D.; and Sigman, B.: Orthopaedic disorders in patients with Down's syndrome. Orthop. Clin. North America,12: 57-71, 1981.1257 1981

Drummond, D. S.: Congenital anomalies of the pediatric cervical spine. In The Textbook of Spinal Surgery, edited by K. H. Bridwell and R. L. DeWald. Ed. 2, vol. 1, pp. 951-968. Philadelphia, Lippincott-Raven, 1997.

Goldberg, M. J.: Syndromes of orthopaedic importance. In Lovell and Winter's Pediatric Orthopaedics, edited by R. T. Morrissy and S. L. Weinstein. Ed. 4, vol. 1, pp. 255-303. Philadelphia, Lippincott-Raven, 1996.

Joshi, V. V.; Kasznica, J.; Ali Khan, M. A.; Amato, J. J.; and Levine, O. R.: Cystic lung disease in Down's syndrome: a report of two cases. Pediat. Pathol.,5: 79-86, 1986.579 1986

Kobori, M.; Takahashi, H.; and Mikawa, Y.: Atlanto-axial dislocation in Down's syndrome. Report of two cases requiring surgical correction. Spine,11: 195-200, 1986.11195 1986 [PubMed]

Nordt, J. C., and Stauffer, E. S.: Sequelae of atlantoaxial stabilization in two patients with Down's syndrome. Spine,6: 437-440, 1981.6437 1981 [PubMed]

Olive, P. M.; Whitecloud, T. S., III; and Bennett, J. T.: Lower cervical spondylosis and myelopathy in adults with Down's syndrome. Spine,13: 781-784, 1988.13781 1988 [PubMed]

Orlicek, S. L.; Walker, M. S.; and Kuhls, T. L.: Severe mycoplasma pneumonia in young children with Down syndrome. Clin. Pediat.,31: 409-412, 1992.31409 1992 [PubMed]

Pueschel, S. M.; Herndon, J. H.; Gelch, M. M.; Senft, K. E.; Scola, F. H.; and Goldberg, M. J.: Symptomatic atlantoaxial subluxation in persons with Down syndrome. J. Pediat. Orthop.,4: 682-688, 1984.4682 1984

Segal, L. S.; Drummond, D. S.; Zanotti, R. M.; Ecker, M. L.; and Mubarak, S. J.: Complications of posterior arthrodesis of the cervical spine in patients who have Down syndrome. J. Bone and Joint Surg.,73-A: 1547-1554, Dec. 1991.73-A1547 1991

Smith, M. D.; Phillips, W. A.; and Hensinger, R. N.: Fusion of the upper cervical spine in children and adolescents. An analysis of 17 patients. Spine,16: 695-701, 1991.16695 1991 [PubMed]

Thase, M. E.: Longevity and mortality in Down's syndrome. J. Ment. Defic. Res.,26: 177-192, 1982.26177 1982 [PubMed]

Wells, T. R.; Landing, B. H.; Shamszadeh, M.; Thompson, J. W.; Bove, K. E.; and Caron, K. H.: Association of Down syndrome and segmental tracheal stenosis with ring tracheal cartilages: a review of nine cases. Pediat. Pathol.,12: 673-682, 1992.12673 1992

Yamaki, S.; Horiuchi, T.; and Takahashi, T.: Pulmonary changes in congenital heart disease with Down's syndrome: their significance as a cause of postoperative respiratory failure. Thorax,40: 380-386, 1985.40380 1985 [PubMed]

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