Elsevier

World Neurosurgery

Volume 108, December 2017, Pages 988.e1-988.e5
World Neurosurgery

Case Report
First Case of Autonomic Dysreflexia Following Elective Lower Thoracic Spinal Cord Transection in a Spina Bifida Adult

https://doi.org/10.1016/j.wneu.2017.07.147Get rights and content

Background

Spinal cord transection is a radical but effective treatment for highly selective cases of symptomatic spinal retethering in paraplegic spina bifida patients. Autonomic dysreflexia (AD) is a potentially life-threatening syndrome involving a dysregulated sympathetic discharge reflex commonly seen following cervical and high thoracic spinal cord injury, leading to a disconnect between autonomic pathways above and below the lesion that can lead to severe complications including uncontrolled hypertension, bradycardia, stroke, and potentially death. Herein we present a case in which a paraplegic spina bifida patient presenting with symptomatic spinal retethering experienced autonomic dysreflexia following an elective spinal cord transection.

Case Description

A 51-year-old male with a history of complex spina bifida presented with an active cerebrospinal fluid leak. Physical examination revealed a thin covering of abnormal epidermis over the large placode. Magnetic resonance imaging revealed a large myelomeningocele defect with posterior element defects spanning from L2 to the sacrum with evidence of tethering. The patient underwent an intradural transection of the spinal cord with a “blind-pouch” closure of the dura at the level of T12/L1. Postoperatively, the patient developed intermittent episodes of hypertension, bradycardia, headaches, altered mental status, severe perspiration, and red flushing of the upper torso, face, and arms. The diagnosis of AD was made clinically and managed with a positive response to a combination of beta- and alpha-blockade along with patient education on avoidance of common AD triggers. At 5-year follow-up the patient has continued to do well on medication.

Conclusion

This case highlights a potential major side effect from elective transection of the spinal cord. If unrecognized and untreated, AD can cause significant distress and morbidity. We hope this first case report serves to supplement existing data and aid in future surgical and medical decision-making.

Introduction

Autonomic dysreflexia (AD) is an acute syndrome of excessive, uncontrolled sympathetic output with potentially serious consequences in patients who have spinal cord injury (SCI). Typically; the syndrome is characterized by lesions at or above the level of sympathetic splanchnic outflow (T6 level).1

AD is the result of an exaggerated autonomic response to pain or other triggers below the level of the SCI, resulting in an increase in blood pressure along with other compensatory symptoms. The most common symptoms of AD are sweating, pounding headache, tingling sensation on the face and neck, blotchy skin around the neck, and piloerection (goose bumps). Not all the symptoms always appear at once, and their severity may vary. AD is considered a potentially life-threatening condition and a medical emergency requiring immediate attention; untreated and extreme cases may lead to stroke and/or death.2, 3

Few procedures are as inherently displeasing to neurosurgeons as the deliberate transection of the spinal cord. Nevertheless, in certain highly selective cases, the surgical division of distally nonfunctional spinal cord elements as a measure to protect proximal functional segments can prove effective.3 Currently, there are limited data on various aspects of the procedure, including postoperative complications, despite its being considered the most common standard for the treatment of symptomatic repetitively tethered cord in spina bifida patients with paraplegia.

Although AD is discussed in the literature as a possible complication of surgical spinal cord transection, it has yet to be reported in the literature. To the best of our knowledge, this case will be the first report describing AD as a possible complication following therapeutic spinal cord transection.1 We hope this case can serve to supplement the present data and aid in the differential diagnosis and surgical and medical decision making of AD resulting from intentional spinal cord transection in spina bifida patients.

Section snippets

Case Description

A 51-year-old male with history of complex spina bifida presented with an active cerebrospinal fluid (CSF) leakage from the lumbar wound and severe intractable back pain. He was born with a complex and large myelomeningocele that was left unrepaired with an initial intention of management via palliative care. Over the years, a layer of abnormal epithelium formed over the placode, and he did relatively well for 50 years without intervention until developing the aforementioned symptoms and sought

Discussion

AD is a potentially serious consequence in patients who have SCI with lesions at or above the level of sympathetic outflow of the most rostral of the thoracic splanchnic nerves4 (Figure 2). The most common symptoms of AD are related to the interruption of descending inhibition of sympathetic motor activity and the consequent development of hyperresponsiveness of peripheral receptors. Such symptoms and signs include sweating; pounding headaches; tingling sensations of the face and neck; blotchy,

Conclusion

Elective transection of the spinal cord is an extreme procedure reserved for a highly selective set of indications. Appropriately, there are limited data on the topic. To the best of our knowledge, this is the first case of AD following elective transection of the spinal cord at the thoracolumbar junction. The case highlights AD as a potential major side effect from such an operation, as well as its successful management. If unrecognized and untreated, AD can cause significant distress and

Acknowledgments

We would like to thank Cyrus Kafai, M.S., and Alex Pham, B.S., for their support writing this manuscript.

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Conflict of interest statement: The authors have no financial interest to declare in relation to the content of this article. Material support was provided by Ochsner Medical Center.

Juanita Garces and Mansour Mathkour are the first authors and contributed equally.

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