Autonomic Dysreflexia in an Individual with Syringomyelia in the Setting of Myelomeningocele and Chiari II Malformation: A Case Report

Case Diagnosis: Patient is a 13-year-old female with known syrinx in the setting of spina bifida and Chiari II malformation who is newly diagnosed with autonomic dysreflexia.

Case Description or Program Description: The patient is a 13-year-old female with lumbar myelomeningocele status post detethering procedure, syringomyelia, hydrocephalus, and Chiari type II malformation, complicated by neurogenic bowel and bladder, scoliosis, spasticity and chronic respiratory failure. She is followed by pediatric rehabilitation medicine for functional impairments secondary to spina bifida and syringomyelia. Her complex care pediatrician communicated to the pediatric rehabilitation physician that the patient had described episodes of flushing with her bowel program and strenuous activity, without other associated symptoms. Her pediatric rehabilitation medicine physician diagnosed her with autonomic dysreflexia (AD), provided her with the appropriate education regarding AD, and developed the appropriate care plan for the patient.

Setting: Outpatient pediatric rehabilitation clinic

Assessment/Results: One year after diagnosis of AD, she was admitted to acute care for hypertensive emergency in the setting of a UTI. The admitting team implemented the AD protocol written by her pediatric rehabilitation physician and contacted this physician for additional safety advice regarding treatment of the episodes. Blood pressures improved with antibiotic treatment and she was discharged. Her care plan has been safely implemented for subsequent episodes secondary to gastrointestinal complications and postoperative pain.

Discussion (relevance): Syrinx can be associated with the onset AD with classic precipitating triggers (bladder, bowel, exercise). Providers should suspect and evaluate for syrinx formation or expansion in patients who develop unexplained AD and have predisposing factors (prior trauma, Chiari II, myelomeningocele). Providers should screen patients with known syrinx for AD, provide appropriate education on triggers and symptoms, and derive an appropriate care plan for these patients.

Conclusions: Individuals with myelomeningocele and Chiari malformation are at risk of syrinx formation and should be effectively screened for signs and symptoms of AD in order to implement the appropriate care plan.