SCI
Gabrielle N. Avancena, MD
Resident Physician
NYU Grossman School of Medicine
Long Island City, New York, United States
Cherie Chen, DO
Resident Physician
NYU Grossman School of Medicine
New York, New York, United States
Renat Sukhov, MD
Attending
NYU
New York, New York, United States
Max Lee, MD
Fellow Physician
Hospital for Special Surgery
New York, New York, United States
Gabrielle N. Avancena, MD
NYU Grossman School of Medicine
Long Island City, New York, United States
Cobb syndrome
Case Description: A 12-year-old with a history of T4–5 AVMs s/p coil embolization (2019) presented with acute progressive back pain, bilateral leg weakness, paresthesias, and urinary retention. MRI showed diffuse spinal cord hemorrhage involving T2–T5. As paresthesias advanced to the midtorso and ambulation was lost, the child was transferred to a higher-level care center. Angiogram revealed a thoracic spinal cord aneurysm with numerous AVMs, treated with multiple embolizations. Post-operatively, the child regained right leg strength against gravity, while the left remained paretic (T4 ASIA C). Neurogenic bladder was managed with a Foley, then intermittent catheterization with eventual spontaneous voiding; neurogenic bowel with timed program; and spasticity with PM&R consultation, initiating baclofen, bilateral AFOs, and IRF admission. There, the child progressed from maximal assistance for mobility and ADLs to partial assistance with modified ambulation using an assistive device.
Discussions: Cobb syndrome, or cutaneomeningospinal angiomatosis, is an extremely rare condition consisting of multiple vascular malformations sharing one or multiple spinal segments. Malformations usually form at the thoracic or lumbar levels and can be cutaneous, muscular, osseous, or, often most devastatingly, spinal. Such arteriovenous malformations can cause spinal cord injury by compression, ischemia, or hemorrhage with resultant neurological deficits, including acute paraplegia, sensory loss, spasticity, and sphincter dysfunction. In the inpatient setting, PM&R can be a valuable consultant in managing SCI sequelae to optimize functional recovery through medication and intensive therapies.
Conclusions: SCI in the pediatric population is rare, with an annual incidence of 24 per 1 million in the US, with approximately 50% being atraumatic in etiology. Timely recognition of Cobb syndrome-induced AVM malformations should lead to embolization procedures and careful monitoring to avoid complications such as Foix-Alajouanine disease. Early involvement of physiatry plays an invaluable role in functional recovery and avoidance of complications for these patients, as demonstrated by this case.
