Split cord malformation and tethered cord syndrome: case series with long-term follow-up and literature review
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ORIGINAL ARTICLE
Split cord malformation and tethered cord syndrome: case series with long-term follow-up and literature review Andrew J. Kobets 1
&
Jeffrey Oliver 1 & Alan Cohen 1 & George I. Jallo 1,2 & Mari L. Groves 1
Received: 19 May 2020 / Accepted: 17 November 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract Purpose To date, the description of the natural course of concurrent tethered cord syndrome with a low-lying conus medullaris and split cord malformation is lacking in the literature. We report a cohort of adult and pediatric patients with concurrent malformations and long-term follow-up. Methods Patients with concurrent diagnoses of split cord malformation and tethered cord (radiographic evidence supporting clinical symptomatology) were identified between 2000 and 2020. Patients without sufficient documentation or at least 6-month follow-up were excluded. Results Nine patients were identified with an average of 8.9 years follow-up (range 2–31 years). The most common symptoms were radiating leg pain and lower extremity paresthesias, occurring in 44% of patients; and bladder/bowel dysfunction, worsening scoliosis, and acute motor deterioration were less common. Two patients were successfully treated conservatively for mild leg pain and paresthesias. For those who underwent surgery, all experienced symptomatic relief upon first follow-up. Two had late symptomatic recurrence; one 4 and 8 years after initial surgery; and the other, 11, 26, and 31 years after initial surgery. Conclusion The rarity of concurrent split cord and tethered cord syndrome with a low-lying conus makes management difficult to formulate. This series supplements our knowledge of the long-term outcomes and lessons learned from the management of these patients. Approximately 25% of patients were managed conservatively and had symptomatic improvement. For surgically managed patients, with intractable pain or worsening neurological function, symptoms can still recur over a decade after intervention. Reoperation, however, can still be beneficial, can provide years of relief, and should be considered. Keywords Diastematomyelia . Split cord . Tethered cord . Lipoma . Low-lying conus . Fatty filum
Abbreviation SBNS Spina Bifida Neurological Scale
Introduction Diastematomyelia, or split cord malformation, is a developmental spinal dysraphism in which a bony, membranous, or fibrous septum inhibits the fusion of a single spinal cord, thus * Andrew J. Kobets [email protected] 1
Department of Neurosurgery, Division of Pediatric Neurosurgery, Johns Hopkins School of Medicine, 600 N Wolfe Street, Phipps Building, 5th Floor, Baltimore, MD, USA
2
Department of Neurosurgery, Division of Pediatric Neurosurgery, All Children’s Hospital, St. Petersburg, FL, USA
resulting in the persistence of two hemicords bound by either a single or duplicated dural sleeve [1]. Pang and others have implicated the presence of an anomalous neuroenteric canal in the development of a split cord, in which this persistent endomesenchymal tra
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