Type I split cord malformation and tethered cord syndrome in an adult patient: A case report and literature review

Abstract

Background: In a split cord malformation (SCM), the spinal cord is divided longitudinally into two distinct hemicords that later rejoin. This can result in a tethered cord syndrome (TCS). Rarely, TCS secondary to SCM presents in adulthood. Here, we present an adult female with Type I SCM resulting in TCS and a review of literature. Case Description: A 57-year-old female with a history of spina bifida occulta presented with a 2-year history of worsening back and left leg pain, difficulty with ambulation, and intermittent urinary incontinence; she had not responded to conservative therapy. Magnetic resonance imaging (MRI) revealed a tethered cord secondary to lumbar type I SCM. The patient underwent an L1–S1 laminectomy for resection of the bony septum with cord detethering. At 2-month follow-up, the patient had improvement in her motor symptoms and less pain. In literature, 25 cases of adult-onset surgically managed SCM with TCS were identified (between 1936 and 2018). Patients averaged 37 years of age at the time of diagnosis, and 56% were female. Conclusion: TCS can present secondary to SCM in adulthood and is characterized predominantly by back and leg pain.

Classification of and individual treatment strategies for complex tethered cord syndrome

“…C-TCS is called “complex” because these tether-causing factors increase the difficulty of release and decompression, with the filum terminal, arachnoid, bone, and diastomyelia types showing greater difficulty of release and the lipoma and cele types showing greater difficulty of decompression ( 24 – 26 ). Since complete release and full decompression are the primary objectives of tethered cord release ( 27 ), an understanding of the tether-causing factors responsible for C-TCS is essential ( 28 , 29 ). The main factor causing a tethered cord of the filum filament type is the filament, while the secondary factors include lipomas and lipomyelomeningocele/meningocele ( 30 , 31 ).…”

Incidentally discovered type 1 split cord malformation in an adult patient

“…SCM can cause variety of symptoms with back pain being the most common [5] . SCM is associated with tethered cord syndrome where it accounts for 10%-38% of adult tethered cord diagnoses [8] .…”

“…Most SCMs are diagnosed in childhood and present with lumbago, lumbosciatica, or perineal dysesthesia often after minor trauma [4] . The clinical manifestations of SCMs in adults include back pain, leg pain, intermittent incontinence, and cutaneous stigmata [5] . In rare cases, adult SCM can be discovered incidentally on neuroimaging [6 , 7] .…”