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Open access

The complex treatment paradigms for concomitant tethered cord and scoliosis: illustrative case

Rose Fluss, Riana Lo Bu, Andrew J Kobets, and Jaime A Gomez

BACKGROUND

Scoliosis associated with tethered cord syndrome is one of the most challenging spinal deformities to manage. Multiple surgical approaches have been developed, including traditional staged and concomitant procedures, spine-shortening osteotomies, and individual vertebral column resections.

OBSERVATIONS

A 10-year-old female presented with congenital kyphoscoliosis with worsening curve progression, tethered spinal cord, and a history of enuresis. The scoliosis had progressed to a 26° coronal curve and 55° thoracolumbar kyphosis. Preoperative magnetic resonance imaging of the spine revealed a tethered cord between the levels of L3–4 and a large kyphotic deformity at L1. The patient underwent laminectomy, during which intraoperative motor signals were lost. A planned hemivertebrectomy at L1 was performed prior to an L4 laminectomy, untethering of the filum terminale, and posterior spinal fusion from T11 to L2. After surgery, the patient experienced transient lower-extremity weakness, with her neurological function improving from baseline over the next 2 months. Ultimately, the goal of this surgery was to halt the progressive decline in motor function, which was successfully achieved.

LESSONS

Much remains to be learned about the treatment of this complicated disease, especially in the setting of concomitant scoliosis. This case serves to exemplify the complex treatment paradigms that exist when attempting to manage this clinical syndrome and that more remains to be learned.

Open access

Techniques for restoring optimal spinal biomechanics to alleviate symptoms in Bertolotti syndrome: illustrative case

Nolan J Brown, Zach Pennington, Hania Shahin, Oanh T Nguyen, and Martin H Pham

BACKGROUND

Lumbosacral transitional vertebrae (LSTVs) are congenital anomalies that occur in the spinal segments of L5–S1. These vertebrae result from sacralization of the lowermost lumbar segment or lumbarization of the uppermost sacral segment. When the lowest lumbar vertebra fuses or forms a false joint with the sacrum (pseudoarticulation), it can cause pain and manifest clinically as Bertolotti syndrome.

OBSERVATIONS

A 36-year-old female presented with severe right-sided low-back pain. Computed tomography was unremarkable except for a right-sided Castellvi type IIA LSTV. The pain proved refractory to physical therapy and lumbar epidural spinal injections, but targeted steroid and bupivacaine injection of the pseudoarticulation led to 2 weeks of complete pain relief. She subsequently underwent minimally invasive resection of the pseudoarticulation, with immediate improvement in her low-back pain. The patient continued to be pain free at the 3-year follow-up.

LESSONS

LSTVs alter the biomechanics of the lumbosacral spine, which can lead to medically refractory mechanical pain requiring surgical intervention. Select patients with Bertolotti syndrome can benefit from operative management, including resection, fusion, or decompression of the pathologic joint.

Open access

Coexisting filum terminale arteriovenous fistula and filum terminale lipoma treated with single-stage surgery: illustrative case

Hiroki Yamazaki, Tomohiko Ozaki, Tomoki Kidani, Yosuke Fujimi, Masahiro Nonaka, Masao Umegaki, Chisato Yokota, and Toshiyuki Fujinaka

BACKGROUND

Both filum terminale arteriovenous fistulas (FTAVFs) and filum terminale lipomas (FTLs) are rare. Because of this rarity, there is a paucity of data regarding concomitant FTAVF and FTL, and the optimal treatment remains to be defined. The authors describe a patient with coexisting FTAVF and FTL treated with single-stage surgery.

OBSERVATIONS

A man in his 70s was referred to the authors’ department because of a suspected spinal vascular malformation seen on magnetic resonance imaging that was performed to investigate lower limb weakness, intermittent claudication, and urinary incontinence. Previous imaging had shown a terminal lipoma with an internal flow void. Computed tomography angiography and digital subtraction angiography revealed an FTAVF, the feeder being a lateral sacral artery. The patient was treated surgically with curative intent. The FTL and tethered cord that had been identified by imaging were treated in the same procedure. Postoperative digital subtraction angiography showed absence of the abnormal vessels. The patient was discharged home on the 19th postoperative day.

LESSONS

When considering treatment, it is important to determine whether symptoms are attributable mainly to FTL, tethered cord, or FTAVF. One-stage treatment is useful because it eliminates both the FTAVF and the factors that led to its development.

Open access

Surgical management of high-grade lumbar spondylolisthesis associated with Hajdu-Cheney syndrome: illustrative case

Davaine J. Ndongo Sonfack, David Bergeron, Zhi Wang, Ghassan Boubez, Daniel Shedid, and Sung-Joo Yuh

BACKGROUND

Hajdu-Cheney syndrome (HCS) is a rare connective tissue disorder characterized by severe bone demineralization. In the spine, it is associated with the early onset of severe osteoporosis and can cause spondylolisthesis. Spinal instrumentation in the setting of severe osteoporosis is challenging because of poor resistance of vertebrae to biomechanical stress.

OBSERVATIONS

A 59-year-old woman with known idiopathic HCS presented with a grade 4 L5-S1 spondylolisthesis and right L5 pedicle fracture associated with a left L5 pars fracture, causing a progressive L5 radiculopathy that was worse on the left side than the right side and bilateral foot drop. The authors performed decompressive lumbar surgery, which included a complete L5 laminectomy and resection of the left L5 pedicle. This was followed by multilevel lumbosacral instrumentation using cement-augmented fenestrated pedicle screws as well as transdiscal sacral screws and bilateral alar-iliac fixation. Postoperatively, the radicular pain resolved, and the left foot drop partially recovered.

LESSONS

Stabilization of high-grade spondylolisthesis in the setting of bone demineralization disorders is challenging. The use of different instrumentation techniques is important because it increases biomechanical stability of the overall instrumentation construct.