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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

Surgical management of pediatric spinal aneurysmal bone cysts: patient series

Benjamin E Flyer, Erik B Vanstrum, Nicholas Chapman, Joseph H Ha, Jacob K Al-Husseini, Jason K Chu, J. Gordon McComb, Susan R Durham, Mark D Krieger, and Peter A Chiarelli

BACKGROUND

Aneurysmal bone cysts (ABCs) are rare, highly vascular osteolytic bone lesions that predominantly affect pediatric populations. This report evaluates the clinicopathological data of pediatric patients with spinal ABCs. The medical records for all patients at Children’s Hospital Los Angeles with biopsy-proven ABCs of the spine between 1998 and 2018 were evaluated.

OBSERVATIONS

Seventeen patients, 6 males and 11 females, were identified. The mean age at surgery was 10.4 years (range, 3.5–20 years). The most common presenting complaint was pain at the lesion site 16/17 (94%), followed by lower-extremity weakness 8/17 (47%). Resection and intralesional curettage were performed in all patients. Three (18%) of 17 patients underwent selective arterial embolization prior to resection. Spinal stability was compromised in 15 of 17 patients (88%), requiring instrumented fusion. Five (29%) of the 17 patients received additional therapy including radiation, calcitonin-methylprednisolone, or phenol. Four (23.5%) of 17 patients experienced a recurrence, and the mean time to recurrence was 15 months. The postoperative follow-up ranged from 6 to 108 months (median, 28 months). Reoperation occurred after an average of 35 months. At the recent follow-up, patients were free of disease.

LESSONS

Gross-total resection by intralesional curettage with case-dependent instrumented spinal fusion for instability remains an effective strategy for managing pediatric spinal ABCs. Long-term follow-up is necessary to detect tumor recurrence.

Open access

Cervical corpectomy in a pediatric patient with chondrodysplasia punctata and C5 dysplasia with spinal cord compression: illustrative case

Nirali P Patel, Mark W Youngblood, Melissa A LoPresti, and Tord D Alden

BACKGROUND

Chondrodysplasia punctata (CDP) describes skeletal dysplasia secondary to a variety of genetic underpinnings characterized by cartilaginous stippling from abnormal calcium deposition during endochondral bone formation. Approximately 20%–38% of patients with CDP have cervical spine abnormalities, resulting in stenosis and cord compression. However, approaches to management differ among patients.

OBSERVATIONS

The authors present an 18-year-old male with a known history of CDP and cervical kyphosis with worsening paresthesias and increased spasticity. Imaging confirmed dysplastic C4 and C5 vertebra with focal kyphosis, bony retropulsion, spinal cord compression, and myelomalacia. To treat the stenosis and deformity, the patient underwent C4 and C5 vertebrectomies with C3 to C6 anterior fusion with resolution of symptoms.

LESSONS

Despite many CDP patients having cervical deformities with spinal cord compression and associated neurological symptoms, there is a paucity of data on surgical management and outcomes. There are only scattered reports, and most authors recommend initial conservative management because of the high risk of operative morbidity and mortality secondary to comorbidities. When surgery is performed, long-term follow-up is recommended because of the high rates of progression of deformity, requiring subsequent operations. The authors hope that their experience adds to the literature describing the surgical management of cervical deformities in these patients.

Open access

Symptomatic spinal arachnoid cyst with spastic diplegia secondary to cerebral palsy: illustrative case

Andrew Guillotte, Abdul-Rahman Alkiswani, Kathryn A Keeler, and Michael D Partington

BACKGROUND

Selective dorsal rhizotomy (SDR) can improve the spastic gait of carefully selected patients with cerebral palsy. Spinal arachnoid cysts are a rare pathology that can also cause spastic gait secondary to spinal cord compression.

OBSERVATIONS

The authors present an interesting case of a child with cerebral palsy and spastic diplegia. He was evaluated by a multidisciplinary team and determined to be a good candidate for SDR. Preoperative evaluation included magnetic resonance imaging (MRI) of the spine, which identified an arachnoid cyst causing spinal cord compression. The cyst was surgically fenestrated, which provided some gait improvement. After recovering from cyst fenestration surgery, the patient underwent SDR providing further gait improvement.

LESSONS

SDR can be beneficial for some patients with spastic diplegia. Most guidelines do not include spinal MRI in the preoperative evaluation for SDR. However, spinal MRI can be beneficial for surgical planning by localizing the level of the conus. It may also identify additional spinal pathology that is contributing to the patient’s spasticity. In rare cases, such as this one, patients may benefit from staged surgery to address structural causes of spastic gait prior to proceeding with SDR.

Open access

Early outcomes in hybrid fixation for idiopathic scoliosis: posterior fusion combined with anterior vertebral body tethering. Patient series

Daniel Cherian, Amer F Samdani, Alexander J Schüpper, Alan A Stein, Zan Naseer, Joshua M Pahys, Emily Nice, and Steven W Hwang

BACKGROUND

Anterior vertebral body tethering (AVBT) and posterior spinal fusion (PSF) are options for patients with idiopathic scoliosis. Combining both procedures in patients with double curves, a procedure in which PSF is performed for the thoracic curve and AVBT for the lumbar curve, provides maximal correction of the thoracic curve with a theoretical maintenance of motion in the lumbar spine.

OBSERVATIONS

The authors retrospectively reviewed 20 skeletally immature patients diagnosed with idiopathic scoliosis at a single institution with an average age of 12.7 ± 1.6 years and who had undergone hybrid treatment with an average follow-up of 8 months. The PSF procedures averaged 276 ± 63 minutes with 442.8 ± 295 mL of blood loss, and the AVBT averaged 275 ± 54 minutes with 118.3 ± 80 mL of blood loss. Following the hybrid correction, the thoracic and lumbar coronal curve angles improved from 67.6° to 21.6° and from 65.2° to 24°, respectively. The three-dimensional kyphosis improved from 3.3° to 24°.

LESSONS

A combined approach of PSF and AVBT is safe and effective for idiopathic scoliosis. This approach combines the gold standard of thoracic fusion with the motion preservation benefits of AVBT in the lumbar spine. This study will continue to refine indications for AVBT.

Open access

Three-stage correction of severe idiopathic scoliosis with limited skeletal traction during a humanitarian surgical mission: illustrative case

J. Manuel Sarmiento, Jordan Fakhoury, Angadh Singh, Cameron Hawk, Khalid Sethi, and Ravi Bains

BACKGROUND

Underprivileged and underserved patients from developing countries often present late with advanced, untreated spinal deformities. We report a three-stage all-posterior approach using limited skeletal traction with Gardner-Wells tongs (GWTs) for the management of severe idiopathic scoliosis during a humanitarian surgical mission trip.

OBSERVATIONS

A 17-year-old high-school female was previously diagnosed with juvenile idiopathic scoliosis (diagnosed at age 8) and progressed to a severe 135° kyphoscoliosis. Procedural stage 1 involved spinal instrumentation and posterior releases via posterior column osteotomies from T3 to L4. She then underwent 7 days of skeletal traction with GWTs in the intensive care unit as stage 2. In stage 3, rod engagement, posterior spinal fusion, and partial T10 vertebral column resection were performed. There were no changes in intraoperative neuromonitoring during either surgery and she woke up neurologically intact after both stages of the surgical procedure.

LESSONS

Skeletal traction with GWTs is a viable alternative to traditional halo-gravity traction in settings with limited resources. Three-stage spinal deformity correction using limited skeletal traction is a feasible and effective approach for managing severe scoliosis during humanitarian surgical mission trips.

Open access

Thoracic pediculectomy for acute spinal cord decompression in high-risk spinal deformity correction: illustrative case

J. Manuel Sarmiento, Christina Rymond, Alondra Concepcion-Gonzalez, Chris Mikhail, Fthimnir M Hassan, and Lawrence G Lenke

BACKGROUND

Neurological complications are higher in patients with severe spinal deformities (Cobb angle >100°). The authors highlight a known technique for thoracic concave apical pedicle resection that is useful for spinal cord decompression in patients with high-risk spinal deformities in the setting of intraoperative neuromonitoring (IONM) changes.

OBSERVATIONS

A 14-year-old female with progressive idiopathic scoliosis presented for evaluation of her clinical deformity. Scoliosis radiographs showed a double major curve pattern comprising a 107° right main thoracic curve and a compensatory 88° left thoracolumbar curve. She underwent 2 weeks of halo-gravity traction that reduced her major thoracic curve to 72°. During thoracic posterior column osteotomies, the authors were alerted to decreases in IONM signals that were not responsive to increases in mean arterial pressure, traction weight reduction, and convex compression maneuvers. The dural surface was tightly draped over the two thoracic apical pedicles of T7 and T8, so emergent pediculectomies were performed at both levels for spinal cord decompression. IONM signals gradually improved and eventually became even better than baseline. The patient woke up without any neurological deficits.

LESSONS

Pediculectomy of the concave apical pedicle(s) should be considered for spinal cord decompression if there are IONM changes during high-risk spinal deformity surgery.

Open access

Pediatric intraspinal arachnoid cyst: successful endoscopic fenestration. Illustrative case

Victoria Jane Horak, Med Jimson D. Jimenez, Melissa A. LoPresti, and Jeffrey S. Raskin

BACKGROUND

Intradural spinal arachnoid cysts (SACs) are a rare cause of spinal cord compression. Treatment is centered on decompression of the spinal cord via laminectomy or laminoplasty followed by resection or fenestration of the cyst. Although laminectomy or laminoplasty access may be needed to achieve the desired result, either procedure can be associated with more extensive surgical dissections and long-term spinal stability concerns, including postsurgical kyphosis.

OBSERVATIONS

The authors present a case of a cervical intradural SAC in a 4-month-old girl presenting with symptomatic compression. The patient was treated by laminotomy and endoscopic fenestration of the SAC with resolution of symptoms and no disease progression 10 months postoperatively, when the patient was 14 months old.

LESSONS

Microsurgical endoscopic fenestration of an intradural SAC can provide a less invasive means of treatment while avoiding the risks associated with more invasive approaches.

Open access

Direct withdrawal of a retained foreign body bisecting the thoracic spinal canal in a neurologically intact pediatric patient: illustrative case

David R. Peters, Trent VanHorn, Brandon Karimian, Benjamin Pruden, Scott D. Wait, Roy T. Daniel, and Constantin Tuleasca

BACKGROUND

Nonmissile penetrating spinal cord injury (NMPSCI) with a retained foreign body (RFB) is rare and usually results in permanent neurological deficits. In extremely rare cases, patients can present without significant neurological deficits despite an RFB that traverses the spinal canal. Given the rarity of these cases, a consensus has not yet been reached on optimal management. In a patient with an RFB and a neurologically normal clinical examination, the risk of open surgical exploration may outweigh the benefit and direct withdrawal may be a better option.

OBSERVATIONS

A 10-year-old female suffered an NMPSCI to the thoracic spine with an RFB that bisected the spinal canal but remained neurologically intact. Direct withdrawal of the RFB was chosen instead of open surgical exploration, leading to an excellent clinical outcome. The literature was reviewed to find other examples of thoracic NMPSCI with RFB and neurologically normal examinations. Management strategies were compared.

LESSONS

For NMPSCI with RFB and without significant neurological deficits, direct withdrawal is a viable and possibly the best treatment option. The use of fast-acting anesthesia without intubation minimizes patient manipulation, speeds up recovery, and allows early assessment of neurological status after removal.

Open access

Management of dynamic cervical kyphosis with dorsal epidural lipomatosis: a Hirayama disease variant? Illustrative case

Adam J. Kundishora, Benjamin C. Reeves, Andres Moreno-De-Luca, Christopher S. Hong, Stephanie M. Robert, Aladine A. Elsamadicy, Dominick Tuason, and Michael L. DiLuna

BACKGROUND

Hirayama disease, a cervical myelopathy characterized most commonly by a self-limiting atrophic weakness of the upper extremities, is a rare entity, scarcely reported in the literature. Diagnosis is made by spinal magnetic resonance imaging (MRI), which typically shows loss of normal cervical lordosis, anterior displacement of the cord during flexion, and a large epidural cervical fat pad. Treatment options include observation or cervical immobilization by collar or surgical decompression and fusion.

OBSERVATIONS

Here, the authors report an unusual case of a Hirayama-like disease in a young White male athlete who presented with rapidly progressive paresthesia in all 4 extremities and no weakness. Imaging showed characteristic findings of Hirayama disease as well as worsened cervical kyphosis and spinal cord compression in cervical neck extension, which has not previously been reported. Two-level anterior cervical discectomy and fusion and posterior spinal fusion improved both cervical kyphosis on extension and symptoms.

LESSONS

Given the disease’s self-limiting nature, and a lack of current reporting, there remains no consensus on how to manage these patients. Such findings presented here demonstrate the potentially heterogeneous MRI findings that can be observed in Hirayama disease and emphasize the utility of aggressive surgical management in young, active patients whereby a cervical collar may not be tolerated.