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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

Spinal epidural lipomatosis in a pediatric patient with a malignant brain tumor: illustrative case

Reed Berlet, Daphne Li, and John Ruge

BACKGROUND

Spinal epidural lipomatosis (SEL) in pediatric patients with concomitant malignant brain neoplasms is rare and can present with rapid deterioration in neurological function.

OBSERVATIONS

A 4-year-old boy with SEL became paraplegic 4 months after completion of chemoradiation for his previously resected, intracranial atypical teratoid rhabdoid tumor. The patient presented with rapid deterioration in lower extremity sensory and motor function, which, given his oncological history, was concerning for disease progression. Of note, 8 months prior, the patient was started on corticosteroid therapy for respiratory dysfunction. Magnetic resonance imaging revealed significant progression of lumbosacral SEL requiring surgical decompression with subsequent neurological improvement.

LESSONS

When evaluating pediatric patients with primary or metastatic brain tumors with new or worsening myelopathy and motor or sensory deficits, it is important to consider SEL.

Open access

Pineal parenchymal tumor of intermediate differentiation with late spinal dissemination 13 years after initial surgery: illustrative case

Hiroyuki Kato, Takafumi Tanei, Yusuke Nishimura, Yoshitaka Nagashima, Motonori Ishii, Tomoya Nishii, Nobuhisa Fukaya, Takashi Abe, and Ryuta Saito

BACKGROUND

Pineal parenchymal tumors of intermediate differentiation (PPTIDs) are rare in the pineal gland. A case of PPTID that disseminated to the lumbosacral spine 13 years after the total resection of a primary intracranial tumor has been reported.

OBSERVATIONS

A 14-year-old female presented with headache and diplopia. Magnetic resonance imaging revealed a pineal tumor that induced obstructive hydrocephalus. A biopsy and endoscopic third ventriculostomy were performed. Histological diagnosis revealed a grade II PPTID. Two months later, the tumor was removed via craniotomy because the postoperative Gamma Knife surgery was ineffective. Histological diagnosis confirmed PPTID, although the grade was revised from II to III. Postoperative adjuvant therapy was not performed, because the lesion had been irradiated and gross total tumor removal was achieved. She has had no recurrence in 13 years. However, pain around the anus newly appeared. Magnetic resonance imaging of the spine revealed a solid lesion in the lumbosacral spine. The lesion was subtotally resected, and histological diagnosis revealed grade III PPTID. Postoperative radiotherapy was performed, and she had no recurrence 1 year after radiotherapy.

LESSONS

Remote dissemination of PPTID can occur several years after the initial resection. Regular follow-up imaging, including the spinal region, should be encouraged.

Open access

Neurosurgical management of vertebral lesions in pediatric chronic recurrent multifocal osteomyelitis: patient series

Nicholas F. Hug, David A. Purger, Daphne Li, Lawrence Rinsky, and David S. Hong

BACKGROUND

Chronic recurrent multifocal osteomyelitis (CRMO) is a rare pediatric autoinflammatory disorder involving 2 or more inflammatory bone lesions separated in time and space associated with pathological vertebral fractures. There are no current guidelines for the role of pediatric spine surgeons in the management of this condition. The authors demonstrate the importance of close and early involvement of neurosurgeons in caring for patients with CRMO with vertebral involvement.

OBSERVATIONS

Fifty-six pediatric patients with clinical and radiographic evidence of CRMO were identified and clinical, radiographic, laboratory, and histopathological data were reviewed. All were evaluated via Jansson and Bristol CRMO diagnostic criteria. Ten had radiographic evidence of vertebral involvement (17.9%). Nine of these had multifocal disease. Five patients had multiple vertebrae affected. Six patients were evaluated for possible surgical intervention and one required intervention due to vertebra plana leading to a progressive kyphotic deformity and significant spinal canal stenosis.

LESSONS

In conjunction with management by the primary pediatric rheumatology team using nonsteroidal anti-inflammatory drugs, disease-modifying anti-rheumatic drugs, immunotherapies, and bisphosphonates, given the risk of pathological fractures and potential resulting long-term neurological deficits, the authors recommend close monitoring and management by pediatric spine surgeons for any patient with CRMO with vertebral lesions.