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Open access

Successful neurosurgical separation of conjoined spinal cords in pygopagus twins: illustrative cases

Chisato Yokota, Naoki Kagawa, Yohei Bamba, Yuko Tazuke, Yasuji Kitabatake, Tomoyoshi Nakagawa, Ryuichi Hirayama, Hiroomi Okuyama, and Haruhiko Kishima

BACKGROUND

Conjoined twins represent a rare congenital malformation. Pygopagus twins are fused at the sacrum and perineum, with union of the spine. The authors report a successful separation of a unique case of pygopagus twins sharing a U-shaped spinal cord, which the authors identified through aberrant nerves by intraoperative physiological spinal root examination.

OBSERVATIONS

The 6-month-old male pygopagus conjoined twins, who were diagnosed in the prenatal period, underwent separation. They had a single dural sac containing a U-shaped continuous spinal cord; their filum terminale appeared completely fused and the anatomical border of the spinal cord was not distinguishable. A triggered electromyogram (tEMG) was used on each nerve root to determine which belonged to one twin versus the other, to detect nerve cross, and to identify functional midline cleavage. Finally, the twins were separated after spinal division. Both twins recovered uneventfully with no lower limb neurological deficits or walking impairment for 16 months.

LESSONS

Pygopagus twins with a conjoined spinal cord are very rare, but a good long-term functional prognosis can be expected with successful separation. Intraoperative tEMG is useful in spinal separation surgery for twins with a conjoined spinal cord.

Open access

Surgical treatment in primary spinal infections in a pediatric population: illustrative case

Ryan S. Beyer, Austin J. Franklin, Matthew J. Hatter, Andrew Nguyen, Nolan J. Brown, Gaston Camino-Willhuber, Nestor R. Davies, Sohaib Hashmi, Michael Oh, Nitin Bhatia, and Yu-Po Lee

BACKGROUND

Primary spinal infections (PSIs) are a group of uncommon but serious infectious diseases that are characterized by inflammation of the endplate-disc unit. Pediatric spinal infection is rare and challenging to diagnose due to vague presenting symptoms. Most cases are conservatively managed with surgery rarely indicated. The authors performed a systematic review to study the baseline characteristics, clinical presentation, and outcomes of pediatric patients with PSIs who underwent surgical treatment.

OBSERVATIONS

PSI in pediatric patients might behave differently in terms of epidemiology, clinical presentation, and outcomes when compared with nonpediatric patients. Overall, PSI ultimately managed surgically in pediatric patients is associated with a high rate of localized pain, neurological compromise, and treatment failure when compared with nonsurgically managed pediatric spinal infections.

LESSONS

PSIs managed surgically in the pediatric population were found to be caused by Mycobacterium tuberculosis in 74.4% of cases and were associated with higher rates of localized pain, neurological compromise, and treatment failure than nonsurgically managed pediatric spinal infections. Thoracic involvement (71.8%) in the spinal infection was reported most commonly in our review. When omitting the cases involving M. tuberculosis infection, it was revealed that 50% of the pediatric cases involved infection in the cervical region, suggesting increased severity and disease course of cervical spinal infections in the pediatric population. Surgical treatment is indicated only in cases of severe neurological compromise and treatment failure.

Open access

Spinal cord stimulator for the treatment of central neuropathic pain secondary to cervical syringomyelia: illustrative case

Bryan A. Schatmeyer, Rakan Dodin, Michael Kinsman, and David Garcia

BACKGROUND

Central neuropathic pain (CNP) of the cervical and/or thoracic spinal cord has many etiologies, both natural and iatrogenic. Frequently, CNP is medically refractory and requires surgical treatment to modulate the perception of pain. Spinal cord stimulation is a modality commonly used in adults to treat this type of refractory pain; however, it is rarely used in the pediatric population.

OBSERVATIONS

The authors reported a case involving a common pediatric condition, Chiari malformation type I with syrinx, that led to a debilitating complex regional pain syndrome. The associated life-altering pain was successfully alleviated following placement of a spinal cord stimulator.

LESSONS

CNP, or the syndromic manifestations of the pain (complex regional pain syndrome), can alter an individual’s life in dramatic ways. Spinal cord stimulator placement in carefully selected pediatric patients should be considered in these difficult pain treatment paradigms.

Open access

Multiple-site neural tube defects complicated by multiple-site split cord malformations and thickened filum terminale: experience at a pediatric neurosurgical teaching hospital in Ethiopia. Illustrative case

Mestet Yibeltal Shiferaw, Yemisirach Bizuneh Akililu, Bethelehem Yesehak Worku, Tsegazeab Laeke T/Mariam, and Abenezer Tirsit Aklilu

BACKGROUND

Multiple-site open neural tube defects (MNTDs) and multiple-site split cord malformations (MSCMs) are extremely rare congenital anomalies that are defined by the simultaneous noncontiguous occurrence of more than one neural tube defect (NTD) and split cord malformation (SCM), respectively, in a single case with normal neural tissue in between. This work shows the cooccurrence of MNTDs and MSCMs, which has never been reported in the literature.

OBSERVATIONS

A single-stage repair for a 13-day-old female neonate with a preoperative diagnosis of MNTDs (thoracic meningocele and thoracolumbar myelomeningocele) plus an additional intraoperative diagnosis of MSCMs (type 3c) of thoracic and thoracolumbar spine, and thickened filum terminale was done with a favorable smooth postoperative course.

LESSONS

The use of intraoperative meticulous surgical technique along with preoperative skin stigmata helped for anticipation, detection, and treatment of associated complex spinal MNTDs, especially in resource-limited settings, where preoperative magnetic resonance imaging is not routinely used. Whether to repair the MNTDs as a single- versus multiple-stage procedure is mainly a function of the patient’s tolerance to the duration of anesthesia and the anticipated blood loss for the patient’s age. The overall developmental biology and long-term clinical outcome of MNTDs compared to single NTD/SCM is poorly understood and needs further study.

Open access

Presacral mature cystic teratoma associated with Currarino syndrome in an adolescent with androgen insensitivity: illustrative case

Grant Koskay, Patrick Opperman, Frank M. Mezzacappa, Joseph Menousek, Megan K. Fuller, Linden Fornoff, and Daniel Surdell

BACKGROUND

Currarino syndrome is a rare disorder that classically presents with the triad of presacral mass, anorectal malformation, and spinal dysraphism. The presacral mass is typically benign, although malignant transformation is possible. Surgical treatment of the mass and exploration and repair of associated dysraphism are indicated for diagnosis and symptom relief. There are no previous reports of Currarino syndrome in an androgen-insensitive patient.

OBSERVATIONS

A 17-year-old female patient presented with lack of menarche. Physical examination and laboratory investigation identified complete androgen insensitivity. Imaging analysis revealed a presacral mass lesion, and the patient was taken to surgery for resection of the mass and spinal cord untethering. Intraoperative ultrasound revealed a fibrous stalk connecting the thecal sac to the presacral mass, which was disconnected without the need for intrathecal exploration. The presacral mass was then resected, and pathological analysis revealed a mature cystic teratoma. Postoperatively, the patient recovered without neurological or gastrointestinal sequelae.

LESSONS

Diagnosis of incomplete Currarino syndrome may be difficult but can be identified via work-up of other disorders, such as androgen insensitivity. Intraoperative ultrasound is useful for surgical decision making and may obviate the need for intrathecal exploration during repair of dysraphism in the setting of Currarino syndrome.

Open access

Co-occurrence of subcutaneous myxopapillary ependymoma, dermal sinus tract, and filum terminale lipoma: a review of the pathobiology of caudal spinal cord development and spinal cord tethering. Illustrative case

Gabrielle W. Johnson, Yuxiao Xu, Ali Y. Mian, and David D. Limbrick Jr

BACKGROUND

Myxopapillary ependymoma (MPE) is typically benign and found in the conus medullaris and/or filum terminale, although rare cases of subcutaneous and extra-axial MPE have been reported. The co-occurrence of MPE, tethered cord syndrome (TCS) with lipoma of the filum terminale, and a dermal sinus tract is extremely rare, with only 6 reported cases in the literature. Here, the authors present the first case, to their knowledge, of an extra-axial, subcutaneous MPE co-presenting with TCS, lipoma of the filum terminale, and a dermal sinus tract and discuss the underlying pathobiology.

OBSERVATIONS

A 14-month-old male who presented for evaluation of a dermal sinus tract underwent magnetic resonance imaging, which revealed a tethered cord with associated lipoma. At 14 months, the patient underwent spinal cord detethering with resection of his sacral dimple and sinus tract. Histopathological evaluation revealed an incidentally found MPE within the dermal sinus tract.

LESSONS

The authors review the underlying biology of MPEs, tethered cord syndrome, and dermal sinus tracts, and explore possible points of convergence within the developmental pathways that may result in this unique concomitant presentation. Additionally, they suggest that extra-axial MPE may be underappreciated and underdiagnosed; this case suggests that extra-axial MPE may be only effectively diagnosed with histological studies.

Open access

Concomitant brain abscess and spinal cord abscess in an immunocompetent teenage male: illustrative case

Piiamaria S. Virtanen, Med Jimson D. Jimenez, V. Jane Horak, Virendra R. Desai, John J. Manaloor, and Jeffrey S. Raskin

BACKGROUND

Multiple bilateral brain abscesses occur rarely in immunocompetent patients. Hematogenous spread to the central nervous system (CNS) allows suppuration and abscess formation in the privileged immune environment of the CNS; hematogenous spread to the spinal cord is extremely rare and the combination of multifocal brain abscesses and intramedullary abscesses has not been reported. This report presents a rare presentation and diagrams a treatment algorithm involving iterative minimal access surgeries and prolonged medical management.

OBSERVATIONS

The authors present a case of an 18-year-old male with numerous multifocal and bilateral intraparenchymal abscesses and a medically resistant C5 intramedullary spinal cord abscess. The symptomatic patient had a left oculomotor palsy and left hemiparesis, ultimately undergoing ultrasound-guided aspiration of abscesses in the left frontal and left cerebral peduncle. Following transient motor improvement, he evolved tetraparesis prompting spinal cord imaging and emergent ultrasound-guided needle aspiration of an occult C5 intramedullary spinal cord abscess. The patient received appropriate medical therapy, completed inpatient rehabilitation, and made a full recovery.

LESSONS

Needle- and ultrasound-guided catheter drainage of CNS abscesses should be considered for symptomatic lesions. Following the neurological examination closely is extremely important; if the expected neurological improvement is delayed or regresses, then expanded imaging is warranted.

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.

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

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.