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Open access

Treatment of an anterior cervicothoracic myelomeningocele together with spine deformity correction in a child: illustrative case

Hudin N Jackson, Nealen Laxpati, and David F Bauer

BACKGROUND

Anterior cervicothoracic myelomeningoceles are a rare pathology. In reported cases, treatment has included shunting, isolated resection and repair without deformity correction, or isolated deformity correction without meningocele repair. The authors describe a pediatric patient with an anterior cervicothoracic myelomeningocele presenting with progressive neurological decline, who underwent simultaneous treatment of the myelomeningocele to detether the spinal cord and achieve major correction of the scoliotic deformity.

OBSERVATIONS

A 15-year-old girl was born with C7-T1-T2 hemivertebrae and anterior cervical myelomeningocele at C7–T1. She developed progressive cervical thoracic scoliosis, left hemiparesis initially, and additional right hemiparesis eventually. She underwent surgical repair via C7, T1, and T2 corpectomies with intradural detethering of the spinal cord. The scoliosis was treated with C7–T2 Ponte osteotomies and C2–T5 posterior fixation, followed by anterior reconstruction with a titanium cage and anterior plate from C6 to T3. The myelomeningocele was adequately treated with good correction of the patient’s deformity. The patient had postoperative improvement in her strength and solid arthrodesis on postoperative imaging.

LESSONS

The authors describe the successful treatment of an anterior cervicothoracic myelomeningocele and associated scoliosis in a child. This is a unique report of a combined strategy to achieve both deformity correction and detethering of the spinal cord.

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

Vacuum-assisted scalp repositioning: a novel temporizing approach to acute sinking skin flap syndrome. Illustrative cases

Evan Courville, Joshua Marquez, Michael Homma, Michael Conley, Georgios P Skandalakis, Peter Shin, James Botros, and Christian Ricks

BACKGROUND

This report describes the use of a novel approach to address acute sinking skin flap syndrome (SSFS), a postcraniectomy complication arising from brain dysfunction beneath the skull defect. The authors present a case series of two patients, emphasizing the prospective application of an external plaster cast in tandem with a vacuum-assisted closure (VAC) device (wound VAC) to promptly reposition the scalp and relieve brain compression.

OBSERVATIONS

Following intervention, one patient showed immediate neurological improvement, with complete resolution of symptoms within hours. Conversely, the second patient developed nonconvulsive status epilepticus. Computed tomography scans postintervention validated the successful scalp repositioning and mass effect resolution in both instances. This temporary approach proved successful in one patient with moderate symptoms, serving as a bridge to cranioplasty.

LESSONS

The integration of an external plaster cast and wound VAC offers a cost-effective and prompt solution for patients with acute SSFS pending cranioplasty. Appropriate patient selection and heightened caution for those with severe symptoms should be exercised.

Open access

Approaches to ventriculoperitoneal shunt scalp erosion: countersinking into the calvarium. Illustrative case

Denise Brunozzi, Melissa A LoPresti, Jennifer L McGrath, and Tord D Alden

BACKGROUND

Ventriculoperitoneal shunting (VPS) is a standard procedure for the treatment of hydrocephalus, and the management of its complications is common in the practice of pediatric neurosurgery. Shunt exposure, though a rare complication, can occur because of thin, fragile skin, a young patient age, protuberant hardware, poor scalp perfusion, and a multitude of other patient factors.

OBSERVATIONS

The authors report a complex case of VPS erosion through the scalp in a young female with Pfeiffer syndrome treated with external ventricular drainage, empirical antibiotics, and reinternalization with countersinking of replaced shunt hardware into the calvarium to prevent internal skin pressure points, reduce wound tension, and allow wound healing.

LESSONS

Recessing the shunt hardware, or countersinking the implant, into the calvarium is a simple technique often used in functional neurosurgical implantation surgeries, providing a safe surgical strategy to optimize wound healing in select cases in which the skin flap is unfavorable.

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

Intravascular ultrasound to aid in the diagnosis and revision of an intra-aortic pedicle screw: illustrative case

Landon D. Ehlers, Patrick J. Opperman, Jack E. Mordeson, Jonathan R. Thompson, and Daniel L. Surdell

BACKGROUND

Pedicle screw impingement on vessel walls has the potential for complications due to pulsatile effects and wall erosion. Artifacts from spinal instrumentation create difficulty in accurately evaluating this interface. The authors present the first case of intravascular ultrasound (IVUS) used to characterize a pedicle screw breach into the aortic lumen.

OBSERVATIONS

A 21-year-old female with surgically corrected scoliosis underwent computed tomography angiography (CTA) 3 years postoperatively, which revealed a pedicle screw within the thoracic aorta lumen. Metal artifact distorted the CTA images, which prompted the decision to use intraoperative IVUS. The IVUS confirmed the noninvasive imaging findings and guided final decisions regarding aortic endograft size and location during spine hardware revision.

LESSONS

For asymptomatic patients presenting with pedicle screws malpositioned in or near the aorta, treatment decisions revolve around the extent of vessel wall penetration. Intraluminal depth can be obscured by artifact on computed tomography or magnetic resonance imaging or inadequately evaluated by a transesophageal echocardiogram. In our intraoperative experience, IVUS confirmed the depth of vessel lumen violation by a single pedicle screw and no wall penetration by two additional screws of concern. This was useful in deciding on thoracic endovascular aortic repair graft size and landing zone and facilitated safe spinal instrumentation removal and revision.

Open access

Pedicle subtraction metallectomy with complex posterior reconstruction for fixed cervicothoracic kyphosis: illustrative case

Harman Chopra, José Manuel Orenday-Barraza, Alexander E. Braley, Alfredo Guiroy, Olivia E. Gilbert, and Michael A. Galgano

BACKGROUND

Iatrogenic cervical deformity is a devastating complication that can result from a well-intended operation but a poor understanding of the individual biomechanics of a patient’s spine. Patient factors, such as bone fragility, high T1 slope, and undiagnosed myopathies often play a role in perpetuating a deformity despite an otherwise successful surgery. This imbalance can lead to significant morbidity and a decreased quality of life.

OBSERVATIONS

A 55-year-old male presented to the authors’ clinic with a chin-to-chest deformity and cervical myelopathy. He previously had an anterior C2–T2 fixation and a posterior C1–T6 instrumented fusion. He subsequently developed screw pullout at multiple levels, so the original surgeon removed all of the posterior hardware. The T1 cage (original corpectomy) severely subsided into the body of T2, generating an angular kyphosis that eventually developed a rigid osseous circumferential union at the cervicothoracic junction with severe cord compression. An anterior approach was not feasible; therefore, a 3-column osteotomy/fusion in the upper thoracic spine was planned whereby 1 of the T2 screws would need to be removed from a posterior approach for the reduction to take place.

LESSONS

This case highlights the devastating effect of a hardware complication leading to a fixed cervical spine deformity and the complex decision making involved to safely correct the challenging deformity and restore function.

Open access

Vascular anomaly, lipoma, and polymicrogyria associated with schizencephaly: developmental and diagnostic insights. Illustrative case

Kevin K. Kumar, Angus Toland, Nancy Fischbein, Martha Morrell, Jeremy J. Heit, Donald E. Born, and Gary K. Steinberg

BACKGROUND

Schizencephaly is an uncommon central nervous system malformation. Intracranial lipomas are also rare, accounting for approximately 0.1% of brain “tumors.” They are believed to be derived from a persistent meninx primitiva, a neural crest–derived mesenchyme that develops into the dura and leptomeninges.

OBSERVATIONS

The authors present a case of heterotopic adipose tissue and a nonshunting arterial vascular malformation arising within a schizencephalic cleft in a 22-year-old male. Imaging showed right frontal gray matter abnormality and an associated suspected arteriovenous malformation with evidence of hemorrhage. Brain magnetic resonance imaging revealed right frontal polymicrogyria lining an open-lip schizencephaly, periventricular heterotopic gray matter, fat within the schizencephalic cleft, and gradient echo hypointensity concerning for prior hemorrhage. Histological assessment demonstrated mature adipose tissue with large-bore, thick-walled, irregular arteries. Mural calcifications and subendothelial cushions suggesting nonlaminar blood flow were observed. There were no arterialized veins or direct transitions from the arteries to veins. Hemosiderin deposition was scant, and hemorrhage was not present. The final diagnosis was consistent with ectopic mature adipose tissue and arteries with meningocerebral cicatrix.

LESSONS

This example of a complex maldevelopment of derivatives of the meninx primitiva in association with cortical maldevelopment highlights the unique challenges from both a radiological and histological perspective during diagnostic workup.

Open access

Surgical management of Hirayama disease in a pediatric patient presenting with severe cervical kyphosis and focal myelopathy: illustrative case

David B. Kurland, Sean Neifert, Hammad Khan, and Darryl Lau

BACKGROUND

Hirayama disease (HD) is a rare, nonfamilial neuromuscular disease causing cervical myelopathy and deformity, most commonly effecting pubertal Asian males. Patients whose nonoperative treatment fails and who cannot tolerate long-term cervical immobilization, experience relapse after arrest of symptoms, or present with severe features warrant surgical treatment. Here, the authors present an unusual case of HD that resulted in rapid progression of severe cervical kyphosis and discuss surgical management strategies.

OBSERVATIONS

A 15-year-old male presented with unprovoked neck pain, progressive chin-on-chest phenomenon, and cervical myelopathy. Imaging revealed a severe subaxial cervical kyphosis of 88° and severe spinal cord compression secondary to changes within the thecal sac, ligaments, and bony elements. He underwent a multistage surgery involving halo gravity traction, C3–6 anterior cervical discectomy and fusion, and C2 to T2 posterior instrumented fusion with C3–5 Smith-Petersen osteotomies. Cervical subaxial pedicle screws facilitated deformity correction through a cantilever technique.

LESSONS

HD is rare and often self-limited. For severe or refractory cases of HD, guidelines for surgical management have been suggested, with a variety of approaches deemed efficacious. This is the first case of a patient presenting with such severe cervical deformity; early diagnosis and recognition is the first step toward prompt, adequate management.