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BACKGROUND

Hajdu-Cheney syndrome (HCS) is an extremely rare genetic disorder characterized by severe osteoporosis, scoliosis, and persistent open cranial sutures (POCSs). Neurological complications include hydrocephalus, Chiari I malformations, and basilar invagination (BI). Surgical intervention in HCS is challenging due to severe osteoporosis, ligamentous laxity, POCSs, and extreme skeletal deformities. Herein, the authors present a case of BI repair in a patient with HCS and POCSs, requiring a novel technique of cranial vault suspension, with long-term follow-up.

OBSERVATIONS

A 20-year-old female with HCS and progressive symptomatic BI, initially managed with posterior fossa decompression and occipital to cervical fusion, subsequently required cranial vault expansion due to symptomatic shifting of her cranium secondary to POCS. This custom construct provided long-term stabilization and neurological improvement over a follow-up duration of 9.5 years. A literature review performed revealed three other cases of surgical intervention for BI in patients with HCS and clinicopathological characteristics of each case was compared to the present illustrative case.

LESSONS

POCSs in patients with BI complicate traditional surgical approaches, necessitating more invasive techniques to secure all mobile cranial parts for optimal outcomes. Using this cranial vault suspension and fusion technique results in lasting neurological improvement and construct stability.

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.

BACKGROUND

The authors describe a rare case of transient postoperative wrist and finger drop following a prone position minimally invasive surgery (MIS) lateral microdiscectomy.

OBSERVATIONS

Hand and wrist drop is an unusual complication following spine surgery, especially in prone positioning. The authors’ multidisciplinary team assessed a patient with this complication following MIS lateral microdiscectomy. The broad differential diagnosis included radial nerve palsy, C7 radiculopathy, stroke, and spinal cord injury. Given the patient’s supinator weakness, intact pronation and wrist flexion, and transient recovery within 4 weeks, the most likely diagnosis was radial nerve neuropraxia secondary to ischemic compression. After careful consideration of the operative environment and anatomical constraints, the patient’s blood pressure cuff was found to be the most probable source of compression.

LESSONS

Blood pressure cuff–induced peripheral nerve injury may be a source of postoperative radial nerve neuropraxia in patients undergoing spine surgery. Careful considerations must be given to the blood pressure cuff location, which should not be placed at the distal end of the humerus due to higher susceptibility of peripheral nerve compression. Spine surgeons should be aware of and appropriately localize postoperative deficits along the neuroaxis, including central versus proximal or distal peripheral injuries, in order to guide appropriate postoperative management.

BACKGROUND

Posttraumatic intradural hematomas of the cervical spine are rare findings that may yield significant neurological deficits if they compress the spinal cord. These compressive hematomas require prompt surgical evacuation. In certain instances, intradural hematomas may form from avulsion of cervical nerve roots.

OBSERVATIONS

The authors present the case of a 29-year-old male who presented with right upper-extremity weakness in the setting of polytrauma after a motor vehicle accident. He had no cervical fractures but subsequently developed right lower-extremity weakness. Magnetic resonance imaging (MRI) demonstrated a compressive hematoma of the cervical spine that was initially read as an epidural hematoma. However, intraoperatively, it was found to be a subdural hematoma, eccentric to the right, stemming from an avulsion of the right C6 nerve root.

LESSONS

Posttraumatic cervical subdural hematomas require rapid surgical evacuation if neurological deficits are present. The source of the hematoma may be an avulsed nerve root, and the associated deficits may be unilateral if the hematoma is eccentric to one side. Surgeons should be prepared for the possibility of an intradural hematoma even in instances in which MRI appears consistent with an epidural hematoma.

BACKGROUND

Synovial cysts are a common finding in degenerative spine disease, most frequently involving the facet joints of the lumbar spine. Synovial cysts are less common in the cervical spine and rarely involve the atlantoaxial junction.

OBSERVATIONS

In this case report, the authors detail a unique presentation of a left atlantoaxial synovial cyst with large intracranial extension into the cerebellopontine angle causing progressive cranial nerve palsies resulting in tinnitus, vertigo, diminished hearing, gait imbalance, left trigeminal hypesthesia, left facial weakness, and dysarthria. The patient underwent a retromastoid craniectomy for resection of the synovial cyst, resulting in improvement and resolution of symptoms. Follow-up occurred at 6 weeks, 3 months, and 5 months postoperatively without recurrence on imaging.

LESSONS

The authors describe acute and long-term management of a unique presentation of an atlantoaxial synovial cyst including retromastoid craniectomy, intervals for follow-up for recurrence, and possible treatment options in cases of recurrence. A systematic literature review was also performed to explore all reported cases of craniocervical junction synovial cysts and subsequent surgical management.

BACKGROUND

Hürthle cell carcinoma (HCC) is an unusual and aggressive variant of the follicular type of differentiated thyroid cancer (DTC), accounting for less than 3% of DTCs but posing the highest risk of metastasis. Brain metastases are uncommonly reported in the literature but pose a poor prognosis. The low rate of brain metastases from HCC coupled with ambiguous treatment protocols for the extracranial disease complicate successful disease management and definitive treatment strategy. The authors present the case of a patient with HCC metastasis to the skull base, cortex, and spine with recent tibial metastasis.

OBSERVATIONS

Despite the presence of metastasis to the cortex, skull base, and spine, the patient responded very well to radiation therapy, sellar mass resection, and cervical spine decompression and fixation and has made a remarkable recovery.

LESSONS

The authors’ multidisciplinary approach to the patient’s care, including a diverse team of specialists from oncology, neurosurgery, orthopedic surgery, radiology, endocrinology, and collaboration with clinical trial researchers, was fundamental to her successful outcome, demonstrating the utility of intersecting specialties in successful outcomes in neuro-oncological patient care.

BACKGROUND

In surgery for cervical spondylotic myelopathy (CSM) with spondylolisthesis, there is no consensus on the correction and fixation for spondylolisthesis. The authors retrospectively studied whether the correction of single-level fixation with lateral mass screws (LMSs) could be maintained.

OBSERVATIONS

The records of patients with CSM with spondylolisthesis who had been treated with posterior decompression and single-level fusion with LMSs from 2017 to 2021 were retrospectively reviewed. Radiographic measurements included cervical parameters such as C2–7 lordosis, T1 slope, and the degree of spondylolisthesis (percent slippage) before surgery, immediately after surgery, and at the final observation.

Ten cases (mean age 72.8 ± 7.8 years) were included in the final analysis, and four cases (40%) were on hemodialysis. The median observation period was 26.5 months (interquartile range, 12–35.75). The mean percent slippage was 16.8% ± 4.7% before surgery, 5.3% ± 4.0% immediately after surgery, and 6.5% ± 4.7% at the final observation. Spearman’s rank correlation showed a moderate correlation between preoperative slippage magnitude and correction loss (r = 0.659; p = 0.038). Other parameters showed no correlation with correction loss.

LESSONS

For CSM with spondylolisthesis, single-level fixation with LMSs achieved and maintained successful correction in the 2-year observation.

BACKGROUND

This article describes a rare case of cervical spinal arteriovenous malformation (AVM) mimicking a neurogenic spinal tumor.

OBSERVATIONS

A 22-year-old female presenting with a C6–7 AVM with a calcification nodule experienced new-onset acute right upper radiculopathy associated with extradural compression of the spinal cord. Note that spinal AVMs with a calcified nodule are rare. Endovascular embolization is generally used to relieve the symptoms of AVM; however, this procedure cannot relieve cord compression, particularly in cases complicated by calcified nodules. This article discusses treatment options.

LESSONS

Decompression surgery is preferable to endovascular embolization because it alleviates symptoms while preventing cord compression and minimizing the risk of recurrence.

BACKGROUND

Intramedullary schwannomas account for 1.1% of all spinal schwannomas. Preoperative diagnosis is best accomplished by thoroughly evaluating clinical and radiological characteristics, accompanied by a high index of suspicion. The authors report a case of C2–3 intramedullary schwannoma in a young male who presented with neck pain and vertigo. The current literature is also reviewed.

OBSERVATIONS

The authors reviewed the data of a young male with a 2-month history of neck pain and vertigo. Magnetic resonance imaging of the brain and cervical spine showed an intramedullary mass at C2–3 with a syrinx extending into the cervicomedullary junction. Laminectomy, myelotomy, and microsurgical excision of the mass under intraoperative neurological monitoring (IONM) were done. Postoperative pathology reported the specimen as a schwannoma.

LESSONS

Gross-total resection of a schwannoma using IONM is the treatment of choice because of the lesion’s benign nature, a better prognosis, and defined cleavage plane. Schwannomas should be included in the differential diagnosis of intramedullary spinal tumors. Because of its progressive nature, early surgery is recommended in symptomatic patients.

BACKGROUND

Traumatic neuroma typically refers to a reactive process in the injured peripheral nerve, characterized by an excessive growth of axons, Schwann cells, and fibroblasts at the proximal end of the nerve after its interruption. The authors report a case of a traumatic neuroma in the cervical nerve root in a patient with no history of trauma.

OBSERVATIONS

The patient presented with sensation loss in the right-hand ulnar distribution, right flank around the T4–11 region, and right small toe along with motor power weakness over the right upper and lower extremity. Magnetic resonance imaging revealed an intradural extramedullary mass lesion with extension along the C7 nerve root. Histological examination showed traumatic neuroma. A total resection of the lesion along with the resolution of sensory and motor deficits was achieved directly after surgery.

LESSONS

Traumatic neuroma should always be kept in the armamentarium for diagnosis of an intradural nerve sheath tumor.