Multiple myeloma (MM), a malignant lymphoproliferative B-cell disorder, is the second most common hematological malignancy in the United States and makes up 1.6% of all documented cancers. 1 – 3 This abnormal proliferation of plasma cells in bone marrow results in osteolytic bone lesions, which often metastasize to the spine. 4 Patients with spinal metastases are commonly managed by multidisciplinary oncology teams in concordance with principles espoused by the neurological, oncological, mechanical, and systematic (NOMS) decision framework, which guides management in the context of the presence of neurological deficit, degree of epidural spinal cord compression (ESCC), anticipated tumor response to radiation therapy (RT), spinal instability, and systemic disease status. 5 , 6 Goals of surgical management, when indicated, include preservation or restoration of neurological function, maintenance of spinal stability, and alleviation of pain. 5 As MM is highly radiosensitive, conventional RT without surgery remains the mainstay of treatment for patients with spinal metastasis, except for cases with gross mechanical instability. 5 , 7
Here, we discuss a case of spinal MM presenting with a progressive, atraumatic chin-on-chest deformity. This patient was managed initially with axial halo-gravity traction and halo-vest immobilization and chemotherapy and RT and, several months later, surgical stabilization.
Illustrative Case
Clinical Presentation
A 66-year-old male presented with several months of diffuse, progressive neck pain, difficulty ambulating, and an inability to raise his head off his chest. Physical examination revealed a severe 90° kyphotic chin-on-chest deformity and associated stage IV pressure ulcer on the underlying chest. This deformity was noted to be mobile based on the ability to move the neck with gentle manual bedside traction applied with the hands. Neurological examination noted 4/5 strength in bilateral hand grip and finger abduction with Hoffman’s reflex and sustained clonus bilaterally. He also displayed a baseline subtle right residual hemiparesis with a mild right-hand contracture from a prior stroke. Gait testing was deferred for safety concerns but was reported to be unstable. Laboratory evaluation revealed acute kidney injury, anemia, and a kappa/lambda ratio of 3.3. Computed tomography (CT) imaging revealed diffuse osseous lytic mandibular and spinal lesions with significant destruction of the T1 and T2 vertebral bodies, resulting in severe kyphotic deformity and cervicothoracic central canal stenosis ( Fig. 1 ). A biopsy of the right iliac crest was consistent with MM. At presentation, the patient’s medical history included stage 3 chronic kidney disease, chronic heart failure, and hypertension. FIG. 1. Images demonstrating burst deformities at T1–2 with severe kyphosis and chin-on-chest deformity and mild canal stenosis. Cervical ( A ) and thoracic ( B ) CT images were obtained with the patient in a supine position. Sagittal ( C ) and axial ( D ) magnetic resonance imaging sequences.
Initial Management
In summary, this patient presented with overt mechanical instability and spinal cord compression, both of which necessitated surgical decompression and stabilization at the cervicothoracic junction. However, his systemic bony disease would likely significantly increase the risk of hardware failure, especially in the context of deformity correction surgery. It was also likely that both an untreated kyphotic deformity, which impaired the patient’s functional status and ability to participate in activities of daily living (ADLs), and a possibly extensive recovery period from surgery could delay his initiation of systemic chemotherapy and/or radiation.
We know that a short course of systemic treatment along with bisphosphonates can improve the bony density in patients with MM. Taking into account the reducible nature of his deformity, the relative preservation of his neurological function, and his diffuse disease burden, the decision was made to prioritize the initiation of chemotherapy, bisphosphonates, and radiation while realigning and immobilizing the spine using an external halo orthosis to temporarily but immediately stabilize the patient’s deformity and thereby improve his functional status. Axial traction of 65 lbs was incrementally applied to the awake patient in a halo-gravity wheelchair orthosis system to restore appropriate spinal alignment ( Fig. 2 ). Radiographic alignment and neurological status were monitored with serial radiographs and neurological examinations, respectively, until appropriate alignment was established ( Fig. 3 ). The patient was then immobilized in a halo-vest external orthosis and tolerated it well. He was discharged from the hospital and then underwent six cycles of lenalidomide, bortezomib, and dexamethasone chemotherapy along with 30 Gy of RT. At the 6-month follow-up after initial presentation, the patient was compliant with his halo orthosis, had responded well to systemic therapy, and was cleared by his oncologist to undergo definitive spinal surgery. FIG. 2. Halo-gravity chair traction to restore spinal alignment is used for this mobile chin-to-chest deformity. A: The vest is in place and the halo is applied, prior to any added traction weight, in the alignment with which he presented to the hospital. B: After halo-gravity traction has restored adequate spinal alignment, after which the halo-vest system was connected and secured for immobilization. FIG. 3. Lateral radiographs demonstrating cervicothoracic alignment prior to halo traction ( A ), following halo traction ( B ), and following definitive cervicothoracic decompression and fusion ( C ).
Operative Treatment and Postoperative Course
Three weeks after the completion of chemotherapy and RT, the patient underwent a C2–T10 decompression and instrumented fusion under general endotracheal anesthesia with the aid of intraoperative CT imaging ( Fig. 2 ). Four points of fixation (two pedicle screws and two laminar screws) were utilized at C2 to prevent proximal construct failure. Lateral mass screws were placed from C3 to C6, and pedicle screws were placed from T1 to T10. Intraoperative vertebral cement augmentation was utilized from C7 to T10, to reduce the likelihood of screw pullout, and at T11, to help prevent distal junctional failure. Bilateral laminectomies were also completed from C7 to T2 to address stenosis. The wound was closed in multiple layers utilizing local paraspinal muscle flaps with the assistance of plastic surgery. There were no intraoperative or postoperative complications, and intraoperative neuromonitoring signals were stable at the completion of the procedure. After a short inpatient stay, the patient was discharged to a rehabilitation facility with a cervicothoracic orthosis to be worn out of bed for a few months postoperatively and instructions to begin maintenance chemotherapy 2–3 weeks after surgery.
Follow-Up
At 1, 4, and 12 months following surgery, the patient was seen in the neurosurgery clinic in excellent condition, without postoperative neurological sequelae. He reported significant improvement in his baseline symptoms and was able to ambulate and do work around the house along with other ADLs without neck or back pain. Imaging confirmed intact hardware and alignment, with a sagittal vertical axis of +11 cm ( Fig. 3C ).
Unfortunately, over the next 2 years, his disease continued to progress despite multiple attempted chemotherapy combinations and an autologous stem cell transplant, with a course complicated by febrile neutropenia and bacteremia. At 2.5 years after initial treatment, he was transitioned to comfort-directed care and died peacefully with hospice support soon thereafter.
Informed Consent
The necessary informed consent was obtained in this study.
Discussion
Observations
Unlike many patients with metastatic spinal disease, patients with MM can survive for many years, making it paramount to optimize factors that facilitate cancer treatment and minimize long-term surgical complications. 8 , 9 While MM in neurologically intact patients is often effectively treated with chemo- and radiotherapy alone, the NOMS framework emphasizes the degree of ESCC and the presence of mechanical instability as criteria for surgical intervention, the timing of which is driven by the presence and degree of neurological deficits. 5 For those with significant deficits, urgent surgical intervention is necessary to preserve neurological function. In patients without significant deficits, such as the patient discussed here, systemic and bisphosphonate therapies prior to surgical intervention can improve vertebral body bone mineral density, screw pullout strength, construct integrity, and overall safety. 10 – 13 Because the incidence of hardware failure in patients with spinal metastases is reported to be as high as 16%, with MM significantly contributing to this risk due to severe generalized bone demineralization, it was crucial to focus on improving the vertebral bone density for this patient. 14 – 16
Halo traction and immobilization were employed to restore as much normal spinal alignment as possible, enabling the patient to improve functional status and, therefore, to better tolerate systemic therapy and RT and to continue enteric feeding. Although surgical intervention for unstable pathological fractures in patients with metastatic cancer is often initiated before systemic therapy and RT, the absence of significant neurological deficit in this patient with MM allowed for systemic therapies to improve bone density and optimize construct integrity before surgery. 17 Ultimately, the unique features of MM relative to other metastatic or primary tumors, including a lower likelihood of neurological deficits and an improvement in bone density after radiation and chemotherapy, permitted the delay of surgical treatment and initiation of systemic therapy.
While systemic myeloma therapy improved bone quality before surgery, significantly reducing the risk of construct failure, several intraoperative decisions were also made to further prevent construct failure in this illustrative case. Furthermore, given the patient’s limited life expectancy, the surgical team prioritized a conservative approach focused on spinal column stabilization and moderate correction of the deformity rather than achieving perfect spinal alignment correction. 17 Fixation was accomplished with pedicle, lateral mass, and laminar screws with cement augmentation.
Vertebroplasty, which entails the injection of polymethylmethacrylate bone cement into the vertebral body, provided structural support, further reducing the risk of vertebral collapse by stabilizing prior fractures. 18 – 20 Bone cement is also toxic to tumor cells and is the mainstay material for reconstructing bony defects in patients with metastases. 21 Although minimally invasive approaches for cement augmentation and screw fixation are viable for patients with MM, an open approach was chosen in this case due to the need for spinal cord decompression and the extent of instrumentation. 21 – 24 Because this patient presented with severe cervicothoracic kyphosis, a long-segment posterior fusion construct extending up to C2 and employing four-point fixation at the upper instrumented vertebra was used to reduce the risk of pseudarthrosis and junctional kyphosis. 25 , 26 Hardware-related complications are common following posterior segmentation fusion procedures, often necessitating reoperation, which is particularly taxing and risky for oncological patients whose conditions are medically complex and who may need to resume systemic therapy without interruption as soon as possible after recovery from surgery. 27 Although anterior column reconstruction could have improved long-term construct durability and cervicothoracic lordosis, it was not pursued due to the significant risks associated with the complex anterior approach (via sternotomy) or a posterior transpedicular corpectomy at T1, which would have posed substantial risks to the C8 and T1 nerve roots. 28 , 29
Addressing postoperative wound-related complications is another critical aspect of care for oncology patients undergoing spine surgery, given the various factors that can impair healing and increase infection risk. Immunosuppression due to malignancy increases the risk of postoperative complications such as surgical site infection (SSI). 30 , 31 RT, experienced by approximately 60% of patients, can also result in complications such as skin atrophy, soft tissue fibrosis, and desquamation, impairing postoperative healing. 32 , 33 While the standard of care for open spine surgery entails primary closure via layered tissue re-approximation, the utilization of paraspinal muscle flaps can reduce tension at wound edges, increase vascularized tissue, and minimize dead space. Paraspinal flap closure techniques have been shown to decrease the incidence of wound complications after spinal reconstruction surgery, particularly in spinal oncology patients. 25 , 34 Preoperative medical treatment of MM corrected the patient’s acute kidney injury, which has also been associated with an increased risk of SSI.
Ultimately, the patient experienced significant improvement in his quality of life postoperatively, with no significant pain complaints, progressive deformity, or construct failure throughout his remaining life.
Lessons
In our illustrative case of a 66-year-old patient with severe spinal deformity and myelopathy due to disseminated MM, we highlight the unique sequence of treatment events that facilitated optimal outcomes. The patient underwent a carefully timed sequence of halo traction and immobilization, followed by systemic therapies (chemo- and radiotherapy) before surgical intervention.
Unlike the typical approach for metastatic spine tumors where immediate surgery is often prioritized, the initial use of halo traction and immobilization allowed for spinal realignment and temporary stabilization. This allowed the patient to begin MM-directed systemic therapy almost immediately after diagnosis. 7 , 35 , 36 These therapies were critical in improving bone quality in a medically complex patient before definitive surgical stabilization, ultimately reducing the risk of pseudarthrosis and junctional kyphosis. Definitive surgery was undertaken 6 months later and successfully reduced the spine-related burden of his disease.
The decision to delay surgery until after systemic therapy reflects the unique aspects of MM, such as the lower likelihood of neurological deficits and the potential for bone density improvement following radiation and chemotherapy. This approach is atypical in metastatic spine tumor management, where immediate surgical intervention is often the norm to prevent devastating neurological injury. Ultimately, this individualized treatment plan alleviated the patient's deformity-related pain and discomfort, allowing him to regain mobility and perform activities independently during his last years of life with MM. Our case illustrates the value of individualized treatment planning and the potential benefits of delaying surgical intervention to optimize patient outcomes in complex oncological cases.
While unstable spinal fractures and/or high-grade ESCC are relatively uncommon presentations of MM, among patients presenting with either pathological fracture or ESCC secondary to malignancy of unknown origin, more than 50% are diagnosed with MM or plasmacytoma. 4 , 7 These patients often require urgent triage and collaboration with medical oncologists, radiation oncologists, and spine surgeons to establish a diagnosis and prevent devastating neurological injury. 35 Our case, therefore, also underscores the importance of a tailored, multidisciplinary approach in managing complex spinal deformities in patients with MM.
Disclosures
The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.
Author Contributions
Conception and design: Niu, Sastry, Shao, Abdulrazeq. Acquisition of data: Karimi, Sastry, Shao, Li. Analysis and interpretation of data: Sastry, Shao, Li. Drafting the article: Karimi, Sastry. Critically revising the article: all authors. Reviewed submitted version of manuscript: Karimi, Sastry, Shao, Abdulrazeq. Approved the final version of the manuscript on behalf of all authors: Niu. Administrative/technical/material support: Sastry. Study supervision: Niu, Sastry.
Correspondence
Tianyi Niu: Warren Alpert School of Medicine, Brown University, Rhode Island Hospital, Providence, RI. tniu@lifespan.org .
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