A rare case of intervertebral disc calcification combined with ossification of the posterior longitudinal ligament in a child: a case report and literature review.

BACKGROUND: Intervertebral disc calcification (IDC) combined with calcification in children has been sporadically reported, while ossification of the posterior longitudinal ligament (OPLL) in the cervical spine in pediatric patients is exceedingly rare. The aim of this study is to investigate the potential prognosis and outcomes associated with this condition. CASE PRESENTATION: We present an unusual case involving a 10-year-old Chinese child diagnosed with calcified cervical disc herniation and ossification of the posterior longitudinal ligament. Conservative treatment measures were implemented, and at the 1-month and 6-month follow-up, the patient's pain exhibited significant improvement. Subsequent cervical MRI and CT scans revealed the complete disappearance of OPLL and substantial absorption of the calcified disc. During the three-month follow-up, CT demonstrated slight residual disc calcification, however, the patient remained asymptomatic with no discernible limitation in cervical motion. CONCLUSIONS: We conducted a comprehensive review of several cases presenting with the same diagnosis. It is noteworthy that IDC combined with OPLL in children constitutes a rare clinical entity. Despite imaging indications of potential spinal canal occupation, the majority of such cases demonstrate complete absorption following conservative treatment, with OPLL exhibiting a faster absorption rate than calcified discs.

Consensus statement on diagnosis and treatment of cervical ossification of posterior longitudinal ligament from Asia Pacific Spine Society (APSS) 2020.

PURPOSE: The study aimed to develop an evidence-based expert consensus statement on diagnosis and treatment of cervical ossification posterior longitudinal ligament (OPLL). METHOD: Delphi method was used to perform such survey, and the panel members from Asia Pacific Spine Society (APSS) 2020 were invited to answer the open-ended questions in rounds 1 and 2. Then the results were summarized and developed into a Likert-style questionnaire for voting in round 3, and the level of agreement was defined as 80%. In the whole process, we conducted a systematic literature search on evidence for each statement. RESULTS: Cervical OPLL can cause various degrees of neurological symptoms, an it's thought to be more common in Asia population. CT reconstruction is an important imaging examination to assist diagnosis and guide surgical choice. Segmental, continuous, mixed, and focal type is the most widely used classification system. The non-surgical treatment is recommended for patients with no or mild clinical symptoms, or irreversible neurological damage, or failed surgical decompression, or condition cannot tolerant surgery, or refusing surgery. As OPLL may continue to develop gradually, surgical treatment would be considered in their course inevitably. The surgical choice should depend on various conditions, such as involved levels, thickness, and type of OPLL, skill-experiences of surgeons, which are listed and discussed in the article. CONCLUSION: In this statement, we describe the clinical features, classifications, and diagnostic criteria of cervical OPLL, and review various surgical methods (such as their indications, complications), and provide a guideline on their choice strategy.

The Effect of an Educational and Interactive Informed Consent Process on Patients With Cervical Spondylotic Myelopathy Caused by Ossification of the Posterior Longitudinal Ligament.

STUDY DESIGN: Prospective observational cohort study. OBJECTIVE: In this study, an educational and interactive informed consent (EIC) program was proposed for patients with cervical spondylotic myelopathy caused by ossification of the posterior longitudinal ligament (OPLL-CSM) to improve their comprehension level during the informed consent process. SUMMARY OF BACKGROUND DATA: OPLL-CSM is a slow progressive disease, and it is difficult for patients to understand the disease. Few studies have evaluated very specific programs to improve the informed consent process for these patients. METHODS: This prospective study evaluated patients with OPLL-CSM who either underwent the proposed EIC process (n = 63) or the standard consent process (n = 124). The standard consent process only included a physician-patient interview. During the EIC process, information was provided regarding OPLL-CSM through information booklets, a video, verbal information, and initial and second physician-patient interviews. After the second physician-patient interview, the patient was requested to answer 14 medical questions to assess their knowledge about OPLL-CSM. The proposed EIC process took approximately 90 minutes. They were asked to report the most useful educational method and the most effective method of reinforcing verbal communication. RESULTS: The mean questionnaire scores were higher in the EIC group than in the control group (P < 0.001). Video was selected by 50/63 patients (79.4%) as the most useful EIC process method, and the most effective method of reinforcing verbal communication was video (n = 61; 96.8%). Patients in the EIC group reported having higher satisfaction with surgery (P = 0.024) than did those in the control group. CONCLUSION: The proposed EIC process was shown to result in good patient comprehension and recall regarding OPLL-CSM. Using a video was the most informative and effective reinforcement of verbal communication. The enhanced educational group had better knowledge and improved satisfaction following surgery. The EIC process might help physicians educate and counsel patients regarding OPLL-CSM and its treatment. LEVEL OF EVIDENCE: 2.

Calcification of the intervertebral disc and ossification of posterior longitudinal ligament in children.

BACKGROUND: IDC in children, first reported by Baron in 1924, is very rare. OPLL of the cervical spine mainly affect people ages 50-70 years. The coexistence of IDC and OPLL in children is very rare, only six cases with 3 to 24 months' follow-up were reported to date. CASE PRESENTATION: A 6-year-old boy presented with complains of neck pain at July 2007. The boy was treated by conservative treatment and observed up for 9 years. Neck pain greatly improved after a one-month conservative treatment and never recur. Laboratory tests revealed elevated ESR and CRP at admission and found nothing abnormal at 19-month and 9-year follow-up. Computed tomography and magnetic resonance imaging revealed IDC at C2/3, C3/4 and OPLL at C3/4 at admission and found minor calcification at C2/3 remained but calcification at C3/4 and OPLL at C3/4 completely disappeared at 19-month and 9-year follow-up. Nineteen months after initial diagnosis, restoration of T2-weighted signal intensity of C2/3 and C3/4 discs was observed through MRI. Loss of T2-weighted signal intensity of C2/3 disc and decrease of T2-weighted signal intensity of C3/4 disc was observed at 9-year follow-up. CONCLUSIONS: IDC with OPLL in children is very rare. Conservative treatments are recommended with affirmative short-term and long-term clinical effects. More intensive observation with long-term follow-ups may be needed to warrant the clinical effects.

[Ossification of the posterior longitudinal ligament (OPLL) and the ligamentum flavum (OLF)].

The adult population is frequently sustained with ossification of the posterior longitudinal ligament (OPLL) and/or the ligamentum flavum (OLF) of the spine and the diseases may subsequently result is serious spinal cord insult with profound paralysis of the extremities. These pathologies are genetically denoted metaplasia of the elastic fibers of the ligament with consequent ectopic ossification process. The spinal cord is chronically compressed and will result in profound motor paralysis with sensory deficit. Both diseases are well imaged on CT and MRI, showing a various magnitude of spinal cord compression.

Laminoplasty versus conservative treatment for acute cervical spinal cord injury caused by ossification of the posterior longitudinal ligament after minor trauma.

BACKGROUND CONTEXT: No reports to date have accurately evaluated the management for acute spinal cord injury (SCI) caused by ossification of the posterior longitudinal ligament (OPLL) after minor trauma. PURPOSE: To assess whether outcomes of laminoplasty is better than conservative treatment. STUDY DESIGN/SETTING: A retrospective study. PATIENT SAMPLE: Thirty-one patients underwent surgery (L group) and 29 patients underwent conservative treatment (C group). OUTCOME MEASURES: Disability, muscle strength, sensation, and general health status. METHODS: Patients were managed according to routine clinical practice and the results between groups were compared. Clinical and radiographic outcomes were assessed at admission, discharge, 6 months and at the final visit. Causes for trauma, duration of hospital stay, and complication were also evaluated. RESULTS: Causes for trauma included falling, traffic accidents and sports. Mixed and segmental types were the most frequent cause of OPLL resulting into SCI. Duration of hospital stay and complications were less in the L group. Motor and sensory scores increased in the L group at discharge (p<.05) and at 6 months (p<.05), and maintained thereafter (p>.05); scores improved significantly in the C group at 6 months (p<.05), with a slight deterioration with time (p>.05); scores in the L group were higher than in the C group at each time point after surgery (p<.05). Bodily pain and mental health in SF-36 improved at discharge in the L group (p<.05); all scores improved at 6 months in both the groups (p<.05), with better improvements in the L group (p<.05). The canal diameter increased and occupation ratio decreased in the L group (p<.05), and maintained thereafter (p<.05); a slight increase of occupation ratio was observed in the C group (p>.05). Lordotic angle and range of motion were maintained in both the groups, with no significance between groups (p>.05). High-signal intensity decreased at 6 months (p<.05) in the L group; no significant change was found in the C group during the follow-up (p>.05); Significant difference was detected between the groups at 6 months and at the final visit (p<.05). CONCLUSIONS: Most of the OPLL patients displayed as incomplete SCI after minor trauma. Although spontaneous improvement of SCI without surgery is often observed, laminoplasty has more satisfactory outcomes, prevents late compression of cord, and reduces perioperative complications, although with no significant benefit in cervical alignment and range of motion.

Proline/arginine-rich end leucine-rich repeat protein converts stem cells to ligament tissue and Zn(II) influences its nuclear expression.

Our objective was to facilitate ligament tissue reconstruction by characterizing the mechanism of expression of ligament tissue. To accomplish this, we searched for proteins specific to the tissue and introduced them into mesenchymal stem cells. In the two-dimensional phosphorescent gel electrophoresis, the spots in common with the normal human ligament tissue were selected after removing the spots of the normal bone tissue from those of the ossified tissue in the spinal ligament. Proline/arginine-rich end leucine-rich repeat protein (PRELP) was identified in ligament-specific locations by liquid chromatography-tandem mass spectrometry. Transfection of PRELP into mouse mesenchymal stem cells yielded ligament-like connective tissue comprised of parallel fibers. Thus, expression of the PRELP protein could reconstruct the ligament tissue. Since zinc-related proteins were found with high incidence as a result of an array analysis of PRELP's ProtoArray, it was considered that there is a relationship to the zinc metabolism. Tissue induction was mediated by the tumor necrosis factor (TNF)-α via the zinc pathway. PRELP may be a useful gene in syndesmoplasty, provided zinc is present for tissue reconstruction. Chromosome division becomes active with the addition of zinc, and rapid tissue induction takes place in the presence of zinc and TNF-α. Currently, the reconstruction of a ruptured ligament tissue is difficult, but we expect that the PRELP protein expression may facilitate this process. This study describes the discovery of the gene responsible for the differentiation of stem cells into ligament tissue. This important finding may lead to treatments for gonarthrosis, cruciate ligament, and periodontal ligament ruptures, and ossification of the posterior longitudinal ligament.

Conservatively treated ossification of the posterior longitudinal ligament increases the risk of spinal cord injury: a nationwide cohort study.

The optimal treatment strategy for ossification of the posterior longitudinal ligament (OPLL) depends on symptoms and is uncertain. Whether the risk of spinal cord injury (SCI) is increased in patients with cervical spinal stenosis or myelopathy caused by OPLL remains unclear. This study aimed to evaluate the risk of SCI in patients with OPLL of the cervical spine when managed with conservative treatment (no surgery). Study subjects were identified from a nationwide cohort of 26,544,883 people from 1998 to 2005 and were divided into the OPLL group (n=265), who were hospitalized for OPLL but had conservative treatment (no surgery), and the comparison group (n=5339), composed of age- and sex-matched people. Until the end of 2008, a total of 5604 subjects were followed-up for 34,723.5 person-years. The propensity score method was used to adjust for covariates. Kaplan-Meier and Cox regression analyses were performed. The incidence rate of cervical SCI in the OPLL group was found to be significantly higher than in the comparison group (4.81 versus 0.18 per 1000 person-years; p<0.001). Cervical SCI was more likely to happen in the OPLL group than in the comparison group (crude hazard ratio [HR] 25.64; p<0.001). After adjustments, the OPLL group had a 32.16-fold (p<0.001) higher risk for cervical SCI. Disability caused by SCI had an even higher risk (HR=110.72, adjusted HR=104.78; p<0.001) for the OPLL group. Therefore, cervical SCI and related disabilities are more likely to happen in OPLL patients, who should be cautioned for subsequent SCI if treated conservatively.

Management of cerebrospinal fluid leaks after anterior decompression for ossification of the posterior longitudinal ligament: a review of the literature.

OBJECT: Anterior decompression is an effective way to treat cervical myelopathy associated with ossification of the posterior longitudinal ligament (OPLL); however, this approach is associated with an increased risk of a dural tear and resultant CSF leak because fusion of the dura with the ossified PLL is common in these cases. The authors review the literature and present an algorithm for treatment of CSF leaks in these patients. METHODS: A MEDLINE review was performed to identify papers related to CSF leak after anterior decompression for OPLL, and data were summarized to identify treatment options for various situations. A treatment algorithm was identified based on these findings and the experience of the authors. RESULTS: Eleven studies were identified that presented data on intra- and postoperative management of a CSF leak during ventral surgery for OPLL. The incidence of cervical dural tears and CSF leaks after anterior decompression procedures for OPLL ranged from 4.3% to 32%. Techniques including preventative measures, intraoperative dural repair with various materials, and postoperative drainage or shunt placement have all been used. CONCLUSIONS: Although direct dural repair is the preferred treatment for CSF leak, this technique is not always technically possible. In these cases, intraoperative adjuncts in combination with postoperative measures can be used to decrease the pressure gradient across the dural tear.

Conservative management of ossification of the posterior longitudinal ligament. A review.

OBJECT: Ossification of the posterior longitudinal ligament (OPLL) can result in significant myelopathy. Surgical treatment for OPLL has been extensively documented in the literature, but less data exist on conservative management of this condition. METHODS: The authors conducted a systematic review to identify all reported cases of OPLL that were conservatively managed without surgery. RESULTS: The review yielded 11 published studies reporting on a total of 480 patients (range per study 1-359 patients) over a mean follow-up period of 14.6 years (range 0.4-26 years). Of these 480 patients, 348 (72.5%) were without myelopathy on initial presentation, whereas 76 patients (15.8%) had signs of myelopathy; in 56 cases (15.8%), the presence of myelopathy was not specified. The mean aggregate Japanese Orthopaedic Association score on presentation for 111 patients was 15.3. Data available for 330 patients who initially presented without myelopathy showed progression to myelopathy in 55 (16.7%), whereas the other 275 (83.3%) remained progression free. In the 76 patients presenting with myelopathy, 37 (48.7%) showed clinical progression, whereas 39 (51.5%) remained clinically unchanged or improved. CONCLUSIONS: Patients who present without myelopathy have a high chance of remaining progression free. Those who already have signs of myelopathy at presentation may benefit from surgery due to a higher rate of progression over continued follow-up.

Intervertebral thoracic disc calcification associated with ossification of posterior longitudinal ligament in an eleven-year-old child.

STUDY DESIGN: Intervertebral disc calcification of T6-T7, T7-T8 discs associated with ossification of posterior longitudinal ligament (OPLL) in a child is reported. OBJECTIVE: To discuss the natural history and management of calcification of T6-T7, T7-T8 discs with OPLL. SUMMARY OF BACKGROUND DATA: Calcified intervertebral discs are rare in children. Cervical disc calcification has already been described. However, thoracic disc calcification associated with OPLL has not been reported. METHODS: An 11-year-old boy presented with progressive back pain for 6 months. Neurologic examination showed numbness in both lower extremities. The knee jerk reflex of the patient was hypertonic. The muscle strength of both lower extremities were Grade 4, with reduction. He was treated with a lumbar belt for 2 weeks. RESULTS: Initial thoracic spine radiograph and CT scan showed two adjacent calcified discs of T6-T7, T7-T8 associated with T6-T7 OPLL, resulting in marked spinal canal stenosis. His neurologic symptoms subsided and his back pain disappeared after a 2-week conservative treatment. Three months later CT scan showed that the calcification of T6-T7, T7-T8 discs was aggravated, but the T6-T7 OPLL was relieved. CONCLUSION: The natural history of intervertebral disc calcification is usually benign. In this case, the improvement of OPLL is associated with the stabilization of the maturely fused calcified disc. Spontaneous resolution of the OPLL and recovery of normal neurologic function can be expected with conservative treatment.

[Updates on ossification of posterior longitudinal ligament. Ossification front of posterior longitudinal ligament and cellular biological assessment of chronic mechanical compressed spinal cord].

Mechanisms of ossification processes, pathological changes, and treatment/assessment of myelopathy symptoms because of ossification of the posterior longitudinal ligament (OPLL) remain obscure. Enchondral ossification process of OPLL was closely associated with degenerative changes of elastic fibers and cartilage formation, together with the appearance of metaplastic hypertrophic cartilage cells and neovascularization. There are differences in expression degrees of cytokines and transcription factors between mixed and localized OPLL. While the chronic compressed spinal cord may have plasticity ; the use of stem cell implants, supplementation of neurotrophic factors, in addition to surgical treatment, may bring a better clinical outcome,encouraging the development of these basic research studies. Assessment using new imaging techniques needs to determine the affected level and judge the severity of symptoms.

[Bone disease with pain. The pain caused by ossification of the posterior longitudinal ligament of the cervical spine].

Ossification of the posterior longitudinal ligament of the cervical spine is a hyperostotic condition of spine. The spinal cord is compressed by OPLL in the spinal canal. As a result, the myelopathy is caused. The pain is a symptom which is invited by OPLL. Non-steroidal anti-inflammatory drugs usually cure the pain ; in addition antidepressant and anticonvulsant are effective. The operation is performed for decompression of the spinal cord. Laminoplasty is ordinarily indicated, however, anterior decompression should be chosen for the patient with kyphotic alignment of cervical spine and with highly canal narrowing ratio.

Clinical course of patients with ossification of the posterior longitudinal ligament: a minimum 10-year cohort study.

OBJECT: Ossification of the posterior longitudinal ligament (OPLL) may produce quadriplegia. The course of future neurological deterioration in patients with radiographic evidence of OPLL, however, is not known. The authors conducted a long-term follow-up cohort study of more than 10 years to clarify the clinical course of this disease progression. METHODS: A total of 450 patients, including 304 managed conservatively and 146 treated by surgery, were enrolled in the study. All patients underwent neurological and radiographical follow-up examinations for a mean of 17.6 years. Myelopathy was graded using Nurick classification and the Japanese Orthopaedic Association scale. Fifty-five (17%) of 323 patients without myelopathy evident at the first examination developed myelopathy during the follow-up period. Risk factors associated with the evolution of myelopathy included greater than 60% OPLL-induced stenotic compromise of the cervical canal, and increased range of motion of the cervical spine. Using Kaplan-Meier analysis, the myelopathy-free rate in patients without first-visit myelopathy was 71% after 30 years. A significant difference in final functional outcome was not observed between nonsurgical and surgical cases in which preoperative Nurick grades were 1 or 2. In patients with Nurick Grade 3 or 4 myelopathy, however, only 12% who underwent surgery eventually became wheelchair bound or bedridden compared with 89% of those managed conservatively. Surgery proved ineffective in the management of patients with Grade 5 disease. CONCLUSIONS: Results of this long-term cohort study elucidated the clinical course of OPLL following conservative or surgical management. Surgery proved effective for the management of patients with Nurick Grades 3 and 4 myelopathy.

Pathogenesis of myelopathy in patients with ossification of the posterior longitudinal ligament.

OBJECT: The goal of this study was to clarify the pathogenesis of myelopathy in patients with ossification of the posterior longitudinal ligament (OPLL) based on the relationship between static compression factors and dynamic factors. METHODS: There was a total of 247 patients, including 167 patients who were conservatively followed for a mean of 11 years and 2 months and 80 patients who had myelopathy at initial consultation and underwent surgery. The changes in clinical symptoms associated with OPLL in the cervical spine were examined periodically. During the natural course of OPLL in the cervical spine, 37 (22%) of 167 patients developed or suffered aggravated spinal symptoms. All of the patients with a space available for the spinal cord (SAC) less than 6 mm suffered myelopathy, whereas the patients with an SAC diameter of 14 mm or greater did not. No correlation was found between the presence or absence of myelopathy in patients whose SAC diameter ranged from 6 mm to less than 14 mm. In patients with myelopathy whose minimal SAC diameter ranged from 6 mm to less than 14 mm, the range of motion of the cervical spine was significantly greater. CONCLUSIONS: These results indicate that pathological compression by the ossified ligament above a certain critical point may be the most significant factor in inducing myelopathy, whereas below that point dynamic factors may be largely involved in inducing myelopathy.

[Protrusion of a calcified cervical intervertebral disk in a 5-year-old boy]. / Protrusion einer verkalkten zervikalen Bandscheibe bei einem 5-jährigen Jungen.

Herniation of the calcified nucleus pulposus is a complication of intervertebral disc calcification. The herniated nucleus pulposus may assume an intraspinal location which may lead to cord compression and produce alarming neurological symptoms and radiographic images. Surgical intervention is, however, rarely necessary as complete resorption of the calcified disc material will generally occur. We report the case of a five years old boy with the symptoms of an acute tortcollis, radiographs of the cervical spine revealed calcification of C5/C6 intervertebral disc and MRI narrowing of the spinal canal. With conservative treatment his symptoms resolved within three weeks, three months later X-rays and MRI showed complete resolution of the calcification and herniation.