Development of a Scoring System to Evaluate the Severity of Craniocervical Spinal Cord Compression in Patients with Mucopolysaccharidosis IVA (Morquio A Syndrome).
ABSTRACT: BACKGROUND:As spinal cord compression at the craniocervical junction (CCJ) is a life-threatening manifestation in patients with mucopolysaccharidosis (MPS) IVA, surgical decompression should be performed before damage becomes irreversible. We evaluated the diagnostic value of several examinations for determining the need for decompression surgery. METHODS:We retrospectively analysed results of clinical neurological examination, somatosensory evoked potential (SEP) and magnetic resonance imaging (MRI) in 28 MPS IVA patients. A scoring system - based on the severity of findings - was used to compare results of patients with and without indication for decompression surgery. Individual test scores and two composite scores were evaluated for their potential to assess severity of CCJ impairment. RESULTS:Sixteen patients had an indication for surgery; 12 of them had undergone surgery. Twelve patients had no indication for surgery; none had received surgery. Neurological (P = 0.004), MRI (P < 0.001) and atlantoaxial subluxation (P = 0.006) scores, but not SEP and odontoid hypoplasia scores, differed significantly between patients with and without surgical indication. Both the abbreviated CCJ score, i.e. sum of neurological and MRI scores, and the extended CCJ score, i.e. sum of abbreviated CCJ and atlantoaxial subluxation score, discriminated between patients with and without surgical indication (abbreviated: 0-2 points vs 2-5 points, P < 0.001; extended: 0-3 points vs 3-7 points; P < 0.001). Although CCJ instability plays a major role in cervical cord pathology, decompression surgery without occipito-cervical stabilisation may yield good postoperative results. CONCLUSIONS:The abbreviated and extended CCJ scores are objective, transparent and reproducible tools for assessing the CCJ pathology and the need for surgery.
Project description:Cervical myelopathy occurs in approximately 2.5% of patients suffering from rheumatoid arthritis (RA) and is associated with notable morbidity and mortality. However, the surgical management of patients affected by cervical involvement in the setting of RA remains challenging and not well studied. To address this, we conducted a retrospective analysis of our clinical database between May 2007 and April 2017, and report on nine patients suffering from cervical myelopathy due to RA. We included patients treated surgically for cervical myelopathy on the basis of diagnosed RA. Clinical findings, treatment and outcome were assessed and reported. In addition, we conducted a narrative review of the literature. Four patients were male. Mean age was 64.8 ± 20.5 years. Underlying cervical pathology was anterior atlantoaxial instability (AAI) associated with retrodental pannus in four cases, anterior atlantoaxial subluxation (AAS) in two cases and basilar invagination in three cases. All patients received surgical treatment via posterior fixation, and in addition two of these cases were combined with a transnasal approach. Preoperative modified Japanese orthopaedic association scale (mJOA) improved from 12 ± 2.4 to 14.6 ± 1.89 at a mean follow-up at 18.8 ± 23.3 months (range 3-60 months) in five patients. In four patients, no follow up was available, and the mJOA of these patients at time of discharge was stable compared to the preoperative score. One patient died two days after surgery, where a pulmonary embolism was assumed to be the cause of mortality, and one patient sustained a temporary worsening of his neurological deficit postoperatively. Surgery is generally an effective treatment method in patients with inflammatory arthropathies of the cervical spine. Given the nature of the RA and potential instability, fixation in addition to cord decompression is generally required.
Project description:The objectives of this study are to investigate the clinical curative effect of Gallie technique and atlantoaxial screw-rod constructs (SRC) on atlantoaxial sagittal instability and determine the indication of Gallie technique.Data of 49 patients with atlantoaxial sagittal instability from February 2008 to May 2015 were analyzed retrospectively. The visual analog scale (VAS) score and the neck disability index (NDI) were used to evaluate the curative effect. Postoperative radiological outcomes were used to evaluate the stability of atlantoaxial joint and bone fusion. Perioperative parameters such as blood loss, operation time, radiographic exposure times, and hospital expense were also recorded and analyzed.Forty-nine patients (36 men and 13 women) were included in this study. The mean age was 41.4 ± 8.9 (range from 19 to 64). All patients were followed up for 24-67 months. Among these patients, 25 of these patients underwent Gallie surgery and 24 underwent SRC surgery. The pain in the occipitocervical area of all the patients has been relieved. NDI scores and VAS scores were lower in Gallie group than in SRC group in early postoperative period. The proportion of the patients who achieved good bone fusion within 3 months after operation was 88.0% (22/25) in the Gallie group and 100% (24/24) in the SRC group. The Gallie group is lower than the SRC group in blood loss, operation time, radiographic exposure times, and hospital expense. Statistical difference was observed between the two groups.For patients with atlantoaxial instability who has (1) the atlantodental interval (ADI) which is bigger than 5 mm on lateral flexion-extension X-ray, or Anderson-D'Alonzo type II odontoid fracture, (2) no asymmetry between odontoid process and lateral mass on open-mouth anterior-posterior X-ray, and (3) no dislocation of lateral mass joint on the CT 3D reconstruction, Gallie technique can be chosen as a safe and effective method if atlantoaxial reduction can be achieved preoperatively. Compared with SRC, Gallie technique can relieve the pain in the occipitocervical area earlier and it can shorten operation time and reduce intraoperative bleeding, radiographic exposure times, and hospital expense effectively. However, for patients with irreducible atlantoaxial dislocation, the Gallie technique should be used with caution.
Project description:Resection of the odontoid process and anterior arch of the atlas results in atlantoaxial instability, which if left uncorrected may lead to severe neurological complications. Currently, such atlantoaxial instability is corrected by anterior and/or posterior C1-C2 fusion. However, this results in considerable loss of rotation function of the atlantoaxial complex. From the viewpoint of retaining the rotation function and providing stability, we designed an artificial atlanto-odontoid joint based on anatomical measurements of 50 pairs of dry atlantoaxial specimens by digital calipers and 10 fresh cadaveric specimens by microsurgical techniques. The metal-on-metal titanium alloy joint has an arc-shaped atlas component, and a hollow cylindrical bushing into which fits a rotation axle of an inverted v-shaped axis component and is implanted through a transoral approach. After the joint was implanted onto specimens with anterior decompression, biomechanical tests were performed to compare the stability parameters in the intact state, after decompression, after artificial joint replacement, and after fatigue test. Compared to the intact state, artificial joint replacement resulted in a significant decrease in the range of motion (ROM) and neutral zone (NZ) during flexion, extension, and lateral bending (P < 0.001); however, with regard to axial rotation, there was no significant difference in ROM (P = 0.405), a significant increase in NZ (P = 0.008), and a significant decrease in stiffness (P = 0.003). Compared to the decompressed state, artificial joint replacement resulted in a significantly decreased ROM (P B 0.021) and NZ (P B 0.002) and a significantly increased stiffness (P \ 0.001) in all directions. Following artificial joint replacement, there was no significant difference in ROM (P C 0.719), NZ (P C 0.580), and stiffness (P C 0.602) in all directions before and after the fatigue test. The artificial joint showed no signs of wear and tear after the fatigue test. This artificial atlanto-odontoid joint may be useful in cases of odontoid resection due to malunion or nonunion of odontoid fracture, atraumatic odontoid fracture, irreducible atlas dislocation, posterior atlantoaxial subluxation, or congenital skull base abnormalities.
Project description:INTRODUCTION:Recently, a novel hypothesis has been proposed concerning the origin of craniovertebral junction (CVJ) abnormalities. Commonly found in patients with these entities, atlantoaxial instability has been suspected to cause both Chiari malformation type I and basilar invagination, which renders the tried and tested surgical decompression strategy ineffective. In turn, C1-2 fusion is proposed as a single solution for all CVJ abnormalities, and a revised definition of atlantoaxial instability sees patients both with and without radiographic evidence of instability undergo fusion, instead relying on the intraoperative assessment of the atlantoaxial joints to confirm instability. METHODS:The authors conducted a comprehensive narrative review of literature and evidence covering this recently emerged hypothesis. The proposed pathomechanisms are discussed and contextualized with published literature. CONCLUSION:The existing evidence is evaluated for supporting or opposing sole posterior C1-2 fusion in patients with CVJ abnormalities and compared with reported outcomes for conventional surgical strategies such as posterior fossa decompression, occipitocervical fusion, and anterior decompression. At present, there is insufficient evidence supporting the hypothesis of atlantoaxial instability being the common progenitor for CVJ abnormalities. Abolishing tried and tested surgical procedures in favor of a single universal approach would thus be unwarranted.
Project description:Craniocervical junction (CCJ) anomalies and secondary syringomyelia are commonly diagnosed in Cavalier King Charles spaniel (CKCS). Familiarity with the natural history of these abnormalities is vital to understanding the disease syndrome.To evaluate magnetic resonance imaging (MRI) predictors of worsening clinical signs, syringomyelia, and morphology in CKCS longitudinally.Fifty-four client-owned CKCS, 5-13 years old; 50% currently symptomatic.Longitudinal observational study. We enrolled CKCS with an MRI of the CCJ performed ?3 years earlier. We used questionnaires and neurologic examinations to grade initial and current clinical status. Dogs that could be anesthetized were reimaged. Morphologic assessments included the presence and severity of: Chiari-like malformations, medullary position, atlantooccipital overlapping (AOO), dorsal atlantoaxial bands, and syringomyelia. Cranial cavity volumes and foramen magnum height were measured.Clinical status was evaluated in 54 dogs; 36/54 were reimaged. Mean follow-up was 71 months. Of initially asymptomatic dogs, 32% were symptomatic at re-evaluation. Of initially symptomatic dogs, 56% had worsened; 13% had improved with medical management. The morphology of the CCJ at initial imaging did not predict development of either new or worsened signs or syringomyelia by the time of re-evaluation.Craniocervical junction anomalies assessed in this study did not appear predictive of future clinical status or syringomyelia in our cohort. The impacts of syringomyelia, AOO, and atlantoaxial bands on future clinical status merit further study in larger groups of CKCS. Clinical progression in our cohort of medically managed CKCS did not differ substantially from published reports of those treated surgically.
Project description:Study Design Retrospective case review. Objective Atlantoaxial instability with and without basilar invagination poses a considerable challenge in management regarding reduction, surgical approach, decompression, instrumentation choice, and extent of fusion. A variety of strategies have been described to reduce and stabilize cranial settling with basilar invagination. Modern instrumentation options included extension to the occiput, C1-C2 transarticular fixation, and C1 lateral mass-C2 pars among others. Since not all cases of cranial settling are the same, their treatment strategies also differ. Factors such as local vascular anatomy, amount of subluxation, need for distraction, and shape of occipital condyles will dictate level and type of instrumentation. The objective of this study was to outline treatment options and provide a rationale for the surgical plan. Methods Two cases of C1-C2 instability in patients with Down syndrome are described. Case 2 underwent C1-C2 instrumented fusion, whereas case 1 involved posterior instrumented fusion to the occiput. Results Both patients tolerated the procedures well. There were no complications. Minimum follow-up was 1 year. There was no loss of reduction. Solid arthrodesis was achieved in both cases. Conclusion Successful reduction can be achieved with both C1-C2 instrumented fusion as well as O-C instrument fusion. Factors such as local vascular anatomy, amount of subluxation, need for distraction, and shape of occipital condyles will dictate level and type of instrumentation.
Project description:Introduction:Atlantoaxial rotatory subluxation (AARS) is not uncommon in paediatric emergencies, however, the complications might be fatal. Long onset before presentation is correlated with higher recurrence and persistent deformity. There is no consensus on the treatment of AARS yet. Selected patients may benefit from conservative approaches; however, retention might be difficult, and subluxation may recur. Presentation of case:A 6-year-old boy was admitted to our institution with AARS for three months before admission. Typical Cock-Robin position was observed. Computed tomography (CT) indicated AARS Fielding and Hawkins grade III. We treated the case conservatively by closed reduction and cervical traction using Gardner-Wells tongs. However, poor compliance resulted recurrence of subluxation, so we decided to fuse the atlantoaxial joint using transarticular screws, posterior wiring, and autologous bone grafting. Posterior fusion resulted in a satisfactory outcome, in which the wound healed accordingly. Six months of follow up examination revealed normal motoric and sensory function. The patient was able to perform daily activities with no significant issues. Discussion:Patients with fixed deformity of more than three weeks have a higher rate for recurrence or persistent deformity, as reduction is harder and difficult to maintain. The use of posterior wiring alone is limited in maintaining reduction, while using transarticular screws alone is considered better in maintaining reduction; however, not providing it. Conclusion:The use of posterior cervical fusion using C-wire, transarticular screws, and autologous bone grafting may be applied in recurrent case of AARS to ensure adequate reduction and fixation of the atlantoaxial joint.
Project description:Study Design Case report and literature review. Objective To describe a case of nontraumatic atlantoaxial rotatory subluxation (Grisel syndrome) and to review clinical and radiologic aspects, physiopathology, and treatment of this lesion. There is no well-established protocol in the management of patients without spontaneous reduction. The authors discuss the available strategies to achieve reduction and when to operate on these patients. Methods Case presentation of a 7-year-old patient who presented with torticollis ∼1 week after the onset of an upper airway infection. There was no history of head or neck trauma. Computed tomography demonstrated atlantoaxial rotatory subluxation and a normal atlantodental interval. Results The patient was treated with nonsteroidal anti-inflammatory drugs and antibiotics and by progressively increasing the soft cervical collar height. Clinical reduction of the subluxation occurred after 48 hours. He wore the rigid collar for 6 weeks. At that moment, the patient was completely asymptomatic and follow-up cervical spine radiograph demonstrated an anatomical C1-C2 relation. The patient was instructed to return to daily life activities in a gradual manner. Conclusions Grisel syndrome should be considered in the differential diagnosis of torticollis, especially in children. The management can be planned according to the classification of Fielding and Hawkins. The initial treatment involves medicines, injury reduction, and cervical spine immobilization. Surgical treatment is indicated only in cases of failure of conservative treatment, recurrences of subluxation, and irreducible subluxations.
Project description:The posterior screw fixation in atlas via posterior arch and lateral mass, also called C1 "pedicle" screw, combined with C2 pedicle screw fixiation has shown better biomechanical stability in unstable atlantoaxial fractures. However, its popularization has to fulfill the limitation imposed by anatomical characteristics. The aim of this study was to explore the manipulation, effect, and safety of the atlantoaxial transpedicular screw fixation under "direct vision" for the treatment of unstable atlantoaxial fracture.All the patients diagnosed with unstable atlantoaxial fracture, who received surgery treatment of C1,C2 internal fixation from January 2012 to December 2014 were reviewed. Only these patients that were diagnosed with atlantoaxial unstability secondary to trauma and were treated with atlantoaxial transpedicular screw fixation under "direct vision" and iliac autograft were included. The safety of transpedicular screw placement, postoperative outcome, atlantoaxial stability, autograft fusion, and complications was observed and analyzed retrospectively. The pain visual analog scale (VAS) and the Japanese Orthopedic Association (JOA) score were used as surgical curative effect evaluation standards.We reviewed a total of 92 patients diagnosed with unstable atlantoaxial fracture, who received surgery treatment of C1,C2 internal fixation from January 2012 to December 2014, and 87 patients were treated with atlantoaxial transpedicular screw fixation under "direct vision" and were included this analysis. A total of 306 transpedicular screws in atlas and axis were placed successfully. All cases were followed-up >12 months. The overall breach rate was 11.36%. None of the breaches resulted in new-onset neurological sequela. The neurological status in cases with bilateral upper extremities numbness and lower extremities weakness had improved after surgery. At the latest follow-up, the neck VAS and JOA scores were significantly improved (P?<?.01) than those preoperatively. No cases demonstrated implantation failure and bone graft absorption on the postoperative x-ray films and CT scans.Atlantoaxial transpedicular screw fixation under "direct vision" and iliac autograft for the treatment of unstable atlantoaxial fracture has shown simple manipulation and efficient performance. Thus, the technique of C1-C2 fixation is feasible in treating unstable atlantoaxial fracture.
Project description:Background:Ulnar nerve entrapment at the elbow (UNE) is overrepresented in patients with diabetes, but the outcome of surgery is unknown. We aimed to evaluate patient-reported outcome in patients with and without diabetes, and to assess potential sex differences and compare surgical treatment methods. Methods:Data on patients operated for UNE (2010-2016, n = 1354) from the Swedish National Registry for Hand Surgery were linked to the Swedish National Diabetes Register. Symptoms were assessed preoperatively (n = 389), and 3 (n = 283), and at 12 months postoperatively (n = 267) by QuickDASH and HQ-8 (specific hand surgery questionnaire-8 questions). Only simple decompressions were included when comparing groups. Results:Men with diabetes reported higher postoperative QuickDASH scores than men without diabetes. Women scored their disability higher than men on all time-points in QuickDASH, but showed larger improvement between preoperative and 12 months postoperative values. Patients operated with transposition scored 10.8 points higher on QuickDASH than patients who had simple decompression at 12 months (95% confidence interval 1.98-19.6). Conclusions:Women with diabetes benefit from simple decompression for UNE to the same extent as women without diabetes. Men with diabetes risk not to benefit from simple decompression as much as women do. Ulnar nerve transposition had a higher risk of residual symptoms compared to simple decompression.