scholarly journals Reduction of atlantoaxial dislocation prevented by pathological position of the transverse ligament in fixed, irreducible os odontoideum: operative illustrations and radiographic correlates in 41 patients

2017 ◽  
Vol 27 (1) ◽  
pp. 20-28 ◽  
Author(s):  
Brian J. Dlouhy ◽  
Bruno A. Policeni ◽  
Arnold H. Menezes
Keyword(s):  
Down Syndrome ◽  
Surgical Treatment ◽  
Pediatric Patients ◽  
Clinical Presentation ◽  
Odontoid Process ◽  
Atlantoaxial Dislocation ◽  
Os Odontoideum ◽  
Transverse Ligament ◽  
Radiographic Findings ◽  
Hyperintense Signal

OBJECTIVEOs odontoideum (OO) is a craniovertebral junction (CVJ) abnormality in which an ossicle (small bone) is cranial to a hypoplastic dens by a variable gap. This abnormality can result in instability, which may be reducible or irreducible. What leads to irreducibility in OO is unclear. Therefore, the authors sought to better understand the causes of irreducibility in OO.METHODSA retrospective review was conducted, which identified more than 200 patients who had undergone surgical treatment for OO between 1978 and 2015 at the University of Iowa Hospitals and Clinics. Only the 41 patients who had irreducible OO were included in this study. All inpatient and outpatient records were retrospectively reviewed, and patient demographics, clinical presentation, radiographic findings, surgical treatment, and operative findings were recorded and analyzed.RESULTSThe cohort of 41 patients who were found to have irreducible OO included both children and adults. A majority of patients were adults (61% were 18 years or older). Clinical presentation included neck pain and headache in the majority of patients (93%). Weakness, sensory disturbances, and myelopathy were invariably present in all 41 patients (100%). Down syndrome was much more common in the pediatric cohort than in the adult cohort; of the 16 pediatric patients, 6 had Down syndrome (38%), and none of the adults did. Of the 16 pediatric patients, 5 had segmentation failure (31%) in the subaxial spine, and none of the adults did. A form of atlantoaxial dislocation was seen in all cases. On CT imaging, atlantoaxial facets were dislocated in all 41 cases but did not have osseous changes that would have prevented reduction. On MRI, the transverse ligament was identified anterior and inferior to the ossicle and superior to the hypoplastic odontoid process in all cases in which these studies were available (i.e., post-MRI era; 36 of 36 cases). The ligament was hypointense on T2-weighted images but also had an associated hyperintense signal on T2 images. Intraoperatively, the transverse ligament was identified anterior and inferior to the ossicle and superior to the hypoplastic odontoid process in all 41 cases.CONCLUSIONSIn the largest series to date of irreducible OO and the only study to examine variable factors that lead to irreducibility in OO, the authors found that the position of the transverse ligament anterior and inferior to the ossicle is the most common factor in the irreducibility of OO. The presence of granulation tissue and of the dystopic variant of OO is also associated with irreducibility. The presence of Down syndrome and segmentation failure probably leads to faster progression of ligamentous incompetence and therefore earlier presentation of instability and irreducibility. This is the first study in which intraoperative findings regarding the transverse ligament have been correlated with MRI.

scholarly journals Surgical treatment for basilar invagination with irreducible atlantoaxial dislocation: transoral atlantoaxial reduction plate fixation vs occipitocervical fixation

2020 ◽  
Vol 21 (1) ◽  
Author(s):  
Xiaobao Zou ◽  
Bieping Ouyang ◽  
Haozhi Yang ◽  
Binbin Wang ◽  
Su Ge ◽  
...  
Keyword(s):  
Surgical Treatment ◽  
Early Stage ◽  
Plate Fixation ◽  
Japanese Orthopaedic Association ◽  
Odontoid Process ◽  
Basilar Invagination ◽  
Atlantoaxial Dislocation ◽  
Clinical Effects ◽  
Occipitocervical Fixation ◽  
Irreducible Atlantoaxial Dislocation

Abstract Background Transoral atlantoaxial reduction plate (TARP) fixation or occipitocervical fixation (OF) is an effective treatment for basilar invagination (BI) with irreducible atlantoaxial dislocation (IAAD). But, all current clinical studies involved a single surgical procedure. The clinical effects of TARP and OF operation for BI with IAAD have yet to be compared. We therefore present this report to compare the treatment of TARP and OF procedure for BI with IAAD. Methods Fifty-six patients with BI with IAAD who underwent TARP or OF operation from June 2011 to June 2017 were retrospectively analyzed. Among these, 35 patients underwent TARP operation (TARP group), and 21 patients underwent OF operation (OF group). We compared the difference of clinical, radiological, and surgical outcomes between the TARP and OF groups postoperatively. Results Compared with OF group, the operative time and blood loss in TARP group were lower. There was no statistical difference in the atlantodental interval (ADI), clivus canal angle (CCA), cervicomedullary angle (CMA), distance between the top of the odontoid process and the Chamberlain line (CL) and Japanese Orthopaedic Association (JOA) score between the TARP and OF groups preoperatively, but the improvements of these parameters in the TARP group were superior to those in the OF group postoperatively. The fusion rates were higher in the TARP group than those in the OF group at the early stage postoperatively. Conclusions TARP and OF operations are effective surgical treatment for BI with IAAD, but the performance of reduction and decompression and earlier bone fusion rates of TARP procedure are superior to those of OF.

Surgical treatment of neglected C2 odontoid process fracture with anterior atlantoaxial dislocation

2020 ◽  
pp. 1-5
Author(s):  
Vladimir Klimov ◽  
Murodzhon Kosimshoev ◽  
Aleksey Evsyukov ◽  
Vitaly Stepanenko ◽  
Jamil Rzaev
Keyword(s):  
Surgical Treatment ◽  
Odontoid Process ◽  
Atlantoaxial Dislocation ◽  
Odontoid Process Fracture

scholarly journals Atlantoaxial dislocation with congenital “sandwich fusion” in the craniovertebral junction: a retrospective case series of 70 patients

2020 ◽  
Vol 21 (1) ◽  
Author(s):  
Yinglun Tian ◽  
Nanfang Xu ◽  
Ming Yan ◽  
Peter G. Passias ◽  
Frank A. Segreto ◽  
...  
Keyword(s):  
Surgical Treatment ◽  
Clinical Features ◽  
Japanese Orthopaedic Association ◽  
Case Series ◽  
Atlantoaxial Dislocation ◽  
Os Odontoideum ◽  
Cranial Neuropathy ◽  
Atlantoaxial Joint ◽  
The Mean

Abstract Background In the setting of congenital C1 occipitalization and C2–3 fusion, significant strain is placed on the atlantoaxial joint. Vertebral fusion both above and below the atlantoaxial joint (i.e., a “sandwich”) creates substantial instability. We retrospectively report on a case series of “sandwich fusion” atlantoaxial dislocation (AAD), describing the associated clinical characteristics and detailing surgical treatment. To the best of our knowledge, the present study is the largest investigation to date of this congenital subgroup of AAD. Methods Seventy consecutive patients with sandwich fusion AAD, from one senior surgeon, were retrospectively reviewed. The clinical features and the surgical treatment results were assessed using descriptive statistics. No funding sources or potential conflict of interest-associated biases exist. Results The mean patient age was 42.2 years (range: 5–77 years); 36 patients were male, and 34 were female. Fifty-eight patients (82.9%) had myelopathy, with Japanese Orthopaedic Association (JOA) scores ranging 4–16 (mean: 12.9). Cranial neuropathy was involved in 10 cases (14.3%). The most common presentation age group was 31 to 40 years (24 cases, 34.3%). Radiological findings revealed brainstem and/or cervical-medullar compression (58 cases, 82.9%), syringomyelia (16 cases, 22.9%), Chiari malformation (12 cases, 17.1%), cervical spinal stenosis (10 cases, 14.3%), high scapula deformity (1 case, 1.4%), os odontoideum (1 case, 1.4%), and dysplasia of the atlas (1 case, 1.4%). Computed tomography angiography was performed in 27 cases, and vertebral artery (VA) anomalies were identified in 14 cases (51.9%). All 70 patients underwent surgical treatment, without spinal cord or VA injury. Four patients (5.7%) suffered complications, including 1 wound infection, 1 screw loosening, and 2 cases of bulbar paralysis. In the 58 patients with myelopathy, the mean JOA score increased from 12.9 to 14.5. The average follow-up time was 50.5 months (range: 24–120 months). All 70 cases achieved solid atlantoaxial fusion at the final follow-up. Conclusions Sandwich fusion AAD, a unique subgroup of AAD, has distinctive clinical features and associated malformations such as cervical-medullar compression, syringomyelia, and VA anomalies. Surgical treatment of AAD was associated with myelopathy improvement and minimal complication occurrence.

Clinical Presentation and Stroke Incidence Differ by Moyamoya Etiology

2020 ◽  
pp. 088307382096716
Author(s):  
John R. Gatti ◽  
Adriana Gonzalez Torriente ◽  
Lisa R. Sun
Keyword(s):  
Radiation Therapy ◽  
Down Syndrome ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Clinical Presentation ◽  
Cell Disease ◽  
Stroke Incidence ◽  
Radiographic Findings ◽  
Cranial Radiation

Introduction: Moyamoya arteriopathy, which can be idiopathic or associated with sickle cell disease, neurofibromatosis, Down syndrome, or cranial radiation therapy, is a progressive cerebral arteriopathy associated with high rates of incident and recurrent stroke. Little is known about how these subgroups differ with respect to clinical presentation, radiographic findings, stroke risk, and functional outcomes. Methods: Using ICD codes, we identified children ages 28 days to 18 years treated for moyamoya arteriopathy at our tertiary care center between 2003 and 2019. Demographic, clinical, and radiographic data were extracted from the medical record. The Pediatric Stroke Recurrence and Recovery Questionnaire was administered to consenting participants. Results: Sixty-nine patients met inclusion criteria (33 idiopathic, 18 sickle cell disease, 11 neurofibromatosis, 6 Down syndrome, 1 cranial radiation therapy). Median follow-up time was 7.7 years; 24 patients had at least 5 years of follow-up data. Frequency of stroke at presentation differed by subgroup ( P < .001). Of patients with at least 2 years of follow-up, 33 (55%) experienced stroke. The proportion of patients experiencing stroke differed by subgroup (50% of idiopathic cases, 72% of sickle cell disease, 11% of neurofibromatosis, and 100% of Down syndrome, P = .003). The frequency of bilateral versus unilateral disease ( P = .001) and stroke-free survival following presentation ( P = .01) also differed by subgroup. Conclusions: In this single-center cohort, moyamoya subgroups differed with respect to clinical and radiographic characteristics, with neurofibromatosis-associated moyamoya syndrome having a milder phenotype and Down syndrome–associated moyamoya portending a more aggressive course. These findings need confirmation in a larger, multi-center cohort with longer duration of follow-up.

Intracranial arachnoid cysts in children

1986 ◽  
Vol 64 (6) ◽  
pp. 835-842 ◽  
Author(s):  
Griffith Rutherford Harsh ◽  
Michael S. B. Edwards ◽  
Charles B. Wilson
Keyword(s):  
Magnetic Resonance Imaging ◽  
Pediatric Patients ◽  
Clinical Presentation ◽  
Arachnoid Cysts ◽  
Anatomical Location ◽  
Resonance Imaging ◽  
Cyst Size ◽  
Radiographic Findings ◽  
Content Type ◽  
Fine Print

✓ The clinical and radiographic findings, surgical treatment, and outcome in 16 pediatric patients with intracranial arachnoid cysts are reviewed. The clinical presentation reflected the anatomical location of the lesions. Computerized tomography or magnetic resonance imaging scans were diagnostic in all cases. Of the nine cysts treated primarily or secondarily by craniotomy for fenestration and drainage into the basilar cisterns, five recurred. Cyst-peritoneal shunting led to diminished cyst size and clinical improvement in all seven cases in which it was used as the initial treatment and in all four cases in which fenestration had been unsuccessful. The results in this series show that cyst-peritoneal shunting is the treatment of choice for most intracranial arachnoid cysts in children.

Hydrocephalus in Spinal Muscular Atrophy: A Case Report and Review of the Literature

2020 ◽  
Author(s):  
Jeetendra P. Sah ◽  
Aaron W. Abrams ◽  
Geetha Chari ◽  
Craig Linden ◽  
Yaacov Anziska
Keyword(s):  
Spinal Muscular Atrophy ◽  
Clinical Characteristics ◽  
Clinical Presentation ◽  
Muscular Atrophy ◽  
Communicating Hydrocephalus ◽  
Type I ◽  
Review Of The Literature ◽  
Radiographic Findings ◽  
Comprehensive Review ◽  
The Relationship

AbstractIn this article, we reported a case of spinal muscular atrophy (SMA) type I noted to have tetraventricular hydrocephalus with Blake's pouch cyst at 8 months of age following intrathecal nusinersen therapy. The association of hydrocephalus with SMA is rarely reported in the literature. Development of hydrocephalus after intrathecal nusinersen therapy is also reported in some cases, but a cause–effect relationship is not yet established. The aim of this study was to describe the clinical characteristics of a patient with SMA type I and hydrocephalus, to review similar cases reported in the literature, and to explore the relationship between nusinersen therapy and development of hydrocephalus. The clinical presentation and radiographic findings of the patient are described and a comprehensive review of the literature was conducted. The adverse effect of communicating hydrocephalus related to nusinersen therapy is being reported and the authors suggest carefully monitoring for features of hydrocephalus developing during the course of nusinersen therapy.

Long-term outcomes of lumbar microdiscectomy in the pediatric population: a large single-institution case series

2019 ◽  
Vol 24 (5) ◽  
pp. 549-557
Author(s):  
Malia McAvoy ◽  
Heather J. McCrea ◽  
Vamsidhar Chavakula ◽  
Hoon Choi ◽  
Wenya Linda Bi ◽  
...  
Keyword(s):  
Conservative Management ◽  
Functional Outcomes ◽  
Pediatric Patients ◽  
Clinical Presentation ◽  
Case Series ◽  
Csf Leak ◽  
Single Institution ◽  
The Mean

OBJECTIVEFew studies describe long-term functional outcomes of pediatric patients who have undergone lumbar microdiscectomy (LMD) because of the rarity of pediatric disc herniation and the short follow-up periods. The authors analyzed risk factors, clinical presentation, complications, and functional outcomes of a single-institution series of LMD patients over a 19-year period.METHODSA retrospective case series was conducted of pediatric LMD patients at a large pediatric academic hospital from 1998 to 2017. The authors examined premorbid risk factors, clinical presentation, physical examination findings, type and duration of conservative management, indications for surgical intervention, complications, and postoperative outcomes.RESULTSOver the 19-year study period, 199 patients underwent LMD at the authors’ institution. The mean age at presentation was 16.0 years (range 12–18 years), and 55.8% were female. Of these patients, 70.9% participated in competitive sports, and among those who did not play sports, 65.0% had a body mass index greater than 25 kg/m2. Prior to surgery, conservative management had failed in 98.0% of the patients. Only 3 patients (1.5%) presented with cauda equina syndrome requiring emergent microdiscectomy. Complications included 4 cases of postoperative CSF leak (2.0%), 1 case of a noted intraoperative CSF leak, and 3 cases of wound infection (1.5%). At the first postoperative follow-up appointment, minimal or no pain was reported by 93.3% of patients. The mean time to return to sports was 9.8 weeks. During a mean follow-up duration of 8.2 years, 72.9% of patients did not present again after routine postoperative appointments. The total risk of reoperation was a rate of 7.5% (3.5% of patients underwent reoperation for the same level; 4.5% underwent adjacent-level decompression, and one patient [0.5%] ultimately underwent a fusion).CONCLUSIONSMicrodiscectomy is a safe and effective treatment for long-term relief of pain and return to daily activities among pediatric patients with symptomatic lumbar disc disease in whom conservative management has failed.

scholarly journals Epilepsy in Down Syndrome: A Highly Prevalent Comorbidity

2021 ◽  
Vol 10 (13) ◽  
pp. 2776
Author(s):  
Miren Altuna ◽  
Sandra Giménez ◽  
Juan Fortea
Keyword(s):  
Down Syndrome ◽  
Functional Outcomes ◽  
Clinical Presentation ◽  
Late Onset ◽  
Current Knowledge ◽  
Epileptic Seizures ◽  
Myoclonic Epilepsy ◽  
Increased Risk ◽  
Number Of Individuals

Individuals with Down syndrome (DS) have an increased risk for epilepsy during the whole lifespan, but especially after age 40 years. The increase in the number of individuals with DS living into late middle age due to improved health care is resulting in an increase in epilepsy prevalence in this population. However, these epileptic seizures are probably underdiagnosed and inadequately treated. This late onset epilepsy is linked to the development of symptomatic Alzheimer’s disease (AD), which is the main comorbidity in adults with DS with a cumulative incidence of more than 90% of adults by the seventh decade. More than 50% of patients with DS and AD dementia will most likely develop epilepsy, which in this context has a specific clinical presentation in the form of generalized myoclonic epilepsy. This epilepsy, named late onset myoclonic epilepsy (LOMEDS) affects the quality of life, might be associated with worse cognitive and functional outcomes in patients with AD dementia and has an impact on mortality. This review aims to summarize the current knowledge about the clinical and electrophysiological characteristics, diagnosis and treatment of epileptic seizures in the DS population, with a special emphasis on LOMEDS. Raised awareness and a better understanding of epilepsy in DS from families, caregivers and clinicians could enable earlier diagnoses and better treatments for individuals with DS.

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