Idiopathic syringomyelia: retrospective case series, comprehensive review, and update on management

2011 ◽  
Vol 31 (6) ◽  
pp. E15 ◽  
Author(s):  
Anil K. Roy ◽  
Nicholas P. Slimack ◽  
Aruna Ganju
Keyword(s):  
Conservative Management ◽  
Incidental Finding ◽  
Imaging Techniques ◽  
Case Series ◽  
Neurological Dysfunction ◽  
Type I ◽  
Retrospective Case ◽  
Chiari Malformation Type I ◽  
Comprehensive Review ◽  
Idiopathic Syringomyelia

Object A syrinx is a fluid-filled cavity within the spinal cord that can be an incidental finding or it can be accompanied by symptoms of pain and temperature insensitivity. Although it is most commonly associated with Chiari malformation Type I, the advancement of imaging techniques has resulted in more incidental idiopathic syringes that are not associated with Chiari, tumor, trauma, or postinfectious causes. The authors present a comprehensive review and management strategies for the idiopathic variant of syringomyelia. Methods The authors retrospectively identified 8 idiopathic cases of syringomyelia at their institution during the last 6 years. A PubMed/Medline literature review yielded an additional 38 articles. Results Two of the authors' patients underwent surgical treatment that included a combination of laminectomy, lysis of adhesions, duraplasty, and syrinx fenestration. The remaining 6 patients were treated conservatively and had neurologically stable outcomes. Review of the literature suggests that an etiology-driven approach is essential in the diagnosis and management of syringomyelia, although conservative management suffices for most cases. In particular, it is important to look at disturbances in CSF flow, as well as structural abnormalities including arachnoid webs, cysts, scars, and a diminutive posterior fossa. Conclusions The precise etiology for idiopathic syringomyelia (IS) is still unclear, although conceptual advances have been made toward the overall understanding of the pathophysiology of IS. Various theories include the cerebellar piston theory, intramedullary pulse pressure theory, and increased spinal subarachnoid pressure. For most patients with IS, conservative management works well. Continued progression of symptoms, however, could be approached using decompressive strategies such as laminectomy, lysis of adhesions, and craniocervical decompression, depending on the level of pathology. Management for patients with progressive neurological dysfunction and the lack of flow disturbance is unclear, although syringosubarachnoid shunting can be considered.

Progressive Neurological Deficit in Adult Idiopathic Syringomyelia (IS): Case Report and Literature Review

2016 ◽  
Vol 1 (3) ◽  
Author(s):  
Farid Yudoyono ◽  
Anugrah O. Widhiatmo ◽  
Beny A. Wirjomartani
Keyword(s):  
Case Report ◽  
Literature Review ◽  
Neurological Deficit ◽  
Chiari Malformation ◽  
Imaging Techniques ◽  
Lower Extremities ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Progressive Neurological Deficit ◽  
Idiopathic Syringomyelia

A syringomyelia is a clinical entity of any tubular fluid-filled cavity within the spinalcord that causes slowly but relentlessly progressive symptoms as well as expansion of thecavity, which is most commonly associated with Chiari malformation Type I, which due toadvancement of imaging techniques has resulted in more incidental idiopathic syringes that arenot associated with tumor, trauma, or postinfectious causes. Idiopathic syringomyelia (IS) is apathological entity in which no overt etiology is evident for a syrinx. In this study, we describe acase in a 45 year-old woman presented with progressive difficulties in walking and also hadmyelopathic signs evidenced by hyperreflexia in the lower extremities and underwent foramenmagnum decompression and C1 lamiectomy. Idiopathic syringomyelia is a pathological entityin which no overt etiology is evident for a syrinx. It can be managed succesfully byconservative treatment but if there is a progression of neurological deficit, surgicaldecompression is a mandatory.Keywords: idiopathic syringomyelia, neurological deficit

Incidental mastoiditis on magnetic resonance imaging scans: clinical relevance and cost implications

2018 ◽  
Vol 132 (11) ◽  
pp. 1010-1012 ◽  
Author(s):  
Y Abbas ◽  
H S Yuen ◽  
A Trinidade ◽  
G Watters
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Incidental Finding ◽  
Case Series ◽  
Resonance Imaging ◽  
Retrospective Case ◽  
Reporting Practice ◽  
Significant Pathology ◽  
Cost Implications ◽  
Rule Out

AbstractObjectivesTo determine: (1) the incidence of incidental ‘mastoiditis’ reported on magnetic resonance imaging scans performed in patients with asymmetrical sensorineural hearing loss and/or unilateral tinnitus; (2) how many of those patients have actual otological pathology and/or require treatment; and (3) the financial implications of such a reporting practice.MethodRetrospective case series.ResultsBetween October 2015 and November 2016, 500 patients underwent magnetic resonance imaging of the internal auditory meatus to rule out cerebellopontine angle lesions. There was an incidental finding of increased mastoid signalling in 5.8 per cent (n= 29), of which 20.7 per cent (6 of 29) were reported as bilateral cases. The diagnosis of ‘mastoiditis’ was found in 39.7 per cent (29 of 73). None of these patients had any pathology identified clinically. Other significant pathology was identified in a further 8.8 per cent (n= 44).ConclusionThe diagnosis of mastoiditis is primarily clinical. An incidental finding of high signalling in the mastoid region on magnetic resonance imaging is highly unlikely to represent actual clinical disease. In patients who are scanned for other reasons and who do not complain of otological symptoms, such findings are unlikely to require otolaryngology input.

SP6.1.10 Can gallbladder polyp surveillance be justified? Experience of a large DGH

2021 ◽  
Vol 108 (Supplement_7) ◽  
Author(s):  
Nicholas Bradley ◽  
Michael Wilson ◽  
Christopher Shearer ◽  
Timothy Heron ◽  
Katie Robertson ◽  
...  
Keyword(s):  
Incidental Finding ◽  
Case Series ◽  
District General Hospital ◽  
Pathological Examination ◽  
Histopathological Analysis ◽  
Low Grade ◽  
Retrospective Case ◽  
Gallbladder Polyps ◽  
Abnormal Pathology ◽  
Gallbladder Malignancy

Abstract Aims Gallbladder polyps are typically an incidental finding on sonographic or pathological examination with an estimated prevalence of 0.3 – 9.5%. Their role as a precursor to gallbladder malignancy is disputed. The 2017 European Joint Society Guidelines (ESGAR/EAES/EFISDS/ESGE) aim to standardise management of gallbladder polyps detected prior to cholecystectomy. We aim to describe our experience in the management of gallbladder polyps in a district general hospital. Methods This single centre retrospective case series included consecutive cholecystectomies over an 8-year period, identified through pathology records. Medical records were interrogated to identify the presence of gallbladder polyps identified pre-operatively and at subsequent histopathological analysis. Results 3835 cases were included. Four cases (0.1%) had an adenocarcinoma identified; none of these had pre-operatively identified polyps. Two cases (0.05%) were found to have lesions with high-grade dysplasia, 1 of which had a 17mm polyp preoperatively. One case (0.03%) had cellular atypia, without a pre-operative polyp. Forty six cases (1.2%) were found to have low-grade dysplasia; 1 (2.2%) of these had adenomyomatosis pre-operatively but none had pre-operative polyps. Overall, 53 patients (1.4%) had abnormal pathology with only 1 (1.9%) of these having a pre-operative polyp identified. Conclusions Our results suggest that in the real-world setting, intensive surveillance of gallbladder polyps has limited utility in identifying cases of gallbladder malignancy. The burden on services produced by adherence to strict surveillance guidelines is difficult to justify and a less arduous approach is unlikely to significantly influence the management or outcome in patients with gallbladder malignancy.

CRPS of Lower Extremity– A Case Report and Review

2016 ◽  
Vol 27 (1) ◽  
pp. 22-28
Author(s):  
Mahima Agrawal
Keyword(s):  
Case Report ◽  
Lower Extremity ◽  
Lower Limb ◽  
Relaxation Training ◽  
Single Case ◽  
Case Series ◽  
Pain Syndrome ◽  
Type I ◽  
Retrospective Case ◽  
Single Case Report

Abstract Complex regional pain syndrome (CRPS) of the lower limb is a relatively uncommon entity as compared to CRPS of the upper extremity. Literature search has revealed only 2 retrospective case series and a single case report of lower extremity CRPS type I from 1975 to 2014 on Pubmed, isolated cases of CRPS type I of lower extremity have also been reported following knee surgeries and arthroscopies. This report presents a case of lower limb CRPS type I following blunt trauma to right foot, treatment of which was directed towards management of allodynia, vasomotor symptoms and surgical correction of deformity which had developed because of the disease, coping mechanisms were also reinforced through counselling and relaxation training. The individual responded well to treatment with a reported 75% reduction in the disabling symptoms and improvement in ambulatory status.

Surgical outcomes in adult patients with syringomyelia associated with Chiari malformation Type I: the relationship between scoliosis and neurological findings

2007 ◽  
Vol 6 (3) ◽  
pp. 216-221 ◽  
Author(s):  
Atsushi Ono ◽  
Futoshi Suetsuna ◽  
Kazumasa Ueyama ◽  
Toru Yokoyama ◽  
Shuichi Aburakawa ◽  
...  
Keyword(s):  
Chiari Malformation ◽  
Clinical Status ◽  
Japanese Orthopaedic Association ◽  
Foramen Magnum ◽  
Neurological Dysfunction ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Group A ◽  
The Mean ◽  
Group B

Object The clinical characteristics of pediatric scoliosis associated with syringomyelia have been reported in previous studies, but scoliosis associated with syringomyelia in adults is rarely treated, and there is a paucity of detailed studies. In the present study of adult syringomyelia associated with Chiari malformation Type I, the authors investigated the relationships among the syrinx, scoliosis, and neurological data. Methods The population was composed of 27 patients (≥ 20 years of age) who underwent foramen magnum decompression for the treatment of syringomyelia. The patients were divided into two groups: those with scoliosis of 10° or more (Group A) and those without scoliosis (Group B). The authors assessed the length of the syrinx, duration of morbidity, and clinical status before and after surgery based on the Japanese Orthopaedic Association (JOA) Scale. There were 15 cases in Group A and 12 in Group B. The mean length of the syrinx was 12.8 vertebral bodies (VBs) in Group A and 7.2 VBs in Group B. The mean duration of morbidity was 14.2 years in Group A and 6.8 years in Group B. The mean preoperative JOA score was 10.1 in Group A and 14.4 in Group B, whereas the mean postoperative JOA scores were 11.9 and 15.8, respectively. There were significant differences between Groups A and B in length of the syrinx, duration of morbidity, and pre- and postoperative JOA scores. Conclusions In patients with syringomyelia and scoliosis the syringes spanned a greater number of VBs, the duration of morbidity was greater, neurological dysfunction was more severe, and surgical results were poorer. Scoliosis could be a predicting factor of the prognosis in patients with syringomyelia and Chiari malformation Type I.

Sever’s Disease: What Does the Literature Really Tell Us?

2008 ◽  
Vol 98 (3) ◽  
pp. 212-223 ◽  
Author(s):  
Rolf W. Scharfbillig ◽  
Sara Jones ◽  
Sheila D. Scutter
Keyword(s):  
Health Professionals ◽  
Case Series ◽  
Review Of The Literature ◽  
Retrospective Case ◽  
Level Of Evidence ◽  
Comprehensive Review ◽  
Musculoskeletal Conditions ◽  
Low Level ◽  
Retrospective Case Series

Background: Sever’s disease is typical of many musculoskeletal conditions where observational annotations have slowly been accepted as fact with the passing of years. Acceptance of these nontested observations means that health professionals seeking information on this condition access very low-level evidence, mainly being respectable opinion or poorly conducted retrospective case series. Methods: A comprehensive review of the literature was undertaken gathering available articles and book references relating to Sever’s disease. This information was then reviewed to present what is actually known about this condition. Results: Respectable opinion and poorly conducted retrospective case series make up the majority of evidence on this condition. Conclusion: The level of evidence for most of what we purport to know about Sever’s disease is at such a level that prospective, well-designed studies are a necessity to allow any confidence in describing this condition and its treatment. (J Am Podiatr Med Assoc 98(3): 212–223, 2008)

Absent right superior caval vein in situs solitus

2015 ◽  
Vol 26 (2) ◽  
pp. 334-339 ◽  
Author(s):  
Rebekka Lytzen ◽  
Karin Sundberg ◽  
Niels Vejlstrup
Keyword(s):  
Incidental Finding ◽  
Clinical Manifestations ◽  
Case Series ◽  
Retrospective Case ◽  
Caval Vein ◽  
Cardinal Vein ◽  
Benign Condition ◽  
Superior Caval Vein ◽  
Case Series Study ◽  
The Right

AbstractIntroductionIn up to 0.07% of the general population, the right anterior cardinal vein obliterates and the left remains open, creating an absent right superior caval vein and a persistent left superior caval vein. Absent right superior caval vein is associated with additional congenital heart disease in about half the patients. We wished to study the consequences of absent right superior caval vein as an incidental finding on prenatal ultrasonic malformation screening.Material and methodsThis is a retrospective case series study of all foetuses diagnosed with absent right superior caval vein at the national referral hospital, Rigshospitalet, Denmark, from 2009 to 2012.ResultsIn total, five cases of absent right superior caval vein were reviewed. No significant associated cardiac, extra-cardiac, or genetic anomalies were found. Postnatal echocardiographies confirmed the diagnosis and there were no postnatal complications. All children were found to have healthy hearts at follow-up.ConclusionsIn all cases, the findings proved to be a benign condition with no clinical manifestations or complications. Although isolated absent right superior caval vein does not seem to affect the outcome, associated anomalies may be serious. Absent right superior caval vein should, therefore, prompt a search for additional malformations. Furthermore, the diagnosis of an isolated absent right superior caval vein is important, because knowledge of the anomaly can prevent future problems when invasive procedures are necessary.

Familial Chiari malformation: case series

2011 ◽  
Vol 31 (3) ◽  
pp. E1 ◽  
Author(s):  
Benjamin D. Schanker ◽  
Brian P. Walcott ◽  
Brian V. Nahed ◽  
Kristopher T. Kahle ◽  
Yan Michael Li ◽  
...  
Keyword(s):  
Chiari Malformation ◽  
Familial Aggregation ◽  
Association Studies ◽  
Case Reports ◽  
Case Series ◽  
Twin Studies ◽  
Type I ◽  
Genome Wide Association Studies ◽  
Chiari Malformation Type I ◽  
Genome Wide

Chiari malformations (Types I–IV) are abnormalities of the posterior fossa that affect the cerebellum, brainstem, and the spinal cord with prevalence rates of 0.1%–0.5%. Case reports of familial aggregation of Chiari malformation, twin studies, cosegregation of Chiari malformation with known genetic conditions, and recent gene and genome-wide association studies provide strong evidence of the genetic underpinnings of familial Chiari malformation. The authors report on a series of 3 family pairs with Chiari malformation Type I: 2 mother-daughter pairs and 1 father-daughter pair. The specific genetic causes of familial Chiari malformation have yet to be fully elucidated. The authors review the literature and discuss several candidate genes. Recent advances in the understanding of the genetic influences and pathogenesis of familial Chiari malformation are expected to improve management of affected patients and monitoring of at-risk family members.

scholarly journals Attenuated androgen discontinuation in patients with hereditary angioedema: a commented case series

2022 ◽  
Vol 18 (1) ◽  
Author(s):  
Marcus Maurer ◽  
Markus Magerl ◽  
Emel Aygören-Pürsün ◽  
Konrad Bork ◽  
Henriette Farkas ◽  
...  
Keyword(s):  
Side Effects ◽  
Hereditary Angioedema ◽  
Treatment Strategies ◽  
Case Series ◽  
Additional Patient ◽  
Type I ◽  
Retrospective Case ◽  
Androgen Withdrawal ◽  
Life Threatening ◽  
Attenuated Androgen

Abstract Background Hereditary angioedema (HAE) is characterized by potentially severe and life-threatening attacks of localized swelling. Prophylactic therapies are available, including attenuated androgens. Efficacy of attenuated androgens has not been assessed in large, randomized, placebo-controlled trials and can be associated with frequent, and sometimes severe, side effects. As better tolerated targeted therapies become available, attenuated androgen withdrawal is increasingly considered by physicians and their patients with HAE. Attenuated androgens withdrawal has not been systematically studied in HAE, although examination of other disorders indicates that attenuated androgen withdrawal may result in mood disturbances and flu-like symptoms. Standardized protocols for attenuated androgen discontinuation that continue to provide control of attacks while limiting potential attenuated androgen withdrawal symptoms are not established as the outcomes of different withdrawal strategies have not been compared. We aim to describe the challenges of attenuated androgen discontinuation in patients with HAE and how these may continue into the post-androgen period. Case presentation We present a retrospective case series of 10 patients with confirmed type I HAE who have discontinued prophylactic treatment with attenuated androgens. The most common reason for attenuated androgen discontinuation was side effects. Attenuated androgens were either immediately withdrawn, tapered and/or overlapped with another treatment. The major challenge of discontinuation was the management of an increased frequency and severity of HAE attacks in some patients. Conclusions Healthcare teams need to undertake careful planning and monitoring after attenuated androgens discontinuation, and modify treatment strategies if HAE control is destabilized with an increased number of attacks. Discontinuation of attenuated androgens is definitively an option in an evolving HAE treatment landscape, and outcomes can be favourable with additional patient support and education.

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