scholarly journals Syringomyelia in children with closed spinal dysraphism: long-term outcomes after surgical intervention

2020 ◽  
Vol 25 (3) ◽  
pp. 319-325 ◽  
Author(s):  
Amy K. Bruzek ◽  
Jordan Starr ◽  
Hugh J. L. Garton ◽  
Karin M. Muraszko ◽  
Cormac O. Maher ◽  
...  

OBJECTIVEThe nature of the relationship between spinal cord syrinx and tethered cord is not well known. It is unclear if surgical cord untethering results in resolution or improvement of an associated syrinx. The objective of this study was to report the response of spinal cord syrinx to surgical cord untethering.METHODSThe authors retrospectively reviewed all patients with a syrinx and tethered cord who presented to a single institution over an 11-year interval. Patients with open neural tube defects were excluded. Thirty-one patients were identified, 25 of whom had both clinical and imaging follow-up after surgery. Patients were grouped according to etiology of the tethered cord. Clinical outcomes and syrinx characteristics were recorded.RESULTSOf the 25 patients with tethered cord, 68% (n = 17) were male. The average age at presentation was 2.5 years (0–10.1 years) and age at surgery was 3.7 years (range 1 day to 17 years). Etiologies of tethered cord were lipomyelomeningocele (n = 8), thickened/fatty filum (n = 7), intradural lipoma (n = 5), myelocystocele (n = 2), meningocele (n = 2), and diastematomyelia (n = 1). Twenty-three of the patients underwent primary untethering, whereas 2 patients had received untethering previously at another institution. The average syrinx length and width prior to surgery were 4.81 vertebral levels (SD 4.35) and 5.19 mm (SD 2.55 mm), respectively. Conus level ranged from L1 to S3. Patients were followed for an average of 8.4 years (1.35–15.85 years). Overall there was no significant change in syrinx length or width postoperatively; the average syrinx length increased by 0.86 vertebral levels (SD 4.36) and width decreased by 0.72 mm (SD 2.94 mm). Seven of 25 patients had improvement in at least one presenting symptom, including scoliosis, weakness, bowel/bladder dysfunction, and pain. Eight patients had stable presenting symptoms. Six patients were asymptomatic and 5 patients had new or worsening symptoms, which included scoliosis, pain, or sensory changes.CONCLUSIONSAlthough some syrinxes improved after surgery for tethered cord, radiological improvement was not consistent and did not appear to be associated with change in clinical symptoms. The decision to surgically untether a cord should be focused on the clinical symptoms and not the presence of a syrinx alone. Further studies are needed to confirm this finding.

2011 ◽  
Vol 15 (3) ◽  
pp. 258-270 ◽  
Author(s):  
Jörg Klekamp

Object The treatment of tethered cord syndromes in adults is discussed regarding the natural history and surgical indications. The author analyzes data obtained in patients who were diagnosed with a tethered cord in adulthood and either underwent surgical or conservative therapy between 1991 and 2009. Methods Since 1991, data obtained in 2515 patients with spinal cord pathologies were entered into the spinal cord database, and prospective follow-up was performed through outpatient visits and questionnaires. Of the 2515 patients, 85 adults with a tethered cord syndrome formed the basis of this study. The tethering effect was caused either by a split cord malformation, a thick filum terminale, a conus medullaris lipoma with extradural extension, or various combinations of these mechanisms. The mean age of the patients was 46 ± 13 years (range 23–74 years) and the mean follow-up duration was 61 ± 62 months. Two groups were distinguished based on the absence (Group A, 43 patients) or presence (Group B, 42 patients) of an associated lipoma or dysraphic cyst (that is, dermoid, epidermoid, or neurenteric cyst). Surgery was recommended for patients with symptoms only. Short-term results were determined within 3 months of surgery, whereas long-term outcomes (clinical recurrences) were evaluated using Kaplan-Meier statistics. Results For all patients, pain was the most common major complaint. Severe neurological deficits were rare. In Group A, 20 of 43 patients underwent surgery, whereas in Group B 23 of 42 patients underwent surgery. Among individuals who did not undergo surgery, 17 patients refused surgery and 25 patients underwent recommended conservative treatment. Short-term postoperative results indicated a significant improvement of pain and a stabilization of neurological symptoms. Long-term results showed a good prognosis in patients in whom first-time (that is, nonrevision) surgery achieved successful untethering, with a 10-year rate of neurological stabilization in 89% of Group A and a 10-year rate of neurological stabilization in 81% of Group B patients. The benefit of secondary operations in Group B was limited, with eventual clinical deterioration occurring in all patients within 10 years. For patients treated conservatively, follow-up information could be obtained in 33 of 42 patients. Twenty-eight patients remained in stable clinical condition. Only 5 of the conservatively treated patients experienced clinical deterioration over time; in 4 of these individuals with deterioration, surgery had been recommended but was refused by the patient. The clinical recurrence rate in all conservatively treated patients was 21% after 10 years. With a recommendation for surgery this figure rose to 47% within 5 years. Conclusions Surgery in adult patients with a tethered cord syndrome should be reserved for those with symptoms. In surgically treated patients, pain relief can often be achieved, and long-term neurological stabilization tends to persist more often than it does in conservatively treated patients. A conservative approach is warranted, however, in adult patients without neurological deficits. Revision surgery in patients with complex dysraphic lesions should be performed in exceptional cases only.


1995 ◽  
Vol 82 (3) ◽  
pp. 394-400 ◽  
Author(s):  
Mark Lee ◽  
Ali R. Rezai ◽  
Rick Abbott ◽  
Daniel H. Coelho ◽  
Fred J. Epstein

✓ Spinal cord lipomas are rare lesions, accounting for approximately 1% of all spinal cord tumors. True intramedullary spinal cord lipomas are extremely rare and are represented in the literature as scattered, single case reports. The authors present a series of six patients with intramedullary spinal cord lipomas managed at our institution from July, 1985 to July, 1993. The patients' ages ranged from 8 to 45 years. Four patients presented with newly diagnosed tumors and two had undergone previous surgery. Patients usually presented with long histories of disability followed by rapid progression of their symptoms. Most patients were in poor neurological condition on presentation. Presenting symptoms included spinal pain, dysesthetic sensory changes, gait difficulties, weakness, and incontinence. Three patients had cervical tumors, two had cervicothoracic tumors, and one patient had a thoracic tumor. Diagnostic studies, including magnetic resonance imaging, were obtained in all patients. No patient exhibited any form of spinal dysraphism or had a dural defect. All patients underwent decompressive, subtotal resections of 40% to 70% of their lesions. Follow-up times ranged from 12 to 96 months. All patients had resolution of their pain, but they generally showed no neurological improvement. As of their most recent follow-up visit, none of the patients was neurologically normal; three can function independently, although with neurological deficits. The other three patients cannot function independently and have severe neurological deficits. The authors conclude that patients with intramedullary spinal cord lipoma who present with significant neurological compromise have a very poor prognosis with regard to neurological function and generally show no improvement with surgical resection.


1998 ◽  
Vol 88 (6) ◽  
pp. 958-961 ◽  
Author(s):  
Bermans J. Iskandar ◽  
Benjamin B. Fulmer ◽  
Mark N. Hadley ◽  
W. Jerry Oakes

Object. The management of tethered spinal cord syndrome with onset of symptomatology occurring in adulthood remains controversial, although the necessity of early surgery in the pediatric tethered cord syndrome population is well established. To ascertain the results of surgery in adult patients with this anomaly, the authors undertook a retrospective review of 34 cases. Methods. The authors studied the hospital records of 34 consecutive patients who presented in adulthood with tethered cord syndrome and conducted follow-up phone interviews with 28 of them. The population consisted of 12 men and 22 women, ranging in age from 18 to 70 years (mean 34 years). The most common presenting feature was pain, followed by weakness and incontinence. All patients underwent surgery. The most common operative findings were tight filum terminale, split cord malformation, and lipomyelomeningocele, paralleling those observed in pediatric studies. Long-term surgical results and patient outcome ratings were encouraging. After a mean clinical follow-up period of 4 years, significant improvement occurred in 22 of 27 patients presenting with pain, 13 of 27 patients with motor or sensory dysfunction, and 11 of 18 patients with bowel and bladder disturbance. In addition, telephone interviews were obtained after a period of 8.6 years. Twenty-two (79%) of 28 patients called the operation a long-term success; 21 (75%) of 28 patients believed that they had significant postoperative improvement (and not just stabilization) in pain and/or neurological function. Surgical complications were generally minor. Nineteen (86%) of 22 employed patients returned to work after surgery. Two (33%) of six patients who were not employed before surgery worked full time postoperatively. Only two of the 28 patients interviewed had received Workers' Compensation benefits; both of these had good outcomes and returned to work. Conclusions. Tethered spinal cord syndrome in adults is an uncommon entity that can become symptomatic. Although surgery in adults involves greater risk of neurological injury than in children, it is a low-risk procedure with encouraging results. Because neurological deficits are generally irreversible, early surgery is recommended.


2010 ◽  
Vol 12 (3) ◽  
pp. 233-242 ◽  
Author(s):  
Gautam U. Mehta ◽  
Ashok R. Asthagiri ◽  
Kamran D. Bakhtian ◽  
Sungyoung Auh ◽  
Edward H. Oldfield ◽  
...  

Object Spinal cord hemangioblastomas are a common protean manifestation of von Hippel-Lindau (VHL) disease and can be associated with significant morbidity. To better define expected outcome and optimal management of these tumors in the context of this neoplasia syndrome, the authors analyzed the findings from patients with VHL disease who underwent resection of spinal cord hemangioblastomas. Methods Consecutive patients with VHL disease who underwent surgery for spinal cord hemangioblastomas with > 6 months follow-up were included in the study. Serial clinical examinations, functional scores, imaging findings, and operative records were analyzed. Results One hundred eight patients (57 male, 51 female) underwent 156 operations for resection of 218 spinal cord hemangioblastomas. One hundred forty-six operations (94%) were performed for symptom-producing tumors. The most common presenting symptoms included hypesthesia (64% of resections), hyperreflexia (57%), dysesthesia (43%), and weakness (36%). Mean follow-up was 7.0 ± 5.0 years (range 0.5–20.9 years). Complete resection was achieved for 217 tumors (99.5%). At 6-months follow-up, patients were stable or improved after 149 operations (96%) and worse after 7 operations (4%). Ventral tumors (OR 15.66, 95% CI 2.54–96.45; p = 0.003) or completely intramedullary tumors (OR 10.74, 95% CI 2.07–55.66; p = 0.005) were associated with an increased risk of postoperative worsening. The proportion of patients remaining functionally stable at 2, 5, 10, and 15 years' follow-up was 93, 86, 78, and 78%. Long-term functional decline was caused by extensive VHL-associated CNS disease (6 patients), VHL-associated visceral disease (1 patient), or non-VHL disease (2 patients). Conclusions Resection of symptomatic spinal cord hemangioblastomas is a safe and effective means of preserving neurological function in patients with VHL disease. Tumor location (ventral or completely intramedullary) can be used to assess functional risk associated with surgery. Long-term decline in neurological function is usually caused by VHL-associated disease progression.


2009 ◽  
Vol 4 (1) ◽  
pp. 28-36 ◽  
Author(s):  
Wajd N. Al-Holou ◽  
Karin M. Muraszko ◽  
Hugh J. Garton ◽  
Steven R. Buchman ◽  
Cormac O. Maher

Object After primary repair of a myelomeningocele or a lipomyelomeningocele, patients can present with symptoms of secondary tethered cord syndrome (TCS). After surgical untethering, a small percentage of these patients can present with multiple repeat TCS. In patients presenting with secondary or multiple repeat TCS, the role as well the expected outcomes of surgical untethering are not well defined. Methods Eighty-four patients who underwent spinal cord untethering after at least 1 primary repair were retrospectively evaluated using scaled and subjective outcome measures at short-term and long-term follow-up visits. Outcomes were analyzed for predictive measures using multivariate logistic regression. Results Surgical untethering was performed in 66 patients with myelomeningoceles and 18 patients with lipomyelomeningoceles. Fourteen patients underwent multiple repeat spinal cord untethering. Patients were followed up for an average of 6.2 years. Most patients had stability of function postoperatively. Motor function and weakness improved in 7 and 16% of patients at 6 months, respectively, and 6 and 19% of patients at long-term follow-up evaluation, respectively. Of the patients who presented with back pain, 75% had improvement in symptoms at 6 months postoperatively. Younger age at untethering was significantly associated with worse long-term neurological outcomes. The number of previous untethering procedures, original diagnosis, sex, anatomical level, and degree of untethering had no effect on surgical outcomes. Conclusions Patients presenting with secondary or multiple repeat TCS may benefit from surgical untethering.


1994 ◽  
Vol 80 (4) ◽  
pp. 689-693 ◽  
Author(s):  
Hiroshi K. Inoue ◽  
Satoshi Kobayashi ◽  
Katsumi Ohbayashi ◽  
Hideaki Kohga ◽  
Masaru Nakamura

✓ The incidence of the tethered cord syndrome after repair of spinal dysraphism is not insignificant. A retethered spinal cord may also develop after an untethering operation. In order to treat and/or prevent the tethered and retethered spinal cord, the authors developed and successfully used a new method in 12 cases. After complete release and reconstruction of the spinal cord, a Gore-Tex surgical membrane was placed over the cord and fixed to the lateral dural surface with stay sutures. During a postoperative follow-up period ranging from 23 months to 7 years, no further neurological deterioration was observed in the 12 patients and magnetic resonance imaging studies showed no adhesion of the spinal cord to the operative site. It is concluded that this simple new method is effective for the treatment and prevention of tethering and/or retethering of the spinal cord, although a longer follow-up study is required.


2001 ◽  
Vol 10 (1) ◽  
pp. 1-5 ◽  
Author(s):  
Bermans J. Iskandar ◽  
Benjamin B. Fulmer ◽  
Mark N. Hadley ◽  
W. Jerry Oakes

Object The management of tethered cord syndrome with onset of symptomatology occurring in adulthood remains controversial, although the necessity of early surgery in the pediatric tethered cord syndrome population is well established. To ascertain the results of surgery in adult patients with this anomaly, the authors undertook a retrospective review of 34 cases. Methods The authors studied the hospital records of 34 consecutive patients who presented in adulthood with tethered cord syndrome and conducted follow-up phone interviews with 28 of them. The population consisted of 12 men and 22 women, ranging in age from 18 to 70 years (mean 34 years). The most common presenting feature was pain, followed by weakness and incontinence. All patients underwent surgery. The most common operative findings were tight filum terminale, split cord malformation, and lipomyelomeningocele, paralleling those observed in pediatric studies. Long-term surgical results and patient outcome ratings were encouraging. After a mean clinical follow-up period of 4 years, significant improvement occurred in 22 of 27 patients presenting with pain, 13 of 27 patients with motor or sensory dysfunction, and 11 of 18 patients with bowel and bladder disturbance. In addition, telephone interviews were obtained after a period of 8.6 years. Twenty-two (79%) of 28 patients called the operation a long-term success; 21 (75%) of 28 patients believed that they had significant postoperative improvement (and not just stabilization) in pain and/or neurological function. Surgical complications were generally minor. Surgery-related complications included one cerebrospinal fluid leak and five pseudomeningoceles. Two patients complained of worsening motor or sensory dysfunction and one of worsening bladder dysfunction, whereas four patients experienced persistent pain and one complained of worsening pain postoperatively. According to the patients, the vast majority of these complications were minor. Nineteen (86%) of 22 employed patients returned to work after surgery. Two (33%) of six patients who were not employed before surgery worked full time postoperatively. Only two of the 28 patients interviewed had received Workers' Compensation benefits; both of these had good outcomes and returned to work. Conclusions Tethered spinal cord syndrome in adults is an uncommon entity that can become symptomatic. Although surgery in adults involves greater risk of neurological injury than in children, it is a low-risk procedure with encouraging results. Because neurological deficits are generally irreversible, early surgery is recommended.


Neurosurgery ◽  
2012 ◽  
Vol 71 (1) ◽  
pp. 23-29 ◽  
Author(s):  
Ji Yeoun Lee ◽  
Ji Hoon Phi ◽  
Jung-Eun Cheon ◽  
Seung-Ki Kim ◽  
In-One Kim ◽  
...  

Abstract BACKGROUND: There has been controversy regarding the management of syringomyelia associated with tethered spinal cord. Previous reports on the topic have included only a small number of patients, considered open/closed spinal dysraphism together, or had a short follow-up. OBJECTIVE: To review a uniform group of patients with syringomyelia associated with tethered cord and treated mainly by untethering alone. METHODS: Of the 135 patients operated on for closed spinal dysraphism between 2003 and 2008, 33 patients with preoperative syringomyelia were identified. The preoperative/postoperative clinical data and syrinx index (ratio of the syrinx area and the cord area) were retrospectively reviewed. The syrinx index of each patient was plotted as an individual graph to outline the temporal change of the syrinx before and after untethering surgery. RESULTS: Five patients showed symptom progression during the preoperative period, and 4 of the 5 had an additional magnetic resonance imaging before the operation that showed progression of the syringomyelia. Postoperatively, 31 of 32 patients (97%) who underwent postoperative follow-up imaging showed long-term stability or a decrease in the syrinx index. Four symptomatically stable patients showed a transient increase in the syrinx index during the initial postoperative 6 months, which later decreased spontaneously. In 1 patient with retethering, the syrinx index increased 6 months before the onset of new urinary symptoms. CONCLUSION: Untethering alone may be sufficient for the management of syringomyelia associated with tethered cord. A transient increase in the syrinx index during the initial postoperative period may be observed without additional surgery if patients are symptomatically stable.


Author(s):  
Sean Wei Xiang Ong ◽  
Siew-Wai Fong ◽  
Barnaby Edward Young ◽  
Yi-Hao Chan ◽  
Bernett Lee ◽  
...  

Abstract Background The complications and sequelae of coronavirus disease 2019 (COVID-19) and their effect on long-term health are unclear, and the trajectory of associated immune dysregulation poorly understood. Methods We conducted a prospective longitudinal multi-center cohort study at four public hospitals in Singapore. COVID-19 patients were monitored for a median of six months after recovery from acute infection. Clinical symptoms and radiologic data were collected, along with plasma samples for quantification of immune mediators. The relationship between clinical symptoms and immune cytokine profiles was investigated. Results 288 participants were recruited, and follow-up data were available for 183, 175, and 120 participants at days 30, 90 and 180 post-symptom onset respectively. Symptoms related to COVID-19 were present in 31 (16.9%), 13 (7.4%), and 14 (11.7%) at days 30, 90 and 180. In a multivariable model, age >65 years, non-Chinese ethnicity, and the severity of acute infection were associated with increased likelihood of persistent symptoms. Recovered COVID-19 patients had elevated levels of pro-inflammatory IL-17A, SCF, IL-12p70, IL-1βand pro-angiogenic MIP-1β, BDNF, VEGF at day 180 compared to healthy controls. Higher levels of MCP-1 and PDGF-BB were detected in patients with persistent symptoms, versus symptom-free patients. Conclusions About 10% of recovered patients had persistent symptoms six months after initial infection. Immune cytokine signatures of the recovered patients reflected ongoing chronic inflammation and angiogenesis. COVID-19 patients should be monitored closely for emerging long-term health consequences.


2021 ◽  
Vol 12 ◽  
Author(s):  
Renata Toth-Bencsik ◽  
Peter Balicza ◽  
Edina Timea Varga ◽  
Andras Lengyel ◽  
Gabor Rudas ◽  
...  

IntroductionPhospholipase A2-associated Neurodegeneration (PLAN) is a group of neurodegenerative diseases associated with the alterations of PLA2G6. Some phenotype-genotype association are well known but there is no clear explanation why some cases can be classified into distinct subgroups, while others follow a continuous clinical spectrum.MethodsLong-term neurological, and psychiatric follow-up, neuropathological, radiological, and genetic examinations, were performed in three affected girls and their family.ResultsTwo 24-years old twins and their 22-years old sister harbored the p.P622S, and p.R600W mutation in PLA2G6. The age of onset and the most prominent presenting symptoms (gaze palsy, ataxia, dystonia, psychomotor regression indicated atypical neuroaxonal dystrophy (ANAD), however, optic atrophy, severe tetraparesis would fit into infantile neuroaxonal dystrophy (INAD). All siblings had hyperintensity in the globi pallidi and substantiae nigrae which is reported in ANAD, whereas it is considered a later neuroradiological marker in INAD. The slow progression, rigidity, bradykinesis, and the prominent psychiatric symptoms indicate PLA2G6-related dystonia-parkinsonism. Abnormal mitochondria, lipid accumulation and axonal spheroids were observed in the muscle and nerve tissue. Brain deposition appeared 6 years following the initial cerebellar atrophy. Mild MRI alterations were detected in the asymptomatic carrier parents.ConclusionThe colorful clinical symptoms, the slightly discordant phenotype, and the neuroimaging data in the family supports the view that despite the distinct definition of age-related phenotypes in PLAN, these are not strict disease categories, but rather a continuous phenotypic spectrum. The mild MRI alterations of the parents and the family history suggest that even heterozygous pathogenic variants might be associated with clinical symptoms, although systematic study is needed to prove this.


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