Intradural inclusion cysts following in utero closure of myelomeningocele: clinical implications and follow-up findings

2008 ◽  
Vol 2 (6) ◽  
pp. 406-413 ◽  
Author(s):  
Enrico Danzer ◽  
N. Scott Adzick ◽  
Natalie E. Rintoul ◽  
Deborah M. Zarnow ◽  
Erin S. Schwartz ◽  
...  
Keyword(s):  
Mr Imaging ◽  
Tethered Cord ◽  
Bladder Function ◽  
Clinical Implications ◽  
Clean Intermittent Catheterization ◽  
Lower Extremity Function ◽  
Normal Bladder ◽  
Increased Risk ◽  
Clinical Consequences

Object The goal in this study was to evaluate the incidence and clinical implications of the development of cutaneously derived intradural inclusion cysts (ICs) following fetal myelomeningocele (fMMC) closure. Methods Retrospective databases and responses to a parental questionnaire were reviewed to determine the incidence, clinical presentation, and outcomes of fMMCs in children in whom ICs developed at follow-up. Results Prior to the National Institutes of Health (NIH)-sponsored Management of Myelomeningocele Study (MOMS), 54 patients underwent fMMC closure at the authors' institution. Sixteen (30%) presented with symptomatic tethered cord syndrome (TCS) at a median age of 27 months (range 4–93 months). Ten (63%) of the 16 (19% of the total) developed TCS in association with an intradural IC. In 9 (90%) of 10 patients, the IC was seen on preoperative MR imaging, and in 1 it was found during surgery. Four additional children (7% of the total) with evidence of an IC on surveillance MR imaging are currently asymptomatic at 94, 84, 60, and 60 months of age, respectively. All but 1 (an L-3 level lesion) IC developed in infants with L-4 and L-5 defects. After cyst removal, 6 children are asymptomatic at a median follow-up of 36 months (range 12–63 months). Following IC removal, 4 children lost normal bladder function and now require clean intermittent catheterization, and 1 lost normal leg function and now requires a walking aid for ambulation. Histologically, 8 lesions were dermoid, 1 was an epidermoid, and 1 was a mixed dermoid-epidermoid IC. Three patients developed another IC and required its removal at 24, 39, and 51 months, respectively. One required another tethered cord release within 57 months after IC removal. Conclusions Cutaneously derived intradural ICs can develop following fMMC surgery. Deterioration of bladder function, risk of recurrence, and loss of lower-extremity function appear to be the most important long-term complications of IC in children with fMMCs. The ongoing NIH-sponsored MOMS may help determine whether children with fMMC are at increased risk of IC development compared with children treated with postnatal MMC closure. Parents seeking fMMC closure should be informed about the possibility of IC formation and the potential clinical consequences.

Progression of carotid near-occlusion to complete occlusion: related factors and clinical implications

2020 ◽  
pp. neurintsurg-2019-015638
Author(s):  
Andrés García-Pastor ◽  
Antonio Gil-Núñez ◽  
José María Ramírez-Moreno ◽  
Noelia González-Nafría ◽  
Javier Tejada ◽  
...  
Keyword(s):  
Collateral Circulation ◽  
Demographic Data ◽  
Clinical Manifestations ◽  
Treatment Modalities ◽  
Clinical Implications ◽  
Related Factors ◽  
Complete Occlusion ◽  
Clinical Consequences ◽  
Near Occlusion

BackgroundThe clinical consequences and factors related to the progression from a carotid near-occlusion (CNO) to a complete occlusion are not well established. Our aim is to describe the rate, predictive factors and clinical implications of the progression to complete carotid occlusion (PCCO) in a population of patients with symptomatic CNO.MethodsWe conducted a multicenter, nationwide, prospective study from January 2010 to May 2016. Patients with angiography-confirmed CNO were included. We collected information on demographic data, clinical manifestations, radiological and hemodynamic findings, and treatment modalities. A 24 month carotid-imaging follow-up of the CNO was performed.Results141 patients were included in the study, and carotid-imaging follow-up was performed in 122 patients. PCCO occurred in 40 patients (32.8%), and was more frequent in medically-treated patients (34 out of 61; 55.7%) compared with patients treated with revascularization (6 out of 61; 9.8%) (p<0.001). 7 of the 40 patients with PCCO (17.5%) suffered ipsilateral symptoms. Factors independently related with PCCO in the multivariate analysis were: age ≥75 years (OR 2.93, 95% CI 1.05 to 8.13), revascularization (OR 0.07, 95% CI 0.02 to 0.20), and collateral circulation through the ipsilateral ophthalmic artery (OR 3.25, 95% CI 1.01 to 10.48).ConclusionsPCCO occurred within 24 months in more than half of the patients under medical treatment. Most episodes of PCCO were not associated with ipsilateral symptoms. Revascularization reduces the risk of PCCO.

Tumor suppressor p16 methylation in multiple myeloma: biological and clinical implications

Blood ◽  
2006 ◽  
Vol 109 (3) ◽  
pp. 1228-1232 ◽  
Author(s):  
Natalia Gonzalez-Paz ◽  
Wee J. Chng ◽  
Rebecca F. McClure ◽  
Emily Blood ◽  
Martin M. Oken ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Cell Labeling ◽  
Clinical Implications ◽  
Gene Methylation ◽  
P16 Gene ◽  
Specific Pcr ◽  
Long Term Follow Up ◽  
Clinical Consequences

Abstract The biological and clinical implications of p16 gene methylation in multiple myeloma (MM) are still unclear despite previous studies. In this comprehensive study, using methylation-specific PCR (MS-PCR), we show that p16 methylation is relatively common and occurs in monoclonal gammopathy of undetermined significance (MGUS; n = 17), smoldering multiple myeloma (SMM; n = 40), and MM (n = 522) at a prevalence of 24%, 28%, and 34%, respectively. However, p16 methylation does not appear to affect gene expression level. In a large cohort of patients with long-term follow-up information (n = 439), there was no difference in overall survival between patients with or without p16 methylation. We also found no association between p16 methylation and the main cytogenetic categories, although it was more common among patients with 17p13.1 deletions (p53 locus), a genetic progression event in MM. In addition, p16 methylation has no apparent effect on the cycle because there was also no difference in the plasma cell labeling index (a direct measurement of proliferation) between patients with and without p16 methylation. Our results question a major role for p16 methylation in the oncogenesis of the PC neoplasm, and we now believe p16 methylation may be a marker for overall epigenetic changes associated with disease progression, with no obvious direct biological or clinical consequences.

scholarly journals Feasibility and outcome of clean intermittent catheterization for children with sensate urethra

2013 ◽  
Vol 4 (6) ◽  
pp. 403 ◽  
Author(s):  
Khalid Fouda Neel
Keyword(s):  
Nous Avons ◽  
Urinary Diversion ◽  
Surgical Intervention ◽  
Prise En Charge ◽  
Bladder Function ◽  
Intermittent Catheterization ◽  
Clean Intermittent Catheterization ◽  
Neuropathic Bladder

Objective: Clean intermittent catheterization (CIC) is an importantasset in managing children with noncompliant bladders. We reviewthe feasibility and late outcomes of patients with normal urethralsensation who began CIC.Materials and methods: We reviewed all patients with posteriorurethral valve (PUV) or non-neuropathic bladder sphincter dysfunction(NNBSD) who began on CIC, and had at least 2 years offollow-up. We considered their age, indication, acceptance andcompliance with CIC. Additionally, we examined the late outcomeof bladder function and the need for any surgical intervention atfollow-up.Results: Between 1999 and 2006, 52 patients with PUV (38patients) or NNBSD (14 patients) were started on CIC. Of these 52patients, 48% were under the age of 4. A total of 44 patients (85%)accepted the recommendation for CIC, and 34 patients (65%) werecompliant with the protocol after at least 2 years of follow-up. Theage of the patients was the only significant factor related to thesuccess of the protocol (4 years old or younger, p = 0.03). Afterat least 2 years of follow-up, 28 patients (54%) remained on CICand have not required urinary diversion or bladder reconstruction.Conclusion: The current study showed that CIC is a feasible optionfor patients with sensate urethra, which necessitates the introductionof treatment as early as possible. Further, those patients whoare compliant with the CIC demonstrated a better chance of avoidingsubsequent surgical intervention for the management of a noncompliantbladder.Objectif : Le cathétérisme intermittent propre (CIP) est un élémentimportant de la prise en charge de l’insuffisance vésicale chez lesenfants. Nous passons ici en revue la faisabilité et les résultats tardifschez des patients affichant des sensations urétrales normalesqui ont entrepris un traitement par CIP.Matériel et méthodologie : Nous avons examiné tous les patientsatteints d’une dysfonction de la valve urétrale postérieure (DVUP)ou d’une dysfonction non névrotique du sphincter vésical (DNNSV)ayant entrepris un traitement par CIP, et suivis pendant au moins2 ans. Nous avons tenu compte de leur âge, de l’indication dutraitement, de leur acceptation et leur observance du traitementpar CIP. En outre, nous avons examiné les résultats tardifs de lafonction vésicale et le besoin de recourir à tout type d’interventionchirurgicale lors du suivi.Résultats : Entre 1999 et 2006, 52 patients atteints de DVUP(38 patients) ou de DNNSV (14 patients) ont entrepris un traitementpar CIP. De ce nombre, 48 % avaient moins de 4 ans. Au total,44 patients (85 %) ont accepté la recommandation de procéderau CIP, et 34 patients (65 %) observaient toujours le traitementaprès au moins 2 ans de suivi. L’âge des patients représentaitle seul facteur significatif lié à la réussite du traitement (4 ansou moins, p = 0,03). Après au moins 2 ans de suivi, 28 patients(54 %) poursuivaient leur traitement par CIP et n’avaient pas besoind’un détournement urinaire ou d’une reconstruction de la vessie.Conclusion : Notre étude a montré que le CIP est une optionraisonnable pour les patients avec urètre sensible, qui nécessitel’instauration d’un traitement le plus rapidement possible. Par ailleurs,les patients qui observent bien le traitement par CIP présentaientde meilleures chances d’éviter une intervention chirurgicalesubséquente pour le traitement d’une insuffisance vésicale.

scholarly journals Desmopressin 120 mcg, 180 mcg, 240 mcg: The right treatment for the right patient

2018 ◽  
Vol 90 (2) ◽  
pp. 127 ◽  
Author(s):  
Pietro Ferrara ◽  
Ester Del Vescovo ◽  
Francesca Ianniello ◽  
Giulia Franceschini ◽  
Luciana Romaniello ◽  
...  
Keyword(s):  
Drug Therapy ◽  
Nocturnal Enuresis ◽  
Bladder Function ◽  
Efficacy And Safety ◽  
Nocturnal Polyuria ◽  
First Line ◽  
Normal Bladder ◽  
Increased Risk ◽  
The Right ◽  
Line Drug

Background: The first-line drug therapy for patients with nocturnal enuresis (NE) associated with nocturnal polyuria and normal bladder function is desmopressin (dDAVP). Objective: To evaluate if increasing dose of oral desmopressin lyophilisate (MELT) can improve response rates to dDAVP and is useful in enuretic children. Materials and methods: We enrolled a total of 260 children all diagnosed with NE. Enuretic children were treated with increasing MELT at a dose of 120, 180 and 240 mcg a day.Results. We included in our study a total of 237 children, 164 males (69.2%) and 73 females (30.8%) aged between 5 and 18 years (mean age 10.32 ± 2.52 years). Of the 237 patients enrolled in the study and treated with MELT 120 mcg, a full response was achieved in 135 (56.9%). A partial response was achieved in 21 (8.9%) patients, therefore the dose was increased up to 180 mcg, with further improving symptoms (14.3%) or full response (9.5%), and up to 240 mcg, without usefulness. Conclusions: MELT at the dose of 120 mcg resulted efficacy and safety; the increased dose up to 180 mcg resulted poorly efficacy; finally, the further increase up to 240 mcg did not improve the symptoms with the increased risk of side effects.

scholarly journals Clinical Implications of MR Imaging Findings in the White Matter in Very Preterm Infants: A 2-year Follow-up Study

Radiology ◽  
2011 ◽  
Vol 261 (3) ◽  
pp. 899-906 ◽  
Author(s):  
Francisca T. de Bruïne ◽  
Annette A. van den Berg-Huysmans ◽  
Lara M. Leijser ◽  
Monique Rijken ◽  
Sylke J. Steggerda ◽  
...  
Keyword(s):  
White Matter ◽  
Mr Imaging ◽  
Preterm Infants ◽  
Clinical Implications ◽  
Imaging Findings ◽  
Very Preterm Infants ◽  
Very Preterm ◽  
Follow Up Study

Tethered Cord and Anorectal Malformations: A Case Series

2017 ◽  
Vol 28 (06) ◽  
pp. 484-490 ◽  
Author(s):  
Lorena Canazza ◽  
Milena Meroni ◽  
Giorgio Selvaggio ◽  
Cecilia Parazzini ◽  
Laura Valentini ◽  
...  
Keyword(s):  
Demographic Data ◽  
Case Series ◽  
Tethered Cord ◽  
Anorectal Malformations ◽  
International Classification Of Functioning ◽  
Clean Intermittent Catheterization ◽  
Critical Elements ◽  
Long Term Follow Up

Introduction The clinical manifestation of a stretched low-lying cone (LLC) is represented by the tethered cord syndrome (TCS) with cutaneous, urologic, neurologic, and orthopaedic dysfunctions. TC is frequently found in patients with anorectal malformations (ARMs). The aim of our article is to report a series of patients affected by LLC and ARMs and evaluate their management and long-term follow-up results. Materials and Methods We performed a retrospective analysis over a period of 15 years including patients with ARM and TC and excluding those with severe polymalformations. We collected information related to the types of malformations and demographic data. We analyzed their management (perinatal protocol, radiological investigations, surgery, and multidisciplinary follow-up). QOL was assessed through the International Classification of Functioning, Disability, and Health for Children and Youth (ICF-CY). Results Thirty-three patients among 210 ARM cases had TC (16%). Eleven of them underwent neurosurgery. One patient had retethering; out of 11 patients, 4 remained stable and 6 improved after surgery (UDS normalization and resolution of symptoms). At a mean follow-up of 10 years, four patients were on clean intermittent catheterization and five on the Peristeen transanal irrigation. The majority of patients were defined as 3 (from 1-bad to 5-excellent) for their physical and mental state. Conclusion The use of MRI is considered to complete the ARM screening in detecting TC. The multidisciplinary approach is crucial and helps in defining the management of patients. In fact, it is not clear how the features of ARM and TC affect each other. The selection of cases for surgery should take into account the critical elements.

Prediction of recurrence in solid nonfunctioning pituitary macroadenomas: additional benefits of diffusion-weighted MR imaging

2020 ◽  
Vol 132 (2) ◽  
pp. 351-359 ◽  
Author(s):  
Ching-Chung Ko ◽  
Tai-Yuan Chen ◽  
Sher-Wei Lim ◽  
Yu-Ting Kuo ◽  
Te-Chang Wu ◽  
...  
Keyword(s):  
Mr Imaging ◽  
Cox Proportional Hazards ◽  
Imaging Features ◽  
Large Tumor ◽  
Adc Value ◽  
Diffusion Weighted ◽  
Increased Risk ◽  
Adc Values ◽  
Pituitary Macroadenomas

OBJECTIVEA subset of benign, nonfunctioning pituitary macroadenomas (NFMAs) has been shown to undergo early progression/recurrence (P/R) during the first years after surgical resection. The aim of this study was to determine preoperative MR imaging features for the prediction of P/R in benign solid NFMAs, with emphasis on apparent diffusion coefficient (ADC) values.METHODSWe retrospectively investigated the preoperative MR imaging features for the prediction of P/R in benign solid NFMAs. Only the patients who had undergone preoperative MRI and postoperative MRI follow-ups for more than 1 year (at least every 6–12 months) were included. From November 2010 to December 2016, a total of 30 patients diagnosed with benign solid NFMAs were included (median follow-up time 45 months), and 19 (63.3%) patients had P/R (median time to P/R 24 months).RESULTSBenign solid NFMAs with cavernous sinus invasion, failed chiasmatic decompression, large tumor height and tumor volume, high diffusion-weighted imaging (DWI) signal, and lower ADC values/ratios were significantly associated with P/R (p < 0.05). The cutoff points of ADC value and ADC ratio for prediction of P/R are 0.77 × 10−3 mm2/sec and 1.01, respectively, with area under the curve (AUC) values (0.9 and 0.91) (p < 0.01). In multivariate Cox proportional hazards analysis, low ADC value (< 0.77 × 10−3 mm2/sec) is a high-risk factor of P/R (p < 0.05) with a hazard ratio of 14.07.CONCLUSIONSBenign solid NFMAs with low ADC values/ratios are at a significantly increased risk of P/R, and aggressive treatments accompanied by close follow-up with imaging studies should be considered.

scholarly journals Antenatally detected urinary tract dilatation: a 12–15-year follow-up

2020 ◽  
Vol 35 (11) ◽  
pp. 2129-2135 ◽  
Author(s):  
Maria Herthelius ◽  
Rimma Axelsson ◽  
Karl-Johan Lidefelt
Keyword(s):  
Urinary Tract ◽  
Kidney Damage ◽  
Long Term Outcome ◽  
Urinary Tract Dilatation ◽  
Normal Bladder ◽  
Increased Risk ◽  
Bladder Pathology ◽  
Tract Infections

Abstract Background Antenatally diagnosed urinary tract dilatation (UTD) still burdens healthcare providers and parents. This study was conducted to establish long-term outcome in an unselected group of children with antenatally detected UTD. Methods Seventy-one out of 103 children born in 2003–2005 and diagnosed with antenatal UTD agreed to participate in a 12–15-year follow-up including blood and urine samples, a kidney ultrasound exam, and kidney scintigraphy. The records were searched for previous urinary tract infections. Results Among children with an anteroposterior diameter (APD) ≤ 7 mm and no calyceal, kidney, ureteral, or bladder pathology in the early postnatal period, no one tested had reduced estimated glomerular filtration rate (eGFR), albuminuria, or UTD at the follow-up at a mean age of 13.6 years. One child had kidney damage not affecting kidney function. Among children with postnatal APD > 7 mm and/or kidney, calyceal, ureteral, or bladder pathology, 15% had persistent UTD and 32–39% (depending on the method used) had kidney damage. Major postnatal urinary tract ultrasound abnormalities and a congenital anomalies of the kidney and urinary tract (CAKUT) diagnosis were factors associated with an increased risk for permanent kidney damage (odds ratios 8.9, p = 0.016; and 14.0, p = 0.002, respectively). No one had reduced eGFR. One child (1/71, 1%) had a febrile urinary tract infection after the age of 2. Conclusions We conclude that in children with postnatal APD ≤ 7 mm, no calyceal dilatation, normal bladder, ureters, and kidney parenchyma, the outcome is excellent. There is no need for long-term follow-up in these patients.

Progressive posttraumatic myelomalacic myelopathy: treatment with untethering and expansive duraplasty

1997 ◽  
Vol 86 (4) ◽  
pp. 624-628 ◽  
Author(s):  
Thomas T. Lee ◽  
Jose M. Arias ◽  
Heather L. Andrus ◽  
Robert M. Quencer ◽  
Steven F. Falcone ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Mr Imaging ◽  
Motor Function ◽  
Tethered Cord ◽  
Sensory Function ◽  
Autonomic Dysreflexia ◽  
Nerve Roots ◽  
Content Type ◽  
Fine Print

✓ Patients with progressive posttraumatic myelomalacic myelopathy (PPMM), or tethered cord syndrome, present with symptoms and signs similar to those observed in cases of progressive posttraumatic cystic myelopathy, that is, sensorimotor function deterioration, local and/or radicular pain, increased spasticity, increased autonomic dysreflexia, and sphincter dysfunction. The authors investigated surgical outcomes of untethering combined with expansive duraplasty. Forty patients with PPMM who presented with functional deterioration underwent untethering of the spinal cord and nerve roots with an expansive duraplasty. Meticulous dissections of adhesions on the dorsal and lateral aspects of the spinal cord and nerve roots were performed. Intraoperative ultrasonography was used to detect the presence of a confluent cyst and to assess the success of untethering. After surgery, the patients were treated using a protocol that involved frequent turning for 48 hours and subsequently mobilization. Preoperative magnetic resonance (MR) imaging, with and without administration of a contrast agent, was obtained in all patients, except one patient who underwent immediate and delayed computerized tomography (CT) myelography. The mean follow-up period was 3 years (range 20–57 months) for the 36 patients available for follow-up review. Spinal cord tethering was observed in all patients preoperatively. Trauma was the most common cause of this pathology, accounting for 31 of the 40 cases. Preoperative MR imaging did not demonstrate tumor recurrence in the group of five patients who had undergone an initial operation for tumor excision. The interval between the causative event and the operation was less than 5 years in half of the patients (20 of 40), with the longest interval lasting up to 37 years. Motor function deterioration was the most frequent manifestation; it was present in 31 of 40 patients. Improvements in motor function, autonomic dysreflexia, pain, sphincter dysfunction, and sensory function were found during the most recent follow-up examination in 79%, 75%, 62%, 50%, and 43% of the patients, respectively. Two patients experienced retethering of the spinal cord and one underwent a second operation. Surgical untethering and expansive duraplasty, followed by postoperative position rotation to avoid retethering, provide symptomatic relief for patients with PPMM.

Combination of myxopapillary ependymoma and fatty filum in a child with tethered cord syndrome

2008 ◽  
Vol 1 (5) ◽  
pp. 386-388 ◽  
Author(s):  
Konstantina Karabatsou ◽  
Daniel Crooks ◽  
Dawn Williams ◽  
Neil Buxton
Keyword(s):  
Mr Imaging ◽  
Tumor Recurrence ◽  
Developmental Delays ◽  
Histological Analysis ◽  
Tethered Cord ◽  
Myxopapillary Ependymoma ◽  
Good Recovery ◽  
Surgical Exploration ◽  
Pathophysiological Process

✓ The authors present a case of a child with a tethered spinal cord associated with a myxopapillary ependymoma. This 16-month-old boy presented to the authors' institution with developmental delays in standing and walking. Magnetic resonance (MR) imaging demonstrated a fatty terminal filum and tethered cord. The child underwent surgical exploration of the spine with resection of the fatty filum tissue and release of the cord. Histological analysis of the fatty filum suggested the presence of a coexisting myxopapillary ependymoma. The child made a good recovery with no evidence of tumor recurrence after 4-years of follow-up with serial MR imaging. This unusual combination has not previously been reported in children, and to the authors' knowledge there is only one reported case in an adult. The likelihood of a common pathophysiological process in these conditions is also discussed.

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