scholarly journals Imaging characteristics and growth of subependymal giant cell astrocytomas

2006 ◽  
Vol 20 (1) ◽  
pp. 1-4 ◽  
Author(s):  
Michelle J. Clarke ◽  
Andrew B. Foy ◽  
Nicholas Wetjen ◽  
Corey Raffel
Keyword(s):  
Tumor Growth ◽  
Contrast Enhancement ◽  
Giant Cell ◽  
Pediatric Population ◽  
Obstructive Hydrocephalus ◽  
Signs And Symptoms ◽  
Foramen Of Monro ◽  
Imaging Characteristics ◽  
The Mean

Object Subependymal giant cell astrocytomas (SEGAs) are a common manifestation of tuberous sclerosis (TS). These evolving tumors have a propensity to cause obstructive hydrocephalus, usually due to obstruction at the level of the foramen of Monro. Differentiating SEGAs from subependymal nodules (SENs) before obstruction occurs may improve the morbidity associated with these tumors. In this study the authors' aim was to determine imaging characteristics of proven tumors in a single-center pediatric population. Methods The authors retrospectively reviewed all records and images obtained in patients with TS in whom results of biopsy sampling had proven that their tumors were SEGAs. Time to presentation, signs and symptoms at presentation, and imaging characteristics of the evolving tumors were noted. Twelve patients with 14 SEGAs proven by the results of biopsy sampling were reviewed. Resection was recommended for symptomatic and neuroimaging evidence of hydrocephalus (41%), tumor growth without evidence of hydrocephalus (33%), and for poorly controlled seizures (25%). The mean diameter of the tumors at the time of resection was 1.9 cm (range 0.3–4 cm), and no tumor recurred. Because of the pathological and radiographic continuum of SENs and SEGAs, it remains difficult to predict whether and when a given lesion will progress. Tumor growth and contrast enhancement are the most common signs of progression on neuroimages, and may be seen prior to the development of obstructive hydrocephalus. Conclusions Patients with SENs and SEGAs should undergo follow-up neuroimaging at yearly intervals, and if lesions show signs of progression (contrast enhancement or growth), these intervals should be shortened and consideration given to early resection.

scholarly journals P205 The contemporary presentation and management of giant cell arteritis with large vessel vasculitis

Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_1) ◽  
Author(s):  
Owen Cronin ◽  
Neil D McKay ◽  
Hannah Preston ◽  
Helen Harris ◽  
Barbara Hauser
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Polymyalgia Rheumatica ◽  
Large Vessel Vasculitis ◽  
Large Vessel ◽  
Ct Angiogram ◽  
Pet Ct ◽  
The Mean ◽  
Vessel Involvement

Abstract Background/Aims  Giant cell arteritis with large vessel vasculitis (LV-GCA) represents a distinct, less researched sub-category of giant cell arteritis (GCA). In comparison to cranial GCA, the patient’s diagnostic pathway is less well described and it is thought that LV-GCA is underdiagnosed, including in patients with polymyalgia rheumatica and cranial-GCA. Advances in imaging (e.g. PET-CT) and treatment (tocilizumab), have provided additional options in the diagnosis and management of LV-GCA. The aim was to describe the contemporary clinical journey for patients diagnosed with LV-GCA. Methods  The electronic patient health record system in NHS Lothian (TrakCare) was used to collect relevant data. Patients with imaging-confirmed large vessel vasculitis, diagnosed with GCA after 1 January 2017 were included. Follow-up was until August 2020. Results  Eighteen patients with LV-GCA were included. The mean age was 65 years and 66.7% were female. Two patients had known cranial-GCA but 89% of patients were diagnosed exclusively with large vessel involvement. The most common symptoms were malaise (55%), weight loss (55%), polymyalgia rheumatica (55%) and limb claudication (44%). Pyrexia of unknown origin was a feature in only 17% of patients. Two patients were asymptomatic and were investigated on the basis of raised inflammatory markers. Mean CRP at baseline was 99mg/L and ESR 85mm/hour. The mean time from symptom-onset to diagnosis was 6.8 months (range 1 to 15 months). Sixteen patients (89%) were reviewed by at least one other secondary care specialist. One third of patients were referred from General Medicine followed by Vascular Surgery (16%) and General Practice (16%). 7/18 patients were inpatients at the time of referral. 56% of patients required two modalities of imaging to confirm large vessel involvement. The most commonly used imaging techniques (in descending order) were CT-Chest/Abdomen/Pelvis, CT-angiogram, PET-CT and Vascular Ultrasound. 50% of patients underwent follow-up imaging, most commonly MR- or CT-angiography. Mean follow-up was for 1.6 years. The mean prednisolone dose at 3 months (n = 18) was 24mg daily and 8mg at 12 months (n = 12). 28% of patients relapsed during the follow-up period at 4, 5, 8, 9 and 24 months post-diagnosis. 7/18 patients were commenced on methotrexate for steroid-side effects or for relapse. 8/18 received subcutaneous tocilizumab in combination with methotrexate in two cases. Three patients were started on azathioprine but only one continued. Conclusion  In modern-day clinical practice, patients with LV-GCA experience a longer time to diagnosis than those with cranial symptoms. Patients with LV-GCA can experience an array of constitutional symptoms. Frequently, more than one imaging modality is required to confirm LV-GCA and the majority of patients will have seen other hospital specialists or have been admitted to hospital before diagnosis. Methotrexate and tocilizumab are the most frequently-used and effective steroid-adjunct in this single-centre cohort. Disclosure  O. Cronin: None. N.D. McKay: Consultancies; Gilead. Other; Has received support for conference attendance from Pfizer and Gilead, Has received educational support from UCB, Gilead, Celgene, Biogen, Sanofi, Abbvie, Novartis, Pfizer. H. Preston: None. H. Harris: None. B. Hauser: None.

Seven years follow-up of corneal cross-linking (CXL) in pediatric patients: Evaluation of treated and untreated eye

2021 ◽  
pp. 112067212110206
Author(s):  
Iliya Simantov ◽  
Lior Or ◽  
Inbal Gazit ◽  
Biana Dubinsky-Pertzov ◽  
David Zadok ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Pediatric Patients ◽  
Pediatric Population ◽  
Corneal Thickness ◽  
Cross Linking ◽  
Uncorrected Distance Visual Acuity ◽  
Mild Reduction ◽  
Change In The Mean ◽  
The Mean

Background: Retrospective cohort study evaluating long term keratoconus progression amongst cross-linking (CXL) treated pediatric patients in the treated and the fellow untreated eyes. Methods: Data on 60 eyes of 30 patients, 18 years old or younger, who underwent CXL in at least one eye was collected and analyzed. Follow-up measurements taken from the treated and untreated eye up to 7 years after CXL treatment, were compared to baseline measurements. Parameters included uncorrected distance visual acuity (UCDVA), best-corrected spectacle visual acuity (BCSVA), manifest refraction, pachymetry, corneal tomography, and topography. Results: Mean age of patients was 16 ± 2.1 years. For the treated eyes, during follow-up period mean UCDVA had improved (from 0.78 ± 0.22 at baseline to 0.58 ± 0.26 logMAR at 7 years; p = 0.13), as well as mean BCSVA (from 0.23 ± 0.107 at baseline to 0.172 ± 0.05 logMAR at 7 years; p = 0.37). The mean average keratometry showed a significant flattening (from 49.95 ± 4.04 to 47.94 ± 3.3 diopters (D); p < 0.001), However there was no change in the mean maximal keratometry. The mean minimal corneal thickness (MCT) showed a significant mild reduction of 26 µm ( p = 0.006). Although statistically insignificant, the mean manifest cylinder was also reduced to 2D ( p = 0.15). During the follow-up period, eight untreated eyes (26.6%) deteriorated and underwent CXL, while only one treated eye (3.33%) required an additional CXL. Conclusion: CXL is a safe and efficient procedure in halting keratoconus progression in the pediatric population, the fellow eye needs to be carefully monitored but only a 25% of the patients will require CXL in that eye during a period of 7 years.

scholarly journals Laser interstitial thermal therapy for subependymal giant cell astrocytoma: technical case report

2016 ◽  
Vol 41 (4) ◽  
pp. E9 ◽  
Author(s):  
David Y. A. Dadey ◽  
Ashwin A. Kamath ◽  
Eric C. Leuthardt ◽  
Matthew D. Smyth
Keyword(s):  
Giant Cell ◽  
Pediatric Population ◽  
Thermal Therapy ◽  
Subependymal Giant Cell Astrocytoma ◽  
Complication Rates ◽  
Open Resection ◽  
Laser Interstitial Thermal Therapy ◽  
Intracranial Lesions ◽  
Giant Cell Astrocytoma

Subependymal giant cell astrocytoma (SEGA) is a rare tumor occurring almost exclusively in patients with tuberous sclerosis complex. Although open resection remains the standard therapy, complication rates remain high. To minimize morbidity, less invasive approaches, such as endoscope-assisted resection, radiosurgery, and chemotherapy with mTOR pathway inhibitors, are also used to treat these lesions. Laser interstitial thermal therapy (LITT) is a relatively new modality that is increasingly used to treat a variety of intracranial lesions. In this report, the authors describe two pediatric cases of SEGA that were treated with LITT. In both patients the lesion responded well to this treatment modality, with tumor shrinkage observed on follow-up MRI. These cases highlight the potential of LITT to serve as a viable minimally invasive therapeutic approach to the management of SEGAs in the pediatric population.

scholarly journals Idiopathic retroperitoneal fibrosis: A report on 15 patients

2015 ◽  
Vol 72 (10) ◽  
pp. 928-931 ◽  
Author(s):  
Jovan Hadzi-Djokic ◽  
Tomislav Pejcic ◽  
Dragoslav Basic ◽  
Ivana Vukomanovic ◽  
Zoran Dzamic ◽  
...  
Keyword(s):  
Surgical Procedures ◽  
Retroperitoneal Fibrosis ◽  
Early Recognition ◽  
Signs And Symptoms ◽  
Omental Flap ◽  
Definitive Treatment ◽  
Pathological Examination ◽  
Idiopathic Retroperitoneal Fibrosis ◽  
The Mean

Background/Aim. Retroperitoneal fibrosis (RPF) represents a chronic pathological process characterized by fibrosis which entraps and compresses the ureters and the great blood vessels in the retroperitoneal space. A specific form of RPF is idiopathic RPF, an uncommon collagen vascular disease of unclear etiology. The series of 15 patients which underwent open surgical repair due to idiopathic RPF is presented herein. Methods. From 1989 to 2012, 11 male and 4 female patients underwent surgery due to primary RPF. The ureters were entrapped unilaterally (7 patients), or bilaterally (8 patients). Major symptoms included low back pain due to hydronephrosis (9 patients), uremia (4 patients), and urinary tract infection (2 patients). The diagnosis was based on intravenous urography (IVU), retrograde ureteropyelography and computed tomography (CT). Results. Surgical procedures included intraperitoneal ureteral displacement (8 patients) and ureteral wrapping with omental flap (6 patients). One patient underwent bilateral ureteral stenotic segments resection and oblique ureterography, followed by wrapping with omental flap. Pathological examination confirmed primary RPF in all patients. The mean operative time was 3.5 h (range 2.5-4.5 h). The average intrahospital stay was 21 days (range 16-26 days). The mean follow up was 32 months (6-46 months). During the follow up, 12 patients had improvement on IVU. Conclusion. Early recognition of signs and symptoms of RPF is of the utmost importance for the outcome. Surgical procedures, including ureteral wrapping with omental flap, or intraperitoneal ureteral displacement, usually represent definitive treatment.

scholarly journals Pediatric Peritoneal Epithelial Malignant Mesothelioma Case Report

2021 ◽  
Vol 2021 ◽  
pp. 1-7
Author(s):  
Elizabeth Bellew ◽  
Samantha Lee ◽  
Hiren Patel ◽  
Carolyn Fein Levy ◽  
Rachelle Goldfisher ◽  
...  
Keyword(s):  
Case Report ◽  
Malignant Mesothelioma ◽  
Crucial Role ◽  
Clinical Course ◽  
Pediatric Population ◽  
Adult Population ◽  
Initial Evaluation ◽  
Peritoneal Mesothelioma ◽  
Imaging Characteristics

We present a 14-year-old boy with peritoneal epithelial malignant mesothelioma (PEMM). While pathology is required to make this diagnosis, radiology plays a crucial role throughout the clinical course of this disease. The key imaging characteristics of peritoneal mesothelioma have been previously well-described in the adult population, but there are rare reports in the pediatric population. This pediatric report highlights the multidimensional use of imaging in this disease, from the initial evaluation to therapeutic supplementation and subsequent follow-up.

Surgical Outcome of Large Solid Posterior Fossa Hemangioblastoma without Preoperative Embolization

2021 ◽  
Author(s):  
Prashant Raj Singh ◽  
Raghvendra Kumar Sharma ◽  
Jitender Chaturvedi ◽  
Nitish Nayak ◽  
Anil Kumar Sharma
Keyword(s):  
Posterior Fossa ◽  
Surgical Outcome ◽  
Karnofsky Performance Status ◽  
Obstructive Hydrocephalus ◽  
Case Series ◽  
Preoperative Embolization ◽  
Initial Symptom ◽  
Lower Cranial Nerve ◽  
The Mean

Abstract Background Large solid hemangioblastoma in the posterior fossa has an abundant blood supply as an arteriovenous malformation. The presence of adjacent vital neurovascular structures makes them vulnerable and difficult to operate. Complete surgical resection is always a challenge to the neurosurgeon. Material and Method We share the surgical difficulties and outcome in this case series of large solid hemangioblastomas without preoperative embolization as an adjunct. This study included five patients (three men and two women, with a mean age of 42.2 years). Preoperative embolization was attempted in one patient but was unsuccessful. All the patients have headache (100%) and ataxia (100%) as an initial symptom. A ventriculoperitoneal shunt was inserted in one case before definite surgery due to obstructive hydrocephalus. The surgical outcome was measured using the Karnofsky Performance Status (KPS) score. Result The tumor was excised completely in all the cases. No intra- and postoperative morbidity occurred in four patients; one patient developed transient lower cranial nerve palsy. Mean blood loss was 235 mL, and no intraoperative blood transfusion was needed in any case. The mean follow-up period was 14.2 months. The mean KPS score at last follow-up was 80.One patient had a KPS score of 60. Conclusion Our treatment strategy is of circumferential dissection followed by en bloc excision, which is the optimal treatment of large solid hemangioblastoma. The use of adjuncts as color duplex sonography and indocyanine green video angiography may help complete tumor excision with a lesser risk of complication. Preoperative embolization may not be needed to resect large solid posterior fossa hemangioblastoma, including those at the cerebellopontine angle location.

Neurogenic claudication associated with posterior vertebral rim fractures in children

2012 ◽  
Vol 10 (3) ◽  
pp. 241-245 ◽  
Author(s):  
Jonathan G. Thomas ◽  
Jerome Boatey ◽  
Alison Brayton ◽  
Andrew Jea
Keyword(s):  
Blood Loss ◽  
Hospital Stay ◽  
Length Of Hospital Stay ◽  
Signs And Symptoms ◽  
Leg Pain ◽  
Neurogenic Claudication ◽  
Pediatric Age Group ◽  
Fractures In Children ◽  
The Mean

Object Outside of the patient population with achondroplasia, neurogenic claudication is rare in the pediatric age group. Neurogenic claudication associated with posterior vertebral rim fracture is even more uncommon but nonetheless causes pain and disability in affected children and adolescents. The purpose of this study was to describe the surgical results of 3 adolescents presenting with neurogenic claudication and posterior vertebral rim fracture when treated with laminectomy alone. Methods The medical and operative records of the 3 pediatric patients were retrospectively reviewed. Presenting signs and symptoms and CT findings, such as the interpedicular distances between T-12 and L-5, were obtained. Perioperative results were assessed, including operative time, blood loss, length of hospital stay, and complications. Findings at latest follow-up were also recorded, including a patient satisfaction survey. Results The 3 patients (1 girl and 2 boys) had a mean age of 14.7 years (range 14–15 years) and underwent follow-up for a mean of 11.3 months (range 5–18 months). Notable preoperative signs and symptoms included back pain (all patients), leg pain (all patients), leg numbness (1 patient), and leg weakness (1 patient). No patient presented with bowel and/or bladder dysfunction. The mean blood loss during laminectomy was 123 ml (range 20–300 ml), and the mean length of hospital stay was 4.3 days (range 3–6 days). On average, decompression was performed at 2.2 levels (range 2–2.5 levels). All 3 patients reported at most recent follow-up that they were “satisfied” with the surgery. There was 1 complication of instability from an iatrogenic pars fracture, which required reoperation and posterior instrumented fusion. Conclusions To the best of the authors' knowledge, this report represents the first surgical series of pediatric neurogenic claudication associated with posterior vertebral rim fractures. Pediatric neurosurgeons may infrequently encounter neurogenic claudication associated with a posterior vertebral rim fracture in children. To treat children with neurogenic claudication associated with posterior vertebral rim fractures, a simple laminectomy may be a safe and efficacious alternative to discectomy and removal of fracture fragments.

First experiences with an adjustable gravitational valve in childhood hydrocephalus

2009 ◽  
Vol 3 (2) ◽  
pp. 90-93 ◽  
Author(s):  
Veit Rohde ◽  
Ernst-Johannes Haberl ◽  
Hans Ludwig ◽  
Ulrich-W. Thomale
Keyword(s):  
Success Rate ◽  
Clinical Improvement ◽  
Pediatric Population ◽  
Clinical Results ◽  
Opening Pressure ◽  
Pressure Unit ◽  
The Mean ◽  
Childhood Hydrocephalus ◽  
Gravitational Unit

Object The goal of this report was to describe the authors' initial experiences with an adjustable gravity-assisted valve (GAV) called the ProGAV in treating childhood hydrocephalus. Methods The ProGAV was implanted in 53 children (29 boys and 24 girls, median age 7.3 years) with hydrocephalus of various origins. The ProGAV consists of a differential-pressure unit with adjustable opening pressures and a gravitational unit with a fixed opening pressure. Results The mean follow-up period was 15.2 months (range 6–44 months). The authors did not observe any valve-related complications. Four infections (7.5%) occurred, warranting the removal of the shunt. In 19 children, the opening pressure was changed at least once during the follow-up period, for underdrainage in 10, overdrainage in 8, and shunt weaning in 1, with substantial clinical improvement in 18 children. Overall, good clinical results were obtained in 47 (88.7%) of the 53 valve placements. Conclusions With an overall success rate of 88.7%, the first experiences with the ProGAV in childhood hydrocephalus are promising and justify its further use in the pediatric population.

GIANT CELL TUMOURS OF TENDON SHEATH OF HAND: CAUSES AND STRATEGIES TO PREVENT RECURRENCE

Hand Surgery ◽  
2011 ◽  
Vol 16 (02) ◽  
pp. 149-154 ◽  
Author(s):  
Azal Jalgaonkar ◽  
Baljinder Dhinsa ◽  
Howard Cottam ◽  
Ganapathyraman Mani
Keyword(s):  
Risk Factors ◽  
Local Recurrence ◽  
Giant Cell ◽  
Recurrence Rate ◽  
Case Series ◽  
Tendon Sheath ◽  
Giant Cell Tumours ◽  
High Incidence ◽  
The Mean

Giant cell tumours of tendon sheath of hand present a surgical dilemma due to their high incidence of local recurrence. We present a case series of 46 patients with 47 histologically confirmed giant cell tumours of tendon sheath over a ten-year period from 1998 to 2008. The mean follow-up was 47 months (range 25–124 months). We identified tumours with bony erosions and piecemeal resections as predictors of recurrence. Our recurrence rate of 9% was at the lower end of spectrum of previously published reports (range 7%–44%). We recommend "en-masse" excision of these tumours. All the patients with suspicion of these tumours should have preoperative radiographs to identify erosions. A thorough curettage of the bone should be done in cases with osseous erosion to prevent recurrence. Patients with these risk factors should be followed up annually for five years and be warned about recurrence.

Endoscopic endonasal skull base surgery in the pediatric population

2013 ◽  
Vol 11 (3) ◽  
pp. 227-241 ◽  
Author(s):  
Srinivas Chivukula ◽  
Maria Koutourousiou ◽  
Carl H. Snyderman ◽  
Juan C. Fernandez-Miranda ◽  
Paul A. Gardner ◽  
...  
Keyword(s):  
Skull Base ◽  
Pituitary Adenomas ◽  
Skull Base Surgery ◽  
Pediatric Population ◽  
Skull Base Tumors ◽  
Endoscopic Endonasal Surgery ◽  
Endonasal Surgery ◽  
Endoscopic Endonasal ◽  
The Mean

Object The use of endoscopic endonasal surgery (EES) for skull base pathologies in the pediatric population presents unique challenges and has not been well described. The authors reviewed their experience with endoscopic endonasal approaches in pediatric skull base surgery to assess surgical outcomes and complications in the context of presenting patient demographics and pathologies. Methods A retrospective review of 133 pediatric patients who underwent EES at our institution from July 1999 to May 2011 was performed. Results A total of 171 EESs were performed for skull base tumors in 112 patients and bony lesions in 21. Eighty-five patients (63.9%) were male, and the mean age at the time of surgery was 12.7 years (range 2.3–18.0 years). Skull base tumors included angiofibromas (n = 24), craniopharyngiomas (n = 16), Rathke cleft cysts (n = 12), pituitary adenomas (n = 11), chordomas/chondrosarcomas (n = 10), dermoid/epidermoid tumors (n = 9), and 30 other pathologies. In total, 19 tumors were malignant (17.0%). Among patients with follow-up data, gross-total resection was achieved in 16 cases of angiofibromas (76.2%), 9 of craniopharyngiomas (56.2%), 8 of Rathke cleft cysts (72.7%), 7 of pituitary adenomas (70%), 5 of chordomas/chondrosarcomas (50%), 6 of dermoid/epidermoid tumors (85.7%), and 9 cases of other pathologies (31%). Fourteen patients received adjuvant radiotherapy, and 5 received chemotherapy. Sixteen patients (15.4%) showed tumor recurrence and underwent reoperation. Bony abnormalities included skull base defects (n = 12), basilar invagination (n = 4), optic nerve compression (n = 3) and trauma (n = 2); preexisting neurological dysfunction resolved in 12 patients (57.1%), improved in 7 (33.3%), and remained unchanged in 2 (9.5%). Overall, complications included CSF leak in 14 cases (10.5%), meningitis in 5 (3.8%), transient diabetes insipidus in 8 patients (6.0%), and permanent diabetes insipidus in 12 (9.0%). Five patients (3.8%) had transient and 3 (2.3%) had permanent cranial nerve palsies. The mean follow-up time was 22.7 months (range 1–122 months); 5 patients were lost to follow-up. Conclusions Endoscopic endonasal surgery has proved to be a safe and feasible approach for the management of a variety of pediatric skull base pathologies. When appropriately indicated, EES may achieve optimal outcomes in the pediatric population.

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