Incidence, clinical features, and treatment of familial moyamoya in pediatric patients: a single-institution series

OBJECTIVELimited data exist on familial moyamoya in children. The purpose of this study was to characterize presentation and outcomes of pediatric moyamoya patients who have relatives diagnosed with moyamoya.METHODSThe authors performed a single-institution retrospective analysis of a case series including all surgically treated children with moyamoya with first- or second-degree relatives with moyamoya. Clinical and radiographic characteristics were analyzed, along with surgical outcomes.RESULTSA total of 537 patients underwent surgery for moyamoya during the study period. Eighteen of those patients (3.4% of the total series) had moyamoya and a family history of moyamoya and were included in this study. Of these 18 patients, 14 were non-twin siblings, and the remaining 4 represented 2 pairs of identical (affected) twins.The presentation was predominantly ischemic (72%), but 4 patients (33%) were asymptomatic when they were found to have moyamoya. Bilateral disease was present in 13 patients (72%). Radiographic stroke prevalence (67%), Suzuki grade (3.3), and angiographic findings were comparable to findings in nonfamilial moyamoya patients. Thirty revascularization procedures were performed, with a 3.3% operative stroke rate per hemisphere and no new strokes in an average follow-up period of 4.5 years.CONCLUSIONSIn a North American surgical series, familial moyamoya existed in 3.4% of cases, and was distinguished by manifesting in a broad range of ethnic groups, with a higher proportion of male patients and increased rates of asymptomatic and unilateral disease in comparison to nonfamilial moyamoya. Screening indications remain controversial and the current data are used to suggest guidelines. Surgical therapy is warranted, effective, and durable in these patients, but patients should be carefully selected.

Download Full-text

ISCHEMIC STROKE

The Professional Medical Journal ◽

10.29309/tpmj/2016.23.08.1664 ◽

2016 ◽

Vol 23 (08) ◽

pp. 925-931

Author(s):

Ramesh Kumar ◽

Muhammad Iqbal Shah ◽

Manzoor Ali Lakhair ◽

Zaheer Memon

Keyword(s):

Diabetes Mellitus ◽

Ischemic Stroke ◽

Case Series ◽

Risk Elements ◽

Stroke Prevalence ◽

Stroke Study ◽

Study Results ◽

Low Hdl ◽

History Of

Objectives: To determine the frequency of Dyslipidemia and other risk factorsin patients with ischemic stroke. Study design: Descriptive and case series. Setting: Thisresearch study was carried out in the Medicine department of Liaquat University HospitalJamshoro/Hyderabad. Duration of study: Six months. Sample size: Total 100 patients of bothmale and female sex were enrolled in this research study. Results: Total 100 cases of ischemicstroke were included. The mean age + SD (range) was 59.72 + 6.40 (45 – 70 years), 76(76.0%)were males and 24(24.0%) were females. Speech deficits was present in 28(28.0%, n = 100)patients. Eighty seven (87.0%, n = 100) patients had difficulty in walking, 17(17.0%, n = 100)patients had seizures, confusion was observed in 35(35.0%, n = 100) patients, 35(35.0%, n =100) had headache, Vertigo was seen in 11(11.0%n = 100) patients, Visual disturbances wasseen in 14(14.0%, n = 100) cases and 20(20.0%n = 100) patients had vomiting. High bloodpressure was in 51(51.0%, n = 100), diabetes mellitus and dyslipidemia in 30(30.0%, n = 100)respectively. Eighteen (18.0%, n = 100) patients were known cases of cardiovascular diseases,36(36.0%, n = 100) were smokers, 22(22.0%, n = 100) patients had family history of strokeand only 2(2.0%, n = 100) patients had the history of alcoholism. High cholesterol (mg/dL) wasseen in 21(21.0% n = 30) patients, High LDL (mg/dL) in 37(37.0%, n = 30), Low HDL (mg/dL) in90(90.0%, n = 30) and High Triglycerides (mg/dL) were seen in 40(40.0%, n = 30). Conclusion:High Blood pressure, Diabetes mellitus, Dyslipidemia and Smoking are chief risk elements ofischemic stroke. Prevalence of stroke is high in poor socioeconomic demographics with singleor multiple risk elements and this may be because of unawareness about these risk elements,unaffordability of treatment or proper follow up.

Download Full-text

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

Journal of Neurosurgery Pediatrics ◽

10.3171/2019.6.peds18716 ◽

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.

Download Full-text

THU0319 DEVELOPMENT AND OUTCOME OF AORTIC COMPLICATIONS DURING TOCILIZUMAB TREATMENT OF GCA AND HISTOPATHOLOGIC EVIDENCE OF RESIDUAL INFLAMMATION-A CASE SERIES.

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.4660 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 388.2-389

Author(s):

A. Rubbert-Roth ◽

P. K. Bode ◽

T. Langenegger ◽

C. Pfofe ◽

T. Neumann ◽

...

Keyword(s):

Structural Changes ◽

Case Series ◽

Aortic Aneurysms ◽

Imaging Data ◽

Limited Data ◽

Retrospective Case ◽

Pet Ct ◽

Fdg Pet Ct ◽

Pet Ct Scan

Background:Giant cell arteritis (GCA) may affect the aorta and the large aortic branches and lead to dissections and aortic aneurysms. Tocilizumab (TCZ) treatment has the capacity to control aortic inflammation as has been demonstrated by CRP normalization and imaging data. However, limited data are available on the histopathological findings obtained from patients who underwent surgery because of aortic complications during TCZ treatment.Objectives:We report on 5 patients with aortitis who were treated with TCZ and developed aortic complications.Methods:We describe a retrospective case series of patients with GCA treated with TCZ, who presented in our clinic between 2011 and 2019. Three patients underwent surgery. Histopathologic examination was performed in specimen from all of them.Results:Five female patients were diagnosed with GCA (4/5) or Takaysu arteritis (1/5) involving the aorta, all them diagnosed by MR angiography and/or FDG PET CT scan. Three patients (one with aortic aneurysm, one with dissection) underwent surgery after having been treated with TCZ for seven weeks, nine months and four years, respectively. Imaging before surgery showed remission on MRI and/or PET-CT in all cases. At the time of surgery, all patients showed normalized CRP and ESR values. Histopathological evaluation of the aortic wall revealed infiltrates, consisting predominantly of CD3+CD4+ T cells. Enlargement of pre-existing aneuryms was observed in the other two patients 10 weeks and 4 months after discontinuation of TCZ, respectively. Both patients were not eligible for surgical intervention and died during follow-up.Conclusion:Our case series suggests that during treatment with TCZ, regular imaging is necessary in this patient population to detect development of structural changes such as aneurysms or dissections. Despite treatment, residual inflammation might persist which could contribute to eventual aortic complications.Disclosure of Interests:Andrea Rubbert-Roth Consultant of: Abbvie, BMS, Chugai, Pfizer, Roche, Janssen, Lilly, Sanofi, Amgen, Novartis, Peter Karl Bode: None declared, Thomas Langenegger: None declared, Claudia Pfofe: None declared, Thomas Neumann: None declared, Olaf Chan-Hi Kim: None declared, Johannes von Kempis Consultant of: Roche

Download Full-text

Ultrasound Biomicroscopy Documented Anterior Uveal Melanoma Regression after Ruthenium-106 Plaque Therapy

Ocular Oncology and Pathology ◽

10.1159/000512030 ◽

2021 ◽

pp. 1-9

Author(s):

Biljana Kuzmanović Elabjer ◽

Mladen Bušić ◽

Andrej Pleše ◽

Mirjana Bjeloš ◽

Daliborka Miletić ◽

...

Keyword(s):

Uveal Melanoma ◽

Tumor Regression ◽

Corneal Thickness ◽

Case Series ◽

Ultrasound Biomicroscopy ◽

Close Monitoring ◽

Single Institution ◽

Retrospective Case ◽

Caucasian Patients

Introduction: Ultrasound biomicroscopy (UBM) is the only widely used method for the evaluation of anterior uveal melanoma (AUM). Objective: Documentation of regression of AUM treated with ruthenium-106 (Ru-106) plaque types CCB and CCC using UBM. Methods: This single institution-based retrospective case series involved 10 Caucasian patients with AUM followed after brachytherapy with UBM from January 2014 until February 2019. The largest prominence of the tumor perpendicular to the sclera or the cornea (including scleral/corneal thickness) (D) and the largest basal dimension (B) were measured in millimeters with UBM for all patients prior to the brachytherapy and at 4-month interval follow-up. Tumor regression was calculated as a percentage of decrease in the initial D and B values. Results: The study involved 10 patients with a mean age of 64.4 years (yr) (range 46–80 yr). D ranged from 1.82 to 5.5 mm (median 2.99 mm) and B from 2.32 to 12.38 mm (median 4.18 mm). The apical radiation dose in all patients was 100 Gy. The median follow-up was 42.02 months. Regression for D was 21.11 ± 13.66%, 31.09 ± 14.66%, and 34.92 ± 19.86% at 1st, 2nd, and 3rd year of the follow-up, respectively, while for B it was 21.58 ± 16.05%, 28.98 ± 17.71%, and 32.06 ± 18.96%, respectively. Tumor recurrence was documented in 2/10 patients. Conclusion: The major regression of AUM, treated with Ru-106 plaque types CCB and CCC, was documented in the first 2 years after brachytherapy in our study group. In the following years, only minimal regression was documented that warns of the need for close monitoring and active search for local recurrences.

Download Full-text

Cryobiopsy as a New Tool for Complications Diagnosis During Follow-up After Lung Transplantation: Single Institution Case Series

Transplantation Proceedings ◽

10.1016/j.transproceed.2021.03.041 ◽

2021 ◽

Author(s):

Sławomir Żegleń ◽

Wojtek Karolak ◽

Gabriela Mikołajczyk ◽

Nicole Geryk ◽

Zuzanna Gogulska ◽

...

Keyword(s):

Lung Transplantation ◽

Case Series ◽

Single Institution

Download Full-text

Total Ankle Arthroplasty Survivorship, Complication, and Revision Rates in Patients Younger Than 55 Years

Foot & Ankle Specialist ◽

10.1177/1938640020980925 ◽

2021 ◽

pp. 193864002098092

Author(s):

Devon W. Consul ◽

Anson Chu ◽

Travis M. Langan ◽

Christopher F. Hyer ◽

Gregory Berlet

Keyword(s):

Case Series ◽

Ankle Arthrodesis ◽

Total Ankle Replacement ◽

Implant Survival ◽

Ankle Arthritis ◽

Single Institution ◽

Retrospective Case ◽

Ankle Replacement ◽

End Stage

Total ankle replacement has become a viable alternative to ankle arthrodesis in the surgical management of advanced ankle arthritis. Total ankle replacement has generally been reserved for patients who are older and for those who will have a lower demand on the replacement. The purpose of the current study is to review patient outcomes, complications, and implant survival in patients younger than 55 years who underwent total ankle replacement at a single institution. A single-center chart and radiographic review was performed of consecutive patients who underwent total ankle replacement for treatment of end-stage ankle arthritis. All surgeries were performed by 1 of 5 fellowship-trained foot and ankle surgeons at a single institution. A total of 51 patients met inclusion criteria with a mean follow-up of 31.2 months (SD = 16.2). Implant survival was 94%, There were 7 major complications (13%) requiring an unplanned return to the operating room and 8 minor complications (15%) that resolved with conservative care. The results of this study show that total ankle replacement is a viable treatment option for patients younger than 55 years. Levels of Evidence: A retrospective case series

Download Full-text

GCT-27. CLINICAL FEATURES AND PROGNOSTIC FACTORS OF NONGERMINOMATOUS GERM CELL TUMORS IN 111 CONSECUTIVE PATIENTS IN A SINGLE INSTITUTION: IMPACT OF IRRADIATION AND CHEMOTHERAPY CYCLES ON SURVIVAL

Neuro-Oncology ◽

10.1093/neuonc/noaa222.247 ◽

2020 ◽

Vol 22 (Supplement_3) ◽

pp. iii333-iii333

Author(s):

Lei Wen ◽

Juan Li ◽

Qingjun Hu ◽

Mingyao Lai ◽

Cheng Zhou ◽

...

Keyword(s):

Prognostic Factors ◽

Germ Cell ◽

Chinese Population ◽

Germ Cell Tumors ◽

Tumor Diameter ◽

Limited Data ◽

Single Institution ◽

Large Institution ◽

Significant Difference

Abstract BACKGROUND Limited data is available in intracranial nongerminomatous germ cell tumors (NGGCTs) in Chinese population. Here we aimed to retrospectively assess the clinical-pathological and prognostic factors of NGGCTs in a single large institution in China. METHODS From June 2003 to December 2018, 111 consecutive NGGCTs were treated in Guangdong Sanjiu Brain Hospital, China. RESULTS The median follow-up was 36.2 months (range, 1.2 to 131.2 months). Three-year EFS and OS for 111 NGGCTs patients were 78.5%±4.5% and 82.8%±4.0%, respectively. 98 patients received CSI plus boost yielded better survival than those who received reduced-volume radiotherapy or no radiotherapy (3y OS, 86.7% vs. 51.4%, p=0.007). Patients had at least four cycles of chemotherapy were strongly associated with improved 3-year OS, compared to those received less than 4 cycles (94.1% vs. 63.6%, p＜0.001). There was no significant difference in survival of patients stratified by age, surgery, hydrocephalus, as well as tumor diameter. Multivariate analysis identified chemotherapy cycles less than 4 was the only prognostic factor that conferring a worse OS (p=0.003). Patients both received CSI and at least 4 courses of chemotherapy were correlated with lower incidence of relapse (p=0.044). CONCLUSIONS Multimodal approach including CSI and enough courses of chemotherapy was effective and should be recommended for the treatment of newly diagnosed NGGCTs in Chinese population.

Download Full-text

Sialendoscopy With Intraductal Steroid Irrigation in Patients With Sialadenitis Without Sialoliths

Ear Nose & Throat Journal ◽

10.1177/0145561319841207 ◽

2019 ◽

Vol 98 (5) ◽

pp. 291-294 ◽

Cited By ~ 4

Author(s):

Saudamini J. Lele ◽

Mickie Hamiter ◽

Torrey Louise Fourrier ◽

Cherie-Ann Nathan

Keyword(s):

Treatment Option ◽

Case Series ◽

Complete Response ◽

Definitive Treatment ◽

Invasive Technique ◽

Effective Treatment Option ◽

Patient Reported ◽

History Of ◽

The Mean

Sialendoscopy has emerged as a safe, effective and minimally invasive technique for management of obstructive and inflammatory salivary gland disease. The aim of our study was to analyze outcomes of sialendoscopy and steroid irrigation in patients with sialadenitis without sialoliths. We performed a retrospective analysis of patients who underwent interventional sialendoscopy with steroid irrigation from 2013 to 2016, for the treatment of sialadenitis without sialolithiasis. Twenty-two patients underwent interventional sialendoscopy with ductal dilation and steroid irrigation for the treatment of sialadenitis without any evidence of sialolithiasis. Conservative measures had failed in all. Eleven patients had symptoms arising from the parotid gland, 4 patients had symptoms arising from the submandibular gland, while 6 patients had symptoms in both parotid and submandibular glands. One patient complained of only xerostomia without glandular symptoms. The mean age of the study group which included 1 male and 21 females was 44.6 years (range: 3-86 years). Four patients had autoimmune disease, while 7 patients had a history of radioactive iodine therapy. No identifiable cause for sialadenitis was found in the remaining 11 patients. The mean follow-up period was 378.9 days (range: 16-1143 days). All patients underwent sialendoscopy with ductal dilation and steroid irrigation. Twelve patients showed a complete response and 9 patients had a partial response, while 1 patient reported no response. Only 3 patients required repeat sialendoscopy. The combination of sialendoscopy with ductal dilation and steroid irrigation is a safe and effective treatment option for patients with sialadenitis without sialoliths refractory to conservative measures. Prospective studies with a larger case series are needed to establish its role as a definitive treatment option.

Download Full-text

Cerebrovascular fibromuscular dysplasia

Neurology Clinical Practice ◽

10.1212/cpj.0000000000000339 ◽

2017 ◽

Vol 7 (3) ◽

pp. 225-236 ◽

Cited By ~ 9

Author(s):

Andrea M. Harriott ◽

Eli Zimmerman ◽

Aneesh B. Singhal ◽

Michael R. Jaff ◽

Mark E. Lindsay ◽

...

Keyword(s):

Disease Progression ◽

Fibromuscular Dysplasia ◽

Massachusetts General Hospital ◽

Case Series ◽

Antiplatelet Agents ◽

Cerebrovascular Event ◽

Symptomatic Disease ◽

Surveillance Imaging ◽

History Of

AbstractBackground:Fibromuscular dysplasia (FMD) is a rare noninflammatory, nonatherosclerotic arteriopathy of medium-sized arteries affecting up to 7% of the population. The disease can affect any artery but commonly affects renal, extracranial carotid, and vertebral arteries. The epidemiology and natural course of cerebrovascular FMD is unknown and requires further investigation.Methods:We present demographic and outcomes data on a case series of 81 patients with cerebrovascular FMD from Massachusetts General Hospital presenting between 2011 and 2015 followed by a review of the peer-reviewed literature.Results:Patients were a median age of 53 years (±12 SD) and the majority were women. Approximately 50% had a history of tobacco use and more than two-thirds had hypertension. Most patients were on monoplatelet therapy with aspirin; during follow-up, 7 of 67 had progressive disease or additional symptoms. One of 67 patients had a cerebrovascular event: TIA. There were 5 of 67 who had noncerebrovascular events or disease progression and 1 death of unclear cause.Conclusions:Cerebrovascular FMD may present with myriad symptoms. Our data support that patients with FMD with symptomatic disease have a low rate of recurrent symptoms or disease progression and can be managed conservatively with stroke risk modification, antiplatelet agents, surveillance imaging, and counseling.

Download Full-text

Effectiveness and safety of endoscopic radial incision and cutting for severe benign anastomotic stenosis after surgery for colorectal carcinoma: a three-case series

Endoscopy International Open ◽

10.1055/s-0043-124470 ◽

2018 ◽

Vol 06 (03) ◽

pp. E335-E339 ◽

Cited By ~ 3

Author(s):

Naoki Asayama ◽

Shinji Nagata ◽

Kenjiro Shigita ◽

Taiki Aoyama ◽

Akira Fukumoto ◽

...

Keyword(s):

Colorectal Carcinoma ◽

Surgical Resection ◽

Case Series ◽

Anastomotic Stenosis ◽

Endoscopic Dilation ◽

Limited Data ◽

Radial Incision ◽

Hiroshima City ◽

Median Interval

AbstractBenign colonic anastomotic stenosis sometimes occurs after surgical resection and usually requires surgical or endoscopic dilation. Limited data are available on the effectiveness and safety of the endoscopic radial incision and cutting (RIC) method at sites other than the esophagus. The aim of this retrospective study was to investigate the effectiveness and safety of RIC dilation for severe benign anastomotic colonic stenosis. Subjects were 3 men (median age 72 years, range 65 – 76 years) who developed severe benign anastomotic stenosis after surgical resection for colorectal carcinoma and were subsequently treated by RIC dilation at Hiroshima City Asa Citizens Hospital between May 2014 and December 2016. Severe anastomotic stenosis was defined as a narrowed anastomosis through which a standard colonoscope could not be passed. The median interval from surgery to RIC was 21 months (range 9 – 29 months). RIC was successful in all 3 patients and reduced the severity of dyschezia postoperatively; 2 patients experienced improvement after a single RIC session and the other after 6 RIC sessions. No treatment-related adverse events or re-stenosis requiring repeat dilation was noted during a median follow-up of 27 months (range 8 – 37 months). Our findings indicate that the RIC technique can be applied safely and effectively to various sites in the colon, avoiding the need for reoperation.

Download Full-text