Long-term follow-up of surgically treated juvenile patients with moyamoya disease

2012 ◽  
Vol 10 (5) ◽  
pp. 451-456 ◽  
Author(s):  
Maki Mukawa ◽  
Tadashi Nariai ◽  
Yoshiharu Matsushima ◽  
Yoji Tanaka ◽  
Motoki Inaji ◽  
...  

Object Surgical revascularization is considered an effective treatment for juvenile patients with moyamoya disease (MMD). Yet the long-term outcome in surgically treated patients still needs to be clarified. More than 30 years have passed since the authors' department started intensively treating pediatric patients with MMD using indirect anastomosis techniques. In this study the authors surveyed the current status of these patients. Methods Activities of daily living (ADLs) were surveyed and present clinical status was assessed based on the modified Rankin Scale (mRS). Cerebrovascular events subsequent to surgical treatment were also recorded. Results Since 1979, 208 patients younger than 19 years of age with MMD were surgically treated and followed up for > 3 years. Data were available on 172 patients (83%), who had been followed up for a mean of 14.3 years (range 3–32 years). Activity of daily living outcomes were as follows: 138 patients (80.2%) had mRS scores of 0–2, 29 (16.9%) a score of 3, 1 (0.6%) a score of 4, 1 (0.6%) a score of 5, and 3 (1.7%) a score of 6. Cerebrovascular events occurred 8 or more years after surgery in 6 patients (3.4%), that is, 6 hemorrhages and 3 infarctions. The cumulative risk of late-onset stroke at 10, 20, and 30 years after surgical intervention was 0.8%, 6.3%, and 10.0%, respectively. Conclusions This long-term survey demonstrated that most surgically treated pediatric patients with MMD maintain good ADL outcomes. However, a significant number of new cerebrovascular events occurred more than 10 years after the initial surgery. Additional follow-up will help to identify which events may occur during the adult years of patients treated as children.

Stroke ◽  
2020 ◽  
Vol 51 (Suppl_1) ◽  
Author(s):  
Satoshi Kuroda ◽  
Naoki Nakayama ◽  
Shusuke Yamamoto ◽  
Daina Kashiwazaki ◽  
Haruto Uchino ◽  
...  

Background and Purpose: Surgical revascularization is now known to improve the outcome in patients with moyamoya disease. However, majority of previous studies reported their short-term (<5 years) outcome. Therefore, this study was aimed to evaluate long-term (5 to 20 years) outcome after STA-MCA anastomosis and ultimate indirect bypass, encephalo-duro-myo- arterio-pericranial synangiosis (EDMAPS). Methods: Cumulative incidence of late morbidity/mortality and disease progression were evaluated among 93 patients who underwent STA-MCA anastomosis and EDMAPS. All of them were prospectively followed up for longer than 5 years post-surgery (mean, 10.5±4.4 years). There were 35 pediatric and 58 adult patients. Clinical diagnosis included TIA or ischemic stroke in 80 patients, hemorrhagic stroke in 10, and asymptomatic in 3. STA-MCA anastomosis and EDMAPS were performed onto their 141 hemispheres. MRI and MRA were performed every 6 or 12 years during follow-up periods. Results: During follow-up periods, 92/93 patients were free from any stroke or death, but one recurred hemorrhagic stroke (0.10% per patient-year). Disease progression occurred in the territory of the contralateral carotid or posterior cerebral artery (PCA) in 19 hemispheres of 15 patients (1.5% per patient-year). The interval between initial surgery and disease progression varied from 0.5 to 15 years. Repeat bypass surgery for anterior and posterior circulations resolved ischemic attacks in all 10 patients. Conclusion: STA-MCA anastomosis and EDMAPS would be the best choice to prevent further cerebrovascular events for longer than 10 years by widely providing surgical collaterals to both the MCA and ACA territories. However, regular follow-up would be essential for longer than 10 years post-surgery to identify the disease progression in the territory of contralateral carotid artery and PCA and prevent late cerebrovascular events.


2020 ◽  
Vol 58 (1) ◽  
pp. 86-93 ◽  
Author(s):  
Erik Beckmann ◽  
Andreas Martens ◽  
Heike Krüger ◽  
Wilhelm Korte ◽  
Tim Kaufeld ◽  
...  

Abstract OBJECTIVES Aortic valve-sparing root replacement is an excellent treatment option for patients with intact tricuspid aortic valves. However, the durability in patients with bicuspid aortic valves is still a matter of debate. The aim of this study was to analyse the short- and long-term outcomes in patients with bicuspid aortic valves. METHODS Between July 1993 and October 2015, a total of 582 patients underwent the David I procedure, 50 of whom had a bicuspid aortic valve. In these patients, the mean age was 46 ± 13 years, and 80% (n = 40) were men. Follow-up was complete for 100% of patients and comprised a total of 552 patient-years with a mean follow-up time of 11 ± 5.2 years. RESULTS In addition to the David procedure, cusp plication was performed in 34% (n = 17) of patients and commissure repair in 8% (n = 4) of patients. There were no in-hospital deaths, and 1 patient (2%) had a perioperative stroke. The 1-, 5-, 10- and 20-year survival rates of patients after discharge were 98%, 94%, 88% and 84%, respectively. The rates for freedom from valve-related reoperation at 1, 5, 10 and 20 years after initial surgery were 98%, 88%, 79% and 74%, respectively. Long-term freedom from reoperation was comparable to that of patients with tricuspid aortic valves (P = 0.2). CONCLUSIONS The David procedure has excellent short-term results in patients with a bicuspid aortic valve. In this study, the long-term durability of reimplanted bicuspid aortic valves was comparable to that of tricuspid valves. However, careful patient selection might have contributed to this, and further studies might be needed to definitely clarify this issue.


2020 ◽  
Vol 11 ◽  
Author(s):  
Bin Gao ◽  
Kaijiang Kang ◽  
Jia Zhang ◽  
Dong Zhang ◽  
Xingquan Zhao

Background: Headache associated with Moyamoya disease (HAMD) in the Chinese population is not well-described. The long-term outcome of surgical revascularization and natural course of HAMD has not been disclosed either.Methods: A headache screening questionnaire in China based on the ICHD2 and a face-to-face interview performed by an experienced neurologist were used to investigate headache characteristics and frequency and pain intensity in the 3 months before admission, and a telephone interview was used for the follow-up of a large cohort of 119 Chinese patients with HAMD.Results: Headache intensity was rated as scores of 5.9 ± 2.0 on a visual analog scale (VAS), ranging from 0 to 10, in the 3 months before admission. Forty-six patients (38.6%) were categorized as having migraine-like headaches, 29 patients (24.3%) were categorized as having tension type-like headaches, and 44 patients (36.9%) had a combination of both. The majority of patients had migraine-like headaches (n = 34, 73.9%) with a migrainous aura. Both the frequency and intensity of the headache improved significantly in patients treated with surgical revascularization (n = 96, 80.7%) or the conservative treatment (n = 23, 19.3%) in a long-term follow-up.Conclusion: HAMD frequently presented with a migraine-like headache (75.5% in total). A tension type headache was present in 60.9% of patients. The symptom of dizziness is common in patients with HAMD (60.5%), and 19 of them (26.4%) met the diagnose of vestibular migraine. Both intensity and frequency of HAMD show a trend of spontaneous remission in a long-term follow-up, and there is no difference in long-term outcomes of HAMD between surgical revascularization and conservative treatment, which indicates that the effect of bypass intervention on HAMD may be a placebo effect.


2011 ◽  
Vol 114 (3) ◽  
pp. 747-755 ◽  
Author(s):  
Marco Schiariti ◽  
Pablo Goetz ◽  
Hussien El-Maghraby ◽  
Jignesh Tailor ◽  
Neil Kitchen

Object Hemangiopericytomas are rare tumors that behave aggressively with a high rate of local recurrence and distant metastases. With the aim of determining the outcome and response to various treatment modalities, a series of 39 patients who underwent microsurgical resection for primary meningeal hemangiopericytoma over a 24-year period is presented. Methods Patients with hemangiopericytoma were identified from histopathology records and their medical records were analyzed retrospectively by 2 independent reviewers to collect data on surgical treatment, adjuvant therapy, postoperative course, local or distant recurrence, and follow-up. Results Of the 39 patients, 19 were male and 20 were female. Mean patient age was 44.1 years. Thirty-four tumors were intracranial and 5 were spinal. The mean follow-up period was 123 months. Twenty-eight patients developed local recurrence. The recurrence rate at 1, 5, and 15 years was 3.5%, 46%, and 92%, respectively. Extraneural metastasis occurred in 8 patients (26%) at an average of 123 months after initial surgery. Recurrences and metastases were treated by surgical excision, external beam radiation therapy (EBRT), chemotherapy, and/or stereotactic radiosurgery. Adjuvant EBRT following initial surgery was found to extend the disease-free interval from 154 months to 254 months, although it did not prevent the development of metastasis. In those patients with EBRT and complete resection, the mean recurrence-free interval was found to be 126.3 months longer (p = 0.04) and overall survival 126 months longer than without EBRT. Furthermore, adjusting for resection, patients undergoing EBRT had 0.33 times increased risk of recurrence compared with those who did not (p = 0.03). A majority of patients remained able to live independently despite revision surgery for recurrence. Conclusions The mean follow-up of this patient series represents the longest follow-up duration published to date and demonstrates extended survival in a significant number of patients with hemangiopericytoma. Gross-total resection followed by adjuvant EBRT provides patients with the highest probability of an increased recurrence-free interval and overall survival. Prolonged survival justifies long-term follow-up and aggressive treatment of initial, recurrent, and metastatic disease.


Author(s):  
Erik Beckmann ◽  
Andreas Martens ◽  
Heike Krueger ◽  
Wilhelm Korte ◽  
Tim Kaufeld ◽  
...  

Introduction Aortic valve-sparing root replacement (David's procedure) is an especially appealing treatment option for young patients. Here, we present the short-, mid, and long-term outcomes of this operation in adolescent patients. Methods Between September 1994 and March 2014, 29 patients aged 6 to 21 years underwent the David-I procedure at our center. We conducted a retrospective study with follow-up. Results The mean age was 16.8 ± 3.4 years and 90% (n = 26) were male. Marfan's syndrome was present in 86% (n = 25) of cases. Ninety-seven percent (n = 28) of cases were performed electively, and one case (3%) was performed emergently for acute aortic dissection type A. There were no early perioperative deaths (0%). Follow-up, which was completed on 100% of patients, comprised a total of 394 patient years and a mean follow-up time of 13.6 ± 5.4 years. The estimates for survival at 1, 5, and 10 years after initial surgery were 100, 97, and 93%, respectively. During follow-up, there were four (14%) late deaths and five (17%) aortic valve-related reoperations. The 1-, 5-, and 10-year estimates for freedom from valve-related reoperation were 100, 86, and 83%, respectively. The perioperative mortality for these five reoperations was 0%. Discussion Aortic valve–sparing root replacement can be performed in adolescents with a very low perioperative risk. Long-term survival seems to be affected by connective tissue diseases.


2011 ◽  
Vol 30 (2) ◽  
pp. 81-88 ◽  
Author(s):  
Jinping Zhang ◽  
Ben H. Lee ◽  
Chao Chen

AbstractNeonatal osteomyelitis is a rare and challenging diagnosis, particularly in the early onset period. Neonatal osteomyelitis is predominantly caused by Staphylococcus aureus with single bone involvement. Here, we report two cases of neonatal osteomyelitis in premature infants caused by Klebsiella pneumoniae with multiple bone lesions. Both cases presented with sepsis and meningitis and were initially diagnosed by incidental findings on plain films, with follow-up bone scan imaging. In both cases, diagnosis was timely and treatment was successful. These cases highlight the need to include neonatal osteomyelitis in the differential diagnosis when late-onset or prolonged neonatal sepsis is present, particularly because long-term outcome is dependent on rapid diagnosis and initiation of treatment.


2018 ◽  
Vol 33 (14) ◽  
pp. 901-908 ◽  
Author(s):  
Chengjun Wang ◽  
Meng Zhao ◽  
Jia Wang ◽  
Shuo Wang ◽  
Dong Zhang ◽  
...  

The purpose of this study is to investigate the surgical results and long-term outcomes of encephaloduroarteriosynangiosis for moyamoya disease in pediatric patients. We performed a retrospective analysis of 67 pediatric patients with moyamoya disease who underwent encephaloduroarteriosynangiosis in Beijing Tiantan Hospital. The case series included 36 boys and 31 girls. All the patients underwent surgical revascularization, and a total of 93 encephaloduroarteriosynangiosis procedures were performed (41 unilateral, 26 bilateral). The mean follow-up period after surgery was 30 months. During follow-up, ischemic stroke events were detected in 5 patients and the stroke rate for pediatric patients who underwent encephaloduroarteriosynangiosis procedure was 7.1% per patient-years. At the last follow-up, favorable outcomes (modified Rankin Scale score ≤ 2) were observed in 65 cases (97.0%). Our study suggests that long-term surgical outcome of encephaloduroarteriosynangiosis in pediatric moyamoya disease patients is satisfactory, and this technique has a positive impact on the prevention of stroke recurrence.


2012 ◽  
Vol 9 (6) ◽  
pp. 636-645 ◽  
Author(s):  
Päivi Koroknay-Pál ◽  
Hanna Lehto ◽  
Mika Niemelä ◽  
Riku Kivisaari ◽  
Juha Hernesniemi

Object Population-based data on pediatric patients with aneurysms are limited. The aim of this study is to clarify the characteristics and long-term outcomes of pediatric patients with aneurysms. Methods All pediatric patients (≤ 18 years old) with aneurysms among the 8996 aneurysm patients treated at the Department of Neurosurgery in Helsinki from 1937 to 2009 were followed from admission to the end of 2010. Results There were 114 pediatric patients with 130 total aneurysms during the study period. The mean patient age was 14.5 years (range 3 months to 18 years). The male:female ratio was 3:2. Eighty-nine patients (78%) presented with subarachnoid hemorrhage. The majority of the aneurysms (116 [89%]) were in the anterior circulation, and the most common location was the internal carotid artery bifurcation (36 [28%]). The average aneurysm diameter was 11 mm (range 2–55 mm) with 16 giant aneurysms (12%). Eighty aneurysms (62%) were treated microsurgically, and 37 (28%) were treated conservatively due to poor medical and neurological status of the patient or due to technical reasons during the early years of the patient series. No connective tissue disorders common to pediatric aneurysm patients were diagnosed in this series, with the exception of 1 patient with tuberous sclerosis complex. The mean follow-up duration was 24.8 years (range 0–55.8 years). At the end of follow-up, 71 patients (62%) had a good outcome, 3 (3%) were dependent, and 40 (35%) had died. Twenty-seven deaths (68%) were assessed to be aneurysm-related. Factors correlating with a favorable long-term outcome were good neurological condition of the patient on admission, aneurysm location in the anterior circulation, complete aneurysm closure, and absence of vasospasm. Six patients developed symptomatic de novo aneurysms after a median of 25 years (range 11–37 years). Fourteen patients (12%) had a family history of aneurysms. There was no increased incidence for cardiovascular diseases in long-term follow-up. Conclusions Most aneurysms were ruptured and of medium size. Internal carotid artery bifurcation was the most frequent location of the aneurysms. There was a male predominance of pediatric patients with aneurysms. Most patients experienced good recovery, with 91% of the long-term survivors living at home independently without assistance and meaningfully employed. Altogether, almost a third of these patients finished high school and one-fifth had a college or university degree. Pediatric patients had a tendency to develop de novo aneurysms.


2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Alessandra Leopizzi ◽  
Francesco Santoro ◽  
Adriana Mallardi ◽  
Enrica Vitale ◽  
Natale Daniele Brunetti

Abstract Aims Several studies have shown that Takotsubo syndrome (TTS) secondary to neurological disorders is associated with higher rate of in-hospital complications. Imaging brain studies found that atrophy or damage of some regions and their altered connectivity with other brain regions are typical features of TTS patients. The aim of the study is to evaluate rates of in hospital and long-term Follow-up events in TTS patients with history or acute cerebrovascular events (CVE). Methods and results 395 consecutive patients were enrolled in a multicentre prospective registry. History, clinical data, echocardiographic parameters and in-hospital and long-term follow-up events of all patients were evaluated. Prevalence of cerebrovascular events among TTS patients was 9.4% (num = 37). Compared to the control group, these patients were older (80 ± 7 vs. 71 ± 12, P = 0.01) and predominantly men (24% vs. 9%, P = 0.01). No differences in term of cardiovascular risk factors and left ventricular ejection fraction at admission and discharge were found among the two groups. The prevalence of physical stressor was higher in the CVE group (67% vs. 44% P = 0.01). The incidence of in-hospital adverse events was higher in the CVE than in the control one (70% vs. 29%), mainly driven by higher rate of Cardiogenic shock (19 vs. 8% P = 0.01) and in-hospital death (19 vs. 4%, P = 0.01). At long-term follow-up, patients in CVE group hat higher mortality rate (38% vs. 20% P = 0.01)). Patients presenting with acute CVE, 10 out of 37 patients (27%) when compared with chronic CVE had higher in-hospital mortality (40% vs. 11% P = 0.01). Conclusions TTS patients with history or acute cerebrovascular events had higher rate of in-hospital events and death at long-term follow-up. Acute cerebrovascular events in the setting of TTS are featured with worst prognosis.


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