Moyamoya syndrome and PHACE syndrome: clinical and radiographic characterization of the intracranial arteriopathy and response to surgical revascularization

2019 ◽  
Vol 23 (4) ◽  
pp. 493-497 ◽  
Author(s):  
Sarah Jernigan ◽  
Armide Storey ◽  
Christine Hammer ◽  
Coleman Riordan ◽  
Darren B. Orbach ◽  
...  

OBJECTIVEPHACE syndrome (PHACES) has been linked to cervical and cerebral vascular anomalies, including persistent embryonic anastomoses and progressive steno-occlusive disease. However, no prior studies have documented the long-term response of PHACES patients with moyamoya disease to surgical revascularization with pial or myosynangiosis. The authors present their experience with 8 consecutive patients with PHACES and moyamoya disease.METHODSRetrospective review of patients who underwent pial synangiosis revascularization for moyamoya disease with concurrent diagnosis of PHACES.RESULTSA total of 8 patients out of 456 surgically treated moyamoya patients had a diagnosis of PHACES. All patients were female, and their average age at the time of surgical treatment was 9.3 years (range 1.8–25.8 years). Five patients had associated basilar artery anomalies or stenosis. All patients had symptomatic narrowing of the petrous segment of the internal carotid artery with tortuous collateralization. Three patients underwent unilateral pial or myo-synangiosis and 5 underwent bilateral procedures. The average hospital length of stay was 5.0 days (range 3–7 days). There were no postoperative complications. Follow-up ranged from 8 to 160 months (average 56 months). Seven of 8 patients have had follow-up angiograms and all had Matsushima grade A or B collateralization without progression of stenosis in other locations. All patients had reduced cortical FLAIR signal on 6-month follow-up MRI and no evidence of new radiographic or clinical strokes.CONCLUSIONSPatients with moyamoya disease and PHACES had an intracranial arteriopathy characterized by ectactic anterior vasculature with concomitant basilar artery stenosis, and were all female. The patients had both radiographic and clinical responses to pial synangiosis. The surgical treatment of these patients can be challenging given facial hemangiomas located near the surgical field. Patients with unilateral disease did not have evidence of progression in other cerebral circulation during the given follow-up period.

2015 ◽  
Vol 16 (3) ◽  
pp. 322-328 ◽  
Author(s):  
Hari T. Vigneswaran ◽  
Zachary J. Grabel ◽  
Craig P. Eberson ◽  
Mark A. Palumbo ◽  
Alan H. Daniels

OBJECT Adolescent idiopathic scoliosis (AIS) can cause substantial morbidity and may require surgical intervention. In this study, the authors aimed to evaluate US trends in operative AIS as well as patient comorbidities, operative approach, in-hospital complications, hospital length of stay (LOS), and hospital charges in the US for the period from 1997 to 2012. METHODS Patients with AIS (ICD-9-CM diagnosis codes 737.30) who had undergone spinal fusion (ICD-9-CM procedure codes 81.xx) from 1997 to 2012 were identified from the Kids' Inpatient Database. Parameters of interest included patient comorbidities, operative approach (posterior, anterior, or combined anteroposterior), in-hospital complications, hospital LOS, and hospital charges. RESULTS The authors identified 20, 346 patients in the age range of 0–21 years who had been admitted for AIS surgery in the defined study period. Posterior fusions composed 63.4% of procedures in 1997 and 94.1% in 2012 (r = 0.95, p < 0.01). The mean number of comorbidities among all fusion groups increased from 3.0 in 1997 to 4.2 in 2012 (r = 0.92, p = 0.01). The percentage of patients with complications increased from 15.6% in 1997 to 22.3% in 2012 (r = 0.78, p = 0.07). The average hospital LOS decreased from 6.5 days in 1997 to 5.6 days in 2012 (r = -0.86, p = 0.03). From 1997 to 2012, the mean hospital charges (adjusted to 2012 US dollars) for surgical treatment of AIS more than tripled from $55,495 in 1997 to $177,176 in 2012 (r = 0.99, p < 0.01). CONCLUSIONS Over the 15-year period considered in this study, there was an increasing trend toward using posterior-based techniques for AIS corrective surgery. The number of comorbid conditions per patient and thus the medical complexity of patients treated for AIS have increased. The mean charges for the treatment of AIS have increased, with a national bill over $1.1 billion per year in 2012.


Author(s):  
Raman Mitra ◽  
Eric Pagan ◽  
David Chang ◽  
James Gabriels ◽  
Amtul Mansoor ◽  
...  

Background: Coronavirus disease (COVID-19) has overwhelmed healthcare systems worldwide often at the cost of patients with serious non-COVID-19 conditions. Outcomes and risks of contracting COVID-19 in patients hospitalized during the pandemic are unknown. Objective: To report our experience in safely performing electrophysiology procedures during the COVID-19 pandemic. Methods: We examined non-COVID-19 patients who underwent electrophysiology procedures during the peak of the pandemic between March 16, 2020 and May 11, 2020 at seven Northwell Health hospitals. We developed a priority algorithm to stratify inpatients and outpatients requiring electrophysiology procedures and instituted a protocol to minimize hospital length of stay (LOS). All patients underwent post discharge 30-day tele-health follow-up and chart review up to 150 days. Results: A total of 217 patients underwent electrophysiology procedures, of which 86 (39%) patients were outpatients. A total of 108 (49.8%) patients had a LOS less than 24 hours, including 74 device implantations and generator changes, 24 cardioversions, five ablations, and one electrophysiology study. There were eleven (5.1%) procedure or arrhythmia related re-admissions and two (0.9%) minor procedural complications. Overall average hospital LOS was 83.4±165.1 hours and a median of 24.0 hours. For outpatient procedures, average hospital LOS was 9.4±13.4 hours and a median of 4.3 hours. Overall follow-up time was 83.9 ±42 days and a median of 84 days. During follow-up, two (0.9%) patients tested positive for COVID-19 and recovered uneventfully. No deaths occurred. Conclusion: During the peak of the COVID-19 pandemic, patients safely underwent essential electrophysiological procedures without increased incidence of acquiring COVID-19.


2020 ◽  
Vol 49 (4) ◽  
pp. 51-54
Author(s):  
O. Yu. Usenko ◽  
V. M. Kopchak ◽  
I. V. Khomiak ◽  
A. I. Khomiak ◽  
A. V. Malik

Introduction. Up to date, no consensus exists on the surgical treatment of paraduodenal pancreatitis (PDP). Most authors prefer to perform pancreaticoduodenectomy when surgical treatment is indicated. However, such an aggressive approach may not always be justified for the treatment of benign disease. The aim of our study was to investigate the results of duodenum-preserving pancreatic head resections (DPPHR) for the treatment of PDP. Materials and methods. We performed a retrospective analysis of a database consisting of 112 patients with PDP treated in Shalimov National Institute of Surgery and Transplantology from 2014 to 2019. A total of 45 patients after DPPHR were included to the study. Such modifications of DPPHR as Frey’s, Beger’s and Berne’s procedures were used. The primary study endpoint was pain control assessed according to the Izbicki pain score before surgery and at follow-up visits. Secondary endpoints were defined as complication rate (Clavien — Dindo >2), hospital length of stay and 90-day mortality. All patients were followed-up for the assessment of pain cessation with a median of 33 months (range 8–54 months). Results. There were 42 males (93.3%) and 3 females (6.7%) in the study group. Preoperative Izbicki pain score result was 52.6 points. Follow-up pain score results were significantly lower at 11.7 points. Postoperative complication rate (Clavien — Dindo >2) was measured at 8.9%. Median hospital length of stay was 17.4 days. No mortality was recorded in the study group. All results were statistically significant (p<0.05). Conclusion. Application of DPPHR for the surgical treatment of PDP allows to achieve excellent results in terms of pain control (52.6 and 11.7 points on the Izbicki pain score before surgical intervention and at follow-up), while maintaining low complication (8.9%) and mortality (0%) rates.


2019 ◽  
Vol 80 (03) ◽  
pp. 143-148
Author(s):  
George Williams ◽  
William Jones ◽  
Rabail Chaudhry ◽  
Chunyan Cai ◽  
Greesha Pednekar ◽  
...  

Background Moyamoya disease (MMD) is a rare cerebrovascular condition, often presenting as a headache or stroke in adults. Anesthetic management of this illness may challenge providers because it can affect the long-term neurologic outcome and hospital length of stay (LOS) in patients with MMD. Materials and Methods A literature search was conducted to assess etiology and epidemiology, as well as existing reports of intraoperative management of MMD. Due to sparse findings, the search was expanded to include studies of the use of intraoperative anesthetic agents during other neurosurgical procedures. We also retrospectively reviewed all MMD cases from January 1, 2009, to December 31, 2015, at Memorial Hermann Hospital-Texas Medical Center, where intraoperative management involved craniotomy and surgical revascularization. Data were collected primarily on the use of several anesthetic agents. The LOS and any adverse events were also recorded for each case. The data were divided into two equivalent case cohorts: (1) January 1, 2009, to February 18, 2013, and (2) February 19, 2013, to December 31, 2015. Results Remifentanil use notably increased between the first and second time periods while fentanyl use decreased. Desflurane usage also demonstrated an observed increase when our two cohorts were compared. Additionally, there was a decrease in the mean LOS between the first and second periods of 3.9 and 3.3 days, respectively. Conclusion Increasing use of remifentanil in MMD cases could be attributed to its ability to provide more stable hemodynamics during induction, maintenance, and emergence of anesthesia when compared with fentanyl. Lower systolic pressures, diastolic pressures, and heart rates were reported in patients receiving remifentanil over fentanyl.


2002 ◽  
Vol 9 (3) ◽  
pp. 295-298 ◽  
Author(s):  
John W. York ◽  
W. Charles Sternbergh ◽  
Michael R. Lepore ◽  
Moises Yoselevitz ◽  
Samuel R. Money

Purpose: To show that AneuRx aortic cuffs might be used in a “stacked” configuration to effectively treat saccular abdominal aortic aneurysm (AAA). Methods: In a recent 1-year period, 147 patients underwent endovascular AAA repair. Of these, 5 (4 men; mean age 61.6 years, range 55–69) had saccular AAAs with a mean diameter of 3.7 ± 0.2 cm (range 3.0–4.7). AneuRx aortic cuff prostheses (3.75-cm length) were deployed sequentially in these 5 patients via a right femoral approach; the devices were overlapped ∼1.5 to 2.0 cm until complete exclusion of the aneurysm was achieved. Endograft surveillance was performed using computed tomography at 4 weeks postoperatively and then every 6 months. Results: Successful exclusion of the saccular infrarenal aortic aneurysms was achieved in all 5 patients using 2 or 3 “stacked” stent-graft extensions. Four of the 5 procedures were performed under spinal anesthesia; the average procedural time was 96 ± 41 minutes. The average hospital length of stay was 1.6 days; no major morbidity or mortality was encountered. There were no early or late endoleaks, aneurysm expansion, or device migration over a follow-up that ranged to 12 months. Conclusions: Saccular AAA provides ideal anatomy for endovascular repair with a “tube” endograft. “Stacked” aortic cuffs create a customized stent-graft that is not otherwise commercially available.


2021 ◽  
pp. 107110072110175
Author(s):  
Jordan R. Pollock ◽  
Matt K. Doan ◽  
M. Lane Moore ◽  
Jeffrey D. Hassebrock ◽  
Justin L. Makovicka ◽  
...  

Background: While anemia has been associated with poor surgical outcomes in total knee arthroplasty and total hip arthroplasty, the effects of anemia on total ankle arthroplasty remain unknown. This study examines how preoperative anemia affects postoperative outcomes in total ankle arthroplasty. Methods: A retrospective analysis was performed using the American College of Surgeons National Surgery Quality Improvement Project database from 2011 to 2018 for total ankle arthroplasty procedures. Hematocrit (HCT) levels were used to determine preoperative anemia. Results: Of the 1028 patients included in this study, 114 patients were found to be anemic. Univariate analysis demonstrated anemia was significantly associated with an increased average hospital length of stay (2.2 vs 1.8 days, P < .008), increased rate of 30-day readmission (3.5% vs 1.1%, P = .036), increased 30-day reoperation (2.6% vs 0.4%, P = .007), extended length of stay (64% vs 49.9%, P = .004), wound complication (1.75% vs 0.11%, P = .002), and surgical site infection (2.6% vs 0.6%, P = .017). Multivariate logistic regression analysis found anemia to be significantly associated with extended hospital length of stay (odds ratio [OR], 1.62; 95% CI, 1.07-2.45; P = .023) and increased reoperation rates (OR, 5.47; 95% CI, 1.15-26.00; P = .033). Anemia was not found to be a predictor of increased readmission rates (OR, 3.13; 95% CI, 0.93-10.56; P = .066) or postoperative complications (OR, 1.27; 95% CI, 0.35-4.56; P = .71). Conclusion: This study found increasing severity of anemia to be associated with extended hospital length of stay and increased reoperation rates. Providers and patients should be aware of the increased risks of total ankle arthroplasty with preoperative anemia. Level of Evidence: Level III, retrospective comparative study.


2021 ◽  
Vol 108 (Supplement_2) ◽  
Author(s):  
K Daga ◽  
M T Ahmad ◽  
M Taneja

Abstract The current literature regarding the morphology and presentations of strokes due to basilar artery stenosis/occlusion is limited. This pathology is a rare cause of stroke and its management is not clearly decided in guidelines or published literature. Moreover, posterior circulation strokes are reported to be more devastating than anterior circulation. We present a case of a 68-year-old male who presented in an acute setting with reduced consciousness, myoclonic jerks, weakness and nausea due to a stroke from an occluded mid-segment basilar artery. MRI showed evidence of left cerebellar and right occipital infarcts. Emergent cerebral angiography was performed, and he was immediately treated by thrombectomy and stenting (Stryker Wingspan stent). The patient made a full recovery within one week and remained well at follow-up 3 months post-procedure. Emergency recanalization of basilar artery strokes, by clot retrieval and stenting, should be considered as a treatment option.


2021 ◽  
pp. 1-7
Author(s):  
Carlos Castillo-Pinto ◽  
Jessica L. Carpenter ◽  
Mary T. Donofrio ◽  
Anqing Zhang ◽  
Gil Wernovsky ◽  
...  

Abstract Objective: Children with CHD may be at increased risk for epilepsy. While the incidence of perioperative seizures after surgical repair of CHD has been well-described, the incidence of epilepsy is less well-defined. We aim to determine the incidence and predictors of epilepsy in patients with CHD. Methods: Retrospective cohort study of patients with CHD who underwent cardiopulmonary bypass at <2 years of age between January, 2012 and December, 2013 and had at least 2 years of follow-up. Clinical variables were extracted from a cardiac surgery database and hospital records. Seizures were defined as acute if they occurred within 7 days after an inciting event. Epilepsy was defined based on the International League Against Epilepsy criteria. Results: Two-hundred and twenty-one patients were identified, 157 of whom were included in our analysis. Five patients (3.2%) developed epilepsy. Acute seizures occurred in 12 (7.7%) patients, only one of whom developed epilepsy. Predictors of epilepsy included an earlier gestational age, a lower birth weight, a greater number of cardiac surgeries, a need for extracorporeal membrane oxygenation or a left ventricular assist device, arterial ischaemic stroke, and a longer hospital length of stay. Conclusions: Epilepsy in children with CHD is rare. The mechanism of epileptogenesis in these patients may be the result of a complex interaction of patient-specific factors, some of which may be present even before surgery. Larger long-term follow-up studies are needed to identify risk factors associated with epilepsy in these patients.


Stroke ◽  
2013 ◽  
Vol 44 (suppl_1) ◽  
Author(s):  
Satoshi Kuroda ◽  
Naoki Akioka ◽  
Daina Kashiwazaki ◽  
Hideo Hamada ◽  
Naoya Kuwayama ◽  
...  

Introduction —It is well known that surgical revascularization can improve cerebral hemodynamics and prevent further ischemic cerebrovascular events in moyamoya disease. However, a certain subgroup of patients repeats ischemic attacks even after surgery because of insufficient surgery or disease progression during follow-up periods. Hypothesis —Relevant designs and techniques in additional bypass surgery can resolve ischemic cerebrovascular events in patients with moyamoya disease refractory to previous bypass surgery. Methods —This study included totally 7 patients (9 hemispheres) with moyamoya disease refractory to previous bypass surgery. There were 5 children and 2 adults. They underwent previous bypass surgery in Japan and Europe 6 to 240 months before admission. Based on precise clinical and radiological analysis, cerebrovascular events were considered to occur because of insufficient bypass surgery in 5 patients and disease progression in the ipsilateral posterior cerebral artery in 2. Surgical strategies included wide craniotomy to cover the area where cerebral hemodynamics is still impaired and appropriate bypass procedures such as STA-MCA anastomosis, OA-PCA anastomosis, and indirect bypass. Using [123]I-IMP SPECT or [15]O-gas PET, cerebral hemodynamics was precisely examined before and after surgery Results —Postoperative course was uneventful and cerebral hemodynamics significantly improved in all 7 patients. Postoperative cerebral angiography revealed that additional bypass provided collateral blood flow to ischemic area before surgery. Ischemic cerebrovascular events rapidly resolved in 5 patients and gradually decreased in 2. Conclusion —This study strongly suggests adequate surgical design and procedures can resolve ischemic cerebrovascular events in patients with moyamoya disease refractory to previous bypass surgery.


Author(s):  
Antonio Tarasconi ◽  
Fausto Catena ◽  
Hariscine K. Abongwa ◽  
Belinda De Simone ◽  
Federico Coccolini ◽  
...  

Unlike other surgical fields, such as cardiac surgery, where many trials have been made about safety, feasibility and outcome of surgical procedures in the elderly, there is lack of literature about emergency abdominal surgery in very old patients, especially in people over 90 years of age. The available data reported survival of about 50% one year after the operation. The aim of the study is to determine the survival rate two years after emergency abdominal surgery in a nonagenarian population and to identify any demographic and surgical parameters that could predict a poor outcome in this type of patient. The study was a retrospective multicenter trial. Patient inclusion criteria were: age 90 years old or older, urgent abdominal surgery. The medical charts reviewed and data collected were: gender, age, the American Society of Anesthesiologists (ASA) score and comorbidities, diagnosis, time elapsed between arrival to the Emergency Room and admission to the Operatory Room, surgical procedures, open versus laparoscopic procedure, type of anesthesia and outcomes with hospital length of stay. Phone call follow-up was performed for patient discharged alive and Kaplan-Meier analysis was used to evaluate survival. We identified 72 (20 males and 52 females) nonagenarian patients who underwent abdominal emergency surgery at 6 Italian hospitals (Parma, Bergamo, Bologna, Brescia, Chiari, Adria). Mean age was 92.5 years [range 90-100, standard deviation (SD) 2.6], median ASA score was 3 (range 2-5, mean 3.32) and only 7 patients were without comorbidities. Mean hospital length of stay was 13 days (range 1-60, SD 11.52); 56 patients (77.7%) were discharged alive; 2 years survival rate was 23% [mean follow-up=10 months (range 1-27)]. Among all the parameters analyzed, only ASA score was significantly correlated with survival. Neither the presence of malignancy nor the absence of comorbidities seems to correlate with survival. Nonagenarian patients undergoing emergent abdominal surgical procedures have a high overall in-hospital mortality rate (23%) and a low 2 years survival rate (51.4%). Except for ASA score, there are no other factors predicting poor outcome. Based on the present study emergency abdominal surgery in frail patients over 90 years of age has to be carefully evaluated: only 1 out 5 patients will be alive after 2 years.


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