scholarly journals Perioperative Considerations in Osteogenesis Imperfecta: A 20-Year Experience with the Use of Blood Pressure Cuffs, Arterial Lines, and Tourniquets

Children ◽  
2020 ◽  
Vol 7 (11) ◽  
pp. 214
Author(s):  
Kirsten E. Ross ◽  
Joseph T. Gibian ◽  
Christy J. Crockett ◽  
Jeffrey E. Martus

Osteogenesis imperfecta (OI) is a rare genetic connective-tissue disorder with bone fragility. To avoid iatrogenic fractures, healthcare providers have traditionally avoided using non-invasive blood pressure (NIBP) cuffs and extremity tourniquets in the OI population in the perioperative setting. Here, we hypothesize that these procedures do not lead to iatrogenic fractures or other complications in patients with OI. A retrospective study of all children with OI who underwent surgery at a single tertiary care children’s hospital from 1998 to 2018 was performed. Patient positioning and the use of NIBP cuffs, arterial lines, and extremity tourniquets were documented. Fractures and other complications were recorded. Forty-nine patients with a median age of 7.9 years (range: 0.2–17.7) were identified. These patients underwent 273 procedures, of which 229 were orthopaedic operations. A total of 246 (90.1%) procedures included the use of an NIBP cuff, 61 (22.3%) an extremity tourniquet, and 40 (14.7%) an arterial line. Pediatric patients with OI did not experience any iatrogenic fractures related to hemodynamic monitoring or extremity tourniquet use during the 20-year period of this study. Given the benefits of continuous intra-operative hemodynamic monitoring and extremity tourniquets, we recommend that NIBP cuffs, arterial lines, and tourniquets be selectively considered for use in children with OI.

2019 ◽  
Vol 39 (1) ◽  
pp. e68-e70 ◽  
Author(s):  
Brian T. Sullivan ◽  
Adam Margalit ◽  
Vaibhav S. Garg ◽  
Dolores B. Njoku ◽  
Paul D. Sponseller

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
D Vondrakova ◽  
D V Vondrakova ◽  
A K Kruger ◽  
M J Janotka ◽  
P N Neuzil ◽  
...  

Abstract Introduction Continuous reliable evaluating of left ventricular (LV) contractile function in patients with advanced heart failure requiring intensive care remains challenging. Recently, continual monitoring of dP/dtmax from arterial line became available for hemodynamic monitoring. However, the relation between arterial dP/dtmax and LV dP/dtmax measurement is not fully understood. Purpose The aim of our study was to determine the relation of arterial dP/dtmax and LV dP/dtmax assessed by echocardiography in patients with acute heart failure. Methods Forty-eight patients with acute heart failure requiring intensive care and hemodynamic monitoring were recruited into the study (mean age 70.4 years, 65% were males). Hemodynamic variables including arterial dP/dtmax were continually monitored using arterial line pressure waveform analysis. LV dP/dtmax was assessed using continuous-wave Doppler analysis of mitral regurgitation flow. Results The values from continual arterial dP/dtmax monitoring significantly correlated with the LV dP/dtmax assessed by echocardiography (r=0.72, 95% confidence interval [CI] 0.54–0.83, P<0.0001). Linear regression revealed that (LV dP/dtmax) = 0.87×(arterial dP/dtmax) + 291, P<0.0001. Arterial dP/dtmax significantly correlated also with the stroke volume (r=0.55, P<0.0001), cardiac output (r=0.32, P=0.0289), mean arterial blood pressure (r=0.43, P=0.0155) and systolic blood pressure (r=0.79, P<0001). On the other hand arterial dP/dtmax did not correlate with the systemic vascular resistance (SVR), heart rate, dynamic arterial elastance, diastolic blood pressure or central venous pressure. Conclusion Our results revealed that arterial dP/dtmax values tightly and highly significantly correlate with LV dP/dtmax. Arterial dP/dtmax could be, therefore, used for continual monitoring of LV contractility. Acknowledgement/Funding Institutional grant MH CZ - DRO (Na Homolce Hospital- NNH, 00023884), IG150501


Critical Care ◽  
2016 ◽  
Vol 20 (1) ◽  
Author(s):  
Rachel Joffe ◽  
Jonathan Duff ◽  
Gonzalo Garcia Guerra ◽  
Jodie Pugh ◽  
Ari R. Joffe

2018 ◽  
Author(s):  
Karen M. Kruger ◽  
Angela Caudill ◽  
Mercedes Rodriguez Celin ◽  
Sandesh CS Nagamani ◽  
Jay R Shapiro ◽  
...  

BackgroundOsteogenesis imperfecta (OI) is a genetic connective tissue disorder characterized by increased bone fragility and recurrent fractures. The phenotypic severity of OI has a significant influence on the ability to walk but little is known about the ambulatory characteristics, strength, or functional abilities in individuals with OI, especially in the more severe forms. To advance clinical research in OI, the Linked Clinical Research Centers, network of clinical centers in North America with significant experience in treating patients with OI, was established in 2009. The purpose of this work was to characterize mobility in OI using standard clinical assessment tools. and determine if any patient characteristics could be used to predict mobility outcomes.MethodsData were collected at five clinical sites and included age, gender, ethnicity, height, weight, use of assistive device, and bisphosphonate use and mobility metrics (age at first walk, Gillette Functional Assessment Questionnaire, Functional Mobility Scale, and distance walked in the 6 minute walk test). Linear mixed models were developed to explore the relationships between subject demographics and mobility metrics.ResultsThe study identified 491 individuals age 3 and older. In general, the results showed minor limitations in the type I group while the more severe types showed more significant limitations in all mobility metrics analyzed. Height and weight were shown to be the most significant predictors of mobility metrics. Relationships with mobility and bisphosphonates varied with OI type and whether oral or IV was used.ConclusionThis paper is the most comprehensive report of mobility in individuals with OI to date. These results are vital to understanding the mobility limitations of specific types of OI and beneficial when developing rehabilitation protocols for this population. It is important for physicians, patients, and caregivers to gain insight into severity and classification of the disease and the influence of disease-related characteristics on the prognosis for mobility.


2021 ◽  
Vol 8 (30) ◽  
pp. 2790-2796
Author(s):  
Utpal Dutta ◽  
Mahin Aboobacker B ◽  
Rituparna Bora

BACKGROUND Blood pressure monitoring in intraoperative period is one of the basic parameters in haemodynamic monitoring. This guides an anaesthesiologist to maintain an optimal condition between the patient’s stress condition and the depth of anaesthesia. Blood pressure can be measured either invasively or non-invasively in patients undergoing surgery. Invasive measurement from an arterial line is the preferred method even though there may be calibration errors, movement artefacts and over or under damping. The gold standard in cases of high-risk prolonged surgery is continuous monitoring of blood pressure by means of invasive blood pressure (IBP) measurement. The purpose of this study was to compare the non-invasive and invasive blood pressure monitoring in patients undergoing high risk surgeries METHODS After getting institutional ethical committee (H) clearance, study was conducted on 40 patients undergoing high-risk prolonged surgeries. Non-invasive blood pressures (NIBP) were recorded by oscillometric method before intubation, after intubation, half an hour after intubation and one hour after intubation. Simultaneously invasive blood pressure monitoring was done by establishing radial artery line. Bland-Altman plot was used to compare the blood pressure measurement by the non-invasive and invasive method. RESULTS Systolic blood pressure readings using non-invasive blood pressure monitoring was overestimated, diastolic and mean arterial pressure (MAP) readings using noninvasive blood pressure monitoring was underestimated in all instances. In all time points non-invasive blood pressure showed a good correlation with invasive blood pressure. CONCLUSIONS The study revealed that there is statistically significant difference between noninvasive and invasive blood pressure readings. We recommend invasive blood pressure monitoring in high-risk surgeries and critically ill patients, although a large-scale study is required to arrive at a conclusion. KEYWORDS Non-invasive Blood Pressure, Invasive Blood Pressure, Bland-Altman Analysis, High Risk Surgery


2018 ◽  
Vol 22 (4) ◽  
pp. 348-351
Author(s):  
Winson S. Ho ◽  
John A. Jane

Osteogenesis imperfecta (OI) is an inherited connective tissue disorder that causes bone fragility and deformity. Neurological manifestations, including macrocephaly and hydrocephalus, have been reported. Increased vascular fragility or bleeding diathesis also predisposes OI patients to intracranial hemorrhage. The development of chronic subdural fluid collections or hydrocephalus may require CSF diversion. The authors report a previously unrecognized complication of CSF diversion in a patient with OI, that is, a delayed severe cranial deformity, presumably due to over-shunting. In addition to the cosmetic concern, the deformity caused severe headaches and tenderness. The patient underwent craniectomy and titanium mesh cranioplasty, which resulted in the complete resolution of symptoms. This report raises the possibility that over-shunting in patients with OI could predispose to the formation of cranial deformity requiring surgical intervention.


2021 ◽  
Vol 22 (9) ◽  
pp. 4963
Author(s):  
Victoria L. Gremminger ◽  
Charlotte L. Phillips

Bone and muscle are highly synergistic tissues that communicate extensively via mechanotransduction and biochemical signaling. Osteogenesis imperfecta (OI) is a heritable connective tissue disorder of severe bone fragility and recently recognized skeletal muscle weakness. The presence of impaired bone and muscle in OI leads to a continuous cycle of altered muscle–bone crosstalk with weak muscles further compromising bone and vice versa. Currently, there is no cure for OI and understanding the pathogenesis of the skeletal muscle weakness in relation to the bone pathogenesis of OI in light of the critical role of muscle–bone crosstalk is essential to developing and identifying novel therapeutic targets and strategies for OI. This review will highlight how impaired skeletal muscle function contributes to the pathophysiology of OI and how this phenomenon further perpetuates bone fragility.


2018 ◽  
Vol 27 (6) ◽  
pp. 368-375 ◽  
Author(s):  
Anneli Talvik ◽  
Paola Rebora ◽  
Marika Heinpalu-Kuum ◽  
Sabrina Salerno ◽  
Eliza Miszkowska-Nagórna ◽  
...  

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