scholarly journals Characterization of Clinical Outcomes in Patients with Cutis Marmorata Telangiectatica Congenita

2021 ◽  
Vol 5 (4) ◽  
pp. 399-402
Author(s):  
Nate Bowers ◽  
Wasim Haidari ◽  
Jennifer Su ◽  
Jesus Cardenas-de la Garza ◽  
Steve Feldman ◽  
...  

Cutis marmorata telangiectatica congenita (CMTC) is an uncommon, congenital, cutaneous vascular disease with an unknown pathogenesis. Although considered as a benign condition, anomalies such as body asymmetry are frequently associated. Herein we present a series of patient with diagnosis of CMTC with a focus on clinical outcomes. In our series, limb length abnormalities were the most common associate anomaly, occurring in 24% of the subjects, similar to other series with rates of 33% and 27% but higher than the general population (6.7%). Importantly, dermatologist should be aware of the frequently associated anomalies in CMTC, such as leg length discrepancy, which may have serious consequences if not recognized and treated.    

2019 ◽  
Vol 14 (1) ◽  
Author(s):  
Teresa Nu Phuong Trinh Bui ◽  
Ayse Corap ◽  
Anette Bygum

Abstract Background Cutis marmorata telangiectatica congenita (CMTC) is a rare capillary malformation characterised by persistent reticulated marbled erythema. It tends to be associated with cutaneous atrophy, ulcerations and body asymmetry. CMTC is usually reported to be a benign condition; however, associated anomalies are not rare. Here, we have compiled information on published CMTC patients with the aim to evaluate the proposed diagnostic criteria by Kienast et al. and address the clinical manifestations, associated anomalies, differential diagnoses, management and prognosis. Our review is based on a search of the PubMed database which retrieved studies between 1922 and April 2019. The search yielded 148 original articles with a total of 485 patients. Results Of the identified patients, 24.5% had generalised CMTC, 66.8% had localised and 8.7% had a non-specified distribution of CMTC. Associated anomalies were observed in 42.5% of patients, predominantly body asymmetry and neurological defects like seizure and developmental delay. Fewer patients (10.1%) had ophthalmological defects, usually glaucoma. The major criterium “absence of venectasia” was not met in 20.4% of patients. Conclusion We suggest that children with CMTC should be referred to an ophthalmologist for regular follow-up, and children with CMTC affecting the legs should be monitored for leg length discrepancy throughout the growth period. Furthermore, we suggest reconsideration of the major criterium “absence of venectasia” from the proposed diagnostic criteria, and instead include body asymmetry.


Author(s):  
Han-Jun Lee ◽  
Seong Hwan Kim ◽  
Nicolas Pujol ◽  
Yong-Beom Park

AbstractThe purpose of this study is to compare perception of leg length discrepancy (LLD) and clinical results of total knee arthroplasties (TKA) in patients with or without previous ipsilateral hip arthroplasty. Between 2008 and 2015, navigation-assisted TKA was performed in 43 patients with previous hip arthroplasty after hip fracture. After 1:3 propensity score matching was performed, 108 patients of primary navigation-assisted TKA (group 1) and 36 patients with hip arthroplasty (group 2) were included. Knee Society (KS) scores, Western Ontario and McMaster Universities Index (WOMAC) scores, and patients' satisfaction including perception of LLD were evaluated. Radiographic evaluation included mechanical axis, component position, and LLD. Logistic regression analysis was performed to find the factors that affect the clinical outcomes. No significant differences in radiologic and clinical evaluations, except for KS function score, patient's satisfaction and LLD (p< 0.001), were detected between the groups. LLD and its perception were significantly higher in group 2 (1.8 ± 3.4 mm in group 1 and 9.7 ± 4.1 mm in group 2, p = 0.000). Risk factors for the low KS function score were found as LLD (odds ratio [OR]: 1.403, p = 0.008) and previous hip arthroplasty itself (OR: 15.755, p = 0.002), but much higher OR was found in previous hip arthroplasty. Although the outcomes of TKA in patients with ipsilateral hip arthroplasty are comparable to those of primary TKA, LLD was high and patient's satisfaction and functional outcomes were low in patients with previous ipsilateral hip arthroplasty. Care should be taken when considering TKA in patients with previous hip arthroplasty. This is a Level III, case control study.


2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Hidetatsu Tanaka ◽  
Norikazu Yamada ◽  
Hiroaki Kurishima ◽  
Yu Mori ◽  
Takashi Sakamoto ◽  
...  

Abstract Background The anterolateral muscle-sparing total hip arthroplasty (THA) in the supine position is advantageous owing to the very low-dislocation rate and excellent leg length discrepancy control. However, femur exposure is challenging. Although the conjoined external rotators tendon (CERT) release is effective in improving femoral access, the effects on clinical outcomes remain unclear. The purpose of this study was to evaluate the clinical and radiographic results of CERT release in the anterolateral muscle-sparing THA approach. Methods The study was performed as a retrospective cohort study and included 85 hips in 85 patients who underwent primary anterolateral THA. Clinical and radiographic outcomes were investigated 6 months and 1 year after THA (CERT-preserved and non-released patients). The Japanese Orthopaedic Association (JOA) hip score, JOA Hip-disease Evaluation Questionnaire (JHEQ), forgotten joint score (FJS), and the 36 short-form questionnaires (SF-36 mental and physical) were evaluated. The leg length discrepancy, cup inclination and stem orientation were evaluated with radiographs. Results Among all the included hips, 37 patients (43.5%) retained the CERT, and 48 patients (56.5%) included the released CERT. There were no significant differences in the JOA hip scores, JHEQ, FJF-12 and SF-36 between the released and non-released groups. There were significant differences in sagittal stem alignments between groups. Conclusion The CERT release in anterolateral muscle-sparing THA has a limited effect on post-operative clinical outcomes. The CERT release improved the femur exposure and is more invasive than the preserved CERT. We infer that the CERT should be maintained in patients with a wide range of motions, and release the CERT in inadequate femur canal preparation cases.


2019 ◽  
Vol 30 (5) ◽  
pp. 536-543
Author(s):  
Nicolas Reina ◽  
Jonathan Hourtal ◽  
Christopher G Salib ◽  
Gauthier Gracia ◽  
Etienne Cavaignac ◽  
...  

Background: Leg-length discrepancy (LLD) is a frequent and recurring issue after total hip arthroplasty (THA). It can lead to discomfort and litigation due to the patient’s perception of this inequality. Purpose: The aim of this study was to evaluate the functional impact of postoperative LLD and length correction in patients after primary THA. We hypothesised that clinical outcomes are inversely correlated to length correction and residual discrepancy. Methods: In a prospective cohort, we included 121 patients undergoing unilateral primary THA. Patients were categorised into 3 groups based on their residual LLD using routine EOS imaging; Shorter (<-5 mm); Equal (-5 mm to +5 mm); and Longer (>+5 mm). Delta of Correction (DC) was defined as the difference between pre- and postoperative discrepancy measured on EOS imaging. Functional scores (HHS, Postel Merle d’Aubigné, and WOMAC) were measured preoperatively and at last follow-up. The average follow-up was 24 months. We evaluated the clinical and radiographic outcomes regarding the residual LLD and DC. Results: All patients had improvement in their functional scores at last follow-up. There was no difference in clinical outcomes, regardless of preoperative or residual LLD. The highest scores were found in the population with the smallest variations (DC -5 to +5 mm, HHS 96). Conversely, poorer results were found in shortened patients (DC <-5 mm), HHS 86, and lengthened patients, corrected by > 15 mm, with HHS of 83, p < 0.005. Patient perception of postoperative LLD correlated with the DC ( p = 0.004). Conclusion: The DC measurement is a more effective assessment than arthroplasty.


2021 ◽  
Author(s):  
hidetatsu tanaka ◽  
Norikazu Yamada ◽  
Hiroaki Kurishima ◽  
Yu Mori ◽  
Takashi Sakamoto ◽  
...  

Abstract Background: The anterolateral muscle-sparing total hip arthroplasty (THA) in the supine position is advantageous owing to the very low-dislocation rate and excellent leg length discrepancy control. However, femur exposure is challenging. Although the conjoined external rotators tendon (CERT) release is effective in improving femoral access, the effects on clinical outcomes remain unclear. The purpose of this study was to evaluate the clinical and radiographic results of CERT release in the anterolateral muscle-sparing THA approach.Methods: The study was performed as a retrospective cohort study and included 85 hips in 85 patients who underwent primary anterolateral THA. Clinical and radiographic outcomes were investigated 6 months and 1 year after THA (CERT-preserved and non-released patients). The Japanese Orthopaedic Association (JOA) hip score, JOA Hip-disease Evaluation Questionnaire (JHEQ), forgotten joint score (FJS) and the 36 short-form questionnaires (SF-36 mental and physical) were evaluated. The leg length discrepancy, cup inclination and stem orientation were evaluated with radiographs.Results: Among all the included hips, 37 patients (43.5%) retained the CERT, and 48 patients (56.5%) included the released CERT. There were no significant differences in the JOA hip scores, JHEQ, FJF-12 and SF-36 between the released and non-released groups. There were significant differences in sagittal stem alignments between groups.Conclusion: The CERT release in anterolateral muscle-sparing THA has a limited effect on post-operative clinical outcomes. The CERT release improved the femur exposure and is more invasive than the preserved CERT. We infer that the CERT should be maintained in patients with wide range of motions, and release the CERT in inadequate femur canal preparation cases.


Author(s):  
Kentaro Iwakiri ◽  
Yoichi Ohta ◽  
Takashi Fujii ◽  
Yukihide Minoda ◽  
Akio Kobayashi ◽  
...  

2021 ◽  
Vol 7 (7) ◽  
pp. eabe6855 ◽  
Author(s):  
Carolina Beltrán-Pavez ◽  
Sebastián Riquelme-Barrios ◽  
Aarón Oyarzún-Arrau ◽  
Aracelly Gaete-Argel ◽  
Roxana González-Stegmaier ◽  
...  

Chile has one of the worst numbers worldwide in terms of SARS-CoV-2 positive cases and COVID-19–related deaths per million inhabitants; thus, characterization of neutralizing antibody (NAb) responses in the general population is critical to understanding of immunity at the local level. Given our inability to perform massive classical neutralization assays due to the scarce availability of BSL-3 facilities in the country, we developed and fully characterized an HIV-based SARS-CoV-2 pseudotype, which was used in a 96-well plate format to investigate NAb responses in samples from individuals exposed to SARS-CoV-2 or treated with convalescent plasma. We also identified samples with decreased or enhanced neutralization activity against the D614G spike variant compared with the wild type, indicating the relevance of this variant in host immunity. The data presented here represent the first insights into NAb responses in individuals from Chile, serving as a guide for future studies in the country.


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