scholarly journals Short-term comparison of the use of static and expandable intramedullary rods in the lower limbs of children with osteogenesis imperfecta

2021 ◽  
Vol 20 (1) ◽  
Author(s):  
Louis J de Jager ◽  
Pieter Η Mare ◽  
David Μ Thompson ◽  
Leonard C Marais

ABSTRACT BACKGROUND: Children with osteogenesis imperfecta suffer from frequent fractures and deformities due to skeletal fragility. Stabilisation of fractures, correction of deformity and intramedullary rodding result in decreased pain and improved function. Modern expandable intramedullary rods aim to provide lasting stability during growth, without an increase in complications. The aim of our study was to determine and compare the outcome of static Rush rods and expandable Fassier-Duval rods in terms of complications and reoperation rate. METHODS: We reviewed the records of a cohort of 17 children (seven female) with osteogenesis imperfecta (11 Sillence type III, six Sillence type IV) who were treated with intramedullary rods in the lower limb between 2011 and 2017. They had 64 rodding (38 femoral and 26 tibial) procedures (26 Rush rods and 38 Fassier-Duval rods). These were a primary procedure in 46, and a revision procedure after previous Rush rodding in 18 cases. RESULTS: The overall complication rate was 66% (n=42). There was a higher complication rate in the Rush rod group (81%, n=21) when compared to the Fassier-Duval group (55%, n=21)(p=0.035). The most frequent complication in the Rush rod group was distal deformity as the rod is outgrown (69%, n=18). The most frequent complication in the Fassier-Duval rod group was intramedullary migration due to a failure to expand (45%, n=17). Factors that were associated with increased risk of complications included younger age (p=0.031), type of rod (p=0.035), and deformity as an indication for surgery (77% complications, p=0.033). At a mean follow-up of 3.1 years, the reoperation rate in the Rush rod group was 58% (n=15). Comparatively, at a mean of 3.7 years follow-up, there were no reoperations in the Fassier-Duval group. CONCLUSIONS: Despite the numerous innovations, the surgical management of lower limb deformities and fractures in children with osteogenesis imperfecta remains challenging with a relatively high complication rate. The use of Fassier-Duval rods may result in a lower reoperation rate when compared to Rush rods, in the short term. Level of evidence: Level 4 Keywords: osteogenesis imperfecta, intramedullary rod, Rush rod, Fassier-Duval rod, fracture, deformity

2014 ◽  
Vol 24 (1) ◽  
pp. 48-53 ◽  
Author(s):  
Alejandra Martínez ◽  
Cristophe Pomel ◽  
Thomas Filleron ◽  
Marjolein De Cuypere ◽  
Eliane Mery ◽  
...  

ObjectiveThe aim of the study was to report on the oncologic outcome of the disease spread to celiac lymph nodes (CLNs) in advanced-stage ovarian cancer patients.MethodsAll patients who had CLN resection as part of their cytoreductive surgery for epithelial ovarian, fallopian, or primary peritoneal cancer were identified. Patient demographic data with particular emphasis on operative records to detail the extent and distribution of the disease spread, lymphadenectomy procedures, pathologic data, and follow-up data were included.ResultsThe median follow-up was 26.3 months. The median overall survival values in the group with positive CLNs and in the group with negative CLNs were 26.9 months and 40.04 months, respectively. The median progression-free survival values in the group with metastatic CLNs and in the group with negative CLNs were 8.8 months and 20.24 months, respectively (P = 0.053). Positive CLNs were associated with progression during or within 6 months after the completion of chemotherapy (P = 0.0044). Tumor burden and extensive disease distribution were significantly associated with poor progression-free survival, short-term progression, and overall survival. In multivariate analysis, only the CLN status was independently associated with short-term progression.ConclusionsDisease in the CLN is a marker of disease severity, which is associated to a high-risk group of patients with presumed adverse tumor biology, increased risk of lymph node progression, and worst oncologic outcome.


2021 ◽  
Author(s):  
wenbin jiang ◽  
Shuyun Jiang ◽  
Yan Yu ◽  
Qijia Zhan ◽  
Min Wei ◽  
...  

Abstract Background Selective Dorsal Rhizotomy (SDR) guided by our modified protocol can decrease spasticity in certain muscles. This study aimed to investigate gait parameters changes in cerebral palsy (CP) with focal spasticity after SDR in short-term follow-up. Methods CP classified as Gross Motor Function Classification System (GMFCS) level Ⅰ and Ⅱ who underwent SDR were included. Changes of spasticity, gait parameters and gait deviation index (GDI) were retrospectively reviewed. Results This study contained 26 individuals with 44 affected and 8 intact lower limbs (4 monoplegia, 4 hemiplegia and 18 diplegia). Mean age was 5.7 ± 1.9 years-old and follow-up duration was 9.9 ± 6.6 months. After SDR, average spasticity of 108 target muscles decreased from 2.9 ± 0.8 to 1.8 ± 0.6 in Modified Ashworth Scale (MAS). Kinematic curves changed after the surgery in sagittal and transverse plane in affected sides, further investigation showed improvements in ankle and knee. No changes were found in temporal-spatial parameters except decrease in cadence in affected sides. GDI improved significantly in affected limbs. Conclusion In short-term follow up, the new-protocol-guiding SDR can lower focal spasticity, GA showed improvements in kinematic parameters and GDI. Longer follow-up duration is needed to clarify the long-term outcome.


Circulation ◽  
2017 ◽  
Vol 135 (suppl_1) ◽  
Author(s):  
Sridharan Raghavan ◽  
Wenhui G Liu ◽  
P. Michael Ho ◽  
Mary E Plomondon ◽  
Anna E Baron ◽  
...  

Background: Diabetes is a significant risk factor for cardiovascular disease, but optimal glycemic control strategies remain unclear. In particular, trials of intensive glycemic control have highlighted a tension between increased mortality risk and macrovascular benefits. In this study we aimed to assess whether the burden of coronary artery disease (CAD) modifies the association between glycemic control and short-term mortality. Methods: We studied veterans with diabetes who underwent elective cardiac catheterization between 2005 and 2013 in a retrospective analysis of data from the VA Clinical Assessment, Reporting, and Tracking (CART) Program. Primary exposures were time-varying HbA1c over two years of follow-up after index catheterization, categorized as <6%, 6-6.49%, 6.5-6.99%, 7-7.99%, 8-8.99%, and >=9%, and burden of CAD, categorized as no CAD, non-obstructive CAD, or obstructive CAD. Primary outcome was two-year all-cause mortality. A total of 17394 participants had, on average, five HbA1c measurements over two years of follow-up. We used multivariable Cox proportional hazards regression to estimate the association between HbA1c and mortality, adjusting for demographic and clinical covariates and CAD burden, and including a term for interaction between HbA1c and CAD burden. Results: In adjusted models with 6.5 ≤ HbA1c ≤ 6.99% as the reference category, HbA1c < 6% was associated with increased risk of mortality (HR 1.55 [1.25, 1.92]), whereas HbA1c categories above 7% were not. We observed significant interaction between glycemic control and CAD burden (interaction p=0.0005); the increased risk of short-term mortality at HbA1c < 6% was limited to individuals with non-obstructive and obstructive CAD (Figure 1). Conclusions: HbA1c below 6% was associated with increased risk of short-term mortality, but only in individuals with CAD. CAD burden may thus inform individualized diabetes management strategies, specifically treatment de-escalation in individuals with any angiographically-defined CAD.


2020 ◽  
Author(s):  
Benjamin Fick ◽  
Daniel Stover ◽  
Ruth Chimenti ◽  
Mederic Hall

Abstract Background: Ultrasound guided tenotomy (USGT) is a minimally invasive treatment option for patients with chronic tendinopathy who fail to benefit from conservative exercise interventions. The complication rate and effectiveness of USGT remain poorly defined in the literature. Purpose: This study aimed to evaluate the risks associated with USGT and outcomes across upper extremity and lower extremity tendinopathy/fasciopathy sites. Methods Patients who had USGT at the elbow, patellar, or Achilles tendons or along the plantar fascia were identified by retrospective review of charts. Screening for complications (infection, tendon rupture, and hypersensitivity) and satisfaction with the procedure were assessed at routine short-term follow-up visits and at long-term follow-up via phone/email. Outcomes (pain, quality of life) were assessed using the region specific pain scales and the Short Form-12, respectively, at baseline prior to the procedure, short-term follow up, and long term follow up. Results: A total of 262 patients were identified through chart review. There was a low complication rate of 0.7% including one superficial wound infection and one case of wound hypersensitivity. Prior to USGT, the majority of patients reported moderate/daily pain that decreased by short-term and long-term follow-up to mild/occasional pain (p < 0.05). Additionally, most patients reported abnormally low physical function prior to USGT that was within normal range of physical function by long-term follow-up (p < 0.05). The majority of responders (63% at the plantar fascia to 92% at the Achilles midportion) reported being either ‘very satisfied’ or ‘somewhat satisfied’ with the procedure at short-term follow-up. Conclusions: This study found that USGT is a safe procedure with a low complication rate in a heterogeneous sample. Study findings provide preliminary evidence on the utility of USGT to reduce pain and improve function with a high rate of patient satisfaction.


2000 ◽  
Vol 15 (3-4) ◽  
pp. 144-148
Author(s):  
F. Mercier ◽  
F. Cormier ◽  
J. M. Fichelle ◽  
F. Duarte ◽  
J. M. Cormier

Aim: To review the investigation and treatment of iliac vein obstruction. Method: A review of current literature in the field of management of iliac venous obstruction has been conducted. Synthesis: Iliac venous obstruction results in chronic or acute symptoms in the lower limb presenting as pain, swelling, oedema and discomfort of the lower limb. Intrinsic or extrinsic obstruction of the iliac veins may be the cause. Cockett syndrome is the classic aetiology for chronic intermittent or fixed left inferior limb venous obstruction. Other causes include tumours, vascular grafts or lymph node compression and retroperitoneal fibrosis. Duplex ultrasound imaging is now the first-choice investigation. CT scanning is useful where external vein compression is suspected. Phlebography is used when an endovascular procedure is to be done. The surgical treatment of Cockett syndrome described by Cormier is transposition of the common right iliac artery in the left internal iliac artery. This is being replaced by endovascular balloon venoplasty completed by stenting of the left iliac vein. We reviewed the experience of surgical correction of Cockett syndrome with Cormier's technique in 70 patients operated on between 1976 and 1990; 55 patients had a follow-up of 12-177 months. Anatomical and functional results were perfect for all patients except when endoluminal synechiae or iliac venous thrombosis were associated with postural compression. In this case a 50% success rate was achieved. The endovascular revolution offers a less invasive technique for treatment of chronic iliac venous obstruction. Follow-up is short at present in the few publications found in the literature. Conclusions: Iliac vein obstruction results in symptoms of swelling in the lower limbs. These may be managed conservatively. Where there is an indication for venous reconstruction, investigation by duplex ultrasonography is the first step. Endovascular procedures including stenting offer significant benefit. The long-term outcome of these interventions has yet to be established.


1995 ◽  
Vol 109 (4) ◽  
pp. 320-323 ◽  
Author(s):  
P. Murthy ◽  
W. S. Mckerrow

AbstractAn audit project was undertaken to appraise the outcome of 95 cases of septal surgery over a 12-month period, taking into account the subjective alteration of symptoms, appearances at anterior rhinoscopy and complications. Seventy-three patients (76.8 per cent) attended the follow-up clinic three to six months (median four months) after surgery. There was an improvement in short-term nasal breathing and in the clinical appearance of the septum in 84.9 and 96.9 per cent respectively; a complication rate of 10.9 per cent and an overall satisfaction rate of 81.3 per cent were also noted. These results support those of other reported series and suggest that routine follow-up of uncomplicated cases of septal surgery is unnecessary.


2020 ◽  
Author(s):  
Daryl Oswald Cheng ◽  
Claire Jacqueline Calderwood ◽  
Erik Wilhelm Skyllberg ◽  
Adam Denis Jeremy Ainley

AbstractBackgroundDescriptions of clinical characteristics of patients hospitalised with coronavirus disease 2019 (COVID-19), their clinical course and short-term in- and outpatient outcomes in deprived urban populations in the United Kingdom are still relatively sparse. We describe the epidemiology, clinical course, experience of non-invasive ventilation and intensive care, mortality and short-term sequalae of patients admitted to two large District General Hospitals across a large East London NHS Trust during the first wave of the pandemic.MethodsA retrospective analysis was carried out on a cohort of 1,946 patients with a clinical or laboratory diagnosis of COVID-19, including descriptive statistics and survival analysis. A more detailed analysis was undertaken of a subset of patients admitted across three Respiratory Units in the trust.ResultsIncreasing age, male sex and Asian ethnicity were associated with worse outcomes. Increasing severity of chest X-ray abnormalities trended with mortality. Radiological changes persisted in over 50% of cases at early follow up (6 weeks). Ongoing symptoms including hair loss, memory impairment, breathlessness, cough and fatigue were reported in 67% of survivors, with 42% of patients unable to return to work due to ongoing symptoms.ConclusionsUnderstanding the acute clinical features, course of illness and outcomes of COVID-19 will be vital in preparing for further peaks of the pandemic. Our initial follow up data suggest there are ongoing sequalae of COVID-19 including persistent symptoms and radiological abnormalities. Further data, including longer term follow up data, are necessary to improve our understanding of this novel pathogen and associated disease.Section 1: What is already known on this topicPrevious studies have reported that increasing age, male sex, Black and Asian ethnicity increased risk of death for patients admitted to hospital with coronavirus disease 2019 (COVID-19). There is little published literature regarding the follow up of patients with COVID-19.Section 2: What this study addsOur study is one of the first with follow up data for patients admitted to hospital with COVID-19. We show that radiological abnormality persisted at 6 weeks in over 50% of patients, as well as significantly increased breathlessness in patients without baseline dyspnoea. Our study confirms that increasing age, male sex and Asian ethnicity increased risk of death for patients, but also in an ethnically and socioeconomically diverse population in East London.


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
M Maga ◽  
A Wachsmann ◽  
P Maga

Abstract Background Peripheral artery disease (PAD) is one of the common atherosclerosis manifestations. However endovascular treatment (percutaneous transluminal angioplasty - PTA) remains the most frequently applied treatment method, it is less successful in patients with recurrent restenosis. Even though the influence of cysteinyl eicosanoids on atherosclerosis progression is well-known, their role in restenosis after endovascular treatment has not been studied yet. Purpose The aim of this study was to establish prognostic features of urinary leukotrienes E4 (uLtE4) in occurrence of restenosis. Methods 180 patients admitted for PTA with lower limbs ischemia on 3 or 4 stage of Rutherford scale were recruited in this prospective innovative study. Urine samples were collected before intervention as well as 2 hours after and day after it. During control visits were performed 1,3,6 and 12 months after PTA including uLtE4 level measurement, evaluation of treatment success and complex assessment of restenosis basing on ankle-brachial index, Doppler ultrasound and general condition of patient. To perform the analysis all of patients were divided into 2 groups basing on presence of complications on each follow-up meeting: A-without restenosis; B-with restenosis. Results Significant differences in level of uLtE4 were observed between group A and B on 3-month (85.57 vs 157.64, p<0.002) 6-month (83.17 vs 158.28, p<0.001) and 12-month (76.81 vs 150.25, p<0.001) follow-up visits (Fig1). Patients with increased uLtE4 level in 1month had higher risk of restenosis in 3-month observation (OR=3.57, p=0.002) and elevated uLtE4 in 3-month follow-up increased the risk of restenosis in 6 months (OR=2.38, p=0.016). The same trend was observed in 6 months uLtE4 levels regarding 12-months restenosis however it wasn't statistically significant (OR=1.82, p=0.1). However post-operative uLtE4 were significantly higher compared to the pre-operative ones, they did not correlated with future restenosis in none of the follow-ups. Figure 1 Conclusions Our study shows that, elevated level of uLtE4 correlates with increased risk of restenosis occurrence in PAD patients after EVT. Leukotrienes rise can also predict the restenosis in the following months. These results confirms the role of inflammatory mediators in atherosclerosis and let us believe, that one day uLtE4 level may become the significant biomarker of complications after lower limbs endovascular treatment. Acknowledgement/Funding Grant of Polish Ministry of Science and Higher Education - “Best of the best 2.0” (S/FS0/0267)


2013 ◽  
Vol 31 (15_suppl) ◽  
pp. 10539-10539
Author(s):  
Toshirou Nishida ◽  
Tsuyoshi Takahashi ◽  
Mari Kaneda ◽  
Maiko Ako ◽  
Takeshi Omori ◽  
...  

10539 Background: Most gastrointestinal stromal tumors (GIST) have either KIT or PDGFRA mutations. Neurofibromatosis type 1 pts caused by mutations in the NF1 gene have increased risk of GIST development, which may have no mutation in both genes. In this study, we analyzed clinical and pathological features of NF-1 associated GISTs. Methods: Study 1: We have screened 95 adults NF1 pts (age 31-66, 35 male and 60 female) by enhanced MDCT between 2003 and 2012. Study 2: We collected 1,184 sporadic GISTs from community hospitals in Japan between 2001 and 2010 retrospectively, and found 24 primary NF1-GISTs (1.7% of sporadic) and 2 recurrent NF1-GISTs, of whom clinicopathological features were analyzed. Results: Study1: By MDCT screening, we have found histologically confirmed 6 GISTs (4 males and 2 female; 6/1,000 NF1-persons/year) in the small intestine. Median age of NF1-GIST was 45, and five pts had multiple tumors, ICC hyperplasia in the normal intestine and no mutation in the KIT and PDGFRA genes. Study 2: Median age of 26 NF1-GIST (12 male and 14 female) was 58. 25 GISTs were located in the small bowel and one in the stomach. 17 pts had multiple GISTs and 9 pts single lesion. Pathologically, KIT was positive for all NF1-GISTs. 24 pts had spindle cell tumors and 2 had mixed or epithelioid. No mutation was found in the KIT and PDGFRA genes of 11 pts examined. Median values of mitosis (0/50HPF) and Ki67 (0.5%) were lower than those of sporadic GIST (3/50HPF and 2.5%). With media follow-up of 3.6 years, 8 pts had recurrences and 4 pts died of the disease. By western blotting, KIT was faintly phosphorylated but its downstream kinases including MEK, p44/22, AKT, mTOR, p38 and STAT3, were activated. Six pts received imatinib and had no response and, subsequently, 5 pts received sunitinib with 4 PD and 1 short-term SD. Conclusions: NF1-associated GIST is a rare entity of GIST and has distinctive features from conventional sporadic GISTs. KIT-targeted TKI appeared to be ineffective to recurrent and advanced NF1-GISTs.


Author(s):  
Balaji Zacharia ◽  
Jittu Alex ◽  
Ashwin Rajmohan

AbstractWe present a case of a 14-year-old girl who was first treated when she was 4 years old. She had progressive limping of the left lower limb from the age of 3 years. She was diagnosed to have developmental coxa vara of the left hip and treated by a subtrochanteric valgus osteotomy of the left femur. Later, she developed hypertrophy of the left upper and lower limbs. There were port-wine stains over the left lower limbs with multiple superficial varicosities. Her diagnosis was Klippel–Trénaunay syndrome (KTS). She is asymptomatic at the final follow-up. Both developmental coxa vara and KTS are rare conditions. We present this case to demonstrate the rarest combination of two rare conditions occurring in the same limb.


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