Foot Macrodactyly Associated with Klippel-Trenaunay Syndrome

2021 ◽  
Vol 23 (5) ◽  
pp. 375-380
Author(s):  
Maurizio De Pellegrin ◽  
Désirée Moharamzadeh ◽  
Giacomo Placella ◽  
Vincenzo Salini

Klippel-Trenaunay syndrome is a rare congenital malformation which may be associated with macrodactyly. The main problem is the need for custom-made shoes. We describe the case of a female newborn affected by Klippel-Trenaunay syndrome, with a larger and longer right foot; the difference increased progressively and, at 5 years of age, the right foot was 50% larger and 38% longer than the left one. Due to the progression of the deformity, reduction surgery was advised to reduce the foot’s width. Resection of the second ray and 2nd cuneiform was performed. The result was excellent and there were no complications during a 10-year follow-up period with a decrease of width and length difference to 10% and 4%, respectively, in comparison to the contralateral foot. There were no gait anomalies. Abnormal foot width and length represented the main problems and guided the surgical strategy. Second ray resection was effective, without complications and with a good long-term functional outcome. Possibility of wearing fashionable conventional shoes without insoles was achieved to the patient’s satisfaction

Cells ◽  
2021 ◽  
Vol 10 (3) ◽  
pp. 594
Author(s):  
Lukas Prantl ◽  
Eva Brix ◽  
Sally Kempa ◽  
Oliver Felthaus ◽  
Andreas Eigenberger ◽  
...  

Lipofilling is a popular technique to treat volume loss in aging patients. The isolated adipose tissue is composed of adipocytes and stromal vascular fraction cells, which include adipose-derived stem cells (ASC). We hypothesize that the patient’s wrinkle severity scale (WSS) and patient’s satisfaction on the global aesthetic improvement scale (GAIS) can be improved after using concentrated lipoaspirate. Fourteen patients (54 years ± 11.09 years) with volume loss in the midface area underwent waterjet-assisted liposuction (Human Med AG, Schwerin, Germany). Fat was centrifuged in an ACP Double Syringe (Arthrex GmbH, Munich, Germany) using Rotofix 32A centrifuge (Andreas Hettich, GmbH & Co.KG, Tuttlingen, Germany). Homogenization was performed using the double syringe and a 1.4 mm female–female luerlock connector. After a second centrifugation, patients received periorbital (PO) and nasolabial (NL) lipografting. ASC count was performed after enzymatical digestion. Vitality of cells was assessed using a resazurin assay. During long-term follow up (12 months, n = 10), we found a high patient’s satisfaction (GAIS 1+/−0.52) and a good improvement of the WSS during short- and long-term follow-up. The ASC count of processed lipoaspirate was 2.1-fold higher than of unprocessed lipoaspirate (p < 0.001). The difference of ASC in sedimented and simply centrifuged lipoaspirate was also significant (p < 0.05). Facial rejuvenation with concentrated fat graft offers good results concerning objective aesthetic outcome and patient’s satisfaction.


2021 ◽  
pp. 263246362097804
Author(s):  
Vanita Arora ◽  
Pawan Suri

Anatomy and physiology are the basis of human body functioning and as we have progressed in management of various diseases, we have understood that physiological intervention is always better than an anatomical one. For more than 50 years, a standard approach to permanent cardiac pacing has been an anatomical placement of transvenous pacing lead at the right ventricular apex with a proven benefit of restoring the rhythm. However, the resultant ventricular dyssynchrony on the long-term follow-up in patients requiring more than 40% ventricular pacing led to untoward side effects in the form of heart failure and arrhythmias. To counter such adverse side effects, a need for physiological cardiac pacing wherein the electrical impulse be transmitted directly through the normal conduction system was sought. His bundle pacing (HBP) with an intriguing alternative of left bundle branch pacing (LBBP) is aimed at restoring such physiological activation of ventricles. HBP is safe, efficacious, and feasible; however, localization and placement of a pacing lead at the His bundle is challenging with existing transvenous systems due to its small anatomic size, surrounding fibrous tissue, long-learning curve, and the concern remains about lead dislodgement and progressive electrical block distal to the HBP lead. In this article, we aim to take the reader through the challenging journey of HBP with focus upon the hardware and technique, selective versus nonselective HBP, indications and potential disadvantages, and finally the future prospects.


2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Shuntaro Oribe ◽  
Takafumi Toyohara ◽  
Eikan Mishima ◽  
Takehiro Suzuki ◽  
Koichi Kikuchi ◽  
...  

Abstract Background Fibromuscular dysplasia (FMD) often causes renal artery stenosis with renovascular hypertension. Recent clinical outcomes encourage percutaneous transluminal renal angioplasty (PTRA) to treat FMD; however, the necessary follow-up period remains unclear. Moreover, previous studies have not revealed the difference in the period until recurrence between two major types of FMD—multifocal and focal. Case presentation We describe two patients with multifocal FMD who developed hypertension during their teenage years and had recurrence of FMD > 10 years after PTRA. We further examined the types of FMD and age of onset in 26 patients who underwent PTRA. The period until recurrence of multifocal FMD was longer than that of focal FMD. Moreover, patients with early-onset multifocal FMD are likely to have a delayed recurrence after PTRA compared to other types. Conclusions Our report suggests that patients with multifocal FMD, especially those with onset at an early age, may need long-term follow-up for at least ≥ 10 years.


2002 ◽  
Vol 12 (3) ◽  
pp. 224-228 ◽  
Author(s):  
Haifa Abdul Latiff ◽  
Mazeni Alwi ◽  
Hasri Samion ◽  
Geetha Kandhavel

This study reviewed the short-term outcome of transcatheter closure of the defects within the oval fossa using an Amplatzer® Septal Occluder. From January 1997 to December 2000, 210 patients with defects within the oval fossa underwent successful transcatheter closure. We reviewed a total of 190 patients with left-to-right shunts, assessing the patients for possible complications and the presence of residual shunts using transthoracic echocardiogram at 24 h, 1 month, 3 months and one year. Their median age was 10 years, with a range from 2 to 64 years, and their median weight was 23.9 kg, with a range from 8.9 to 79 kg. In 5 patients, a patent arterial duct was closed, and in 2 pulmonary balloon valvoplasty performed, at the same sitting. The median size of the Amplatzer® device used was 20 mm, with a range from 9 to 36 mm. The median times for the procedure and fluoroscopy were 95 min, with a range from 30 to 210 min, and 18.4 min, with a range from 5 to 144 min, respectively. Mean follow-up was 20.8 ± 12.4 months. Complete occlusion was obtained in 168 of 190 (88%) patients at 24 h, 128 of 133 (96.2%) at 3 months, and 103 of 104 (99%) at one year. Complications occurred in 4 (2.1%) patients. In one, the device became detached, in the second the device embolized into the right ventricular outflow tract, the lower end of the device straddled in the third, and the final patient had significant bleeding from the site of venupuncture. There were no major complications noted on follow-up. We conclude that transcatheter closure of defects within the oval fossa using the Amplatzer® Septal Occluder is safe and effective. Long-term follow-up is required, nonetheless, before it is recommended as a standard procedure.


2018 ◽  
Vol 46 (6) ◽  
pp. 1362-1370 ◽  
Author(s):  
Philipp Schuster ◽  
Markus Geßlein ◽  
Michael Schlumberger ◽  
Philipp Mayer ◽  
Raul Mayr ◽  
...  

Background: High tibial osteotomy (HTO) is a widely used treatment option for medial osteoarthritis and varus malalignment, especially in young patients with early osteoarthritis. Limited outcome data are available for this procedure in severe osteoarthritis, and no long-term data are available using newer implants. Purpose: To determine survivorship and functional results of medial open-wedge HTO combined with a chondral resurfacing (CR) procedure (abrasion plus microfracture) in severe medial osteoarthritis (Kellgren-Lawrence grade 3 and 4) and varus malalignment. Furthermore, factors that potentially influence the outcome were analyzed. Study Design: Case series; Level of evidence, 4. Methods: From September 2005 to December 2008, all cases of HTO (fixation with an angular-stable internal fixator) combined with CR were prospectively surveyed with regard to survival (Kaplan-Meier-method, not requiring arthroplasty) and functional outcome (subjective International Knee Documentation Committee [IKDC] score). Cartilage regeneration at the time of hardware removal, tibial bone varus angle (TBVA), pre- and postoperative mechanical medial proximal tibial angle (MPTA), and postoperative alignment were analyzed with regard to the result. Results: Seventy-nine knees were included (73 patients; mean age 50.9 ± 7.6 years). The follow-up rate was 90% at 10.0 ± 1.2 years (range, 8.3-12.1 years). Pre- and postoperative mechanical tibiofemoral axis were 9.6° ± 3.0° of varus and 0.6° ± 2.7° of valgus, respectively. Survival rate was 81.7% (95% CI, 72.5%-90.9%) at 10 years. Subjective IKDC score significantly improved from 44 ± 11 preoperatively to 70 ± 13 at one, 66 ± 15 at three, 66 ± 15 at five, and 65 ± 17 at ten years ( P < .001 at any point of follow-up). Poor cartilage regeneration and low preoperative IKDC score (<40) were associated with decreased survival. High preoperative TBVA was associated with better and an overcorrected MPTA (>95°) with inferior functional outcome at final follow-up, respectively. Conclusion: Even in cases of severe medial osteoarthritis and varus malalignment, HTO in combination with a CR procedure is a good to excellent treatment option. The role of the CR procedure remains unclear. Although good results are obtained with overcorrected MPTA, long-term functional outcome is inferior.


2021 ◽  
Vol 20 (1) ◽  
pp. 15-17
Author(s):  
Caroline Apsey ◽  
◽  
Muhammad Jawad ◽  
Martin Daschel ◽  
Daniel Woosey ◽  
...  

We assessed the efficacy of thrombolysis in avoiding long-term complications. Notes of patients thrombolysed for PE in the 2-year period were reviewed. The initial CTPA and echocardiogram results before thrombolysis were compared to the results of follow up imaging repeated after 6 months. Twenty-two patients were thrombolysed for PE. 14 patients had sub-massive PE and 8 patients had massive PE. The right ventricle (RV) was dilated on pre-thrombolysis echocardiogram in 16 patients. On follow up echocardiography all patients with massive PE (6 studies) had a normal RV size, with pulmonary artery pressures (PAP) of 29mmHg. Follow up echocardiography of patients with submassive PE (13 studies) showed 11 patients with a normal RV, with PAP of 28 mmHg.


Stroke ◽  
2017 ◽  
Vol 48 (suppl_1) ◽  
Author(s):  
Miguel A Barboza ◽  
Rodrigo Uribe ◽  
Fabiola Serrano ◽  
Luis C Becerra-Pedraza ◽  
D. K Mantilla-Barbosa ◽  
...  

Background and purpose: Atherosclerotic ischemic stroke is the second most frequent etiology of stroke in the adult population. Functional outcome, mortality and recurrence of stroke rates on the long-term follow-up are poorly studied. This study investigates long-term outcome among patients with ischemic stroke secondary to atherosclerotic causality, and identifies the main factors associated with poor outcome, recurrence, and death. Methods: We analyzed data from our consecutive acute ischemic stroke database, over a period of 25 years (1990-2015). The endpoints were: bad outcome (Modified Rankin Score ≥3), recurrence and mortality at discharge, and final follow-up. Multivariate Cox and Kaplan-Meier analysis were used to estimate the probability of death and recurrence. Results: A total of 946 consecutive atherosclerotic stroke patients were included (571 [60.4%] males, median age 65 years [interquartile range 57-73 years] for the entire population); dyslipidemia (64.2%), hypertension (63.3%), diabetes (35.0%), and active smoking history (31.8%) were the most prevalent risk factors.After a median follow-up of 38 months (IQR 12-75 months), 59.3% patients had a bad outcome at discharge. A result of 26.1% had stroke recurrence (median time until recurrence: 9 months [IQR 12-84 months], with 12.9% cases presenting ≥2 recurrences), and 24.1% were dead (median time to death: 18.5 months [IQR 11-74 months]) at the final follow-up period. After multivariate adjustment, hypertension (HR 4.2, CI 95% 2.8-6.1; p<0.001) was the strongest predictor of recurrence. Additionally, diabetes (HR 2.6, CI 95% 2.0-3.5; p<0.001), bad functional outcome after recurrence (HR 2.3, CI 95% 1.9-2.9; p<0.001), age ≥65 years (HR 2.2, CI 95% 1.7-2.9; p<0.001), and active smoking (HR 1.8, CI 95% 1.3-2.3; p<0.001) were the strongest predictors of mortality. Conclusions: Atherosclerotic ischemic stroke has a high rate of recurrence, associated mainly with hypertension. Mortality is predicted by diabetes, bad functional outcome at recurrence, and older age.


2018 ◽  
Vol 75 (5) ◽  
pp. 512-515 ◽  
Author(s):  
Sasa Hinic ◽  
Jelena Saric ◽  
Predrag Milojevic ◽  
Jelena Gavrilovic ◽  
Tijana Durmic ◽  
...  

Introduction. Myxoma is the most common primary benign heart tumor. The most frequent location is the left atrium, the chamber of the heart that receives oxygen- rich blood from the lungs. Myxomas usually develop in women, typically between the ages of 40 and 60. Symptoms may occur at any time, but most often they are asymptomatic or oligosymptomatic for a long period of time. Symptoms usually go along with body position, and are related to compression of the heart cavities, embolization and the appearance of general symptoms. The diagnosis of benign tumors of the heart is based on anamnesis, clinical features and findings of the tumor masses by use of non-invasive and invasive imaging methods. Extensive surgical resection of the myxoma is curative with minimal mortality. Long term clinical and echocardiographic follow-up is mandatory. Case report. We reported a case of a 62-year-old male, presented with 15 days of intermittent shortness of breath, dizziness and feeling of heart palpitations and subsequently diagnosed with right atrial myxoma based on transthoracic echocardiography . The patient was emergently operated in our hospital. Long-term followup did not reveal recurrence. Conclusion. Our case was an atypical localisation of right atrial myxoma. Whether the intracardiac mass is benign or malignant, early surgery is obligatory in order to prevent complications.


Neurosurgery ◽  
2017 ◽  
Vol 80 (6) ◽  
pp. 967-974 ◽  
Author(s):  
Christoph Schwartz ◽  
Thomas Pfefferkorn ◽  
Caroline Ebrahimi ◽  
Caroline Ottomeyer ◽  
Gunther Fesl ◽  
...  

Abstract BACKGROUND: Detailed data on long-term functional outcome of patients with World Federation of Neurosurgical Societies (WFNS) grades IV and V aneurysmal subarachnoid hemorrhages (aSAH) are still scarce. OBJECTIVE: Assessment of long-term outcome of WFNS IV and V aSAH patients. METHODS: Functional outcome and quality of life were assessed by the modified Rankin scale (mRS) and the 36-item short-form health survey in consecutively treated aSAH WFNS IV and V patients between 2005 and 2010. Scores from the 36-item short-form health survey were compared to a healthy German population. Prognostic factors were analyzed by uni- and multivariate models. RESULTS: One hundred and seven eligible patients (median age: 53.0 years) were identified. After interdisciplinary consensus on optimal treatment, aneurysms were obliterated either by clipping (n = 35) or by coiling (n = 72). Ten patients were lost to long-term follow-up; the median clinical follow-up period was 3.2 years for the remaining 97 cases. Twenty-five of 97 died during the acute hospital phase and another 10 patients over the follow-up period leaving 62 long-term survivors. At the end of clinical follow-up, 40/97 patients, including 40/62 of long-term survivors, reached functional independence (mRS ≤ 2). Twelve of 97 patients were moderately (mRS = 3), 10/97 patients were severely disabled (mRS ≥ 4). Younger age (≤ 53 years; P = .001) and radiological absence of cerebral infarction (P = .03) were the strongest predictors for favorable outcome. Quality of life was perceived to be only moderately reduced compared to the healthy control group. CONCLUSION: Poor-grade aSAH is not necessarily associated with poor long-term functional outcome; after aneurysm repair ∼60% of patients survived and among long-term survivors ∼ 60% regained functional independence.


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