scholarly journals Conservative management of knee arthropathy in a patient with Klippel Trenaunay syndrome

2020 ◽  
Vol 19 ◽  
Author(s):  
Fanny Rodríguez Santos ◽  
Victoria Loson ◽  
Agustín Coria ◽  
Hugo Martínez

Abstract Klippel-Trenaunay syndrome (KTS) is a rare vascular malformation characterized by capillary malformation, venous malformations, and soft tissue or bone hypertrophy that affect the extremities in most cases. Knee or hip arthropathy are common associated conditions and cause serious disability. We present the case of a patient with a diagnosis of KTS and severe knee arthropathy. A 34-year-old man with KTS was referred to our hospital with severe knee arthropathy, with the joint fixed in a 90° position. CT Angiography and MRI of the left leg showed important varicose development of the superficial venous system with intraarticular vessels. After discussion of the case by a multidisciplinary committee, the patient was enrolled on a physiotherapy program and had achieved significant improvements in movement and quality of life at 12-month follow-up. Treatment of KTS is primarily conservative and a multidisciplinary approach is necessary.

2008 ◽  
Vol 192 (4) ◽  
pp. 300-305 ◽  
Author(s):  
Claire A. G. Wolfs ◽  
Alfons Kessels ◽  
Carmen D. Dirksen ◽  
Johan L. Severens ◽  
F. R. J. Verhey

BackgroundAn integrated multidisciplinary approach to dementia is often recommended but has rarely been evaluated.AimsTo evaluate the clinical effects of an integrated multidisciplinary diagnostic facility for psychogeriatric patients.MethodPatients suspected of having complex psychogeriatric problems were randomly allocated to the intervention (n=137) or to treatment as usual (n=93). They were assessed at baseline, and at 6 months and 12 months follow-up by means of personal interviews with the patient's proxy. The primary outcome was health-related quality of life, assessed using the visual analogue scale (VAS) of the EuroQd measure, EQ-5D.ResultsHealth-related quality of life had improved at 6 months in the intervention group, whereas that of the control group had decreased. Furthermore, more patients in the intervention group experienced a clinically relevant improvement of 10 points or more on the VAS at both follow-up measurements.ConclusionsAn integrated multidisciplinary approach improves dementia care.


2021 ◽  
Vol 12 ◽  
Author(s):  
Ruben M. Strijbos ◽  
Louise V. Straatman ◽  
Tim G. A. Calon ◽  
Martin L. Johansson ◽  
Arthur J. G. de Bruijn ◽  
...  

Objective: Comparing the surgical outcomes of the Minimally Invasive Ponto Surgery (MIPS) technique with the linear incision technique with soft tissue preservation (LITT-P) for bone conduction devices after a follow-up of 22 months.Methods: In this multicenter randomized controlled trial, there was the inclusion of 64 adult patients eligible for unilateral surgery. There was 1:1 randomization to the MIPS (test) or the LITT-P (control) group. The primary outcome was an (adverse) soft tissue reaction. Secondary outcomes were pain, loss of sensibility, soft tissue height/overgrowth, skin sagging, implant loss, Implant Stability Quotient measurements, cosmetic scores, and quality of life questionnaires.Results: Sixty-three subjects were analyzed in the intention-to-treat population. No differences were found in the presence of (adverse) soft tissue reactions during complete follow-up. Also, there were no differences in pain, wound dehiscence, skin level, soft tissue overgrowth, and overall quality of life. Loss of sensibility (until 3-month post-surgery), cosmetic scores, and skin sagging outcomes were better in the MIPS group. The Implant Stability Quotient was higher after the LITT-P for different abutment lengths at various points of follow-up. Implant extrusion was nonsignificantly higher after the MIPS (15.2%) compared with LITT-P (3.3%).Conclusion: The long-term results show favorable outcomes for both techniques. The MIPS is a promising technique with some benefits over the LITT-P. Concerns regarding nonsignificantly higher implant loss may be overcome with future developments and research.Clinical Trial Registration:www.ClinicalTrials.gov, identifier: NCT02438618.


Author(s):  
Deepak Sharma ◽  
Sachin Lamba ◽  
Aakash Pandita ◽  
Sweta Shastri

Klippel–Trénaunay syndrome (KTS or KT) is an infrequently seen dermatological syndrome, which is often viewed as a triad of vascular malformation (capillary malformations or port-wine brands), venous varicosity, and soft tissue and/or bony hypertrophy. We report a case of a 12-year-old male who presented to us with the symptoms of varicose plaques over both lower limbs and was diagnosed as a case of KTS. Management is normally conservative and includes stockings for compression of the branches to reduce edema because of chronic venous insufficiency; modern devices that cause on and off pneumatic compression; and rarely, surgical correction of varicose veins with lifelong follow-up. The orthopedic abnormalities are treated with epiphysiodesis in order to prevent (stop) overgrowing of limb and correction of bone deformity.


2020 ◽  
Vol 35 (8) ◽  
pp. 597-604
Author(s):  
Claude Laurian ◽  
Pierre Cerceau ◽  
Nikos Paraskevas ◽  
Claudine Massoni ◽  
Veronique Marteau ◽  
...  

Objectives To report the outcomes of surgical treatment of calf intramuscular venous malformations (IMVMs) on pain, functional limitation, and quality of life. Methods We retrospectively reviewed 57 consecutive patients who had surgery for IMVM of the posterior compartment of the leg between 2010 and 2015. Treatments were all done at a single institution. Results Patients presented with pain (52), muscle contracture (14), or pulmonary embolism (4). Muscle involvement included the soleus muscle (n = 28, 49%), the gastrocnemius muscle (n = 25, 43%), and deep muscles (n = 4, 7%). Complete excision was possible in 52 patients (91%) and partial excision in 5 (9%). Thirty-five of 46 patients who had an MRI follow-up at six months had no residual venous malformation. At the final follow-up (mean 39 months), 32 of 40 patients seen had no residual pain and 37 had no residual functional impairment. Conclusion In cases where IMVM is located in one muscle in the leg, we demonstrated that surgery yielded improvement in pain, function, and quality of life.


Ultrasound ◽  
2019 ◽  
Vol 28 (2) ◽  
pp. 91-102
Author(s):  
Olga Ivanitskaya ◽  
Elena Andreeva ◽  
Natalia Odegova

Klippel–Trenaunay syndrome is a rare disease with a classic triad of port wine stains, varicose veins, and bony and soft tissue hypertrophy of an extremity. The quality of life in these patients is significantly affected, making the prenatal diagnosis of Klippel–Trenaunay syndrome important. We present four prenatally diagnosed cases of this anomaly with a unique case of ectrodactyly of the hand in foetus with Klippel–Trenaunay syndrome. Such a combination has not been previously reported prenatally. A review of the literature for similar cases is also presented.


2019 ◽  
pp. 244-251
Author(s):  
A. S. Yunusov ◽  
T. I. Garashchenko ◽  
P. I. Belavina ◽  
A. G. Ryazanskaya ◽  
E. V. Molodtsova ◽  
...  

Rationale. Currently, the literature describes about 374 cases of Marshall syndrome. According to some authors, the prevalence of Marshall syndrome is 2.3 per 10,000 children, in general, the epidemiology of PFAPA syndrome in the pediatric population is unknown.Purpose. The aim of our study is to justify surgical treatment in children with Marshall syndrome. Objectives. The objectives of this study are to give a comparative assessment of the effectiveness of tonsillectomy and adenotonzyllectomy for this syndrome and to evaluate the quality of life indicators before and after surgical treatment.Materials and methods. The study included 26 patients with Marshall syndrome, of which 18 patients underwent adenotonzyllectomy and 8 patients underwent bilateral tonsillectomy. A clinical case of a typical manifestation of Marshall syndrome is also described, and follow-up results of the effectiveness of surgical treatment for Marshall syndrome are presented. In the course of the work, an adapted questionnaire was developed to assess the quality of life of parents of children with Marshall syndrome.Results. The result of our work is the fact that in relation to the relief of PFAPA-syndrome, children of preschool and primary school age have more effective adenotonsillectomy (88.2%), while tonsillectomy was effective only in 55.6% of patients.Conclusion. A multidisciplinary approach is required to address the diagnosis and treatment of Marshall syndrome.


2016 ◽  
Vol 4 (1) ◽  
pp. 294
Author(s):  
Krishnappa Santosh Kumar ◽  
Nanjappa Mohan Kumar

Klippel Trenaunay Syndrome (KTS) is a cutaneous vascular malformation in combination with bone and soft tissue overgrowth, with or without lymphatic malformation. Symptoms appear by birth and approximately 90% of the cases are diagnosed by the age of 12 years. Prompt diagnosis at the earliest and conservative management with regular follow up is crucial in prognosis of KTS.


2004 ◽  
Vol 62 (4) ◽  
pp. 963-968 ◽  
Author(s):  
Maria M.M. Ulsenheimer ◽  
Sérgio A. Antoniuk ◽  
Lúcia H.C. dos Santos ◽  
Marcos P. Ceccatto ◽  
Antônio Ernesto da Silveira ◽  
...  

We analyzed 31 children with myelomeningocele born between July 1990 and July 2000. Follow-up median was 24 months (6-68months). Only 2 mothers had a known etiologic factor (diabetes mellitus). Twelve had the correct prenatal diagnosis. All children were born at term; 23 by cesarean; 13 had rupture of the membrane. Surgical correction had a 4 days median (1 to 44 days). Lumbosacral lesions were the most frequent (46%). Thirty patients were hydrocephalic, shunt was placed in 27. Meningitis was 4 times more frequent in shunted patients. Seven became epileptic (19.4%). Denver II test showed significant delay in gross motor development . Neurogenic bladder was diagnosed in 12 patients. Congenital clubfoot was the main orthopedic malformation (53%). Six infants died. Nowadays, 17 patients are being followed. A multidisciplinary approach probably helps for a better quality of life.


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