Monomelic Maffucci syndrome

2021 ◽  
Vol 14 (3) ◽  
pp. e239619
Author(s):  
Gopalkrishna G Verma ◽  
Vijay Kumar Jain ◽  
Karthikeyan P Iyengar
Keyword(s):  
Lower Limb ◽  
Corrective Osteotomy ◽  
Valgus Deformity ◽  
Left Knee ◽  
Leg Length ◽  
Maffucci Syndrome ◽  
Successful Clinical Outcome ◽  
Genu Valgus Deformity ◽  
Angular Deformities

Maffucci syndrome is a rare congenital, non-hereditary condition characterised by presence of multiple enchondromas and haemangiomas. Enchondromatous lesions affecting epiphysial growth plates can lead to angular deformities and leg-length discrepancy in the lower limb. We describe a 12-year-old girl with monomelic Maffucci syndrome affecting her left lower limb. She presented with progressive genu valgus deformity of her left knee. This caused her to limp during her gait and was a cosmetic dissatisfaction. The deformity affected her quality of life. She underwent a supracondylar distal femoral corrective osteotomy with a successful clinical outcome and restoration of her gait and cosmetic deformity.

scholarly journals Tension band plates have greater risks of complications in temporary epiphysiodesis

2021 ◽  
Vol 15 (2) ◽  
pp. 106-113
Author(s):  
You-Hung Cheng ◽  
Wei-Chun Lee ◽  
Yi-Feng Tsai ◽  
Hsuan-Kai Kao ◽  
Wen-E Yang ◽  
...  
Keyword(s):  
Tibial Plateau ◽  
Distal Femur ◽  
Mechanical Axis ◽  
Valgus Deformity ◽  
Tension Band ◽  
Leg Length ◽  
Level Of Evidence ◽  
Grade Ii ◽  
Angular Deformities

Purpose This study aimed to compare the efficacy of decreasing leg-length discrepancy (LLD) and postoperative complications between tension band plates (TBP) and percutaneous transphyseal screws (PETS). Methods This retrospective study reviewed LLD patients who underwent temporary epiphysiodesis at the distal femur and/or proximal tibia from 2010 to 2017 (minimum two years follow-up). Efficacy of decreasing LLD was assessed one and two years postoperatively. Complications were classified with the modified Clavien-Dindo-Sink complication classification system. Knee deformities were assessed by percentile and zone of mechanical axis across the tibial plateau. Results In total, 53 patients (25 boys, 28 girls) underwent temporary epiphysiodesis (mean age, 11.4 years). The efficacy of decreasing LLD at two years between the TBP (n = 38) and PETS (n = 15) groups was comparable. Seven grade III complications were recorded in six TBP patients and in one PETS patient who underwent revision surgeries for knee deformities and physis impingement. Four grade I and two grade II complications occurred in the TBP group. The mechanical axis of the leg shifted laterally in the PETS group and medially in the TBP groups (+7.1 percentile versus -4.2 percentile; p < 0.05). Shifting of the mechanical axis by two zones was noted medially in four TBP patients and laterally in two PETS patients. Conclusion More implant-related complications and revision surgeries for angular deformities were associated with TBP. A tendency of varus and valgus deformity after epiphysiodesis using TBP and PETS was observed, respectively. Patients and families should be informed of the risks and regular postoperative follow-up is recommended. Level of evidence Level III

Total knee arthroplasty in a young patient with achondroplasia

2021 ◽  
Vol 14 (7) ◽  
pp. e242909
Author(s):  
Hassan Zmerly ◽  
Marco Russo ◽  
Manuela Moscato ◽  
Ibrahim Akkawi
Keyword(s):  
Total Knee Arthroplasty ◽  
Knee Osteoarthritis ◽  
Knee Arthroplasty ◽  
Lower Limb ◽  
Skeletal Dysplasia ◽  
Total Joint Replacement ◽  
Limb Lengthening ◽  
Valgus Deformity ◽  
Left Knee ◽  
Total Knee

Achondroplasia is a genetic skeletal dysplasia, characterised by dwarfism, with upper and lower limb deformities, that can lead to knee osteoarthritis; the surgical treatment is a challenge due to the particular joint anatomy variances. We present a 40-year-old woman, affected by achondroplasia with severe left knee osteoarthritis; the patient was treated conservatively with poor results. Between the ages of 12 and 17 years, she underwent multiple operative procedures for lower limb lengthening and a partial correction of the valgus deformity, by applying an external fixator. The patient was successfully treated with cemented posterior stabilised, fixed-bearing, total knee arthroplasty. The patient affected by achondroplasia, with severe knee osteoarthritis, should be considered for total joint replacement, which can be carried out with or without additional extra-articular osteotomy for alignment correction. Due to skeletal dysplasia in joint deformities, knee replacement requires careful preoperative planning and special technical considerations.

scholarly journals Economic assessment of a community-based care package for people with lower limb disorder caused by lymphatic filariasis, podoconiosis and leprosy in Ethiopia

2020 ◽  
Vol 114 (12) ◽  
pp. 1021-1034
Author(s):  
Natalia Hounsome ◽  
Mersha Kinfe ◽  
Maya Semrau ◽  
Oumer Ali ◽  
Abraham Tesfaye ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Lymphatic Filariasis ◽  
Lower Limb ◽  
Life Quality ◽  
Psychosocial Care ◽  
Northwest Ethiopia ◽  
World Health ◽  
Care Package ◽  
The Cost

Abstract We conducted an implementation research study to integrate a holistic package of physical health, mental health and psychosocial care for podoconiosis, lymphatic filariasis and leprosy into routine healthcare in Gusha cluster, Guagusa Shikudad district, northwest Ethiopia. The healthcare package included training patients in lower limb hygiene and skin care and provision of shoes, hygiene supplies and medication. The implementation activities included training events, workshops, awareness raising, self-help groups, supportive supervision, staff secondments and advisory board meetings. The cost of implementing the care package in Gusha cluster, with a population of 30 558 people, was 802 655 Ethiopian birr (ETB) (£48 159) and the cost of delivering care to 235 participants was 204 388 ETB (£12 263), or 870 ETB (£52) per person. There was a 35% decrease in the mean disability scores (measured using the World Health Organization Disability Assessment Schedule 2.0) and a 45% improvement in the dermatology-specific quality of life (measured using the Dermatology Life Quality Index) at the 3-month follow-up compared with baseline. There were reductions in the number of days with symptoms, days off usual activities/work and days with reduced activity due to illness, all of which were statistically significant. Our pilot suggests that integration of the care package into routine healthcare in Ethiopia may be effective in improving health-related quality of life and disability and reducing time out of economic activity due to illness.

scholarly journals Lower Limb Kinematics in Individuals with Hip Osteoarthritis during Gait: A Focus on Adaptative Strategies and Interlimb Symmetry

2021 ◽  
Vol 8 (4) ◽  
pp. 47
Author(s):  
Micaela Porta ◽  
Massimiliano Pau ◽  
Bruno Leban ◽  
Michela Deidda ◽  
Marco Sorrentino ◽  
...  
Keyword(s):  
Lower Limb ◽  
Functional Limitations ◽  
Hip Osteoarthritis ◽  
Movement Analysis ◽  
Human Movement ◽  
Point Of View ◽  
Negative Effects ◽  
Gait Patterns ◽  
Limb Kinematics

Among the functional limitations associated with hip osteoarthritis (OA), the alteration of gait capabilities represents one of the most invalidating as it may seriously compromise the quality of life of the affected individual. The use of quantitative techniques for human movement analysis has been found valuable in providing accurate and objective measures of kinematics and kinetics of gait in individuals with hip OA, but few studies have reported in-depth analyses of lower limb joint kinematics during gait and, in particular, there is a scarcity of data on interlimb symmetry. Such aspects were investigated in the present study which tested 11 individuals with hip OA (mean age 68.3 years) and 11 healthy controls age- and sex-matched, using 3D computerized gait analysis to perform point-by-point comparisons of the joint angle trends of hip, knee, and ankle. Angle-angle diagrams (cyclograms) were also built to compute several parameters (i.e., cyclogram area and orientation and Trend Symmetry) from which to assess the degree of interlimb symmetry. The results show that individuals with hip OA exhibit peculiar gait patterns characterized by severe modifications of the physiologic trend at hip level even in the unaffected limb (especially during the stance phase), as well as minor (although significant) alterations at knee and ankle level. The symmetry analysis also revealed that the effect of the disease in terms of interlimb coordination is present at knee joint as well as hip, while the ankle joint appears relatively preserved from specific negative effects from this point of view. The availability of data on such kinematic adaptations may be useful in supporting the design of specific rehabilitative strategies during both preoperative and postoperative periods.

scholarly journals Effect of Leg-length Discrepancy on Lower-limb Muscle Activities during Gait: Focus on Equinus Gait

2015 ◽  
Vol 30 (6) ◽  
pp. 961-965
Author(s):  
Yuichi HONDA ◽  
Eiichi SAKAKI ◽  
Hisayoshi YOSHIZUKA ◽  
Yasushi NAGAO
Keyword(s):  
Lower Limb ◽  
Leg Length Discrepancy ◽  
Lower Limb Muscle ◽  
Limb Muscle ◽  
Leg Length ◽  
Length Discrepancy

A Prospective Study in Detection of Lower-Limb Lymphedema and Evaluation of Quality of Life After Vulvar Cancer Surgery

2012 ◽  
Vol 22 (6) ◽  
pp. 1081-1088 ◽  
Author(s):  
Marta Novackova ◽  
Michael J. Halaska ◽  
Helena Robova ◽  
Ivana Mala ◽  
Marek Pluta ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Lower Limb ◽  
Bioelectrical Impedance Analysis ◽  
Vulvar Cancer ◽  
Bioelectrical Impedance ◽  
Impedance Analysis ◽  
Cancer Surgery ◽  
Control Group ◽  
Inguinofemoral Lymphadenectomy

BackgroundLower-limb lymphedema is one of the most disabling adverse effects of vulvar cancer surgery. Multifrequency Bioelectrical Impedance Analysis (MFBIA) is a modern noninvasive method to detect lymphedema. The first aim of this study was to prospectively determine the prevalence of secondary lower-limb lymphedema after surgical treatment for vulvar cancer using objective methods, circumference measurements and MFBIA technique. The second aim was to compare quality of life (QoL) before and 6 months after vulvar surgery.MethodsTwenty-nine patients underwent vulvar cancer surgery in our study: 17 underwent inguinofemoral lymphadenectomy (RAD), and 12 underwent sentinel lymph node biopsy (CONS). Patients were examined before and 6 months after vulvar surgery by measuring the circumference of the lower limbs and with MFBIA. A control group of 27 healthy women was also measured. To evaluate QoL, the European Organisation for Research and Treatment of Cancer (EORTC) QoL questionnaires (QLQ-C30 and QLQ-CX24) were administered to patients before and 6 months after surgery.ResultsUsing circumference measurement, 9 lymphedemas (31%) were diagnosed: 3 (25%) in the CONS and 6 (37.5%) in the RAD group (P= 0.69). After vulvar surgery, patients in the RAD group reported more fatigue and worsening of physical and role functioning. When comparing both groups, the RAD group had significantly worse parameters in social functioning, fatigue, and dyspnea.ConclusionsLower radicality in inguinofemoral lymphadenectomy shows a trend toward lower morbidity and significantly improves QoL. Multifrequency Bioelectrical Impedance Analysis was tested in these patients as a noninvasive, objective method for lymphedema detection. Detection of lymphedema based on subjective evaluations proved to have an unsatisfactory sensitivity. Less radical surgery showed objectively better results in QoL.

scholarly journals Rates and risk factors for amputation in people with diabetes in Japan: a historical cohort study using a nationwide claims database

2021 ◽  
Vol 14 (1) ◽  
Author(s):  
Masanori Kaneko ◽  
Kazuya Fujihara ◽  
Mayuko Yamada Harada ◽  
Taeko Osawa ◽  
Masahiko Yamamoto ◽  
...  
Keyword(s):  
Lower Limb ◽  
Cox Regression ◽  
Lower Limb Amputation ◽  
Limb Amputation ◽  
Historical Cohort ◽  
Cox Regression Analysis ◽  
Claims Database ◽  
Younger Age

Abstract Background The prevalence of diabetes is rising, and diabetes develops at a younger age in East Asia. Although lower limb amputation negatively affects quality of life and increases the risk of cardiovascular events, little is known about the rates and predictors of amputation among persons with diabetes from young adults to those in the “young-old” category (50–72 y). Methods We analyzed data from a nationwide claims database in Japan accumulated from 2008 to 2016 involving 17,288 people with diabetes aged 18–72 y (mean age 50.2 y, HbA1c 7.2%). Amputation occurrence was determined according to information from the claims database. Cox regression model identified variables related to lower limb amputation. Results The mean follow-up time was 5.3 years, during which time 16 amputations occurred (0.17/1000 person-years). Multivariate Cox regression analysis showed that age (hazard ratio [HR] 1.09 [95% confidence intervals] 1.02–1.16, p = 0.01) and HbA1c (HR 1.46 [1.17–1.81], p < 0.01) were independently associated with amputations. Compared with those aged < 60 years with HbA1c < 8.0%, the HR for amputation was 27.81 (6.54–118.23) in those aged ≥60 years and HbA1c ≥8.0%. Conclusions Age and HbA1c were associated with amputations among diabetic individuals, and the rates of amputation were significantly greater in those ≥60 years old and with HbA1c ≥8.0%.

Application of Sit-To-Stand Assistive Device Based on Hip Support

2021 ◽  
Vol 14 ◽  
Author(s):  
Huaiqiang Zhang ◽  
Qiang Xue ◽  
Shuo Yang ◽  
Tongtong Wang ◽  
Binwei Zhou
Keyword(s):  
Lower Limb ◽  
Independent Living ◽  
Structural Characteristics ◽  
Assistive Devices ◽  
Assistive Device ◽  
Technology Application ◽  
Assist Devices ◽  
Advantages And Disadvantages ◽  
Sit To Stand

Background: Completing the transition from a sitting position to a standing position is a basic skill in people’s daily lives and is crucial for independent living. Lower limb dysfunction will bring many inconveniences into a person’s life and greatly affect their quality of life. Patients with lower limb dysfunction are a specialized group, and nursing problems for this group are becoming increasingly serious. Helping patients with lower limb dysfunction restore their lower limb mobility or assisting them to walk is a social problem necessary to be solved. Objective: : To review the recent sit-to-stand assistive devices based on hip support, classify them systematically and introduce their characteristics, including the mechanisms and the types of patients for which such mechanisms are applicable; to help patients with lower limb dysfunction or doctors (therapists) understand and choose a reasonable sit-to-stand assist device based on hip support. Methods: This paper summarizes literatures and patents about sit-to-stand assistive devices. From the aspects of structural characteristics, drive type and support modes based on the hip and applications situation, the advantages and disadvantages of the typical sit-to-stand assist devices are represented. Results: Current and future development trends on the structural characteristics, drive type and support modes based on the hip and applications situation of sit-to-stand assist devices are discussed to improve the humanization, modularization and applicability of sit-to-stand assist devices. Conclusion: Sit-to-stand assistive devices based on hip support can help patients improve the quality of their life, assist patients carrying out rehabilitation training, and delay the decline of lower limb function. However, the existing sit-to-stand assistive devices based on hip support need further improvement in the aspects of motion mechanism, new technology application and ergonomics design.

Poster 1: Quality of Life and Satisfaction are Strongly Related to Mobility for Patients with a Lower Limb Prosthesis

PM&R ◽  
2018 ◽  
Vol 10 ◽  
pp. S1-S1
Author(s):  
Shane R. Wurdeman ◽  
Phillip M. Stevens ◽  
James H. Campbell
Keyword(s):  
Quality Of Life ◽  
Lower Limb ◽  
Lower Limb Prosthesis ◽  
Limb Prosthesis
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