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congenital femoral deficiency

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https://www.readbyqxmd.com/read/27826907/the-unstable-knee-in-congenital-limb-deficiency
#1
REVIEW
Gabriel T Mindler, Christof Radler, Rudolf Ganger
PURPOSE: Instability of the knee is a common finding in patients with congenital limb deficiency. The instability can be attributed to soft tissue abnormalities, frontal, sagittal or rotational deformity of the lower limb and bony dysplasia of the patella or of the femoral condyles. In most of the cases, these pathomorphologic changes stay asymptomatic in daily activity. However, instability can appear during deformity correction and bone-lengthening procedures, leading to flexion contracture or subluxation of the knee...
December 2016: Journal of Children's Orthopaedics
https://www.readbyqxmd.com/read/27782934/proximal-femoral-focal-deficiency-associated-with-fibular-duplication-and-diplopodia-and-complete-agenesis-of-the-tibia-a-case-report
#2
Vivek S Chawathe, Anil K Gaur, Badrinath D Athani, Shefali Gupta
A 14-year-old boy reported with congenital deformity of the left lower limb, with gross shortening, hip and knee flexion deformities, ankle with equinus deformity, and polydactyly of the left foot. Radiologic examination showed proximal femoral focal deficiency, double fibula, and duplication of the tarsal bones, and a diagnosis of 'proximal femoral focal deficiency associated with fibular duplication and diplopodia with complete agenesis of tibia' was made. Such association of deformities is very rare and poses difficulties in rehabilitation of the case...
October 25, 2016: Journal of Pediatric Orthopedics. Part B
https://www.readbyqxmd.com/read/27726750/imaging-findings-of-metabolic-bone-disease
#3
Connie Y Chang, Daniel I Rosenthal, Deborah M Mitchell, Atsuhiko Handa, Susan V Kattapuram, Ambrose J Huang
Metabolic bone diseases are a diverse group of diseases that result in abnormalities of (a) bone mass, (b) structure mineral homeostasis, (c) bone turnover, or (d) growth. Osteoporosis, the most common metabolic bone disease, results in generalized loss of bone mass and deterioration in the bone microarchitecture. Impaired chondrocyte development and failure to mineralize growth plate cartilage in rickets lead to widened growth plates and frayed metaphyses at sites of greatest growth. Osteomalacia is the result of impaired mineralization of newly formed osteoid, which leads to characteristic Looser zones...
October 2016: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/27694593/outcomes-following-femoral-lengthening-an-initial-comparison-of-the-precice-intramedullary-lengthening-nail-and-the-lrs-external-fixator-monorail-system
#4
M Laubscher, C Mitchell, A Timms, D Goodier, P Calder
AIMS: Patients undergoing femoral lengthening by external fixation tolerate treatment less well when compared to tibial lengthening. Lengthening of the femur with an intramedullary device may have advantages. PATIENTS AND METHODS: We reviewed all cases of simple femoral lengthening performed at our unit from 2009 to 2014. Cases of nonunions, concurrent deformities, congenital limb deficiencies and lengthening with an unstable hip were excluded, leaving 33 cases (in 22 patients; 11 patients had bilateral procedures) for review...
October 2016: Bone & Joint Journal
https://www.readbyqxmd.com/read/27678508/hip-stability-during-lengthening-in-children-with-congenital-femoral-deficiency
#5
Mark Eidelman, Julio J Jauregui, Shawn C Standard, Dror Paley, John E Herzenberg
BACKGROUND: Congenital femoral deficiency (CFD) is one of the most challenging and complex conditions for limb lengthening. We focused on the problem of hip instability during femoral lengthening because subluxation and dislocation are potentially catastrophic for hip function. METHODS: We assessed for hip stability in 69 children (91 femoral lengthenings) who had CFD Paley type 1a (43 children) and 1b (26 children). The mean age at first lengthening was 6.4 years...
September 27, 2016: International Orthopaedics
https://www.readbyqxmd.com/read/27622419/proximal-femoral-focal-deficiency-of-the-fetus-early-3d-4d-prenatal-ultrasound-diagnosis
#6
Marek J Kudla, Aleksandra Beczkowska-Kielek, Katarzyna Kutta, Justyna Partyka-Lasota
Proximal Femoral Focal Deficiency (PFFD) is a rare congenital syndrome of unknown etiology. Additional disorders can be present up to 70% of PFFD cases. Management (including termination) depends on the severity of the malformation. We present a case of a 32-year-old woman referred for routine ultrasound examination in the 12th week of pregnancy. Detailed 3D/4D evaluation revealed asymmetry of lower limbs and diagnosis of isolated PFFD was established. Parents were fully informed and decided to continue the pregnancy...
September 2016: Medical Ultrasonography
https://www.readbyqxmd.com/read/27227952/clinical-results-and-complications-of-lower-limb-lengthening-for-fibular-hemimelia-a-report-of-eight-cases
#7
Kenichi Mishima, Hiroshi Kitoh, Koji Iwata, Masaki Matsushita, Yoshihiro Nishida, Tadashi Hattori, Naoki Ishiguro
Fibular hemimelia is a rare but the most common congenital long bone deficiency, encompassing a broad range of anomalies from isolated fibular hypoplasia up to substantial femoral and tibial shortening with ankle deformity and foot deficiency. Most cases of fibular hemimelia manifest clinically significant leg length discrepancy (LLD) with time that requires adequate correction by bone lengthening for stable walking. Bone lengthening procedures, especially those for pathological bones, are sometimes associated with severe complications, such as delayed consolidation, fractures, and deformities of the lengthened bones, leading to prolonged healing time and residual LLD at skeletal maturity...
May 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27185866/long-term-glucocorticoid-effect-on-bone-mineral-density-in-patients-with-congenital-adrenal-hyperplasia-due-to-21-hydroxylase-deficiency
#8
Filippo Ceccato, Mattia Barbot, Nora Albiger, Marialuisa Zilio, Pietro De Toni, Giovanni Luisetto, Martina Zaninotto, Nella Augusta Greggio, Marco Boscaro, Carla Scaroni, Valentina Camozzi
INTRODUCTION: Patients with 21-hydroxylase deficiency (21OHD) assume a lifelong glucocorticoid (GC) therapy. Excessive GC treatment increases the risk of osteoporosis and bone fractures, even though the role of substitutive therapy is not fully established: we analyzed the effect of GC dose on bone metabolism and bone mineral density (BMD) over time in patients with 21OHD. METHODS: We studied bone metabolism markers and BMD in 38 adult patients with 21OHD (19-47 years, 24 females and 14 males) and 38 matched healthy control...
August 2016: European Journal of Endocrinology
https://www.readbyqxmd.com/read/26979810/foetal-fibular-hemimelia-with-focal-femoral-deficiency-following-prenatal-misoprostol-use-a-case-report
#9
P Pallavee, Rupal Samal, Jasmina Begum, Seetesh Ghose
Misoprostol is a well known abortifacient. It can cause teratogenicity like Mobius sequence and terminal transverse limb defects. We report a rare case of proximal focal femoral deficiency with fibular hemimelia in a woman who had attempted abortion with self-administered misoprostol and later continued the pregnancy. Though the absolute risk of congenital malformations with its use is low ∼1%, this should be clearly communicated to the women requesting abortion to help them make fully informed reproductive health decisions...
March 16, 2016: Journal of Obstetrics and Gynaecology: the Journal of the Institute of Obstetrics and Gynaecology
https://www.readbyqxmd.com/read/26650581/prophylactic-intramedullary-rodding-following-femoral-lengthening-in-congenital-deficiency-of-the-femur
#10
Amr A Abdelgawad, Julio J Jauregui, Shawn C Standard, Dror Paley, John E Herzenberg
INTRODUCTION: Femoral fracture after lengthening of congenital femoral deficiency (CFD) is a common complication with a high incidence, up to 50%. The purpose of this study is to determine whether prophylactic intramedullary Rush rodding after lengthening for CFD is an effective method to prevent femoral fracture and to assess any complications that may result. METHODS: Forty-five femoral lengthenings (43 CFD patients) were performed using external fixation. At the time of frame removal (except for 3 cases, 8 d later), prophylactic intramedullary Rush rods were inserted...
December 8, 2015: Journal of Pediatric Orthopedics
https://www.readbyqxmd.com/read/26600294/avascular-necrosis-in-the-contralateral-hip-in-patients-with-congenital-femoral-deficiency-a-report-of-3-cases
#11
Timothy S Brown, Robert L Wimberly, John G Birch
BACKGROUND: Congenital femoral deficiency is an uncommon clinical entity. We report 3 patients who developed avascular necrosis of the hip in the long (normal) leg during longitudinal observation and/or treatment of congenital femoral deficiency. METHODS: Patients were identified in limb length discrepancy clinic and their charts were retrospectively reviewed for clinical and radiographic data collection. RESULTS: We describe the occurrence of idiopathic avascular necrosis in the normal limb in patients being followed for limb length discrepancy...
January 2017: Journal of Pediatric Orthopedics
https://www.readbyqxmd.com/read/26437563/-successful-anesthetic-management-of-a-patient-with-critical-bleeding-during-hepatectomy-using-recombinant-activated-factor-vii-and-intraoperative-blood-salvage
#12
Hikaru Yamaguchi, Takashi Suzuki, Yuko Shimura, Satoko Goto, Ken-ichi Takahashi, Hanae Awakura, Tadamichi Someya, Kazuyuki Serada
A 58-year-old 79 kg male with metastatic liver cancer was scheduled for hepatectomy. Preoperative examination did not reveal any hemostatic abnormalities. Nine hours into the surgery, a vascular clip attached to the middle hepatic vein was disconnected and rapid bleeding followed. Unscheduled intraoperative cell salvage was employed. Despite surgical hemostasis as well as transfusion with fresh frozen plasma and platelets, significant oozing persisted for 10 hours, and cumulative blood loss amounted to 30,000 ml...
June 2015: Masui. the Japanese Journal of Anesthesiology
https://www.readbyqxmd.com/read/26408260/prenatal-diagnosis-of-proximal-focal-femoral-deficiency-literature-review-of-prenatal-sonographic-findings
#13
REVIEW
Valentina D'Ambrosio, Gaia Pasquali, Antonia Squarcella, Eleonora Marcoccia, Angela De Filippis, Silvia Gatto, Aliberti Camilla, Antonio Pizzuti, Renato La Torre, Antonella Giancotti
Proximal focal femoral deficiency (PFFD) is a rare musculoskeletal malformation that occurs in 0.11-0.2 per 10,000 live births. This congenital anomaly involves the pelvis and proximal femur with widely variable manifestations, from mild femoral shortening and hypoplasia to the absence of any functional femur and acetabular aplasia. Prenatal diagnosis of PFFD is still a challenge, but early recognition of this malformation could provide useful information to both parents and physicians concerning management and therapeutic planning...
May 2016: Journal of Clinical Ultrasound: JCU
https://www.readbyqxmd.com/read/26333739/lengthening-in-congenital-femoral-deficiency-a-comparison-of-circular-external-fixation-and-a-motorized-intramedullary-nail
#14
COMPARATIVE STUDY
Sheena R Black, Michael S Kwon, Alexander M Cherkashin, Mikhail L Samchukov, John G Birch, Chan-Hee Jo
BACKGROUND: Circular external fixation for limb-lengthening is associated with frequent and numerous complications. Intramedullary lengthening devices represent a potential advance in limb-lengthening. The purpose of this study was to compare the outcomes of femoral lengthening in pediatric patients treated by either circular external fixation or a motorized intramedullary nail. METHODS: All patients with a diagnosis of congenital femoral deficiency who had undergone femoral lengthening with either circular external fixation or a motorized intramedullary nail were identified...
September 2, 2015: Journal of Bone and Joint Surgery. American Volume
https://www.readbyqxmd.com/read/26251945/congenital-absence-of-the-anterior-cruciate-ligament
#15
Jothi Murali, Keith Monchik, Paul Fadale
The incidence of congenital absence of the anterior cruciate ligament (ACL) is extremely low. Congenital ACL absence has most often been found in association with conditions such as knee dislocation, knee dysplasia, proximal focal femoral deficiency, and fibular hemimelia. We report on the incidental finding of ACL aplasia in a patient with a medial meniscal tear and history of leg-length discrepancy. As has been found in prior case studies, this patient had hypertrophy of the meniscofemoral ligament of Humphrey, which likely lent her stability...
August 2015: American Journal of Orthopedics
https://www.readbyqxmd.com/read/26216721/lubricin-restoration-in-a-mouse-model-of-congenital-deficiency
#16
Adele Hill, Kimberly A Waller, Yajun Cui, Justin M Allen, Patrick Smits, Ling X Zhang, Ugur M Ayturk, Steven Hann, Samantha G Lessard, David Zurakowski, Matthew L Warman, Gregory D Jay
OBJECTIVE: Congenital deficiency of the principal boundary lubricant in cartilage (i.e., lubricin, encoded by the gene PRG4) increases joint friction and causes progressive joint failure. This study was undertaken to determine whether restoring lubricin expression in a mouse model would prevent, delay, or reverse the disease process caused by congenital deficiency. METHODS: Using genetically engineered lubricin-deficient mice, we restored gene function before conception or at ages 3 weeks, 2 months, or 6 months after birth...
November 2015: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/26201943/bone-involvement-in-males-with-kallmann-disease
#17
Giovanni Iolascon, Laura Frizzi, Massimiliano Bianco, Francesca Gimigliano, Vincenzo Palumbo, Antonia Maria Sinisi, Antonio Agostino Sinisi
BACKGROUND: Kallmann syndrome (KS) is a rare genetic condition characterized by congenital early-onset hypogonadotropic hypogonadism and anosmia or hyposmia. Male subjects are more frequently affected and present absent/delayed puberty, low testosterone levels with higher risk for osteoporosis. Therefore, to maintain normal levels of sex steroids and prevent bone loss, male KS needs life-long hormonal replacement therapy (HRT). AIMS: The objective of our study is to assess bone involvement in subjects with KS currently treated with HRT...
October 2015: Aging Clinical and Experimental Research
https://www.readbyqxmd.com/read/26194561/lengthening-with-external-fixation-is-effective-in-congenital-femoral-deficiency
#18
Daniel E Prince, John E Herzenberg, Shawn C Standard, Dror Paley
BACKGROUND: Treatment of congenital femoral deficiency is a complex, multistage protocol and a variety of strategies have been devised to address joint instability, limb length inequality, and deformities. Despite being an important part of the algorithmic approach to the overall treatment of patients with congenital femoral deficiency, a reproducible, safe, and functional treatment for femoral length discrepancy in patients with mild and moderate congenital femoral deficiency has not been reported...
October 2015: Clinical Orthopaedics and related Research
https://www.readbyqxmd.com/read/26172360/common-patterns-of-congenital-lower-extremity-shortening-diagnosis-classification-and-follow-up
#19
REVIEW
Maria A Bedoya, Nancy A Chauvin, Diego Jaramillo, Richard Davidson, B David Horn, Victor Ho-Fung
Congenital lower limb shortening is a group of relatively rare, heterogeneous disorders. Proximal focal femoral deficiency (PFFD) and fibular hemimelia (FH) are the most common pathologic entities in this disease spectrum. PFFD is characterized by variable degrees of shortening or absence of the femoral head, with associated dysplasia of the acetabulum and femoral shaft. FH ranges from mild hypoplasia to complete absence of the fibula with variable shortening of the tibia. The development of the lower limb requires complex and precise gene interactions...
July 2015: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/25920687/imaging-features-of-lower-limb-malformations-above-the-foot
#20
REVIEW
A Bergère, E Amzallag-Bellenger, G Lefebvre, A Dieux-Coeslier, A Mezel, B Herbaux, N Boutry
Lower limb malformations are generally isolated or sporadic events. However, they are sometimes associated with other anomalies of the bones and/or viscera in patients with constitutional syndromes or disorders of the skeleton. This paper reviews the main imaging features of these abnormalities, which generally exhibit a broad spectrum. This paper focuses on several different bone malformations: proximal focal femoral deficiency, congenital short femur and femoral duplication for the femur, tibial hemimelia (aplasia/hypoplasia of the tibia) and congenital bowing for the tibia, fibular hemimelia (aplasia/hypoplasia) for the fibula, and aplasia, hypoplasia and congenital dislocation for the patella...
September 2015: Diagnostic and Interventional Imaging
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