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Congenital Hip Dysplasia

Annick Moens, Karen van Hoeve, Evelien Humblet, Jean-François Rahier, Peter Bossuyt, Sophie Dewit, Denis Franchimont, Elisabeth Macken, Jochen Nijs, Annelies Posen, Beatrijs Strubbe, Anneleen Van Hootegem, Wouter Van Moerkercke, Séverine Vermeire, Marc Ferrante
Background and Aims: Vedolizumab is an IgG1 anti-α4β7 integrin antibody approved for the treatment of inflammatory bowel diseases [IBD], but without clear safety data during conception, pregnancy and nursing. Animal studies showed that mucosal vascular addressin cell adhesion molecule 1 [MAdCAM-1] is expressed by maternal vessels in the placenta and recruits α4β7-expressing cells that are considered important for maternal/fetal tolerance. Blocking this interaction by vedolizumab might affect this process...
October 3, 2018: Journal of Crohn's & Colitis
Ayman H Jawadi, Anwar Wakeel, Waleed Tamimi, A Nasr, Zafar Iqbal, Abdullah Mashhour, Mohamed A Fattah, Nawaf Alkhanein, Ahmad S Abu Jaffal
Developmental dysplasia of the hip (DDH) is a congenital condition characterized by abnormality in acetabulum size and/or shape. The incidence rate of DDH differs between different populations with risk factors including positive family history, breech presentation, sex, firstborn status, side of the hip, mode of delivery and oligohydramnios. It is recognized that DDH has a genetic component that exhibit autosomal dominant patterns. Many candidate genes have been studied and found to be associated with the disease; most of them are normally involved in cartilage development and joint metabolism...
September 2018: Journal of Genetics
Luisella Pedrotti, Barbara Bertani, Gabriella Tuvo, Redento Mora, Mario Mosconi, Federica De Rosa
A 4 months and half female child come to our attention for congenital dislocation of the left hip, previously treated in another hospital with abduction bracing, without satisfactory results. After progressive longitudinal bilateral traction, closed reduction under general anesthesia was performed and a spica cast was applied in the so-called human position. The patients remained in the spica cast for 6 weeks and then the plaster cast was renewed in narcosis for another 6 weeks. Once the second cast has been removed left femoral nerve palsy was detected...
September 26, 2018: La Pediatria Medica e Chirurgica: Medical and Surgical Pediatrics
Frank A Segreto, Dennis Vasquez-Montes, Avery E Brown, Cole Bortz, Samantha R Horn, Bassel G Diebo, Peter L Zhou, Shaleen Vira, Joseph F Baker, Anthony M Petrizzo, Renaud Lafage, Virginie Lafage, Thomas J Errico, Peter G Passias
Introduction: Early Onset and Adolescent Idiopathic Scoliosis, relatively common diagnoses (∼3% general population), have been associated with developmental dysplasia of the hip (DDH); a more rare spectrum of anomalies related to the abnormal development of acetabulum, proximal femur, and hip joint. To the best of our knowledge, no high powered investigations have been performed in an attempt to assess incidence and associated risks of DDH in scoliosis patients. Methods: The KID database was queried for ICD-9 codes from 2003 to 2012 pertaining to EOS (Congenital and Idiopathic <10y/o) and AIS patients...
September 2018: Journal of Orthopaedics
R Placzek, M Gathen, S Koob, C Jacobs, M M Ploeger
OBJECTIVE: Growth disorders (e. g. caused by congenital hip dislocation, Perthes disease or bacterial coxitis) often lead to an infantile deformity of the proximal femur with a shortened femoral neck and displaced grater trochanter. In 1988, Morscher and Buess described a femoral neck lenghtening osteotomy for treatment of adults. For the first time, we show a modification of this osteotomy for children and adolescents with a locking plate system. The aim is to restore the normal anatomy of the femoral neck and biomechanics of the proximal femur...
October 2018: Operative Orthopädie und Traumatologie
Yves Salentiny, Lukas Zwicky, Peter E Ochsner, Martin Clauss
INTRODUCTION: Total hip arthroplasty in patients with altered anatomy of the hip and femur, such as in congenital dysplasia of the hip, is challenging and often requires specially designed stems. Müller straight stems have shown excellent long-term results; however, long-term data on the analogous cemented Müller CDH stem are still missing. The aim of this study was to analyze long-term survival, identify potential risk factors for aseptic loosening, and analyze radiological outcome of the cemented Müller CDH stems...
October 2018: Archives of Orthopaedic and Trauma Surgery
Jun Chen, Wen-Bing Zhang, Jin-Zhou He, Run Zhang, Yin-Qiang Cao, Xing Liu
Developmental dysplasia of the hip (DDH) is one of the most common congenital disorders in childhood. Its diverse pathological changes require different treatments and result in different outcomes. Although many studies have been conducted on DDH, some special pathology is still unrecognized. We here presented a rare case of a one-year and eleven-month old girl with DDH; a half-free intra-articular osteocartilaginous tissue was found in her right hip joint. X-ray, computer assisted tomography (CT) and magnetic resonance imaging (MRI) were performed to evaluate the pathological changes...
August 2018: Chinese Journal of Traumatology, Zhonghua Chuang Shang za Zhi
Stephan Arlt, Hansrudi Noser, Andreas Wienke, Florian Radetzki, Gunther Olaf Hofmann, Thomas Mendel
BACKGROUND: Acetabular fracture surgery is directed toward anatomical reduction and stable fixation to allow for the early functional rehabilitation of an injured hip joint. Recent biomechanical investigations have shown the superiority of using an additional screw in the infraacetabular (IA) region, thereby transfixing the separated columns to strengthen the construct by closing the periacetabular fixation frame. However, the inter-individual existence and variance concerning secure IA screw corridors are poorly understood...
May 21, 2018: Journal of Orthopaedic Surgery and Research
Jung-Keun Ko, Dirga Kumar Lamichhane, Hwan-Cheol Kim, Jong-Han Leem
Little information is available on the prevalences of birth defects in Korea. The aims of this study were to estimate recent prevalences of selected birth defects and to analyze the prevalence trends of these defects during the period from 2008 to 2014. Prevalences were calculated for 69 major birth defects using health insurance claim data obtained from the Korea National Health Insurance Service (NHIS). Prevalence rate ratios were calculated using Poisson regression to analyze trends over the 7-year study period...
May 5, 2018: International Journal of Environmental Research and Public Health
G D'Amours, F Lopes, J Gauthier, V Saillour, C Nassif, R Wynn, N Alos, T Leblanc, Y Capri, S Nizard, E Lemyre, J L Michaud, V-A Pelletier, Y D Pastore, J-F Soucy
Inherited bone marrow failure syndromes (IBMFS) are caused by mutations in genes involved in genomic stability. Although they may be recognized by the association of typical clinical features, variable penetrance and expressivity are common, and clinical diagnosis is often challenging. DNAJC21, which is involved in ribosome biogenesis, was recently linked to bone marrow failure. However, the specific phenotype and natural history remain to be defined. We correlate molecular data, phenotype, and clinical history of 5 unreported affected children and all individuals reported in the literature...
August 2018: Clinical Genetics
Amanda T Whitaker, James Kasser, Young-Jo Kim
RATIONALE: The sciatic nerve runs a predictable course combining L4-S3 nerve roots through the true pelvis and under the greater sciatic notch. There are reports of bony protuberances from the sacrum and ilium in cases of spinal dysraphism; however advanced imaging, treatment, or outcomes are not described. There are no cases with associated fibular hemimelia in the current literature. PATIENT CONCERNS: This is a 4-year-old girl with tethered cord, acetabular dysplasia with hip subluxation, congenital short femur, anterior cruciate ligament (ACL) deficiency, and fibular hemimelia with her sciatic nerve coursing through the ilium...
March 2018: Medicine (Baltimore)
Lun-Qing Zhu, Guang-Hao Su, Jin Dai, Wen-Yan Zhang, Chun-Hua Yin, Fu-Yong Zhang, Zhen-Hua Zhu, Zhi-Xiong Guo, Jian-Feng Fang, Cheng-da Zou, Xing-Guang Chen, Ya Zhang, Cai-Ying Xu, Yun-Fang Zhen, Xiao-Dong Wang
Developmental dysplasia of the hip (DDH) is a common congenital malformation characterized by mismatch in shape between the femoral head and acetabulum, and leads to hip dysplasia. To date, the pathogenesis of DDH is poorly understood and may involve multiple factors, including genetic predisposition. However, comprehensive genetic analysis has not been applied to investigate a genetic component of DDH. In the present study, 10 pairs of healthy fathers and DDH daughters were enrolled to identify genetic hallmarks of DDH using high throughput whole genome sequencing...
February 25, 2018: Genomics
Matthew Sloan, Atul F Kamath
Colonna capsular arthroplasty represents an option for the management of chronic hip dislocation in young patients with dysplasia. In the appropriate patient, modern capsular arthroplasty procedures may provide an opportunity for hip preservation in patients with preserved femoral head cartilage and not appropriate for total hip arthroplasty. Here, we review our experience with surgical dislocation of the hip and capsular arthroplasty in a 27-year-old female with congenital hip dysplasia and chronic superior hip dislocation...
January 2018: Journal of Hip Preservation Surgery
Ata Can, Ilker A Sarikaya, Necip S Yontar, Ayse O Erdogan, Baris Gorgun, Fahri Erdogan
BACKGROUND: We argue that 1-stage bilaterally total hip arthroplasty (THA) could be acceptable in bilateral coxarthrosis because of high-riding developmental dysplasia of the hip (DDH). METHODS: Sixty-nine cases (51 patients) of high-riding DDH in patients who underwent THA from 2010 to 2013 were reviewed. Patients were divided into 2 groups: unilateral (group 1) and 1-stage bilateral surgery (group 2). The clinical measurements were the visual analog scale and Harris Hip Score...
May 2018: Journal of Arthroplasty
C Glorion
Surgical reduction of congenital hip dislocation is technically challenging. In our practice, surgical reduction is usually reserved for patients who have failed non-operative treatment, which is the first-line strategy. However, primary surgery may be indicated if the dislocation is diagnosed late and can be performed until 8 years of age. The reduction step is crucial. It starts with painstaking exposure of the capsule. Identifying the lower part of the acetabulum is the key to accurate repositioning of the epiphysis...
February 2018: Orthopaedics & Traumatology, Surgery & Research: OTSR
Abayneh Girma Demisse, Ashenafi Berhanu, Temesgen Tadesse
BACKGROUND: Prune belly syndrome is a rare congenital malformation of unknown etiology, with the following triad of findings: abdominal muscle wall weakness, undescended testes, and urinary tract abnormalities. In most cases, detection of prune belly syndrome occurs during neonatal or infancy period. In this case report, we describe a 12-year-old boy from Ethiopia with the triad of findings of prune belly syndrome along with skeletal malformations. We are unaware of any previous report of prune belly syndrome in Ethiopia...
December 4, 2017: Journal of Medical Case Reports
Shin-Young Yim, Kihong Chang, Ah-Reum Ahn, Eun Ji Park, Jongwoo Kim
Congenital muscular torticollis (CMT) is known to concur with some conditions such as developmental dysplasia of the hip or brachial plexus injury, which gives us some insights for pathogenesis of CMT. Although clavicular fracture is the most common fracture in newborns, little is known about concurrence of CMT and clavicular fracture. Our clinical experience led us to realize that concurrence of CMT and clavicular fracture tended to occur on the contralateral side for each other rather than the ipsilateral side...
May 2018: American Journal of Physical Medicine & Rehabilitation
Charlotte L Williams, Susie Weller, Lisa Roberts, Isabel Reading, Andrew Cook, Louisa Little, Wendy Wood, Louise Stanton, Andreas Roposch, Nicholas Mp Clarke
BACKGROUND: Developmental dysplasia of the hip (DDH) is a very common congenital disorder, and late-presenting cases often require surgical treatment. Surgical reduction of the hip may be complicated by avascular necrosis (AVN), which occurs as a result of interruption to the femoral head blood supply during treatment and can result in long-term problems. Some surgeons delay surgical treatment until the ossific nucleus (ON) has developed, whereas others believe that the earlier the reduction is performed, the better the result...
October 2017: Health Technology Assessment: HTA
Tamas Halasi, Declan Reidy, Shane Guerin
INTRODUCTION: The Smith-Petersen vitallium mold arthroplasty was a real landmark in arthroplasty surgery as this was the first technique which produced predictable and satisfactory results. CASE REPORT: We present the longest known follow-up of any hip arthroplasty in literature. The arthroplasty was performed in 1949 in London on a 30-years-old female patient with congenital hip dysplasia, and it was revised in 2014 after 65 years. CONCLUSION: Total hip arthroplasties nowadays give better functional results, but the fact that the patient got 65 years of the relatively good function is noteworthy and is a tribute to Dr...
May 2017: Journal of Orthopaedic Case Reports
Mathias Rathe, Michael Boe Møller, Pernille Wied Greisen, Niels Fisker
The congenital dyserythropoietic anemias (CDAs) are a group of rare inherited blood disorders characterized by ineffective erythropoiesis as the principal cause of anemia. We present a child with CDA 1b-the rarest and least well-described type-due to a mutation in the C15orf41 gene. The patient presented with severe in utero and neonatal manifestations, typical peripheral limb anomalies as well as rarely reported cardiac manifestations, visual impairment, short stature, and hip dysplasia. Anemia was complicated by iron overload and pronounced extra medullary erythropoiesis leading to skull deformities...
March 2018: Pediatric Blood & Cancer
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