ARTROGRIPOSE MLTIPLA CONGNITA PDF

Arthrogryposis multiplex congenita is a clinical or imaging descriptor that denotes congenital non-progressive joint contractures involving two or more body regions. Mostly reported in individuals of Asian, African and European descent with equal incidence in males and females It is thought to occur in approximately , live births 6,8. It can result from a number of pathologies.

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CiteScore measures average citations received per document published. Read more. SRJ is a prestige metric based on the idea that not all citations are the same. SJR uses a similar algorithm as the Google page rank; it provides a quantitative and qualitative measure of the journal's impact.

SNIP measures contextual citation impact by wighting citations based on the total number of citations in a subject field. This article presents the clinical case of 21 years old female patient reporting history of Arthrogryposis Multiplex Congenita AMC. The extraoral examination disclosed clinical AMC pathognomonic signs. The intraoral examination revealed slight compression of the maxillary arch, conical upper lateral incisors, absence of 17 and 35, 53 persistence, upper cuspids inclusion and agenesis of third molars.

The extraction of 23 was planned due to its maxillary position. Relatively to the 13, the orthodontical traction with a microimplant was the option. Treatment planning established orthodontics to restore esthetics and function followed by rehabilitation with implants. Arthrogryposis Multiplex Congenita AMC is a rare congenital disorder, 1 affecting 1 in 2—4 to 1 in 12, 5 newborns.

It involves the presence of multiple non-progressive, 1,3 symmetric joint contractures, 1,3,4 sometimes associated with muscle weakness and fibrosis. This disease can present an isolated form or it can also be associated with other congenital anomalies, as part of a syndrome, with or without central nervous system involvement.

This condition's etiology is considered multifactorial and may be presented as a monogenic disease autosomal recessive transmission, autosomal dominant or associated with the X chromosome , as a chromosomal disorder or as a congenital malformation involving various organs. In the recognition of early clinical signs of AMC, in the last months of pregnancy, decreased fetal movement fetal akinesia is considered a common denominator to all AMC affected individuals, conditioning a variety of minor fetal deformities.

It is important to notice the absence of movement, essential for joints and periarticular tissues development, leads to an increase of connective tissue around the immobilized joint with rippling of the skin covering the joint, muscle atrophy and changes in the joint surface depending on the position of the immobilization.

This paper focused on the presentation of a clinical case of AMC, emphasizing oral and craniofacial abnormalities and proposing a treatment approach. A female patient, 21 years old, attended a dental appointment to assess orthodontic treatment need, referring the closure of existing dental gaps in the upper anterior arch as a priority.

During the anamnesis, the patient reported an AMC history, diagnosed since childhood. The extraoral clinical examination Fig. The analysis stressed a thin hypertonic upper lip and a low smile line TMJ clinical examination by palpation, auscultation and mandibular kinetics evaluation discarded clinical signs of temporomandibular disorder.

The intraoral clinical examination Fig. In order to assess dental arch discrepancy, Bolton analysis 12 revealed a discrepancy with excess on lower anterior arch Fig. Facial appearance at rest — A right side, B left side, C front, D smile.

Intraoral photographic status A upper occlusal, B lower occlusal, C right side, D front side, E left side. Panoramic X-ray Fig. Diagnostic records A panoramic X-ray, B periapical radiographs, C cephalometric radiography, D ricketts cephalometric tracing. Ricketts cephalometric analysis Fig. Functional analysis according to Multifunction System MFS classification 13 showed type 1 nasal collapse narrow nostrils without collapse , type 2 adenoids slightly convex , type 2 tonsils appear slightly , normal swallowing, nasal breathing, type 1 tongue mobility level tongue touches the palate.

In order to improve the perception of cuspid location, a computed tomography CT was requested Fig. Root shortening is a rare finding occurring in about 1. In the present study the Lind method 15 was the one selected for the assessment. According to it, the interproximal concavities between root and crown correspond to x and y points, being m the midpoint of x - y segment. The relative root length is calculated from the apex to m point and the relative coronal length is the distance between m point and the midpoint of the incisal edge.

According to Jakobsson et al. In AMC cases, despite the limitations caused by dental morphology, orthodontic treatment can be considered in order to enable opening spaces for rehabilitation with dental implants in an attempt to restore the appearance and function.

Partial fixed appliances may be used to avoid compromising periodontal structures of adjacent teeth while opening spaces for rehabilitation. However, partial appliances do not fix all dental positions and may pledge reaching goals related to the establishment of an ideal functional occlusion. Relative position and possibility of orthodontic traction of impacted teeth should be carefully considered prior to treatment.

Bringing the impacted canine into a normal position is important in achieving a functional occlusion and final esthetics of orthodontic treatment, but factors such as height of the impacted canine, angulation of the long axis to the upper midline, canine mesiodistal position of the tip relative to the midline, adjacent incisors and the anteroposterior position of the canine root apex should also be considered.

In this particular case, the left canine presented a horizontal position and was in proximity with the roots of adjacent teeth. Therefore, extraction seems to be the most suitable option.

As for the right canine, a more conservative approach would be adequate and it seems that the use of a mini-screw as a traction auxiliary device would be indicated to be possible to traction using a mini-screw Fig. Representation of the microimplant location. This may avoid loss of anchorage and outline periodontal limitations. Patients should be informed about the limitations of these procedures, such as, tooth ankylosis, possible compromise of adjacent teeth during root repositioning and the anatomy and position of the remaining teeth.

The decision of tooth traction or extraction should be decided with the patient. Rehabilitation phase can be done based on implant supported rehabilitation or using a removable partial acrylic denture. As skeletal prosthesis require too much support on the abutment teeth, this option should be discarded. In order to improve dental esthetics of conoid teeth, when present, esthetic restorations with composite resin may be held due to the already mentioned limited prognosis offered by fixed prosthesis.

In conclusion, a multidisciplinary approach is essential to solve this type of cases, covering areas such as genetics, oral surgery, orthodontics, implantology and esthetic dentistry. Comprehensive treatment must be individualized and adjusted to patient needs and, when required, subjected to adjustments in face of individual clinical response.

Especially in orthodontic patients, this problem is emphasized and gets critical dimension, due to the high rate of root resorption in patients with previous root shortenings.

The authors declare that no experiments were performed on humans or animals for this study. The authors declare that they have followed the protocols of their work center on the publication of patient data. The authors have obtained the written informed consent of the patients or subjects mentioned in the article. The corresponding author is in possession of this document. The authors have no conflicts of interest to declare.

Indexed in: Scopus See more Follow us:. Discontinued publication For more information click here. Previous article Next article. Issue 3. Pages July - September Artrogryposis multiplex congenita associated with intraoral changes — Multidisciplinary approach.

Download PDF. Helena Maltez Rodrigues. Corresponding author. This item has received. Under a Creative Commons license. Article information. Show more Show less. Introduction Arthrogryposis Multiplex Congenita AMC is a rare congenital disorder, 1 affecting 1 in 2—4 to 1 in 12, 5 newborns. Case report A female patient, 21 years old, attended a dental appointment to assess orthodontic treatment need, referring the closure of existing dental gaps in the upper anterior arch as a priority.

Alfonso, J. Paez, J. Int Pediatr, 15 , pp. Mielnik-Blaszczak, M. Arthrogryposis multiplex congenita AMC — case report. Binkiewicz-Glinska, A. Sobierajska-Rek, S. Bakula, J. Wierzba, K. Drewek, I. Kowalski, et al. Arthrogryposis in infancy, multidisciplinary approach: case report.

BMC Pediatr, 13 , pp. Bamshad, A. Van Heest, D. J Bone Joint Surg Am, 91 , pp. Hoff, M. Loane, N. Gilhus, S. Rasmussen, A. Arthrogryposis multiplex congenita: etiology, genetics, classification, diagnostic approach, and general aspects. J Pediatr Orthop B, 6 , pp. Alves, L. Zhao, P. Patel, A.

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Arthrogryposis

Skip to search form Skip to main content You are currently offline. Some features of the site may not work correctly. A artrogripose multipla congenita classica e caracterizadapor contraturas articulares incapacitantesdiagnosticadas ao nascimento. Descreve-seum caso de amioplasia, a forma mais frequentedessa doenca, coexistente com puberdade isossexualprecoce que se iniciou em uma menina comsete anos e dez meses de idade. A associacao deamioplasia com puberdade precoce nao foi previamenterelatada. Embora essa associacao possaconstituir um fenomeno casual, descreve-se o estudode caso por sua raridade.

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Artrogripose múltipla congênita coexistente com puberdade precoce idiopática isossexual

Arthrogryposis multiplex congenita AMC , or simply arthrogryposis , describes congenital joint contracture in two or more areas of the body. Children born with one or more joint contractures have abnormal fibrosis of the muscle tissue causing muscle shortening, and therefore are unable to perform active extension and flexion in the affected joint or joints. AMC has been divided into three groups: amyoplasia , distal arthrogryposis, and syndromic. Amyoplasia is characterized by severe joint contractures and muscle weakness.

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