Amyoplasia congenita is a diagnostic subgroup of children with arthrogryposis multiplex congenita AMC. AMC is a relatively rare syndrome characterized by multiple joint contractures at birth. Amyoplasia congenita is the most common type of this syndrome with an occurrence rate of 1 in 10, live births, and mainly refers to the disorders with limb involvement. In this report, the author presents a premature baby with amyoplasia congenita, whose hips showed flexion, abduction, and external rotation contractures.
|Country:||Central African Republic|
|Published (Last):||3 May 2019|
|PDF File Size:||3.80 Mb|
|ePub File Size:||3.10 Mb|
|Price:||Free* [*Free Regsitration Required]|
If your institution subscribes to this resource, and you don't have a MyAccess Profile, please contact your library's reference desk for information on how to gain access to this resource from off-campus. Rare congenital disorder constituting about one third of cases of Arthrogryposis Multiplex Congenita characterized by multiple contractures of joints. Congenital disorder that may result from an intrauterine vascular accident affecting the fetal spinal cord. Sporadic cases. Incidence is approximately , live births.
Higher incidence is found in twins. Two forms have been described:. Neuropathic form in which anterior horn cells are reduced in number, pyramidal tract and motor roots are demyelinated, and axons in peripheral nerves are reduced. Myopathic form in which joint deformities result from lack of movements in utero. Based on clinical findings at birth in a child with contracture of numerous joints in a flexed position arthrogryposis , hypoplasia of the attached musculature, and development of multiple pterygia in affected joints.
In approximately two thirds of patients, all four limbs are affected; in approximately one fourth of patients, the lower extremities are more severely affected than the arms; and in the remaining cases, the arms are more severely affected than the lower extremities.
The term amyoplasia refers to replacement of muscles by fibrous tissue in different areas. Intelligence in these patients is normal. Common features involve the head and neck low-set ears, ptosis, limited eye movements laterally and upward, abnormal retinal pigmentation, flattened nose, round facies, frontal midline capillary hemangioma, micrognathia, cleft palate , skeleton scoliosis, dislocated hips, internally rotated and adducted shoulders, fixed extended elbows, wrist deformities consisting of flexion and ulnar deviation, talipes varus, syndactyly, camptodactyly, amputation of digits , gastrointestinal tract gastroschisis, intestinal atresia, hypertrophic pyloric stenosis, gastric ulcers , and chest hypoplastic lungs, diaphragmatic hernia, hydrothorax.
Lymphedema has been observed. Evaluate respiratory function clinical, chest radiographs, pulmonary function tests if possible, arterial blood gases analysis, CT scans.
Assess for difficult airway management micrognathia, cleft palate. Tracheal intubation may be difficult and may require adapted anesthetic management.
Spontaneous ventilation should be maintained until the airway is secured. If significant, pleural effusions should be drained preoperatively.
Postoperative mechanical ventilatory support may be necessary and should be arranged beforehand. Careful intraoperative positioning is mandatory and can be challenging secondary to limited joint mobility. Complications resulting from positioning, such as pressure sores and fractures, have been described. Venous access can be difficult with all the contractures.
Avoid bag-mask ventilation in the presence of diaphragmatic hernia. Muscle deficiency and respiratory function may require lower doses of muscle relaxants. Opioids should be used judiciously.
No relation between malignant hyperthermia and arthrogryposis syndromes has been demonstrated. However, hyperpyrexia and hypermetabolism have been described in association with volatile anesthetics, although these reactions were thought to be distinct from malignant hyperthermia.
See Arthrogryposis Multiplex Congenita for a summary. This div only appears when the trigger link is hovered over. Otherwise it is hidden from view. Forgot Username? About MyAccess If your institution subscribes to this resource, and you don't have a MyAccess Profile, please contact your library's reference desk for information on how to gain access to this resource from off-campus.
Sign in via OpenAthens. Sign in via Shibboleth. AccessBiomedical Science. AccessEmergency Medicine. Case Files Collection. Clinical Sports Medicine Collection.
Davis AT Collection. Davis PT Collection. Murtagh Collection. About Search. Enable Autosuggest. Previous Chapter. Next Chapter. Bruno Bissonnette, et al. Accessed June 05, MLA Citation. Download citation file: RIS Zotero. Reference Manager. Autosuggest Results. Sign In. Username Error: Please enter User Name. Password Error: Please enter Password. Forgot Password? What is MyAccess? Best Value. View All Subscription Options. Pop-up div Successfully Displayed This div only appears when the trigger link is hovered over.
AMC: amyoplasia and distal arthrogryposis
This is a rare disorder occurring in 1 out of every 3, live births. The incidence of true amyoplasia occurs in 1 out of every 10, live births. Arthrogryposis, also called arthrogryposis multiplex congenita AMC , involves a variety of non-progressive conditions that are characterized by multiple joint contractures stiffness and involves muscle weakness found throughout the body at birth. This disorder should be considered a symptom complex or a descriptive term rather than a disease and an underlying cause should be sought when possible. The most common type of arthrogryposis is called amyoplasia in which the hands, wrists, elbows, shoulders, hips, feet and knees are affected.
Amyoplasia Congenita of the Lower Extremity: Report in a Premature Baby
If your institution subscribes to this resource, and you don't have a MyAccess Profile, please contact your library's reference desk for information on how to gain access to this resource from off-campus. Rare congenital disorder constituting about one third of cases of Arthrogryposis Multiplex Congenita characterized by multiple contractures of joints. Congenital disorder that may result from an intrauterine vascular accident affecting the fetal spinal cord. Sporadic cases.