From the Greek literally means "curved or hooked joints." … Used to describe multiple joint contractures present at birth. [ibis-birthdefects.org]

[…] ar·thro·gry·po·sis mul·'ti·plex con·gen·'i·ta [MIM*108110] limitation of range of joint motion and contractures present at birth, usually involving multiple joints; a syndrome probably of diverse etiology that may result from changes in spinal cord, muscle [medical-dictionary.thefreedictionary.com]

Of the PWS cases, 75% present paternal deletion of segment 15q11q13, 20 to 25% of the PWS present maternal uniparental disomy, and approximately 5% of the PWS present translocation or other structural chromosomal anomaly involving chromosome 15, around [nature.com]

Present On Admission POA Help "Present On Admission" is defined as present at the time the order for inpatient admission occurs — conditions that develop during an outpatient encounter, including emergency department, observation, or outpatient surgery [icd10data.com]

• Chest Deformity

  Lethal form with severe contractures, scoliosis, chest deformities, micrognathia, and death by age of 3 months. Psychomotor retardation may occur. 2. [ibis-birthdefects.org]

  Several types are recognized: myopathic type is relatively rare and is characterized by muscle changes with fixed flexion deformities of the limbs and gross deformities of the chest and spine. [icd10data.com]

  deformities Inguinal hernia Hand and foot Overlapping fingers, tapered fingers, camptodactyly, or syndactyly Clenched fists Positional foot deformities or clubfoot Single or bridged palmar creases Absent or hypoplastic distal flexion creases Extremities [en.wikibooks.org]

• Skin Atrophy

  Also called amyoplasia congenita [əmī′ōplā′zhə]. arthrogryposis multiplex congenita A rare sporadic condition characterised by joint contractures, dislocations, rigid skeletal deformities (e.g., clubfoot or talipes equinovarus), skin atrophy and replacement [medical-dictionary.thefreedictionary.com]

• Low-Set Posteriorly Rotated Ears

  posteriorly rotated ears 0000368 Lymphedema Swelling caused by excess lymph fluid under skin 0001004 Polyhydramnios High levels of amniotic fluid 0001561 Scoliosis Abnormal curving of the spine 0002650 Talipes 0001883 Ulnar deviation of finger Finger [rarediseases.info.nih.gov]

• Polyuria

  The child had polyuria renal tubular acidosis, glucosuria, phosphaturia, hypophosphatemia, hyperchloremia, generalized aminoaciduria and proteinuria. [cags.org.ae]

The final diagnosis or underlying cause may not be known until a child can come to the USA and have a thorough workup by specialists. [adoption.umn.edu]

[…] dislocations, esp knees); - Pena Shokeir syndrome (growth deficiency, unusual facial features, multiple ankylosis; - camptodactyly (when only a finger is involved); - talipes equinovarus; - congenital myasthenia gravis; - whistling face syndrome; - Initial Workup [wheelessonline.com]

B: preoperative radiological workup at the talonavicular dislocation was irreducible.provement in the foot, which is maintained atfter surgical correction. [dokumen.tips]

• Liver Biopsy

  Liver biopsy reported by showed intracellular and intracanalicular cholestasis with paucity of intrahepatic bile ducts. [cags.org.ae]

Treatment  This comprehensive approach is based on a triad of treatment tools:  Firstly, rehabilitation including physiotherapy, manipulation of contractures, and later social and occupational rehabilitation.  Secondly, individually tailored orthotic [de.slideshare.net]

all children walked without crutches or canes; - authors recommend a complete circumferential capsulotomy followed by release of all contracted soft tissues; - references: - The hip in arthrogryposis multiplex congenita. - A plan of management and treatment [wheelessonline.com]

Links: Marfan's syndrome arachnodactyly arthrogryposis General Practice Notebook General Practice Notebook The information provided herein should not be used for diagnosis or treatment of any medical condition. [gpnotebook.co.uk]

Treatment and management varies based on the case, though in general, there is no specific treatment for this syndrome. Description Last Updated: Jan 27, 2018 [diseaseinfosearch.org]

Prognosis - Arthrogryposis multiplex congenita The prognosis is not well defined and therefore it is difficult to search, because of the small number of patients. [checkorphan.org]

Prognosis Prognosis depends on the underlying cause but most have a normal lifespan. If, however, there is a central nervous system problem in addition, about half of patients die in the first year. [patient.info]

The prognosis is highly variable and is dependent on associated anomalies and severity. [radiopaedia.org]

Prognosis The prognosis depends on how severely affected an individual is and how many joints are involved. Some of the more severe cases may be associated with an early death due to sudden respiratory failure and difficulty breathing properly. [encyclopedia.com]

Prognosis The main point to remember is that AMC is not a progressive disorder. [nationwidechildrens.org]

[…] ar·thro·gry·po·sis mul·'ti·plex con·gen·'i·ta [MIM*108110] limitation of range of joint motion and contractures present at birth, usually involving multiple joints; a syndrome probably of diverse etiology that may result from changes in spinal cord, muscle [medical-dictionary.thefreedictionary.com]

Arthrogryposis in children: Etiological assessments and preparation of a protocol for etiological investigations. Arch Pediatr. 2018 Jun 15. [Medline]. Hall JG. [emedicine.medscape.com]

Arthrogryposis (Multiple Congenital Contractures): Diagnostic Approach to Etiology, Classification, Genetics, and General Principles. Eur J Med Genet 2014;57:464-472. Filges I, Hall JG. [rarediseases.org]

FSS No mutation detected – risk for FSS reduced but not eliminated Differential Diagnosis Sheldon-Hall syndrome Distal arthrogryposis type 1 Trismus-pseudocamptodactyly syndrome Congenital contractual arachnodactyly (Beals-Hecht syndrome) Background Epidemiology [arupconsult.com]

Introduction Nonprogressive congenital disorder involving multiple rigid joints (usually symmetric) leading to severe limitation in motion Epidemiology incidence 1:3000 live births Mechanism symmetry of contractures due to immobilization in utero neurogenic [orthobullets.com]

Lethal congenital contracture syndrome (LCCS) and other lethal arthrogryposes in Finland--an epidemiological study. Am J Med Genet A. 2006 Sep 1. 140A(17):1834-9. [Medline]. Lin IW, Chueh HY, Chang SD, Cheng PJ. [emedicine.medscape.com]

Epidemiology The prevalence of arthrogryposis is about 1/3000 [ 3 ]. [ojrd.biomedcentral.com]

Epidemiology It may occur to some extent in 1 in 3,000 to 1 in 5,000 live births. [ 3 ] The condition is usually detected at birth or before by ultrasound examination. It is often secondary to other conditions. [patient.info]

[…] inheritance – ~70% of cases represent new mutations Mutations in MYH3 gene – 93% of cases 2 common missense mutations occur in exon 17 of MYH3 – 72% of FSS cases c.2014C>T (p.R672C) c.2015G>A (p.R672H) No other FSS-associated genes identified to date Pathophysiology [arupconsult.com]

[…] congenital disorder involving multiple rigid joints (usually symmetric) leading to severe limitation in motion Epidemiology incidence 1:3000 live births Mechanism symmetry of contractures due to immobilization in utero neurogenic (90%) myopathic (10%) Pathophysiology [orthobullets.com]

[…] characterized by multiple joint contractures found throughout the body at birth. [1] The designation is currently used in connection with a very heterogeneous series of disorders that all include the common feature of multiple congenital joint contractures. [2] Pathophysiology [emedicine.medscape.com]

[…] associated with congenital Zika virus infection Tests to evaluate arthrogryposis were consistent with a neurogenic pattern, with electromyelographic findings and spinal magnetic resonance imaging suggesting involvement of the lower motor neurones The pathophysiology [bmj.com]

Centers for Disease Control and Prevention. [acog.org]

Arthrogryposis, Congenital multiple arthrogryposis, Fibrous ankylosis of multiple joints, Myodystrophia fetalis deformans, Congenital arthromyodysplasia Symptoms - Arthrogryposis multiplex congenita Causes - Arthrogryposis multiplex congenita Prevention [checkorphan.org]

Prevention Genetic advice may be essential to prevent arthrogryposis. Extrinsically derived contractures have a low recurrence risk but the recurrence risk for intrinsically derived contractures depends on the aetiology. [patient.info]

However, it is necessary to seek treatment to prevent further impediments of the joints. What causes Arthrogryposis Multiplex Congenita? There are many causes of the disease, some of which are inherited. [aanem.org]

… Research on animals has shown that anything which prevents normal joint movement before birth can result in joint contractures. The joint itself may be normal … Arthrogryposis Clinics Avenues, 2006 [Support Groups] A.I. [ibis-birthdefects.org]