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#1
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10-13-2014, 10:51 PM
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Arthrogryposis Multiplex Congenita
I hope it's not callous, but I hope the baby died to avoid suffering, unless they somehow fixed all of it. |
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#2
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10-13-2014, 11:12 PM
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Re: Arthrogryposis Multiplex Congenita
some info to go with your image baby did die few hours after photo taken Congenital contractures, fetal akinesia sequence, Pena-Shokeir syndrome. Definition: This heterogeneous set of conditions shares limitation of movements and joint ankylosis as main findings. Figure 1: Newborn with Arthrogryposis Multiplex Congenita. Note the pronounced anomalies of the limbs that are very rigid. Also note the linear fingers with lack of visible flexion joints. The newborn died of respiratory failure a few hours after the images was obtained. (Image courtesy of the mother) Incidence: 1-3:10,000 Etiology: Possibly autosomal dominant. Pathogenesis: Arthrogryposis results from decreased in-utero motion, either from neural , muscular, connective tissue, infectious origin. Diagnosis: Although the anomalies are obvious when recognized and in particular when the baby is born, the prenatal diagnosis may be challenging when fluid is decreased, and the abnormal limb position appears attributable to the oligohydramnios. Some forms are associated with polyhydramnios and then the abnormal limb position (knocked knee), genu recurvatum, clubfeet and hand) make the diagnosis easy. Polyhydramnios is often a manifestation of decreased swallowing, which may be part of the same pathogenesis as the arthrogryposis itself (muscular or neuronal deficit). An increased in nuchal lucency as well as the characteristic decreased movement can also be seen in the first trimester. |