Journal List > Korean J Obstet Gynecol > v.55(9) > 1088515

Kim, Oh, and Hong: A case of arthrogryposis multiplex congenita associated with maternal septate uterus

Abstract

Arthrogryposis multiplex congenita (AMC) is a congenital disorder showing multiple joint contractures. Although there are characteristic features in morphology, it is difficult to diagnose prenatally by ultrasonography. The causes of AMC include abnormalities of central nervous system, myopathies, connective tissue disorders, and intrauterine environmental factors such as oligohydramnios or uterine anomalies. We describe a case of prenatally diagnosed and postnatally evaluated AMC associated with maternal septate uterus.

Figures and Tables

Fig. 1
Coronal scan of gravid uterus shows that a thick septum is extended to lower uterine cavity. The head and upper portion of the fetus is located in the right uterine cavity and lower portion in the left cavity (arrow, septum).
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Fig. 2
3-D scan image of multiple joint contractures (S, setum; arrow, wrist deformity and club foot).
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Fig. 3
Gross appearances of the fetus with multiple joint contractures corresponding arhtrogryposis multiplex congenita.
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Fig. 4
(A) High power microscopic findings of calf muscle shows mild size variation of skeletal muscle fiber and interstitial edema (H&E, ×400). (B) Electron microscope shows well preserved myofbrils and sarcomeres (arrow). There is no abnormal mitochondria nor cytoplasmic inclusion (×1,500).
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