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Kim, Oh, and Hong: A case of arthrogryposis multiplex congenita associated with maternal septate uterus
Case Report
Korean Journal of Obstetrics & Gynecology

Korean Journal of Obstetrics & Gynecology 2012; 55(9): 669-672.

Published online: 17 September 2012

DOI: https://doi.org/10.5468/KJOG.2012.55.9.669

A case of arthrogryposis multiplex congenita associated with maternal septate uterus

Hae Kyung Kim, MD1, Hoon Kyu Oh, MD2, Seong Yeon Hong, MD1

1Department of Obstetrics and Gynecology, Catholic University of Daegu School of Medicine, Daegu, Korea.

2Department of Pathology, Catholic University of Daegu School of Medicine, Daegu, Korea.

Corresponding author: Seong Yeon Hong, MD. Department of Obstetrics and Gynecology, Daegu Catholic University Medical Center, 33 Duryugongwon-ro 17-gil, Nam-gu, Daegu 705-718, Korea. Tel: +82-53-650-4078, Fax: +82-53-650-4078, magu815@cu.ac.kr

Received 5 June 2012       Revised 17 July 2012       Accepted 26 July 2012

Copyright © 2012. Korean Society of Obstetrics and Gynecology

(open-access):

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

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.

Keywords: Arthrogryposis multiplex congenita, Septate uterus, Prenatal diagnosis

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).
kjog-55-669-g004

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