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Obstet Gynecol Sci > Volume 55(6); 2012 > Article
Korean Journal of Obstetrics & Gynecology 2012;55(6):398-402.
DOI: https://doi.org/10.5468/KJOG.2012.55.6.398    Published online June 16, 2012.
Antenatal sonographic features of intestinal neuronal dysplasia type A associated with polydactyly and micromelia.
Hyun Joo Son, Yun Sung Jo, Ji Han Jung, Dong Gyu Jang, Guisera Lee
1Department of Obstetrics and Gynecology, St. Vincent Hospital, The Catholic University of Korea School of Medicine, Seoul, Korea. leegsr@catholic.ac.kr
2Department of Pathology, St. Vincent Hospital, The Catholic University of Korea School of Medicine, Seoul, Korea.
Abstract
Type A intestinal neuronal dysplasia is a congenital abnormality that is a very rare disease. Here, we report on a patient who had intestinal dilatation with serial changes and polydactyly, as shown on prenatal ultrasound. Bowel obstruction symptoms were shown at 16 days of life. An open abdominal surgery was performed. Malrotation of the bowel and narrowing of the area from 15 cm above the ileocecal valve were noted. Therefore, a right hemicolectomy, including lesions was performed. The patient was diagnosed with type A intestinal neuronal dysplasia by pathology.
Key Words: Intestinal neuronal dysplasia, Polydactyly, Micromelia, Ultrasonography
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