Prune Belly Syndrome: Report of Twelve Autopsy Cases and Possible Pathogenesis |
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Authors: | Kenji KAWAMOTO Takayoshi IKEDA Takeshi MATSUO Hiroshi MAEDA Naomasa OKAMOTO Yukio SATOW Naotaka AKIMOTO Juing-Yi LEE |
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Affiliation: | 1st Department of Pathology, Nagasaki University School of Medicine, 12-4 Sakamoto-machi, Nagasaki 852, Japan;*Department of Geneticopathology, Research Institute for Nuclear Medicine and Biology, Hiroshima University, 1-2-3 Kasumi, Minami-ku, Hiroshima 734, Japan |
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Abstract: | Abstract Twelve autopsy cases (9 males, 3 females) of the prune belly syndrome are presented. Principal anomalies of this syndrome are a prune-like abdominal feature and a giant bladder. Urethral atresia was observed in most but two cases. Imperforate anus and rectovesical fistula were observed in 8 and 7 cases respectively. Associated anomalies were those which may or may not be embryologically related to the principal anomalies. As regards the pathogenesis, the authors propose that a primary defect may occur during any developmental stage of the somitic mesoderm, genital tubercle and urethra. In conclusion, the prune belly syndrome may be of spectrum anomalies depending on the stage specificity mainly in the abdominal wall and genito-urinary organs. |
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Keywords: | prune belly syndrome absence of abdominal muscles pathogenesis |
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