Droste S, Fitzsimmons J, Pascoe-Mason J, Shepard T H, Mack L A
Department of Obstetrics and Gynecology, University of Washington School of Medicine, Seattle.
Obstet Gynecol. 1990 Aug;76(2):206-9.
Bilateral renal agenesis is a fetal malformation incompatible with extrauterine life. Accurate prenatal diagnosis is essential for patient counseling. False-negative diagnoses have been reported and were attributed to the sonographic misidentification of apparently hypertrophied fetal adrenal glands as fetal kidneys. To study the relationship between renal agenesis and adrenal size, we reviewed autopsy records from 11 affected fetuses that had undergone careful autopsy and organ weight determination in our laboratory. Anomalies of distant structures were present in five affected fetuses. A sonographic diagnosis of adrenal hypertrophy had been made in two cases. In four of 11 fetuses, the glands had taken on a flattened discoid appearance. The autopsy records of 240 normal fetuses were similarly reviewed, and regression lines were generated for adrenal weight based on foot length and crown-rump length. The adrenal weights from affected fetuses were well within normal limits when compared with these normal regression lines and with organ weight standards from the literature. We conclude that adrenal hypertrophy is not a common finding in this syndrome and that the reported false-negative diagnoses are more likely attributable to a change in adrenal shape rather than a true increase in adrenal mass.
双侧肾缺如是一种与宫外生活不相容的胎儿畸形。准确的产前诊断对患者咨询至关重要。已有假阴性诊断的报道，其原因是超声检查将明显肥大的胎儿肾上腺误识别为胎儿肾脏。为了研究肾缺如与肾上腺大小之间的关系，我们回顾了在我们实验室进行过仔细尸检和器官重量测定的11例受累胎儿的尸检记录。5例受累胎儿存在远处结构异常。有2例曾作出肾上腺肥大的超声诊断。在11例胎儿中有4例，肾上腺呈现扁平盘状外观。我们同样回顾了240例正常胎儿的尸检记录，并根据足长和顶臀长生成了肾上腺重量的回归线。与这些正常回归线以及文献中的器官重量标准相比，受累胎儿的肾上腺重量完全在正常范围内。我们得出结论，肾上腺肥大在该综合征中并非常见表现，且所报道的假阴性诊断更可能归因于肾上腺形状的改变而非肾上腺质量的真正增加。
