Kesim Selin, Turoğlu Halil Turgut, Özgüven Salih, Öneş Tunç, Erdil Tanju Yusuf
Marmara University Faculty of Medicine, Department of Nuclear Medicine, İstanbul, Turkey.
Mol Imaging Radionucl Ther. 2022 Feb 2;31(1):69-71. doi: 10.4274/mirt.galenos.2020.84755.
Caudal regression syndrome (CRS) or sacral agenesis is a rarely seen malformation with a varying degree of structural abnormalities, including multiorgan system dysfunctions, reported with higher incidence among children of mothers with diabetes, as in this case. Spinal anomalies can range from coccyx hemiagenesis to the total absence of lower lumbar vertebrae and sacrum in most severe cases. Herein, we have presented a 9-year-old patient with CRS who had renal failure. Technetium-99m dimercaptosuccinic acid renal scintigraphy revealed bilaterally non-functioning kidneys with no renal cortical uptake. Renal anomalies in CRS with vertebral, anorectal, cardiac, trachea-esophageal, renal, and limb anomalies association include one-sided renal agenesis, multicystic dysplastic kidneys, and ureter duplications.
尾椎退化综合征(CRS)或骶骨发育不全是一种罕见的畸形,伴有不同程度的结构异常,包括多器官系统功能障碍,如本病例所示,在患有糖尿病的母亲所生儿童中发病率较高。脊柱异常的范围可从尾骨半发育不全到最严重病例中腰椎和骶骨完全缺失。在此,我们报告了一名患有CRS且伴有肾衰竭的9岁患者。锝-99m二巯基丁二酸肾闪烁显像显示双侧无功能肾,无肾皮质摄取。CRS合并脊柱、肛门直肠、心脏、气管食管、肾脏和肢体异常时的肾脏异常包括单侧肾缺如、多囊性发育不良肾和输尿管重复。