Lotan D, Hertz M, Aladjem M, Braf Z, Boichis H
Isr J Med Sci. 1981 Jun;17(6):437-40.
Five cases of partial or complete agenesis of the sacrum (also called the caudal regression syndrome) diagnosed at the Chaim Sheba Medical :Center are reported. Neurological dysfunction of the bladder was present in all. Two patients were diagnosed at the ages of four months and three years, and had dilated collecting systems and neurological deficits of the upper motor neuron type. In three of the patients, the malformation was recognized later, at the ages of 7 1/2, 11 and 22 years. All three had a normal urinary tract, on examination by intravenous urography, and neurological deficits of the lower motor neuron type. Although no overt reduction of renal function was noted in those of our patients in whom diagnosis was delayed, it appears that early diagnosis and treatment of the neurogenic bladder associated with the caudal regression syndrome may reduce both morbidity and discomfort.
报告了在海姆·谢巴医疗中心诊断出的5例部分或完全骶骨发育不全(也称为尾椎退化综合征)病例。所有患者均存在膀胱神经功能障碍。两名患者分别在4个月和3岁时被诊断出,有集合系统扩张和上运动神经元型神经功能缺损。另外三名患者在7岁半、11岁和22岁时较晚才发现畸形。通过静脉肾盂造影检查,这三名患者的尿路均正常,存在下运动神经元型神经功能缺损。虽然在诊断延迟的患者中未观察到明显的肾功能减退,但似乎早期诊断和治疗与尾椎退化综合征相关的神经源性膀胱可降低发病率和不适感。