摘要
目的：了解儿童先天性马蹄肾（HSK）合并泌尿系统及脊柱异常的发生率，提高对本病的临床及影像表现的认识。方法：回顾性分析22例儿童先天性马蹄肾合并泌尿系统及脊柱畸形的临床资料和影像学表现。男14例，女8例，年龄20天~11岁，平均3.5岁。就诊时的临床表现主要包括发现肾积水6例，发现神经管闭合不全5例，发现脊柱发育异常4例，出生后发现无肛3例，偶尔发现马蹄肾畸形4例。所有病例均给予MRI检查。结果：①本组病例, 仅合并泌尿系统异常8例（36.4%），仅合并脊柱异常5例（22.7%），同时合并泌尿系统及脊柱异常的6例（27.3%），无明显泌尿系统及脊柱异常的3例（13.6%）。②14例泌尿系统异常包括右肾积水6例，左肾积水3例，双肾积水4例，1例肾重复畸形不伴肾积水；3例左肾积水中的1例同时合并左肾重复畸形。6例右肾积水中1例合并左肾重复畸形和双侧输尿管积水。4例双肾积水中1例合并双侧输尿管上段积水。③11例脊柱畸形，分别含有以下一种或多种畸形，包括脊髓脊膜膨出（5例），骶尾椎发育不良（7例），多发椎体畸形合并脊柱侧弯（6例），椎管内脂肪瘤或脂肪变性（3例），脊髓空洞（2例）。结论：先天性马蹄肾是一种少见的先天性畸形，常容易合并泌尿系统及脊柱异常，MRI对马蹄肾及泌尿系统、脊柱异常的发现有独特优势，有利于对本病进行完整的评估。
Abstract
Objective: To improve the knowledge of imaging appearances and clinical presentations of horse shoe kidney(HSK) combined with urinary system and spine malformations in children. Methods: Clinical and imaging data of 22 cases of HSK in children were retrospectively reviewed with 14 males and 8 females. The age was from 20 days to 11 years with a mean age of 3.5 years. 6 cases presented with uronephrosis, 5 cases presented with dysrhaphia. 4 cases presented with spine malformations. 3 cases presented with aproctia. 4 cases were found occasionally. MRI was performed for these 22 cases. Results: ①In these 22 HSK cases, 8 cases were combined with urinary system abnormalities(36.4%), 5 cases were combined with spine abnormalities(22.7%), 6 cases were combined with both urinary system and spine abnormalities(27.3%), and 3 cases had no other abnormalities(13.6%). ②14 cases of urinary system abnormalities included 6 right uronephrosis, 3 left uronephrosis, 4 bilateral uronephrosis, 1 renal duplication malformation. 1 left uronephrosis was associated with left renal duplication malformation. 1 right renal uronephrosis was associated with left renal duplication malformation and bilateral ureterectasia. 1 bilateral uronephrosis was associated with bilateral upper ureterectasia. ③11 cases of spine malformations included one or more types of malformations, including 5 dysrhaphia, 7 lumbosacral dysplasia, 6 multiple vertebral deformity combined with scoliosis, 3 intraspinal lipoma and 2 syringomyelia. Conclusion: As a rare congenital deformity, HSK can be associated with urinary system and/or spine malformations. MRI examinations have advantages in diagnosing and evaluating these malformations.
关键词
泌尿生殖系统畸形 /
脊柱 /
磁共振成像
Key words
Urogenital abnormalities /
Spine /
Magnetic resonance imaging
周 莺;邵 虹;潘慧红;张 弘;朱 铭.
儿童先天性马蹄肾合并泌尿系统及脊柱异常的MRI表现[J]. 中国临床医学影像杂志. 2015, 26(5): 337-340
ZHOU Ying;SHAO Hong;PAN Hui-hong;ZHANG Hong;ZHU Ming.
MRI manifestations of horse shoe kidney combined with urinary system and spine malformations in children[J]. Journal of China Clinic Medical Imaging. 2015, 26(5): 337-340
中图分类号：
Ｒ726.921.3
Ｒ726.911
R445.2
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