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.