小儿重复尿道畸形

Urethral duplication in children

目的探讨小儿重复尿道畸形的合理治疗方法。方法重复尿道畸形患儿18例，均为男性，年龄1～12岁，平均4．5岁。18例畸形均为矢状位排列，其中尿道上裂型6例、下裂型4例、会阴分叉型8例。尿道上裂型行副尿道结扎切除术3例，3例无临床症状者未行手术；尿道下裂型行腹侧尿道成形术3例，1例先行尿道造口，术后半年行尿道成形术；会阴分叉型腹侧尿道为主尿道者7例一期行阴茎根部尿道造口、二期自尿道造口至阴茎头行尿道成形术，1例背侧尿道发育正常腹侧尿道发育不良者行腹侧尿道切除术。结果18例术后随访1～7年，尿道上裂手术治疗3例术后症状消失，外观满意；尿道下裂型2例术后排尿通畅，1例合并尿道狭窄、尿道瘘者行尿道修复术后治愈，1例因合并神经原性膀胱行膀胱造瘘；会阴分叉型二期术后治愈1例，一期术后并发尿道狭窄、尿道直肠瘘各1例，二期术后尿道狭窄1例，分别经尿道扩张、尿道瘘修补术后治愈，3例待行二期手术，1例腹侧尿道切除者术后效果满意。结论重复尿道畸形临床表现依分型各异，手术时分清主、副尿道至关重要；腹侧尿道为主尿道，发育、功能完善，手术应予以保留。治疗方式应根据患儿病变的解剖特点及临床表现合理选择。

Objective To discuss the management of urethral duplication in children. Methods The clinical data of 18 children （all are males） with urethral duplication were retrospectively analyzed. All the 18 patients were sagittal plane included epispadias in 6, hypospadias in 4 and Y type in the remaining 8. In 6 cases with epispadias, 3 patients underwent excision of the accessory dorsal urethra, 3 patients without operation. Urethroplasty was necessary in the patient with a duplicated hypospadiac urethra. Of 8 patients with Y-type urethral duplication, 7 patients underwent 2-stage repair including displacement of the urethra from the anal canal to the perineum at stage 1 and subsequent urethral reconstruction at stage 2. The ventral urethra which was hypospadias had been excised in the other one. Results All the patients were followed-up. Good cosmetic and functional results were achieved in the 3 boys who underwent excision of the accessory dorsal urethra. In the hypospadiac duplication, 2 patients could void spontaneously through a normally positioned granular meatus. One boy presented with urethral stenosis after operated. One boy had a vesicostomy for neuropathic bladder. In Y-type urethral duplications, only 1 patient was successful after 2-stage urethroplasty, 3 patients developed urethra stricture or fistula, 3 boys need the 2-stage operation, the other one who underwent excision of the ventral urethra could void spontaneously. Conclusions Urethral duplication is a rare congenital anomaly. The clinical presentation varies because of the different anatomical patterns of this abnormality. Commonly the ventral urethra is most functional and maintained after surgical correction. Surgical management should be individual and depend on the anatomical configuration of the du- plicated urethras.