Al Mosawi Aamir Jalal
Department of Pediatrics, Al Kadhimiyia University Hospital, Al Kadimiyia, Baghdad, Iraq.
Urology. 2007 Aug;70(2):355-6; discussion 356-7. doi: 10.1016/j.urology.2007.04.049.
To report the third series of the severe neonatal form of Allen-Hinman syndrome. Hinman and Baumann (1973) and Allen (1977) reported the first two series (14 and 21 patients, respectively) of children with severe functional vesicourethral dysfunction producing changes indistinguishable from an obstructive factor.
From January 1995 to April 2006, 7 boys with Allen-Hinman syndrome were observed. They had been symptomatic since early infancy with a poor stream and chronic urinary retention. They had bilateral hydroureteronephrosis on renal ultrasonography and intravenous urography (grade 4 to 5 vesicourethral reflux) during the first year of age. They were initially thought by urologists to have a posterior urethral valve. When cystoscopy excluded anatomic obstruction, the urologists were unable to provide an alternative diagnosis.
The patients' age on referral ranged from 5 months to 15 years (mean 6 years). They had been followed up for a period of 2 weeks to 6 years. Four patients had been treated with chronic catheterization and were referred after the age of 5 years with end-stage renal failure. One of them underwent cutaneous vesicotomy after the onset of chronic renal failure and the need for dialysis. I recommended early vesicotomy for the remaining 3 patients. One boy had undergone vesicotomy during the first 6 months of age and had been followed up for 6 years. At 6 years of age, the dilation had disappeared and his renal function had been preserved.
The nonneurogenic neurogenic bladder represents a rare, severe form of dysfunctional voiding that can present even in the neonatal period and can lead to renal failure.
报告艾伦 - 欣曼综合征严重新生儿型的第三组病例。欣曼和鲍曼(1973年)以及艾伦(1977年)分别报告了前两组病例(分别为14例和21例患儿),这些患儿患有严重的功能性膀胱尿道功能障碍,产生的变化与梗阻因素难以区分。
1995年1月至2006年4月,观察到7例患有艾伦 - 欣曼综合征的男孩。他们自婴儿早期就出现症状,表现为尿流不畅和慢性尿潴留。在一岁时,肾脏超声和静脉肾盂造影显示他们有双侧肾盂输尿管积水(膀胱尿道反流4至5级)。泌尿外科医生最初认为他们患有后尿道瓣膜。当膀胱镜检查排除解剖性梗阻后,泌尿外科医生无法给出其他诊断。
转诊时患者年龄从5个月至15岁不等(平均6岁)。他们接受了2周至6年的随访。4例患者接受了长期导尿治疗,5岁后因终末期肾衰竭前来就诊。其中1例在慢性肾衰竭且需要透析后接受了膀胱造瘘术。我建议对其余患者早期行膀胱造瘘术。1名男孩在6个月大时接受了膀胱造瘘术,并接受了6年的随访。6岁时,扩张消失,肾功能得以保留。
非神经源性神经源性膀胱是一种罕见的、严重的排尿功能障碍形式,甚至可在新生儿期出现,并可导致肾衰竭。