Ghavamian R, Wilcox D T, Duffy P G, Milla P J
Department of Urology, Great Ormond Street Hospital for Sick Children, NHS Trust, London, United Kingdom.
J Urol. 1997 Sep;158(3 Pt 2):1286-90. doi: 10.1097/00005392-199709000-00164.
Hollow visceral myopathy is a rare clinical entity characterized by impaired intestinal function in the absence of mechanical occlusion. It can affect the smooth muscle of the whole or segments of the gastrointestinal tract and occasionally the urinary tract. We examined the urological manifestations of hollow visceral myopathy and management in the pediatric population.
We reviewed the records of 14 male patients 1 day to 2 years old (mean age 4.6 months) and 10 female patients 1 day to 5 years old (mean age 9.4 months) at presentation to our institution with hollow visceral myopathy. In all patients genitourinary tract ultrasound, voiding cystourethrography and serum creatinine measurement were done at presentation.
All patients had gastrointestinal obstructive symptoms at presentation and 11 (46%) had urological symptoms, including urinary retention in 2, urinary tract infection in 3, and a prenatal diagnosis of megacystis and hydroureteronephrosis in 6. Overall 22 patients (92%) had urological abnormalities, all had poor bladder emptying and recurrent urinary tract infections, and 13 had megacystis associated with bilateral hydroureteronephrosis in 9 and unilateral hydroureteronephrosis in 2. There were 9 deaths from extensive gastrointestinal involvement and sepsis. Of the surviving 15 patients 13 have urological abnormalities, including 8 who perform and tolerate clean intermittent catheterization via the urethra and are well. Of the 4 male infants who did not tolerate clean intermittent catheterization appendicovesicostomy was done in 1, a Casale tube was placed in 1 and vesicostomy was performed in 2. The remaining female patient has day and night wetting.
Urological abnormalities are common in hollow visceral myopathy and they can contribute to presenting symptoms. Clean intermittent catheterization via the urethra to aid in bladder emptying and decrease the frequency of urinary tract infections is the mainstay of treatment but surgery to construct an alternative catheterizable channel or vesicostomy may be required in intolerant patients.
中空脏器肌病是一种罕见的临床病症，其特征为在无机械性梗阻的情况下肠道功能受损。它可影响整个胃肠道或部分胃肠道的平滑肌，偶尔也会影响泌尿道。我们研究了中空脏器肌病在儿科人群中的泌尿系统表现及治疗方法。
我们回顾了14例年龄在1天至2岁（平均年龄4.6个月）的男性患者以及10例年龄在1天至5岁（平均年龄9.4个月）的女性患者的病历，这些患者均因中空脏器肌病前来我院就诊。所有患者在就诊时均进行了泌尿生殖道超声检查、排尿性膀胱尿道造影以及血清肌酐测定。
所有患者在就诊时均有胃肠道梗阻症状，11例（46%）有泌尿系统症状，包括2例尿潴留、3例尿路感染以及6例产前诊断为巨膀胱和输尿管肾积水。总体而言，22例患者（92%）有泌尿系统异常，均存在膀胱排空障碍和反复尿路感染，13例有巨膀胱，其中双侧输尿管肾积水者9例，单侧输尿管肾积水者2例。9例因广泛的胃肠道受累和败血症死亡。在存活的15例患者中，13例有泌尿系统异常；其中8例能经尿道进行并耐受清洁间歇性导尿，情况良好。在4例不能耐受清洁间歇性导尿的男婴中，1例行阑尾膀胱造口术，1例置入卡萨莱管，2例行膀胱造口术。其余女性患者存在日夜遗尿现象。
泌尿系统异常在中空脏器肌病中很常见，且可导致出现症状。经尿道进行清洁间歇性导尿以帮助膀胱排空并减少尿路感染的频率是主要治疗方法，但对于不耐受的患者可能需要手术构建替代的可导尿通道或进行膀胱造口术。
