El Madi Aziz, Khattala Khalid, Rami Mohammed, Bouabdallah Youssef
Department of Pediatric Surgery, Hassan II University Hospital; Fes, Morocco.
Pan Afr Med J. 2014 Jul 16;18:218. doi: 10.11604/pamj.2014.18.218.4906. eCollection 2014.
Hernia through a defect of the broad ligament is extremely rare in children. These defects can result from a developmental defect or a spontaneous rupture of cystic formations remnants of mesonephric ducts or Müller. Genital anomalies associated with unilateral renal agenesis are more common in females. We report the case of a 13 months girl allowed for assessment of recurrent urinary tract infection; abdominal examination did not objectified palpable mass, the external genitalia were without abnormalities. Abdominal ultrasound revealed a left renal space is empty with a retrovesical cyst. Cystography was requested that objectified a large pelvic cystic mass retrovesical communicating with the bladder, there was also a left vesicoureteral reflux. Uro- MRI showed a cystic formation retrovesical communicating with the bladder, the right pelvic kidney; uterus is normal size for age. DMSA scintigraphy confirmed the absence of the left kidney with the right kidney that ensures 100% of total renal function. To surgical exploration we found a hernia of the bladder through the left broad ligament, the uterus was dislocated on the right side; left ovary was hypoplasic; the ipsilateral ureter was blind with renal agenesis, we performed by reduction of the bladder then closing the hernial orifice, dissection of the ureter with its ligation and section at the vesical stoma. The postoperative course was uneventful. Evolution is favorable. This observation illustrates a hernia of the bladder through the broad ligament associated with ovarian hypoplasia, renal agenesis and ipsilateral ureter blind ending; this association was not described to our knowledge in the literature.
通过阔韧带缺损形成的疝在儿童中极为罕见。这些缺损可能源于发育缺陷或中肾管或苗勒管囊性结构残余的自发性破裂。与单侧肾缺如相关的生殖器异常在女性中更为常见。我们报告了一名13个月大女童的病例，该女童因反复尿路感染前来评估；腹部检查未触及可触及的肿块，外生殖器无异常。腹部超声显示左肾区空虚，膀胱后有一个囊肿。随后进行了膀胱造影，发现膀胱后有一个巨大的盆腔囊性肿块与膀胱相通，同时还存在左侧膀胱输尿管反流。泌尿磁共振成像显示膀胱后有一个与膀胱相通的囊性结构，右侧盆腔肾；子宫大小与年龄相符。二巯基丁二酸闪烁扫描证实左肾缺如，右肾承担100%的总肾功能。手术探查时，我们发现膀胱通过左侧阔韧带形成疝，子宫向右移位；左侧卵巢发育不全；同侧输尿管盲端伴肾缺如，我们先将膀胱复位，然后关闭疝孔，游离输尿管并在膀胱造口处结扎和切断。术后过程顺利。病情进展良好。该病例说明了通过阔韧带形成的膀胱疝与卵巢发育不全、肾缺如和同侧输尿管盲端相关；据我们所知，这种关联在文献中尚未有描述。
