背景:修剪型腹部综合征是一种病因不明的罕见先天性畸形,有以下三位一体的发现:腹肌壁无力,睾丸未降,尿路异常.在大多数情况下,在新生儿或婴儿期检测到修剪腹部综合征。在这个案例报告中,我们描述了一个来自埃塞俄比亚的12岁男孩,他发现了西梅腹部综合症和骨骼畸形。我们不知道埃塞俄比亚以前有任何关于西梅腹部综合症的报道。
方法:一名来自西北贡达尔·阿姆哈拉地区州的12岁的阿姆哈拉男孩因过去3个月的左侧腹侧肿胀而到我们的转诊医院就诊。产妇的怀孕过程和病史是非共同的,他在一家健康中心进行了接生。他有七个兄弟姐妹,他们都没有类似的症状。经检查,他腹部扩张,不对称,左翼凸出,可见水平线,向上的脐带缝,腹直肌缺失。他的腹部柔软,囊性柔软,在左侧的双侧可见肿块,大小为13×11厘米。两个睾丸都没有下降,他还患有臀部发育异常。腹部超声检查显示他的左肾区域有一个大的囊性肿块,有回声碎片,髋部X射线显示双侧发育性髋部发育不良。术中发现为左肾囊性,两个睾丸都是腹膜内的,左肾静脉曲折,肿大的膀胱到达脐以上,然后离开了Megaureter.
方法:进行双侧睾丸切除术和左肾切除术。他被静脉给予抗生素治疗肾盂肾炎,并出院回家,预约随访和可能的腹部成形术。
结论:在当前的报告中,延迟的表现导致睾丸萎缩和睾丸切除术的决定。此外,他有性激素异常的潜在风险.因此,在资源有限的环境中,对修剪型腹部综合征的诊断需要高度怀疑。我们建议进一步研究,以确定在资源有限的环境中修剪腹部综合征的最佳管理和早期诊断。
BACKGROUND: Prune belly syndrome is a rare congenital malformation of unknown etiology, with the following triad of findings: abdominal muscle wall weakness, undescended testes, and urinary tract abnormalities. In most cases, detection of prune belly syndrome occurs during neonatal or infancy period. In this
case report, we describe a 12-year-old boy from Ethiopia with the triad of findings of prune belly syndrome along with skeletal malformations. We are unaware of any previous report of prune belly syndrome in Ethiopia.
METHODS: A 12-year-old Amhara boy from the Northwest Gondar Amhara regional state presented to our referral hospital with a complaint of swelling over his left flank for the past 3 months. Maternal pregnancy course and medical history were noncontributory, and he had an attended birth at a health center. He has seven siblings, none of whom had similar symptoms. On examination he had a distended abdomen, asymmetric with bulging left flank, visible horizontal line, upward umbilical slit, and absent rectus abdominis muscles. His abdomen was soft with a tender cystic, bimanually palpable mass on the left flank measuring 13 × 11 cm. Both testes were undescended and he also has developmental dysplasia of the hips. An abdominal ultrasound revealed a large cystic mass in his left kidney area with echo debris and a hip X-ray showed bilateral developmental dysplasia of the hip. Intraoperative findings were cystic left kidney, both testes were intraperitoneal, tortuous left renal vein, enlarged bladder reaching above umbilicus, and left megaureter.
METHODS: bilateral orchidectomy and left nephrectomy were done. He was given intravenously administered antibiotics for treatment of pyelonephritis and discharged home with an appointment for follow up and possible abdominoplasty.
CONCLUSIONS: In the current report delayed presentation contributed to testicular atrophy and decision for orchidectomy. Furthermore, he will be at potential risk for sex hormone abnormality. Therefore, diagnosis of prune belly syndrome in resource-limited settings requires a high index of suspicion. We recommend further research to determine the optimal management and early diagnosis of prune belly syndrome in resource-limited settings.