DeRosa Raffaella, Musser John E, Rooks Veronica J, McPherson John, McMann Leah P
Division of Urology, Department of Surgery, Tripler Army Medical Center, Honolulu, HI.
Department of Urologic Oncology, Memorial Sloan Kettering Cancer Center, New York, NY; Urology Service, Department of Surgery, New York, NY.
Urology. 2014 Nov;84(5):1211-3. doi: 10.1016/j.urology.2014.07.017. Epub 2014 Sep 18.
Children with omphalocele, exstrophy, imperforate anus, and spinal defects complex present with the most severe form of birth defects in the exstrophy-epispadias spectrum. Prenatal diagnosis is difficult, but improved survival over the past several decades makes understanding the potential anatomic manifestations imperative for expeditious and appropriate surgical care. The upper urinary tract is often normal in children with omphalocele, exstrophy, imperforate anus, and spinal defects complex, but malposition of one of the kidneys has previously been reported. We present the first case of bilateral kidney herniation into the omphalocele sac.
患有脐膨出、膀胱外翻、肛门闭锁和脊柱裂综合征的儿童呈现出膀胱外翻-尿道上裂谱系中最严重的出生缺陷形式。产前诊断困难，但过去几十年生存率的提高使得了解潜在的解剖学表现对于迅速且恰当的手术治疗至关重要。患有脐膨出、膀胱外翻、肛门闭锁和脊柱裂综合征的儿童上尿路通常正常，但此前曾有一侧肾脏位置异常的报道。我们报告首例双侧肾脏疝入脐膨出囊的病例。
