神经纤维瘤侵犯膀胱，表现为排便梗阻症状及巨大会阴疝。
Neurofibroma invading into urinary bladder presenting with symptoms of obstructed defecation and a large perineal hernia.
作者信息
Subasinghe Duminda, Keppetiyagama Chathuranga Tisara, De Silva Chandu, Perera Neville D, Samarasekera Dharmabandhu N
机构信息
The National Hospital of Sri Lanka, University Surgical Unit, Colombo, Sri Lanka.
出版信息
BMC Surg. 2014 Apr 16;14:21. doi: 10.1186/1471-2482-14-21.
BACKGROUND
Pelvic floor hernias pose a diagnostic and a treatment challange. Neurofibromatosis is a rare systemic disease, and urinary tract involvement is rare.
CASE PRESENTATION
Here we report a case of a 54-year-old female with multiple neurofibromatosis who presented with features of obstructed defecation and was found to have a large perineal hernia. At surgery, we found an unusual herniation of a large neuropathic bladder and rectum through a perineal defect. She underwent reduction cystoplasty and repair of the pelvic floor using a prolene mesh. Subsequent histopathological examination confirmed a large neurofibroma infiltrating the urinary bladder.
CONCLUSION
Neurofibromatosis of the bladder is rare it should be considered as a differential diagnosis in patients presenting with symptoms of obstructed defecation.
背景
盆底疝带来诊断和治疗方面的挑战。神经纤维瘤病是一种罕见的全身性疾病，累及泌尿道的情况罕见。
病例报告
我们在此报告一例54岁患有多发性神经纤维瘤病的女性，其表现为排便梗阻特征，经检查发现有一个巨大的会阴疝。手术中，我们发现一个巨大的神经性膀胱和直肠通过会阴缺损发生了异常疝出。她接受了膀胱复位成形术并用普理灵网片修复盆底。随后的组织病理学检查证实有一个巨大的神经纤维瘤浸润膀胱。
结论
膀胱神经纤维瘤病罕见，对于出现排便梗阻症状的患者应考虑将其作为鉴别诊断之一。
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