Irfan Omar, Ladiwala Zoya Fatima Rizwan, Zaidi Zafar
Pediatrics, Centre for Global Child Health, Hospital for Sick Children, Toronto, CAN.
Medicine, Civil Hospital Karachi, Dow University of Health Sciences, Karachi, PAK.
Cureus. 2020 Apr 18;12(4):e7723. doi: 10.7759/cureus.7723.
Bladder exstrophy-epispadias complex (EEC) is a rare congenital defect where the abdominal muscles and bones fail to close in the mid-pelvis. It is crucial to understand the health-related quality of life (QOL) of exstrophy patients who have undergone multiple correctional surgeries. We herein discuss a case of bladder EEC that was repaired through a series of procedures at a resource-limited hospital in Karachi, Pakistan. A 21-year-old male, who was born with EEC, underwent bladder augmentation, Mitrofanoff procedure, bladder neck reconstruction, ureter implantation and a right nephrectomy in a single one-stage procedure during late childhood for urinary incontinence. However, this required a further revision because the urinary incontinence persisted, with difficulty in catheterizing the Mitrofanoff channel. On follow-up after 10 years, our patient currently describes normal QOL with near-normal sexual function. Validated questionnaires for QOL, erectile dysfunction, incontinence and prostatic function were used to assess the patient's post-operative status in these domains. Through our report, we conclude that such patients can have a normal QOL by means of a holistic multidisciplinary management, which includes timely surgical corrections along with an additional focus on the psycho-social and sexual aspects of this condition.
膀胱外翻-尿道上裂复合畸形（EEC）是一种罕见的先天性缺陷，盆腔中部的腹部肌肉和骨骼未能闭合。了解接受过多次矫正手术的膀胱外翻患者的健康相关生活质量（QOL）至关重要。我们在此讨论一例膀胱EEC病例，该病例在巴基斯坦卡拉奇一家资源有限的医院通过一系列手术进行了修复。一名21岁男性，出生时患有EEC，在儿童晚期接受了一期手术，包括膀胱扩大术、米氏术、膀胱颈重建术、输尿管植入术和右肾切除术，以治疗尿失禁。然而，由于尿失禁持续存在，米氏通道插管困难，需要进一步修复。在10年的随访中，我们的患者目前表示生活质量正常，性功能接近正常。使用经过验证的生活质量、勃起功能障碍、尿失禁和前列腺功能问卷来评估患者在这些领域的术后状况。通过我们的报告，我们得出结论，通过全面的多学科管理，包括及时的手术矫正以及对该疾病心理社会和性方面的额外关注，此类患者可以拥有正常的生活质量。
