Rahayatri Tri Hening, Pramudito Baskoro Cahyo, Soedibyo Sastiono
Faculty of Medicine, Universitas Indonesia, Department of Pediatric Surgery, Cipto Mangunkusumo Hospital, Jakarta, Indonesia.
Faculty of Medicine, Universitas Indonesia, Department of Surgery, Cipto Mangunkusumo Hospital, Jakarta, Indonesia.
Int J Surg Case Rep. 2021 Feb;79:76-83. doi: 10.1016/j.ijscr.2020.12.085. Epub 2021 Jan 5.
Vaginal agenesis with anorectal malformations is a complex pediatric condition that adversely affects various physiological processes in the body. It may cause disturbances in defecation and urination, abnormalities in the urinary and gastrointestinal tract, dysfunction of the genital and reproductive organs, and sexual function disorders. The complexity in the surgical management of vaginal agenesis includes the selection of a functional reconstruction technique for anal and vaginal formation, timing of the reconstruction, and management of complications in the associated organ system. Herein, we describe a patient with Mayer-Rokitansky-Küster-Hauser syndrome accompanied by a rectovesical fistula. Other abnormalities, such as microcephaly, polydactyly, long urethral abnormalities resembling the male urethra, and complications in the kidney and urinary tract, were observed in the patient. The associated complications included recurrent urinary tract infections, urinary overflow incontinence, vesicoureteric reflux, hydroureter, and left renal hydronephrosis. The patient underwent posterior sagittal anorectoplasty surgery and vaginal reconstruction. The long-term vaginal physiological development of patients with this condition remains unknown.
阴道闭锁合并肛门直肠畸形是一种复杂的儿科疾病，会对身体的各种生理过程产生不利影响。它可能导致排便和排尿障碍、泌尿和胃肠道异常、生殖器官功能障碍以及性功能障碍。阴道闭锁手术管理的复杂性包括选择用于肛门和阴道成形的功能性重建技术、重建时机以及相关器官系统并发症的处理。在此，我们描述一名患有迈耶-罗基坦斯基-库斯特-豪泽综合征并伴有直肠膀胱瘘的患者。该患者还观察到其他异常情况，如小头畸形、多指畸形、类似男性尿道的长尿道异常以及肾脏和泌尿系统并发症。相关并发症包括复发性尿路感染、充溢性尿失禁、膀胱输尿管反流、输尿管积水和左肾积水。该患者接受了后矢状位肛门直肠成形术和阴道重建术。这种情况下患者阴道的长期生理发育情况尚不清楚。
