Uterovajinal agenezili rektovestibüler fistül: İki olgu sunumu
Küçük Resim Yok
Tarih
2004
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Erişim Hakkı
info:eu-repo/semantics/closedAccess
Özet
Anorektal bozukluklar arasında çok nadir rastlanan, iki uterovajinal agenezili rektovestibüler fistül olgusu sunuldu. Bozukluk rektovestibüler fistüllü anal atrezi ya da normal üretral ve vajinal açıklığı olan fistülsüz anal atrezi şeklinde yanlış tanı alabilmektedir. Her iki hastaya yenidoğan döneminde tanı konarak kolostomileri açıldı. Anorektal bozukluğun düzeltici ameliyatları hastalar 6 aylıkken yapıldı. Posterior sagital girişim ile her iki hastada distal rektum yeni vajina oluşturulmasında kullanıldı. Proksimal rektum ise, Pena ve ark.'nın tanımladığı şekilde serbestleştirilip sfinkter içerisinden geçirilerek anal bölgeye ağızlaştırıldı. Hasta-ların overleri normal olup Fallop tüpleri orta çizgiye yaklaştıkça fibröz bant halini almakta idi, uterus ve vajinaları yoktu. 4 yaşında olan ilk hastada idrar ve dışkı tutma sağlandı. 3 yaşın altında olan diğer hastada klinik değerlendirme henüz yapılamamaktadır. Oluşturulan yeni vajinanın hastalarda cinsel etkinliğe izin verip vermeyeceği konusunda henüz bilgimiz yoktur. Posterior sagittal yaklaşım, bu bozuklukların onarımında karından girmeye gerek kalmadan uygulanabilecek bir seçenek olabilir. Anorektal bozukluğu olan kız hastalarda nadir bozuklukları saptayabilmek için perinenin dikkatli şekilde gözle muayenesi gerekmektedir.
A very unusual anorectal defect in two patients, consisting of a rectovestibular fistula and uterovaginal agenesis is presented. The defect can be misdiagnosed as imperforate anus with rectovestibular fistula or imperforate anus without fistula and with normal urethral and vaginal openings. A colostomy was opened at the neonatal period in the patients, and definitive repair of the anorectal anomaly was performed when the patients were 6-month-old. With the posterior sagittal approach, the distal rectum was used to create the neovagina in the patients. The proximal rectum was mobilized and placed within the limits of the sphincter mechanism as described by Pena et al. All patients had normal ovaries, but fallopian tubes gradually became cordlike structures as they approached the midline, and the uterus and vagina were completely absent. One patient, who is 4 years old, is continent of urine and has voluntary bowel movements. The other patient is under 3 years of age and is therefore not old enough to determine a clinical result. We don't have evidence that the neovagina functions as a vagina in the patients to allow sexuall activity. The posterior sagittal approach represents another alternative to treat these defects without the need for laparotomy. It is mandatory to perform a meticulous inspection of the perineum in female patients with anorectal malformations to detect unusual defects.
A very unusual anorectal defect in two patients, consisting of a rectovestibular fistula and uterovaginal agenesis is presented. The defect can be misdiagnosed as imperforate anus with rectovestibular fistula or imperforate anus without fistula and with normal urethral and vaginal openings. A colostomy was opened at the neonatal period in the patients, and definitive repair of the anorectal anomaly was performed when the patients were 6-month-old. With the posterior sagittal approach, the distal rectum was used to create the neovagina in the patients. The proximal rectum was mobilized and placed within the limits of the sphincter mechanism as described by Pena et al. All patients had normal ovaries, but fallopian tubes gradually became cordlike structures as they approached the midline, and the uterus and vagina were completely absent. One patient, who is 4 years old, is continent of urine and has voluntary bowel movements. The other patient is under 3 years of age and is therefore not old enough to determine a clinical result. We don't have evidence that the neovagina functions as a vagina in the patients to allow sexuall activity. The posterior sagittal approach represents another alternative to treat these defects without the need for laparotomy. It is mandatory to perform a meticulous inspection of the perineum in female patients with anorectal malformations to detect unusual defects.
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Kaynak
Pediatrik Cerrahi Derg.
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Cilt
18
Sayı
1
