Department of Pediatric Surgery, University of Bologna, Via Massarenti, 11, 40138 Bologna, Italy.
J Pediatr Surg. 2010 Oct;45(10):2087-91. doi: 10.1016/j.jpedsurg.2010.05.016.
BACKGROUND/PURPOSE: The absence of vagina is rare in the pediatric population. It can occur as a result of congenital malformations such as an aplasia of mullerian ducts (46,XX Mayer-Rokitansky-Küster-Hauser syndrome) or a complete androgen insensitivity syndrome (46,XY testicular feminizing syndrome). Intersex patients, who underwent reassessment of a female sex, need a genital reconstruction toward a feminine phenotype. Patients with congenital adrenogenital syndrome with high urogenital sinus could have a severe hypoplastic vagina. In all these cases, a vaginal replacement is required. We reviewed our experience of vaginal replacement using a sigmoid conduit.
In 34 years, we evaluated 47 patients. The observation period was from 1 to 34 years (mean: 12 years). The preoperative diagnosis was Mayer-Rokitansky-Küster-Hauser syndrome in 17 cases, androgen insensitivity syndrome in 24 cases, adrenogenital syndrome with high urogenital sinus in 5 and 1 patient was affected by penile agenesis. Forty-six patients were treated with vaginal reconstruction by interposition of sigmoid colon. Only in 1 case we performed a vaginal construction with an ileal loop: in this case, the sigmoid colon was extremely dilated by a chronic constipation secondary to a high anorectal malformation corrected at birth.
The outcome for 47 patients is excellent: 18 are sexually active and 4 are married. Only 1 patient with adrenogenital syndrome died of endocrine problems. Complications occurred in 17 cases: in 1 patient a necrosis of the replaced vagina occurred, thus requiring vaginal exeresis; now she is waiting for a second operation. Another patient had an abdominal abscess, which was surgically treated. In 12 cases a second procedure was required: 6 had stenotic new-vaginal introitus, 4 had new-vaginal prolapse, and 2 had intestinal obstruction.
We believe that the preferable technique for vaginal replacement is the use of intestinal conduit. The sigmoid colon is the best intestinal tract to be used owing to its size, location and preserved blood supply. Our experience leads us to believe that the sigmoid segment is the segment of choice, although we consider ileal loop as a good alternative when the sigmoid colon is not available.
背景/目的：儿童中阴道缺失较为罕见。它可能是由于先天性畸形引起的，例如苗勒管发育不全（46，XX Mayer-Rokitansky-Küster-Hauser 综合征）或完全雄激素不敏感综合征（46，XY 睾丸女性化综合征）。需要重新评估为女性性别的两性畸形患者需要进行生殖器重建以达到女性表型。患有高尿生殖窦先天性肾上腺生殖综合征的患者可能会出现严重的阴道发育不良。在所有这些情况下，都需要进行阴道替代。我们回顾了使用乙状结肠管进行阴道替代的经验。
在 34 年中，我们评估了 47 例患者。观察期为 1 至 34 年（平均：12 年）。术前诊断为 17 例 Mayer-Rokitansky-Küster-Hauser 综合征，24 例雄激素不敏感综合征，5 例高尿生殖窦先天性肾上腺生殖综合征，1 例阴茎发育不全。46 例患者采用乙状结肠间置法进行阴道重建。仅在 1 例中我们使用回肠环进行阴道构建：在这种情况下，由于出生时纠正的高位肛门直肠畸形引起的慢性便秘，乙状结肠极度扩张。
47 例患者的治疗结果均良好：18 例有性生活，4 例已婚。仅 1 例患有先天性肾上腺生殖综合征的患者因内分泌问题死亡。17 例患者发生并发症：1 例替代阴道发生坏死，因此需要阴道切除术；现在她正在等待第二次手术。另一位患者发生了腹部脓肿，经手术治疗。12 例需要进行第二次手术：6 例新阴道入口狭窄，4 例新阴道脱垂，2 例肠梗阻。
我们认为，阴道替代的首选技术是使用肠管。乙状结肠是最好的肠段，因为它的大小、位置和保留的血液供应。我们的经验使我们相信，虽然我们认为回肠环是乙状结肠不可用时的良好替代，但乙状结肠段是首选的肠段。
