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泄殖腔杂种|Overview

什么是泄殖腔杂菌(OEIS综合征)?

Cloacal exstrophy, also known as OEIS Syndrome, occurs when a portion of the large intestine lies outside of the body, and on either side of it —and connected to it — are the two halves of the bladder. In boys, the penis is usually flat and short, with the exposed inner surface of the urethra on top. The penis is sometimes split into a right and left half. In girls, the clitoris is split and there may be one or two vaginal openings. Cloacal exstrophy (OEIS syndrome) is a very rare birth defect, affecting 1 in every 250,000 births. Although cloacal exstrophy is a serious condition and requires a series of operations, the long-term outcome is good for many children.

What are the signs of cloacal exstrophy?

In cloacal exstrophy, a baby’s bladder is open in the front and the inner surface is exposed on the lower surface of the abdominal wall. The exposed bladder appears as a right and left half, with the large intestine exposed and connected in the middle. This abnormal connection of the intestine and the bladder allows mixing of stool and urine. Cloacal exstrophy can occur as part of OEIS syndrome, which includes anomphalocele, extrophy, imperforate anus and spinal defects. In an omphalocele, some of the abdominal organs protrude through an opening in the lower abdominal wall muscles in the area of the umbilical cord. A translucent membrane covers the protruding organs. The omphalocele may be small, with only a portion of the intestine protruding, or large, with most of the abdominal organs (including intestine, liver and spleen) protruding outside of the body. In an imperforate anus, the anus has not been formed, and the colon connects to the bladder. Sometimes intestine is also present behind the bladder that is not in continuity. Spinal defects may either be major or minor. Many times, children born with cloacal exstrophy are also born with varying degrees of脊柱裂

What causes cloacal exstrophy?

泄殖腔外翻的原因是未知的。它not appear that any medications or activities of the expectant parents have any impact on the development of cloacal exstrophy. Based on our current knowledge, it is very unlikely that this condition can be prevented.

我们如何照顾泄殖腔卵石

泄殖腔通过一系列操作纠正。临床医生结直肠和骨盆畸形中心at Boston Children’s Hospital have extensive experience in cloacal exstrophy repair and supporting you and your baby throughout the process. Our multidisciplinary team of physicians works together in coordinating initial surgical repair as well as long term follow-up and reconstructive needs.

泄殖腔杂种|诊断和治疗

How is cloacal exstrophy diagnosed?

在某些情况下,可以在产前超声出生前诊断出泄殖腔肿瘤(OEIS综合征),这可以通过磁共振成像(MRI)证实。出生后,您的婴儿医生可以通过身体检查进行或确认这种诊断。

Other diagnostic tests and procedures can include:

  • Magnetic resonance imaging (MRI)
  • 在麻醉和内窥镜检查下检查。这涉及插入一种称为内窥镜的小仪器,以查看空心器官的内部,例如直肠,尿道或阴道
  • 腹部超声(sonography). This imaging method is used to view the anatomy of the internal organs as they function, and to assess blood flow

泄殖腔如何治疗?

泄殖腔外丛需要手术修复。为您的孩子设计的治疗计划将取决于异常的类型和程度。

上演重建

这是多年来涉及多项操作的多步骤处理。手术技术的进步使我们的外科医生团队能够在重建过程中进行合作,以最大程度地减少所涉及的阶段数量。

t这些程序的确切时间,性质和结果将取决于您孩子的特殊情况。您的外科医生将与您讨论您的孩子计划,以及您期望能够成功的计划。

腹部repair

Within the first 24 to 48 hours after your child is born, surgery will likely be performed to repair the omphalocele, to return the protruding organs to the abdomen and close the opening in the abdominal wall. The surgeons will work as a team and include a pediatric urologist and a pediatric colorectal surgeon.

如果可能的话,外科医生会将膀胱与肠道分开,可能关闭膀胱,并为您的孩子取消粪便。一旦肠与膀胱分离,它就会成管。通常,任何其他肠道的肠子都与此管状肠道(一种称为后肠救援的程序)相连。通常,会产生末端造口,以便将肠中的粪便传入收集袋中。这允许尿液和粪便疏散的正常分离。造口后,宝宝的消化不会受到损害。

Your child may also need to have a catheter passed intermittently in order to help eliminate urine or a surgical procedure to reroute urine flow.

一些脊柱缺陷也可能需要修复。这些可以在生命的头几个月中通过手术治疗。

如果您在初次手术后要访问我们,以获取第二意见,有时可能需要进行后肠救援程序。我们的团队将在麻醉下进行仔细检查,并可能进行范围的范围,以确定是否需要进一步手术。

After treatment

下一步是让您的孩子足够健康回家。这涉及使您的孩子从手术中愈合。如果您的孩子过早出生,并确保您准备好照顾孩子的额外需求,从而使您的孩子在身体和情感上都要照顾好自己的额外需求,这也可能涉及允许您的孩子成长。

与婴儿外科医生一起工作的护理人员和其他医疗保健专业人员可以帮助您学习结肠造口术。在此期间,本地和国家支持小组也可能有所帮助。

Subsequent surgery

Depending on the amount of colon your child was born with and the nerve and muscle function in your baby's bottom, surgeons may eventually create a rectum and close the stoma. If your child has a significant amount of colon and is able to form solid stool, a surgical procedure known as a "pull-through" may eventually be performed. This surgery involves opening the abdomen to connect the colon to the rectum.

随后的程序还将涉及重大的尿液重建手术和进一步的生殖器重建。

泄殖腔杂种|Programs & Services

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