Hirschsprung Disease

Changing poopy diapers is one of those unpleasant tasks of parenting that most people are able to joke about. But when your baby is unable to poop, this topic is no laughing matter.

Babies who have trouble emptying their bowels sometimes have a problem called Hirschsprung disease. Treatment for this condition almost always requires surgery. Fortunately, most children who have surgery are fully cured and able to pass bowel movements normally.

About Hirschsprung Disease

Hirschsprung（Hersh-sproong）疾病影响新生儿，婴儿和幼儿的大肠（结肠）。由于冒号的下部缺失神经细胞导致的状态 - 阻止肠道运动（粪便）通过肠道 - 是由出生缺陷引起的。大多数时候，大量的问题在出生时开始，虽然在较温和的情况下可能出现几个月或几年后的症状。

Hirschsprung disease can cause constipation, diarrhea, and vomiting and sometimes lead to serious colon complications, like小肠结肠炎和中毒性巨结肠life-threatening. So it’s important that Hirschsprung disease be diagnosed and treated as early as possible.

原因

The large intestine moves digested material through the gut by a series of contractions calledperistalsis。This is controlled by nerves in between the layers of muscle tissue in the intestine.

Hirschsprung疾病的儿童沿着他们冒号的一部分缺少那些神经。这可以防止冒号放松，这可能导致消化的材料堵塞并使其难以通过粪便。

Hirschsprung病可以影响整个大肠，称为长段病，或者它可以影响更接近直肠（短段疾病）的结肠长度。神经细胞更常见的是停止更接近直肠，因为在子宫细胞上发生在途径上，该途径在大肠顶部开始，并且在末端靠近直肠附近。（因此，随着Hirschsprung疾病，神经细胞只是在那种途径上停止发展。）

医生并不完全确定为什么有些孩子得到Hirschsprung疾病，但他们确实知道它可以在家庭中奔跑，比女孩更频繁地影响男孩。事实上，Hirschsprung疾病比女性在男性中常见约为5倍。患有综合征和遗传心脏病的儿童也有患有Hirschsprung病的风险增加。

症状

Hirschsprung疾病的症状可能因病症的严重程度而有所不同。严重患者的儿童通常会在生命的前几天内有症状。直到稍后，可能无法发现升级案件。

Newborns with Hirschsprung disease may show these signs:

• 无法在生命的第一个或第二天中通过粪便
• 肿胀的腹部，腹胀或煤气
• diarrhea
• vomiting, which may include vomiting a green or brown substance

在生命的前48小时内无法大便，往往是在新生儿中检测Hirschsprung病的关键。这种红旗可以在诊断条件时非常有价值。

Less severe cases of Hirschsprung disease may go unnoticed until later childhood, or sometimes even adolescence or adulthood. The symptoms in these cases are usually milder but can be long-lasting, chronic conditions. Symptoms may include:

• 腹部肿胀
• constipation
• 难以获得体重
• vomiting
• 气体

由于Hirschsprung疾病会影响身体吸收营养的能力，因此增长可能会延迟拥有它的老年人。

诊断

为了诊断Hirschsprung疾病，医生经常叫做一个测试barium enema。钡是一种使用灌肠将结肠的染料。钡在X射线上发表更好，可以帮助医生了解冒号的图片。（在患有Hirschsprung疾病的孩子中，肠道通常看起来太窄，在神经细胞缺失。）

In some cases, the doctor might do a直肠抽吸活组织检查。This test, which can often be done in the office, involves using a suction device to remove some cells from the colon’s mucous lining. This test will reveal whether nerve cells are missing and can help make the diagnosis of Hirschsprung disease.

对于年龄较大的孩子，医生可以使用不同的测试，例如manumetr.or asurgical biopsy。Manometry is a test in which a balloon is inflated inside the rectum to see if the anal muscle relaxes as a result. If the muscle doesn’t relax, the child may have Hirschsprung disease. In a surgicalbiopsy, the doctor removes a sample of tissue from the colon to examine under a microscope.

Treatment

手术被认为是Hirschsprung疾病最有效的治疗方法。这可以在一步中完成，具体取决于条件的严重程度。在手术时（由于发炎的结肠或营养差）非常恶心的儿童可能需要两步进行手术。

The most common surgery to correct Hirschsprung disease involves removing the section of the colon without nerves and reattaching the remaining portion of the colon to the rectum. Often, this can be done through minimally invasive (laparoscopic) surgery immediately after the condition is diagnosed.

在某些情况下，医生可以分两步进行手术。在第一步中，医生将移除冒号的不健康部分，然后执行一个名为AN的过程ostomy.。In an ostomy, the doctor creates a small hole, or stoma, in the child’s abdomen and attaches the upper, healthy portion of the colon to the hole.

两种类型的造口术是：

1. 奥洛洛搬弄：去除整个大肠并将小肠连接到造口
2. Colostomy：删除冒号的一部分

The child’s stool passes through the stoma into a bag that is connected to it and needs to be emptied several times a day. This allows the lower part of the colon to heal prior to the second surgery. In the second surgery, the doctor closes up the hole and attaches the normal portion of the colon to the rectum.

After surgery, kids often getconstipated。泻药可以提供一些救济，但如果您决定使用它们，请与您的医生联系这是最适合您的孩子。对于足够大的孩子吃固体食物，高度 -fiber饮食可以缓解和防止便秘。喝大量水也很重要，有助于预防脱水。The large intestine helps absorb water from food, so dehydration can be a concern for children who have had part of their intestine removed.

在手术后继续有症状或开发新的孩子（如爆炸性和水腹泻，发烧，肿胀的腹部或从直肠出血）应该立即进行医疗注意。这些可以是小肠结肠炎的迹象，肠道炎症。

Outlook

The general outlook for children who have been treated surgically for Hirschsprung disease is excellent. After surgery, most can pass stool normally and have no lasting complications. However, a few kids might continue to have symptoms, including constipation and bowel control problems.

审查：J.Fernando del Rosario，MD
Date reviewed: January 2015