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孙俊杰 三甲
孙俊杰 副主任医师
深圳市儿童医院 泌尿外科

输尿管异位开口Ectopic Ureter

输尿管异位开口

我们大多数人天生有两条输尿管,每条从一个肾脏引流尿液进入膀胱。但大自然给了我们中的某一些人超过了正常分配数量的输尿管。在大多数情况下,额外的输尿管不会引起麻烦。然而,如果这些输尿管中哪条连接不正确---引流不正确,会怎么样?对于异位输尿管儿童,额外的一条不是简单多一条的问题。幸运的是,医学给了泌尿科医生很多的的诊断检查和外科手术来处理这种异常。因此,阅读下面来看看你孩子的医生可能怎样去纠正这种情况。深圳市儿童医院泌尿外科孙俊杰

输尿管异位开口的原因是什么?

正常情况下,从每个肾脏仅有一条输尿管将尿液排出到膀胱。尿液然后存储在膀胱,直到人主动排尿。偶尔,一个肾脏有两条输尿管引流尿液。一条输尿管引流肾脏的上部分和第二条输尿管引流肾脏的下部分。只要它们都正常进入膀胱,这种“重复的集合系统”是没有问题的。很少的是,孩子可能天生有输尿管异位开口。这种情况是说,一条输尿管未能正确地连接到膀胱,而把尿液引到了膀胱外的某个地方。在女孩中,输尿管异位开口通常排入尿道,甚至是阴道。在男孩中,它通常排入前列腺附近的尿道或排入生殖道系统。异位输尿管开口可以发生于非重复的集合系统,但更常见于重复的系统。

输尿管异位开口的症状是什么?

输尿管堵塞或无法控制排尿(尿失禁)可以预示输尿管异位开口。引流不畅,伴有相反的压力,可能会导致输尿管及其所服务的肾脏部分变得扩张或膨胀。这种状况被称为肾积水,在超声上可容易发现。出于这个原因,许多输尿管异位的婴儿是在孕母进行产前超声检查时被检测到的。然而,并非所有的输尿管异位都是积水的,因此他们不能通过超声波来检测到。

输尿管异位开口引流不畅可能会让孩子更容易有尿路感染。除了肾积水,女孩输尿管异位开口可能导致尿失禁,因为输尿管引流的尿液会直接进入阴道或阴道附近。这个问题在如厕训练后会变得更明显。它通常与其他形式的女孩尿失禁有区别,因为这种尿失禁是一种持续性的滴尿湿裤子,而不是膀胱失去控制地发作。有些女孩在输尿管异位开口正确诊断之前,会用药物或其它疗法治疗了很多年。输尿管异位开口的男孩一般没有尿失禁,因为输尿管异位开口把尿液引流到体内。然而,它们可能仍然显示出肾积水或尿路感染的症状。

当存在输尿管异位开口时,在肾脏和膀胱之间的正常输尿管连接也可以有轻微缺陷。这种缺陷可能会导致膀胱输尿管返流,尿液从肾脏通过输尿管、膀胱最后从尿道排出的这一经过受到破坏。随着反流,由于膀胱充盈或排空,一些尿液流回到肾内。膀胱输尿管反流将患者置于肾脏感染的较高风险之中,这也是一些输尿管异位开口儿童表现尿路感染迹象的另一个原因。

如何诊断输尿管异位开口?

输尿管异位开口的评价取决于病人(通常是孩子)所表现的问题。例如,如果产前超声检测到肾积水,然后孩子出生后通常会再次超声检查。然后会进行一种称为排泄性膀胱尿道造影(VCUG)的膀胱X线检查,来排作为肾脏和输尿管的肿胀原因的膀胱输尿管返流。该VCUG还用于确定与输尿管异位开口相关的第二条输尿管是否有回流。一般结合超声和VCUG结果,医生就可以确定是否有肾积水。有时,其他诊断性检查,如肾血流扫描或者一种被称为静脉肾盂造影(IVP)的正规肾X线检查,均可能有助于搞清楚解剖。经输尿管异位开口引流的肾脏或者部分肾脏的功能往往较差。这可以用肾血流扫描来评估。这两种检查都涉及注射被肾脏摄取的造影剂,然后通过标准的X线图像(对于IVP)来显示,或者通过用于检测染料中少量的放射性(对于肾血流扫描)的特殊照相机来显示。此功能信息可能对选择治疗的方式很重要。最后,可能会进行膀胱镜检查(通常在决定性治疗的时间)。在此检查中,通常在全身麻醉下进行,小型镜头被置入尿道和阴道,确认来自两侧肾脏的输尿管的开口。不幸的是,不是总能看到异位输尿管的开口。然而,通过识别其他输尿管开口的数量和位置,通常是可以确定诊断的。

当一个孩子表现尿失禁症状,通常进行相同的系列检查。然而,如果输尿管不肿大,并且没有相关联的反流,超声和VCUG可以是正常的。如果症状提示异位输尿管开口,那么有时这在肾血流扫描或IVP中可以看出。偶尔,需要CT扫描去看异位输尿管和它所引流的肾脏部分。由于儿童尿失禁的其它原因十分常见,诊断并不总是很容易,有的孩子得到诊断前,可能已尿失禁很年了。

异位输尿管开口如何治疗?

治疗异位输尿管开口的方法就是手术。为了控制感染的风险,患者可在手术前予以低剂量抗生素。

有三种手术方式 ---- 肾切除术,肾盂输尿管吻合和输尿管再植术 ---- 去解决这个问题,每个术式都有优点和缺点。

肾切除术(上极半肾切除术):在这个手术中,肾脏或异位输尿管引流的部分肾脏被切除。这将使尿液停止流入异位输尿管,从而治愈尿失禁,并减少感染的机会。技术上操作最简单,并发症风险也最低。当通过异位输尿管引流的肾脏或肾脏部分功能很差时,这是特别具有吸引力的。如果对侧肾脏功能正常,它也可以用于部分肾脏正常时。此手术传统上是通过肋下切口进行的,但现在有一些患者可以在腹腔镜下完成。主要缺点是潜在功能的肾组织可被切除掉,而且异位输尿管的底端被留下。虽然通常这都不是问题,异位输尿管的剩余部分可能是一个未来感染的来源。

肾盂输尿管吻合术:在此手术中,异位输尿管在靠近肾脏的位置被截断,然后缝合到肾下部的正常集合系统。这使得从肾脏上部来的尿液得到正常引流。这具有保护所有肾组织的优势,但仍然会在原位留下底部的异位输尿管。它的并发症发生率也稍高于其它手术。

输尿管再植:在该手术中,异位输尿管在底部附近被截断,然后缝合到膀胱,通过这样一种方式,尿液引流好且不会反流。通常是通过耻骨上切口进行,该手术比其他两种手术具有稍高的并发症率。如果对小婴儿进行这个手术,技术上也会困难。然而,如同肾盂输尿管吻合术,此手术将保留所有的肾脏组织。此外,它比其他两个手术切除了更多异常的异位输尿管,而且使得外科医生能止住任何膀胱输尿管反流。

输尿管异位开口治疗后会有什么情况?

恢复取决于所选择的手术。但是,婴儿和幼儿通常手术后住院一至五天。手术时可能会留有一条小导管,这条管可能在孩子回家前或在随访时在诊室被无痛地很快拔除。该导管进去位置的那个微小切口,会自行愈合而不需要缝合。

常见问题:

是男孩还是女孩更容易有输尿管异位开口呢?

这种情况是女孩比男孩更常见,但两种性别均可以发生。

异位输尿管手术的最佳年龄是多少?

婴儿满月后可以随时进行肾切除术和半肾切除术。一些外科医生倾向于等到孩子长大一些,通常在满一岁后再进行输尿管膀胱再植术。

什么会是异位输尿管的孕期风险因素?

异位输尿管没有已知的危险因素。它是一个先天性问题,可能发生于在输尿管和膀胱之间连接的发育失败。

这是怀孕期间发生了一些事造成的吗?

也没有证据表明,这种异常是由母亲怀孕期间做过什么或者是暴露于什么而引起的。

异位输尿管开口对我孩子未来的性功能是否产生任何影响?

虽然输尿管异位开口会引流到生殖道,它不会影响性功能,也很少会损害生育能力。对于男孩,与异位输尿管同侧的生殖道可能有异常,但如果对侧不受影响(这是通常的情况),则生育力仍应该是正常的。

我其他孩子有异位输尿管的危险吗?

肾脏集合系统的重复畸形通常是作为常染色体显性基因遗传的,这意味着每个兄弟或姐妹有二分之一的机会具有两条输尿管引流一个或两个肾脏。如果两条输尿管都进入膀胱,然而,他们应该不会遇到任何泌尿问题或者需要对这种状况手术。患有异位输尿管的孩子应当注意,他的每个子女有二分之一的可能会患有集合系统重复畸形,但是,在大多数情况下,将不存在异位输尿管。

如果部分或全部肾脏功能不佳或被切除,我的孩子会有终身性的肾脏问题吗?

不会的,只要另一个肾脏是正常的。大多数的异位输尿管只会影响到一个肾脏的上部,它只提供身体三分之一的肾功能。即使当整个肾脏受到影响,也不太可能有长期的问题。儿童经常有天生的单肾不为人所知,捐了一个肾的人也会生活得很好。这唯一意味着,病人不再有“备用的”肾。因此,万一在事故中,伤害了他们唯一的肾脏,如果进行了一个全肾切除术,那么他们将发展成肾功能衰竭。如果肾切除术中只是切除了肾脏的一部分,那么患者长期情况也会很好。

 

Ectopic Ureter

Most of us are born with two ureters, one to drain the urine from each kidney into the bladder. But nature has given some of us more than the normal allotment. In most cases, a bonus ureter causes no problems. Yet what if one of these ureters it is not connected correctly - and drains incorrectly? That is the case for children with an ectopic ureter, a bonus that is not a plus. Luckily, medicine has given urologists a bevy of diagnostic tests and surgical techniques to deal with this abnormality. So read below to see how your child's doctor might correct this condition.

What are the causes of ectopic ureter?

Normally, there is a single ureter draining the urine from each kidney to the bladder. The urine is then stored in the bladder until one voluntarily urinates. Occasionally, there may be two ureters draining a single kidney. One ureter drains the upper part of the kidney and the second ureter drains the lower portion. So long as they both enter the bladder normally, this "duplicated collecting system" is not a problem. Rarely a child may be born with an ectopic ureter. This is a ureter which fails to connect properly to the bladder and drains somewhere outside the bladder. In girls, the ectopic ureter usually drains into the urethra or even the vagina. In boys, it usually drains into the urethra near the prostate or into the genital duct system. An ectopic ureter can occur in a non-duplicated collecting system but is more common in a duplicated system.

What are the symptoms of ectopic ureter?

Blockage of the ureter or the inability to control urination (incontinence) can indicate an ectopic ureter. Poor drainage, accompanied by back pressure, can cause the ureter and portion of the kidney it services to become distended or swollen. This condition is called hydronephrosis and can be spotted easily on an ultrasound. For this reason, many babies with an ectopic ureter are detected when the pregnant mother undergoes a prenatal ultrasound. However, not all ectopic ureters are hydronephrotic so they may not be detected by an ultrasound.

Poor drainage from an ectopic ureter may make children more likely to have urinary tract infections. In addition to hydronephrosis, ectopic ureters in girls may cause incontinence since the ureter drains urine directly into or near the vagina. This problem becomes evident after toilet-training. It is usually distinguished from other forms of incontinence in girls because the incontinence is a constant dripping moistness rather than episodes of loss of bladder control. Some girls will be treated with medication and other therapies for many years before the correct diagnosis of an ectopic ureter is made. Boys with ectopic ureters do not generally have incontinence since the ectopic ureter drains inside the body. However, they may still show symptoms of hydronephrosis or a urinary tract infection.

When an ectopic ureter is present, there may also be a slight flaw in the normal ureter's connection between the kidney and bladder. This flaw can result in vesicoureteral reflux, a disruption of the passage of urine from the kidney, through the ureter, to the bladder and finally out the urethra. With reflux, as the bladder fills or empties some urine flows backward into the kidney. Vesicoureteral reflux places patients at a higher risk for kidney infections and is another reason some children with ectopic ureters show signs of a urinary tract infection.

How is ectopic ureter diagnosed?

The evaluation of an ectopic ureter depends on the problem shown by the patient (usually a child). For instance, if hydronephrosis is detected on a prenatal ultrasound, then the ultrasound is usually repeated after the child is born. A bladder X-ray, called a voiding cystourethrogram (VCUG) is then taken to rule out vesicoureteral reflux as the cause for swelling of the kidney and ureter. The VCUG is also used to determine if there is reflux in a second ureter associated with the ectopic ureter. Usually with the combination of an ultrasound and a VCUG the doctor can determine if there is hydronephrosis. Sometimes other diagnostic studies such as renal flow scan or a formal kidney X-ray, called an intravenous pyelogram (IVP), may help to clarify the anatomy. The kidney or portion of the kidney drained by the ectopic ureter often functions poorly. This can be assessed with a renal flow scan. Both tests involve an injection of contrast dye picked up by the kidney and then seen either by standard X-ray pictures (for an IVP) or with a special camera for detecting small amounts of radioactivity in the dye (for the renal flow scan). This functional information may be important in selecting the form of treatment. Finally, a cystoscopy may be performed (often at the time of definitive treatment). In this test, usually performed under a general anesthesia, a small telescope is placed into the urethra and vagina and the openings of the ureters from both kidneys are identified. Unfortunately, the ectopic ureter's opening cannot always be identified. However, by identifying the number and location of the other ureter openings, the diagnosis can usually be confirmed.

When a child shows symptoms of urinary incontinence, the same sequence of tests is usually undertaken. However, if the ureter is not swollen and there is no associated reflux, the ultrasound and VCUG may be normal. If the symptoms suggest an ectopic ureter, then sometimes this can be seen on a renal flow scan or IVP. Occasionally, a CT scan is needed to see the ectopic ureter and the portion of the kidney it drains. The diagnosis is not always easy to make and since other causes of incontinence are very common in children, some children may be incontinent for years before the diagnosis is made.

How is an ectopic ureter treated?

The treatment for ectopic ureter is surgery. To control the risk of infection, the patient may be placed on a low dose of antibiotics prior to surgery.

While there are three surgical techniques – nephrectomy, ureteropyelostomy and ureteral reimplantation – to correct this problem, each has advantages and disadvantages.

Nephrectomy (upper pole heminephrectomy): In this surgery, the kidney or the portion of it drained by the ectopic ureter is removed. This stops the flow of urine into the ectopic ureter, thus curing the incontinence and reducing the chance of infection. Technically the simplest operation, also has the lowest complication risk. It is particularly attractive when the kidney or portion of the kidney draining through the ectopic ureter is functioning poorly. It may also be used when that kidney portion is functioning properly if the opposite kidney is normal. This operation has been traditionally performed through an incision under the ribs but can now be done laparoscopically in some patients. The main disadvantages are that the potentially functioning kidney tissue may be removed and the bottom end of the ectopic ureter is left in place. While usually not a problem, the remaining part of the ectopic ureter can be a future source for infection.

Ureteropyelostomy: In this procedure, the ectopic ureter is divided near the kidney and sewn into the normal collecting system of the lower part of the kidney. This allows the urine from the upper part of the kidney to drain normally. It has the advantage of protecting all the kidney tissue but still leaves the bottom half of the ectopic ureter in place. It also has a slightly higher complication rate than the other operations.

Ureteral reimplantation: In this operation, the ectopic ureter is divided near the bottom and sewn into the bladder in such a way that urine drains well and does not flow backwards. Usually performed through an incision above the pubic bone, this procedure has a slightly higher complication rate than the other two surgeries. It can also be technically difficult if performed in small infants. However, like ureteropyelostomy, this operation preserves all kidney tissue. Furthermore, it removes more of the abnormal ectopic ureter than the other two procedures and allows the surgeon to stop any vesicoureteral reflux.

What can be expected after treatment for ectopic ureter?

Recovery depends on the operation selected. However, infants and small children are usually hospitalized from one to five days after the surgery. A small catheter may be left at the time of surgery, which is removed painlessly and quickly before the child goes home or in the office at a follow-up visit. The small openings, where the catheter went in, heal on their own without the need for stitches.

Frequently asked questions:

Are boys or girls more likely to have an ectopic ureter?

This condition is more common in girls than boys, but can occur in either sex.

What is the optimal age for ectopic ureter surgery?

Nephrectomies and heminephrectomies can be performed anytime after an infant reaches one month. Some surgeons prefer to wait until a child is older, usually after a first birthday, to perform a ureteral reimplantation.

What are the risk factors for an ectopic pregnancy?

There are no known risk factors for an ectopic ureter. It is a congenital problem that probably occurs because of a failure in the development of the connection between the ureter and bladder.

Was this caused by something that happened during pregnancy?

There is also no evidence that this abnormality is caused by anything a mother does or was exposed to during pregnancy.

Does an ectopic ureter have any impact on my child's future sexual function?

Although an ectopic ureter drains to the genital tract, it does not affect sexual function and rarely impairs fertility. In boys, the genital tract on the same side of the ectopic ureter may be abnormal but if the other side is unaffected (which is usually the case), then fertility should still be normal.

Are my other children at risk for an ectopic ureter?

The duplicated drainage system of the kidney is usually transmitted genetically as an autosomal dominant condition, meaning that each brother or sister has a one-half chance of having two ureters draining one or both kidneys. If both ureters drain into the bladder, however, they should not experience any urological problem or require surgery for the condition. The child with an ectopic ureter should be advised that each of their children have a one-half likelihood of having a duplicated drainage system, but that in most cases an ectopic ureter will not be present.

If part or all of a kidney is functioning poorly or removed, will my child have life-long kidney problems?

No, not as long as the other kidney is normal. Most ectopic ureters affect just the upper part of one kidney, which provides only one-third of that kidney's function to the body. Even when an entire kidney is affected, long-term problems are unlikely. Children are frequently born with a single kidney and never know it and patients who donate a kidney also do fine. The only implication is that the patient no longer has a "spare" kidney. Therefore, in the unlikely event that the person was to injure their only kidney in an accident, then they would develop kidney failure if a complete nephrectomy were performed. If only a portion of the kidney was removed during the nephrectomy, the patient will do very well in the long-term.

 

中山大学附属第一医院小儿外科

孙俊杰医生

Department of Pediatric Surgery, the First Affiliated Hospital of Sun Yat-Sen University

Dr. SUN Junjie

 

 

孙俊杰
孙俊杰 副主任医师
深圳市儿童医院 泌尿外科
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