输卵管解剖变异和先天性异常及其对生育能力的影响概述

输卵管的解剖变异和先天性异常 (UTAVsCAs) 是罕见病症，通常未被确诊，或因不相关病症在影像学、剖腹手术、腹腔镜检查或剖宫产期间意外确诊。 UTAVsCA 通常没有症状，但它们的临床相关性在于它们可能对生育能力产生不利影响。 由于它们很少发生，它们通常作为病例报告发表。 最典型的描述是：输卵管发育不全 (UT)、附件 UT（UT 重复）、附件 UT 口和输卵管旁囊肿（例如 Morgagni 包虫囊肿）。 UTAVsCA 被归类为苗勒管异常 (MDA) 的总括类别，其中包括从副中肾 (苗勒) 管发育的所有器官的异常发育，即 UT、子宫和阴道上部。 有趣的是，大多数 MDA 分类系统仅讨论子宫和阴道异常，而 UT 常常被完全忽略。 这可能源于这样一个事实，即许多临床医生不再对 UT 感兴趣，因为他们认为 UT 是多余的器官，其功能可以在体外受精 (IVF) 实验室中轻松替换。 事实上，现代生殖医学对不育夫妇的受孕有很大帮助。 在许多情况下，UTs 实际上被成功绕过，一个“试管”婴儿诞生了。 尽管如此，UT 在为受精和早期胚胎发育提供合适的环境方面仍然是绝对独特的 - 这些过程尚未完全了解。 这一事实可以部分解释为什么 IVF 的成功率“仅”在 30-50% 左右，具体取决于年龄。 因此，UTAVsCA 的研究在生殖医学的背景下仍然具有临床意义，不应从研究工作中省略。

子宫或输卵管(UT) 是大约 10 厘米长的肌肉管，从每个卵巢附近延伸到子宫角。 UT 占据称为输卵管系膜的阔韧带的上部。 UT 可以细分为几个解剖区域。 离卵巢最近的是漏斗状部分，漏斗部通向腹膜腔的骨盆部分。 漏斗部的边缘有指状突起，即伞。 在排卵期间，卵子被漏斗部捕获，并通过输卵管上皮衬里的纤毛跳动向子宫方向输送。 下一个解剖部分是宽壶腹（通常的受精部位），然后是较窄的峡部，最后是在子宫壁内延伸的 UT 部分，即壁间（子宫）部分。 UT 的子宫/壁内部分有一个小管腔，直径为一毫米或更小，尽管管腔的尺寸趋于向卵巢末端增加（直径约 2 毫米或更小）。 UT 在精子和卵子/早期胚胎运输过程中以及通过衬里上皮分泌物提供的营养资源对卵子/早期胚胎的支持中起主要作用。 最近的研究表明，由于表观遗传编程，UT 微环境对于胚胎发育及其未来成年后的健康至关重要。

从历史的角度来看，UTs研究的主要突破可以追溯到启蒙时期，也就是所谓的“理性时代”，主要强调严谨的科学研究。 就在这个时期，有史以来最伟大的解剖学家之一 Frederik Ruysch（1638-1731 年）出生于荷兰。 在他积极的职业生涯中，他能够解剖和记录无数正常和异常的标本，其中许多仍然是圣彼得堡（俄罗斯）彼得大帝人类学和民族志博物馆（Kunstkamera）展览的组成部分 ). 其中一个展品是所谓的“案例 47”，它描述了用三个 UT 对女性内生殖器进行的解剖准备。 处于科技黄金时代的荷兰是改变游戏规则的科学家的无底洞。 另一个 UT 异常，即其异常长度首先由 Regnier de Graaf 在他的著名著作 De Mulierum Organis Generationi Inservientibus 1672 中首次描述，该著作仅在他英年早逝前一年出版。 根据异常的类型和严重程度，它可能完全没有被注意到，或者最初的症状直到青春期、第一次性交或生育问题才会变得明显。 施勒等人总结说，目前女性生殖系统先天性异常 (FRSCA) 的发病率估计在普通人群中约为 0.2-0.4%，在诊断为和/或治疗不孕症的患者中上升至 3-13%。 然而，估计这种罕见病症的真实发病率几乎是不可能的，因为它们通常是偶然被诊断出来的。 尽管输卵管的解剖变异和先天性异常 (UTAVsCAs) 属于副中肾缪勒管异常 (MDAs) 的总括类别，但它们偶尔被提及和分类。 在大多数情况下，重点主要放在子宫异常上。 引人注目的是，罗宾斯等人，发表了一篇关于 MDA 的评论论文，其中作者指出 MDA 是影响子宫、子宫颈和阴道的先天性异常。 他们的定义中甚至没有提到 UT。

这篇综述文章的主要目的是从胚胎起源、诊断、管理、分类和与辅助生殖技术相关的临床意义的角度，概述这些重要但被忽视的器官的选定解剖变异和先天性异常。

输卵管解剖变异和先天性异常及其对生育能力的影响概述

图 1
美国生育协会对Müllerian异常的分类。

输卵管解剖变异和先天性异常及其对生育能力的影响概述

图 2
完全单侧发育不全。

输卵管解剖变异和先天性异常及其对生育能力的影响概述

图 3
节段性（近端）发育不全。

输卵管解剖变异和先天性异常及其对生育能力的影响概述

图 4
中段发育不全。

输卵管解剖变异和先天性异常及其对生育能力的影响概述

图 5
附件 UT 连接到正常 UT 的壶腹。

输卵管解剖变异和先天性异常及其对生育能力的影响概述

图 6
附属口。

输卵管解剖变异和先天性异常及其对生育能力的影响概述

图 7
Morgagni 包虫囊肿。

尽管在某些情况下很少发生、经常没有症状、意外诊断或临床意义不大，但 UTAVsCA 仍然是一个高度相关的话题。 第一个原因是，更好地了解任何先天性异常可以拓宽对正常和异常胚胎发育的大量知识。 这很重要，因为这些见解可以进一步转化为其他临床相关和相关的研究领域，例如其他 MDA 的调查和管理，例如子宫异常。 第二个原因在于误诊的可能性，这可能导致未经证实的诊断，如特发性不孕症。 诚然，整个生殖医学领域的现代成功，尤其是体外受精 (IVF) 技术可以处理广泛的不孕症输卵管因素。 这就是为什么许多研究人员和临床医生认为 UT 不再值得研究的原因，因为它们很容易被 IVF 技术绕过。 然而，即使是最先进的 IVF 技术也无法完全复制输卵管微环境，从而导致各种并发症，包括移植胚胎植入失败。 任何关于发育异常或成熟 UT 的基础研究都具有丰富临床实践的巨大潜力，这符合所有不育夫妇的最大利益。 不孕症在全球范围内一直在上升，而且这种趋势估计会继续下去，今天不得不面对更糟糕的数字。 认为认为研究 UT 不值得付出努力的观点是短视的，可能会在未来造成可怕的后果。

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