Gouw A S, Elema J D, Bink-Boelkens M T, de Jongh H J, ten Kate L P
Eur J Pediatr. 1985 Nov;144(4):316-23. doi: 10.1007/BF00441771.
Splenogonadal fusion is a rare congenital malformation in which there is an abnormal fusion between the spleen and the gonad or derivatives of the mesonephros. It can occur in both sexes but has been reported far more frequently in males. There are two types of this malformation: the continuous and the discontinuous type, depending on the presence or absence of a structural connection between the regular spleen and the ectopic splenic tissue that is fused to the gonad. In one-third of all reported cases splenogonadal fusion is associated with other congenital defects. This association is predominantly found within the continuous type. Peromelia is present in a high percentage of cases, varying from total absence of all limbs to absence of parts of the lower limbs. On the basis of a case report and a review of 84 published cases, the causal, pathogenetic and nosologic aspects of this malformation are discussed. We hypothesise that splenogonadal fusion with peromelia and splenogonadal fusion without peromelia represent two ends of one spectrum determined by the developmental stage during which the causal factor acts.
脾性腺融合是一种罕见的先天性畸形，表现为脾脏与性腺或中肾衍生物之间的异常融合。两性均可发生，但据报道在男性中更为常见。这种畸形有两种类型：连续型和间断型，取决于正常脾脏与融合到性腺的异位脾组织之间是否存在结构连接。在所有报道的病例中，三分之一的脾性腺融合与其他先天性缺陷有关。这种关联主要见于连续型。四肢不全在高比例病例中存在，从所有肢体完全缺如到下肢部分缺如不等。基于一例病例报告和对84例已发表病例的回顾，讨论了这种畸形的病因、发病机制和疾病分类学方面。我们推测，伴有四肢不全的脾性腺融合和不伴有四肢不全的脾性腺融合代表了由致病因素起作用的发育阶段所决定的同一谱系的两端。
