Rare congenital malformation. It mainly affects boys (95 %) and the left side (98 %). It is the result of an abnormal connection between the primitive spleen and the gonad or another derivative of the mesonephros during gestation (between the 4th and 10th weeks): part of the splenic tissue then accompanies the gonad during its descent. 

Splenogonadal fusion may be :

-   continuous (55 %):         a  trans- or retroperitoneal parenchymatous or fibrous cord,, joins the spleen to its ectopic nodule (photo). This form is often (48 %) associated with other anomalies: micrognathia, microglossia, anorectal malformation, cranial anomalies, spina bifida, crytorchidism, pulmonary hypoplasia. In the case of limb anomalies, it is called Splenogonadal Fusion with Limb Defects (SFLD), which is similar to Hanhart syndrome and faciofemoral syndrome. This malformation has been described in some cases of Moebius and Poland syndromes (see these terms).

- discontinuous (45 %):         there is no connection between the spleen and the ectopic splenic tissue, which is sometimes an accessory spleen. The ectopic splenic nodule may lie along the spermatic cord, in the epididymis, or in direct contact with the testicular parenchyma. 

Clinical presentation: often discovered incidentally during surgery for cryptorchidism (continuous form) or testicular mass (discontinuous form).

Anesthetic implications: 

 none, except those of the possibly associated syndromes.

References : 

-        Margi M, Boumart K, Nkhili H, Oulahyane R, Cherkaoui A, Abdelhak M, Benhmamouch MN.
Fusion splénogonadique : à propos d’un cas.
Arch Pédiatr 2010; 17: 42-4

-        Fernandez Atuan R, Burgues Prades P, Gonzalez Esgueda A, Ruiz de Temiño M, Estors Sastre B, Bragagnini Rodruiguez P, Alvarez Garcia N, Elias Pollina J.
Splenogonadal fusion: Case report and review of literature.
J Ped Surg Case Reports 2013 ;  1 : 375-7

-        Patre V, Netam S, Luka P, Mandle H.
Discontinuous type of splenogonadal fusion syndrome with limb defects.
Indian J Urol 2012;28:94-5.

-        Salehi M, Kenari FA, Ghasemi M, Rehman S, Salehi M, Jafari HR.
Splenogonadal fusion: A rare case report and literature review.
International Journal of Surgery Case Reports 2023 ; 109 : 108480

Updated: January 2024