[MIM 183 300]

(SFLD, acronym for Splenogonadal Fusion with Limb Defects)

Rare. Association of abnormal fusion of the spleen with a gonad (or more rarely with remnants of the mesonephros) with limb anomalies (severe amelia or reduction leading to rudimentary upper and/or lower limbs), and orofacial anomalies such as cleft palate, bifid uvula, microglossia and mandibular hypoplasia. Other malformations may be associated, such as cryptorchidism, anal stenosis/atresia, hypoplastic lungs and cardiac malformations.

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

Updated: January 2024