Abstract
In this report of a 13-year old boy, we describe the first case of splenogonadal fusion (SGF) associated with growth hormone deficiency, delayed skeletal maturation and stunting, and provide a review of the literature on SGF based on a search in major medical indexes using the descriptors splenogonadal, splenogonadal fusion and congenital anomalies of the spleen. Predominant in males (15:1), SGF is a rare congenital anomaly with only around 200 cases described involving the presence of splenic tissue in the gonads and, in some cases, a fibrous cord connecting the two structures. SGF may be associated with severe limb anomalies, micrognathism and testicle cancer. Despite frequently increased testicle volume, the condition is usually asymptomatic. Knowledge of SGF is important in the differential diagnosis of malignancies, avoiding unnecessary orchiectomy. Nevertheless, SGF has been observed in association with malignant tumors, especially in patients with cryptorchidism.
Highlights
Splenogonadal fusion (SGF) is a rare congenital anomaly resulting from a connection between the spleen and the gonad or mesonephric remnants during gestation
In this report of a 13-year old boy, we describe the first case of splenogonadal fusion (SGF) associated with growth hormone deficiency, delayed skeletal maturation and stunting, and provide a review of the literature on SGF based on a search in major medical indexes using the descriptors “splenogonadal”, “splenogonadal fusion” and “congenital anomalies of the spleen”
A number of theories have been proposed to explain SGF. The earliest of these in 1913 suggests that inflammation between the peritoneal surfaces over the gonadal ridge and spleen could produce an adhesion or partial fusion of the two organs [6]. Another theory postulates the existence of a retroperitoneal pathway allowing communication between the splenic tissue and the gonad or mesonephric remnants [7]
Summary
Splenogonadal fusion associated with delayed skeletal maturation: A case report and review of the literature. José Urbano de Medeiros Neto, Carlos Gustavo Hirth, Conceição Aparecida Dornelas, João Batista Gadelha de Cerqueira, Dennis Tomio Fujiike, Emília Tomé de Sousa
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