What to Do in Case of Splenogonadal Fusion in Non Palpable Testis?

Splenogonadal fusion (SGF) is a rare congenital anomaly characterised by abnormal association between aberrant accessory splenic tissue and the gonads [1, 2]. In literature, almost 221 case reports described this condition [3] and it is most frequent in male on the left side. There is underestimation of SGF occurrence in females because females' gonads are inside the body and therefore less accessible for examination. This condition is often found incidentally in evaluation of cryptorchidism, inguinal hernia, or testicular mass [1, 3, 4]. The embryologic mechanism is uncertain, although theorised to originate between the fifth and sixth weeks of gestation before the start of gonadal descent. The close proximity between the left gonad and spleen during embryogenesis explains the fact that SGF almost always occurs on the left side [1, 5]. SGF is classified in two types as discontinuous and continuous. The first one is fusion of accessory spleen tissue with gonadal derivatives without direct connection to the spleen. The latter one is a direct cord-like connection between the spleen and gonadal derivatives [6]. This is a case of a 12-month old male infant presented with prenatal diagnosis of aortic coarctation and dysplastic aortic valve. At birth, the patient was diagnosed with bilateral cryptorchidism and genetic evaluation showed a genetic duplication (22q11). In the first week of life, he underwent coarctectomy and ligation of patent ductus. We performed an ultrasound visualising the left and right testes in the lower abdominal cavity and normal appearance of abdominal organs. At 10 months of life, laparoscopic examination showed an undescended right testis close to the bladder and on the left side multiple accessory spleen with the distal one attached with the left undescended testis (Figure 1). We performed on the right side a laparoscopic orchiopexy placing the testis in the apical part of the scrotum. On the left side we decided to perform the first stage Fowler-Stephen orchiopexy. Six months later, the left testis appeared well trophic, therefore a dissection between accessory spleen and the testis was done with a preservation of all accessory spleens. The second stage Fowler-Stephen was successfully performed. The patient had an uneventful recovery and follow up after surgery revealed no abnormalities. In conclusion, SGF is a rare congenital anomaly often diagnosed incidentally during surgery for abdominal testis. Surgical treatment of SGF is debated in the literature, treatment options include orchiectomy versus orchiopexy depending on findings at surgery. Separate two-stage laparoscopic staged Fowler-Stephen orchiopexy could be performed safely in this condition with no complications in order to avoid unnecessary orchiectomy. We confirm that neither the manuscript nor any parts of its content are currently under consideration or published in another journal. All authors have approved the manuscript and agree with its submission to your journal. All authors have made a substantial contribution to the original article. No data have been fabricated or manipulated (including images) to support our conclusions. All authors have full control of primary data and have contributed to the scientific work. All authors have no direct or indirect commercial financial incentive. No financial support has been received for this research/study. No funding agreement limits our ability fairly to complete and publish our research/study. Considering the observational nature of the study and the absence of any interventions outside standard practice, ethical approval was waived. The consent was given from the patient's family included in the medical chart in order to use clinical information for reaserch and or publications. The authors declare no conflicts of interest.