Splenogonadal fusion: a case report and review of the literature

Abstract

Background Splenogondal fusion (SGF) is a rare congenital anomaly characterized by abnormal association between the splenic tissue and the gonads or mesonephric remnants. SGF that requires separate two-stage laparoscopic staged Fowler-Stephen orchiopexy on both the left and right sides is extremely rare. SGF could be misdiagnosed as testicular malignancy and leads to unnecessary orchiectomy. Case presentation This is a case of an 8-month old male infant presented with bilateral cryptorchidism, B-mode ultrasound visualized the left and right testes in the lower abdominal cavity and the upper margin of the left testicle as a hypoechoic mass extending to the spleen, indicating an undescended right testis and possible SGF on the left side. Single-site laparoscopic examination confirmed the diagnosis of SGF on the left side and an undescended right testis. As both testes were high and the right spermatic vessel was poorly developed and short, a routine single stage orchiopexy would be difficult and risky, therefore, separate two-stage laparoscopic staged Fowler-Stephen orchiopexies for both sides were implemented. Stage 1 of the staged Fowler-Stephen orchiopexy for the right side was performed first without treating the left side, Stage 2 for the right side, separation of the left testis from the spleen as well as Stage 1 for the left side were performed 7 months later, and Stage 2 for the left side was performed 7 months after that. Follow-up ultrasound 1 year after the surgery revealed no obvious abnormalities in the shapes of the testes or their blood supply. This treatment strategy prevented unnecessary orchiectomy. Conclusions We reported a rare case of SGF that needed separate two-stage laparoscopic staged Fowler-Stephen orchiopexies for both sides, and a review of the recent literature. SGF is a rare congenital anomaly often diagnosed incidentally during exploration/surgery for scrotal swelling/mass, cryptorchidism or inguinal hernia in young patients. Surgeons, especially pediatric surgeons should be aware of this rare condition to avoid unnecessary, life-altering radical orchiectomy. When routine single stage orchiopexy is not feasible or risky for either side, separate two-stage laparoscopic staged Fowler-Stephen orchiopexies could be performed on both the left and right sides to avoid unnecessary orchiectomy.

Splenogonadal fusion: A case series of two challenging diagnoses

“…Our second case report is also another condition of SGF associated with a disorder of sex differentiation. The age of diagnosis ranged from stillborn to 81 years, 8 but it is common in people under the age of 20 1,3 …”

“…These two types occur approximately with the same frequency 1,3,5 and with a little predominance of continuous forms 5 . Congenital malformations occur most frequently in the continuous form 1,3–5,11 . Cryptorchidism and inguinal hernias are the most frequently associated malformations 1,3,5,11 …”

“…Splenogonadal fusion is a very rare, benign congenital abnormality, occurring most frequently in male patients 1,3,5 . To date, one case has occurred in a newborn with a 46, XY genotype who is phenotypically female 7 .…”

“…Congenital malformations occur most frequently in the continuous form 1,3–5,11 . Cryptorchidism and inguinal hernias are the most frequently associated malformations 1,3,5,11 …”

“…9 Some patients develop asymptomatic testicular mass; however, patients may rarely develop symptoms linked to splenic involvement (leukemia, mononucleosis, malaria, and salmonellosis) or to intestinal obstruction caused by the intraperitoneal cord, or further symptoms related to a traumatic rupture of the ectopic spleen. 3,5,10 Our two cases were incidentally discovered during the exploration of or the treatment for other congenital malformations.…”