Continuous-type splenogonadal fusion: A case report

Abstract

Abstract. Splenogonadal fusion (SGF) is a rare congenital malformation. Since it lacks characteristic features, very few cases of SGF have been diagnosed preoperatively. Laparoscopy was effective in both diagnosing and surgically treating this condition. Herein, we reported left side SGF in a male patient who was diagnosed during laparoscopic exploration, and Fowler-Stephens orchidopexy was implemented at the same time. The patient was followed up for one year. At a 6-month follow-up, the left scrotum demonstrated swelling and the internal contents were hard. An ultrasound of this testicle indicated non-uniform, splenic-like organization. However, at the one-year follow-up, the volume of splenic-like organization was reduced but the testicular size did not exhibit further atrophy. IntroductionSplenogonadal fusion (SGF) is a rare congenital malformation. The condition was first described in 1883, but fewer than 200 cases have been reported to date (1). Only a few cases have been diagnosed preoperatively, which were typically regarded as cryptorchidism, testicular tumors or inguinal hernia, and accidentally determined to be SGF after exposure of the inguinal canal during surgery (2). This disease commonly leads to a preoperative misdiagnosis of testicular cancer, causing testes to be mistakenly removed in nearly a third of SGF cases (3,4). In order to avoid these errors, increased awareness of the diagnosis of and treatment for SGF therefore is required. In this case report, we summarize our experiences of treating SGF and review the literature to improve the understanding of this disease and provide helpful suggestions for future treatment strategies. Case reportA 4-year-old boy presented to the Department of Pediatric Surgery, West China Hospital of Sichuan University (Chengdu, China) with bilateral undescended testes from birth in April 2013. Physical examination indicated that the bilateral scrota were empty, the testes were also not present in the inguinal region, and the patient's penis was normal. An ultrasound confirmed that there were no testicular masses in the bilateral scrota and inguinal regions. In accordance with the above clinical findings, the patient was diagnosed with bilateral cryptorchidism, and therefore laparoscopic gonad exploration was performed. Complete abdominal exploration revealed gonad-like tissue connected with a smooth, fleshy, brown, cord-like structure beside the left colon, but left spermatic cord blood vessels were not observed. The cord-like structure was connected to the spleen (which was normal), and was approximately 5-6 cm long and 1.5 cm in diameter ( Fig. 1A and B). The spleen cord was cut off and the gonad-like tissues were pulled down into the scrotum through the inguinal canal (Fig. 1C-E). A small incision was made in the scrotum skin to expose the gonad-like tissue. A small piece of gonad-like tissue was harvested for pathological examination. The result of pathological examination demonstrated that the gonad-like tissue was a normal testis. As a result, the spleen c...

A case of splenogonadal fusion accompanied by accessory spleen in a 4-year-old boy

“…If an aberrant fusion happens, during the gonadal descent (which starts at 8 th week) the splenic tissue would descend with the gonad and may be found anywhere along the normal descent path. [ 3 4 ] This theory also is consistent with the common association of limb and jaw defects with the continuous SGF because those mesenchymal derivatives (limb buds and Meckel's cartilage) also develop during the critical fusion time window.…”

“…It has been reported that 25%–35% of SGF will necessitate orchiectomy because of testicular atrophy or inseparability of tissues. [ 3 4 ] This was the case for our patient where the testicular remnant was very much atrophied and later found to be of primitive histology. This was probably due to pressure applied on the testicular parenchyma by the splenic tissue enclosed within the unyielding tunica vaginalis.…”

“…This is probably because the aberrant splenic tissue interferes with the normal gonadal descent and/or the closure of processus vaginalis. [ 3 ] As previously mentioned, continuous SGF is commonly accompanied by other congenital anomalies such as micrognathia, cleft palate, cardiac defects, and limb deformities. [ 5 6 ] Such is not the case with the discontinuous cases of SGF.…”

A rare case of discontinuous type splenogonadal fusion in a 13 year old boy: a case report and review of literature

“…5,6 This entity is almost entirely left sided (98%) and demonstrates a profound male predominance (incidence ~ 16:1). 7 In 1956 Putschar and Manion classified SGF into two groups: continuous and discontinuous. In the continuous type, a cord of splenic tissue, a fibrous cord beaded with splenic nodules or a pure fibrous band anatomically connects the main spleen to the gonad.…”

Discontinuous Splenogonadal Fusion in a Patient With Left Testicular Mass

“…It is widely accepted that once an accurate pre-or intraoperative diagnosis is made, surgery is not needed in the absence of significant complications [17]. In patients with continuous SGF who undergo orchiopexy, the splenic band can be easily separated from the testicular vessels and removed alongside with the splenic tissue affecting the testis [18][19][20].…”