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Splenogonadal Fusion: A Rare Finding During Routine Orchiopexy


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Splenogonadal fusion remains a rare congenital anomaly, with limited reports in the literature. It is important to be aware of this benign condition as orchiectomy can result from confusion with malignant processes. We present a case of splenogonadal fusion in a 12-month-old healthy male with history of cryptorchidism found at the time of surgery. Keywords: Splenogonadal fusion, Orchiopexy, Undescended testicle
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Urology Case Reports
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Splenogonadal fusion: A rare nding during routine orchiopexy
Catherine J. Chen
, Nicholas Kavoussi
, Micah A. Jacobs
University of Texas Southwestern, Department of Urology, United States
Vanderbilt University, Department of Urology, United States
Splenogonadal fusion
Undescended testicle
Splenogonadal fusion remains a rare congenital anomaly, with limited reports in the literature. It is important to
be aware of this benign condition as orchiectomy can result from confusion with malignant processes. We
present a case of splenogonadal fusion in a 12-month-old healthy male with history of cryptorchidism found at
the time of surgery.
Splenogonadal fusion (SGF) is a rare congenital anomaly, resulting
in the fusion between spleen and gonad. It was rst reported in lit-
erature in 1883 by Bostroem with approximately 150 cases reported in
literature since then.
SGF can present as an isolated nding or in
association with limb defects, micrognathia, anal atresia and other
congenital anomalies.
Awareness of this anomality is important as
almost 37% of cases reported in literature is associated with un-
necessary orchiectomy.
Case presentation
A 12-month-old healthy male patient was referred for evaluation of
left cryptorchidism. On physical exam, he was noted to have normal
descended right testicle and a palpable mass in the left groin presumed
to be his left testicle. He was subsequently taken to the operating room
for a left orchiopexy.
The orchiopexy began through a scrotal approach. The testis was
delivered through the scrotal incision, at which time an adherent
splenule was also delivered (Fig. 1). The splenic tissue, which was ap-
proximately 2 cm × 3 cm and contained a tail extending into the ret-
roperitoneal space, was carefully dissected free from the testicle and
cord structures and released into the retroperitoneum through a sub-
sequent inguinal incision (Fig. 2). A left hernia sac was also identied,
divided and ligated. Once freed from the splenic tissue, the testicle
easily reached the dependent scrotum and was secured with monocryl
suture. 6 weeks post-operatively follow-up revealed the left testicle well
positioned in the scrotum with no hernias or masses in the groin.
Although SGF is more commonly reported males with a male:female
ratio of 15:1, this discrepancy is likely due to under diagnosis in females
due to the intraabdominal location of the gonad.
While the exact pa-
thophysiology of the fusion is unclear, it is thought that the fusion
between the spleen and gonad occurs between the 5th and 8th week of
gestation, prior testicular descent.
There are two forms of SGF, continuous and discontinuous. In the
continuous form, there is a structural attachment between the spleen
and gonad. This structural attachment can range from a cord-like -
brous structure, to completely splenic or beaded with multiple splenic
In the discontinuous form, accessory splenic tissue is fused to
the gonad and there is no attachment to the orthotopic spleen. Slightly
more than half of the cases (55%) reported in literature were cases of
continuous SGF.
In both forms, the fused splenic mass is most com-
monly fused to the testicle, though they can also be fused to the epi-
didymis or spermatic cord.
The splenic tissue is usually encapsulated
by its own capsule, allowing for it to be separated from the testicle;
however, there has been 1 reported case of intermingling of splenic and
testicular tissue.
Histological evaluation of the fused splenic tissue is
usually normal.
The continuous form of SGF is more commonly associated with
other congenital anomalies, including cryptorchidism, limb defects,
cardiac defects, micrognathia, cleft palate, anal atresia, and spina bi-
Of these malformations, limb defects are most common and is
termed splenogonadal fusion-limb defect syndrome.
Splenogonadal fusion is a rare benign entity that is important to
Received 2 April 2019; Received in revised form 29 April 2019; Accepted 6 May 2019
Corresponding author. 2350 Stemmons Freeway, 4th Floor, F4.04, Dallas, TX, 75207, United States.
E-mail address: (C.J. Chen).
Urology Case Reports 27 (2019) 100904
Available online 07 May 2019
2214-4420/ © 2019 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license
recognize as it can lead to unnecessary orchiectomy. The accessory
splenic tissue can be easily separated from the testicular tissue and cord
structures, thus allowing the undescended testicle to be brought down
into the scrotum.
1. Varga I, Galova P, Adamkov M, et al. Congenital Anomalies from the Spleen from an
Embryological Point of View.
2. Carragher A. One hundred years of splenogonadal fusion. Urology. 1990;35:471475.
3. Gouw ASH, Elema JD, Bink-Boelkens MTE, Jongh HJ, ten Kate LP. The spectrum of
splenogonadal fusion. Eur J Pediatr. 1985;144:316323.
4. Karaman MI, Gonzales ET. Splenogonadal fusion: report of 2 case and review of the
literature. J Urol. 1996;155:309311.
Fig. 1. Accessory splenule attached to the spermatic cord.
Fig. 2. Accessory splenule freed from the spermatic cord.
C.J. Chen, et al. Urology Case Reports 27 (2019) 100904
... If diagnosed in time, the testis can be salvaged in majority of these cases. Usually there is a cleavage plane between the testis and the aberrant splenic tissues through which the splenic tissue can be shelled out easily, as it was in our case [7,15,16]. In cases with fibrous cord connecting testis upper pole to spleen, just detaching the cord usually helps in orchidopexy [8,12]. ...
... In cases with fibrous cord connecting testis upper pole to spleen, just detaching the cord usually helps in orchidopexy [8,12]. Leaving some nodules behind especially in cases of intra-testicular splenic tissue or even a larger piece of spleen attached to testis, does not seems to cause any significant issues [16]. However, regular testicular USS is suggested as these tissues are can be involved in any problems which can affect a normal spleen like hemolytic anemia, idiopathic thrombocytopenia, leukemia, mumps, infectious mononucleosis or malaria [5]. ...
... Az anomália lényege, hogy embrionális korban a léppé differenciálódó őssejttelep és a gonadtelep között abnormális kapcsolat alakul ki. A gyermeksebészek a legtöbbször véletlen leletként -rejtett here vagy lágyéksérv mi-atti műtétek kapcsán -szembesülnek ezzel az entitással [1,2]. A gonad melletti kis léptelep a legtöbbször tumornak imponál, mely miatt szükségtelenül orchiectomia történhet [3]. ...
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Összefoglaló. A splenogonadalis fúzió ritka fejlődési rendellenesség, melynek lényege az ivarmirigy (leggyakrabban a here) és a lépszövet rendellenes kapcsolódása. A szerzők egy féléves csecsemő esetét kívánják bemutatni, akinél már a megszületést követően észlelhető volt a bal oldali here megnagyobbodása. A műtét előtti képalkotó vizsgálatok (ultrahang, MRI), a fizikális vizsgálat és az intraoperatív lelet a splenogonadalis fúzió lehetőségét vetette fel. A műtét során a makroszkóposan lépszövetnek imponáló elváltozás eltávolításra került, majd rutinorchiopexia történt. A hisztológiai vizsgálat alátámasztotta a feltételezett diagnózist. Az entitást 1883-ban írták le először, azóta kevesebb mint 200 esetről számoltak be az angol nyelvű irodalomban. Az utóbbi 30 évben magyar szerzőktől egy közlemény jelent meg. Az elváltozás a banális szülési trauma okozta haematomára, újszülöttkori heretorzióra, malignus heretumorra is hasonlíthat, komoly differenciáldiagnosztikai problémát okozva. A splenogonadalis fúzió jóindulatú elváltozás, a helyes diagnózis esetén elkerülhető a felesleges orchiectomia. Orv Hetil. 2022; 163(7): 288-290. Summary. Splenogonadal fusion is a rare congenital malformation specified as the presence of splenic tissue connected to the gonads (mainly testis). The authors present the case of a 6-month-old male infant, in whom left inguino-scrotal mass was noticed soon after birth. The mass, based on preoperative diagnostic setup (ultrasound, MRI), physical examination and intraoperative findings were suspicious for splenogonadal fusion. At surgery, the mass was resected from the testis, which macroscopically resembled a splenic tissue. Following the resection, orchiopexy was performed. Histology confirmed splenogonadal fusion. Since the first description of the malformation in 1883, only less than 200 cases have been reported in the English literature. Only a single article has been published in Hungary in the last 30 years. Splenogonadal fusion represents a serious differential diagnostic problem. Hematoma caused by trauma, neonatal testicular torsion, benign or malignant testicular tumors may also present similarly. In our case, proper presumptive diagnosis made it possible to avoid unnecessary orchiectomy. Orv Hetil. 2022; 163(7): 288-290.
... However, in the discontinuous type, there is no connection between the ectopic spleen and the abdominal spleen (2, 3). Many cases have been reported since the anomaly was first described, with the latest ones being reported from the United States, France, and Iraq (4)(5)(6). Herein, we report the case of a 7-month-old boy with splenogonadal fusion presenting with a painless palpable scrotal mass and a sudden increase in the size. ...
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Introduction: Splenogonadal fusion is a rare congenital anomaly characterized with anomalous fusion of the spleen and the gonad or mesonephric derivatives. Herein, we report the case of a 7-month-old boy with unusual presentation of splenogonadal fusion.
... Such sequestrations are known in other developing organs such as small intestine (heterotopic gastric mucosa, pancreas), large intestine (heterotopic gastric mucosa, pancreas, prostatic glands), and testis (ectopic adrenal and splenic tissue) but their association with a duplicated urethra is not yet reported. [10][11][12][13][14] To conclude, the association of penoscrotal masses lined with colonic epithelium and a urethral duplication is extremely rare. Besides a standard urethrogram to assess urethral duplication, MRI may be useful to understand the origin of the mass and its relation with the urinary tract as well as the anorectal canal which can help in planning the surgery in a better way. ...
We present a rare case of a 7 years old male with a perineal mass with Urethral duplication. Perineal mass histopathology showed colonic mucosa. The association of heterotopic colonic mucosa with urethral duplication, hypospadias, bifid scrotum and normal anus has not been described previously in the literature.
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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.
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The spleen is the major accumulation of lymphoid tissue in the human body, an organ which prenatally produces and postnatally controls blood cells. Normally, a developed spleen lies in the upper left quadrant in parallel with the long axis of the 10th rib. It is a mesodermal derivate which first appears as a condensation of mesenchymal cells inside the dorsal mesogastrium at the end of the fourth embryonic week. Some congenital anomalies of the spleen are common, such as splenic lobulation and accessory spleen, while other conditions are rare, such as wandering spleen and polysplenia. Splenogonadal fusion is also a rare developmental anomaly, resulting from abnormal fusion of the splenic and gonadal primordia during prenatal development. The purpose of this article is to describe the normal development of the human spleen, supplemented with our own photomicrographs and a review of congenital anomalies of the spleen with their possible embryonic basis.
Splenogonadal fusion is a rare congenital malformation in which there is an abnormal fusion between the spleen and the gonad or derivatives of the mesonephros. It can occur in both sexes but has been reported far more frequently in males. There are two types of this malformation: the continuous and the discontinuous type, depending on the presence or absence of a structural connection between the regular spleen and the ectopic splenic tissue that is fused to the gonad. In one-third of all reported cases splenogonadal fusion is associated with other congenital defects. This association is predominantly found within the continuous type. Peromelia is present in a high percentage of cases, varying from total absence of all limbs to absence of parts of the lower limbs. On the basis of a case report and a review of 84 published cases, the causal, pathogenetic and nosologic aspects of this malformation are discussed. We hypothesise that splenogonadal fusion with peromelia and splenogonadal fusion without peromelia represent two ends of one spectrum determined by the developmental stage during which the causal factor acts.
hrs reported in the literature by Bostroem in~ i883 but it was Pommer, who in 1889 described in detail a ease which was associated with limb defects and other congenital abnormalities, no- tably mierognathia and anal atresia. It has been suggested that this teratologie syndrome of splenogonadal fusion, limb defects, and other Congenital abnormalities, which constitutes a subgroup of patients with splenogonadal fu- sion, be called the Pommer syndrome. Putschar and Manion in 1956 published the first detailed literature review of splenogonadal fusion in which details of 30 clinical eases and a classifi- cation system were discussed. Since their review was published the urologic and pediatric surgi- cal literature have contributed almost another 100 cases which have been mainly in the form of single ease reports with exceptionally, 2 and 3 cases described. Nevertheless, many of the re- ports conclude with the performance of an or- chieetomy, in the mistaken belief that this entity represents a malignant process. Thus it is vital that urologists, pediatric and general surgeons are aware of this condition and appreciate its benign nature.
We reviewed the presentation and clinical findings in an unusual scrotal mass seen in children. This review involves a detailed description of 2 cases of splenogonadal fusion, and a complete survey and assessment of the published literature on this subject. A detailed assessment of 137 cases (135 previously reported, 2 added) of splenogonadal fusion was undertaken. Splenogonadal fusion can be classified as either continuous or discontinuous type. The disorder was rarely recognized before surgery. In 37% of reported patients orchiectomy was performed because of suspicion that the lesion represented a primary neoplasm. However, orchiectomy is not necessary, since the splenic tissue can be dissected safely off of the tunica albuginea. Therefore, an awareness of the possibility that this anomaly may exist can avoid an unnecessary orchiectomy.