Journal of Pediatric Surgery Case Reports 43 (2019) 5–7

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Journal of Pediatric Surgery Case Reports

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Splenogonadal fusion presenting as a scrotal mass T

a,∗ b a a
Moaied A. Hassan , Hasan K. Gatea , Thura K. Ja'afar , Ahmed O. Fahad
a
Pediatric Surgery Department, Basrah Children's Specialty Hospital, Basrah, 61003, Iraq
b
Iraqi Board for Medical Specializations, Baghdad, 10001, Iraq

A R T I C LE I N FO A B S T R A C T

Keywords: Splenogonadal fusion is an extremely rare congenital anomaly in which there is fusion between the spleen and
Splenogonadal fusion gonad or derivatives of the mesonephros. Due to its extreme rarity, surgeons and radiologists lack the awareness
Inguinoscrotal mass enough to diagnose and sometimes even suspect the diagnosis, and most of the cases are discovered incidentally
Spleen during various inguinoscrotal procedures. Asymptomatic inguinoscrotal mass is the most common presentation
in children, and cryptorchidism is the most common associated anomaly. We present a case of splenogonadal
fusion in a two years old child who was referred to our institution principally with right sided undescended
testis. A left sided scrotal mass was found during examination, and this finding prompted us to conduct a
thorough clinical evaluation. Preoperative diagnosis was not possible in this case and testicular tumor was the
provisional diagnosis. Testicular sparing approach with biopsy of the lesion and histopathological examination
helped to confirm the diagnosis and salvage the testis.

1. Introduction sided empty scrotum. The family stated that they have just recently
noticed that the right testis is not present within the scrotum. On ex-
Splenogonadal fusion is an extremely rare congenital anomaly in amination, the child has right sided undescended impalpable testis with
which there is fusion between the spleen and gonad or derivatives of a left sided painless scrotal mass (Fig. 1). General clinical examination
the mesonephros [1]. The pathology was first described by Bostroem in revealed no significant findings. The family has been informed about
1883 and since then, there were less than 200 cases reported in the this important new finding and the need of their child to have thorough
literature [2,3]. Due to its extreme rarity, the real incidence of the clinical evaluation.
anomaly is unknown and may be underestimated [4]. There is an ob- Inguinoscrotal ultrasound was the first diagnostic tool and revealed
vious male preponderance to the pathology with a male to female ratio that the left testis was replaced by a large(18 × 10 mm) hypervascular
of 16:1(4). Some undefined teratogenic insult during the 5th-8th weeks mass like lesion with another small lesion of similar texture near the
of gestation when the spleen and gonad are in close proximity to each deep inguinal ring with the suggestion of testicular tumor. The right
other is the proposed etiology for the development of this abnormal testis was found located anterior to the distal part of right psoas muscle.
connection between the two organs [1,5]. Putschar and Manion have Complete blood count revealed mild microcytic anemia with
described two types of the anomaly. In the continuous type (55%), the otherwise normal blood counts and cell morphology. Tumor markers
normal spleen is connected to the heterotopic spleen with a cord of including serum alpha-fetoprotein, lactate dehydrogenase and β-human
splenic or fibrous tissue, whereas in the discontinuous type (45%), the chorionic gonadotropin were all within normal limits.
heterotopic spleen is attached to the gonad but has no connection to the Magnetic resonance imaging revealed that the left testis was en-
orthotopic spleen [1,6]. Children with splenogonadal fusion usually larged, heterogenous and hypervascular with the suggestion of acute
present with asymptomatic inguinoscrotal mass; however, most of the orchitis. The latter finding was inconvenient as the scrotal mass was
cases are seen incidentally during surgery for inguinal hernia repair, painless and not tender on examination.
orchiopexy or suspected testicular tumor [1]. Under these circumstances, a decision has been made to explore the
scrotal mass and family's consent was obtained. A transverse left in-
2. Case presentation guinal incision was made with delivery of the testis and cord structures.
A large, solid, dark purple resembling liver tissue mass was found
A two years old child was referred to our institution with a right densely attached to a smaller normal looking testis with another smaller

∗
Corresponding author.
E-mail address: moaied70@yahoo.com (M.A. Hassan).

https://doi.org/10.1016/j.epsc.2019.01.009
Received 28 December 2018; Accepted 12 January 2019
Available online 14 January 2019
2213-5766/ © 2019 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license
(http://creativecommons.org/licenses/BY-NC-ND/4.0/).
M.A. Hassan et al. Journal of Pediatric Surgery Case Reports 43 (2019) 5–7

anatomical continuity between the native spleen and ectopic spleen [3].
The continuous type is the most common (55%), and is known to be
frequently associated with other congenital anomalies including cleft
palate, micrognathia, cardiac defects, limb deformities and anorectal
malformations [3].
Due to its extreme rarity, it is nearly impossible to establish the
diagnosis of splenogonadal fusion on clinical grounds alone. Surgeons
and radiologists lack the awareness enough to make and sometimes
even suspect the diagnosis. In fact, most of the cases are discovered
incidentally during surgery for inguinal hernia repair, orchiopexy and
suspected testicular tumor [1].
In general, children with splenogonadal fusion present with
asymptomatic inguinoscrotal mass. Cryptorchidism is the most frequent
associated anomaly. Cortes et al. reported a 31% cryptorchidism rate in
their review of 111 splenogonadal fusion cases [2,9].
The patient in this study was presented with asymptomatic left
sided scrotal mass with right sided undescended testicle. In fact, cryp-
Fig. 1. Left sided scrotal mass with right sided empty scrotum. torchidism was the alarming sign that made the family seek medical
advice. We believe that the presence of painless left sided scrotal mass
with contralateral undescended testis can be considered alarming for
the pediatric surgeons and urologists to suspect the diagnosis or at least
consider it in the differential diagnosis.
Whenever there is a clinical suspicion, several diagnostic tools may
help in establishing the diagnosis. Ultrasonography reveals an en-
capsulated, iso or hypoechoic, homogeneous extratesticular mass that is
more hypervascular at doppler analysis when compared to the neigh-
boring testis. Computed tomography and magnetic resonance may
identify the continuous type of the anomaly by detecting the connecting
fibrous or splenic tissue. Technicium-99m sulfur colloid scan will reveal
uptake of the radioactive material by the heterotopic splenic tissue [6].
In this study, the ultrasonographer was able to detect the presence of a
Fig. 2. Discontinuous type splenogonadal fusion with another accessory spleen mass lesion attached to the testis and even detected another similar
along the spermatic cord. lesion near the deep inguinal ring which proved later to be an accessory
spleen attached to the spermatic cord with the suggestion of testicular
lesion of the same texture along the spermatic cord (Fig. 2). tumor. Magnetic resonance imaging suggested the possibility of orchitis
At operation, the impression was that the child may suffer some based on the hypervascularity of the lesion. Lack of awareness among
testicular tumor and that the other similar but smaller lesion along the radiologists has driven them away from suspecting the diagnosis.
spermatic cord may represent a metastatic lymph node; however, a Lack of definitive preoperative diagnosis have resulted in un-
decision has been made to obtain a wedge biopsy from the mass and necessary orchidectomy of otherwise benign testicular lesion in many
excise what appeared to be a lymph node and send the resected spe- cases of splenogonadal fusion fearing of the possibility of malignancy.
cimens for histopathological examination and not to rush with orchi- In 1996 Karaman and Gonzales, reviewed 137 cases of splenogonadal
dectomy, based on the general wellbeing of the child, normal blood fusion and surprisingly reported orchidectomy in 37% of the patients
counts and cell morphology and within normal limits tumor markers. because of the suspicion of a testicular tumor [10]. In this study, pre-
Frozen section facility was not available in our institution at time of operative diagnosis was not confirmed; however, the decision was
surgery. made not to rush with orchidectomy and obtain a biopsy waiting for
Histopathological examination of both specimens revealed the histopathological diagnosis, based on the general wellbeing of the child,
presence of red and white pulps of splenic tissue with lymphoid follicles normal blood counts and cell morphology, within normal limits tumor
and germinal center surrounded by thick capsule with no evidence of markers along with the presence of contralateral intraabdominal tes-
malignancy and the diagnosis of splenogonadal fusion was confirmed. ticle. We believe it is important to follow a testicular sparing approach
depending on thorough clinical evaluation and avoid unnecessary
orchidectomy as much as possible.
3. Discussion
4. Conclusion
The spleen develops from the left dorsal mesogastrium, and then
undergoes rotation with the embryonic gut to come and lie in close Splenogonadal fusion is an extremely rare congenital anomaly in
proximity with the developing gonad during the 5th-8th weeks of ge- which there is fusion between the spleen and gonad or derivatives of
station. During this time, an undefined teratogenic insult will lead to the mesonephros. Due to its extreme rarity, the real incidence of the
fusion of these two organs [2,7]. One theory tried to explain the de- anomaly is unknown and may be underestimated. Preoperative diag-
velopment of this abnormal fusion, stated that an inflammatory process nosis is nearly impossible, and most of the cases are discovered in-
of the peritoneal surfaces overlying the spleen and gonad will cause cidentally during surgery for inguinal hernia, orchiopexy and suspected
adhesion between them which eventually brings the two organs to fuse testicular tumor. In children, asymptomatic left sided scrotal mass is the
together [4,8]. Testicular descent begins during the 8th −10th weeks of most common presentation, and when associated with contralateral
gestation, so that it will drag with it a remnant of the attached splenic undescended testis, it may be a considerable clue to suspect the diag-
tissue along the course of its descent down to the scrotum [2]. nosis. Thorough evaluation with the appropriate diagnostic tools will
In 1956, Putschar and Manion classified splenogonadal fusion into help confirm the diagnosis. Whether diagnosed preoperatively or not,
two categories: continuous and discontinuous, depending on the unnecessary orchidectomy should be avoided as the whole pathology is

6
M.A. Hassan et al. Journal of Pediatric Surgery Case Reports 43 (2019) 5–7

benign and associated with fewer complications. References

Patient consent [1] Chiaramonte C, Siracusa F, Li Voti G. Splenogonadal fusion: a genetic disorder? –
report of a case and review of the literature. Urol Case Rep 2014;2:67–9.
[2] Bal K, Ermete M, Balci U, Dincel C. Splenogonadal fusion: a very rare congenital
Consent to publish the case report was not obtained. This report anomaly in the differential diagnosis of a testicular mass. Turk J Urol 2014;40:62–4.
does not contain any personal information that could lead to the [3] Shakeri A, Shakeri A, Rasolmali R, Shakeri S. A case of splenogonadal fusion ac-
identification of the patient. companied by accessory spleen in a 4-year-old boy. Urol Ann 2018;10:406–8.
[4] Stamatiou K, Marinis A, Moschouris H, Manoloudaki K, Koulia K, Tsavari A,
Vasilakaki T. Splenogonadal fusion. A case report. Sci Chron 2016;21(1):104–10.
Authorship [5] Kumar S, Jayant K, Agrawal S, Parmar KM, Singh SK. A rare case of continuous type
splenogonadal fusion in a young male with primary infertility. Case Rep Urol
2014;2014:796761. 3 pages.
All authors attest they meet the current ICMJE criteria for [6] Morgado M, Albuquerque J, Concalves M. Splenogonadal fusion- Rare etiology tes-
Authorship. ticular mass. J Ped Surg Case Rep 2016;10:39–41.
[7] Varma DR, Sirineni GR, Rao MV, Pottala KM, Mallipudi BV. Sonographic and CT
features of splenogonadal fusion. Pediatr Radiol 2007;37:916–9.
Conflict of interest
[8] Rena DM, Dennis BL. Splenogonadal fusion: an unusual case of an acute scrotum. Rev
Urol 2013;15(4):197–201.
The following authors have no financial disclosures: (M.A.H, H.K.G, [9] Cortes D, Thorup JM, Visfeldt J. The pathogenesis of cryptorchidism and splenogo-
T.K.J, A.O.F). nadal fusion: a new hypothesis. Br J Urol 1996;77:285–90.
[10] Morjan Mohamad, Akil Nizar, Nassan Safi, Al-Salem, Ahmed H. Splenogonadal
fusion: a forgotten cause of testicular swelling in children. Ann Pediatr Surg January
Funding 2014;10(1):25–6.

No funding or grant support.