Keywords
Bilateral Empty Scrotum - orchidopexy - discontinued splenogonadal fusion
Introduction
Splenogonadal fusion is a rare benign congenital anomaly defined as the presence of
splenic tissue adherent to gonads. It was first described in 1883 by Bostroem, a German
pathologist. This condition is usually discovered incidentally during inguinal hernia
or a testicular mass.[1]
[2]
[3] There are approximately 175 cases reported in the literature that showed left side
and male predominance. Splenogonadal fusion can be subgrouped into continuous and
discontinuous types based on splenic attachments.[4] It commonly presents as inguinal hernia or testicular mass and may be misdiagnosed
as testicular malignancy; in some cases of continuous types, it might be associated
with congenital anomalies such as limb defects, microganthia, cleft palate, and cardiac
defects.[3]
[5] We present the case of an 18-month-old boy who was referred as a case of bilateral
empty scrotum since birth.
Case Report
An 18-month-old boy was referred as a case of bilateral empty scrotum since birth.
When we reviewed the case, we found a male baby of average weight and height with
uncircumcised well-formed penis and urethral meatus in normal position with bilateral
impalpable undescended testes. There were no associated anomalies. The patient was
scheduled for laparoscopic exploration and orchidopexy, if possible. Laparoscopic
exploration revealed no abnormalities and the spleen in its normal place. On the right
side, the vas deferens and testicular vessels were attenuated, atrophic, and entering
the right deep inguinal ring. Therefore, an exploration of the right inguinal region
was performed, which revealed a blind ending vas deferens and testicular vessels.
The left testicle was found intra-abdominally near the left internal ring and therefore
the testicle was delivered easily to the left inguinal region through an inguinal
incision with minimal dissection. We noticed a mass of the upper pole of the left
testicle with no clear demarcation between the mass and the testicular parenchyma.
The surface of the mass was smooth, irregular in shape, and firm ([Fig. 1)]. The options were either of the following: (1) orchiectomy including the mass if
considered as malignant, but the patient had no other testicle, (2) excisional biopsy
of the mass and orchidopexy, which was not feasible, or, lastly, (3) incisional biopsy
of the mass and scrotal orchidopexy. A wedge biopsy was taken from the upper pole
of the testicle (site of the mass) for tissue diagnosis followed by orchidopexy. The
patient passed uneventful postoperative days. Histopathology showed no malignant or
inflammatory cells but lymphatic (splenic) tissue. The diagnosis of splenogonadal
fusion (discontinuous type) of intra-abdominal left undescended testis and vanished
right testicle was made. As the patient had only one testicle with a splenic tissue
remnant in the upper pole, the plan for meticulous dissection to excise the residual
splenic tissue was made. The child was booked for complete excision of mass from the
upper pole of left testicle after 6 months of observation.
Fig. 1 Figure shows the left testicle. S, Splenic tissue; T, testis; E, epididym.
Discussion
Splenogonadal fusion is a rare benign congenital anomaly defined as the presence of
splenic tissue adherent to the gonads discovered incidentally.[1]
[2]
[3]
[6] Male-to-female ratio is approximately 16:1 with predominance on the left side. Splenogonadal
fusion can be subgrouped based on the attachment to the spleen into a continuous and
discontinuous type.[4]
[7] The continuous type represents approximately 55% of cases and is characterized by
a connection between the spleen and the gonads through a cord of splenic or fibrous
tissue. The discontinuous type (44% of cases) has no direct connection of the gonads
with accessory spleens or ectopic splenic tissue.[3]
[7] The exact pathogenesis for splenogonadal fusion is not yet understood, but it is
thought to develop between the fifth and the eighth week of gestation based on the
close location between the splenic tissue and left gonadal ridge. When the spleen
develops in the dorsum of the mesogastrium from a group of mesenchymal cells, the
gonads start to develop in the posterolateral wall of the embryo at the same time.
During embryonic gut rotation, the spleen development comes into close approximation
to the left urogenital fold. Furthermore, subsequent inflammation or adhesions on
the peritoneal surface may facilitate splenogonadal fusion.[4]
[7]
[8]
[9] This condition commonly presents as an inguinal hernia or a testicular mass and
may be misdiagnosed with testicular malignancy. In the literature, four cases of malignancies
associated with splenogonadal fusion have been described. These patients presented
as adults with different histopathological findings and, in cases of the continuous
type, with congenital anomalies such as limb defects, microganthia, cleft palate,
and cardiac defects.[3]
[5]
[10] The diagnosis is usually made by ultrasonography. In case of insufficient resolution,
a computed tomography or magnetic resonance imaging, and lastly technetium-99m spleen
scintigraphy, which is the most recommended tool in the literature, may be performed.[3]
[7] However, the most useful diagnostic test is surgical exploration. In our case, the
availability of frozen section in first operation saved the child from orchiectomy,
but frozen section may not be available at all times.[3]
[4]
[11]
[12] Splenogonadal fusion is a benign condition. Surgery is not required if clinical
signs are absent, especially in the scrotum. If surgery is performed, the testis can
be preserved as the splenic tissue can usually be easily separated from the gonad.[13]
Conclusion
Splenogonadal fusion is a rare benign congenital anomaly. Pediatric surgeons should
be aware of this disease entity to avoid unnecessary aggressive interventions such
as orchiectomy.
