Summary: A 4-year-old male was brought to a urologist by his mother
because at birth there was concern that he might have a neurologic
bladder. Since birth, the patient did not have any problems with
urinary infection and established satisfactory urinary
Summary: Physical examination revealed severe extremity anomalies. The
abdomen was soft with no palpable masses present. The patient was
circumcised and his testes were well positioned in the scrotum. On
the left side, there was a discrete mass palpated on top of the
testicle, near the location of the epididymis. Based on this
ﬁnding an initial testicular ultrasound was requested
(Figures 1 and 2). Following this, a technetium 99m (
Tc) sulfur colloid scan was performed (Figure 3).
Splenogonadal fusion. (The urologist felt there was no
indication for surgical intervention at the time and decided to
closely observe the patient as follow-up).
The differential diagnosis includes malignant testicular tumor,
adenomatoid tumor, epididymitis, hemangioma and hematoma.
Testicular ultrasound revealed a solid, hypervascular mass in
the left scrotum, which may either be intratesticular or
paratesticular in nature.The possibility of splenogonadal fusion
was entertained, based on the extremity anomalies and a
99mTc sulfur-colloid examination was suggested for
The 99mTc sulfur colloid scan (Figure 3) demonstrated
evidence of functional hyposplenia and focal activity in the left
scrotum, suggesting ectopic reticulo-endothelial or possibly
splenic tissue. A track of activity was also noted to extend from
the spleen to the left scrotum.
A renal ultrasound (Figure 4) that was performed a year earlier
was within normal limits. Magnetic resonance imaging (MRI) of the
spine was also performed, when the patient was 3 months old, and
the spinal cord was not found to be tethered.
Splenogonadal fusion is a rare congenital anomaly that affects
both sexes, with a male-to-female ratio of 16:1. It was ﬁrst
described in 1889 and about 150 cases have been reported in the
English literature.1 In males, the left testicle, or
other derivatives of the mesonephros, connect to splenic tissues,
usually an accessory spleen. In females, a similar fusion occurs
between the left ovary and splenic tissue.
The condition is classiﬁed into two types: continuous
(56%) and discontinuous (44%). In the continuous type, a strand of
tissue directly connects the the spleen and gonad, whereas, there
is no anatomic connection between the gonadal tissue and the spleen
in the discontinuous type.2
The malformation in males manifests as a testicular mass, and
the diagnosis is seldom made preoperatively. A deﬁnite
diagnosis cannot be made solely on sonographic ﬁndings and
the use of 99mTc sulfur colloid scintigraphy to identify
splenic tissue (either accessory or native) activity adds more
information to the imaging diagnosis.3 Surgical
exploration is however, required to exclude malignancy, since the
accessory spleen that is attached to the testicle may be
misinterpreted as a primary, malignant testicular tumor or an
Patients often exhibit congenital anomalies that include limb
defects; reduction deformity of the arms and forearms; no digits or
missing digits on the hands; reduction deformity of the legs; hind
legs; no digits or missing digits on the feet; spinal dysraphism;
congenital diaphragmatic hernia; lung hypoplasia or lung agenesis;
thoracopagus; micrognathia; microgastria; craniostenostosis; anal
atresia or anal stenosis;hypoplasia; and, polysplenia.
The discontinuous type usually is not associated with congenital
Splenogonadal fusion is a rare congenital anomaly. The
malformation in males manifests as a testicular mass, and surgical
exploration is required to exclude malignancy. However, unnecessary
orchiectomy can be avoided because splenic tissue can be dissected
away from the tunica albuginea.
- Imperial SL, Sidhu JS. Nonseminomatous germ cell tumor arising
in splenogonadal fusion. Arch Pathol Lab Med.
- Putschar WG, Manion WC. Splenicgonadal fusion. Am J
- Guarin U, Dimitrieva Z, Ashley SJ. Splenogonadal fusion-a rare
congenital anomaly demonstrated by 99Tc-sulfur colloid imaging:
Case report. J Nucl Med. 1975;16:922-924.