PEDIATRIC UROLOGY

III I

INCIDENCE OF THE BELL'CLAPPER DEFORMITY

IN AN AUTOPSY SERIES
RICHARD E. CAESAR, M.D.
GEORGE W. KAPLAN, M.D.

From the Division of Urology, University of California, San Diego, and

Children's Hospital and Health Center, San Diego, California

ABSTRACT--Objectives. Testicular torsion is seen in all age groups. The bell-clapper de-
formity (BCD) when present predisposes the patient to torsion. This study was under-
taken to determine the incidence of the BCD.
Methods. An autopsy series of 51 males with 101 testes was dissected by the senior
author to determine the incidence of the BCD.
Results. The manner of testicutar investment by the tunica vaginalis was classified as
normal, intermediate, or absent (BCD). Seventy-six testes were normal, 13 were interme-
diate, and 12 were of the bell-clapper type.
Conclusions. The incidence of the BCD in this autopsy series was 1 2%. Because the in-
cidence of testicular torsion in the general population is far less than 1 2%, other factors
in addition to anatomic predisposition must be involved.

Testicular torsion is seen in infants, children, and
adults; however, the incidence of this event in any
given group of males is unknown. It has been pos-
tulated that the vanishing testis syndrome may be
secondary to testicular torsion. ~ It has been fur-
ther postulated that patients with a unilateral van-
ishing testis as well as those who have experienced
intravaginal torsion often have a contralateral bell-
clapper deformity (BCD). z'3 In most cases, testicu-
lar torsion outside of infancy is thought to be sec-
ondary to abnormal attachments of the testis to
the tunica vaginalis.4 However, the prevalence of
these abnormal attachments in the male popula-
tion is unknown. This study was undertaken to
determine the incidence of abnormal attachments
(BCD) in an autopsy series. Because development
of the processus vaginalis, which normally sur-
rounds the testis, is considered an integral step in
the process of testicular descent,5 the nature of the
testicular attachments was also correlated with the
position of the testis at autopsy.
METHODS
Fifty-one consecutive male postmortem exami-
nations at Sharp Memorial Hospital and Children's
Hospital and Health Center form the basis for this
report. The location of the testes was noted and th~
spermatic cord and testis enclosed by the tunics
vaginalis then was removed by the pathology stafili
The chronologic age of the patient was recorded
The formalin-fixed material was then dissected b:
one of us (G.W.K.). The manner in which the tesfi
was invested by the parietal tunica vaginalis wa
recorded The location of the testis was recorded ~s
scrotal, canalicular, or intra-abdominal. The rna~
ner of testicular investment by the parietal tuniei!
vaginalis was classified as normal, intermediate, ~
absent (BCD). ii
In the normal testis, the parietal tunica sui
rounds the entire testis and most of the epididymi
(Fig. 1, top). A small area of the epididymis ani
the entire spermatic cord are outside the pariet~!!!
and visceral tunica vaginalis. In the intermeaia!~
type, the tail of the epididymis and the entire spe~
matic cord are outside the tunica vaginalis (Fig,:!
left). In the BCD and the intra-abdominal teSfiI
the testis, epididymis, and part of the spermatl;
cord are inside of the tunica vaginalis or pe~!
toneum (Fig. 1, right). Statistical analysis was use!
to correlate position of the testis to the extent
testicular investment.RESULTS ~i!

Submitted: October 29, 1993, accepted (with revisions): Febru- The patients ranged in age from i day to 75 yearil
ary 1O, 1994 at death. Many of the infants were premature. Th~
 NORMAL

Tunica vagina~is

runica vagina~is
"-'w'scera/ lamina

:FIGURE 1. (Fop] Normal testicular investment w i n N e tunica vaginalis parietal lamina

(TI/PL) attached to a large portion of the epididgmN• (Left) Intermediate type; IVPL attaches
to a small portion of the epididgmis; however, the tail of the epididgmis is free. (Right) Bell-
clapper deformity; TVPL does not attach to either the testis or epididymis.

nths and the modal age was
(80%) that had descended
• One testis was surgically
r position was noted to be
ix males. One infant had a
.nd a right intra-abdominal
td bilateral intra-abdominal
5%) had a normal testicular
[. The intermediate type of
red in 13 testes (13%) and
in 12 testes (12%)•
9F TESTICULAR DESCENT
;TrCULAR INVESTMENT
; within the scrotum and 2
r position that had a normal
Three testes in the scrotum
dar position were noted to
~-. . . . . . . . . . . . ~ype of investment. The BCD
TABLE I. Extent of testicular descent compared
with degree of investment
Normal Intermediate Absent*
Scrotal 74 3 4
Canalicular 2 10 1
Abdominai 0 0 7
'~Bell-clapperdeformity
was observed in 4 scrotal, 1 canalicular, and 7
intra-abdominal testes (Table I). One child had bi-
lateral scrotal BCDs and one had a right BCD and a
left intermediate testicular investment in the scro-
tal position. Thus, two of three (66%) boys with a
scrotal BCD had an abnormal contralateral testicu-
lar investment.
COMMENT
The mechanism of testicular descent represents
an enigma• In the sixth week of gestation an indif-
ferent gonad arises as a ventromedial outgrowth
f r o m the u r o g e n i t a l ridge. 6 Over the n e x t 5
months, the maximal distance the testis moves
within the abdomen is 1.0 ram. 7 At 28 weeks, the
testis lies at the internal inguinal ring and usually

/ l. Y 199a / e4, NvMBE 1 115

descends sometime before birth. The events that
initiate descent and the role of the gubernaculum
are obscure. However, the processus vaginalis must
form for descent to occur, s
Development of the processus vaginalis begins at
8 weeks of gestation when a pouch of peritoneum
bulges into the inguinal canal, perhaps secondary
to increased abdominal pressure from organogene-
sis. 5 P r i o r to t e s t i c u l a r d e s c e n t in the t h i r d
trimester, the processus vaginalis undergoes rapid
extension into the gubernacular mesenchyme. The
extension of the processus vaginalis into the gu-
bernaculum results in the separation of a central
core of mesenchyme that lies within the inguinal
canal from a tube of mesenchyme lying outside the
processus vaginalis. This outer tube becomes the
cremaster muscle, s
Muschat ~ described the association of the BCD
with testicular torsion. In the normal scrotum,
there is a strong attachment between the testis and
the epididymis, which in turn is attached to the
inner wall of the scrotum. The normal testis and
epididymis are covered by an invagination of peri-
toneum during the process of descent. Only the
posterior aspect of the epididymis has a bare area
not covered by peritoneum. This anatomic situa-
tion does not allow any significant rotation of the
testis, thus precluding torsion.
In patients with the BCD, the testis, epididymis,
and distal spermatic cord together become an in-
travaginal body, hanging freely within the vaginal
sac. The epididymis lacks its attachment to the
scrotum and is, along with the testis and distal
spermatic cord, completely surrounded by the tu-
nica vaginalis. 9 This anatomic lack of surrounding
scrota1 attachments predisposes the testis to rota-
tion around its vascular axis. Patients who have
this anatomic variation on one side often have it bi-
laterally. Scorer and Farrington 3 recorded an 80%
incidence of bilateral BCDs in patients having sper-
matic cord torsion.
tshizuka et alJ ° attempted to classify the manner
in which the testis was covered by the parietal tu-
nica vaginalis in intravaginal torsion by the extent
of fixation of the epididymis to the posterior aspect
of the scrotum. The BCD was documented in 6 of
10 patients presenting with testicular torsion. The
remaining four testes were classified in an inter-
mediate group and were observed to have the
lower border of the reflected tunica vaginalis be-
hind the posterior margin of the inferior aspect of
the epididymis. The anatomic variant described in
the intermediate group was also shown to pred~s:
pose the testis to a limited amount of mobility p~.
tentially leading to intravaginal torsion.
Our findings in this autopsy study confirm till
observation of Ishizuka et al. to regarding the prfi~i
ence of an intermediate type of testicular inv4~
ment. Although the mterme&ate mvestment m 0N
series was most c o m m o n m the canahcular test~
there was no statistical relationship of it
investment to the canalicular positior
also initially suggested an association k
extent of testicular descent and the co
of testicular investment; however, this 1
was also not statistically significant.
The prevalence of the BCD in o u r s e r
over i0% and suggests that this subset
ulation may be at risk for torsion of the
series might not be representative of
population because it is skewed tow~
However, there are no data to suggest t
"maturation" of the manner in which
invested by the tunica vaginalis. Becaus
the testis affects far fewer than i0% ot
etiology of testicular torsion involves
just an anatomic predisposition to the
!ig
Rmhard E. C a ~
Division of U!"~i~ ~
University of California, San f)~.~!j!~
San Diego, California 92103 ~
REFERENCES ;
1. AbeyarameMR, Aherne WA, and ScottJE: The va
ing testis. Lancet 2: 822-824, 1969.
2. BellingerMF: The blind-ending vas: the fate of t h ~
tralateral testis.J Urol 133: 644-645, 1985. ,;~
3. Scorer CG, and Farrington GH: Con,,enital Defol"T~
of the Testis and Epididymis, London, Butterworth 1971i:~
4. Muschat M: The pathological anatomy of testicul~¢~
sion: explanation of its mechanism• Surg Gynecol Obstii$~
758-763, 1932. ~
• : - . !!~
TheiSh:°CokJ d,Three Prj2e~susdv agiun~hc;rid g;br : h n A ~
1263-1268, 1971.
6. Moore K: The DevelopingHuman. ClinicallyO ~ i l
Embryology.4th ed Philadelphia WB Saunders Co, 1988~
7. Wyndham ~NR. A morphol'o gical study of testicuE~!~
scent. J Anat 77: 179-188, 1943.
8. Backhouse KM: Embryology of testicular descerii NIm
maldescent. Urol Clin North Am 9: 315-325, 1982. i,~_.
9. Skoglund RW, MeRoberts JW, a n d 2 g de H T°}Wi!
r ~
the spermatic cord: a review of the liter e and an a ~
of 70 new cases. J Urol 104: 604-607, 1970. ii~
10. Ishizuka E, Noguchi S, Sato K, Iwasak! A',!!I
Fukuoka H: A classification for intravaginal torsion ~.,~
testis. Eur Urol 15: 108-112, 1988.
.1 V ~ J