This study was posted before, but only in abstract. I think the whole document is interesting:
AN UNUSUAL CASE OF PRIAPISM
Mehdi Jam, MD, Nand S. Datta, MD, and Asghar Askari, MD
Los Angeles, California
J Nati Med Assoc. 1993;85:473-474
A 24-year old black man was diagnosed to have
sickle cell disease (SS hemoglobin) at the age of 2
years. He developed the first episode of priapism at the
age of 17 years when he was seen by our service for the
first time. This episode of priapism was associated with
vaso-occlusive crisis. ……..Since then he
has had 62 more episodes of priapism. Of these, 24
episodes were associated with other vaso-occlusive
crises, whereas 39 were isolated episodes of priapism.
…….
The range of duration of priapism was 2 to 8 days,
the average being 4 days. ……..
Successful resolution of the initial episodes of
priapism with medical management and no loss of
potency had convinced him that he did not need surgical
treatment.
Initial physical examination revealed normal-looking
external genitalia. However, subsequently the penis
gradually showed excessive growth and at the last visit
the penis measured 18 cm in length (from the pubic symphysis
to the tip of the penis) and 161/2 cm in circumference
in flaccid state. The hypertrophy was confined to
the corpora cavernosa. The glans penis and the scrotum
were of normal size. During an episode of priapism, the
penis further increased in size and got very tender.
…..
The exact mechanism of priapism is not well understood.
However, in sickle cell disease, it is believed to be due to blockage of venous
drainage by sickled cells. This results in ischemia of
the cavernous tissues of the corpora cavernosa. If
priapism is not treated within a reasonable period of
time, the ischemic injury to the delicate cavernous
tissue results in subsequent fibrosis and impotence.
Our patient did not develop impotence despite the
fact that he had several episodes of priapism. On the
contrary, he developed hypertrophy of his penis. The
exact reason for such occurrence is not clear. It appears
that the degree of venous obstruction in our patient was
such as to produce filling and engorgement of the
cavernous spaces resulting in prolonged erection and at
the same time the obstruction was not complete so that
the circulation through the cavernous spaces continued
to the extent that ischemic injury did not occur. Lack of
ischemic injury to the cavernous tissue was possibly
responsible for the preservation of potency.
When sickling occurs within the cavernous spaces of
the corpora cavernosa in sickle cell disease, it appears to
result in a spectrum of hemodynamic changes. On the
one end of the spectrum is total venous occlusion
resulting in priapism, cavernous ischemia and subsequent
fibrosis, and impotence, and on the other end is
the minimal venous occlusion with no priapism and no
ischemic changes. In the middle of the spectrum, there
appears to be various degrees of partial venous
obstruction, resulting in priapism associated with
adequate oxygenation of the cavernous tissue because
of continued circulation in the presence of incomplete
venous occlusion. Our patient represents such an
example. Furthermore, repeated episodes of mild
hypoxia resulting from the transient partial venous
obstruction in our patient might have been a stimulus
for the hypertrophy of corpora cavernosa, because
hypoxia is known to result in hypertrophy of tissues.4 It
is interesting to note that the penile hypertrophy was
confined to the corpora cavernosa and that the glans and
the corpus spongiosum were of normal size. It is well
known that the latter structures are not involved in
priapism5-7 and hence they are not subjected to hypoxic
stimulus to under hypertrophy.
Literature Cited
1. Tarry WF, Duckett JW, Synder H. Urological complications
of sickle cell disease in a pediatric population. J Urol.
1987; 1 38:592-594.
2. Edmond AM, Holman R, Hayes RJ, Serjeant GR.
Priapism and impotence in homozygous sickle cell disease.
Arch Intem Med. 1980;1 40:1434-1437.
3. Datta NS. Megalophallus in sickle cell disease. J Urol.
1977;1 17:672-673.
4. Florey L. General Pathology. 4th ed. Philadelphia, Pa:
WB Saunders Co; 1970:638-640.
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