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|Obstructed Hernia in Male Intersex
Ali Reza Shojai*, Kundan K Patil**, Vaibhav Kulkarni**
|The male intersex (male pseudohermaphroditism)
with masculine external genitalia and fully developed uterus is
an uncommonly encountered condition in clinical practice. Laparotomy
or Herniotomy usually reveals the uterine condition. Here we report
a case of obstructed hernia in a male intersex..
Hernia uterine inguinale is one of the rare causes of
male pseudohermaphroditism. These individuals are phenotypically
males. They commonly present with inguinal hernia or undescended
testis or both. Our patient presented with obstructed
A 28 year old male presented
with a left sided obstructed inguinal hernia of one day
Further interrogation revealed left inguinal swelling
of 10 years duration and absence of both testicles since
birth. He had 8 years of happy married life with no issues.
No family history of similar complaints.
Physical examination revealed male muscular built with
well developed secondary sex characters. The penis, urethra
and scrotum were fully formed but, both testicles were
The routine investigation was within normal limits. Exploration
of the inguinal canal region reveals an indirect complete
sac with bowel contents. The sac was opened, the contents
were reduced and then the presence of well formed uterus
and cervix attached to posterior part of prostate by thick
fibrous band was seen. Both ovaries, mesorchium were attached
to uterus (Fig. 1). Total hysterectomy was done along
Pathology report Gross specimen of uterus with bilateral
broad ligament with tubes and ovaries measuring 7.5 X
4.5 X 2.5cm. Cut section shows atrophic endometrial lining
and seminiferous tubules like structure
a) Left and right ovaries: Section shows atrophic and
hyalinised seminiferous tubules. No evidence of spermatogenesis.
b) Tubular tissue : Section shows epididymal tissue and
also features of fallopian tube.
Chromosomal analysis and routine karyotyping revealed
normal 46XY pattern. Post operative, patient’s recovery
Male pseudohermaphroditism is a condition in which
gonads are testis but the internal genitalia are not completely
virilised, male intersex may present:
(i) With masculine external genitalia but fully developed
uterus (Present case)
(ii) With purely feminine external genitalia.
(iii) With external genitalia of equivocal sexuality.
A brief review of embryology would make the understanding
of this case easy. Gonads are not developed till seven
weeks. It begins to differentiate as a testis under the
influence of testis determining region Y.1 Foetal sertoli
cells appears first and secrets anti-mullerian hormone
(AMH) by seven weeks. 2,3 It causes regression of mullerian
ducts in male. The gene has been localized to chromosome
19. Leydig cells appears at 8-9 weeks and begins to synthesise
testosterone.3 The latter binds to androgen receptors
and acts on wolffian ducts. The latter differentiates
into epididymis, vas deferens, seminal vesicles, and ejaculatory
ducts in male. External genitalia are not differentiated
till eight weeks of foetal life.3 Testosterone is converted
by enzyme 5a reductase in target cells into Dihydrotestosterone
(DHT). Testosterone and DHT combines with androgen receptors.
The gene for androgen receptor is located on the long
arm of X chromosome. The activated androgen receptor complex
binds to DNA within the nucleus, inducing protein synthesis
and resulting in androgenic effects.4 DHT causes differentiation
of urogenital sinus (UGS) and external genitalia into
male type. Lack of AMH, testosterone and DHT causes feminine
The possibility of an intersex abnormality was not conceived
in the present case until the herniotomy revealed the
presence of the uterus. In male intersex the penis, urethra
and scrotum are fully developed but one or both testicles
are undescended and usually there is an inguinal hernia.
The testicles are histologically normal for undescended
organs but menstruation does not occur as there is no
endometrial development in the uterus. The general bodily
development is masculine in type as in our case.
||Page DC, Mosher R, Simpson
EM, et al. The sex determining region of
Y chromosome encoded a finger protein. Cell
1987; 51: 1091.
|| Simpson JL. Disorders of gonads and
internal reproductive ducts. In principles and practice
of medical genetics, eds Emery AH, Rimoin DL 2nd ed.
Churchill Liringstone, London, 1990 : 1593 - 1617.
|| George IW, Wilson JD. Embryology of
genital tract. In campbell’s urology, eds Walsh
PC, Retik AB, Stamey TA et al 6th ed., WB
Saundars Co., Philadelphia, 1992: 1492, 1496-1508.
|| Conte FA, Grumbach MM. Abnormalities
of sexual determination and differentiation. In smith’s
General Urology, eds Tanagho EA, Me Anninch JW, 14th
ed, Prentice Hall International Inc, London, 1995:
NEW CARDIAC SURGEONS IMPROVE THEIR RESULTS
The outcome of surgery under newly appointed
cardiac surgeons improves during the first four
years of independent practice. Reviewing surgical
outcomes in 18 913 people undergoing coronary
artery surgery in northern England between 1997
and 2003, Bridgewater and colleagues found that
mortality in patients operated on by 15 newly
appointed surgeons was similar to mortality in
patients of established colleagues, and that it
decreased from 2.2% in the first year to 1.2%
in the fourth year of independent practice. These
data should be interpreted with caution, says
Treasure in a commentary as conclusions about
learning curves based on such a small difference
in mortality are insecure.
BMJ, 2004; 329 : 424.