Page 12 - World Association of Laparoscopic Surgeons - Journal

WJOLS

Laparoscopic Gonadectomy for Complete Androgen Insensitivity Syndrome

duct and the left, 3 × 2 × 2 cm. Bilateral sertoli hyperplasia
was noted on histology (Fig. 5).
In view of this, long-term conjugated estrogen 0.625 mg
per day was started.

DISCUSSION

AIS is the most common cause of male pseudoherma-
2
phroditism and third most cause of primary amenorrhea. It
is also known as testicular feminization syndrome. Intersex
male pseudohermaphroditism has an incidence of 1 in
20,000 to 64,000 male births. 1,2 John Morris (1953)
described the anatomical, histological and clinical features
based on 82 cases collected from over nearly 150 years of
medical literatures. 3
The typical mode of presentation is an adolescent female,
who has breast development with the pubertal growth but
has not attained menarche with absent or scanty pubic and
axillary hair. Complete androgen insensitivity syndrome
4
(CAIS) may also be present in early infancy with bilateral
inguinal or labial swellings. Bilateral inguinal hernias are
rare in girls and it has been estimated that 1 to 2% of such
5
cases have CAIS. In review of literature, the case of CAIS
in a 22 years female were the presence of testis, prostatic
tissue, seminal vesicals which was confirmed by ultrasound
of abdomen, hormonal analysis, operative findings and
HPE. 6 Diagnosis of CAIS is usually with the absence of
female internal genital organs on physical examination aided
by pelvic ultrosonography, karyotyping, molecular genetic
testing of the AR gene mutation (chromosomal locus
5
Fig. 2: Karyotype analysis—male karyotype-46XY xq 11q 12) and elevated testosterone LH level. In our case,

Fig. 3: Left gonad Fig. 4: Right gonad

World Journal of Laparoscopic Surgery, January-April 2012;5(1):54-57 55