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A True Hermaphrodite
THE JOURNAL OF UROLOGY
Vol. 88, No. 4 October 1962 Copyright © 1962 by The Williams & Wilkins Co. Printed in U.S.A.
A TRUE HERMAPHRODITE A. WOJEWSKI ANDS. KRASON From the Department of Urology, Pomeranian Medical Academy, Szczecin, Poland
Cases of true hermaphroditism have always aroused great interest due to their rare occurrence and also because of the unexplained causes of their genesis. According to Witschi, Nelson and Segal,1 abnormalities in the development of internal and external sex organs depend on when the damaging factor is acting upon the developing sexual glands. As a consequence of the damage to the egg or blastocyte in the period preceding differentiation of sexual glands the syndrome of true hermaphrodism is originated. In Burns's opinion2 anomalies in sex organ development arise from disorders in the action of hormonal factors. The introduction of androgens or estrogens into the fetal circulation may lead to the transformation of the gonads into inverse sex glands. The sex chromatin pattern of a true hermaphrodite may be either male or female. In the majority of cases it is of female sex. 3 On the basis of world literature Overzier4 collected only 74 cases from 1900 to 1955. Since then the number of recorded true hermaphrodites has markedly increased. 5-u Accepted for publication February 2, 1962. Witschi, E., Nelson, W. 0. and Segal, S. J.: Genetic, developmental and hormonal aspects of gonadal dysgenesis and sex inversion in man. J. Clin. Endocr. & Metab., 17: 737-753, 1957. 2 Burns, R.: Urogenital system. In: Analysis of Development, ed. by B. H. Willier, P. A. Weiss and U. Hamburger. Philadelphia: W. B. Saunders Co., 1955, pp. 47o-49I. 3 Barr, M. L.: The sex chromatin and its bearing on errors of sex development. Canad. M.A. J.,
CASE REPORT
S. M., 4823-61, aged 4, was admitted to the department for the purpose of establishing the sex. The child was born after a normal pregnancy during the course of which the mother took no drugs. The patient was brought up as a girl (fig. 1). Her 6-year-old brother was born without any defects and his development was normal. The child's weight, height and mental de-
Fw. 1. True hermaphrodite, 4 years old
1
74: 419, 1956.
4 Overzier, C.: Hermaphroditismus verus. Acta endocrinol., 20: 63-80, 1955. 5 Schwiebinger, G. W. and Hodges, C. V.: True hermaphrodite. J. Urol., 73: 424-429, 1955. 6 Milner, W. A., Garlick, W. B., Fink, A. J. and Stein, A. A.: True hermaphrodite siblings. J. Urol., 79: 1003-1009, 1958. 7 Lewis, E. L.: A true hermaphrodite, clinical and psychological study. J. Urol., 81: 309-314,
1959.
8 Wojewski, A.: Zur frage des wahren Zwittertums. Urol. Int., 9: 52-61, 1959. 9 Sekowska, J.: Prawdziwe obojnactwo. Pol. Przegl. Chir., 32: 1029-1031, 1960. 10 Austerman, W. and Beach, P.: True hermaphroditism with a report of two cases. J. Urol.,
86: 345-351, 1961.
11 Zielinski, W.: Przypadek obojnactwa prawdziwego. Pediat. Pol., 36: 645-650, 1961.
539
FIG. 2. External sex organs velopment were proper for her age. The penis was 3 cm. long, curved downward. The glans penis was not covered with prepuce. On the lower surface of the penis there was a narrow strip of mucous membrane running from the perineum right up to the glans penis. The external orifice of urethra was on the perineum. Just below the penis there were two skin folds similar to labia majora. The right, larger fold became smaller under pressure. Inside the fold
540
WOJEWSKI AND KRASON
there was an oval formation resembling the testis (fig. 2). On the basis of the examination covering the nuclear chromatin of epithelial cells of mucous membrane in the mouth, feminine
FIG. 3. Urethrovaginogram
chromosome sex was established. Urethrography by means of 2 catheters revealed the presence of rudimentary vagina (fig. 3). The urine 17-ketosteroid level was 0.65 mg./24 hours. During operation on the right inguinal hernia a node the size of a bean was noted in the hernial sac. One half of the node was whitish, its surface being uneven and covered with numerous, bluishly translucent, small cysts, while the other half was smooth, soft, of rosy color. A section was taken from the node. By widening the hernia the abdominal cavity was opened. The anatomical proportions in the region of pelvis were like those in a boy. No opposite gonad was found. There was a round, hard formation of a small hazel-nut size outside the bladder and under the peritoneum. A section was also taken from this node. Histopathological examination: The first section was ovarian tissue with primary vesicles as well as seminal tubules. Stunted spermatogenesis was seen in the preparation. There was no distinct line of demarcation between these two types of tissues (fig. 4). Diagnosis: ovariotestis. The second section contained intermingling bundles of muscular fibers. In the center there was a narrow lumen lined with columnar epithelium.
FIG. 4. Photomicrograph. Testicular and ovarian tissues are seen in preparation. Hematoxylin and eosin, XlOO
A TRUE HER'VIAPH!WDITE
to Dr. Stojalowski this tissue may represent a rudimentary uterus. The hermaphrodite was discharged from the department and advised to return after the period of sexual maturation. In Klebs' classification the case just deseribecl belongs to the group of unilateral true hermaphroditism (hermaphroditism us verus unilateralis). On the right side the testicular and the ovarian tissues fuse to one formation of bean size (ovariotestis), while on the left side no gonad was found. The nuclear chromatin pattern positively pointed to the female sex, but the sex organs were much
more similar to those of operation was postponed maturation so that the make a decision as to sex.
The plusfa
SU1IMARY
A case of unilateral true hermaphroditisrn been described. On one side, in the gonad, tissue of testicle and ovary was present; second gonad was not found. Sex chronwtin pattern was female. The sex organs were developed and similar to male ones. The cfor·i,.ilc,11 on se.\: was no,st1;1onen till sex maturation.
Vol. 88, No. 4 October 1962 Copyright © 1962 by The Williams & Wilkins Co. Printed in U.S.A.
A TRUE HERMAPHRODITE A. WOJEWSKI ANDS. KRASON From the Department of Urology, Pomeranian Medical Academy, Szczecin, Poland
Cases of true hermaphroditism have always aroused great interest due to their rare occurrence and also because of the unexplained causes of their genesis. According to Witschi, Nelson and Segal,1 abnormalities in the development of internal and external sex organs depend on when the damaging factor is acting upon the developing sexual glands. As a consequence of the damage to the egg or blastocyte in the period preceding differentiation of sexual glands the syndrome of true hermaphrodism is originated. In Burns's opinion2 anomalies in sex organ development arise from disorders in the action of hormonal factors. The introduction of androgens or estrogens into the fetal circulation may lead to the transformation of the gonads into inverse sex glands. The sex chromatin pattern of a true hermaphrodite may be either male or female. In the majority of cases it is of female sex. 3 On the basis of world literature Overzier4 collected only 74 cases from 1900 to 1955. Since then the number of recorded true hermaphrodites has markedly increased. 5-u Accepted for publication February 2, 1962. Witschi, E., Nelson, W. 0. and Segal, S. J.: Genetic, developmental and hormonal aspects of gonadal dysgenesis and sex inversion in man. J. Clin. Endocr. & Metab., 17: 737-753, 1957. 2 Burns, R.: Urogenital system. In: Analysis of Development, ed. by B. H. Willier, P. A. Weiss and U. Hamburger. Philadelphia: W. B. Saunders Co., 1955, pp. 47o-49I. 3 Barr, M. L.: The sex chromatin and its bearing on errors of sex development. Canad. M.A. J.,
CASE REPORT
S. M., 4823-61, aged 4, was admitted to the department for the purpose of establishing the sex. The child was born after a normal pregnancy during the course of which the mother took no drugs. The patient was brought up as a girl (fig. 1). Her 6-year-old brother was born without any defects and his development was normal. The child's weight, height and mental de-
Fw. 1. True hermaphrodite, 4 years old
1
74: 419, 1956.
4 Overzier, C.: Hermaphroditismus verus. Acta endocrinol., 20: 63-80, 1955. 5 Schwiebinger, G. W. and Hodges, C. V.: True hermaphrodite. J. Urol., 73: 424-429, 1955. 6 Milner, W. A., Garlick, W. B., Fink, A. J. and Stein, A. A.: True hermaphrodite siblings. J. Urol., 79: 1003-1009, 1958. 7 Lewis, E. L.: A true hermaphrodite, clinical and psychological study. J. Urol., 81: 309-314,
1959.
8 Wojewski, A.: Zur frage des wahren Zwittertums. Urol. Int., 9: 52-61, 1959. 9 Sekowska, J.: Prawdziwe obojnactwo. Pol. Przegl. Chir., 32: 1029-1031, 1960. 10 Austerman, W. and Beach, P.: True hermaphroditism with a report of two cases. J. Urol.,
86: 345-351, 1961.
11 Zielinski, W.: Przypadek obojnactwa prawdziwego. Pediat. Pol., 36: 645-650, 1961.
539
FIG. 2. External sex organs velopment were proper for her age. The penis was 3 cm. long, curved downward. The glans penis was not covered with prepuce. On the lower surface of the penis there was a narrow strip of mucous membrane running from the perineum right up to the glans penis. The external orifice of urethra was on the perineum. Just below the penis there were two skin folds similar to labia majora. The right, larger fold became smaller under pressure. Inside the fold
540
WOJEWSKI AND KRASON
there was an oval formation resembling the testis (fig. 2). On the basis of the examination covering the nuclear chromatin of epithelial cells of mucous membrane in the mouth, feminine
FIG. 3. Urethrovaginogram
chromosome sex was established. Urethrography by means of 2 catheters revealed the presence of rudimentary vagina (fig. 3). The urine 17-ketosteroid level was 0.65 mg./24 hours. During operation on the right inguinal hernia a node the size of a bean was noted in the hernial sac. One half of the node was whitish, its surface being uneven and covered with numerous, bluishly translucent, small cysts, while the other half was smooth, soft, of rosy color. A section was taken from the node. By widening the hernia the abdominal cavity was opened. The anatomical proportions in the region of pelvis were like those in a boy. No opposite gonad was found. There was a round, hard formation of a small hazel-nut size outside the bladder and under the peritoneum. A section was also taken from this node. Histopathological examination: The first section was ovarian tissue with primary vesicles as well as seminal tubules. Stunted spermatogenesis was seen in the preparation. There was no distinct line of demarcation between these two types of tissues (fig. 4). Diagnosis: ovariotestis. The second section contained intermingling bundles of muscular fibers. In the center there was a narrow lumen lined with columnar epithelium.
FIG. 4. Photomicrograph. Testicular and ovarian tissues are seen in preparation. Hematoxylin and eosin, XlOO
A TRUE HER'VIAPH!WDITE
to Dr. Stojalowski this tissue may represent a rudimentary uterus. The hermaphrodite was discharged from the department and advised to return after the period of sexual maturation. In Klebs' classification the case just deseribecl belongs to the group of unilateral true hermaphroditism (hermaphroditism us verus unilateralis). On the right side the testicular and the ovarian tissues fuse to one formation of bean size (ovariotestis), while on the left side no gonad was found. The nuclear chromatin pattern positively pointed to the female sex, but the sex organs were much
more similar to those of operation was postponed maturation so that the make a decision as to sex.
The plusfa
SU1IMARY
A case of unilateral true hermaphroditisrn been described. On one side, in the gonad, tissue of testicle and ovary was present; second gonad was not found. Sex chronwtin pattern was female. The sex organs were developed and similar to male ones. The cfor·i,.ilc,11 on se.\: was no,st1;1onen till sex maturation.