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|Year : 1978 | Volume
| Issue : 4 | Page : 240-242
The Slotnick-Goldfarb's syndrome in infertility- (a case report)
VN Purandare, AV Sathe
Department of Obstetrics & Gynaecology, K.E.M. Hospital and Seth G.S. Medical College, Parel, Bombay 400 012., India
V N Purandare
Department of Obstetrics & Gynaecology, K.E.M. Hospital and Seth G.S. Medical College, Parel, Bombay 400 012.
A case of the unilateral streaked ovary syndrome is presented with documentation by cytologic, chromosomal, laparoscopic and hormonal studies. This, the Slotnick-Goldfarb's syndrome, is characterized by oligomenorrhoea progressing into secondary amenorrhoea in a normal phenotypic female with a streaked ovary in one adnexa and an hypoplastic ovary in the other. This case presented with secondary amenorrhoea and primary sterility. She was tall with an increased carrying angle and had hypo plastic breasts and underdeveloped external and internal genitalia. Investigations revealed a normal female karyotype, a mid-zonal maturation index, low normal urinary ketosteroids and elevated urinary gonadotrophins. Laparoscopy clinched the diagnosis. The aetiology, prognosis and treatment are briefly discussed. Stress is laid on the fact that whilst investigating a case of sterility and amenorrhoea, this new syndrome must be kept in mind.
|How to cite this article:|
Purandare V N, Sathe A V. The Slotnick-Goldfarb's syndrome in infertility- (a case report). J Postgrad Med 1978;24:240-2
| :: Introduction|| |
This report concerns a patient with the unilateral streaked ovary syndrome (Slotnick-Goldfarb's syndrome). This new syndrome, first described by Slotnick and Goldfarb in 1972, is characterized by oligomenorrhoea progressing into secondary amenorrhoea in a normal phenotypic female who has a streaked ovary in one adnexa and a hypoplastic ovary in the other. This new entity must be considered when evaluating an in fertile patient with oligomenorrhoea or secondary amenorrhoea with hypergonadotrophic oestrogen deficiency.
The term gonadal dysgenesis encompasses a wide variety of chromosomal anomalies and somatic stigmas. Gonadal dysgenesis is defined as a pathologic syndrome characterized by lack of germ cells in a patient with external and internal features of the female sex. Thus we have the classic form of gonadal dysgenesis as described by Turner in 1938. This syndrome is characterized by primary amenorrhoea, lack of secondary sexual development and multiple somatic variants. Several new variants have been described since. The term "pure gonadal dysgenesis" was first coined by Harnden and Stewart  in 1959 and later Sohval  coined the term "mixed gonadal dysgenesis". Recently a syndrome characterized by an unilateral streaked ovary associated with a contralateral hypoplastic ovary has been described. This, the Slotnick-Goldfarb's syndrome, appears to be a variant of gonadal dysgenesis.  The streaked ovary probably results from unknown congenital factors and the hypoplastic ovary from suspected viral factors in postnatal life. The purpose of this paper is to report one patient with the Slotnick-Goldfarb's syndrome who presented with secondary amenorrhoea and primary sterility.
| :: Case report|| |
The patient, R.P., was the product of a normal pregnancy and labour being the first of two siblings. The only significant past history was that of measles and mumps during childhood. The patient presented with secondary amenorrhoea and primary sterility. Menarche had been at 14 years. The patient had only three spontaneous menses. This was followed by oligomenorrhoea which soon ended in secondary amenorrhoea. The patient has been on hormones off and on, since then.
Examination revealed a tall, thin patient, weighing 35 kgs., with a height of 161 cms and a span of 162 cms. Besides a slightly increased carrying angle, there were no skeletal abnormalities. The breasts and external genitalia were hypoplastic. Axillary and pubic hair were scanty. The vagina was narrow and the uterus was atrophic.
Investigations revealed a haemogram within normal limits. The urine and stool examination revealed no abnormality. The chest X-ray and the lateral skull film for the sella turcica were normal. No bony abnormalities (such as short 4th metacarpal, carpal angle), were detected. The buccal smear revealed 12% Barr bodies and the karyotype revealed a 46 XX (normal female) pattern. The vaginal cytology revealed a maturation index varying from 0/100/0 to 0/90/10 with poor cervical mucous. The progesterone test was negative and the oestrogen test was positive. 17 Keto-steroids were low (normal-6.1 mg/24 hrs), 17 KGS were 5.0 mg/ 24 hrs. Urinary gonadotrophins were elevated. Serial total urinary oestrogens were below 10 µg/24 hrs (on 3 occasions, 1 week apart). Laparoscopy revealed a smaller than normal uterus, normal tubes and a left streak gonad with a linear (1.5 cms) right hypoplastic ovary.
| :: Discussion|| |
A unilateral streaked or absent ovary may not be brought to light till the other ovary fails. This is exactly the mode by which we pick up patients with a unilaterally streaked or absent ovary. The Slotnick-Goldfarb's syndrome concerns a phenotypic female with a streaked ovary in one adnexa and a hypoplastic ovary in the other. These individuals have enough gonadal function to initiate puberty and obtain sexual maturation. The gonadal reserve is limited. Thus these individuals have breast development and axillary and pubic hair. As a result of the limited gonadal reserve oestrogen production fails resulting in amenorrhoea and elevated gonadotrophin levels.
Our case had her menarche at 14 years and soon went in for oligomenorrhoea. This proceeded on to secondary amenorrhoea. She had a fair amount of breast development starting at around 13 years. Her pubic and axillary hair appeared at around 13½ years and have somewhat regressed. The past history of measles and mumps appears significant.
As far as the aetiology of this syndrome is concerned no one is quite sure as to the cause. It could be the result of genetic and/or developmental factors. The unilateral streaked ovary is possibly an expression of gonadal dysgenesis whilst the hypoplastic ovary, an expression of destruction by viral oophoritis.
In our case the mumps oophoritis may have been the factor causing the ovarian failure in the hypoplastic ovary. However, other factors as outlined by Emperaire  cannot he ruled out. Bone survey did not reveal any skeletal anomaly. Gonadal biopsy was not done in our case. This patient's husband's semen was of a poor quality hence gonadotrophin or clomiphene stimulation of the ovary was not carried out. Jones et at  have described a syndrome with hypergonadotrophic amenorrhoea with a normal folicular apparatus which responded weakly to higher than normal doses of gonadotrophins. Neves-Y-Castro et al  have recently described a case of Slotnick-Goldfarb's syndrome with no response to gonadotrophin stimulation.
We thus stress the need for keeping this new syndrome in mind while evaluating a case of sterility and amenorrhoea. As yet, the prognosis is poor. Substitutional therapy is all that we can offer to the patient (as is being done in our case). Gonadectomy does not seem to be indicated as the Y chromosome is absent from the karyotype.
| :: References|| |
|1.||Emperaire, J. C., Audebert, A. and Greenblatt, R. B.: Premature ovarian failure. Amer. J. Obstet. & Gynaec. 108: 445-449,1970. |
|2.||Harnden, D. G. and Stewart, J. S. S.: The chromosomes in a case of pure gonadal dysgenesis. Brit. Med. J., 2: 1285-1287, 1959. |
|3.||Jones, G. E S. and Moraes-Ruchsen, M.: A new syndrome of amenorrhoea in association with hypergonadotrophism and apparently normal follicular apparatus. Amer. J. Obstet. & Gynaec. 104: 597-600, 1969. |
|4.||Neves-Y-Castro, M., Neves-Da Silva, J., Melero-De-Souza, L_ and Reis-Valle, A.: Unilateral streaked ovary syndrome. Obstet. & Gynaec., 47: 86-89, 1976. |
|5.||Slotnick, E. A. and Goldfarb, A. F.: Unilateral streaked ovary syndrome. Obstet. & Gynaec., 39: 269-273, 1972. |
|6.||Sohval, A. R.: Hermaphroditism with "Atypical" or "Mixed" gonadal dysgenesis. Amer. J. Med., 36: 281-292, 1964 |
|7.||Turner, H. H.: A syndrome of infantilism, Congenital webbed neck and cubitus valgus, Endocrinology, 23: 566-574, 1938. |