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CLINICAL IMAGE
Year : 2020  |  Volume : 152  |  Issue : 7  |  Page : 109

A rare case of 46XX ovotesticular disorder of sex development


Department of Endocrinology, Andhra Medical College, Visakhapatnam 530 002, Andhra Pradesh, India

Date of Submission19-Nov-2019
Date of Web Publication25-May-2021

Correspondence Address:
Sagar S.L. Reddy
Department of Endocrinology, Andhra Medical College, Visakhapatnam 530 002, Andhra Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijmr.IJMR_2178_19

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How to cite this article:
Reddy SS, Subrahmanyam K A. A rare case of 46XX ovotesticular disorder of sex development. Indian J Med Res 2020;152, Suppl S1:109

How to cite this URL:
Reddy SS, Subrahmanyam K A. A rare case of 46XX ovotesticular disorder of sex development. Indian J Med Res [serial online] 2020 [cited 2021 Jul 29];152, Suppl S1:109. Available from: https://www.ijmr.org.in/text.asp?2020/152/7/109/316763

Consent to publish clinical information and images obtained from patients parents.


A 10 yr old male child was brought to the department of Endocrinology, Andhra Medical College, Visakhapatnam, India, in October 2019, in view of ambiguous genitalia with non-palpable left gonad and passing urine beneath phallus since birth. Normal anthropometry was noted, and the child was brought up as male since birth. Phallic length was 3.5 cm and right scrotal gonad of 3 ml with no palpable gonad in the left labial fold was seen. Urethral and vaginal openings were noted [Figure 1] and a karyotype of 46XX was observed [Figure 2]. Magnetic resonance imaging revealed severe hypoplastic uterus with the left  Fallopian tube More Details and ovary of 35 × 10 mm [Figure 3]. Pre-pubertal hormonal profile was observed with greater than two times elevation of testosterone upon human chorionic gonadotrophin stimulation. As parents wanted to raise the child as a male, rudimentary uterus and intra-abdominal gonad removal and correction of perineal hypospadias was planned after re-evaluation at 14 yr of age.
Figure 1: Phallus with right hemiscrotum with two opening - urethral opening (open arrows) and vaginal opening (solid arrows).

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Figure 2: Karyotyping of the child showing 46XX.

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Figure 3: Magnetic resonance imaging showing rudimentary uterus (red arrow) and right side spermatic cord (green arrow).

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Conflicts of Interest: None.


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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