patho histo

1. A RARE CASE OF
AMBIGUOUS GENITALIA
Dr Ritu Ranjan- 3rd yr Resident ,Pathology
Dr P. K. Agarwal-- Consultant Pathology
Dr Ashutosh Pandey –Paediatric surgeon
Dr Pooja Singh-1st yr Resident Pathology
Vivekananda Polyclinic & IMS Lucknow

2. CLINICAL HISTORY
 7 year old child
 Presented with swelling in the external genital
region
 No history of abdominal pain
 Referred to the pediatric surgery department of
VPIMS

3. CLINICAL EXAMINATION
 Fully developed vagina and urethral orifice
 Clitoromegaly
 No skin pigmentation
 No labioscrotal fusion
 No palpable gonads
 Adrenarche not attained

5. FAMILY HISTORY
 No significant family history
 No history of any drug intake by the mother in the
antenatal period

6. INVESTIGATIONS DONE
USG-
 Uterus not clearly seen
 Bilateral inguinal hernia
 Right sided streak ovary
 Left sided ovotestis
KARYOTYPING-
 46XY, 45XO
HORMONAL ASSAY-
 Done, but reports not available

7. TREATMENT GIVEN- SURGERY
 LAPAROSCOPIC GONADECTOMY AND
GENITOPLASTY WITH CLITORAL
REDUCTION

8. GROSS EXAMINATION

9. HISTOPATHOLOGICAL EXAMINATION
 THE SPECIMEN WAS RECEIVED IN THE
PATHOLOGY LABORATORY OF THE
DEPARTMENT FOR HISTOPATHOLOGICAL
EXAMINATION
 SPECIMEN WAS FIXED IN 10% FORMAL SALINE
FOR ROUTINE PARAFFIN SECTIONING
 AFTER GROSS EXAMINATION THE TISSUE WAS
SUBMITTED FOR PARAFFIN SECTIONS

10. GROSS APPEARANCE OF THE
SPECIMEN

11. CUT SURFACE OF THE SPECIMEN-

31. POST OPERATIVE

32. DISCUSSION
 GENITAL AMBIGUITY OCCURS IN 1 IN 4,500
BIRTHS, AND OVOTESTICULAR DISORDER OF
SEXUAL DEVELOPMENT OCCURS IN FEWER
THAN 10% OF ALL DISORDERS OF SEXUAL
DEVELOPMENT
 DESPITE THE FACT THAT MOST PEOPLE WITH
TRUE HERMAPHRODITISM PRESENT WITH
GENITAL AMBIGUITY, LESS THAN 20% ARE
DIAGNOSED BEFORE AGE 5 YEARS.
 THE PRESENT CASE WAS 7 YRS OLD ONLY.

33.  RARE CONDITION.
 OVOTESTIS - OVOTESTICULAR DISORDER OF SEXUAL
DEVELOPMENT (OT-DSD), FORMERLY KNOWN AS TRUE
HERMAPHRODITISM.
 CONSISTING OF OVARIAN AS WELL AS TESTICULAR
TISSUES.
 IN CASES OF OT-STD, OVOTESTES ARE THE MOST
FREQUENT GONAD PRESENT, FOLLOWED BY THE
OVARY AND THEN THE TESTIS.
 THE PRESENT CASE HAD BOTH THE ELEMENTS I.E.
OVARY ALONG WITH FALLOPIAN TUBE AND TESTIS

34. CONCLUSION
 AMBIGUOUS GENITALIA
 TRUE HERMAPHRODITE-OVARIAN & TESTICULAR
TISSUES
 INTERSEX-SEXUAL ORGANS- LARGER THAN
CLITORIS & SMALLER THAN PENIS
 MIXED GONADAL DYSGENESIS- ATYPICAL SET OF
SEX CHROMATIN- 46XY, 45XO
 IT IS A DISORDERED SEXUAL DEVELOPMENT (DSD)

35. THANK YOU

36. DEVELOPMENT OF GONADS IN FETUS