A neonate born at 36 weeks of gestation through vaginal route with a
birth weight of 2.1 kg was noted to have abnormal genitalia. There was
bilaterally symmetrical duplication of labia minora, urethral openings,
vaginal openings and anal dimples (Fig. 1). Both the anal
openings were imperforate. Single pair of labia majora was visible on
either side. The child was passing meconium through both the vaginal
openings suggestive of bilateral recto-vaginal fistulae. On
ultrasonography, kidneys were normal with no duplication of
pelvicalyceal system. Child had single urinary bladder, two urethra,
single uterine cavity, single cervix with two vagina. Keeping in view
the clinical and sonographic findings, we made a diagnosis of caudal
duplication syndrome. The neonate later died due to shock and renal
failure.
 |
Fig. 1 Duplication of labia minora
(yellow arrow), urethral openings (blue arrow), vaginal orifices
(red arrow) and anal dimples (black arrow).
|
Caudal duplication syndrome consists of duplication
of genitourinary, gastrointestinal system, especially hind gut,
vertebral column, spinal cord and lower limb. The theories postulated
include abnormal adherence between ectoderm and endoderm; or damage to
caudal cell mass and posterior hind gut at day 23 to 25 of pregnancy. No
risk factor and sex predominance is described. Treatment with
multi-staged corrective surgeries is individualized according to extent
of duplication and functionality of organ systems involved.