External Genital Aplasia with PruneBelly Syndrome in a Female Foetus - A Rare Case Report
The Prune-Belly Syndrome (PBS) is a rare congenital anomaly with unknown etiology that is characterised by the triad of absent or a deficient development of the abdominal muscles, bilateral cryptorchidism and an anomalous urinary tract. This condition occurs only in males in its full form. Howe...
| Main Authors: | , , |
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| Format: | Article |
| Language: | English |
| Published: |
JCDR Research and Publications Pvt. Ltd.
2018-01-01
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| Series: | International Journal of Anatomy Radiology and Surgery |
| Subjects: | |
| Online Access: | http://www.ijars.net/article_fulltext.asp?issn=0973-709x&year=2018&month=January&volume=7&issue=1&page=AC04-AC07&id=2351 |
| Summary: | The Prune-Belly Syndrome (PBS) is a rare congenital
anomaly with unknown etiology that is characterised
by the triad of absent or a deficient development of the
abdominal muscles, bilateral cryptorchidism and an
anomalous urinary tract. This condition occurs only in
males in its full form. However, a similar condition occurs
in females in the absence of cryptorchidism and presence
of triad showing abdominal musculature deficiency,
anomalies of urinary and genital system. A 22-week-old
dead foetus after abortion had foetal abnormalities like
Potter’s facies, distended abdomen, absent genitalia,
absent urethral orifice, absent vaginal orifice, talipes equino
varus and gross oligohydramnios was donated to Anatomy
Department, for further evaluations. On autopsy, it was
found to have grossly dilated urinary bladder filling entire
abdominal cavity, female internal genital organs, urethral
atresia, vaginal atresia, bilateral hydroureteronephrosis,
two hemivaginae entering into the urinary bladder, uterus
didelphys, bowel loops on right side below the liver and
hypoplastic lungs. The histopathological examination
of the uterus, both fallopian tubes and ovary was done.
Only few cases of female counterpart of PBS have been
discussed in literature so far. |
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| ISSN: | 2277-8543 2455-6874 |