The Prune Belly Syndrome in a Female Foetus with Urorectal Septum Malformation Sequence: A Case Report on a Rare Entity with an Unusual Association
The prune belly syndrome is a rare congenital anomaly which is characterized by the triad of an absent or a deficient development of the abdominal muscle, bilateral cryptorchidism and an anomalous urinary tract. In its full form, this condition occurs only in males. However, a similar condition oc...
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JCDR Research and Publications Private Limited
2013-08-01
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| Series: | Journal of Clinical and Diagnostic Research |
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| Online Access: | https://jcdr.net/articles/PDF/3243/62-5672_PF1(M)_E(C)_F(T)_F1(V)_F1(T)_PF1(PVi_R)_PFA(PR)_PF3_(PUH)_B.pdf |
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| author | Dibyajyoti Goswami Giriraj Kusre Hemonta Kumar Dutta Adity Sarma |
| author_facet | Dibyajyoti Goswami Giriraj Kusre Hemonta Kumar Dutta Adity Sarma |
| author_sort | Dibyajyoti Goswami |
| collection | DOAJ |
| description | The prune belly syndrome is a rare congenital anomaly which is characterized by the triad of an absent or a deficient
development of the abdominal muscle, bilateral cryptorchidism and an anomalous urinary tract. In its full form, this condition
occurs only in males. However, a similar condition occurs in females in the absence of cryptorchidism. On the other hand, the
urorectal septum malformation sequence is a lethal congenital malformation which is characterized by the development of a
phallus like structure, a smooth perineum and the absence of urethral, vaginal and anal openings. We are reporting a case
of a female foetus with the prune belly syndrome, which was associated with a urorectal septum malformation sequence.
A dead foetus with a protruded abdomen and ambiguous genitalia, was born at 32 weeks of pregnancy. On autopsy, it was found to
have female internal genital organs. The left kidney, the urinary bladder and the rectum were absent. The sigmoid colon, the ureters and
the fallopian tubes opened into a common cloacal sac. The histopathological examination of the ovary showed the presence of Leydig’s
cells. The occurrence of the female counterpart of the prune belly syndrome is extremely rare and only few of such cases were found
to be discussed in the details in the indexed English literature so far. Hence, we hope that this case report will contribute to the existing
knowledge on the prune belly syndrome. |
| first_indexed | 2024-12-10T23:37:07Z |
| format | Article |
| id | doaj.art-72776f62f1734a48b311ca9cb1826d1a |
| institution | Directory Open Access Journal |
| issn | 2249-782X 0973-709X |
| language | English |
| last_indexed | 2024-12-10T23:37:07Z |
| publishDate | 2013-08-01 |
| publisher | JCDR Research and Publications Private Limited |
| record_format | Article |
| series | Journal of Clinical and Diagnostic Research |
| spelling | doaj.art-72776f62f1734a48b311ca9cb1826d1a2022-12-22T01:29:09ZengJCDR Research and Publications Private LimitedJournal of Clinical and Diagnostic Research2249-782X0973-709X2013-08-01781727172910.7860/JCDR/2013/5672.3243The Prune Belly Syndrome in a Female Foetus with Urorectal Septum Malformation Sequence: A Case Report on a Rare Entity with an Unusual AssociationDibyajyoti Goswami0Giriraj Kusre1Hemonta Kumar Dutta2Adity Sarma3Post Graduate Trainee, Department of Anatomy, Assam Medical College and Hospital, Dibrugarh, Assam-786002, India.Associate Professor, Department of Anatomy, Assam Medical College and Hospital, Dibrugarh, Assam-786002, India.Associate Professor, Department of Paediatric Surgery, Assam Medical College and Hospital, Dibrugarh, Assam-786002, India.Associate Professor, Department of Pathology, Assam Medical College and Hospital, Dibrugarh, Assam-786002, India.The prune belly syndrome is a rare congenital anomaly which is characterized by the triad of an absent or a deficient development of the abdominal muscle, bilateral cryptorchidism and an anomalous urinary tract. In its full form, this condition occurs only in males. However, a similar condition occurs in females in the absence of cryptorchidism. On the other hand, the urorectal septum malformation sequence is a lethal congenital malformation which is characterized by the development of a phallus like structure, a smooth perineum and the absence of urethral, vaginal and anal openings. We are reporting a case of a female foetus with the prune belly syndrome, which was associated with a urorectal septum malformation sequence. A dead foetus with a protruded abdomen and ambiguous genitalia, was born at 32 weeks of pregnancy. On autopsy, it was found to have female internal genital organs. The left kidney, the urinary bladder and the rectum were absent. The sigmoid colon, the ureters and the fallopian tubes opened into a common cloacal sac. The histopathological examination of the ovary showed the presence of Leydig’s cells. The occurrence of the female counterpart of the prune belly syndrome is extremely rare and only few of such cases were found to be discussed in the details in the indexed English literature so far. Hence, we hope that this case report will contribute to the existing knowledge on the prune belly syndrome.https://jcdr.net/articles/PDF/3243/62-5672_PF1(M)_E(C)_F(T)_F1(V)_F1(T)_PF1(PVi_R)_PFA(PR)_PF3_(PUH)_B.pdfabdominal wallautopsycryptorchidismprune belly syndromeleydig’s cells |
| spellingShingle | Dibyajyoti Goswami Giriraj Kusre Hemonta Kumar Dutta Adity Sarma The Prune Belly Syndrome in a Female Foetus with Urorectal Septum Malformation Sequence: A Case Report on a Rare Entity with an Unusual Association Journal of Clinical and Diagnostic Research abdominal wall autopsy cryptorchidism prune belly syndrome leydig’s cells |
| title | The Prune Belly Syndrome in a Female Foetus with Urorectal Septum Malformation Sequence: A Case Report on a Rare Entity with an Unusual Association |
| title_full | The Prune Belly Syndrome in a Female Foetus with Urorectal Septum Malformation Sequence: A Case Report on a Rare Entity with an Unusual Association |
| title_fullStr | The Prune Belly Syndrome in a Female Foetus with Urorectal Septum Malformation Sequence: A Case Report on a Rare Entity with an Unusual Association |
| title_full_unstemmed | The Prune Belly Syndrome in a Female Foetus with Urorectal Septum Malformation Sequence: A Case Report on a Rare Entity with an Unusual Association |
| title_short | The Prune Belly Syndrome in a Female Foetus with Urorectal Septum Malformation Sequence: A Case Report on a Rare Entity with an Unusual Association |
| title_sort | prune belly syndrome in a female foetus with urorectal septum malformation sequence a case report on a rare entity with an unusual association |
| topic | abdominal wall autopsy cryptorchidism prune belly syndrome leydig’s cells |
| url | https://jcdr.net/articles/PDF/3243/62-5672_PF1(M)_E(C)_F(T)_F1(V)_F1(T)_PF1(PVi_R)_PFA(PR)_PF3_(PUH)_B.pdf |
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