Pathogenesis, Diagnosis, and Management of Splenogonadal Fusion: A Literature Review
Introduction. Splenogonadal fusion is a rare congenital anomaly, defined by the presence of ectopic splenic tissue caused by an abnormal connection between the spleen and the gonad or mesonephrotic derivatives during the embryonic period. Materials and Methods. By reporting an observational case and...
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Wiley
2020-01-01
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| Series: | Case Reports in Urology |
| Online Access: | http://dx.doi.org/10.1155/2020/8876219 |
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| author | Youssef Kadouri Damien Carnicelli Hachem El Sayegh Lounis Benslimane Yassine Nouini |
| author_facet | Youssef Kadouri Damien Carnicelli Hachem El Sayegh Lounis Benslimane Yassine Nouini |
| author_sort | Youssef Kadouri |
| collection | DOAJ |
| description | Introduction. Splenogonadal fusion is a rare congenital anomaly, defined by the presence of ectopic splenic tissue caused by an abnormal connection between the spleen and the gonad or mesonephrotic derivatives during the embryonic period. Materials and Methods. By reporting an observational case and performing a review of the literature according to the CARE guidelines (using the PubMed database and guidelines from urology, general surgery, and pediatric learned societies), we present the embryological genesis of the splenogonadal fusion, the associated anatomical anomalies, and the diagnostic procedure. Observation. We report the case of a patient aged 45, with no notable history, reporting left testicular pain. A small nodule on the upper pole of the left testicular was clinically palpable. Tumor markers were normal, and scrotal ultrasound depicted a hypoechoic hypervascular nodule measuring 8∗6∗8 mm. After validation in a multidisciplinary oncology consultation meeting and opinion from a uro-andrologist expert, the patient underwent an inguinal lumpectomy with an extemporaneous examination which did not objectify any signs of malignancy. Ultimately, it is a normal spleen tissue in the testicular ectopic position. Discussion. Splenogonadal fusion corresponds to a rare congenital malformation; less than 200 cases have been published in the literature, most often affecting boys, with a sex ratio of 15/1. Two types are described, depending on the continuity of the link between the orthotopic spleen and the gonad: the continuous and discontinuous forms. In a third of the cases, there are associated congenital malformations and particularly in the continuous forms (44 to 50% of the cases): anomalies of the limbs, micrognathia, microgyria, and hepatic and digestive abnormalities. Cryptorchidism is associated with the continuous form in 31% of cases. The preoperative diagnosis remains difficult because of its morphological and clinical characteristics suggesting a tumor process. |
| format | Article |
| id | doaj-art-dfa55aeb63494649bf505d2ed727a1d7 |
| institution | Kabale University |
| issn | 2090-696X 2090-6978 |
| language | English |
| publishDate | 2020-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Urology |
| spelling | doaj-art-dfa55aeb63494649bf505d2ed727a1d72025-08-20T03:26:24ZengWileyCase Reports in Urology2090-696X2090-69782020-01-01202010.1155/2020/88762198876219Pathogenesis, Diagnosis, and Management of Splenogonadal Fusion: A Literature ReviewYoussef Kadouri0Damien Carnicelli1Hachem El Sayegh2Lounis Benslimane3Yassine Nouini4Faculty of Medicine and Pharmacy of Rabat Morocco, Ibn Sina Hospital, Department of Urology A, Mohammed V University, MoroccoDepartment of Urology and Andrology, Center Hospitalier Métropole Savoie, Chambery, FranceFaculty of Medicine and Pharmacy of Rabat Morocco, Ibn Sina Hospital, Department of Urology A, Mohammed V University, MoroccoFaculty of Medicine and Pharmacy of Rabat Morocco, Ibn Sina Hospital, Department of Urology A, Mohammed V University, MoroccoFaculty of Medicine and Pharmacy of Rabat Morocco, Ibn Sina Hospital, Department of Urology A, Mohammed V University, MoroccoIntroduction. Splenogonadal fusion is a rare congenital anomaly, defined by the presence of ectopic splenic tissue caused by an abnormal connection between the spleen and the gonad or mesonephrotic derivatives during the embryonic period. Materials and Methods. By reporting an observational case and performing a review of the literature according to the CARE guidelines (using the PubMed database and guidelines from urology, general surgery, and pediatric learned societies), we present the embryological genesis of the splenogonadal fusion, the associated anatomical anomalies, and the diagnostic procedure. Observation. We report the case of a patient aged 45, with no notable history, reporting left testicular pain. A small nodule on the upper pole of the left testicular was clinically palpable. Tumor markers were normal, and scrotal ultrasound depicted a hypoechoic hypervascular nodule measuring 8∗6∗8 mm. After validation in a multidisciplinary oncology consultation meeting and opinion from a uro-andrologist expert, the patient underwent an inguinal lumpectomy with an extemporaneous examination which did not objectify any signs of malignancy. Ultimately, it is a normal spleen tissue in the testicular ectopic position. Discussion. Splenogonadal fusion corresponds to a rare congenital malformation; less than 200 cases have been published in the literature, most often affecting boys, with a sex ratio of 15/1. Two types are described, depending on the continuity of the link between the orthotopic spleen and the gonad: the continuous and discontinuous forms. In a third of the cases, there are associated congenital malformations and particularly in the continuous forms (44 to 50% of the cases): anomalies of the limbs, micrognathia, microgyria, and hepatic and digestive abnormalities. Cryptorchidism is associated with the continuous form in 31% of cases. The preoperative diagnosis remains difficult because of its morphological and clinical characteristics suggesting a tumor process.http://dx.doi.org/10.1155/2020/8876219 |
| spellingShingle | Youssef Kadouri Damien Carnicelli Hachem El Sayegh Lounis Benslimane Yassine Nouini Pathogenesis, Diagnosis, and Management of Splenogonadal Fusion: A Literature Review Case Reports in Urology |
| title | Pathogenesis, Diagnosis, and Management of Splenogonadal Fusion: A Literature Review |
| title_full | Pathogenesis, Diagnosis, and Management of Splenogonadal Fusion: A Literature Review |
| title_fullStr | Pathogenesis, Diagnosis, and Management of Splenogonadal Fusion: A Literature Review |
| title_full_unstemmed | Pathogenesis, Diagnosis, and Management of Splenogonadal Fusion: A Literature Review |
| title_short | Pathogenesis, Diagnosis, and Management of Splenogonadal Fusion: A Literature Review |
| title_sort | pathogenesis diagnosis and management of splenogonadal fusion a literature review |
| url | http://dx.doi.org/10.1155/2020/8876219 |
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