Ahmet Celik1,2, Sibel Tiryaki3, Sukran Darcan4, Orkan Ergun3. 1. Department of Pediatric Surgery, Ege University Faculty of Medicine, Izmir, Turkey. ahmet.celik@ege.edu.tr. 2. Department of Pediatrics, Division of Pediatric Endocrinology, Ege University Faculty of Medicine, Izmir, Turkey. ahmet.celik@ege.edu.tr. 3. Department of Pediatric Surgery, Ege University Faculty of Medicine, Izmir, Turkey. 4. Department of Pediatrics, Division of Pediatric Endocrinology, Ege University Faculty of Medicine, Izmir, Turkey.
Abstract
BACKGROUND: Splenogonadal fusion is a rare congenital anomaly which is characterized by fusion formation between the spleen and gonad. METHODS: We report a case of a 14-month boy with spleongonadal fusion-limb deformity syndrome focusing on the importance of awareness of this syndrome. RESULTS: The patient was admitted to our clinic because of a left undescended testis, and preoperative diagnosis was not made. During the operation, "spleen-like" tissue attached to the gonad induced splenogonadal fusion, which was confirmed by laparoscopy. The patient also had a short right femur, hip dysplasia and a syndromic face. CONCLUSION: Splenogonadal fusion anomaly should be considered in the evaluation of undescended testis, especially in patients with facial and limb deformities.
BACKGROUND: Splenogonadal fusion is a rare congenital anomaly which is characterized by fusion formation between the spleen and gonad. METHODS: We report a case of a 14-month boy with spleongonadal fusion-limb deformity syndrome focusing on the importance of awareness of this syndrome. RESULTS: The patient was admitted to our clinic because of a left undescended testis, and preoperative diagnosis was not made. During the operation, "spleen-like" tissue attached to the gonad induced splenogonadal fusion, which was confirmed by laparoscopy. The patient also had a short right femur, hip dysplasia and a syndromic face. CONCLUSION: Splenogonadal fusion anomaly should be considered in the evaluation of undescended testis, especially in patients with facial and limb deformities.
Authors: Fiona McPherson; Jaime L Frias; Diane Spicer; John M Opitz; Enid F Gilbert-Barness Journal: Am J Med Genet A Date: 2003-08-01 Impact factor: 2.802
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