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Splenogonadal fusion-limb deformity syndrome: a rare but important cause of undescended testis

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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.

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Authors and Affiliations

  1. Department of Pediatric Surgery, Ege University Faculty of Medicine, Izmir, Turkey

    Ahmet Celik, Sibel Tiryaki & Orkan Ergun

  2. Department of Pediatrics, Division of Pediatric Endocrinology, Ege University Faculty of Medicine, Izmir, Turkey

    Ahmet Celik & Sukran Darcan

Authors
  1. Ahmet Celik
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  2. Sibel Tiryaki
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  3. Sukran Darcan
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  4. Orkan Ergun
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Correspondence to Ahmet Celik.

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Celik, A., Tiryaki, S., Darcan, S. et al. Splenogonadal fusion-limb deformity syndrome: a rare but important cause of undescended testis. World J Pediatr 12, 246–248 (2016). https://doi.org/10.1007/s12519-015-0046-y

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  • DOI: https://doi.org/10.1007/s12519-015-0046-y

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