Splenogonadal Fusion

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Classification according to ICD-10
Q89.0 Congenital malformations of the spleen
ICD-10 online (WHO version 2019)

The Splenogonadale fusion is a very rare disorder of embryonic development with an abnormal connection between spleen and gonads . There is an adhesion between a gonad and an atypical part of the spleen.

Intra-operative photo with the spleen attached to the left testicle in a one-year-old with splenogonadal fusion

A splenogonadal fusion can also occur together with other malformations, the SGFLD syndrome .

The first description comes from the year 1883 by the Baltic - German pathologist Eugen Woldemar Bostroem (1850–1928).

pathology

It is believed that this fusion between spleen and testicular tissue occurs between the 4th and 10th week of pregnancy .

distribution

The frequency is given as less than 1 in 1,000,000, so far more than 150 people have been reported.

The male sex is almost exclusively affected, mostly the left side and in 95% of the testicles .

Classification

Depending on whether or not there is a connection between the main spleen and the gonad, the following distinction is possible:

  • Continuous with a strand-like connection. Additional malformations are often found here , mostly cryptorchidism
  • Discontinuous with ectopic spleen tissue attached to the gonad

Clinical manifestations

The fusion can remain clinically silent and undetected, it can be found by chance during (groin) surgery or it can appear as a testicular tumor.

Differential diagnosis

Differentiation from germ cell tumors and other testicular tumors is usually not possible with imaging . In individual cases, a vascular connection between the tumor and the spleen can be found in the color Doppler , and the spleen tissue can be recognized as such in magnetic resonance imaging . A liver-spleen scintigraphy with 99m technetium can point the way.

literature

  • A. Abokrecha, A. Almatrfi: Discontinued Splenogonadal Fusion and Bilateral Empty Scrotum in an 18-Month-Old Boy. In: European Journal of Pediatric Surgery Reports. Vol. 05, 2017, p. E1, doi: 10.1055 / s-0037-1598624
  • G. Huang, Y. Huang, L. Zeng, M. Yuan, Y. Wu, L. Huang: Continuous-type splenogonadal fusion: A case report. In: Experimental and therapeutic medicine. Vol. 13, No. 5, May 2017, pp. 2019-2021, doi: 10.3892 / etm.2017.4198 , PMID 28565802 , PMC 5443268 (free full text).
  • J. Preece, S. Phillips, V. Sorokin, D. Herz: Splenogonadal fusion in an 18-month-old. In: Journal of pediatric urology. Vol. 13, No. 2, April 2017, pp. 214-215, doi: 10.1016 / j.jpurol.2016.06.005 , PMID 28129957 .

Individual evidence

  1. ↑ a b Splenogonadal fusion, isolated. In: Orphanet (Rare Disease Database).
  2. ↑ a b c d Radiopaedia
  3. ↑ Splenogonadale Fusion - limb malformation - micrognathia. In: Orphanet (Rare Disease Database).
  4. ↑ E. Bostroem: Demonstration of a preparation of the adhesion of the spleen to the left testicle. In: Society of German Natural Scientists and Doctors, Negotiations of the 56 Assembly, Freiburg, 1883, p. 149.
  5. Jump up ↑  NJ Kocher, JJ Tomaszewski, RB Parsons, BR Cronson, H. Altman, A. Kutikov, RE Smith: Splenogonadal fusion: a rare etiology of solid testicular mass. In: Urology. Vol. 83, No. 1, January 2014, pp. E1 – e2, doi: 10.1016 / j.urology.2013.09.019 , PMID 24200197 .
  6. ↑ Y. Akama, K. Shimanuki, S. Asahi, Y. Watanabe, K. Ko, R. Takano, H. Amano, T. Kawaguchi, E. Uchida: Incidentally detected splenogonadal fusion in a laparoscopic transabdominal preperitoneal hernia repair operation: A case report. In: International journal of surgery case reports. Vol. 35, 2017, pp. 29-32, doi: 10.1016 / j.ijscr.2017.04.004 , PMID 28431326 , PMC 5397575 (free full text).
  7. ↑ AM Harris: Splenogonadal Fusion: A Rare Benign Testicular Mass in a 55-Year-Old Male. In: Urology case reports. Vol. 9, November 2016, pp. 41-42, doi: 10.1016 / j.eucr.2016.08.013 , PMID 27679760 , PMC 5037259 (free full text).
  8. ↑ DR Varma, GR Sirineni, MV Rao, KM Pottala, BV Mallipudi: Sonographic and CT features of splenogonadal fusion. In: Pediatric radiology. Vol. 37, No. 9, September 2007, pp. 916-919, doi: 10.1007 / s00247-007-0526-x , PMID 17581747 .
  9. ↑ U. Guarin, Z. Dimitrieva, SJ Ashley: Splenogonadal fusion-a rare congenital anomaly demonstrated by 99Tc-sulfur colloid imaging: case report. In: Journal of nuclear medicine: official publication, Society of Nuclear Medicine. Vol. 16, No. 10, October 1975, pp. 922-924, PMID 240914 .

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