Lymph nodes & spleen, nonlymphoma

Spleen-congenital anomalies

Splenic malformations

Last author update: 1 October 2012
Last staff update: 22 November 2020

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PubMed Search: Splenic malformations

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Cite this page: Mansouri J. Splenic malformations. website. Accessed September 21st, 2023.
  • Rare, associated with cardiac malformations (80%, usually involving atrioventricular endocardial cushion and ventricular outflow tract), situs inversus, anomalies of blood vessels, lung, abdominal viscera
  • Can present with pneumococcal sepsis (OPSI)
Hepatolienal fusion
  • Atrophy of spleen with concomitant compromised splenic function (Lancet 2011;378:86)
  • Usually acquired but rare congenital forms exist (isolated congenital hyposplenia, Ivemark syndrome, hypoparathyroidism syndrome, autoimmune polyendocrinopathy candidiasis ectodermal dystrophy [APECED] syndrome, Stormorken syndrome)
  • Multiple spleens, found as part of heterotaxy syndromes (malpositioning of organs on opposite side of body, e.g. situs inversus)
  • Associated with extrahepatic biliary atresia (Arch Pathol Lab Med 1980;104:212), cardiac abnormalities and developmental anomalies of pancreas, portal vein, inferior vena cava
Splenic gonadal fusion
  • Rare congenital anomaly in which ectopic splenic tissue unites with a gonad (< 200 cases reported)
  • Continuous: spleen connected to ectopic splenic mass by cord of splenic and fibrous tissue
  • Discontinuous: no connection between spleen and ectopic splenic mass
  • 20% of continuous types associated with other congenital defects, including peromelus (fetus with malformed limbs) and micrognathia; also testicular ectopia, inguinal hernia
  • 90% in males; usually left sided; usually less than 20 years old
  • Diagnosis: Technetium Tc 99m sulfur colloid scan
  • Treatment: Surgical excision of ectopic splenic tissue to prevent testicular atrophy, torsion or infarction and preserve fertility
Splenorenal fusion
  • May be due to splenosis after splenic trauma or splenectomy; less commonly is developmental anomaly resulting in fusion of splenic and renal tissue
  • May present as a renal mass or with symptoms of hypersplenism
Wandering spleen
  • Due to congenital loss, weakness of splenic ligaments or abnormal length of splenic vessels
  • Susceptible to torsion
  • May rarely be associated with polysplenia
Case reports
Gross description
  • Splenic gonadal fusion: ectopic splenic tissue is well demarcated from gonad, only rarely is intermingling of tissue
Microscopic (histologic) description
  • Splenic gonadal fusion:
    • Normal splenic parenchyma but may have thrombosis, calcification, fibrosis, fat degeneration, hemosiderin deposits
    • Testicular tissue may be normal but may have atrophy or fibrosis of seminiferous tubules, increased Leydig cells, thrombosis of spermatic vessels or testicular tumors
Microscopic (histologic) images

Case #309

Splenic gonadal fusion

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