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Adrenal gland and paraganglia

Congenital anomalies

Adrenal hypoplasia


Reviewer: Nat Pernick, M.D. (see Reviewers page)
Revised: 6 February 2013, last major update February 2005
Copyright: (c) 2002-2013, PathologyOutlines.com, Inc.

General
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● Congenital aplasia is very rare; found in 10% with unilateral renal agenesis
● Bilateral hypoplasia is associated with anencephaly due to lack of ACTH cells; causes adrenal insufficiency
● Unilateral absence occurs in 1 per 10,000 live births
● May be associated with sudden infant death syndrome (Arch Pathol Lab Med 1977;101:168)
Infant symptoms: weight loss, vomiting, dehydration and severe electrolyte disturbances - due to adrenal insufficiency
Primary hypoplasia: X linked, associated with mutations or deletions of DAX-1 gene at Xp21; cortex is hypoplastic, but fetal zone is intact and often has cytomegalic features; associated with hypogonadotrophic hypogonadism in young men
Miniature adult type of hypoplasia: sporadic or autosomal recessive; small glands but normal architecture

Gross description
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● Small for age adrenal glands, decreased fetal zone in newborns, scattered cytomegalic cells, cells have decreased lipid

Differential diagnosis
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● Chronic exogenous glucocorticoids: cause acquired hypoplasia

End of Adrenal gland and paraganglia > Congenital anomalies > Adrenal hypoplasia


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