Adrenal pathology
Key points
Embryology
Medulla - derived from neural crest
Cortex - derived from intermediate mesoderm near to urogenital ridge
Adrenal rests
Cortical rests common - precursor mesoderm is taken with gonads during descent due to their proximity in the foetus
Examples of locations are spematic cord, hernia sac, any of the ovarian ligaments, peritoneum
Identified in retroperitoneal, pelvic or groin areas
They do not need to be removed
'Organ of Zuckerkandl' - ectopic medulla found anterior to aorta
Can be location of paraganglioma, phaeochromocytoma
Adrenal blood supply
Superior adrenal artery - inferior phrenic artwey
Middle artery - Aorta
Inferior artery - Renal artery
Single draining vein
Left - renal vein
Right - IVC
Addisons disease
Autoimmune destruction of adrenal cortex
Lethargy, hypovolaemic shock - Addisonian crisis
Low Na, high K+, hypoglycaemia
Management: Resuscitation, glucocorticoid and mineralocorticoid replacement
Can cause mesenteric ischaemia in children
Congenital adrenal HYPOplasia
Very rare
Autosomal recessive or X linked
Causes adrenal insufficiency and hypogonadism
Neonatal adrenal haemorrhage
R>L, bilateral in 10%
BL is antiphospholipid syndrome
Risk factors: Traumatic delivery, macrosomia, maternal hypotension
Signs: 2nd most common cause of haemoperitoneum, abdominal mass, scrotal discoloration, adrenal insufficiency and shock
Abscess: E. coil or S. Aureus, rarely Streptococcus, Bacteriodes, Echovirus, and Herpes simplex. CT scan and/or MRI. Treatment: drainage
Cushing's syndrome and disease
Syndrome: Condition caused by excess cortisol
Disease: Cushing's syndrome specifically caused by adrenocorticotropic hormone (ACTH) secreting pituitary tumour
Diagnosis:
Check 24h urine cortisol or morning serum cortisol
Check ACTH - if low - adrenal cause - if high, ectopic or pituitary cause
If high ACTH do dexamethasone suppression test - if no suppression means ectopic secretion of ACTH (e.g. Need to screen for phaeochromocytoma, medullary thyroid carcinoma, Wilms tumour, thymus, pancreatic tumours)
See adrenal tumours section
Secretory ectopic tissue should be removed - CT/MRI to identify
References
Dutt M, Wehrle CJ, Jialal I. Physiology, Adrenal Gland. [Updated 2023 May 1]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK537260/
Falco EC, Daniele L, Metovic J, Bollito E, De Rosa G, Volante M, Papotti M. Adrenal Rests in the Uro-genital Tract of an Adult Population. Endocr Pathol. 2021 Sep;32(3):375-384. doi: 10.1007/s12022-021-09685-y. Epub 2021 Jun 7. PMID: 34095993; PMCID: PMC8370964.
Di Muzio B, Hartung M, Niknejad M, et al. Organ of Zuckerkandl. Reference article, Radiopaedia.org (Accessed on 23 Jun 2024) https://doi.org/10.53347/rID-13273
Roldan-Martin MB, Rodriguez-Ogando A, Sanchez-Galindo AC, Parente-Hernandez A, Luengo-Herrero V, Sanchez-Sanchez C. Rare presentation of shock and acute mesenteric ischaemia secondary to acute adrenal insufficiency in an 11-year-old male. J Paediatr Child Health. 2013 Jun;49(6):498-500. doi: 10.1111/j.1440-1754.2012.02556.x. Epub 2012 Sep 13. PMID: 22970910.
Saleem F, Baradhi KM. Adrenal Hypoplasia. [Updated 2023 May 22]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK549779/
Toti MS, Ghirri P, Bartoli A, Caputo C, Laudani E, Masoni F, Mele L, Bernardini R. Adrenal hemorrhage in newborn: how, when and why- from case report to literature review. Ital J Pediatr. 2019 May 8;45(1):58. doi: 10.1186/s13052-019-0651-9. PMID: 31068206; PMCID: PMC6507044.
Zessis NR, Nicholas JL, Stone SI. Severe bilateral adrenal hemorrhages in a newborn complicated by persistent adrenal insufficiency. Endocrinol Diabetes Metab Case Rep. 2018 Feb 20;2018:17-0165. doi: 10.1530/EDM-17-0165. PMID: 29479447; PMCID: PMC5820741.
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