Pediatric gastrointestinal pathology

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This article deals with pediatric gastrointestinal pathology. An introduction to pediatric pathology is in the pediatric pathology article.

An overview of (adult) gastrointestinal pathology is in the gastrointestinal pathology article.

Luminal pathology

Abetalipoproteinemia

  • AKA Bassen-Kornzweig syndrome.

General

Microscopic

Features:

  • Enterocytes have clear cytoplasm (due to lipid accumulation).

Notes:

  • Have abnormal erythrocytes with a spiculated cell membranes acanthocyte - seen on blood films.

Microvillous inclusion disease

General

  • AKA Davidson's disease.
  • Autosomal recessive inherited condition - due to mutation in MYO5B.[3]

Microscopic

Features:

  • Flat mucosa; no villi.

IHC:

  • Carcinoembryonic antigen (CEA) +ve.[4]

EM:

Notes:

  • Appearance similar to celiac sprue; however, usually lacks the intraepithelial lymphocytic infiltration characteristic of celiac sprue.

Cystic fibrosis

  • Abbreviated CF.

General

Microscopic (large bowel)

Features:[6]

  • Crypt enlargement.

Notes:

  • Not intracellular and extracellular accumulation of mucus. (?)

Aganglionosis

  • AKA Hirschsprung disease.

General

  • Congenital.
  • Fixed by surgery.

Pathology:

  • Parasympathetic ganglion cells in intramural and submucosal plexuses - not present.[7]

Notes:

  • Most common reason for litigation in paediatric pathology.[8]

Microscopic

Features:[7]

  • Ganglion cells missing in submucosal plexus and myenteric plexus.
  • +/-Submucosal fibrosis.

Stains

  • Acetylcholinesterase: abundant, disorganized, nerve fibers.
  • CD117. (???)

Images:

Meconium ileus

General

Gross

Features:

  • Thick.
    • High viscosity.
  • Green.

Image:

Microscopic

Features:

  • Meconium-laden macrophages. (???)

Meconium peritonitis

General

Microscopic

Features:

  • Brown granular material - key feature.
  • +/-Multinucleated giant cells.
  • Inflammatory infiltrate (PMNs, lymphocytes, plasma cells).

Image:

Necrotizing enterocolitis

  • Abbreviated NEC.

General

  • Disease primarily of premature babies.
  • Diagnosed by imaging.

Note:

  • Enterocolitis = inflammation of small bowel and colon.[11]
    • Necrotizing enteritis = small bowel only.

Microscopic

Features:

  • Large spaces.

Images:

Pancreatic islet cell hyperplasia

General

  • Assoc. with maternal diabetes.

Microscopic

Features:

  • Marked size variation of pancreatic islets.
    • Normal islets ~ 150 micrometers (diameter). Hyperplastic islets - up to ~500 micrometers (diameter).

Image:

Liver

Neonatal (giant cell) hepatitis

General

  • Good prognosis. (???)

Microscopic

Features:

  • Hepatocyte enlargement - "ballooning".
  • Giant cell formation.

See also

References

  1. ↑ URL: http://www.ncbi.nlm.nih.gov/omim/200100. Accessed on: 6 April 2011.
  2. ↑ Bassen FA, Kornzweig AL (April 1950). "Malformation of the erythrocytes in a case of atypical retinitis pigmentosa". Blood 5 (4): 381–87. PMID 15411425.
  3. ↑ Müller T, Hess MW, Schiefermeier N, et al. (October 2008). "MYO5B mutations cause microvillus inclusion disease and disrupt epithelial cell polarity". Nat. Genet. 40 (10): 1163–5. doi:10.1038/ng.225. PMID 18724368.
  4. ↑ Mills, Stacey E; Carter, Darryl; Greenson, Joel K; Oberman, Harold A; Reuter, Victor E (2004). Sternberg's Diagnostic Surgical Pathology (4th ed.). Lippincott Williams & Wilkins. ISBN 978-0781740517.
  5. ↑ Kennea N, Norbury R, Anderson G, Tekay A (2001). "Congenital microvillous inclusion disease presenting as antenatal bowel obstruction". Ultrasound Obstet Gynecol 17 (2): 172–4. doi:10.1046/j.1469-0705.2001.00211.x. PMID 11251929.
  6. ↑ Neutra MR, Trier JS (October 1978). "Rectal mucosa in cystic fibrosis. Morphological features before and after short term organ culture". Gastroenterology 75 (4): 701–10. PMID 710839.
  7. ↑ 7.0 7.1 URL: [[1] [2]]. Accessed on: 11 January 2011.
  8. ↑ GT. 19 January 2011.
  9. ↑ URL: http://pathology.mc.duke.edu/research/PTH225.html. Accessed on: 11 January 2011.
  10. ↑ URL: http://library.med.utah.edu/WebPath/EXAM/IMGQUIZ/pdfrm.html. Accessed on: 3 December 2011.
  11. ↑ URL: http://medical-dictionary.thefreedictionary.com/enterocolitis. Accessed on: 10 October 2011.
  12. ↑ URL: http://cueflash.com/decks/Pathology_Pediatrics. Accessed on: 11 January 2011.
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