Pediatric Surgery International

, Volume 24, Issue 11, pp 1191–1195 | Cite as

Deficient α-smooth muscle actin as a cause of functional intestinal obstruction in childhood

  • Anne-Marie O’ Donnell
  • Takashi Doi
  • Michael Hollwarth
  • Piotr Kalicinski
  • Piotr Czauderna
  • Prem PuriEmail author
Original Article



Most functional intestinal obstruction in childhood is due to defects in the enteric innervation. Functional intestinal obstruction due to smooth muscle disorders is rare. The aim of this study was to describe four patients with functional intestinal obstruction where the only histopathological abnormality was deficient, α-smooth muscle actin (α-SMA) in the circular muscle of their small bowel.

Materials and methods

Resected intestinal segments from four children with functional intestinal obstruction were either snap frozen or embedded in paraffin wax, and transversely sectioned. Sections were examined by routine H&E staining, AChE and NADPH-diaphorase histochemistry, and immunohistochemistry using PGP9.5 and α-SMA antibodies. Normal age-matched tissue was used as controls.


All four patients demonstrated normal innervation in the small and large bowel. We observed striking differences in the expression of α-SMA within the intestinal muscle layers in all four patients. There was a markedly decreased level of α-SMA in the smooth muscle compared to the longitudinal muscle in the small bowel in each patient. All other regions of the intestine examined showed normal levels of α-SMA, with similar levels expressed in both the circular and longitudinal muscles.


The decreased level of α-SMA in the smooth muscle of these patients causes a serious mechanical defect, thus interfering with normal intestine motility. These findings suggest that patients with functional intestinal obstruction should have a comprehensive investigation of the intestinal smooth muscle in addition to the assessment of the enteric nervous system.


Smooth muscle actin Functional intestinal obstruction Enteric nervous system 


  1. 1.
    Smith V, Milla PJ (1997) Histological phenotypes of enteric smooth muscle disease causing functional intestinal obstruction in childhood. Histopathology 31:112–122PubMedCrossRefGoogle Scholar
  2. 2.
    Milla PJ (1994) Clinical features of intestinal pseudo-obstruction in children. In: Kamm M, Lennard-Jones J (eds) Constipation. Wrightson, Petersfield, pp 251–258Google Scholar
  3. 3.
    Dudley H, Sinclair I, Mc Laren I (1958) Intestinal pseudo-obstruction. J R Coll Surg Edinb 3:206–217PubMedGoogle Scholar
  4. 4.
    Mann S, Debinski H, Kamm M (1997) Clinical characteristics of chronic idiopathic intestinal pseudo-obstruction in adults. Gut 41:675–681PubMedCrossRefGoogle Scholar
  5. 5.
    Krishnamurthy S, Schuffler M (1987) Pathology of neuromuscular disorders of the small intestine and colon. Gastroenterology 93:610–639PubMedGoogle Scholar
  6. 6.
    Stromer M (1995) Immunohistochemistry of the muscle cell cytoskeleton. Microsc Res Tech 31:95–105PubMedCrossRefGoogle Scholar
  7. 7.
    Fatigati V, Murphy R (1984) Actin and tropomyosin variants in smooth muscles. Dependence on tissue type. J Biol Chem 259:14383–14388PubMedGoogle Scholar
  8. 8.
    Smith V, Lake B, Kamm M et al (1992) Intestinal pseudo-obstruction with deficient smooth muscle alpha-actin. Histopathology 21:535–542PubMedCrossRefGoogle Scholar
  9. 9.
    Knowles C, Silk D, Darzi A et al (2004) Deranged smooth muscle alpha-actin as a biomarker of intestinal pseudo-obstruction: a controlled multinational case series. Gut 53:1583–1589PubMedCrossRefGoogle Scholar
  10. 10.
    Moore S, Schneider J, Kaschula R (2002) Unusual variations of gastrointestinal smooth muscle abnormalities associated with chronic intestinal pseudo-obstruction. Pediatr Surg Int 18:13–20PubMedCrossRefGoogle Scholar
  11. 11.
    Karnovsky M, Roots L (1965) A ‘direct-coloring’ thiocholine method for cholinesterase. J Histochem Cytochem 12:219–221Google Scholar
  12. 12.
    Hartshorne D (1987) Biochemistry of the contractile process in smooth muscle. In: Johnson L (ed) Physiology of the gastrointestinal tract, vol. 2nd edn, Raven Press, New York pp 423–482Google Scholar
  13. 13.
    Wedel T, Van Eys G, Waltregny D et al (2006) Novel smooth muscle markers reveal abnormalities of the intestinal musculature in severe colorectal motility disorders. Neurogastroenterol Motil 18:526–538PubMedCrossRefGoogle Scholar
  14. 14.
    Rolle U, Puri P (2006) Structural basis of voiding dysfunction in megacystis microcolon intestinal hypoperistalsis syndrome. J Pediatr Urol 2:277–284CrossRefPubMedGoogle Scholar
  15. 15.
    Fromont-Hankard G, Lafer D, Masood S (1996) Altered expression of alpha-smooth muscle isoactin in Hirschsprung’s disease. Arch Pathol Lab Med 120:270–274PubMedGoogle Scholar

Copyright information

© Springer-Verlag 2008

Authors and Affiliations

  • Anne-Marie O’ Donnell
    • 1
  • Takashi Doi
    • 1
  • Michael Hollwarth
    • 2
  • Piotr Kalicinski
    • 3
  • Piotr Czauderna
    • 4
  • Prem Puri
    • 1
    Email author
  1. 1.Children’s Research CentreOur Lady’s Children’s HospitalDublinIreland
  2. 2.Department of Pediatric SurgeryUniversity of Graz Medical SchoolGrazAustria
  3. 3.Department of Paediatric SurgeryThe Children’s Memorial Health InstituteWarsawPoland
  4. 4.Department of Pediatric SurgeryMedical School of GdańskGdańskPoland

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