Summary
Smooth muscle cell biopsies obtained at pyloromyotomy from 37 children with infantile hypertrophic pyloric stenosis (IHPS) were studied by light and electron microscopy and compared with 6 autopsy control cases without any clinical evidence of this disorder. In cases with IHPS an apparently irregular increase in the number of smooth muscle cells by mitosis was accompanied by an increase of the endoplasmic reticulum, proliferation of mitochondria and regressive changes, such as shrinkage, swelling, necrosis and apoptosis of smooth muscle cells. Other alterations, seen in some but not all cases consisted of large numbers of unusual dense granules some of which were clearly associated with actin filaments and, therefore, regarded as derivatives of the normally occurring dense bodies. Furthermore, intermyofibrillar and subsarcolemmal glycogen accumulations, various nuclear abnormalities and pleomorphic membranous cytoplasmic or nuclear bodies occurred. While smooth muscle cell abnormalities predominated in some cases of IHPS, in others there were more severe axonal changes in the myenteric plexus. It is suggested, therefore, that a primarily myogenic type of IHPS can be distinguished from a predominantly neurogenic type.
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Anuras S, Mitros FA, Milano A, Kuminsky R, Decanio R, Green JB (1986) A familial visceral myopathy with dilatation of the entire gastrointestinal tract. Gastroenterology 90:385–390
Anuras S, Mitros FA, Soper RT, Pringle KC, Maves BV, Younoszai MK, Franken AE Jr, Whitington P (1986) Chronic intestinal pseudoobstruction in young children. Gastroenterology 91:62–70
Barnard RJ (1987) Myopathy of detrusor smooth muscle. Br J Urol 59:595–596
Belding HH, Kernohan JW (1953) A morphologic study of the myenteric plexus and musculature of the pylorus with special reference to the changes in hypertrophic pylorus stenosis. Surg Gynecol Obstet 97:322–334
Bonsib SM, Fallon B, Mitros FA, Anuras S (1984) Urological manifestation of patients with visceral myopathy. J Urol 132:1112–1116
Campbell GR, Gibbins I, Allan I, Gannon B (1977) Effects on long-term denervation on smooth muscle of the chicken expansor secundarium. Cell Tissue Res 176:143–156
Carpenter S, Karpati G (1984) Pathology of skeletal muscle. Churchill Livingstone, New York, pp 631–639
Challa VR, Jona JZ, Markesbery WR (1977) Ultrastructural observations of the myenteric plexus of the pylorus in infantile hypertrophic pylorus stenosis. Am J Pathol 88:309–315
Cooke P (1976) A filamentous cytoskeleton in vertebrate smooth muscle fibers. J Cell Biol 68:539–556
Dahl JL, Bloom DD, Epstein ML, Fox DA, Bass P (1987) Effect of chemical ablation of myenteric neurons on transmitter levels in the rat jejunum. Gastroenterology 92:338–344
Dieler R, Schröder JM (1989) Myenteric plexus neuropathy in infantile hypertrophic pyloric stenosis. Acta Neuropathol (Berl) 78:649–661
Friesen SR, Boley JO, Miller DR (1956) The myenteric plexus of the pylorus: its early normal development and its changes in hypertrophic pyloric stenosis. Surgery 39:21–29
Gabella G (1987) Structure of muscles and nerves in the gastrointestinal tract. In: Johnson LR (ed) Physiology of the gastrointestinal tract, vol 1, 2nd edn. Raven Press, New York, pp 335–381
Ghadially FN (1988) Ultrastructural pathology of the cell and matrix, vol 2, 3rd edn. Butterworths, London, pp 954–959
Hoyes AD, Barber P (1983) Degeneration and phagocytosis of smooth muscle cells in the neonatal rat ureter. J Anat 137:583–589
Jedd MB, Melton LJ III., Griffith MR, Kaufman B, Hoffmann AD, Broughton D, O'Brien PC (1988) Factors associated with infantile hypertrophic pyloric stenosis. Am J Dis Child 142:334–337
Jona JZ (1978) Electron microscopic observation in infantile hypertrophic pyloric stenosis (IHPS). J Pediatr Surg 13:17–20
Mayer EA, Schuffler MD, Rotter JI, Hanna P, Mogard M (1986) Familial visceral neuropathy with autosomal dominant transmission. Gastroenterology 91:1528–1535
Ramstedt C (1934) Die operative Behandlung der hypertrophischen Pylorusstenose des Säuglings. Ergeb Chir 27:54–105
Schärli A, Sieber WK, Kiesewetter WB (1969) Hypertrophic pyloric stenosis at the Children's Hospital of Pittsburgh from 1912–1967. A critical review of current problems and complications. J Pediatr Surg 4:108–114
Schuffler MD, Lowe MC, Bill AH (1977) Studies of idiopathic intestinal pseudoobstruction. I. Hereditary hollow visceral myopathy: clinical and pathological studies. Gastroenterology 73:327–338
Schuffler MD, Pagon RA, Schwartz R, Bill AH (1988) Visceral myopathy of the gastrointestinal and genitourinary tracts in infants. Gastroenterology 94:892–898
Smith B (1972) Neuropathology of the alimentary tract. Edward Arnold, London
Stanghellini V, Corinaldesi R, Barbara L (1988) Pseudoobstruction syndromes. Bailliere's Clin Gastroenterol 2:225–254
Stevenson RJ (1985) Non-neonatal intestinal obstruction in children. Surg Clin Am 65:1217–1234
Tam PKH (1985) Observations and perspectives of the pathology and possible aetiology of infantile hypertrophic pylorus stenosis — a histological, biochemical, histochemical and immunocytochemical study. Ann Acad Med Singapore 14:523–529
Tam PKH (1986) An immunochemical study with neuronspecific enolase and substance P of human enteric innervation —The normal developmental pattern and abnormal deviations in Hirschsprung's disease and pyloric stenosis. J Pediatr Surg 21:227–232
Wood JD (1987) Physiology of the enteric nervous system. In: Johnson LR (ed) Physiology of the gastrointestinal tract, vol 1, 2nd edn. Raven Press, New York, pp 67–109
Wyllie AH (1987) Apoptosis: cell death in tissue regulation. J Pathol 153:313–316
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Supported in part by a grant from the Deutsche Forschungsgemeinschaft (Di 386/1-1)
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Dieler, R., Schröder, J.M., Skopnik, H. et al. Infantile hypertrophic pyloric stenosis: myopathic type. Acta Neuropathol 80, 295–306 (1990). https://doi.org/10.1007/BF00294648
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DOI: https://doi.org/10.1007/BF00294648