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Chronic intestinal pseudoobstruction in young children

  • S. Anuras
    Correspondence
    Address requests for reprints to: Sinn Anuras, M.D., Division of Gastroenterology, Department of Internal Medicine, Texas Tech University Health Sciences Center, Lubbock, Texas 79430.
    Affiliations
    Department of Internal Medicine, Texas Tech University Health Sciences Center, Lubbock, Texas USA

    Departments of Medicine, Pediatrics, Pathology, Surgery, and Radiology, University of Iowa, Iowa City, Iowa USA

    Department of Pediatrics, University of Chicago, Chicago, Illinois USA
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  • F.A. Mitros
    Affiliations
    Department of Internal Medicine, Texas Tech University Health Sciences Center, Lubbock, Texas USA

    Departments of Medicine, Pediatrics, Pathology, Surgery, and Radiology, University of Iowa, Iowa City, Iowa USA

    Department of Pediatrics, University of Chicago, Chicago, Illinois USA
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  • R.T. Soper
    Affiliations
    Department of Internal Medicine, Texas Tech University Health Sciences Center, Lubbock, Texas USA

    Departments of Medicine, Pediatrics, Pathology, Surgery, and Radiology, University of Iowa, Iowa City, Iowa USA

    Department of Pediatrics, University of Chicago, Chicago, Illinois USA
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  • K.C. Pringle
    Affiliations
    Department of Internal Medicine, Texas Tech University Health Sciences Center, Lubbock, Texas USA

    Departments of Medicine, Pediatrics, Pathology, Surgery, and Radiology, University of Iowa, Iowa City, Iowa USA

    Department of Pediatrics, University of Chicago, Chicago, Illinois USA
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  • B.V. Maves
    Affiliations
    Department of Internal Medicine, Texas Tech University Health Sciences Center, Lubbock, Texas USA

    Departments of Medicine, Pediatrics, Pathology, Surgery, and Radiology, University of Iowa, Iowa City, Iowa USA

    Department of Pediatrics, University of Chicago, Chicago, Illinois USA
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  • M.K. Younoszai
    Affiliations
    Department of Internal Medicine, Texas Tech University Health Sciences Center, Lubbock, Texas USA

    Departments of Medicine, Pediatrics, Pathology, Surgery, and Radiology, University of Iowa, Iowa City, Iowa USA

    Department of Pediatrics, University of Chicago, Chicago, Illinois USA
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  • E.A. Franken Jr.
    Affiliations
    Department of Internal Medicine, Texas Tech University Health Sciences Center, Lubbock, Texas USA

    Departments of Medicine, Pediatrics, Pathology, Surgery, and Radiology, University of Iowa, Iowa City, Iowa USA

    Department of Pediatrics, University of Chicago, Chicago, Illinois USA
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  • P. Whitington
    Affiliations
    Department of Internal Medicine, Texas Tech University Health Sciences Center, Lubbock, Texas USA

    Departments of Medicine, Pediatrics, Pathology, Surgery, and Radiology, University of Iowa, Iowa City, Iowa USA

    Department of Pediatrics, University of Chicago, Chicago, Illinois USA
    Search for articles by this author
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      Abstract

      We studied 8 young children (4 boys and 4 girls) with chronic intestinal pseudoobstruction. Intestinal pseudoobstruction, recurrent urinary tract infections, and dysuria occurred between the ages of a few weeks to 5 yr old. All had marked dilatation of the entire gastrointestinal tract distal to the esophagus, and megacystis. Conventional pathologic examinations of the full-thickness specimens of the gastrointestinal tract were normal in 5 and abnormal in 2 patients. The abnormalities included increased fibrosis and lipofuscin pigment in the smooth muscle cells. Myenteric plexus examination, using the Smith's method in 2 patients, was normal. Biopsy specimens from urinary bladders examined in 3 patients revealed separation of individual smooth muscle cells by collagen fibers. Intestinal manometric studies performed in 3 patients showed only weak and infrequent contractions during fasting and after feeding. Severe and extensive dysfunction of the gastrointestinal and urinary tracts with relatively normal histologic appearance are typical for these children.
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      References

        • Anuras S
        • Christensen J
        Recurrent or chronic intestinal pseudoobstruction.
        Clin Gastroenterol. 1981; 10: 177-190
        • Anuras S
        • Christensen J
        Primary (or idiopathic) chronic intestinal pseudoobstruction.
        in: 2nd ed. Progress in gastroenterology. Vol. IV. Grune & Stratton, New York1983: 269-281
        • Schuffler MD
        • Pope CE
        Studies of idiopathic intestinal pseudoobstruction II. Hereditary hollow visceral myopathy: family studies.
        Gastroenterology. 1977; 73: 339-344
        • Faulk DL
        • Anuras S
        • Gardner GD
        • Mitros FA
        • Summers RW
        • Christensen J
        A familial visceral myopathy.
        Ann Intern Med. 1978; 89: 600-606
        • Anuras S
        • Mitros FA
        • Nowak TV
        • et al.
        A familial visceral myopathy with external ophthalmoplegia and autosomal recessive transmission.
        Gastroenterology. 1983; 84: 346-353
        • Anuras S
        • Mitros FA
        • Milano A
        • Kuminsky R
        • Decanio R
        • Green JB
        A familial visceral myopathy with dilation of the entire gastrointestinal tract.
        Gastroenterology. 1986; 90: 385-390
        • Tanner MS
        • Smith B
        • Lloyd JK
        Functional intestinal obstruction due to deficiency of argyrophil neurones in the myenteric plexus. Familial syndrome presenting with short small bowel, malrotation, and pyloric hypertrophy.
        Arch Dis Child. 1976; 51: 837-841
        • Schuffler MD
        • Bird TD
        • Sumi SM
        • Cook A
        A familial neuronal disease presenting as intestinal pseudoobstruction.
        Gastroenterology. 1978; 75: 889-898
        • Roy AD
        • Bharucha H
        • Nevin NC
        • Odling-Smec GW
        Idiopathic intestinal pseudoobstruction: a familial visceral neuropathy.
        Clin Genet. 1980; 18: 291-297
        • Burley RS
        Painful enteromegaly of unknown etiology.
        in: 2nd ed. Proc R Soc Med. 52. 1959: 479-480
        • Paul CA
        • Tomiyasu U
        • Mellinkoff SM
        Nearly fatal pseudoobstruction of the small intestine.
        Gastroenterology. 1961; 40: 698-704
        • Naish J
        • Capper WM
        • Brown NJ
        Intestinal pseudoobstruction with steatorrhea.
        Gut. 1960; 1: 62-66
        • Dyer NH
        • Dawson AM
        • Smith BF
        • Todd IP
        Obstruction of bowel due to lesion in the myenteric plexus.
        Br Med J. 1969; 1: 686-689
        • Schuffler MD
        • Jonak Z
        Chronic idiopathic intestinal pseudoobstruction caused by a degenerative disorder of the myenteric plexus: the use of Smith's method to define the neuropathy.
        Gastroenterology. 1982; 82: 476-486
        • Byrne WJ
        • Cipel L
        • Euler AR
        • Halpin TC
        • Ament ME
        Chronic idiopathic intestinal pseudoobstruction syndrome in children—clinical characteristics and prognosis.
        J Pediatr. 1977; 90: 585-589
        • Puri P
        • Lake BD
        • Nixon HH
        Adynamic bowel syndrome: report of a case with disturbance of the cholinergic innervation.
        Gut. 1977; 18: 754-759
        • Kapila L
        • Haberkorn S
        • Nixon HH
        Chronic adynamic bowel simulating Hirschsprung's disease.
        J Pediatr Surg. 1975; 10: 885-892
        • Greenall MJ
        • Gough MH
        Chronic idiopathic intestinal pseudoobstruction in infancy and its successful treatment with parenteral feeding.
        Dis Colon Rectum. 1983; 26: 53-54
        • Bonsib SM
        • Fallon B
        • Mitros FA
        • Anuras S
        Urological manifestations of patients with visceral myopathy.
        J Urol. 1984; 132: 1112-1116
        • Mitros FA
        • Schuffler MD
        • Teja K
        • Anuras S
        Pathologic features of familial visceral myopathy.
        Hum Pathol. 1982; 13: 825-833
        • Royer P
        • Ricour C
        • Nihoul-Fekete C
        • Pellerin D
        Le syndrome familial de grele court avec malrotation intestinale et stenose hypertrophique du pylore chez le nourrison.
        Arch Fr Pediatr. 1974; 31: 223-229
        • Berdon WE
        • Baker DH
        • Blank WA
        • Gay B
        • Santulli TV
        • Donovan C
        Megacystis-microcolon-intestinal hypoperistalsis syndrome: a new cause of intestinal obstruction in the newborn. Report of radiologic findings in five newborn girls.
        AJR. 1976; 126: 957-964
        • Young LW
        • Yunis EJ
        • Girdany BR
        • Sieber WK
        Megacystismicrocolon-intestinal hypoperistalsis syndrome: additional clinical, radiologic, surgical and histopathologic aspects.
        AJR. 1981; 137: 749-755
        • Wiswell TE
        • Rawlings JS
        • Wilson JL
        • Pettett G
        Megacystis-microcolon-intestinal hypoperistalsis syndrome.
        Pediatrics. 1979; 63: 805-808
        • Amoury RA
        • Fellows RA
        • Goodwin CD
        • Hall RT
        • Holder TM
        • Ashcraft KW
        Megacystis-microcolon-intestinal hypoperistalsis syndrome: a cause of intestinal obstruction in the newborn period.
        J Pediatr Surg. 1977; 12: 1063-1065
        • Sieber WK
        • Girdany BR
        Functional intestinal obstruction in newborn infants with morphologically normal gastrointestinal tract.
        Surgery. 1963; 53: 357-361
        • Ramanathan K
        • Grossman A
        Pseudo-intestinal obstruction.
        Clin Pediatr (Phila). 1968; 7: 109-111
        • Boller M
        • Fiocchi C
        • Brown CH
        Pseudoobstruction in ceroidosis.
        Am J Roentgenol Radium Ther Nucl Med. 1976; 127: 277-279
        • Braunstein H
        Tocopherol deficiency in adults with chronic pancreatitis.
        Gastroenterology. 1961; 40: 224-231
        • Shilkin KB
        • Gracey M
        • Joski RA
        Idiopathic intestinal pseudoobstruction. Report of a case with neuropathological studies.
        Aust Paediatr J. 1978; 14: 102-106
        • Bogomoletz WV
        • Birembaut P
        • Gaillard D
        • Dupouy D
        • Camilleria JP
        • Phat VN
        Chronic idiopathic intestinal pseudoob-struction with myenteric plexus damage.
        Lancet. 1979; i: 679-680
        • Hanks JB
        • Meyers WC
        • Andersen DK
        • et al.
        Chronic primary intestinal pseudoobstruction.
        Surgery. 1981; 89: 175-182
        • Kumpuris DD
        • Brannan PG
        • Goyal RK
        Characterization of motor activity in the jejunum of normal subjects and two patients with idiopathic intestinal pseudoobstruction syndrome (IIPS) (abstr).
        Gastroenterology. 1979; 76: 1177
        • Schuffler MD
        • Baird HW
        • Fleming CR
        • et al.
        Intestinal pseudoobstruction as the presenting manifestation of small cell carcinoma of the lung.
        Ann Intern Med. 1983; 98: 129-134
        • Schuffler MD
        • Rohrmann CA
        • Templeton FE
        The radiologic manifestations of idiopathic intestinal pseudoobstruction.
        Am J Roentgenol Radium Ther Nucl Med. 1976; 127: 729-736