Architectural Malformation of the Muscularis Propria as a Cause for Intestinal Pseudo-Obstruction: Two Cases and a Review of the Literature

被引:10
作者
Kapur, Raj P. [1 ,2 ]
Correa, Hernan [3 ]
机构
[1] Childrens Hosp & Reg Med Ctr, Dept Labs, Seattle, WA USA
[2] Univ Washington, Sch Med, Dept Pathol, Seattle, WA 98195 USA
[3] Vanderbilt Univ, Dept Pathol, Nashville, TN USA
关键词
abnormal layering; Fryns syndrome; intestinal pseudo-obstruction; muscularis propria; segmental megacolon; visceral myopathy; HOLLOW VISCERAL MYOPATHY; SMOOTH-MUSCLE; SONIC HEDGEHOG; FRYNS-SYNDROME; CHRONIC CONSTIPATION; PATHOLOGY; FIBROSIS; DISEASE; COLON; GUT;
D O I
10.2350/08-07-0495.1
中图分类号
R36 [病理学];
学科分类号
100104 ;
摘要
Two patients are presented with intestinal pseudo-obstruction and malformations of their intestinal muscularis propria. Histopathological findings in each case indicate a distinct alteration of intestinal muscle architecture. The 1st patient, a teenager with lifelong constipation, had an additional circular muscle tunic in 2 dilated segments of his large intestine. The 2nd patient, an infant with multiple malformations, had diffuse abnormal layering of his small intestine. The rare reports of other individuals with similar pathological findings are discussed.
引用
收藏
页码:156 / 164
页数:9
相关论文
共 32 条
[1]   Fryns syndrome with Hirschsprung disease: Support for possible neural crest involvement [J].
Alkuraya, FS ;
Lin, AE ;
Irons, MB ;
Kimonis, VE .
AMERICAN JOURNAL OF MEDICAL GENETICS PART A, 2005, 132A (02) :226-230
[2]   Advances in our understanding of the pathology of chronic intestinal pseudo-obstruction [J].
De Giorgio, R ;
Sarnelli, G ;
Corinaldesi, R ;
Stanghellini, V .
GUT, 2004, 53 (11) :1549-1552
[3]   FAMILIAL VISCERAL MYOPATHY - EVIDENCE OF DIFFUSE INVOLVEMENT OF INTESTINAL SMOOTH-MUSCLE [J].
FITZGIBBONS, PL ;
CHANDRASOMA, PT .
AMERICAN JOURNAL OF SURGICAL PATHOLOGY, 1987, 11 (11) :846-854
[4]   Embryonic development of the ganglion plexuses and the concentric layer structure of human gut: a topographical study [J].
Fu, M ;
Tam, PKH ;
Sham, MH ;
Lui, VCH .
ANATOMY AND EMBRYOLOGY, 2004, 208 (01) :33-41
[5]   Chronic intestinal pseudo-obstruction syndrome in pediatric patients [J].
Goulet, O ;
Jobert-Giraud, A ;
Michel, JL ;
Jaubert, F ;
Lortat-Jacob, S ;
Colomb, V ;
Cuenod-Jabri, B ;
Jan, D ;
Brousse, N ;
Nihoul-Fékéte, C ;
Ricour, C .
EUROPEAN JOURNAL OF PEDIATRIC SURGERY, 1999, 9 (02) :83-90
[6]   Four cases of desmosis coli:: severe chronic constipation, massive dilatation of the colon, and hypoperistalsis due to of changes in the colonic connective-tissue net [J].
Hübner, U ;
Meier-Ruge, W ;
Halsband, H .
PEDIATRIC SURGERY INTERNATIONAL, 2002, 18 (2-3) :198-203
[7]  
ISAACSON C, 1985, S AFR MED J, V67, P1015
[8]  
JACOBS E, 1979, GASTROENTEROLOGY, V77, P745
[9]   Cleft-palate lateral synechia syndrome: Insight into the phenotypic spectrum of Fryns syndrome? [J].
Jaeger, A ;
Kapur, R ;
Whelan, M ;
Leung, E ;
Cunningham, M .
BIRTH DEFECTS RESEARCH PART A-CLINICAL AND MOLECULAR TERATOLOGY, 2003, 67 (06) :460-466
[10]   PATHOLOGY OF NEUROMUSCULAR DISORDERS OF THE SMALL-INTESTINE AND COLON [J].
KRISHNAMURTHY, S ;
SCHUFFLER, MD .
GASTROENTEROLOGY, 1987, 93 (03) :610-639