Obstructive circular-muscle defect in the small bowel in a one-year-old child

Histological phenotypes of enteric smooth muscle disease causing functional intestinal obstruction in childhood

“…In children these disorders most commonly are congenital, but can also be acquired. Some defects appear to be primarily of morphogenesis with an absence of one of the intestinal muscle coats 3 , absence of all intestinal muscle layers 4,5 , or the presence of an additional oblique muscle coat 6 being described. In others the abnormality is at the cellular level consisting of fibrosis and myocyte vacuolation detected by light microscopy on conventionally stained histological sections [7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25] .…”

Segmental absence of intestinal musculature in newborn: Case report

“…Most neonatal cases share the following clinical features: prematurity and low birth weight, fetal compromise, cyanosis, hypoxia, respiratory distress, sepsis [ 7 ]. There are eight patients older than one year in which the clinical evolution may be more torpid [ [8] , [9] , [10] , [11] ], presented as constipation of difficult management or simulating Crohn's disease (anemia, long-standing abdominal pain, stenosis), and they improve after resection of the SAIM affected segment and no reproduce the previous symptoms again [ 10 ].…”

“…Therefore, the pathology of the lesion could be variable [ 22 ]. In some cases, the etiology is unknown, and, in others, the lesion may be acquired in utero by vascular insufficiency or ischemia [ 8 ] or after birth due to hypoxia or respiratory distress in preterm infants. In older children and adults, it can be produced secondarily in association with another adjacent pathology – there was an accidental SAIM finding in a patient with appendicitis [ 26 ].…”