Abstract
Background: Segmental absence of the intestinal musculature (SAIM) is a known but clinically rare entity involving partial or complete absence of the intestinal muscularis propria. Clinical presentation is consistent with peritonitis, and treatment involves an emergent laparotomy, bowel resection, and reanastamosis or possible ostomy creation. Diagnosis results from histopathologic evaluation of the resected intestinal specimen. Most of the publications are case reports. Methods: We retrospectively reviewed all surgical pathology files at a tertiary pediatric hospital from 2003 to 2010, identifying children who were noted to have absence of intestinal musculature on pathology. Patients meeting criteria were reviewed in detail, and data regarding clinical presentation, diagnostic testing, radiologic findings, treatment, and outcome were recorded. Results: Five patients were identified between 2003 and 2010 who received the diagnosis of SAIM. Patient age ranged from 1 to 99 days of life. All children were born preterm by cesarean section, had a mean birth weight of 828 ± 338 g, and were intubated after birth. Conclusions: The 5 patients presented are reviewed and contrasted with previous cases presented in the literature. Theories of pathogenesis and classification are discussed, and the cases are labeled as primary versus secondary SAIM.
| Original language | English |
|---|---|
| Pages (from-to) | 1566-1571 |
| Number of pages | 6 |
| Journal | Journal of Pediatric Surgery |
| Volume | 47 |
| Issue number | 8 |
| DOIs | |
| State | Published - Aug 1 2012 |
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Keywords
- Acute abdomen
- Intestinal perforation
- Newborn
- Prematurity
- Segmental absence of intestinal musculature (SAIM)
- Spontaneous intestinal perforation
ASJC Scopus subject areas
- Surgery
- Pediatrics, Perinatology, and Child Health
Cite this
Segmental absence of intestinal musculature : An increasingly reported pathology. / Davis, James S.; Ryan, Mark L.; Shields, John M.; Sola, Juan E; Perez, Eduardo; Neville, Holly; Rodriguez, Maria.
In: Journal of Pediatric Surgery, Vol. 47, No. 8, 01.08.2012, p. 1566-1571.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Segmental absence of intestinal musculature
T2 - An increasingly reported pathology
AU - Davis, James S.
AU - Ryan, Mark L.
AU - Shields, John M.
AU - Sola, Juan E
AU - Perez, Eduardo
AU - Neville, Holly
AU - Rodriguez, Maria
PY - 2012/8/1
Y1 - 2012/8/1
N2 - Background: Segmental absence of the intestinal musculature (SAIM) is a known but clinically rare entity involving partial or complete absence of the intestinal muscularis propria. Clinical presentation is consistent with peritonitis, and treatment involves an emergent laparotomy, bowel resection, and reanastamosis or possible ostomy creation. Diagnosis results from histopathologic evaluation of the resected intestinal specimen. Most of the publications are case reports. Methods: We retrospectively reviewed all surgical pathology files at a tertiary pediatric hospital from 2003 to 2010, identifying children who were noted to have absence of intestinal musculature on pathology. Patients meeting criteria were reviewed in detail, and data regarding clinical presentation, diagnostic testing, radiologic findings, treatment, and outcome were recorded. Results: Five patients were identified between 2003 and 2010 who received the diagnosis of SAIM. Patient age ranged from 1 to 99 days of life. All children were born preterm by cesarean section, had a mean birth weight of 828 ± 338 g, and were intubated after birth. Conclusions: The 5 patients presented are reviewed and contrasted with previous cases presented in the literature. Theories of pathogenesis and classification are discussed, and the cases are labeled as primary versus secondary SAIM.
AB - Background: Segmental absence of the intestinal musculature (SAIM) is a known but clinically rare entity involving partial or complete absence of the intestinal muscularis propria. Clinical presentation is consistent with peritonitis, and treatment involves an emergent laparotomy, bowel resection, and reanastamosis or possible ostomy creation. Diagnosis results from histopathologic evaluation of the resected intestinal specimen. Most of the publications are case reports. Methods: We retrospectively reviewed all surgical pathology files at a tertiary pediatric hospital from 2003 to 2010, identifying children who were noted to have absence of intestinal musculature on pathology. Patients meeting criteria were reviewed in detail, and data regarding clinical presentation, diagnostic testing, radiologic findings, treatment, and outcome were recorded. Results: Five patients were identified between 2003 and 2010 who received the diagnosis of SAIM. Patient age ranged from 1 to 99 days of life. All children were born preterm by cesarean section, had a mean birth weight of 828 ± 338 g, and were intubated after birth. Conclusions: The 5 patients presented are reviewed and contrasted with previous cases presented in the literature. Theories of pathogenesis and classification are discussed, and the cases are labeled as primary versus secondary SAIM.
KW - Acute abdomen
KW - Intestinal perforation
KW - Newborn
KW - Prematurity
KW - Segmental absence of intestinal musculature (SAIM)
KW - Spontaneous intestinal perforation
UR - http://www.scopus.com/inward/record.url?scp=84865100968&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84865100968&partnerID=8YFLogxK
U2 - 10.1016/j.jpedsurg.2012.01.002
DO - 10.1016/j.jpedsurg.2012.01.002
M3 - Article
C2 - 22901918
AN - SCOPUS:84865100968
VL - 47
SP - 1566
EP - 1571
JO - Journal of Pediatric Surgery
JF - Journal of Pediatric Surgery
SN - 0022-3468
IS - 8
ER -