Segmental absence of intestinal musculature: an increasingly reported pathology

• Published in: Journal of pediatric surgery Vol. 47; no. 8; pp. 1566 - 1571
• Main Authors: Davis, James S, Ryan, Mark L, Shields, John M, Sola, Juan E, Perez, Eduardo A, Neville, Holly L, Rodriguez, Maria M
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01.08.2012
• Subjects: Abdomen, Acute - etiology; Abnormalities, Multiple - epidemiology; Acute abdomen; Adult; Cesarean Section; Digestive System Abnormalities - diagnosis; Digestive System Abnormalities - epidemiology; Digestive System Abnormalities - surgery; Ductus Arteriosus, Patent - drug therapy; Ductus Arteriosus, Patent - epidemiology; Fatal Outcome; Female; Humans; Indomethacin - adverse effects; Indomethacin - therapeutic use; Infant; Infant, Newborn; Infant, Premature; Infant, Premature, Diseases - diagnosis; Infant, Premature, Diseases - epidemiology; Infant, Premature, Diseases - surgery; Intestinal perforation; Intestinal Perforation - etiology; Intestines - abnormalities; Male; Maternal Age; Muscle, Smooth - abnormalities; Newborn; Pediatrics; Pneumoperitoneum - etiology; Pregnancy; Prematurity; Retrospective Studies; Segmental absence of intestinal musculature (SAIM); Sepsis - etiology; Spontaneous intestinal perforation; Surgery; Acute abdomen; Segmental absence of intestinal musculature (SAIM); Spontaneous intestinal perforation; Newborn; Prematurity; Intestinal perforation
• ISSN: 0022-3468; 1531-5037
• DOI: 10.1016/j.jpedsurg.2012.01.002

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.