Idiopathic Intestinal Perforations in Premature Infants without Evidence of Necrotizing Enterocolitis

Abstract

In the past three years five premature very low birth weight infants (VLBW, birth weight 720-1,300 g) developed spontaneous localized perforations of the small intestine during the second week after birth. There was no evidence of intestinal obstruction or necrotizing enterocolitis (NEC). The clinical presentation and laboratory values as well as the radiologic, intraoperative and histologic findings were different from those of NEC. In four cases the initial symptoms presented as a gray-green discoloration of the flank and inguinal region with an otherwise unremarkable general condition. All patients exhibited a leukocytosis (range 14,700-19,300) and increased neutrophil count (range 9,900-14,800). Additionally, a pronounced increase in the activity of alkaline phosphatase (> 2.000 U/l in 3 cases) and a renewed increase of serum bilirubin was observed. Four of the five infants survived following laparotomy with ileostomy (n = 2) or primary anastomosis (n = 3).

Is pneumoperitoneum an absolute indication for surgery in necrotizing enterocolitis?

“…Spontaneous intestinal perforation (SIP), [24][25][26] which mimics NEC, can be distinguished from NEC by lack of systemic involvement, absence of other clinical signs common to bowel perforation, lack of pneumatosis intestinalis, its earlier onset in infants of smaller birth weight, and extreme prematurity. Focal intestinal perforation [27] seen in a few premature infants of low birth weight is similar to SIP in appearance.…”

A Case Report of a Low-Birth-Weight Infant With a Subcapsular Liver Hematoma and Spontaneous Bowel Perforation

“…33 There is speculation that the discoloration results from the presence of meconium and bile pigments in the peritoneum. 29,37,38 Abdominal distension and bilious gastric aspirates are less frequently reported [38][39][40] compared to NEC, where abdominal distension, tenderness, and erythema, bilious vomiting, gastric residuals, or bloody diarrhea are prominent features. 3,6,36 SBP may also present benignly, with a paucity of clinical signs or symptoms (Fig 4).…”

“…2,34,41 Although gram-negative sepsis may be present, there appears to be a preponderance of grampositive organisms. Disseminated intravascular coagulation (DIC), 38 thrombocytopenia, and a falling hematocrit 33 may also be associated with both SBP and NEC. 33,36,38 As is the case with clinical symptoms and radiographic findings, laboratory investigations are not reliable in confirming a diagnosis of SBP.…”

“…Disseminated intravascular coagulation (DIC), 38 thrombocytopenia, and a falling hematocrit 33 may also be associated with both SBP and NEC. 33,36,38 As is the case with clinical symptoms and radiographic findings, laboratory investigations are not reliable in confirming a diagnosis of SBP. All three parameters taken into consideration may assist in distinguishing SBP from NEC.…”

“…2,3,29,35,38 Infants with SBP are frequently bacteremic; coagulase-negative staphylococcus is typically the responsible organism, occurring in 35% to 85% of infants studied. 2,3,37 Other microorganisms include Staphylococcus aureus, Escherchia coli, Enterobacter cloacae, Klebsiella pneumoniae, and Candida.…”