Neonatal Intestinal Perforation Caused by Congenital Defects of the Intestinal Musculature

Litwin, Aviva; Avidor, I; Schujman, E; Grunebaum, Michael F.; Wilunsky, E; Wolloch, Y; Reisner, Salomon H.

doi:10.1093/ajcp/81.1.77

Cited by 48 publications

(32 citation statements)

References 8 publications

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“…5 As evidence for this hypothesis, previous authors had noted the same pathologic findings subsequently found in SIP of the ELBW infant. 6,7 Our ability to capture this subset of patients, who are significantly more likely to be male and seem to have perforations in transit out of the womb, reinvigorates the postulate that SIP can be acquired congenitally.…”

Section: Discussionmentioning

confidence: 97%

“…Before the improved survival of ELBW infants, SIP was thought to be a congenitally acquired disease that affects term and near term infants (old SIP). [5][6][7] These case reports described segmental absence of the muscularis in the distal intestine either as an isolated or multifocal process, with some infants having a single occurrence and others having recurrent presentations. The difference in presumed etiology between the old SIP and the new SIP has never been resolved but reports of the old SIP have declined in recent years.…”

Section: Introductionmentioning

confidence: 99%

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New insights into spontaneous intestinal perforation using a national data set: (2) two populations of patients with perforations

et al. 2006

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Background: Spontaneous intestinal perforation (SIP) is increasingly common in the premature infant and has been demonstrated to be associated with early postnatal administration of glucocorticoids and indomethacin. Before survival of the extremely low birth weight (ELBW) infant, SIP was thought to be a rare, congenitally acquired disease sporadically affecting the muscularis of the distal intestine. These disparate views of etiology have not been previously reconciled in the literature.Objectives: (1) To establish a cohort of SIP patients in a national data set. (2) To use timing of diagnosis as a unique data element and thereby differentiate between SIP cases which are susceptible to postnatal risk factors versus those occurring at or immediately after birth (and therefore not exposed to postnatal risk factors).Methods: A large deidentified national data set was used to retrospectively look at timing of diagnosis and then the cohort was divided into postnatal treatment groups for further subanalyses. This analysis resulted in the division of the cohort into early and late diagnosis SIP subcohorts. These were then queried retrospectively by univariate analysis to look for differences in demographics between the two (using a P-value < 0.05) Results: There were 633 patients with SIP evaluated in this data set. The early SIP cohort (0-3 days) was made up of 116 infants with a median BW of 1.401 kg, whereas the late cohort (4-14 days) held 386 infants with a median BW of 775 g. Infants with early SIP were significantly more likely to: be male, have higher Apgar scores, have not received antenatal steroids, surfactant or required mechanical ventilation.Conclusions: Two populations of infants acquire SIP: ELBW infants acquire SIP on average between 7 and 10 days of life after exposure to indomethacin and glucocorticoids (either endogenous or exogenous), and infants with early SIP (0-3 days) who are significantly less likely to have been exposed to postnatal risk factors and are less premature.

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Section: Discussionmentioning

confidence: 97%

Section: Introductionmentioning

confidence: 99%

New insights into spontaneous intestinal perforation using a national data set: (2) two populations of patients with perforations

et al. 2006

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“…In the literature, the pathogenesis of the muscular defect is divided into two types, congenital and acquired [3]. In the congenital type [2,4], the intact mucosa with long villi, good epithelialization, and the edematous, congested submucosal layer make it appear unlikely that the cause of the muscle defect is transmural ischemia, suggesting that the defect may be congenital. Although there were short mucosal villi and dilated vessels in our cases, short villi can regenerate after ischemic damage.…”

Section: Discussionmentioning

confidence: 99%

“…There are no prodromal GI symptoms or radiographic sings of NEC such as persistent dilated loops or pneumatosis intestinalis, or portal-vein gas. The etiologic factors that have been proposed [1] include umbilical catheters, indomethacin therapy, cytomegalovirus infection, coagulase-negative staphylococcal infection [5], and congenital defects of the intestinal musculature [4].…”

Section: Introductionmentioning

confidence: 99%

The mechanism of focal intestinal perforations in neonates with low birth weight

Tatekawa

Muraji

Imai

et al. 1999

Pediatric Surgery International

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Among 36 neonates with intestinal perforations (IP) between 1975 and 1996, 5 had necrotizing enterocolitis (NEC IP) and 10 had focal IPs (FIP). A histologic review of the bowel near the perforations was made to see if there was any difference between cases of NEC IP and FIP. In 1 case of NEC IP, a defect in the musculature was found in addition to disappearance of the mucosal villi and dilated vessels or hemorrhage in the submucosa. Thinning or absence of the intestinal musculature and short villi in the mucosa was observed in 3 cases of FIP, but the acute ischemic changes in FIP were much less than in NEC IP. Hypothesizing that the defective musculature in FIP may be acquired by a vascular accident either before or after birth, we examined the histology of the latest consecutive infants diagnosed as having meconium peritonitis (MP) due to in-utero volvulus and perforation. In the tissue near the perforation, there was an identical focus of thinning and interruption of the musculature while the acute ischemic changes were minimal. We speculate that thinning or absence of the intestinal musculature in FIP may be a result of a transient ischemic event occurring in-utero and that FIP may develop in the damaged intestine after birth when it is fully dilated.

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“…Regardless of being congenital [16] or acquired [17], defects in the intestinal musculature have been reported as a cause of FIP. From these histological findings and the fact that FIP occurs exclusively in very-low-birth-weight neonates, we speculate that immature or uncoordinated bowel movement causes a localized increase in intraluminal pressure and leads to focal perforation or bulging at the sites of defects of muscular layer.…”

Section: Histological Findings Around the Perforationmentioning

confidence: 99%

Focal intestinal perforation in extremely-low-birth-weight neonates: etiological consideration from histological findings

et al. 2007

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In Japan, mortality due to intestinal perforation has been increasing for the last 15 years, as the survival rate of extremely-low-birth-weight neonates (ELBWs) has been increasing. In our NICU, although the incidence of necrotizing enterocolitis has been decreasing, that of focal intestinal perforation (FIP) has been increasing. To elucidate the pathogenesis of FIP, a histological study was performed. For the last 20 years, in our NICU, 11 ELBWs with FIP, including one with multiple perforations and impending rupture, underwent laparotomy and enterostomy. The intestinal wall around the perforation was resected for histological study. Histological studies revealed well-developed ganglion cells in all the cases. Although some lymphocytic infiltration and hemorrhage were observed, no apparent neutrophilic infiltration, or necrotic change was identified in any case. No evidence of microcirculatory impairment was identified. The intestinal musculature discontinued abruptly with thinning in three cases, and without thinning in the others. The histology of impending rupture showed an absence of the muscularis with preservation of the remaining components of the bowel wall. The findings that the punched-out perforations had the appearance of Hirschsprung's disease, FIP exclusively occurred in ELBWs, and that neither mechanical obstruction nor necrotic change could be identified as the cause of the perforation suggest that immature bowel movement and congenital muscle defects may be involved in the pathogenesis of FIP.

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Neonatal Intestinal Perforation Caused by Congenital Defects of the Intestinal Musculature

Cited by 48 publications

References 8 publications

New insights into spontaneous intestinal perforation using a national data set: (2) two populations of patients with perforations

New insights into spontaneous intestinal perforation using a national data set: (2) two populations of patients with perforations

The mechanism of focal intestinal perforations in neonates with low birth weight

Focal intestinal perforation in extremely-low-birth-weight neonates: etiological consideration from histological findings

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