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Functional ileus in neonates: Hirschsprung's disease-allied disorders versus meconium-related ileus
1Department of Pediatric Surgery, Osaka Medical Center and Research Institute for Maternal and Child Health, Japan.
Insights
Neonatal functional ileus cases were analyzed, revealing that Hirschsprung's disease-allied disorders (HAD) and meconium-related ileus (MRI) have distinct histological findings and clinical outcomes. Low birth weight is a key factor in MRI development.
Area of Science:
- Gastroenterology
- Neonatalogy
- Pathology
Background:
- Functional ileus is a significant concern in neonates.
- The myenteric plexus (MP) plays a crucial role in intestinal motility.
- Distinguishing between different causes of neonatal ileus is essential for appropriate management.
Purpose of the Study:
- To investigate the histological and clinical characteristics of neonatal functional ileus.
- To differentiate between Hirschsprung's disease-allied disorders (HAD) and meconium-related ileus (MRI).
- To explore the role of myenteric plexus (MP) immaturity in the etiology of these conditions.
Main Methods:
- Retrospective review of 68 neonates diagnosed with functional ileus.
- Histological examination of the myenteric plexus (MP) in surgically treated cases.
- Clinical data analysis including birth weight, treatment, and outcomes.
- Radiological assessment (contrast enema) for conservative cases.
Main Results:
- Seven cases (Group A) showed decreased MP ganglia, categorized as HAD, with normal birth weight and poor outcomes.
- Five cases (Group B) had normal MP histology, categorized as MRI, with low birth weight and mixed outcomes.
- Fifty-six cases (Group C) resolved with conservative therapy, categorized as MRI, predominantly in low birth weight neonates.
- Macroscopic findings in HAD and MRI were similar, but histological MP abnormalities correlated with clinical outcomes.
Conclusions:
- Neonatal functional ileus can be classified into HAD and MRI based on MP histology and clinical presentation.
- Functional immaturity of the MP is strongly suggested as a contributing factor in MRI, particularly in low birth weight neonates.
- Early identification and differentiation of HAD and MRI are critical for improving neonatal outcomes.
Abstract:
Sixty-eight neonates with functional ileus were reviewed. Twelve required laparotomy; in seven, histological studies revealed decreased ganglia and ganglion cells of the myenteric plexus (MP) (Group A), and in five, MP was normal (Group B). In the remaining 56 cases, obstructive symptoms were relieved following conservative therapy (Group C). All Group A cases except one had normal birth weight, while Group B and C cases showed significantly lower birth weights. A marked caliber change of the small intestine and/or small-caliber distal intestine with meconium stagnation in the proximal intestine was commonly demonstrated at operation in Group A and B, or on contrast enema in Group C. Four Group A cases died of enteritis, and three survivors suffered from prolonged obstructive symptoms. The grade of histological abnormality of MP correlated with the clinical outcome. In Group B, three died of sepsis shortly after surgery, but two survivors have been free from symptoms. Group A can be categorized as Hirschsprung's disease-allied disorders (HAD). Group B and C can be categorized as meconium-related ileus (MRI). The similarity of the macroscopic findings of HAD and MRI, and the occurrence of MRI exclusively in low birth weight neonates, strongly suggest that functional immaturity of MP plays a role in the etiology of MRI.