Intestinal obstruction secondary to adhesions in an infant with cystic fibrosis

Mohammed Jawad1, Gillian Klafkowski, Warren Lenney

  • 1Academic Department of Child Health, University Hospital of North Staffordshire, Stoke on Trent, UK.

BMJ Case Reports
|July 31, 2013
PubMed

Insights

Cystic fibrosis (CF) patients can develop intestinal obstruction from surgical causes like adhesions, not just distal intestinal obstruction syndrome (DIOS). Adhesions can form even without prior abdominal surgery, highlighting the need for thorough evaluation.

Area of Science:

  • Pediatric Surgery
  • Gastroenterology
  • Cystic Fibrosis Research

Background:

  • Intestinal obstruction is a significant complication in pediatric patients with cystic fibrosis (CF).
  • Distal intestinal obstruction syndrome (DIOS) is the most frequent cause of bowel obstruction in CF patients.
  • Conservative management is typically the first line of treatment for DIOS.

Observation:

  • A 9-week-old infant with CF presented with intestinal obstruction, initially diagnosed as DIOS.
  • Conservative treatment with laxatives and gastrograffin failed, necessitating exploratory laparotomy.
  • Extensive intra-abdominal adhesions were found to be the cause of obstruction, despite no prior surgical history.

Findings:

  • The patient had a history of conservative management for possible necrotizing enterocolitis in the neonatal period.
  • A hypothesis suggests a subclinical bowel perforation during the neonatal period led to adhesion formation.
  • Intra-abdominal adhesions causing obstruction can occur in CF patients without a history of abdominal surgery.

Implications:

  • This case underscores the importance of considering surgical etiologies for intestinal obstruction in CF patients beyond DIOS.
  • It highlights that intra-abdominal adhesions, a surgical cause, can develop in the absence of previous surgical interventions.
  • Early consideration of surgical causes may prevent delayed diagnosis and treatment in pediatric CF patients with bowel obstruction.

Related Concept Videos

Intestinal Obstruction II: Pathophysiology01:07

Intestinal Obstruction II: Pathophysiology

Intestinal obstruction triggers a series of physiological responses, starting with gas and fluid accumulation in the bowel segment proximal to the obstruction, leading to distension. This distended intestine compresses the diaphragm, hindering lung expansion and potentially leading to reduced respiratory effort, atelectasis, and pneumonia.To overcome the blockage, the gut intensifies contractions, causing colicky abdominal pain, nausea, and vomiting, which reduces fluid and food intake and...
Intestinal Obstruction I: Introduction01:29

Intestinal Obstruction I: Introduction

Intestinal obstruction is a partial or complete blockage of the small or large intestine that disrupts the normal flow of intestinal contents through the lumen. This interruption impairs digestion, absorption, and fluid balance, and may lead to serious complications if not treated promptly.Mechanical ObstructionMechanical obstruction occurs when a physical blockage prevents intestinal contents from passing, arising from within the lumen or the bowel wall, or from external compression.Adhesions,...
Cystic Fibrosis: Pathogenesis01:23

Cystic Fibrosis: Pathogenesis

Cystic fibrosis (CF), an autosomal recessive disorder, significantly affects the function of exocrine glands. This genetically inherited disease is characterized by the production of thick and sticky mucus, which can severely affect various organs and systems in the body.
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation, but...
Inflammatory Bowel Disease V: Surgical Management01:21

Inflammatory Bowel Disease V: Surgical Management

Surgical interventions for inflammatory bowel disease (IBD), which includes ulcerative colitis and Crohn's disease, are essential in managing symptoms and addressing complications. The selection of surgical procedures is contingent upon the specific conditions and complications that stem from these illnesses.
Here are some common surgical interventions for IBD:
Pyloric Obstruction01:11

Pyloric Obstruction

Pyloric obstruction, also referred to as gastric outlet obstruction, is a condition characterized by narrowing or blockage at the pylorus—the muscular valve regulating the flow of stomach contents into the duodenum. When this passage becomes impaired, the stomach cannot effectively empty its contents into the small intestine. This disruption leads to a range of gastrointestinal symptoms, including early satiety, bloating, epigastric pain, postprandial nausea, persistent vomiting, and...
Esophageal Strictures-I: Introduction01:30

Esophageal Strictures-I: Introduction

Esophageal strictures involve abnormal narrowing or tightening of the esophagus. They vary in length and severity, ranging from mild constriction to complete obstruction, and are classified as benign (noncancerous) or malignant (cancerous).
Etiology
The primary cause of esophageal strictures is long-standing gastroesophageal reflux disease (GERD), accounting for about 70 to 80% of adult cases. Chronic acid reflux can lead to injury and scarring of the esophageal lining, culminating in...