Meconium ileus (MI) is the result of the accumulation of thick, dry, inspissated meconium that creates a bowel blockage, most commonly in the terminal ileum. These pockets of meconium prevent passage of stool beyond the point of obstruction, which leads to distention of the proximal bowel, bowel wall thickening, and distal microcolon. Occurring most commonly (90%) in conjunction with cystic fibrosis (CF), the occurrence of MI without CF is rare. The literature describes the incidence of MI associated with CF occurring in as many as 24.9% of those who have a pair (homozygous) of the most common CF gene mutation, Delta F508. The incidence of MI decreases with other CF mutations, of which there are over 2,000. The morbidity and mortality risks are related to whether the MI is simple or complex. Simple MI can be managed clinically with contrast enemas to relieve the obstruction and restore bowel function, while complex MI requires surgical intervention for possible bowel necrosis, perforation, peritonitis, strictures, and/or volvulus that can occur in utero or after birth. This article presents a case report of a 32-week-gestation female infant with gross abdominal distention beginning on day of life 1. The differential diagnosis, necessary testing, and required treatment that led to the final diagnosis will be presented. Additionally, radiographic modalities used to confirm the diagnosis are discussed. Finally, nursing management of the infant with simple or complex MI and short- and long-term challenges for infants and their families will be addressed.

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http://dx.doi.org/10.1891/NN-2024-0033DOI Listing

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Meconium ileus (MI) is the result of the accumulation of thick, dry, inspissated meconium that creates a bowel blockage, most commonly in the terminal ileum. These pockets of meconium prevent passage of stool beyond the point of obstruction, which leads to distention of the proximal bowel, bowel wall thickening, and distal microcolon. Occurring most commonly (90%) in conjunction with cystic fibrosis (CF), the occurrence of MI without CF is rare.

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Background: Distal intestinal obstruction syndrome (DIOS) presents significant management challenges for people with cystic fibrosis (pwC). We evaluated the treatment outcomes and identified risk factors associated with the need for surgical intervention in patients admitted with DIOS.

Method: We conducted a retrospective case series of 96 encounters of DIOS over a 20-year period, observing outcomes between cases of medical management versus those requiring for operative intervention.

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Importance: Textbooks attribute 80% of meconium-related small bowel obstructions to cystic fibrosis and 15% of colonic obstructions to Hirschsprung disease. It is unknown whether these estimates are accurate, particularly among preterm infants, whose immature bowel predisposes them to meconium-related obstruction (MRO).

Objective: To estimate the incidence of MRO by type and to assess its association with clinical outcomes.

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Sutureless Loop Enterostomy in Very Low and Extremely Low Birth Weight Infants.

Cureus

November 2024

Department of Hepato-Biliary-Pancreatic Surgery and Pediatric Surgery, Saitama Medical Center, Saitama Medical University, Kawagoe, JPN.

Introduction Sutureless enterostomy is used as an effective technique for constructing an enterostomy in very low and extremely low birth weight infants in Japan. Sutureless enterostomy is a separate type of enterostomy procedure for low birth weight infants. We adapted this technique and developed sutureless loop enterostomy (SLE), an approach without a skin bridge in which the intestinal wall is not sutured to the abdominal wall.

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As cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies including elexacaftor/tezacaftor/ivacaftor (ETI) have become widely used in eligible patients with cystic fibrosis (CF), the use of these medications in pregnant people has become a critical area of investigation. Since these medications appear generally safe to both mother and fetus when taken by pregnant people with CF, interest has pivoted to the use of ETI in CF carrier mothers to decrease morbidity and mortality from meconium ileus (MI) in fetuses with cystic fibrosis. Here we discuss three infants at our institution with ultrasound findings of MI who were exposed to prenatal ETI through CF carrier mothers for the purposes of treating MI and lowering risk of intestinal complications from this severe manifestation of CF.

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