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Review
. 2024 Jun 4;14(11):1179.
doi: 10.3390/diagnostics14111179.

Diagnosis and Management of Simple and Complicated Meconium Ileus in Cystic Fibrosis, a Systematic Review

Mădălina Andreea Donos  1   2 , Gabriela Ghiga  2 , Laura Mihaela Trandafir  2 , Elena Cojocaru  3 , Viorel Țarcă  4 , Lăcrămioara Ionela Butnariu  5 , Valentin Bernic  6 , Eugenia Moroșan  3 , Iulia Cristina Roca  7 , Dana Elena Mîndru  2 , Elena Țarcă  8
Affiliations
Review

Diagnosis and Management of Simple and Complicated Meconium Ileus in Cystic Fibrosis, a Systematic Review

Mădălina Andreea Donos et al. Diagnostics (Basel). .

Abstract

The early management of neonates with meconium ileus (MI) and cystic fibrosis (CF) is highly variable across countries and is not standardized. We conducted a systematic review according to the Preferred Reporting Items for Systematic Reviews and Meta-analyses statement. The protocol was registered in PROSPERO (CRD42024522838). Studies from three providers of academic search engines were checked for inclusion criteria, using the following search terms: meconium ileus AND cystic fibrosis OR mucoviscidosis. Regarding the patient population studied, the inclusion criteria were defined using our predefined PICOT framework: studies on neonates with simple or complicated meconium which were confirmed to have cystic fibrosis and were conservatively managed or surgically treated. Results: A total of 566 publications from the last 10 years were verified by the authors of this review to find the most recent and relevant data, and only 8 met the inclusion criteria. Prenatally diagnosed meconium pseudocysts, bowel dilation, and ascites on ultrasound are predictors of neonatal surgery and risk factor for negative 12-month clinical outcomes in MI-CF newborns. For simple MI, conservative treatment with hypertonic solutions enemas can be effective in more than 25% of cases. If repeated enemas fail to disimpact the bowels, the Bishop-Koop stoma is a safe option. No comprehensive research has been conducted so far to determine the ideal surgical protocol for complicated MI. We only found three studies that reported the types of stomas performed and another study comparing the outcomes of patients depending on the surgical management; the conclusions are contradictory especially since the number of cases analyzed in each study was small. Between 18% and 38% of patients with complicated MI will require reoperation for various complications and the mortality rate varies between 0% and 8%. Conclusion: This study reveals a lack of strong data to support management decisions, unequivocally shows that the care of infants with MI is not standardized, and suggests a great need for international collaborative studies.

Keywords: cystic fibrosis; diagnostic; management; meconium ileus; neonates.

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Conflict of interest statement

The authors declare no conflicts of interest.

Figures

Figure 1
Figure 1
PRISMA flowchart. Notes: PRISMA figure adapted from Liberati A, et al. [11], Creative Commons.
Figure 2
Figure 2
Clinical appearance of a patient with simple meconium ileus.
Figure 3
Figure 3
Abdominal X-ray showing dilated intestinal loops and a ground glass appearance; antero-posterior incidence and profile incidence.
Figure 4
Figure 4
Intraoperative appearance of a meconium ileus showing a mesenteric defect, dilated intestinal loops and a meconium inspissated distal ileum.
Figure 5
Figure 5
Intraoperative appearance of a dense, adherent meconium extracted from distal ileum.
Figure 6
Figure 6
Clinical appearance of a patient with meconium peritonitis.
Figure 7
Figure 7
Abdominal X-ray showing dilated intestinal loops in the upper part of the abdomen and opacity due to meconium cyst in the rest of the abdomen.
Figure 8
Figure 8
Intraoperative appearance of a meconium peritonitis with removal of the cystic membrane.
Figure 9
Figure 9
Intestinal stenosis with dilation of the ileon above in a patient with CF.
See this image and copyright information in PMC

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