Meconium plug syndrome is a transient functional abnormality of the newborn's digestive system which consists of the delayed passage of the meconium (beyond 24-48 hours), abdominal distension, vomiting, and dilatation of the left colon. The disease is diagnosed and treated by radiographic contrast enema, but Hirschsprung disease and cystic fibrosis should be excluded.
Presentation
Meconium plug syndrome, much like Hirschsprung disease, intestinal and anal atresia, anal stenosis, and intestinal malrotation, presents with progressive abdominal distension, an absence of meconium elimination, and bilious vomiting [1]. In this disease, the probable causes are intestinal hypomotility and increased consistency of fecal matter that adheres to the colonic wall and becomes immobile [2]. Abdominal inspection and palpation reveal dilated bowel loops.
The clinician should also evaluate the presence of predisposing factors of this disease, such as prematurity [3], cystic fibrosis [4], hypermagnesemia, Hirschsprung disease [5], hypotonia, hypothyroidism, and maternal diabetes. Provided that these factors are excluded, the long-term prognosis is excellent.
Entire Body System
- Neonatal Intestinal Obstruction
Abstract A mass of inspissated meconium in the distal colon or rectum is a relatively common cause of neonatal intestinal obstruction. The meconium-plug syndrome is unrelated to cystic fibrosis and meconium ileus. [ncbi.nlm.nih.gov]
Documentation: The meconium plug syndrome is a common cause of neonatal intestinal obstruction with a failure to pass meconium. It may occur by itself or it may occur in conjunction with other bowel disorders. [meducator3.net]
[…] a.k.a Meconium plug syndrome, Functional immaturity of the colon Uncommon cause of neonatal intestinal obstruction characterized by an abrupt intestinal caliber transition at or near the splenic flexure and and normal rectum. [radipedia.com]
References: Failure to pass meconium: diagnosing neonatal intestinal obstruction. Loening-Baucke V. Am Fam Physician. Nov 1999; 60(7): 2043-2050. Bilious vomiting in the newborn: rapid diagnosis of intestinal obstruction. Kimura K. Am Fam Physician. [pedclerk.bsd.uchicago.edu]
Failure to pass meconium: diagnosing neonatal intestinal obstruction. Am Fam Physician 1999;60:2043–2050. 6. Burke MS, Ragi JM, Karamanoukian HL, Kotter M, Brisseau GF, Borowitz DS, et al. New strategies in nonoperative management of meconium ileus. [synapse.koreamed.org]
- Swelling
Above the blockage, the small intestine is enlarged (dilated) resulting in abdominal swelling (distention). [merckmanuals.com]
What to call the doctor for: Call for persistent vomiting, swelling of the belly or failure to pass stool. [eapsa.org]
Symptoms include Severe abdominal pain or cramping Vomiting Bloating Loud bowel sounds Swelling of the abdomen Inability to pass gas Constipation A complete intestinal obstruction is a medical emergency. It often requires surgery. [icdlist.com]
- Surgical Procedure
As longer term outcomes—including bowel function, continence, and quality of life—are better assessed, a decidedly more optimal surgical procedure may be identified. [clinicaladvisor.com]
He has developed surgical procedures for both diseases. Address correspondence to Vera Loening-Baucke, M.D., Department of Pediatrics, University of Iowa Hospitals and Clinics, JCP 2555, 200 Hawkins Dr., Iowa City, IA 52242. [aafp.org]
Postnatal surgical procedures may also be responsible for this radiologic feature. Disease entities manifesting as microcolon include meconium ileus, small left colon syndrome, small intestinal and colonic atresia, and Hirschsprung disease. [jaocr.org]
- Asymptomatic
Previous reports have generally emphasized the normal ganglion-cell content of the colon in affected patients.Two infants are described who fulfilled all criteria for this syndrome but who were not rendered asymptomatic, as normally anticipated, by removal [ncbi.nlm.nih.gov]
The colon is normal in diameter, ruling out microcolon - Case courtesy of Dr Michael Sargent, Radiopaedia.org, rID 6009 Congenital hypothyroidism Most infants are born asymptomatic. [5] Symptoms, if present, may include, but not limited to: Macroglossia [wikidoc.org]
- Falling
Rectal distention inhibits the internal anal sphincter, resulting in a fall in anal canal pressure. FIGURE 2A. [aafp.org]
Gastrointestinal
- Vomiting
Meconium plug syndrome is a transient functional abnormality of the newborn's digestive system which consists of the delayed passage of the meconium (beyond 24-48 hours), abdominal distension, vomiting, and dilatation of the left colon. [symptoma.com]
Clinical findings Non-passage of stool in the first two days of life, vomiting, abdominal distension. Complications Volvulus, intestinal infarction. [medical-dictionary.thefreedictionary.com]
What to call the doctor for: Call for persistent vomiting, swelling of the belly or failure to pass stool. [eapsa.org]
However, newborns with meconium plug syndrome do not pass meconium within the first day or two and also have symptoms of intestinal blockage, including vomiting and abdominal swelling. [merckmanuals.com]
- Abdominal Distension
Meconium plug syndrome is a transient functional abnormality of the newborn's digestive system which consists of the delayed passage of the meconium (beyond 24-48 hours), abdominal distension, vomiting, and dilatation of the left colon. [symptoma.com]
Clinical findings Non-passage of stool in the first two days of life, vomiting, abdominal distension. Complications Volvulus, intestinal infarction. [medical-dictionary.thefreedictionary.com]
Therefore, we reviewed our experience with meconium plugs as a cause of abdominal distension to evaluate the associated conditions and incidence of Hirschsprung's disease. [ncbi.nlm.nih.gov]
Typically presents with failure to pass meconium and abdominal distension with or without vomiting. [wikidoc.org]
Intestinal obstruction with marked abdominal distension This may occur in: ileal atresia Hirschsprung's disease meconium ileus meconium plug syndrome imperforate anus. [www2.health.vic.gov.au]
- Bilious Vomiting
Keywords Cystic Fibrosis Rectal Biopsy Contrast Enema Intestinal Atresia Bilious Vomiting These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves. [link.springer.com]
Clinical and radiologic findings: (1) neonate with evidence of bowel obstruction (1a) failure to pass meconium (1b) abdominal distention (1c) bilious vomiting (2) distended colon with an obstructing filling defect (3) a diagnostic enema results in passage [meducator3.net]
In 80 per cent of cases the papilla of Vater opens into the proximal duodenum causing the vomiting to be bilious. [www2.health.vic.gov.au]
Meconium plug syndrome, much like Hirschsprung disease, intestinal and anal atresia, anal stenosis, and intestinal malrotation, presents with progressive abdominal distension, an absence of meconium elimination, and bilious vomiting. [symptoma.com]
All develop abdominal distension with bilious vomiting or nasogastric aspirates. See Also Hirschsprung [radipedia.com]
- Constipation
Symptoms include Severe abdominal pain or cramping Vomiting Bloating Loud bowel sounds Swelling of the abdomen Inability to pass gas Constipation A complete intestinal obstruction is a medical emergency. It often requires surgery. [icdlist.com]
The classic neonatal presentation is delayed passage of meconium with abdominal distention or neonatal-onset constipation. [clinicaladvisor.com]
A certain percentage of these patients also have Hirschsprung disease, so they must be followed closely to see if they develop symptoms of chronic constipation and require a rectal biopsy to rule out Hirschsprung Disease. [virtualpediatrichospital.org]
If a stool has been passed since birth but not in the last 48 hours, constipation may be the cause. In small bowel obstruction, meconium can be passed and then a decrease or no stools occur. [mhmedical.com]
Distal short segment disease can present later in life with persistent and progressive constipation. The most serious complication is enterocolitis. [www2.health.vic.gov.au]
- Nausea
For other adverse effects, Haiden et al. [ 29 ] reported a high proportion of NEC ≥ stage II (21 vs. 8%), vomiting, nausea and bradycardia in neonates in the Gastrografin group [ 29 ]. [karger.com]
Neurologic
- Irritability
Glycerine acts as a lubricant and a rectal stimulant due to the mildly irritant action of glycerol [ 14 ]. [karger.com]
Workup
The physician should order plain abdominal radiographs in supine and left lateral decubitus positions, that point out the presence of dilated intestinal loops and an absence of rectal gas. Fluid levels are also absent throughout the small and large bowel. Diagnostic protocol implies that contrast enema is administered afterward. Barium and other contrast agents have been used, but some have been hyperosmolar and caused dehydration [6]. Furthermore, some detergents and wetting additives are toxic and should not be used [7], so clinical judgment is required to determine which contrast agent is most appropriate for the patient. This method reveals a dilated area of the colon and the meconium plug, causing total or partial obstruction and presenting a double-contrast effect [8]. Ribbon-like or sausage shaped filling defects are due to the presence of meconium. The rectum has normal size if Hirschsprung disease is absent. In some cases, the left colon may be constitutionally small, so the investigation will highlight a difference between colon diameters before and after the splenic flexure. Normally, an intestinal loop should not be wider than a lumbar vertebral body. The contrast enema can be performed more than once [9]. If the problem persists after one or two enemas, that should also have a therapeutic effect and lead to prompt meconium elimination, another cause like Hirschsprung disease should be evaluated by rectal-suction biopsy. Hirschsprung disease can be mimicked on radiological films in meconium plug syndrome because the descending colon distal to the plug may be collapsed, therefore a biopsy is highly recommended in this case. Radiological evaluation can also exclude meconium ileus, in which case the appearance is that of a microcolon, but is unable to differentiate between a large bowel and small bowel obstruction (duodenal or jejunoileal atresia, malrotation, volvulus). Differential diagnosis may also be aided by anorectal manometry.
X-Ray
- Bowel Distention
Abdominal radiographs show signs of lower intestinal obstruction with variable abdominal bowel gas, bowel distention, and air-fluid levels. These radiographic findings are nonspecific; hence, barium enema must be performed. [jaocr.org]
Treatment
RESULTS: Approximately 30% of patients had spontaneous resolution of the meconium plug without any treatment. Of those patients requiring treatment, contrast barium enema was used, with 97% success. [ncbi.nlm.nih.gov]
Make informed clinical choices for each patient, from diagnosis and treatment selection through post-treatment strategies and management of complications, with new evidence-based criteria throughout. [books.google.de]
Surgical treatment of meconium ileus. Am J Surg 1970;119:99-105. 5. Noblett HR. Treatment of uncomplicated meconium ileus by Gastrografin enema: A preliminary report. J Pediatr Surg 1969;4:190-7. 6. Lennon J, Cavalera M. [tmj.ro]
Treatment (“What will be done to make my child better?”) Contrast enema: Contrast enema helps in both the diagnosis and treatment of meconium plugs. [eapsa.org]
Prognosis
Treatment and prognosis Usually, resolves with time. Initial nonsurgical management includes rectal stimulation and contrast enema 3. On a plain radiograph consider most other causes of bowel obstruction. [radiopaedia.org]
Provided that these factors are excluded, the long-term prognosis is excellent. [symptoma.com]
In certain cases, surgery may have to be performed with the goal of establishing intestinal continuity and preservation of maximal intestinal length. 9 The prognosis for infants with both simple and complicated MI is excellent with reported survival rates [jaocr.org]
Etiology
Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology. [icd10coded.com]
Etiology/Pathophysiology: Ganglion cells are present but immature and this causes a functional colonic inertia which leads to a meconium plug forming in the colon, that causes obstruction and a narrowed descending colon. [virtualpediatrichospital.org]
Duodenal atresia: The etiology of duodenal atresia is from failure to recanalize the lumen after the solid phase of intestinal development. [pedclerk.bsd.uchicago.edu]
Some authors describe 4 types of meconium peritonitis: Adhesive meconium peritonitis Meconium cyst Giant cystic meconium peritonitis Infected meconium peritonitis Bowel perforation is a common etiology in all four of the peritonitis forms. [surgery4children.com]
Prenatal and perinatal insults causing microcolon represent myriad of etiologies, which include hypoperfusion, as well as factors associated with dysmotility and stasis. [jaocr.org]
Epidemiology
Epidemiology: The underlying cause meconium plugs is largely unknown. [eapsa.org]
Epidemiology of congenital innervation defects of the distal colon. Virchows Arch A Pathol Anat Histopathol. 1992;420:171–7. 19. Smith V V. [aafp.org]
Pathophysiology
Etiology/Pathophysiology: Ganglion cells are present but immature and this causes a functional colonic inertia which leads to a meconium plug forming in the colon, that causes obstruction and a narrowed descending colon. [virtualpediatrichospital.org]
(This clinical review offers a concise and current summary of the pathophysiology of Hirshsprung’s disease and associated disorders, and it focuses on clinical evaluation, including radiographic and histopathological diagnostic tools.) Langer, JC. [clinicaladvisor.com]
A review of pathophysiology and management of fetuses and neonates with meconium ileus for the pediatric surgeon. J Pediatr Surg 2012; 47: 772-781. Juang D, Snyder CL. Neonatal bowel obstruction. Surg Clin N Am 2012; 92: 685-711. [jaocr.org]
Prevention
0% Emergent - ED Care Needed - Preventable/Avoidable - 0% Emergent - ED Care Needed - Not Preventable/Avoidable - 0% Primary diagnosis of injury 0% Primary diagnosis of mental health problems 0% Primary diagnosis of substance abuse 0% Primary diagnosis [medicbind.com]
How can Hirschsprung disease be prevented? At present, no preventive treatment is available for Hirschsprung disease. Earlier recognition of Hirschsprung disease and its complications, including enterocolitis, is associated with improved outcomes. [clinicaladvisor.com]
[…] of meconium ileus is not related to the severity of the cystic fibrosis. [11] The obstruction can be relieved in a number of different ways. [12] Meconium ileus should be distinguished from meconium plug syndrome, in which a tenacious mass of mucus prevents [en.wikipedia.org]
Al Faleh K, Anabrees J: Probiotics for prevention of necrotizing enterocolitis in preterm infants. Evid Based Child Health 2014;9:584-671. Author Contacts Mangesh Deshmukh, FRACP, MSC St. [karger.com]
Seattle Children’s is 1 of just 5 hospitals nationwide whose commitment to preventing and relieving kids’ pain has been recognized by ChildKind International. When it comes to your child’s health, experience matters. [seattlechildrens.org]
References
- Dillon PW, Cilley RE. Newborn surgical emergencies. Gastrointestinal anomalies, abdominal wall defects. Pediatr Clin North Am. 1993;40:1289–1314.
- Ellis DG, Clatworthy HW. The meconium plug syndrome revisited. J Pediatr Surg. 1966;1:54–61.
- Vinograd I, Mogle P, Peleg O, Alpan G, Lernau OZ. Meconium disease in premature infants with very low birth weight. J Pediatr. 1983;103:963–966.
- Rosenstein BJ. Cystic fibrosis presenting with the meconium plug syndrome. Am J Dis Child. 1978;132:167–169.
- Hen J, Dolan TF, Touloukian RJ. Meconium plug syndrome associated with cystic fibrosis and Hirschsprung’s disease. Pediatrics. 1980;66:466–468.
- Kim HS, Je BK, Cha SH, et al. Renal excretion of water-soluble contrast media after enema in the neonatal period. Pediatr Neonatol. 2014;55(4):256-61.
- Carroll AG, Kavanagh RG, Ni Leidhin C, et al. Comparative Effectiveness of Imaging Modalities for the Diagnosis of Intestinal Obstruction in Neonates and Infants: A Critically Appraised Topic. Acad Radiol. 2016;23 (5):559-568.
- Burge D, Drewett M. Meconium plug obstruction. Pediatr Surg International. 2004;20(2):108-110.
- Cho HH, Cheon JE, Choi YH, et al. Ultrasound-guided contrast enema for meconium obstruction in very low birth weight infants: Factors that affect treatment success. Eur J Radiol. 2015; 84 10):2024-2031.