Meconium Plug Syndrome (MPS)
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Key Takeaways
- Meconium plug syndrome (MPS) is distinct from meconium ileus and presents as transient large bowel obstruction relieved by plug passage.
- 10-15% of newborns with MPS are ultimately diagnosed with Hirschsprung disease across multiple studies spanning decades.
- Rectal biopsy practice varies: some centers biopsy all MPS cases, others selectively biopsy only those with abnormal stooling patterns.
- No association found between MPS and cystic fibrosis in recent studies, contrary to some early literature.
- Clinical controversy persists on whether 10-15% Hirschsprung incidence justifies universal biopsy versus selective observation approach.
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Okay. So my next topic, I've got uh just a few slides uh. And just to preface this, the reason uh that so both of these presentations that you're presenting with are presentations in which your data disagrees with someone else's data. Would you agree with that statement? Uh for the first one. I'm not sure it disagrees for the second one. All right. We'll see. At least by my reading of the literature. Okay, go ahead. It's Todd. Go ahead. So it's about meconium plug syndrome. Uh and I think it's important to realize this is not meconium ileus. So we're not talking about meconium ileus, we're talking about meconium plug syndrome. And this is the newborn presenting with a transient large bowel obstruction uh that is relieved by the passage of meconium plug, sometimes with a contrast enema, sometimes with uh some rectal stimulation. Uh but it's not meconium ileus. And I did not actually know this Todd, but this uh syndrome was first described by uh William Clatworthy. Uh at Ohio State. Wow. I did not know this in 1956. and so I've shown the the title uh page of his um uh report. Uh and they described nine uh children. Uh one of the nine children uh uh developed uh or was found to have Hirschsprung's disease. Um so our paper was um 2008 and this was a retrospective study uh and we looked uh over a 13-year period uh at newborns who were documented to have meconium plug syndrome from 1994 to 2007 and there were 77 babies. And 10 of those babies were subsequently found to have Hirschsprung's disease. Uh which was the uh about the same as the Clatworthy paper. No baby had cystic fibrosis. Uh a lot of early literature seemed to suggest uh well not necessarily a lot, but some early literature seemed to suggest an association with cystic fibrosis and meconium plug syndrome. And our conclusion with uh was that babies with meconium plug syndrome and abnormal stooling pattern should undergo a rectal biopsy. A recent paper uh that uh just came out, um was from um uh Dr. Lautz uh and colleagues at Northwestern. Uh and this uh came out this year in the Journal of Pediatric Surgery and it was a a fist database uh study. So fist is the Public Health uh information system and that's really a database registry for hospitals participating in the Children's Hospital uh Association uh in the United States and there're about 50 hospitals within the Children's Hospital Association. So this is a a fist database study uh from 50 children's hospitals in the US. It was over a two-year period uh and they found 373 newborns. Um 43 of them had uh Hirschsprung's disease on an early rectal biopsy uh and um a total of 57 or 15% were ultimately found to have Hirschsprung's disease. And so at least from my reading, Todd, the in these three papers, the incidence of Hirschsprung's disease is somewhere between 11 and 15%. So that's why I'm not sure I I think that there's a lot of difference in these um these papers. Okay. So that's because we had okay. So we're all in agreement then. Do you have any comments that were that were. There's some limitations in this paper and I mean the retrospective database study and that they didn't have uh a good code to identify the rectal biopsies. So they used a whole bunch of different uh CPT codes and procedures and diagnostic codes that may make it a little murky for analysis, but Okay. Yeah, I think that uh database studies in general suffer from a lot of the, you know, same problems and this was uh no exception. But it but it comes out to a similar relatively similar um incidence of Hirschsprung's disease. So uh Mack, do you biopsy all of your meconium plugs? I was about to ask you a question. Okay, go ahead. Is the controversy that a 10 to 15% incidence means warrants that all these kids get a a biopsy, right? That's that's the Yeah how do we interpret the data? Yeah, right. So so what do you do? I've done both. Okay. Wit, what do you do? So we uh generally if there's if there's abnormal stooling pattern, then we'll do the biopsy, but we do not biopsy every, there're not that many, but every patient with every baby with meconium plug syndrome. Did any of the babies? the question is that 10 to 15% that you don't biopsy that come back to find out has it ever, you know, have they gotten worsening enterocolitis because you wait, you know, in other words, can you afford to wait and see? Or is it are those patients getting sick and it's worth doing it and even though 85% of them are going to come back negative. Liz, what do you do? Uh, we biopsy. You biopsy all of them. Yes sir. Yes. Okay. Uh, out in the audience here, do you? The question is do you do you routinely biopsy babies that have meconium plug. Van Alman says yes. Wilken says no. Selective. Okay, Mirror is agreeing with Dan. Selective. Okay. Selective based on how they're doing clinically. Yeah. Okay. Okay. All right.