Should we look for Hirschsprung disease in all children with meconium plug syndrome?

Buonpane, Christie; Lautz, Timothy B.; Hu, Yue-Yung

doi:10.1016/j.jpedsurg.2019.02.036

Cited by 6 publications

(11 citation statements)

References 21 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Meconium plug syndrome was excluded, since contrast enema is also a therapeutic measure in this condition. However, in our case the symptoms persisted, regardless [ 6 ]. The diseases that might be a cause for functional constipation were either not suspected because of the course of the disease (lead poisoning, chronic intestinal pseudo-obstruction, and so on) or excluded (hypothyroidism, spinal dysraphism, intestinal neuronal dysplasia, and so on) [ 7 , 8 ].…”

Section: Timeline and Diagnostic Assessmentmentioning

confidence: 59%

Rare presentation and retrograde diagnosis of total colonic aganglionosis in a female infant: a case report

Chakhunashvili

Chakhunashvili²,

Kvirkvelia³

et al. 2023

J Med Case Reports

View full text Add to dashboard Cite

Background Total colonic aganglionosis is an extremely rare variant of Hirschsprung’s disease, which is predominant in males and can be seen in 1:50,000 live births. The presented case not only depicts a rare case, but also unusual clinical, laboratory, and instrumental data. Case presentation A 2-day-old Caucasian female newborn was transferred to our hospital from maternity. The initial presentation was reverse peristalsis, abdominal distention, and inability to pass stool. Fever had started before the patient was transferred. Hirschsprung’s disease was suspected, and tests such as contrast enema and rectal suction biopsy were done. Before enterostomy, the management of the disease included fluid resuscitation, colonic irrigation, antibiotic administration, enteral feeding, and supportive therapy. During ileostomy operation, no transition zone was visualized and full-thickness biopsy samples were retrieved from the rectum and descending colon. After surgical intervention, status significantly improved—defervescence and weight gain most importantly improved. Conclusion It is well known that diagnosis of total colonic aganglionosis may be delayed for months or even years since the transition zone may not be visible and rectal suction biopsy, unlike full-thickness biopsy, is not always reliable. It might be more prudent not to be derailed because of negative radiography and rectal suction biopsy. Also, doctors should be more suspicious of the disease if signs and symptoms are starting to be consistent with Hirschsprung-associated enterocolitis, despite biopsy and radiology results.

show abstract

Section: Timeline and Diagnostic Assessmentmentioning

confidence: 59%

Rare presentation and retrograde diagnosis of total colonic aganglionosis in a female infant: a case report

Chakhunashvili

Chakhunashvili²,

Kvirkvelia³

et al. 2023

J Med Case Reports

View full text Add to dashboard Cite

show abstract

“…One patient had a non-diagnostic rectal biopsy that was not repeated in 15 years of follow up and was diagnosised with MPS. 1 This includes the delayed biopsies. 2 One had NEC, one CMPI whilst the nal one never had a diagnosis reached HD = Hirschsprung's Disease, MPS = Meconium Plug Syndrome, SBO = Small Bowel Obstruction, NEC = necrotising enterocolitis, CMPI = Cow's milk protein intolerance.…”

Section: Resultsmentioning

confidence: 99%

“…Distal bowel obstructions (DBO) in neonates can present a diagnostic challenge due to the various pathologies that require either surgical or non-surgical treatment [1][2][3]. The different aetiologies that necessitate surgical intervention, such as Hirschsprung's disease (HD), complicated meconium ileus and small or large bowel atresia, can manifest clinically in a similar fashion to those that can be managed non-operatively like meconium plug syndrome (MPS), uncomplicated meconium ileus, and small left colon syndrome [1,3]. In neonates presenting with a DBO, a contrast enema (CE) can be used to differentiate the aetiology [3,5,6] and CE have been recognised as useful for determining the need for surgical exploration [2,7].…”

Section: Introductionmentioning

confidence: 99%

“…One study reported a seven fold increase in false negatives of CE when attempting to diagnose HD in the neonatal period [4]. Another reported between 13-38% of patients labelled as having MPS following a CE were subsequently diagnosed with HD [1], indeed when the term "meconium plug syndrome" was rst coined it was described as a mimic of HD [2,7]. We reviewed the CE performed in a single centre over the past 20 years to evaluate the diagnostic yield of various pathologies in neonates with an unclear aetiology of a DBO.…”

Section: Introductionmentioning

confidence: 99%

“…

Neonatal distal bowel obstruction (DBO) can present a diagnostic challenge [1,2] As different aetiologies have similar clinical manifestations with not all surgical intervention [2,3]. Contrast enemas (CE) can help differentiate the aetiology avoiding the need for surgery [2][3][4].

…”

mentioning

confidence: 99%

See 2 more Smart Citations

Contrast Enema for neonatal distal bowel obstruction: The diagnostic and pathological yield

-Women’s,

Taranath,

Goh

2023

Preprint

View full text Add to dashboard Cite

Purpose: Neonatal distal bowel obstruction (DBO) can present a diagnostic challenge [1, 2] As different aetiologies have similar clinical manifestations with not all surgical intervention [2, 3]. Contrast enemas (CE) can help differentiate the aetiology avoiding the need for surgery [2–4]. We reviewed 20years of CE to evaluate their diagnostic and pathological yield in neonatal DBO. Method: CE for DBO undertaken in neonate between 2001 and 2021 were reviewed with studies. Medical records for 98 patients (109 CE) were accessed to review the results, the definitive diagnosis, birth weight, gestation and age at time of CE. Results: Meconium plug syndrome (MPS) (n = 32) was the commonest radiological diagnosis followed by bowel atresia (n = 20), Hirschsprung’s disease (HD) (11) and meconium ileus (10). Of the CE 16% were normal, 14% were non diagnostic. One third of patients with a radiological diagnosis of MPS had a eventual definitive diagnosis that required surgical intervention, either HD, meconium ileus or SBO. The definitive diagnosis was most commonly MPS (n = 29), followed by HD (21) and bowel atresia (16). There were no statistically significant differences in demographics among the definitive diagnoses. Conclusion: CE in neonatal DBO has a high diagnostic and pathological yield aiding in determining which patients require surgery. Level of Evidence: Level IV

show abstract

Five culturally protected water body practices in Fiji: Current status and contemporary displacement challenges

Vave

2021

Ambio

View full text Add to dashboard Cite

Should we look for Hirschsprung disease in all children with meconium plug syndrome?

Cited by 6 publications

References 21 publications

Rare presentation and retrograde diagnosis of total colonic aganglionosis in a female infant: a case report

Rare presentation and retrograde diagnosis of total colonic aganglionosis in a female infant: a case report

Contrast Enema for neonatal distal bowel obstruction: The diagnostic and pathological yield

Five culturally protected water body practices in Fiji: Current status and contemporary displacement challenges

Contact Info

Product

Resources

About