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Aim: The aim of the present study was to compare the diagnostic accuracy of a barium enema in Hirschsprung's disease with respect to a rectal biopsy. Materials And Methods: This study was conducted on 60 consecutive patients of clinically suspected cases of Hirschsprung's disease admitted to a tertiary care hospital in Kashmir over a period of thre...
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... positive sibling history was found in 10% of the patients in our study. All the patients in our study presented with chronic constipation, 40 patients hav- ing abdominal distension (Figure 1) as concomitant findings, 4 patients presenting with failure to thrive and 6 with vomiting. Digital rectal examination revealed rectal gripping in 52 (86.67%) patients. ...
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... A narrow rectum with a dilated proximal colon on contrast enema is diagnostic of (rectosigmoid) short-segment aganglionosis. A transition zone with a variation in lumen size can be easily demonstrated in lateral views of the enema film [11]. ...
Objectives: This article evaluates current data in literature on the definition, outcomes, and current management strategies for patients with Hirschsprung's disease (HD). Methods: Different questions about the definition, management, and outcomes of patients with Hirschsprung's disease (HD) had been created. English-language articles published until 2022 had been gathered by searching Scopus, PubMed, Google Scholar and Web of Science. Results: 61 manuscripts had been included in this review article. The Three well known operations which had been and still available for management of HD are: Soave, Swenson, and Duhamel operations. Transanal endorectal pull-through and laparoscopically assisted Swenson pull-through procedures for Hirschsprung’s disease offer a minimally invasive alternatives to traditional open abdominal operations. Conclusion: The outcomes of laparoscopic assisted trans-anal or complete transanal are slightly superior to other procedures.
Background and Aims
Hirschsprung's disease (HSD) remains a common cause of pediatric intestinal obstruction. Barium contrast enema (BE) is the primary imaging modality for the evaluation of clinically suspected cases. Here, we aimed to assess the diagnostic accuracy of BE in children with clinically suspected HSD when compared to a gold standard full‐thickness rectal biopsy (FTRB).
Methods
We recruited and consecutively enrolled children with clinically suspected HSD at two tertiary teaching hospitals. Participants underwent BE imaging and two radiologists interpreted the findings independently. Participants further underwent FTRB by pediatric surgeons as the confirmatory test. Sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and receiver operating characteristics (ROC) with the area under the curve (AUC) were calculated on Stata version 14.2, taking FTRB as the standard.
Results
We enrolled 55 cases, of which 49 completed the evaluation and were included in the final analysis. The median age was 9.4 months (interquartile range: 2–24], with a male‐to‐female ratio of 4.4:1. The sensitivity, specificity, PPV, and NPV of BE were 0.95 (95% confidence interval [CI] [0.81–0.99]), 0.73 (95% CI [0.39–0.94]), 0.92 (95% CI [0.82–0.97]), and 0.80 (95% CI [0.50–0.94]), respectively. On AUC, the diagnostic accuracy of BE compared to the confirmatory FTRB was 0.84 (95% CI [0.69–0.98]). The diagnostic accuracy was higher in neonates (ROC: 1.00) when compared to infants (ROC: 0.83) or those above 1 year of age (ROC: 0.798). HSD‐suggestive BE findings were associated with absence of ganglion cells on FTRB (χ² = 23.301, p < 0.001). Inverted rectosigmoid ratio and transition zone were more sensitive in detecting HSD of 0.92 (95% CI [0.74–0.98]) and 0.81 (95% CI [0.63–0.92]), respectively.
Conclusion
BE is sufficiently accurate in the diagnosis of children with HSD, suggesting BE would likely be used to inform surgical management in settings where confirmatory biopsy is lacking. However, clinical judgment is warranted in interpreting negative BE findings.
Hirschsprung disease adalah penyakit bawaan sejak lahir dengan tanda dan gejala utama konstipasi atau obstruksi usus, yang biasanya muncul segera setelah lahir sehingga terjadi keterlambatan pengeluaran mekonium. Hirschsprung disease terjadi karena tidak adanya sel ganglion pada kolon distal yang mengakibatkan inhibisi fungsional berupa inkordinasi gerakan peristaltik sehingga terjadi gangguan pasase usus. Pada bayi atau anak dengan hirschsprung disease biasanya akan mengalami gejala lain seperti distensi abdomen, muntah, muncul gejala enterocolitis hingga malnutrisi. Pasien dengan indeks kecurigaan yang tinggi untuk hirschsprung disease harus menjalani evaluasi diagnostik. Salah satu pemeriksaan penunjang yang valid untuk diagnostik hirschsprung disease adalah kontras enema. Kontras enema adalah pemeriksaan penunjang yang tersedia secara luas untuk hirschsprung disease. Kontras yang larut dalam air dimasukkan ke dalam usus besar menggunakan kateter yang ditempatkan melalui anus diikuti dengan pencitraan fluoroskopi langsung. Namun, prosedur kontras enema dihindari pada pasien dengan gejala enterocolitis karena risiko perforasi.
