Hirschsprung's disease in low- and middle-income countries

Trinidad, Stephen; Kayima, Peter; Kotecha, Vihar; Massenga, Alicia; Rymeski, Beth; Frischer, Jason S.; Situma, Martin; Kotagal, Meera

doi:10.1016/j.sempedsurg.2022.151163

Cited by 6 publications

(19 citation statements)

References 39 publications

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“…17 In the current study, 17 (45%) patients were diagnosed at more than a year of age, indicating the delayed presentation prevalent in our set-up relative to the high detection rate at a neonatal age in developed nations. 4 Male predominance was observed among diagnosed cases, which was in line with previous reports. 6,15,16 The Sn, Sp, and accuracy of barium enema in the diagnosis of HSD in this study was 0.95 (0.81-0.99), 0.72 (0.39-0.94), and 0.84 (0.69-0.98), respectively, similar to reports from Indonesia and Taiwan.…”

Section: Discussionsupporting

confidence: 92%

“…ARM is often unavailable in LMICs. 4,6 Although rectal suction biopsy is the preferred method for confirming diagnosis, full-thickness rectal biopsy (FTRB) is performed on patients of all age groups in LMICs due to the unavailability of the former. 10 Many centers in LMICs lack the expertise and infrastructure needed for timely diagnosis of HSD patients.…”

Section: Introductionmentioning

confidence: 99%

“…Its diagnostic utility as a single agent in settings without access to pathology is also understudied. The presence of a transition zone (TZ) with proximally dilated bowel, abnormal rectosigmoid ratio (less than 1), irregular contractions of the colon, mucosal irregularities, and retention of contrast medium on the film after voiding are the major findings indicative of HSD 4 . These can have an Sn and Sp between 40% and 80% 4,8,9 .…”

Section: Introductionmentioning

confidence: 99%

“…[4][5][6] Although early diagnosis and treatment are essential to prevent complications such as enterocolitis and colonic rupture, 1,4 diagnosing or ruling out HSD presents significant challenges for physicians practicing in LMICs. 4 HSD is more common in boys than girls, albeit in familial forms where fair sex distribution is expected. 2,3,6 The cause is thought to be multifactorial, with familial cases associated with a range of genetic mutations identified to date, in addition to sporadic cases.…”

Section: Introductionmentioning

confidence: 99%

“…Hirschsprung's disease (HSD), a common cause of functional intestinal obstruction in children, is caused by congenital absence of ganglion cells in the distal bowel wall 1–4 . HSD is most commonly diagnosed in newborns with delayed passage of meconium or other signs of intestinal obstruction 1,5 .…”

Section: Introductionmentioning

confidence: 99%

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Diagnostic accuracy of barium enema versus full‐thickness rectal biopsy in children with clinically suspected Hirschsprung's disease: A comparative cross‐sectional study

Hailemariam,

Bekele,

Manyazewal

et al. 2024

Health Science Reports

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Background and AimsHirschsprung'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).MethodsWe 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.ResultsWe 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.ConclusionBE 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.

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Section: Discussionsupporting

confidence: 92%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Diagnostic accuracy of barium enema versus full‐thickness rectal biopsy in children with clinically suspected Hirschsprung's disease: A comparative cross‐sectional study

Hailemariam,

Bekele,

Manyazewal

et al. 2024

Health Science Reports

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Hirschsprung disease: a cost analysis study of the direct, indirect costs and financial coping strategies for the surgical management in Western Uganda

Binde,

Oyania,

Ullrich

et al. 2024

Pediatr Surg Int

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Background: Surgical management of Hirschsprung disease (HD) in low-and middle-income countries is typically a staged procedure, necessitating multiple hospitalizations and clinic visits increasing family nancial burden. Currently there is limited information on the costs borne by caretakers of children with Hirschsprung disease seeking surgical intervention. This study seeks to measure the costs and economic burden of surgical treatment for Hirschsprung Disease in western Uganda.Methods: A cross-sectional study using cost analysis was conducted among caretakers of patients who completed surgical treatment of HD between January 2017 to December 2021 at two hospitals in western Uganda. The average direct and indirect costs incurred by caretakers presenting at a public and private hospital were computed.Results: A total of 69 patients (M: F = 7:1) were enrolled in the study. The median age at diagnosis was 60.5 (IQR 3 -151.25) days for children and 2-staged pull through procedure was the common surgery performed. The mean overall cost for treatment was US $960 (SD = $720), with the majority of costs coming from direct medical costs. Nearly half (48%) of participants resorted to distress nancing to nance their child's surgical care. The overwhelming majority of patients (n = 64, 93%) incurred catastrophic expenditure from the total costs of surgery for HD, and 97% of participants fell below the international poverty line at the time treatment was completed.Conclusion:Despite the availability of 'free care' from government hospital and non-pro t services, this study found that surgical management of Hirschsprung disease imposed substantial cost burden on families with Hirschsprung disease patients.

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