Evaluation of suspicious Hirschsprung disease in children using radiologic investigation method: a prospective observational study

Ramesh Kumar Sahu; Sanjay Kothari; Sk. Rafikul Rahaman; Abhishek Chattopadhyay; Sayandev Dasgupta; Shipra Sen

doi:10.18203/2349-2902.isj20171514

Authors

Ramesh Kumar Sahu Department of Radiodiagnosis, ICARE Institute of Medical Sciences and Research, Banbishnupur, Purba Medinipur, Haldia, West Bengal 721645, India
Sanjay Kothari Department of Radiodiagnosis, ICARE Institute of Medical Sciences and Research, Banbishnupur, Purba Medinipur, Haldia, West Bengal 721645, India
Sk. Rafikul Rahaman Department of Pediatrics, ICARE Institute of Medical Sciences and Research, Banbishnupur, Purba Medinipur, Haldia, West Bengal 721645, India
Abhishek Chattopadhyay Department of Orthopedics, ICARE Institute of Medical Sciences and Research, Banbishnupur, Purba Medinipur, Haldia, West Bengal 721645, India
Sayandev Dasgupta Department of General Surgery, ICARE Institute of Medical Sciences and Research, Banbishnupur, Purba Medinipur, Haldia, West Bengal 721645, India
Shipra Sen Department of Pathology, ICARE Institute of Medical Sciences and Research, Banbishnupur, Purba Medinipur, Haldia, West Bengal 721645, India

DOI:

https://doi.org/10.18203/2349-2902.isj20171514

Keywords:

Contrast enema, Diagnosis, Hirschsprung disease, Radiologic investigation, Sensitivity, Specificity

Abstract

Background: Hirschsprung disease (HD) is a developmental disorder characterized by absence of ganglia in the distal colon, resulting in a functional obstruction. It is a common cause of pediatric intestinal obstruction. Objective of present study was to evaluate a checklist of radiologic and clinical signs to determine the probability of HD in suspicious patients.

Methods: In a diagnostic accuracy study, 19 children with clinical manifestations of HD attended pediatric OPD in a tertiary care teaching hospital, Haldia from January 2014 to December 2016 were assessed. A checklist was used to evaluate the items proposed by contrast enema (CE), based on six subscales, including transitional zone, rectosigmoid index (RSI), irregular contractions in aganglionic region, cobblestone appearance, filling defect due to fecaloid materials and lack of meconium defecation during the first 48 hours after birth. The patients were classified as high score and low score. Sensitivity and specificity were calculated for identifying HD, in comparison with pathologically proved or ruled out HD.

Results: Of the 19 patients, 11 (57.89%) cases had HD and 08 (42.11%) cases were without HD. The mean age was 2.793 ± 4.21 months. Abdominal distension, lack of meconium defecation, and constipation were the most common clinical symptoms with frequencies of 15 (78.9%), 11 (57.8%), and 14 (73.68%), respectively. In summary, the mean sensitivity of detecting the radiological signs of transition zone, spastic colon, reversed recto-sigmoid index and the overall impression in histological confirmed HD patients are 59.09%, 49.99%, 59.09% and 56.06% respectively.

Conclusions: The mean specificity of detecting the absence of the radiological signs of transition zone, spastic colon, reversed recto-sigmoid index and the overall impression in histological confirmed non-HD patients are 68.75%, 81.25%, 87.5% and 79.17% respectively. This would in turn give an overall mean specificity rate of 79.17% in successfully excluding HD with the above mentioned radiological signs from the contrast enema.

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Author Biographies

Ramesh Kumar Sahu, Department of Radiodiagnosis, ICARE Institute of Medical Sciences and Research, Banbishnupur, Purba Medinipur, Haldia, West Bengal 721645, India

Radiologist

Sanjay Kothari, Department of Radiodiagnosis, ICARE Institute of Medical Sciences and Research, Banbishnupur, Purba Medinipur, Haldia, West Bengal 721645, India

Professor,

Department of Radiodiagnosis, ICARE Institute of Medical Sciences and Research, Banbishnupur, Purba Medinipur, Haldia, West Bengal 721645, India

References

Hirschsprung H. Stulträgheit Neugeborener in Folge von Diltation und Hypertrophie des Colons. Jb Kinderheilk. 1887;27:1-7.

Zuelzer WW, Wilson JL. Functional intestinal obstruction on a congenital neurogenic basis in infancy. Am J Dis Child. 1948;75(1):40-64.

Moore SW. Hirschsprung disease: current perspectives. Open Access Surgery. 2016;9:39-50.

Swenson O. Hirschsprung's disease: a review. Pediatrics. 2002;109(5):914-8.

Russell MB, Russell CA, Niebuhr E. An epidemiological study of Hirschsprung's disease and additional anomalies. Acta Paediatr. 1994;83(1):68-71.

Puri P, Montedonico S. Hirschsprung disease: Clinical features. Holschneider AM, Puri P, eds. Hirschsprung Disease and Allied Disorders. 3rd ed. New York: Springer. 2008:107-13.

Touloukian RJ, Cicchetti DV. Hirschsprung disease: accuracy of the barium enema examination. Radiology. 1984;150:393-400.

O'Donovan AN, Habra G, Somers S, Malone DE, Rees A, Winthrop AL. Diagnosis of Hirschsprung's disease. Am J Roentgenol. 1996;167:517-20.

Pratap A, Gupta DK, Tiwari A, Sinha AK, Bhatta N, Singh SN, et al. Application of a plain abdominal radiograph transition zone (PARTZ) in Hirschsprung's disease. BMC Pediatr. 2007;7:5.

Proctor ML, Traubici J, Langer JC, Gibbs DL, Ein SH, Daneman A, et al. Correlation between radiographic transition zone and level of aganglionosis in Hirschsprung's disease: Implications for surgical approach. J Pediatr Surg. 2003;38:775-8.

Qin KW, Shi H, Zhang L, Liu PF, Cai WL, Wu KH. The research on screening differentially expressed genes in Hirschsprung's disease by using Microarray. J Pediatr Surg. 2013;48(11):2281-8.

Whitehouse FR, Kernohan JW. Myenteric plexus in congenital megacolon; study of 11 cases. Arch Intern Med (Chic). 1948;82(1):75-111.

Butler Tjaden NE, Trainor PA. The developmental etiology and pathogenesis of Hirschsprung disease. Transl Res. 2013;162(1):1-15.

Alehossein M, Roohi A, Pourgholami M, Mollaeian M, Salamati P. Diagnostic accuracy of radiologic scoring system for evaluation of suspicious hirschsprungdisease in children. Iran J Radiol. 2015;12(2):e12451.

Vorobyov GI, Achkasov SI, Biryukov OM. Clinical features' diagnostics and treatment of Hirschsprung's disease in adults. Colorectal Dis. 2010;12(12):1242-8.

Ryan ET, Ecker JL, Christakis NA, Folkman J. Hirschsprung's disease: associated abnormalities and demography. J Pediatr Surg. 1992;27(1):76-81.

Pini Prato A, Rossi V, Mosconi M, Holm C, Lantieri F, Griseri P. A prospective observational study of associated anomalies in Hirschsprung's disease. Orphanet J Rare Dis. 2013;8:184.

De la Torre L, Ortega A. Transanal versus open endorectal pull-through for Hirschsprung's disease. J Pediatr Surg. 2000;35(11):1630-2.

Caniano DA, Teitelbaum DH, Qualman SJ. Management of Hirschsprung's disease in children with trisomy 21. Am J Surg. 1990;159(4):402-4.

Constipation Guideline Committee of the North American Society for Pediatric Gastroenterology H, Nutrition. Evaluation and treatment of constipation in infants and children: recommendations of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition. J Pediatr Gastroenterol Nutr. 2006;43(3):e1-13.

Kapur RP. Practical pathology and genetics of Hirschsprung's disease. Semin Pediatr Surg. 2009;18(4):212-23.

de Lorijn F, Kremer LC, Reitsma JB, Benninga MA. Diagnostic tests in Hirschsprung disease: a systematic review. J Pediatr Gastroenterol Nutr. 2006;42(5):496-505.

De Lorijn F, Reitsma JB, Voskuijl WP, Aronson DC, Ten Kate FJ, Smets AM, et al. Diagnosis of Hirschsprung's disease: a prospective, comparative accuracy study of common tests. J Pediatr. 2005;146(6):787-92.

Diamond IR, Casadiego G, Traubici J, Langer JC, Wales PW. The contrast enema for Hirschsprung disease: predictors of a false-positive result. J Pediatr Surg. 2007;42(5):792–5.

Boman F, Corsois L, Paraf F. Hirschsprung's disease: practical considerations. Ann Pathol. 2004;24(6):486-98.

Wester T, Olsson Y, Olsen L. Expression of bcl-2 in enteric neutrons in normal human bowel and Hirschsprung disease. Arch Pathol Lab Med. 1999;123(12):1264-8.

Kim HJ, Kim AY, Lee CW, Yu CS, Kim JS, Kim PN, et al. Hirschsprung disease and hypoganglionosis in adults: radiologic findings and differentiation. Radiology. 2008;247(2):428-34.

Garcia R, Arcement C, Hormaza L, Haymon ML, Ward K, Velasco C, et al. Use of the recto-sigmoid index to diagnose Hirschsprung's disease. Clin Pediatr (Phila). 2007;46(1):59-63.

Walsh MC, Kliegman RM. Necrotizing enterocolitis: treatment based on staging criteria. Pediatr Clin North Am. 1986;33:179-201.

Karnovsky MJ, Roots LA. "Direct-coloring" thiocholine method for cholinesterases. J Histochem Cytochem. 1964;12:219-21.

Rosenfield NS, Ablow RC, Markowitz RI, DiPietro M, Seashore JH, Touloukian RJ, et al. Hirschsprung disease: accuracy of the barium enema examination. Radiology. 1984;150:393-400.

Jamieson DH, Dundas SE, Belushi SA, Cooper M, Blair GK. Does the transition zone reliably delineate aganglionic bowel in Hirschsprung's disease? Pediatr Radiol. 2004;34:811-5.

Mozes YN, Rachmel A, Schonfeld T, Schwarz M, Steinberg R, Ashkenazi S. Difficulties in making the diagnosis of Hirschsprung's disease in early infancy. J Paediatr Child Health. 2004;40:716.

Skaba R. Historic milestones of Hirschsprung disease. J Pediatr Surg. 2007;42:249-51.

Tang PMY, Leung MWY, Chao NSY, Liu KKW, Fan TW. Suspected Hirschsprung's Disease in Infants: The Diagnostic Accuracy of Contrast Enema. HK J Paediatr (new series) 2016;21:74-78.

Kessmann J. Hirschsprung's disease: diagnosis and management. Am Fam Physician. 2006;74(8):1319-22.

Hebra A, Smith VA, Lesher AP. Robotic Swenson pull-through for Hirschsprung's disease in infants. Am Surg. 2011;77(7):937-41.

De la Torre-Mondragon L, Ortega-Salgado JA. Transanal en¬dorectal pull-through for Hirschsprung's disease. J Pediatr Surg. 1998;33(8):1283-6.

Shankar KR, Losty PD, Lamont GL, Turnock RR, Jones MO, Lloyd DA, et al. Transanal endorectal coloanal surgery for Hirschsprung's disease: experience in two centers. J Pediatr Surg. 2000;35(8):1209-13.

Nasir AA, Adeniran JO, Abdur- Rahman LO. Hirschsprung's disease: 8 years’ experience in a Nigerian teaching hospital. J Indian Assoc Pediatr Surg. 2007;12(6):66-9.