Lattuf AL-Rudaini, M., Abd Ali, M. (2021). A Clinical Diagnostic Scoring System to Improve Early Treatment of Hirschsprung’s Disease. Alustath, 21(4), 454-460. doi: 10.52573/ipmj.2021.177069
Maha A. Lattuf AL-Rudaini; Mohammed Abdullah Abd Ali. "A Clinical Diagnostic Scoring System to Improve Early Treatment of Hirschsprung’s Disease". Alustath, 21, 4, 2021, 454-460. doi: 10.52573/ipmj.2021.177069
Lattuf AL-Rudaini, M., Abd Ali, M. (2021). 'A Clinical Diagnostic Scoring System to Improve Early Treatment of Hirschsprung’s Disease', Alustath, 21(4), pp. 454-460. doi: 10.52573/ipmj.2021.177069
Lattuf AL-Rudaini, M., Abd Ali, M. A Clinical Diagnostic Scoring System to Improve Early Treatment of Hirschsprung’s Disease. Alustath, 2021; 21(4): 454-460. doi: 10.52573/ipmj.2021.177069

A Clinical Diagnostic Scoring System to Improve Early Treatment of Hirschsprung’s Disease

Iraqi Postgraduate Medical Journal
Article 13, Volume 21, Issue 4, October 2022, Pages 454-460    PDF (197.21 K)
Document Type: Research Paper
DOI: 10.52573/ipmj.2021.177069
Authors
Maha A. Lattuf AL-Rudaini* 1; Mohammed Abdullah Abd Ali2
1Pediatric Surgery Children Welfare Teaching Hospital-Medical City, Baghdad, Iraq.
2Pediatric Surgery, Basra Specialist Hospital for Children, Basra, Iraq.
Abstract
BACKGROUND:  
Hirschsprung’s disease (HD) is a functional intestinal obstruction due to an abnormality of the enteric   nervous system, most of affected patients with HD are presented during the neonatal period, but                        the diagnosis is still non-specific and leading to missing some patients.
THE AIM OF THE STUDY:
 Is to construct a simple clinical predictor scoring system to assist early diagnosis, allow early treatment               of HD and to avoid doing rectal biopsy as much as possible.
METHODS:
A prospective cohort study was done in Children Welfare Teaching Hospital from the first of January 2016 to the end of December 2017; the study included 52 patients who were admitted to the pediatric surgery department at neonatal period with suspicion of HD. Clinical variables which include (low birth weight, prematurity, family history of HD, male gender, passage of meconium over 48 hours, passage of meconium in first stimulation, feeding intolerance, vomiting, abdominal distention, constipation, diarrhea, dependence on anal stimulation or enemas and important radiological findings) were taken for diagnosis of HD. Rectal biopsy (full thickness) or segment biopsy were done for the included patients to divide them into negative and positive (HD) groups.
According to the statistical results, a new clinical scoring system was constructed and only the variables with significant correlation with the dependent variable (histopathology) were selected.
RESULTS:
Male to female ratio among HD patients was (3.7:1). The transitional zone by contrast enema; dependence on anal stimulation and constipation show accuracy of: 92%; 90%; and 90% respectively; while passage of meconium over 48 hours, male gender and diarrhea show accuracy of: 73%; 69%; and 60% respectively. The probability of the variables in the scoring system were measured by the area under (ROC) curve on which constipation, transitional zone by contrast enema and dependence on anal stimulation achieved                    2 scores for each, while delayed passage of meconium; diarrhea and male gender achieved 1 score for each. The developed scoring system designed as 9 points, at the cut-off point (4), the score achieves sensitivity; specificity and accuracy of: 93.9%; 100%; and 96% respectively.
CONCLUSION AND RECOMMENDATION:
A developed clinical scoring system is simple and easily applied, and it is useful tool in the early diagnosis and treatment of HD by allowing a single stage pull through operation in small infants. Rectal biopsy is spared for those patients with ≤ 4 scores who are still suffering in the follow up period.
 
Keywords
Hirschsprung’s disease; scoring system
References
  1. Jay V. Legacy of Harald Hirschsprung. Pediatr Devel Pathol, 2001; 4:203–4.
  2. J. C. Langer, Hirschsprung Disease. G. W.
  3. Holcomb III, MD MBA, J. P. Murphy, MD, Shawn D. St. Peter, MD, ASHCRAFT’S PEDIATRIC SURGERY,7th edition, 2000, Elsevier Inc.,34:557-76.
  4. J.C. Langer, Hirschsprung Disease, A. G. Coran, MD, N. S. Adzick, MD, T. M. Krummel, MD, J.-M. Laberge, MD, R. C. Shamberger, MD, A. A. Caldamone, MD, Pediatric Surgery, Seventh Edition, Elsevier,2012, vol.4,101:1265-68.
  5. Smith GHH, Cass D. Infantile Hirschsprung’s disease—is barium enema useful? Pediatr Surg Int 1991; 6:318–21.
  6. Jacob C. Langer, Hirschsprung's disease. Oldham, Colombani, Foglia, Skinner, Editors; principle and practice of pediatric surgery. Philadelphia, Lippincott Williams & Wilkins, 2nd edition, 2005; 86:1347-64.
  7. N.A. LewisMarc, A.LevittGarret, S.ZallenMona S.ZafarKaren, L.IaconoJon, E.RossmanMichael, G.CatyPhilip, L.Glick. Diagnosing Hirschsprung's disease: Increasing the odds of a positive rectal biopsy result. Journal of Pediatric Surgery. Volume 38, Issue 3, March 2003: 412-416.
  8. P. L. Toledo de Arruda Lourencao MD., PhD., B. K.Takegawa MD, PhD, E.V. P. Ortolan MD, PhD, S. A.Terra MD, M. A. M. Rodrigues MD, PhD, A useful panel for the diagnosis of Hirschsprung disease in rectal biopsies: calretinin immunostaining and acetylcholinesterase histochesmistry, Annals of Diagnostic Pathology, Volume 17, Issue 4, August 2013: 352-56.
  9. Zaidoon A. Musa, Ban J. Qasim, and A. Wahab A Al Shaikly, Diagnostic role of Calretinin in Hirschsprung disease: A comparison to neuron-specific enolase. Saudi J Gastroenterology.2017; 23:60-66.
10. N. K. Alizai, G. Batcup M. F., Dixon M. D. Stringer. Rectal biopsy for Hirschsprung’s disease: What is the optimum method? Pediatr. Surg. Int., Springer-Verlag 1998; 13:121-24.

11. Cusick EL, Buick RG. Injury to the common iliac artery during suction rectal biopsy. J. Pediatr. Surg. 1995; 30:111–12.

12. Rees B.I., Azmy A., Nigam M., Complication of rectal suction biopsy. J. Pediatr. Surg. 1983; 18:273–75.

13. Barr C., Booth J., Filipe M., Clinical evaluation of the histochemical diagnosis of Hirschsprung’s disease. Gut, 1985; 26:393-99.

14. Weihong Guo, Qinming Zhang, Yongwei Chen and Dawei Hou, Diagnostic Scoring System of Hirschsprung's Disease in the Neonatal Period. Asian Journal of Surgery 2006; 29, Issue 3,176 - 79.

15. Suita S, Taguchi T, Ieiri S, Nakatsuji T. Hirschsprung's disease in Japan: analysis of 3852 patients based on a nationwide survey in 30 years. J. Pediatr. Surg., 2005; 40:197-201.

16. Camille L. Stewart, M. Kulungowski, Suhong Tong, Jacob C. Langer, J. Soden, Rectal Biopsies for Hirschsprung Disease: Patient Characteristics by Diagnosis and Attending Specialty, Journal of Pediatric Surgery, April 2016;51: 573-76.

17. Nicola A. Lewis, Marc A. Levitt, Garret S. Zallen and Philip L. Glick, Diagnosing Hirschsprung’s Disease: Increasing the Odds of a Positive Rectal Biopsy Result, Journal of Pediatric Surgery Volume 38, Issue 3, March 2003: 412-16.

18. Wu XJ, Zhang HY, Li N, Yu DH, Feng JX., A new diagnostic scoring system to differentiate Hirschsprung's disease from Hirschsprung's disease-allied disorders in patients with suspected intestinal dysganglionosis. Int J Colorectal Dis, 2013; 28:689-96.

19. A.R. Khan, G. M. Vujanic, S. Huddart  the constipated child: how likely is Hirschsprung’s disease? Pediatr Surg Int, 2003 19: 439–42.

20. Ghosh A, Griffiths DM Rectal biopsy in the investigation of constipation. Archives of Disease in Childhood 1998; 79:266-68.

 

Statistics
Article View: 29
PDF Download: 26


Journal Management System. Powered by ejournalplus.com