Volume 4 No. 2, April 2009

Feasibility of Low Segment Hirschsprung's Disease to Minimal Surgery: Screening By Radiological Criteria

A. Ghobashi (1) & S. M. K. Shehata(2)

Departments of Radiology(1) &  Surgery(2),  Faculty of Medicine, Tanta University, Egypt
Tanta Med. Sc. J 2009; 4(2):176-185

Abstract provided by Publisher   
 

Background/Aim: Hirschsprung's disease is the most common cause of constipation in childhood. Some cases have benefited from the minor surgical intervention in the form of anorectal myectomy especially those of low segment type. Still the radiological signs present an important diagnostic tool in this disease; despite its low accuracy. Detection of transitional zone (TZ) at infra levator or supra levator positions in Hirschsprung's disease (HD) is not stressed as preoperative assessment. The aim of this work is to define radiological parameters in screening cases of low segment HD amenable to minor surgical intervention we propose this study. Patients & Methods: we studied the contrast radiological data of 164 infants and children suffering from chronic constipation to suspect low segment HD. This revision, with review of the literature resulted in five radiological signs suspect the location of TZ in low segment HD to be infra levator. Retrospectively we applied these signs to the patients' preoperative barium radiograms of 25 patients who were subjected to the myectomy and histologically proved HD. The clinical and follow up sheets of cases were revised to evaluate the accuracy of these signs in screening this group of HD amenable to myectomy. Results were expressed using the sensitivity / specificity test. Results: Twenty-one patients were responding well to myectomy, while the rest were non-responders. The five positive radiological parameters defined were: a) Transitional zone (TZ) at or below the pubococcygeal line in lateral view, b) TZ at or below the interacetabular line in postero-anterior view, c) TZ detected in early delayed films at 30 minutes post enema in one of the previous low positions, d) TZ detected in late delayed films at 24 hours in one of the previous low positions, and e) No TZ, bu^Fecfbcolonic dilatation was seen down to the anus. Radiological signs were recognized among responders with the following frequencies as 14/21 for sign a, 13/21 for sign b, 11/21 for sign c, 10/21 for sign d, and 7/21 for sign e. Sign a gives the highest positive predictive value relating the good response to myectomy. Results were evaluated using the sensitivity / specificity test. A prognostic score has been postulated ranged from 0 to 8. Score of 4 or more gives a good prognostic rating more than 70% to myectomy. Conclusions: Infra levator HD gives good response to anorectal myectomy. Our simple score proves this. These five radiological parameters form the base of screening HD amenable to minor intervention when correlated to the clinical and investigative data. A screening system is feasible and valuable to convey a level of diagnostic probability. Although our study is retrospective, we invite the clinicians to apply these radiological screening parameters to Ba enema of HD before time of biopsy. This screening leads to good results from minimal therapeutic surgical procedure and subsequently less hospital stay and less costs.

ICID 897173