Diagnostic criteria and surgical indications of allied diseases of Hirschsprungs disease in children

Objective To determine the diagnostic criteria and surgical indications of allied diseases of Hirschsprung's disease (HAD) in children. Methods During the period from Jun. 2007 to Dec. 2009,65 consecutive patients were performed "radical surgery for megacolone" by this surgery team. Based on the clinical symptoms,anorectal manometry, AChE staining of rectal mucosa and barium enema,the preoperative suspected diagnosis of Hirschsprung's disease (HD) or HAD was made.Surgical indications were determined once the suspected HD diagnose was made. On the patients withsuspected HAD, strictly conservative treatments should be carried out for at least 3 months. If conservative treatments were ineffective or the symptoms were recurrent, surgical indication of HAD patients could be determined. Results The preoperative diagnosis was 38 patients with HD and 27 with HAD.However, the final pathologic diagnosis was 36 with HD and 29 with HAD. The misjudged preoperative diagnosis was made in 10. 5% (4/38) HD patients and 7. 4% (2/27) HAD patients. The HAD patients included 22 (75. 9%) hypoganglionosis (HG) cases and 7 (21.1 %) intestinal neuronal dysplasia (IND) cases. Among the 27 HAD patients, 27 (93. 1%) had anorectal inhibitory reflex, and varied and delayed reflection waveforms. AChE staining was positive in 12 (41.1%) patients. The results of anorectal manometry and AChE staining of the HAD patients were significantly different from those of the HD patients (P＜0. 01 ). Barium enema of the HAD patients appeared severe expanded rectum which occupied more than 90% space of the pelvic cavity. Redundant sigmoid colon was also found in more than 90% patients. All HAD patients had 24-hour barium retention. Subtotal colectomies were performed on 9 (31. 0%) HAD patients,and 8 (22. 2%) HD patients. Conclusions Anorectal manometry, AChE staining and barium enema are still important in determining the diagnosis and surgical indications for HAD. The efficacy of 3 months or longer conservative treatment is crucial to determine the surgical indications for HAD. Key words: Hirschsprung's disease; Manometry; Barium enema