Hirchsprung’s DISEASE is a developmental disorder of the enteric nervous system, characterized by abdsence of ganglion cell in the myenterric and submucosal plexuese along a variable portion of the distal intestine.The purpose of this study is the presentation of a new procedure for reduction of the postoperative complications of HIRSCHSPRUNG’s DISEASE (HD) by offering a modified Swenson’s technique. This was a chieved through a 10 years study of 157 patients with HD, of whom 74% were male and 26% wre female. In 100 cases the DISEASE was diagnosed in the early neonatal period, in 129 cases the involved segments were rectosigmoid or shortsegment. Our choice of operation was a new modification of swenson’s type of pull through (Swenson’s operation + posterior rectal wall myectomy). Phatholgical examination has confirmed the diagnosis of HD in all patients. The overall results of surgery were good in 81% and there was no mortality.

Background: HDR syndrome (hypoparathyroidism, sensorineural deafness and renal DISEASE) is an autosomal dominant condition, defined by the triad hypoparathyroidism, renal dysplasia and hearing loss. HIRSCHSPRUNG (HSCR) DISEASE is a variable congenital absence of ganglion cells of the enteric nervous system resulting in degrees of functional bowel obstruction. Rarer chromosomal anomalies are reported in combination with HIRSCHSPRUNG DISEASE like DiGeorge syndrome, mosaic trisomy 8, XXY chromosomal constitution, partial duplication of chromosome 2q, tetrasomy 9p, and 20p deletion.Case Presentation: Here, we describe an 8 year-old girl with HDR syndrome accompanied by HIRSCHSPRUNG DISEASE. Although the association of HIRSCHSPRUNG DISEASE with chromosomal anomalies has been reported, according to our knowledge, this is the first report of associated HSCR with HDR syndrome.

Introduction & Aims: The study aims to investigate barium enema accuracy in diagnosing Hischsprung's DISEASE (HD). Patients & Methods: This study was performed on 58 patients diagnosed with Hischsprung's DISEASE later confirmed by pathology; the patients had undergone surgical operation in the last 3 years. The radiographs were reached from their medical records and were later assessed by a skilled pediatric radiologist. Bar-ium enema findings were also evaluated. Rectosig-moid index with the sign of transitional zone (TZ), and barium evacuation of delayed radiographs were recorded by a radiologist. Results: We measured accuracy of rectosigmoid index in these patients to be 94.8%, the sign of TZ to be 70.7%, and the barium evacuation of delayed radio-graphs to be 100% for diagnosis of HD. Fifty percent of the cases were neonates, 42% infants, and 7% more than 2 years old. Of all the patients, 76% were male and 24% were female. The most common DISEASEs accompanying HD were cardiac abnormalities. We did not find any total aganglionosis. Conclusion & Discussion: Half of the patients were newborn infants. The fact that the ratio is more than usual shows timely diagnosis of the condition by phy-sicians of this center. Barium enema findings in new-borns of our study contradicted significantly the re-searches done in other countries. In this study, it was found that with increasing age, barium enema find-ings would be defined in such a way that in in-fantsover 2 years, it can reach a diagnosis accuracy of up to 100%. Regarding the availability of barium en-ema and its lower costs than other diagnostic meth-ods, its accuracy seems very promising. Our study emphasizes that perhaps with barium enema, we may operate these infants in one stage so that they can get rid of multiphasic operation difficulties.