No abstract available.
Diagnosis* ; Hirschsprung Disease* ; Manometry*

No abstract available.
Barium* ; Diagnosis* ; Enema* ; Hirschsprung Disease*

Consensus ; Hirschsprung Disease ; diagnosis ; Humans

Diagnosing Hirschprungs disease (HD) is a clinical challenge to pediatric surgeons. The cardinal symptoms are failure of passage of meconium within first 24 hours of life, abdominal distension, and vomiting. The severity of these symptoms and the degree of consitpation vary considerably between patients. HD is suspected on the basis of history and clinical findings and the diagnosis is established by radiological examination, anorectal manometry, and histochemical analysis of biopsy specimens. In this review, the advantages and pitfalls of each diagnostic modality are discussed. And a diagnostic approach utilizing these diagnostic modalities in children with suspicious HD is presented.
Biopsy ; Child ; Diagnosis* ; Hirschsprung Disease* ; Humans ; Manometry ; Meconium ; Vomiting

No abstract available.
Constipation/*diagnosis ; Female ; Hirschsprung Disease/*diagnosis ; Humans ; Leukocyte L1 Antigen Complex/*analysis ; Male

PURPOSE: The term benign transient non-organic ileus of neonates (BTNIN) is applied to neonates who present symptoms and plain radiographic findings of Hirschsprung's disease, but do not have aganglionic bowel and are managed well by conservative treatment. It can often be difficult to diagnose BTNIN because its initial symptoms are similar to those of Hirschsprung's disease. The aim of this study is to evaluate the clinical characteristics and proper treatment of BTNIN. MATERIALS AND METHODS: A retrospective review was made on the clinical data of 19 neonates who were treated for BTNIN between January 2008 and December 2011 at a single facility. RESULTS: Abdominal distension occurred in every patient (19/19). Other common symptoms included emesis (5/19), explosive defecation (5/19), and constipation (4/19). The vast majority of patients (15/19) experienced the onset of symptoms between 2 and 4 weeks of age. Radiograph findings from all of the patients were similar to Hirschsprung's disease. A barium study showed a transition zone in 33.4% (6/18) of the patients. However, rectal biopsy revealed ganglion cells in the distal rectum in 88.2% (15/17) of the patients, and anorectal manometry showed a normal rectoanal inhibitory reflex in 90% (9/10). All patients responded well to conservative treatment. Symptoms disappeared at the mean age of 4.9+/-1.0 months, and the abdominal radiographs normalized. CONCLUSION: BTNIN had an excellent outcome with conservative treatment, and must be differentiated from Hirschsprung's disease. A rectal biopsy and anorectal manometry were useful diagnostic tools in the differential diagnosis.
Female ; Hirschsprung Disease/diagnosis/pathology ; Humans ; Ileus/*diagnosis/pathology ; Infant, Newborn ; Male ; Rectum/pathology ; Retrospective Studies

PURPOSE: To determine the relative accuracy of barium enema findings of Hirschsprung's disease (HD) and to calculate a level of probability for three signs combined. MATERIAL AND METHODS: Barium enema findings in 45 patients who had undergone rectal biopsy to prove or exclude the diagnosis of HD were retrospectively analyzed by evaluating the presence of a transition zone, irregular contractions and delayed evacuation of barium. Seventeen were neonates (group 1) and the other 28 were infants and children (group 2). The sensitivity, specificity, and positive and negative predictive values of the findings were compared. RESULTS: In visualization of a transition zone, sensitivity, specificity and positive predictive value were 76.5%, 72.7% and 89.7%, respectively. Sensitivity for irregular contractions and delayed evacuation of barium was 76.5% and 91.7%, respectively, whereas for specificity, the corresponding values were 63.6% and 40%. Sensitivities for radiologic signs were higher in group 1 than in group 2, but, the specificities were lower. If two or three findings were positive, the level of probability was 85 - 100%. If two findings were negative, however, the corresponding value was 30%. CONCLUSION: We conclude that the most reliable HD finding is the presence of a transition zone. Irregular contractions and the delayed evacuation of barium are not specific. Two or three positive findings may suggest a higher probability of HD than any single positive finding alone.
Barium* ; Biopsy ; Child ; Diagnosis* ; Enema* ; Hirschsprung Disease* ; Humans ; Infant ; Infant, Newborn ; Retrospective Studies ; Sensitivity and Specificity

BACKGROUND/AIMS: It is difficult to clinically distinguish infants with Hirschsprung disease (HD) from those with other causes of intestinal obstruction. Therefore, reliable and safe diagnostic procedures are particularly necessary in infants with early onset symptoms. The purpose of this study was to assess the accuracy of anorectal manometry in the diagnosis of HD in infancy. METHODS: Forty five infants who were suspected of having HD, at Seoul National University Children's Hospital, from May 1999 to July 2002, were enrolled in this study. The diagnostic accuracy of recto-anal inhibitory reflex (RAIR) and transition zone was evaluated. RESULTS: Final diagnosis of HD was made in 18 cases. The presence of transition zone was proved to be very specific (100%), but sensitivity was very low (39%). The sensitivity and specificity of anorectal manometry were 100% and 93% in total 45 infants. However the sensitivity and specificity of this test were 100% in infants older than 3 months. CONCLUSIONS: It could be stated that the diagnostic accuracy of the RAIR is a highly valid diagnostic value for HD in young infancy. It is particularly helpful when clinical and radiological findings are inconclusive.
Diagnosis* ; Hirschsprung Disease* ; Humans ; Infant ; Intestinal Obstruction ; Manometry ; Reflex* ; Sensitivity and Specificity ; Seoul

PURPOSE: In small portion of patients with Hirschsprung's disease, the aganglionic stagment extends only up to the mid-rectum. This report describes an innovative and effective posterior sagittal anorectal myectomy for curative repair of ultrashort segment Hirschsprung's disease in neonates. METHODS: The procedure was performed on ten patients with ultrashort segment Hirschsprung's disease between 1995 to 1998. The procedure was performed by making a sagittal incision in midline posterior perineum to expose the posterior rectum.4 longitudinal strip of muscular layer is removed from the aganglionic portion of the anorectum from the upper rectum to the internal sphincter. The patients were followed postoperatively to determine the effectiveness of the procedure and to observe the presence of any complications. RESULTS: Seven patients (70.0%) were under three months old. Operative biopsy showed that four patients had aganglionosis in the upper rectum, three patients in the middle rectum and three patients in the lower rectum. The posterior sagittal anorectal myectomy was successful in the treatment of ultrashort segment Hirschsprung's disease- symptom recurred for one patient due to total aganglionosis and for another patients due to rectosigmoid aganglionosis. These two patients received the staplingprocedure after colostomy. Some transient complications included mucosal perforation during surgery (30.0%) and incisional wound infection (20.0%). CONCLUSION: For those neonates with ultrashort segment Hirschsprung's disease, the posterior sagittal autorectal myectomy should be considered a safe and effective method for treatment and confirmatory diagnosis. In addition, preliminary colostomy is not required prior to this procedure.
Biopsy ; Colostomy ; Diagnosis ; Hirschsprung Disease* ; Humans ; Infant, Newborn ; Perineum ; Rectum ; Wound Infection

PURPOSE: Hirschsprung’s disease (HD) is a congenital intestinal disorder with absence of ganglion cells in the intestinal muscle and submucosa. Diagnosis is based on histopathological study such as H&E, and acetylcholinesterase (AchE) immunohistochemistry. Calretinin immunohistochemistry was introduced as a new diagnostic method against limitations of other staining. The aim of this study is to investigate the usefulness of calretinin immunohistochemistry for the diagnosis of HD compared to H&E and AchE. METHODS: Ten patients with HD and 22 non-HD patients were included in the study. H&E staining, AchE and calretinin immunohistochemistry were performed in all 32 patients. All slides were evaluated by same single pathologist and the diagnostic value was calculated for each H&E stain, AchE immunohistochemical staining, and calretinin immunohistochemical staining. RESULTS: Calretinin method had sensitivity of 100% and specificity of 100% for diagnosis of HD. Its diagnostic accuracy was 100%. AchE staining showed 100% of specificity and 80% of sensitivity. Diagnostic accuracy of H&E staining was 56.3%. CONCLUSION: We concluded that calretinin immunohistochemistry is a very useful and valuable method to diagnosis HD patient.
Acetylcholinesterase ; Biopsy* ; Calbindin 2* ; Diagnosis ; Ganglion Cysts ; Hematoxylin ; Hirschsprung Disease ; Humans ; Immunohistochemistry* ; Sensitivity and Specificity ; Suction*