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

Consensus ; Hirschsprung Disease ; diagnosis ; Humans

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

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

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

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

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: 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

OBJECTIVEHirschsprung's disease (HD), one of the most common causes resulting in lower intestinal obstruction in children, is prone to be misdiagnosed or to be missed from diagnosis because of its atypical clinical symptoms and inconspicuous morphological findings by barium enema X-ray. Recently, this situation has been largely ameliorated by increased comprehension of anorectal kinetics and improvement of instrument for measurement of anorectal pressure. By now, anorectal manometry (ARMM) has been regarded as a routine means for functional assessment and diagnosis for anorectal disease. Nevertheless, the accuracy rate of diagnosis of HD in neonate by ARMM remains to be elucidated. In this study the clinical evaluation of anorectal manometry as an early diagnostic method for neonates with Hirschsprung's disease was appraised.

METHODSForty-two HD patients defined by pathological study of rectal tissue obtained via rectal mucous membrane biopsy or operation were recruited in this study. ARMM was performed in liquid transmission using PC polygraph high rate gastrointestinal dynamical detection system (PC Polygraf HR, CTD-synectics, Sweden), with 4-lumen catheter with which a small 5-cm-long balloon was connected at the terminus. All children were positioned on their left side or back during the procedure and the pressure transducers were placed in the mid-axillary line level. The results of ARMM performed before operation or biopsy were compared with the results of barium enema X-ray testing. The decrease of internal anal sphincter pressure as rectoanal inhibitory reflex (RAIR) was measured based on the fluctuation curve of pressure detected. HD was defined when no decrease of anal catheter pressure was detected after insufflation (RAIR positive), and suspected HD state was assessed with the presentation of incomplete relaxation or positive/negative results coexisted (RAIR abnormal) in canal.

RESULTSThirty patients (71.43%) were diagnosed as HD by ARMM including 18 patients who showed negative response to RAIR and 12 patients whose response was abnormal. While barium enema examinations were carried out in all the 45 patients, the results showed 5 HD patients and 14 suspected HD patients, giving an overall diagnostic accuracy of 45.24%. There were also 16 patients with positive ARMM response and negative barium enema findings together, and 5 patients with negative ARMM results and positive barium enema findings at the same time. There was a significant difference between the two diagnostic methods (chi(m)(2) = 4.76, P < 0.05).

CONCLUSIONAnorectal manometry seems to be a more reliable method for diagnosis of Hirschsprung's disease in neonate than barium enema X-ray. Because ARMM is a simple, safe and non-invasive method, it can be used as a screening test of choice in neonates with clinically suspected HD. But for final diagnosis, it is reasonable to combine ARMM with other diagnostic methods in HD patients.

Anal Canal ; physiopathology ; Barium Sulfate ; Enema ; Hirschsprung Disease ; diagnosis ; Humans ; Infant, Newborn ; Manometry ; Rectum ; pathology ; physiopathology

Hirschsprung's disease (HD) is one of the most common cause of intestinal obstruction in neonates. Enterocolitis is a well known complication of HD. Since a severe form of enterocolitis occurring in HD is the major cause of death and disability of these patients, early diagnosis is essential. Recently, ischemic hemorrhagic necrotizing enterocolitis have been reported as lethal complications of HD. However little distinction is made between inflammatory enterocolitis which often follows HD and hemorrhagic ischemic necrotizing enterocolitis, which is a rare, and often fatal complication of HD. Most of reported cases of hemorrhagic necrotizing enterocolitis developed very early, generally within 3 days of birth except several cases. Which may be confused with necrotizing enterocolitis (NEC) which was not associated with HD. We report here two cases of hemorrhagic necrotizing enterocolitis associated with Hirschsprung's disease, who were initially diagnosed as having NEC.
Cause of Death ; Early Diagnosis ; Enterocolitis ; Enterocolitis, Necrotizing* ; Hirschsprung Disease* ; Humans ; Infant, Newborn ; Intestinal Obstruction ; Parturition