Hirschsprung's Disease In Infants Research Articles

Clinical utility of anal sphincter relaxation integral in water-perfused and solid-state high-resolution anorectal manometry

We investigated the diagnostic performance of the anal sphincter relaxation integral (ASRI) for infants with Hirschsprung's disease (HD). We performed water-perfused high-resolution anorectal manometry (HRAM) in 18 infants (9 with HD), and solid-state HRAM in another 18 infants (4 with HD). We calculated the ASRI during the rectoanal inhibitory reflex (RAIR) maneuver at pressure cutoffs of <10mmHg (ASRI 10) and <15mmHg (ASRI 15). We investigated the diagnostic performance of the ASRI for HD in infants undergoing water-perfused and solid-state HRAM. HD infants who underwent either water-perfused or solid-state HRAM had significantly lower ASRI 10 and ASRI 15 values, compared with non-HD infants (P<0.05 and P<0.05, respectively). Using the water-perfused HRAM system, ASRI 10 and ASRI 15 values of <7 and<29mmHgs.cm, respectively, exhibited good diagnostic performance for HD (88.89% and 88.89%, respectively). Receiver operating characteristic curve analysis indicated that ASRI 10 and ASRI 15 values of <5.5 and<20mmHgs.cm, respectively, were optimal for the diagnosis of HD infants when using the solid-state HRAM system, with high diagnostic accuracies of 83.33% and 83.33%, respectively. ASRI may assist the diagnosis of HD infants using either water-perfused or solid-state HRAM. These systems require different catheter-specific ASRI cutoffs for the prediction of HD.

Особенности диагностики и тактики хирургического лечения болезни Гиршпрунга у грудных детей

The aim is to present optimal ways of early diagnosis and tactics of Hirschsprung's disease (HD) surgical correction in newborns and young children based on literature data and our own experience. Materials and methods. Clinical observations on early diagnosis and optimization of the tactics of surgical treatment of HD in 58 infants over the past 9 years have been carried out. All patients underwent a complex of general physical examinations, including the collection of anamnesis and follow-up of patients, clinical examination with rectal examination, laboratory, bacteriological, radiological (survey, polypositional irrigography) and morphological (intraoperatively remote area of the colon agangliosis zone) studies. The features of the clinical course and tactics of surgical treatment of HD in infants, the choice of the surgical method were analyzed, and the risk of complications during the operation was studied. The patients were divided into two groups. The first group included 32 (55.2%) infants with a typical clinical course of HD, radiographically confirmed agangliosis in the rectosigmoid zone, and chronic constipation. The second group included 26 (44.8%) infants with an atypical clinical course of HD with diarrheal syndrome against the background of dysbiosis, enterocolitis, among them there were 2 children with symptoms of Hirschsprung-associated enterocolitis. Research methods: General clinical and biochemical analysis of blood and urine. Scatological examination of feces, sowing on the pathogenic flora of feces and urine. EKG. Ultrasound examination of the abdominal cavity and echocardiography. X-ray examination: an overview roentgenogram of the abdominal and thoracic cavity vertically, irrigoraphy, excretory urography. Results and conclusions. In newborns with sub- and decompensated typical course of chronic constipation in the absence of the effect of conservative therapy, after the diagnosis of HD is established, an early radical operation or colostomy application above the agangliosis zone is recommended. In infants with a favorable typical clinical course and a left-sided form of HD, transanal endorectal reduction of the large intestine (TENTC) can be performed according to indications, starting from one month of age. In this case, the operation of choice is TENTK without colostomy. In subtotal and rectosigmoidal forms of HD with a long aganglionic zone, laparoscopic assistance is recommended, which makes it possible to release the splenic flexure of the colon from ligaments and constrictions, take a full-thickness biopsy of the transitional zone of aganglionicosis, and this makes it possible to conduct express diagnostics, establish resection levels and freely lower the proximal area colon during TENTC. The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of all participating institution. The informed consent of the patient was obtained for conducting the studies. No conflict of interest was declared by the authors. Key words: Hirschsprung’s disease, clinic, infants, trans anal colon reduction.

Clinical Features of Early Diagnosis and a Choice of Method of Surgery for Hirschsprung Disease in Infants

The aim of this study is to improve the results of surgical treatment of Hirschsprung’s disease in infants bychoosing the most effective method.This article presents the results of the treatment of Hirschsprung’s disease in 61 patients of infancy. However,immediate and long-term results of the children’s treatment with HD, operated on in infancy, using thetraditional Soave-Boley method and modern minimally invasive technologies according to TENTC andthe Georgeson method, which show different results, have been studied. Among 26 patients who had beenoperated on by the Soave - Boley method, the following results were obtained : excellent - in 10 (38.4%);good - in 8 (30.7%); satisfactory - in 6 (23%) and unsatisfactory - in 2 (7.6%). Among 29 patients operatedon by the TEPT method, except for one patient, excellent and good results were obtained in 28 (96.6%)patients. What is more, among 6 patients who had been operated on by the Georgeson method, excellentand good results were obtained in all cases; there were no satisfactory or unsatisfactory results. It shouldbe noted that among the operated HD patients according to the Soave-Boley method, there were severepatients, of which 7 patients had a colostomy in the neonatal period.At the same time, radical one-stage surgical treatment for distal forms of HD using minimally invasive TEPTmethod is a priority in infancy, which can reduce complications to a minimum with improved functionaloutcomes of treatment, relative to two-stage treatment.

Colonic diverticulosis in an infant: A clue to total colonic Hirschsprung disease

Total colonic aganglionosis is a rare phenotype of Hirschsprung disease (HD). While the diagnosis is generally established within the neonatal period there are reports of delayed presentation. In this case, we describe a 9-month old girl with no previous medical or surgical history who presented with a small bowel obstruction. A contrast enema performed as part of the work up demonstrated right sided colonic diverticula with no other anatomical abnormalities. Surgical intervention was required to manage the obstruction and colonic biopsies were consistent with HD. This case highlights the association of the unusual finding of colonic diverticula with total colonic HD in infants.

Diagnostic Role of Anal Sphincter Relaxation Integral in High-Resolution Anorectal Manometry for Hirschsprung Disease in Infants

To investigate the possible diagnostic role of anal sphincter relaxation integral (ASRI) in high-resolution anorectal manometry (HRAM) for Hirschsprung disease. We performed conventional anorectal manometry (ARM) in 24 infants (8 with Hirschsprung disease and 16 without Hirschsprung disease) and HRAM in another 21 infants (9 with Hirschsprung disease and 12 without Hirschsprung disease) before and after October 2014. All infants underwent rectal suction biopsy for confirmation of Hirschsprung disease. We quantified rectoanal inhibitory reflex (RAIR) adequacy by calculating the ASRI in HRAM study at pressure cutoffs of less than 10, 15, and 20 mm Hg (ASRI10, ASRI15, and ASRI20, respectively) and investigated the diagnostic utility. Patients with Hirschsprung disease who underwent HRAM had significantly lower ASRI10, ASRI15, and ASRI20 values than did infants without Hirschsprung disease (P = .0002, .0002, and .0003, respectively), indicating significant difference in internal anal sphincter relaxation during RAIR test between these 2 groups. ASRI10 exhibited a greater diagnostic accuracy, area under the curve, sensitivity, and specificity than did ASRI15 and ASRI20 for Hirschsprung disease. Moreover, the diagnostic accuracy of HRAM for Hirschsprung disease based on ASRI10 <7 mm Hg.s.cm was significantly greater than that of conventional ARM (P = .02). ASRI10 may be indicative of the adequacy of RAIR by HRAM in infants, thus assisting the diagnosis of Hirschsprung disease. The diagnostic accuracy of HRAM (based on the ASRI10 value) is greater than that of conventional ARM for Hirschsprung disease. ASRI10 may be used in an automatic HRAM analysis system for the diagnosis of anorectal motility disorders.

Clinical efficacy of laparoscopic Soave's surgery for the treatment of congenital Hirschsprung's disease in infants

Objective To investigate the clinical efficacy of laparoscopic Soave's surgery for the treatment of congenital Hirschsprung's disease in infants (age ＜ 2 months).Methods The clinical data of 368 infants with congenital Hirschsprung's disease who received laparoscopic Soave's surgery at the Children's Hospital of Xuzhou City from June 2005 to June 2012 were retrospectively analyzed.Three Trocars with diameter of 0.5 cm were used.The colic ligament was freed and the mesentery was ligated laparoscopically,then the rectal mucosa was stripped for 5-7 cm in length,and was pulled out through the rectal muscle sheath.Patients were followed up via out-patient examination at postoperative month 1,3,6,12 to learn the frequency of defecation,constipation and anal stenosis.The follow-up was ended in June of 2013.Results Two patients were converted to open surgery,and the remaining 366 patients received laparoscopic surgery successfully,with no incidence of wound infection.The operation time,volume of blood loss and duration of hospital stay were (100 ±20)minutes,(5.4 ± 1.5) mL and (7.3 ± 1.5) days,respectively.The average time for antibiotics usage was (3.5 ± 1.6) days and the time for infusion was (3.8 ± 1.4) days.All the infants were followed up,and the mean time for postoperative follow-up was 4.5 years (range,1.0-8.0 years).Eleven patients were complicated with Hirschsprung's disease associated enteritis,7 patients with constipation,5 with anal stenosis,4 with fecal pollution.The condition of the infants was improved after conservative treatment.Conclusion Laparoscopic Soave's surgery is suitable for the treatment of congenital Hirschsprung's diseases in infants aged under 2 months,it has the advantages of small trauma,quick recovery,better cosmetic effects and few complications. Key words: Congenital Hirschsprung's diseases ; Laparoscopy ; Infant

Anal ultraslow waves and high anal pressure in childhood: a clinical condition mimicking Hirschsprung disease

Purpose Anal ultraslow waves (USWs) have been described in several clinical conditions closely related to chronic constipation associated with high anal pressure; however, USW-related clinical manifestations in childhood are poorly understood. The purpose of this study is to elucidate the clinical relevance of USWs in childhood. Methods Manometric recordings of 118 cases including 70 children with constipation and 16 patients with Hirschsprung disease were analyzed. Results Ultraslow waves were seen in 4 of 70 children with constipation. None of the controls or patients with Hirschsprung disease exhibited USWs. The 4 patients comprised 2 infants with marked abdominal distension mimicking Hirschsprung disease and 2 children (aged 4 and 8 years) with intractable constipation accompanying hemorrhoid or anal fissure. The manometric findings of the USW-positive patients showed a markedly high anal resting pressure and high frequency of slow waves compared to controls, patients with constipation not accompanied by USWs or patients with Hirschsprung disease. Conclusion Children with USWs exhibit symptoms mimicking Hirschsprung disease in infants and chronic intractable constipation in older children. In manometric studies of children, more attention should be paid not only to rectoanal reflex, but also USWs.

Histochemical Diagnosis of Hirschsprung Disease

A histochemical staining technique for detection of acetylcholinesterase (AChE) in rectal suction biopsies was compared with the presence or absence of ganglion cells in full-thickness or suction biopsies for the diagnosis of Hirschsprung disease (HD) in infants and children. Biopsies from 55 of 58 children were adequate for both the AChE assay and routine pathologic examination for ganglion cells. Two patterns of AChE staining were noted. With pattern A, prominent nerve fibers staining for AChE were seen throughout the muscularis mucosa and the lamina propria. With pattern B, similar fibers were seen only in the muscularis mucosa and the areas of lamina propria that were immediately adjacent. No "false-negative" AChE staining reactions were found in patients with HD. No "false-positive" reactions showing pattern A were found. This pattern was diagnostic for HD. Three false-positive reactions were found showing pattern B in patients with conditions other than HD. Among 22 patients with HD, 19 were males and three were females. Pattern A occurred in all age groups and in both sexes. Pattern B in patients with HD was seen exclusively in male infants 1 month of age or less. Experience suggests that the AChE staining of rectal suction biopsies is an excellent screening test for HD in infants and children. If pattern B is encountered, however, the specimen should be examined by routine pathologic techniques for the presence of submucosal ganglion cells.