Rectal Suction Biopsy Research Articles

A comparison of suction and full-thickness rectal biopsy in children

BackgroundRectal biopsy evaluation by an experienced pathologist is the gold standard in diagnosis of Hirschsprung's disease (HD). Although both suction rectal biopsy (SRB) and full-thickness (FTRB) rectal biopsy are performed, the ability for SRB to obtain adequate tissue in older children has been questioned. We hypothesized that SRB and FTRB yield tissue specimens of different size but are equally adequate for diagnosis. MethodsRecords of children who underwent rectal biopsy to evaluate for HD between January 2007 and July 2014 were reviewed. Volume, percent submucosa, and specimen adequacy were compared between biopsy techniques, and the effect of age on biopsy adequacy was assessed. Data were analyzed by mixed-effects models with covariate adjustment for age at biopsy and Fisher's exact test. ResultsForty-seven children underwent a total of 58 biopsies, 45 SRB and 13 FTRB. Thirty-seven were performed before 12 mo of age, and 21 after 12 mo of age. Volume of SRB specimens was significantly smaller than FTRB across ages (14.8 ± 7.8 mm3versus 121.3 ± 13.8 mm3, P = 0.0001). Percent submucosa did not differ significantly between SRB and FTRB specimens across ages (63.8 ± 2.7% versus 66.5 ± 4.3%, P = 0.575). The number of inadequate biopsies was low and not significantly different across ages (P = 0.345), or when comparing by biopsy method (P = 0.689). All biopsies were clinically diagnostic. There were no complications. ConclusionsTissue specimens obtained by SRB are smaller than those obtained by FTRB, especially in older children. SRB and FTRB appear equivalent in their ability to provide adequate submucosa. Differences in cost and patient satisfaction between rectal biopsy techniques must be studied to further define the best overall technique.

Maladie de Hirschsprung, diagnostic et prise en charge actuelle

Hirschsprung disease is a congenital malformation in which, due to lack of neural crest cell migration, the distal part of the bowel has no innervation, leading to functional obstruction. This rare disease (1/5000 live birth) has a complex genetic and physiopathologic background. Depending on the disease level, symptoms vary from distal obstruction to short bowel syndrome. Diagnosis is based on suction rectal biopsy examination and contrast enema. After the obstruction relief, surgical cure aim to remove the aganglionic bowel and to perform laparoscopic or transanal pullthrough. Enterocolitis or anal soiling in childhood can disturb follow up.

Rectal suction biopsy for the diagnosis of Hirschsprung's disease: a systematic review of diagnostic accuracy and complications.

Rectal suction biopsy (RSB) combined with acetylcholinesterase (AChE) staining is currently the gold standard for the diagnosis of Hirschsprung's disease (HD). However, some pathologists are still reluctant to diagnose HD in RSBs as it requires thick submucosal tissue to confirm the absence of ganglion cells. Furthermore, insufficient specimens and adverse events have been reported. The objective of this study was to determine the diagnostic accuracy and incidence of complications of RSBs in patients suspected of HD based on a systematic review of the published literature. A literature-based search for relevant publications was conducted using multiple online databases. Staining for AChE/hematoxylin and eosin should have been used to evaluate RSBs. The number of true-positive, false-positive, true-negative and false-negative results was recorded and data on RSB-related complications was extracted. Pooled incidence rates and odds ratios (ORs) with 95% confidence intervals (CI) were calculated using standardized statistical methodology. Fifty-eight studies met defined inclusion criteria, reporting a total of 14,053 RSBs. The median cohort size consisted of 72 patients (range 1-766) with a median age of 14.4 months (range 1 day-66 years) at time of biopsy. In 89.93% (CI 89.11-90.70%), RSB provided adequate tissue for the diagnosis of HD. Insufficient specimens were obtained in 10.07% (CI 9.30-10.89%) and in 8.46 % (CI 7.55-9.46%) RSB was repeated. The incidence of HD was 19.13% (CI 18.25-20.03%). Mean sensitivity of RSB was 96.84% (CI 95.57-97.47%) and mean specificity was 99.42% (CI 99.17-99.57%). The overall complication rate was 0.65% (CI 0.46-0.91%) with persistent rectal bleeding requiring blood transfusion in 0.53% (CI 0.36-0.77%), bowel perforation in 0.06% (CI 0.02-0.18%) and pelvic sepsis in 0.06% (CI 0.02-0.18%). Complications were significantly more frequent in newborns and infants compared to older children (OR 9.00 [CI 4.75-17.07], p < 0.0001). RSB combined with AChE staining is a simple, safe and accurate method for the diagnosis of HD. The risk for RSB-related complications is higher in newborns and infants.

Infant’s Age Influences the Accuracy of Rectal Suction Biopsies for Diagnosing of Hirschsprung’s Disease

Hirschsprung's disease (HD) is a rare birth defect of the distal colon. Analysis of rectal suction biopsy (RSB) is considered to be the most reliable method for its diagnosis in infants. However, the diagnostic accuracy of RSB analysis could be affected by the patient's age, possibly because of rapid development of the enteric nervous system in the first weeks after birth. Because there is a trend toward testing for HD at early ages, we aimed to determine whether the diagnostic accuracy of RSB analysis is associated with the patient's age. We performed a retrospective analysis of all patients from whom 1 or more RSBs were analyzed from 1975 through 2011 (529 RSBs from 441 patients). Outcomes of RSB analyses were categorized as positive, inconclusive, or negative for HD. Primary diagnoses, based only on RSB, were compared with final diagnoses made after at least 1 year of clinical follow-up. Age at time of RSB analysis was corrected for the gestational age. By using these criteria, we determined the diagnostic accuracy of RSB analysis for different age groups. RSB analysis identified HD in patients with sensitivity values of 46% (patients -45 to 7 days old), 47% (8-22 days old), and 62% (23-39 days old) (corrected for gestational age). The average sensitivity with which RSB analysis identified HD in patients older than 39 days was 88%. RSB identified HD in patients younger than 39 days old with significantly lower sensitivity than in older patients (50% vs 88%, P < .001). The specificity with which RSB identified infants without HD was not affected by age (average 95%). Of all RSBs, 11% were inconclusive for the diagnosis of HD. RSB analysis identifies HD in patients younger than 39 days old with only 50% sensitivity. Moreover, RSBs obtained from younger patients often lead to inconclusive outcomes and require additional biopsies. We propose that for infants suspected of HD at these ages, a noninvasive technique, such as anorectal manometry, should be used for a primary diagnosis. RSB should thereafter be used to confirm the diagnosis when the infant is older than 39 days.

Food, Fiber, and Formula

A term infant who failed to pass meconium and developed bilious emesis on the first day of life underwent a suction rectal biopsy, which revealed the absence of ganglion cells consistent with Hirschsprung’s disease. His final postoperative pathology report indicated an absence of ganglion cells in the anorectal mucosa but some eosinophils in the myenteric plexus and surrounding smooth muscle of the transition zone, aganglionic sigmoid colon, and rectum. These findings are consistent with diagnosis of eosinophilic myenteric ganglionitis. At 6 months old, he had a Soave endorectal pull-through with resection. He had significant, prolonged explosive diarrhea leading to severe perianal breakdown and weight loss. An amino-acid-based infant formula was trialed, and the diarrhea resolved quickly. Ongoing nutrition counseling was needed because of milk sensitivity. He was prone to constipation, and a high-fiber diet was needed. This case shows how collaboration between the pediatric dietitian and pediatric surgical team can lead to better nutritional outcomes including optimal growth, improved formula tolerance, and decreased gastrointestinal (GI)-related complications after surgery. It also reinforces how long-term follow-up is critical from both a nutrition and surgical standpoint, to address the long-term nutrition issues that can ensue from surgical intervention of Hirschsprung’s disease in infancy. This particular case is especially interesting as the patient is the youngest documented case found in the literature with a case of eosinophilic myenteric ganglionitis occurring with Hirschsprung’s disease.

High-resolution anorectal manometry in newborns: normative values and diagnostic utility in Hirschsprung disease.

Conventional methods of screening for Hirschsprung disease (HD) in newborns (barium enema, BE; anorectal manometry, ARM; rectal suction biopsy, RSB) have limitations and/or are invasive. High-resolution anorectal manometry (HR-ARM) is a minimally invasive technique that has potential to overcome most of these limitations, but normative data and performance characteristics have not been reported in newborns. The aims of our study were to assess anorectal sphincter metrics including resting pressure (RP), anal canal length (ACL), and rectoanal inhibitory reflex (RAIR) in healthy and asymptomatic newborns, and to explore the role of HR-ARM in the diagnosis of HD using these normal parameters. All procedures were performed using solid state HR-ARM equipment (Medical Measurement Systems, Enchede, The Netherland) by a single operator. In the first phase, 180 asymptomatic newborns (term newborns 95, preterm newborns 85) were studied, and anal RP, ACL, and RAIR were measured. In the second phase, 16 newborns with clinical manifestations of HD were studied (9 of whom had histopathologic confirmation), and parameters compared to asymptomatic newborns. Normative RP values were higher in term newborns compared with preterm newborns (p < 0.05), and correlated with age. Progressive maturation of the anal sphincter was evident with chronologic age, both in preterm and term newborns. RAIR was present in all normal subjects. Using absent RAIR as indicative of HD, HR-ARM had a sensitivity 89% and specificity of 83% compared to RSB; these performance characteristics were better than BE (sensitivity 78%, specificity 17%), with significantly higher diagnostic accuracy (80% vs 53%, respectively, p = 0.009). Anorectal sphincter pressure progressively matures with incremental increase in RP during the first months of life. HR-ARM is an effective and safe method that complements the diagnosis of HD in newborns.

The Case Files

The Case Files

Preterm with appendicular abscess and subcapsular liver haematoma

Neonatal appendicitis is a rare yet serious condition. Known reported associations are Hirschsprung’s disease, necrotising enterocolitis (NEC) and cystic fibrosis. The occurrence of subcapsular liver haematoma in preterm neonates can be life threatening. We present an unreported association of these two serious conditions in a preterm infant with appendicular abscess and subcapsular liver haematoma. She presented with sepsis, enlarged liver and tender right iliac fossa mass. Laparotomy was performed for possible complicated NEC. Intraoperatively, there was an appendicular abscess with a small subcapsular liver haematoma and no evidence of NEC. Appendectomy and peritoneal toilet were performed. She had a stormy postoperative period due to sepsis, thrombocytopaenia and expansion of her liver haematoma. The pathology of her appendix was in keeping with appendicitis, with no evidence of NEC. Her liver haematoma gradually resolved. Suction rectal biopsy showed normal ganglia. She was discharged after 7 weeks and remained well since then. Keywords : hematoma, liver, neonatal appendicitis, preterm, subcapsular

A variant pattern of calretinin immunohistochemistry on rectal suction-biopsies is fully specific of short-segment Hirschsprung's disease.

Calretinin immunohistochemistry is now widely used to diagnose Hirschsprung's disease (HD), since loss of calretinin expression within the mucosa and muscularis mucosae of rectal suction-biopsy is pathognomonic of HD. However, a stippled staining may be observed within hypertrophic nerves in the submucosae in some HD patients. The aim of the study was to test the hypothesis that such findings may announce the beginning of the transitional zone. We retrieved 44 consecutive patients (10 girls and 34 boys; median age 6.5 days), diagnosed with aganglionosis on rectal suction biopsies, followed by surgery. According to calretinin immunohistochemistry performed on all paraffin-embedded rectal biopsies, we defined two HD groups: P- showing an absence of any staining within mucosa, muscularis mucosae and submucosa et P+ showing an absence of staining within the mucosa and muscularis mucosae, but a positivity of some submucosal hypertrophic nerves. These data were correlated to the length of total pathological segment (aganglionic and transitional zones) obtained from the original surgery reports. 18/44 patients (40.9 %) belonged to the P+ group and 26/44 (59 %) patients were within the P- group. In the P+ group, the maximal length of the aganglionic zone was 9 cm [mean 4 (1-9)] and the total pathological zone never exceeded 14 cm [mean 8 (3.8-14)]. In the P- group, the maximal length of aganglionic zone was 55.5 cm [mean 11.3 (2.5; 55.5)] and the total pathological zone extended to 59.5 cm [mean 17.75 (4.5; 59.5)]. Aganglionic segment was significantly shorter in the P+ group (p < 0.0001). Staining of some hypertrophic nerves in the submucosa in suction rectal biopsy of HD patients using calretinin immunohistochemistry is only encountered in short-segment aganglionosis with a pathological zone always restricted to rectal and sigmoid colon. This information could be crucial for the surgeons in the decision to choose a transanal procedure.

A Comparison of Rectal Suction and Full Wall Biopsy in Hirschsprung’s Disease

Introduction: Hirschsprung’s disease (HD) is defined as congenital agangliosis of the colon wall from the rectum extending cranially. There are several radiologic methods for screening for HD but it is a biopsy of the colon wall that confirms the diagnosis. Initially the full wall (FW) biopsy was the method of choice. FW biopsy requires general anaesthesia and creates a full wall laceration of the colon wall. The newer method called rectal suction (RS) biopsy is carried out by means of a catheter inserted into the rectum that blindly cuts a biopsy including mucosa and submucosa. It can be performed bedside. If ganglion cells are seen in the biopsy specimen, HD can be excluded. The question that arises is: Can we safely move on from FW biopsy to RS biopsy without compromising diagnostic accuracy? Method and Material: Our study is a retrospective revision of 20 FW biopsies that were supplemented with RS biopsies. RS biopsies with insufficient submucosa were excluded. The RS biopsies were assessed by a senior pathologist in a double blind fashion and the result was compared with FW biopsies. FW biopsy was considered gold standard. Results: The exact binominal test for the non-inferiority test of congruence of RS biopsy with FW biopsy as gold standard was not statistically significant using an inferiority margin of 80% (p = 0.06918) [H0: p_RS ≥ 0.8]. The estimated probability for a successful RS biopsy was 95% and the one-sided 95%-confidence interval associated with the test was 0.784 to 1.00. Conclusion: Our results indicate that we can move on from FW biopsy to RS biopsy without compromising the diagnostic value of the rectal biopsy in a clinically significant way. A prospective study to confirm our results would be of great value.

How Reliable Are Diagnostic Methods of Hirschsprung Disease?

Purpose: The purpose of this study was to compare the diagnostic accuracy of the non-invasive diagnostic methods and rectal suction biopsy for the detection of Hirschsprung disease (HD). Methods: We reviewed diagnostic methods and results retrospectively in patients who underwent anorectal manometry, barium enema and rectal suction biopsy for the diagnosis of HD at Asan Medical Center from January 2000 to December 2012. Results: There were 97 patients (59 neonates and 38 infants) in the study period. The overall accuracy of anorectal manometry for the diagnosis of HD was 71.1% and its sensitivity was 51.4% (48.1% in neonate and 62.5% in infant, respectively) and its overall specificity was 82.3% (81.3% in neonate and 83.3% in infant, respectively). The Overall accuracy of barium enema was 66.0% (72.8% in neonate and 55.3% in infant, respectively) and specificity of barium enema was 53.2% (56.3% in neonate and 50.0% in infant, respectively). These results were lower than those of anorectal manometry. The overall sensitivity of barium enema was 88.6% (92.6% in neonate and 75.0% in infant, respectively) and it was higher than the sensitivity of anorectal manometry. Histological studies confirmed HD in 35 patients, in one of whom the suction biopsy showed negative finding. Conclusion: Accuracy of non-invasive methods for diagnosis of HD in our study is lower than those in previous study, so we need to improve the quality of diagnostic tools in our hospital. We conclude that the rectal suction biopsy is the most accurate test for diagnosing HD, so the biopsy to confirm the diagnosis of the HD is very important.

Calretinin and microtubule-associated protein-2 (MAP-2) immunohistochemistry in the diagnosis of Hirschsprung's disease

Background/PurposeIdentifying ganglion cells by rectal suction biopsy is a basic diagnostic tool for the diagnosis of Hirschsprung's disease (HD). However, the difficult interpretation of conventionally processed slides often necessitates ancillary staining methods. The aim of this study was to evaluate the usefulness of calretinin and microtubule-associated protein-2 (MAP-2) immunohistochemistry in the diagnosis of HD. MethodsWe analyzed 52 rectal suction biopsy specimens (37 from 15 HD patients and 15 from 7 non-HD patients) for ganglion cells with calretinin and MAP-2 immunohistochemistry. We also analyzed full-thickness, frozen biopsy samples obtained from 15 HD patients who underwent surgery utilizing calretinin and MAP-2 immunohistochemistry. ResultsBoth calretinin and MAP-2 positively stained ganglion cells in the submucosal plexus of the ganglionic bowel but not aganglionic bowel. Calretinin usually stained ganglion cell cytoplasm and nuclei more intensely than MAP-2, which only stained cytoplasm. No nerve fiber staining in the submucosal layer was observed for either antibody. In 21.1% (11/52) of samples, calretinin and MAP-2 staining found ganglion cells which were reported not to have ganglion cells in the original surgical pathology reports. Immunohistochemical staining for calretinin using paraffin-embedded tissue sections after cryostat sections clearly demonstrated decreased staining intensity compared to MAP-2. ConclusionCalretinin and MAP-2 are useful diagnostic markers for diagnosing HD in rectal suction biopsies. These complementary methods could ameliorate the diagnostic difficulties associated with HD.

Isolated hypoganglionosis: results of a nationwide survey in Japan

This study analyzes how isolated hypoganglionosis (IH) is diagnosed and treated in Japanese pediatric surgical departments. A second questionnaire was sent to 90 pediatric surgical departments that took part in an initial survey on variant Hirschsprung's disease. Of 109 cases registered as having a certain diagnosis of IH, we targeted 90 patients and excluded the remaining 19. Symptom onset was neonatal ileus with a male:female ratio of 34:56. In most cases, results from radio-contrast enemas and suction rectal biopsies were normal. Anorectal manometry demonstrated that there was no relaxation in 73 % of the 37 patients examined. Furthermore, out of 55 patients who were examined intraoperatively, IH was diagnosed or suspected in 29 cases. Another 12 cases were eventually diagnosed with IH by other institutions using paraffin-embedded specimens. Stomas were initially created in 89 out of 90 cases and the mortality rate of the group that initially received jejunostomies was significantly lower than that of the ileostomy group (p < 0.05). Preoperative diagnosis of IH is often difficult and intraoperative biopsies were only able to provide a correct diagnosis in 52 % of cases. More accurate diagnosis and the initial creation of jejunostomies rather than ileostomies may assist in improving patient management and outcomes.

Classification and diagnostic criteria of variants of Hirschsprung’s disease

"Variants of Hirschsprung's disease" are conditions that clinically resemble Hirschsprung's disease (HD), despite the presence of ganglion cells in rectal suction biopsies. The diagnosis and management of these patients can be challenging. Specific histological, immunohistochemical and electron microscopic investigations are required to characterize this heterogeneous group of functional bowel disorders. Variants of HD include intestinal neuronal dysplasia, intestinal ganglioneuromatosis, isolated hypoganglionosis, immature ganglia, absence of the argyrophil plexus, internal anal sphincter achalasia and congenital smooth muscle cell disorders such as megacystis microcolon intestinal hypoperistalsis syndrome. This review article systematically classifies variants of HD based on current diagnostic criteria with an additional focus on pathogenesis, epidemiology, clinical presentation, management and outcome.

Hirschsprung Disease and Use of Calretinin in Inadequate Rectal Suction Biopsies

Up to 17% of all rectal suction biopsies performed in the evaluation of Hirschsprung disease are considered inadequate. In most instances, inadequate biopsies contain too little submucosa or are taken within the anal transition zone. To examine the utility of calretinin stain in the workup of inadequate biopsies for patients with clinical suspicion of Hirschsprung disease. A retrospective analysis was conducted of all rectal suction biopsies performed in the evaluation of Hirschsprung disease during the previous 12 years that were considered "inadequate." Seventeen cases were identified, and Hirschsprung disease status was determined by clinical or surgical follow-up. Immunohistochemistry for calretinin was performed for all cases containing columnar mucosa, which were evaluated without knowledge of clinical course. All 12 patients without Hirschsprung disease had calretinin-positive nerve fibers in the lamina propria or muscularis mucosae, and all 5 patients with Hirschsprung disease had no calretinin staining of nerves. In this retrospective series, calretinin immunohistochemistry correctly predicted outcome in all instances. Although the gold standard for the diagnosis of Hirschsprung disease in rectal suction biopsies remains the evaluation of ganglion cells in a hematoxylin-eosin staining with sufficient submucosa, calretinin immunohistochemistry is quite helpful in triaging further workup based on clinical suspicion.

Index of Suspicion

* ALT: : alanine aminotransferase AST: : aspartate aminotransferase BUN: : blood urea nitrogen CBC: : complete blood count CNS: : central nervous system CSF: : cerebrospinal fluid CT: : computed tomography ECG: : electrocardiography ED: : emergency department EEG: : electroencephalography ESR: : erythrocyte sedimentation rate GI: : gastrointestinal GU: : genitourinary Hct: : hematocrit Hgb: : hemoglobin MRI: : magnetic resonance imaging WBC: : white blood cell An 11-year-old boy presents to the emergency department with a 1-week history of frequent episodes of nonbloody, nonbilious emesis associated with diarrhea and abdominal cramping. He has urgency and fecal incontinence. He has been afebrile and denies any rashes, joint pain, sick contacts, headaches, or other symptoms. On further questioning, his mother reveals that he has had 2 similar episodes of abdominal distension and diarrhea during the past 6 to 7 years. Between these episodes, she states that he normally has nonbloody, hard stools accompanied by straining and chronic abdominal pain and pain with defecation. When asked specifically, his mother does not remember when he passed his first stool after he was born. On physical examination, his temperature is 37.6°C, pulse is 100 beats per minute, respiratory rate is 16 breaths per minute, blood pressure is 113/62 mm Hg, oxygen saturation is 100% on room air, height is 63 inches (95%), and weight is 36.7 kg (25%). He has abdominal distention and diffuse abdominal tenderness with voluntary guarding. There is palpable stool in the left quadrant. There is no perianal erythema or skin tag, and rectal examination reveals a low anal tone. A CT scan of the abdomen (Figure 1) reveals a markedly distended rectosigmoid colon with circumferential, confluent, and contiguous mural thickening. Basic metabolic profile, CBC, and urinalysis results are unremarkable, but the C-reactive protein level is elevated at 131 mg/L (1248 nmol/L). Because of the concern for enterocolitis, he is empirically given broad-spectrum intravenous antibiotics and transferred to a children’s hospital for further evaluation. Figure 1. A CT scan of the abdomen shows a markedly distended rectosigmoid colon with circumferential mural thickening. A 4-year-old boy presents for evaluation of intermittent fevers of 4 weeks’ duration after 2 separate antibiotic courses prescribed for acute otitis media. He has experienced clear rhinorrhea and …

Intraoperative rapid diagnosis of Hirschsprung's disease

Objective To evaluate the superiorities and disadvantages of reduced nicotinamide adenine dinucleotide dehydrogenase (NADPH-d),acetylcholinesterase (AchE) enzyme histochemistry and hematoxylin and eosin (HE) staining in the intraoperative rapid diagnosis of Hirschprung' s disease (HD),increase the intraoperative diagnostic accuracy and decrease the risks of reoperation.Methods Eighteen children with HD underwent intraoperative full-thickness intestinal biopsies,including 11 boys,and 7 girls,with average age of (8.6 ± 3) months.Frozen sections at three levels (narrow segments,transitional region and dilated segments) were taken,and the extent of aganglionosis was examined by using NADPH-d staining,traditional rapid HE and AchE staining respectively.TUJ-1,a marker of enteric neural cells,was used for identification of NADPH-d positive myenteric plexus and ganglion cells by immunofluorescence staining.Results The positve rate of NADPH-d staining,HE staining and AchE staining for HD was 94.4％ (17/18),83.3％ (15/18),and 88.9％ (16/18),respectively (P ＞ 0.05).Time comsuming in NADPH-d,HE and AchE staining was (7.0 ± 1.7),(23.2 ± 2.9) and (30.9 ± 2.2) min,respectively(P ＜ 0.01).Conclusion The combination of NADPH-d and HE staining,with high idiosyncrasy and sensibility,simple and convenient,can be used as a routine intraoperative diagnosis method to identify HD and detect cutting range of colon.AchE staining was the best technique to demonstrate hypertrophic nerve trunks in the mucosa and submucosa and was well suited to suction rectal biopsy. Key words: Hirschprung's disease ; Intraoperative diagnosis; Pathology ; Colorectal surgery

Mo1628 A New Potential Method Per Anus Endoscopic Myotomy for Treatment of Internal Anal Sphincter Achalasia

A New Potential Method Per Anus Endoscopic Myotomy for Treatment of Internal Anal Sphincter Achalasia Lei Wang*, Qiang Cai, Chaoqiang Fan, Wei Ren, Jin Yu digestive diseases, xinqiao hospital chongqing, Chongqing, China; medicine, division of digestive diseases, Alanta, GA Background: Internal anal sphincter achalasia (IASA) is a condition with presentation similar to Hirschsprung’s disease (HD), but with the presence of ganglion cells on rectal suction biopsy (RSB). The diagnosis is made on anorectal manometry (ARM) by the absence of the rectosphincteric reflex on rectal balloon inflation. Internal sphincter myectomy (ISM) is the treatment of choice for patients with IASA. Per oral endoscopic myotomy(POEM) is new method for treatment of esophageal achalasia in recent years. By this method, the muscle layer of the GI wall can be incised safely in the submucosal tunnel. Because the pathogenesis of IASA is similar to the esophageal achalasia, we hypothesized that the per anal endoscopic myotomy(PAEM) can be used to treat IASA and evaluated the efficacy and safety of PAEM in animal model. Materials And Methods: Five 8-week-old pigs were used for this experiment. Under ketamine sedation, baseline internal anal sphincter (IAS) pressures were recorded and the pigs underwent normal sigmoidoscopy. The mucosal incision was made at approximately 1cm orally to the proximal margin of the Z line to creat mucosal entrance to the submucosal space. A submucosal tunnel was created from approximately 1cm to 10cm above the Z line of anus with triangle knife in the posterior wall. Then the scope was introduced into the submucosal tunnel and advanced toward the now visible fibers of the IASA. The circular muscle layer was incised 7cm to 9cm length by triangle knife in the submucosal tunnel. The incision was closed with the clips. Manometry was repeated on day 5 after the procedure. The two animals were euthanized on day 7 and the other three animals were euthanized on day 14. Results: All the five pigs were observed for 1 week and the three pigs were observed 2 weeks. All the pigs appeared to do well, with no evidence of fever or loss of appetite during observing time. The anal maximum resting pressure (AMRP) significantly decreased from an average of 44.5 cmH2O to an average of 14.3 cmH2O after the myotomy. There were significant differences between before and post PAEM with respect to histologic evaluation of inflammation and muscle defection. The circular layer appeared to be cleanly cut with no significant adhesions. Necropsy revealed no evidence of perianal inflammation. Conclusion: Per anal endoscopic myotomy is feasible, safe, and effective in the short term. It has the potential for being useful in patients with achalasia.

Su1101 Is Calretinin Immunohistochemistry Able to Decrease the Rate of Inconclusive Results of Rectal Suction Biopsies in Hirschsprung's Disease?

Su1101 Is Calretinin Immunohistochemistry Able to Decrease the Rate of Inconclusive Results of Rectal Suction Biopsies in Hirschsprung's Disease?

The prevalence of Hirschsprung disease in premature infants after suction rectal biopsy

BackgroundThe prevalence of Hirschsprung disease (HD) in the premature infant population is not well documented. However, delayed passage of stool is common in premature infants, and suction rectal biopsy (SRB) is often used to evaluate for HD in this population. The use of SRB is unknown. Therefore, we evaluated the role of SRB in premature infants with abnormal stooling patterns. MethodsAfter Institutional Review Board approval, a retrospective study was conducted on all infants having an SRB performed to exclude HD from January 2000 to December 2010. Infants were divided into two groups according to gestational age (premature < 37 wk; term ≥ 37 wk). Demographics, diagnosis, treatments, and outcomes were collected. A subset analysis was performed on patients diagnosed with HD. ResultsTwo hundred sixty-nine infants were identified (113 premature and 156 term). Six premature infants (5.3%) and 79 term infants (50.6%) were found to have HD (P < 0.01). As expected, gestational age was significantly different between groups (31.7 versus 38.9 wk, P < 0.01) (Table 1). Premature infants were less likely to have prenatal care (35% versus 55%, P < 0.01) and had longer lengths of hospital stay (45.6 versus 17.6 d, P < 0.01). The most common location of aganglionosis was rectosigmoid in both groups (group 1, 50%; group 2, 33%, P = 0.7). ConclusionsHD occurs significantly less often in premature infants than in term infants. Alternative diagnoses should be investigated in this population when delayed stooling patterns are encountered. SRB should be used more selectively in this group.