Neonatal Hirschsprung's Disease Research Articles

Transanal pull-through for Hirschsprung disease in neonates: A single-center experience

Background: Transanal pull-through for the treatment of Hirschsprung disease in the neonatal period remains debatable. This study reported our experience with transanal pull-through in neonates. Methods: We reviewed medical records of neonates with Hirschsprung disease treated in our department with transanal pull-through between the years 2010 and 2016. Results: Of the 40 included patients, 31 were male neonates, and 9 were female. The mean age at the time of surgery was 13 days. The mean operative time was 136 minutes. The level of aganglionosis was rectal in 8 cases, rectosigmoid in 19 cases, descending colon in 7 cases, splenic flexure in 3 cases, transverse colon in 2 cases, and ascending colon in one case. A combined abdominal approach was used in 11 patients. There were no intraoperative complications. Major complications were noted in 4 cases: anastomotic leakage in 3 cases of whom one succumbed 3 days postoperatively; and bowel perforation in one case. The most common late postoperative complication was fecal soiling encountered in 25% of cases. Two among 29 patients who reached the age of continence remain incontinent. Conclusion: Transanal pull-through in neonates has similar outcomes and complications to those of infants and children. Therefore this procedure can be safely employed in neonates however we recommend that the treating team should have an ample learning curve for this procedure in infants.

Design and development of an electroanalytical enzymatic sensor using anodized laser induced graphene for the management of Hirschsprung Disease

Hirschsprung's disease (HD) is widely characterized by the absence of ganglion cells in the colon of infants and children resulting in a spectrum of diseases starting from constipation to intestinal obstruction. The disease can have a serious manifestation of Hirschsprung-associated enterocolitis which can lead to significant morbidity and even mortality. Presently the treatment includes surgical excision of the pathological segment of the colon. Identification of the segment requires histopathology and immunohistochemistry which is time-consuming and requires significant expertise of a pathologist. There is a gap in the management of the disease in the form of absence of suitable, sensitive, and efficient technologies to detect the pathological segment of the intestine, which could significantly reduce surgery duration in the operation theatre, as well as associated risks to patients. It, therefore, becomes a matter of extreme importance to develop a point-of-care platform for early and efficient identification of the occurrence of HD in neonates and older children during surgery. The present work reports on the development of an electrochemical enzymatic biosensor using anodized laser-induced graphene (aLIG) conjugated with acetylcholinesterase enzyme (AchE), for detection of HD as a function of acetylcholine – the key biomarker. The developed sensor was initially characterized using cyclic voltammetry (CV) and electrochemical impedance spectroscopy (EIS), while the sensor's analytical performance was assessed using CV. The sensor exhibited a limit of detection of 0.26 μM with a shelf life of 1 month, while remaining unperturbed in the presence of common interferants. The performance of the developed sensor was also examined in spiked serum samples and was observed to yield a high degree of linearity. • First report on the design and development of electroanalytical biosensor for detection/management of Hirschsprung disease. • Anodized laser induced graphene conjugated with acetylcholinesterase (aLIG/AchE) as a robust platform for detecting acetylcholine – the key biomarker. • High degree of linearity with enhanced limit of detection of acetylcholine in spiked serum. • Simple, cost-effective development of highly selective and stable sensor. • Exhibiting huge potential for deployment in intra-operative scenario for identifying pathological segment of the colon.

Enterocolitis Is a Risk Factor for Bowel Perforation in Neonates With Hirschsprung's Disease: A Retrospective Multicenter Study.

Background and AimWe evaluated the clinical features of neonatal Hirschsprung's disease (HD)-associated bowel perforation (perforated HD) and investigated risk factors related to it.MethodsWe retrospectively collected clinical data of neonates (<1 month of age) with perforated HD from multicenters in China from January 2006 to December 2019. A total of 142 patients (6.7%) with perforated HD were enrolled in the study. A 1:2 matching method was used to compare the clinical information of HD patients with and without bowel perforation during the neonatal period. The risk factors for bowel perforation were identified using univariate and multivariate logistic risk regression analyses.ResultsPerforation site was present in the proximal ganglionic bowel in 101 (71.1%) cases and the distal aganglionosis segment in 41 (28.9%) cases. Adjacent marginal tissue from the perforated intestine revealed varying degrees of inflammatory cell infiltration, and the severity of enterocolitis was higher in the proximal ganglionic bowel than in the distal aganglionosis segment (p < 0.05). In the univariable and multivariable logistic analyses, clinical symptoms, such as vomiting (adjusted OR = 2.06, 95% CI: 2.01–2.88, p < 0.05), and inflammation index in hematologic tests, such as neutrophil proportion (adjusted OR = 1.09, 95% CI: 1.05–1.33, p < 0.05) and CRP (adjusted OR = 2.13, 95% CI: 1.01–3.27, p < 0.05) were associated with increased risk for perforated HD.ConclusionClinical Hirschsprung disease-associated enterocolitis (HAEC) highly correlated with perforated HD. Timely treatment of HAEC should be appropriate therapeutic approaches to prevent perforated HD.

Autopsy Study of Calretinin Immunohistochemistry in the Anorectal Canal in Young Infants and Potential Implications for Rectal Biopsy Approach in the Neonatal Period.

Absent submucosal ganglion cells in biopsies 1-3 cm above the pectinate line establishes the pathologic diagnosis of Hirschsprung Disease (HD). Calretinin stains both ganglion cells and their mucosal neurites and has gained importance in HD diagnosis. Absent calretinin positive mucosal neurites in biopsies at the appropriate level above the pectinate line is highly specific for HD. Whether this applies to lower biopsies is uncertain. To address this, we studied anorectal canal autopsy specimens from infants. We performed an autopsy study of infant anorectal canal specimens to describe calretinin staining in this region. Calretinin staining was correlated with histologic and gross landmarks. In all 15 non-HD specimens, calretinin positive mucosal neurites were present in glandular mucosa up to the anorectal line where neurites rapidly diminished. Age range was preterm 26 weeks to 3 months. Calretinin positive mucosal neurites are present in glandular mucosa up to the anorectal line in young infants. This is potentially important regarding neonatal HD biopsy level and diagnosis. Positive calretinin staining at the anorectal line favors normal innervation making HD unlikely. Absent calretinin positive neurites in glandular mucosa is worrisome for HD in young infants, regardless of location.

Machine learning-based quantitative analysis of barium enema and clinical features for early diagnosis of short-segment Hirschsprung disease in neonate

ObjectiveTo develop a mathematical model based on a combination of clinical and radiologic features (barium enema) for early diagnosis of short-segment Hirschsprung disease (SHSCR) in neonate. MethodsThe analysis included 54 neonates with biopsy-confirmed SHSCR (the cases) and 59 neonates undergoing barium enema for abdominal symptoms but no Hirschsprung disease (the control). Colon shape features extracted from barium enema images and clinical features were used to develop diagnostic models using support vector machine (SVM) and L2-regularized logistic regression (LR). The training cohort included 32 cases and 37 controls; testing cohort consisted 22 cases and 22 controls. Results were compared to interpretation by 2 radiologists. ResultsIn the analysis by radiologists, 87 out of 113 cases were correctly classified. Six SHSCR cases were mis-classified into the non-HSCR group. In the remaining 20 cases, radiologists were unable to make a decision. Both the SVM and LR classifiers contained five clinical features and four shape features. The performance of the two classifiers was similar. The best model had 86.36% accuracy, 81.82% sensitivity, and 90.91% specificity. The AUC was 0.9132 for the best-performing SVM classifier and 0.9318 for the best-performing LR classifier. ConclusionA combination of clinical features and colon shape features extracted from barium enemas can be used to improve early diagnosis of SHSCR in neonate.

Endoscopic Decompression with a Transanal Drainage Tube for the Treatment of Hirschsprung's Disease in Newborns

Background: To evaluate the effectiveness and safety of endoscopic decompression with a transanal drainage tube (ED-TDT) in the treatment of neonatal Hirschsprung's disease (HD). Materials and Methods: Six newborns (4 boys and 2 girls; age at operation 9-29 days, median 24 days) were admitted to our hospital due to the delayed passage of meconium after birth, abdominal distension, and vomiting. HD was diagnosed by the retrograde enema radiography. All patients underwent ED-TDT for decompression of the dilated colon. During the operation, an endoscope was inserted through the anus to the ascending colon or ileocecal region, which was then used to insert a guidewire to the ascending colon. The endoscope was then removed, and a colorectal drainage tube was introduced over the guidewire. This drainage was placed until a pull-through procedure was performed. Results: The ED-TDT was successfully performed in all newborns. The intraoperative rectal biopsy confirmed the diagnosis of HD. The duration of the operation was 25-70 minutes (median 52.5 minutes). There were no perforations, bleeding, or other complications after surgery. After surgery, the abdominal distention was significantly relieved, and the babies could be fed with milk after 6 hours. These babies were discharged after 2-11 days (median 2.5 days) and underwent a pull-through procedure after 1-3 months (median 3 months). Conclusions: ED-TDT is a safe and effective treatment for neonatal HD before a pull-through procedure. It could be used as an alternative therapy to colostomy.

Uncommon and Rare Pediatric Syndromes Associated with Surgical Conditions in Iraqi Children.

Abstract Background: Many uncommon and rare pediatric clinical syndromes including genetic and dysmorphic disorders are known to be associated with surgical conditions and require various types of surgical interventions. Little is known about the pattern of surgical conditions associated with the uncommon and rare childhood syndromes in Iraq. The aim of this paper to describe our experience with uncommon and rare childhood syndromes associated with surgical conditions observed in two tertiary referral centers in Baghdad. Patients and methods: Twenty one unrelated patients (10 males and 11 females) with uncommon and rare syndromes associated with surgical conditions were observed. The patient’s age ranged from 3 days to 18 years. The patients were observed during the period from 1994 to December 2019 in two tertiary referral teaching hospitals (University Hospital in Al-Kadhimiyia which was called later “Al-Kadhimiyia Teaching Hospital” and the Children Teaching Hospital of Baghdad Medical City). Results: The twenty one patients had seventeen uncommon and rare syndromes associated with surgical conditions including ocular conditions (Cataracts and glaucoma), congenital heart disease, gastrointestinal surgical conditions (Imperforated anus, congenital primary cricopharyngeal achalasia, omphalocele and neonatal Hirschsprung disease), urologic surgical condition (Undescended testes) and orthopedic surgical conditions. (Hip dislocation and multiple fractures), gynecologic ocular conditions (Hematocolpos which is caused by hemivaginal obstruction) and surgical conditions needing plastic surgeries. Conclusion: This unique series of pediatric syndromes associated with surgical conditions included a new Iraqi variant of oculocerebrorenal syndrome, the cases number 35 and 36 of cutis laxa type II (Debre type) in the literature, the case number 52 of Ruprecht Majewski-Bosma syndrome in literature, the 58th case of Toriello-Carey syndrome in the literature, the case number 130 of Townes Brocks syndrome, the twenty eighth case of congenital Chevalier Jackson in the literature, the thirty fourth case of Goldberg Shprintzen syndrome and the fourth patient in the literature with the syndrome of microtia/anotia syndrome and congenital facial palsy without other abnormalities.

The Clinical Impact of Delayed Management of Hirschsprungs Disease

Background: Hirschsprung's disease (HD) is a developmental disorder of the enteric nervous system that is characterized by the absence of ganglion cells in the myenteric and submucosal plexus from the anorectum for a variable colonic or intestinal distance. HD is a congenital bowel motility disorder which occurs in approximately one of every 5000 live births. This results in a lack of peristalsis leading to a huge dilatation of the colon proximal to the aganglionic bowel. Aim of work: The purpose of the present study is to investigate the clinical impact of delayed management of children with HD. and how it would affect their outcomes? Methods: The present study was performed in the period from July 2018 to august 2019 in Pediatric Surgery Department of Zagazig University Hospital. 18 patients enrolled inprospective clinical study Results: The delayed diagnosis of HD is associated with a different pattern of presentation compared with neonatal HD. Most of the cases were presented by chronic constipation, abdominal distension and bilious vomiting. Moreover, outcome is worse in older children who are diagnosed with this disease. Conclusions: HD in older children associated with long hospital stay, increased cost of treatment compared with the younger children. Postoperative outcome in older children associated not only with increased risk of serious morbidities but also indirectly affect their long-term outcome.

Single-stage transanal endorectal pull-through procedure for correction of Hirschsprung disease in neonates and nonneonates: A multicenter study

PurposeThe outcomes of single-stage transanal endorectal pull-through (SSTEPT) for Hirschsprung disease (HSCR) in young patients are favorable; however, reports have shown that diagnosis and surgery at young ages increase the risk for postoperative enterocolitis and slows postoperative recovery. The present study was primarily designed to evaluate the outcomes of SSTEPT in a multi-institutional cohort of neonates and nonneonates with HSCR. MethodsBetween August 2005 and May 2012, a total of 650 children with HSCR were divided into the following two groups: group A (neonatal group, operative age<28days [n=186]); and group B (nonneonatal group, operative age>28days [n=464]). The short-term outcomes were postoperative enterocolitis, perianal excoriation, and anastomotic stricture and leakage rates. The midterm outcomes were incomplete continence and constipation rates based on multi-institutional chart review. Statistical analyses were performed using chi-square (χ2) tests. ResultsFollow-up was completed in 112 neonates and 303 nonneonates. Short-term outcomes indicated a higher incidence of perianal excoriation (27.6% vs. 6.6%, χ2=33.70, p<0.05), anastomotic strictures (14.3% vs. 6.0%, χ2=27.18, p<0.05), anastomotic leakage (8.0% vs. 1.7%, χ2=8.36, p<0.05), and postoperative enterocolitis (40.2% vs. 10.2%, χ2=49.05, p<0.05) in group A compared to group B. Midterm outcomes indicated a higher incidence of incomplete continence (35.7% vs. 14.9%, χ2=21.85, p<0.05) in group A compared to group B. ConclusionPerforming single-stage transanal endorectal pull-through in the nonneonatal period may be more appropriate than the neonatal period. There were higher rates of perianal excoriation, anastomotic strictures and leakage, postoperative enterocolitis, and incomplete continence postoperatively in neonates than nonneonates.

The utility of the contrast enema in neonates with suspected Hirschsprung disease

Background/purposeThe contrast enema (CE) is commonly utilized for suspected Hirschsprung disease (HD) patients. We set out to determine the utility of the CE in the newborn for clinically suspicious HD. MethodsAll CEs performed for suspicion of HD in neonates from January 2004 to December 2013 were reviewed by two pediatric radiologists who were blinded to the original interpretations and final diagnoses. A standardized scoring sheet was utilized to document essential radiographic findings. Definitive diagnoses were determined by pathology. Descriptive statistics, likelihood ratios, and interrater agreement were determined. Results158 CEs were reviewed. Interrater agreement was 89% with kappa (95% CI) of 0.63 (0.47–0.76). Common indications for CE were similar between non-HD and HD groups. The positive, inconclusive, and negative likelihood ratios (95% CI) were 38 (10–172), 3.2 (1.3–9.1), and 0.15 (0.06–0.47), respectively, leading to posttest probabilities for positive, inconclusive, and negative tests of 83%, 32%, and 2.5%, respectively. ConclusionsAlthough radiographic positive CE for HD portends a high probability of HD, inconclusive studies still represent a significant increased risk. In clinically suspicious infants for HD, those with inconclusive studies may benefit from a lower threshold to perform follow-up rectal biopsy.

Clinical outcome of transanal endorectal pull-through for neonatal hirschsprung's disease

Objective To analyze the diagnosis and treatment of neonatal Hirschsprung's disease (HD)and to evaluate the outcome of one-stage transanal endorectal pull-through in the treatment of neonatal HD.Methods The records of 48 newborns with HD from July 2004 to July 2013 were reviewed retrospectively.Base data,details of management,complications,and outcomes were examined.Results Totally 48 consecutive newborns with HD were identified,of which 32 were classical type,9 were long-segment type and 7 were short-segment type.All were successfully treated by one-stage transanal endorectal pull-through procedure,of which 39 were transanal only,7 were laparoscopic assisted,2 were open.The average preoperative preparation time was (7 ± 2) d.The operation time was (88 ± 15) min.The average intra-operative blood loss was (7 ± 2) ml.The average length of resected bowel was (18 ± 6) cm.The mean hospital stay was (10± 2) d.The follow-up period for all patients was in the range of 3 months to 8 years,and all grew well.Complications included anastomotic stricture (9 cases),constipation (8 cases),incontinence (8 cases),enterocolitis (10 cases),et al.All of them got better at last after treatment.Barium enema showed that the shape of colon had become normal.The score of bowel movement assessment was 4 for 38 cases,3 for 7 cases and 2 for 1 case.Conclusion It is suggested that one-stage transanal endorectal pull-through procedure for neonatal HD was safe,for its less complication good outcome and long term effect,which is worthy of further promotion. Key words: Neonatal Hirschsprung's disease; Operation; Treatment

Preliminary evaluation of anorectal manometry in diagnosing Hirschsprung’s disease in neonates

The aim of this paper was to assess the clinical value of anorectal manometry (ARMM) in the diagnosing of Hirschsprung's disease (HD) in neonates. From January 2003 to June 2005, 75 patients in whom HD was clinically suspected were analyzed. ARMM was performed using a desk, high rate gastrointestinal dynamic detection system and the results were compared with barium enema and rectal suction biopsy. Based on rectal suction biopsies in 52 of 75 patients, the positive, false positive, negative, and false negative rates of ARMM in the diagnosis of HD in neonates were found to be 92.3, 1.9, 1.9, and 3.8%, respectively. Forty-three of 75 patients were diagnosed with HD by both ARMM and barium enema and the diagnoses were validated by pathologic results. The diagnosis of HD was excluded in 18 patients in whom HD was clinically suspected, but in whom the results of ARMM and barium enema were normal. Twelve patients who had ARMM results consistent with HD and a negative barium enema, had serial ARMM performed; a rectoanal inhibitory reflex (RAIR) was elicited in four patients, thereby excluding HD and the remaining eight patients were diagnosed with HD by review of barium enema and pathologic results. One of two patients with a positive barium enema for HD, but an ARMM showing the presence of RAIR was excluded by pathologic results and the other patient was lost to follow-up. The diagnostic accuracies of ARMM and barium enema for HD in neonates were 93.3 and 86.7%, respectively. There was no difference in rectal resting pressure and anal rhythmic wave frequency between neonates with HD and healthy neonates, but neonates with HD had higher anal sphincter pressures than healthy neonates (P=0.0074). ARMM is a simple, safe, and non-invasive method with high specificity for the diagnosis of HD in neonates.

Comparison of two techniques for single-stage treatment of Hirschsprung disease in neonates

Introduction: The aim of this study is to determine the incidence, morbidity and functional outcome of two techniques for Hirschsprung disease (single-stage Duhamel pull-through and tans-anal pull-through) in the neonatal age group. Method: All the neonates with Hirschsprung disease with short aganglionic segment (transition zone at recto-sigmoid junction) admitted during the last 6 years were included. A total of 44 cases were included in the study of which 24 were treated by Duhamel pullthrough (group A) and 20 were treated with trans-anal pull-through (TEPT, group B). The time of operation, acceptance of first oral feed, duration of hospital stay, complications and anal continence were evaluated for both procedures. Result: There was no difference in age and sex and length of the aganglionic segment of bowel. The average operative time was 140-180 min. in group A and 120-170 min. in group B. Per-oral feed was started on the 5-7th day in group A and on the 5-6th day in group B. Hospital stay was longer in group A whereas complications were more common in group B and were found to be statistically significant. Anal continence was almost similar in both groups. Conclusion: Single-stage Duhamel pull-through operation is better than trans-anal pull-through operation in long-term follow-up although duration of hospital stay was shorter in TEPT.

Anorectal sleeve micromanometry for the diagnosis of Hirschsprung's disease in newborns

An accurate diagnosis is mandatory for surgery in newborns with Hirschsprung's disease (HD). Acetylcholinesterase staining of rectal suction biopsy specimens is widely performed in the diagnosis of HD, but results are sometimes incorrect or atypical in newborns. We report the usefulness of our method of anorectal manometry using a specially designed sleeve microassembly for the diagnosis of neonatal HD. Anorectal manometry was conducted without sedation in 41 newborns, aged 2 to 30 days (19 newborns were within the first week of life), with abdominal distension. A silastic assembly with a 2-cm-long sleeve sensor and 5 side holes arrayed along the sleeve was designed to reduce the effects of displacement of pressure sensors relative to the anal sphincter. Rectoanal inhibitory reflex (RAIR) was examined with rectal balloon distension. Thirty-two subjects who showed falls of anal sphincter pressure fulfilling the criteria for RAIR were diagnosed to be without HD. Nine patients without an appropriate RAIR were subsequently confirmed to have HD based on operative pathologic findings. Parameters of anal sphincter function did not differ significantly between the subjects with and without RAIR. An anorectal sleeve micromanometric technique is useful in the diagnostic workup of newborns suspected of having HD.

Difficulties in making the diagnosis of Hirschsprung disease in early infancy

This study highlights the less common presentations of Hirschsprung disease (HD) and HD-associated enterocolitis (HAE) in neonates and infants. We present three infants whose diagnosis was delayed because of atypical presenting features, especially with failure to gain weight, decreased appetite, episodes of diarrhoea and vomiting and hypoalbuminaemia. The reported incidence of HAE ranges from 0 to 16.2%. To avoid complications of life-threatening HAE, primary care physicians require a high index of suspicion of the more unusual presentations of HD in neonates and infants.

Benign transient non-organic ileus of neonates.

There are a substantial number of neonates who present with Hirschsprung's disease-like symptoms, but respond very well to conservative therapy. However, once Hirschsprung's disease is ruled out, little attention is paid to these infants, because of the lack of necessity for surgical treatment and their excellent prognosis. The purpose of this study was to elucidate the clinical features of functional ileus of neonates, which we named benign transient non-organic ileus of neonates (BTNIN). Out of 61 neonates referred to our institution with suspected neonatal Hirschsprung's disease (NH), 10 were diagnosed as having NH and 51 as having BTNIN. All the cases of BTNIN showed marked abdominal distension, and 12 showed explosive defecation on digital examination at the first visit. Plain X-ray demonstrated marked whole intestinal dilatation in 12 cases including cases with niveau formation and segmental dilatation. These findings were indistinguishable from those of NH. However, all had a normal anorectal reflex, and rectal suction biopsy revealed normal acetylcholinesterase activity and submucosal ganglion cells. All the cases of BTNIN were treated with periodic glycerin enemas until daily spontaneous defecation was established, which took 2 to 14 months, with an average of 5.0 +/- 2.9 months. None of them showed residual symptoms during the follow-up period.

Fourteen-year experience of acetylcholinesterase staining for rectal mucosal biopsy in neonatal Hirschsprung's disease

Background/Purpose: Acetylcholinesterase (AChE) staining of rectal mucosal biopsy specimens is the most important and popular examination for making a definite diagnosis of Hirschsprung's disease. This examination often is performed for patients with constipation in the daily clinic. The results of this examination are reflected immediately in the treatment. However, the authors sometimes encountered difficult cases to diagnose, especially in neonates. Therefore, a retrospective investigation was conducted on the benefits and problems of AChE staining of rectal mucosal biopsy specimens in neonates. Methods: The authors encountered 459 cases (91 neonates) of suspected Hirschsprung's disease, clinically, from April 1986 to March 2000. Mucosal specimens were taken by punch biopsies. Samples were stained by the modified Karnovsky Roots method using rubeanic acid as an amplifier and immediately examined with a light microscope. These results were collected and assessed mainly on neonatal cases. The authors also analyzed the 104 cases of Hirschsprung's disease diagnosed in patients less than 1 year of age to evaluate the relationship between the grade of proliferation of AChE positive fiber and age. Results: Forty-one neonatal cases of Hirschsprung's disease were diagnosed based on the findings of AChE staining. A definite diagnosis of Hirschsprung's disease was confirmed based on the pathologic findings of operative samples. Forty-eight cases that were diagnosed as normal included 4 cases that turned out to be false-negative (3 Hirschspurung's disease cases and 1 case of an allied disorder of Hirschsprung's disease). There were no major complications in mucosal punch biopsy. In the cases of Hirschsprung's disease diagnosed in a patient less than 1 year of age, the grade of AChE-positive fiber tended to increase with the aging of patients. Conclusions: The specificity of AChE staining was high (100%), but its sensitivity was slightly low (91%). Careful long-term follow-up is required for any cases diagnosed as normal. Mucosal biopsies should be repeated in cases of persistent clinical symptoms. J Pediatr Surg 36:1357-1363. Copyright © 2001 by W.B. Saunders Company.

Eighteen year's experience with neonatal Hirschsprung's disease treated by endorectal pull-through without colostomy

Methods : In the past 18 years, the authors have treated 84 patients with Hirschsprung's disease. Of these, 43 patients were under 1 month of age and underwent endorectal pull-through without colostomy. Some have undergone follow-up for as long as 18 years. Results : Thirty-four of these 43 (79%) newborn patients were available for follow-up. Twenty-two were totally continent. The remaining 12 have normal sphincter tone. Of the 41 patients above 1 month of age, 34 (83%) were available for follow-up. Some have undergone follow-up for as long as 18 years. Conclusion : Twenty-two of this latter group (79%) have normal bowel control.

Hirschsprung's disease: One surgeon's experience in one institution

The clinical experience of 137 patients treated by one pediatric surgeon at HanYang University Hospital over 12 years was analyzed. The sex ratio was 3.6:1 with male predominance. Neonatal Hirschsprung's disease (HD) occurred in 70 cases (51.1%). One hundred fourteen cases (83.2%) were short-segment and 23 cases (16.8%) were long-segment disease, of which 4 cases were total colon aganglionosis. Symptoms were abdominal distension, vomiting, constipation, and delayed passage of meconium. Family history was noted in 4 cases: 3 involved siblings and 1 involved relatives. All patients were male and had short-segment diseases. There were 3 pairs of monozygotic twins discordant for HD. Seventy-seven two-stage Duhamel operations were performed at the age of 121 ± 41.3 days. Primary Duhamel operations were performed on 30 children. Anorectal myectomies were performed on 12 cases of short rectal aganglionosis. The incidence of enterocolitis was 25.5% preoperatively and 10.1% postoperatively. Complications after the Duhamel operation were intestinal obstruction (3), wound disruption (4), and septum reformation (3) with fecaloma formation in the blind rectal pouch. Bowel function was normalized within 3 months in 77.3% and within 1 year after operation in 91.6%. Duhamel operation with a GIA stapler after preliminary colostomy could be done at the age of 104 days on average with good functional results and with acceptable morbidity. Primary Duhamel operation could be done safely on the patients whose conditions were diagnosed after 90 days of life with good results. There was no mortality after Duhamel operation.

Management of Hirschsprung's disease ? an alternate approach

Management of neonatal Hirschsprung's disease based on diagnosis by histologic techniques and frozen acceptable method. However, the non-availability of histologic and histochemical techniques often necessitates alternate approaches. In 376 infants below the age of 2 months who were admitted with the presumptive diagnosis of Hirschsprung's disease, a barium enema was the key investigation. Histologic confirmation could be obtained after a colostomy was established. Manometry allowed the detection of any diagnostic errors. The overall error rate was approximately 4%. An alternate algorithm is suggested for the management of Hirschsprung's Disease, particularly in less well-developed centers.