Reducing Underdiagnosis of Hirschsprung-Associated Enterocolitis: A Novel Scoring System.

BACKGROUND: Hirschsprung-Associated Enterocolitis (HAEC) is a life-threatening and difficult to diagnose complication of Hirschsprung Disease (HSCR). The goal of this study was to evaluate existing HAEC scoring systems and develop a new scoring system. METHODS: Retrospective, multi-institutional data collection was performed. For each patient, all encounters were analyzed. Data included demographics, symptomatology, laboratory and radiographic findings, and treatments received. A "true" diagnosis of HAEC was defined as receipt of treatment with rectal irrigations, antibiotics, and bowel rest. The Pastor and Frykman scoring systems were evaluated for sensitivity/specificity and univariate and multivariate logistic regression performed to create a new scoring system. RESULTS: Four centers worldwide provided data on 200 patients with 1450 encounters and 369 HAEC episodes. Fifty-seven percent of patients experienced one or more episodes of HAEC. Long-segment colonic disease was associated with a higher risk of HAEC on univariate analysis (OR 1.92, 95% CI 1.43-2.57). Six variables were significantly associated with HAEC on multivariate analysis. Using published diagnostic cutoffs, sensitivity/specificity for existing systems were found to be 38.2%/96% for Pastor's and 56.4%/86.9% for Frykman's score. A new scoring system with a sensitivity/specificity of 67.8%/87.9% was created by stepwise multivariate analysis. The new score outperformed the existing scores by decreasing underdiagnosis in this patient cohort. CONCLUSIONS: Existing scoring systems perform poorly in identifying episodes of HAEC, resulting in significant underdiagnosis. The proposed scoring system may be better at identifying those underdiagnosed in the clinical setting. Head-to-head comparison of HAEC scoring systems using prospective data collection may be beneficial to achieve standardization in the field.

Evaluation and Management of Persistent Problems After Surgery for Hirschsprung Disease in a Child.

PURPOSE OF REVIEW: Ideally, after operative intervention, a child born with Hirschsprung disease (HD) should thrive, achieve fecal continence, and avoid recurrent episodes of abdominal distention and enterocolitis. However, a significant number of patients continue to struggle following their pull-through procedure. The purpose of this review is to present an organized and practical approach to the evaluation and management of the symptomatic patient post pull-through operation for HD. RECENT FINDINGS: Children diagnosed with HD who are not doing well after their initial operation can be categorized in three distinct groups: (1) those that have fecal incontinence, (2) those with obstructive symptoms, and (3) those with recurrent episodes of enterocolitis. It is important to have a systematic diagnostic approach for these patients based on a comprehensive protocol. All three of these patient groups can be treated with a combination of either medical management, reoperation when a specific anatomic or pathologic etiology is identified, or botulinum toxin for non-relaxing sphincters contributing to the obstructive symptoms or recurrent enterocolitis. For patients not doing well after their initial pull-through, a systematic workup should be employed to determine the etiology. Once identified, a multidisciplinary and organized approach to management of the symptomatic patients can alleviate most post pull-through symptoms.

Perinatal Outcome in Fetuses with Dislodged Thoraco-Amniotic Shunts.

OBJECTIVE: Fetal thoraco-amniotic shunts (TASs) can dislodge in utero, migrating internally into the fetal thorax or externally into the amniotic cavity. Our objective was to evaluate the perinatal and long-term outcome of fetuses with TAS dislodgement and conduct a review of the literature. METHODS: This is a retrospective review of all TAS inserted for primary pleural effusions and macrocystic congenital pulmonary airway malformations (CPAMs) in a tertiary fetal medicine center (1991-2020). Antenatal history, procedural factors, and perinatal and long-term outcomes were reviewed in all fetuses with dislodged shunts and compared to fetuses with shunts that did not dislodge. RESULTS: Of 211 TAS inserted at a mean gestational age of 27.8 weeks ± 5.47 (17.4-38.1 weeks), 187 (89%) were inserted for pleural effusions and 24 (11%) for macrocystic CPAMs. Shunts dislodged in 18 fetuses (8.5%), 17 (94%) of which were for pleural effusions. Shunts migrated into the chest wall/amniotic cavity or into the thorax among 7/18 (39%) and 11/18 (61%) fetuses, respectively. Eleven (61%) fetuses were initially hydropic, which resolved in 8 (72%) cases. Effusions were bilateral in 9 (50%), amnioreduction was required in 6 (33%), and fetal rotation in 8 cases (44%). Four (22%) fetuses underwent repeat shunting, 12 (67%) neonates required ventilatory support, and 2 (11%) neonates required chest tubes. There was no significant difference in technical factors or outcomes between infants with shunts that dislodged and those that did not. Among 11 intrathoracic shunts, 2 (18%) were removed postnatally and the remainder are in situ without any shunt-related or respiratory complications over a follow-up period of 9 months to 22 years. CONCLUSION: TAS dislodged antenatally in 8.5% of fetuses, with 2/3 of shunts migrating into the thorax, and nearly 25% requiring re-shunting. Retained intrathoracic shunts were well tolerated and may not necessarily require surgical removal after birth.

Use of Tissue Plasminogen Activator in Abdominal Abscesses in Children-A Single-Center Randomized Control Trial.

PURPOSE: To establish the efficacy of once-per-day intracavitary tissue plasminogen activator (tPA) in the treatment of pediatric intra-abdominal abscesses. METHODS: A single-center prospective, double-blinded, randomized controlled trial of the use of intracavitary tPA in abdominal abscesses in children. Patients were randomized to either tPA-treatment or saline-treatment groups. Primary outcome was drainage catheter dwell (hours). Secondary outcomes were length of hospital stay, times to discharge, clinical and sonographic resolution, and adverse events (AEs). RESULTS: Twenty-eight children were randomized to either group (n = 14 each). Demographics between groups were not significantly different (age P = .28; weight P = .40; gender P = .44). There were significantly more abscesses in the tPA-treated group (P = .03). Abscesses were secondary to perforated appendicitis (n = 25) or postappendectomy (n = 3). Thirty-four abscesses were drained, 4 aspirated, 3 neither drained/aspirated. There was no significant difference in number of drains (P = .14), drain size (P = .19), primary outcome (P = .077), or secondary outcomes found. No procedural or intervention drug-related AEs occurred. No patient in the saline-treated group required to be switched/treated with tPA. CONCLUSION: No significant difference in the length of catheter dwell time, procedure time to discharge, or time to resolution was found. Intracavitary tPA was not associated with morbidity or mortality. The results neither support nor negate routine use of tPA in the drainage of intra-abdominal abscess in children. It is possible that a multicentre study with a larger number of patients may answer this question more definitively.

Genotype-phenotype correlation in children with hereditary spherocytosis.

Hereditary spherocytosis (HS) is a common inherited haemolytic anaemia attributed to disturbances in five different red cell membrane proteins. We performed a retrospective study of 166 children with HS and describe the clinical phenotype according to the genotype. In 160/166 (97%) children with HS a disease-causing mutation was identified. Pathogenic variants in ANK1, SPTB, SLC4A1 and SPTA1 were found in 49%, 33%, 13% and 5% of patients. Children with SLC4A1-HS had the mildest phenotype, showing the highest haemoglobin (P < 0·001), lowest reticulocyte counts (P < 0·001) and lowest unconjugated bilirubin levels (P = 0·006), and none required splenectomy in childhood (P < 0·001). Conversely, children with autosomal recessive SPTA1-HS had the most severe clinical phenotype, with almost all patients undergoing splenectomy in early childhood. Patients with ANK1 and SPTB variants showed a similar clinical phenotype. Within each gene, variant type or location did not predict disease severity or likelihood of splenectomy. Among patients with a genetic diagnosis, 47 (29%) underwent splenectomy (23 partial; 24 total) while 57 (36%) underwent cholecystectomy. Total splenectomy led to greater improvements in haemoglobin (P = 0·02). Select use of genetic testing (especially in patients without a family history) may help predict clinical phenotype in childhood and guide family counselling.

Fetal Sclerotherapy for Hydropic Congenital Cystic Adenomatoid Malformations of the Lung Refractory to Steroids: A Case Report and Review of the Literature.

Microcystic congenital cystic adenomatoid malformations (CCAM), when associated with hydrops, carry a dismal prognosis. Options for treatment are limited and experimental, including antenatal corticosteroids, open fetal surgery, laser ablation and, more recently, sclerotherapy. We describe a case of a large, predominantly microcystic CCAM in a hydropic fetus treated successfully with direct interstitial injection of a sclerosant agent (3% sodium tetradecyl sulfate) at 23+3 weeks gestation, after multiple failed courses of steroids. Elective thoracoscopic right lower lobectomy was performed at 1 year of life and there have been no respiratory or other medical morbidities since. A literature review of fetal lung masses treated with sclerosants antenatally reveals that sclerotherapy may represent a novel treatment option for large hydropic microcystic CCAMs, which are unresponsive to corticosteroids. Further studies are required to evaluate the utility and safety of fetal sclerotherapy, as this may represent an alternative minimally invasive treatment option to fetal lobectomy.

Variability among Canadian pediatric surgeons and pediatric urologists in the management of cryptorchidism in boys before the publication of major guidelines: a retrospective review of a single tertiary centre

Background: Before 2014, there was a lack of recommendations on managing cryptorchidism, or undescended testis (UDT), from a large pediatric urological or surgical organization. We assessed the variability in management of UDT among pediatric urologists and pediatric surgeons at a single tertiary pediatric referral centre before publication of major guidelines. Methods: We performed a retrospective review of the electronic records of patients who underwent primary unilateral or bilateral orchidopexy at our centre between January 2012 and January 2014. Results: A total of 488 patients (616 testes) were identified, of whom 405 (83.0%) and 83 (17.0%) were managed by pediatric urologists and pediatric surgeons, respectively. There was no difference in baseline characteristics, including age seen in clinic or at surgery, testis location/palpability and availability of preoperative ultrasonograms, of patients seen by the 2 groups. Pediatric surgeons ordered preoperative ultrasonography more often than pediatric urologists (25.3% v. 3.7%, p < 0.001). With palpable UDTs, although both groups used open approaches, pediatric urologists preferred a scrotal approach (56.9%), and pediatric surgeons approached most testes inguinally (98.8%). With nonpalpable UDTs, laparoscopic approaches were preferred by both groups; however, pediatric urologists used a 2-stage Fowler­Stephens approach more often than pediatric surgeons (48.4% v. 15.8%, p < 0.001). Conclusion: There was wide variation in the management of primary UDT between pediatric urologists and pediatric surgeons before the publication of guidelines. The most prominent difference between the 2 groups was in the ordering of preoperative ultrasonography. Future assessment of change in practice patterns may elucidate whether guidelines are an effective tool for standardization of practice.

Contexte: Avant 2014, on ne disposait pas de recommandations émanant d'une grande organisation urologique ou chirurgicale pédiatrique pour la prise en charge de la cryptorchidie (absence d'un ou des deux testicules dans le scrotum). Nous avons évalué les divers types de prise en charge de la cryptorchidie chez les urologues et les chirurgiens pédiatriques dans un seul centre tertiaire de référence pédiatrique avant la publication de lignes directrices majeures. Méthodes: Nous avons procédé à une revue rétrospective des dossiers électroniques de patients ayant subi une orchidopexie unilatérale ou bilatérale primaire dans notre centre entre janvier 2012 et janvier 2014. Résultats: En tout, 488 patients (616 testicules) ont été identifiés, dont 405 (83,0 %) et 83 (17,0 %) ont été traités respectivement par des urologues et des chirurgiens pédiatriques. On n'a noté aucune différence quant aux caractéristiques de départ des patients vus par les 2 groupes, telles que l'âge lors de la consultation à la clinique ou lors de la chirurgie, la localisation/palpabilité des testicules et le recours à l'échographie préopératoire. Les chirurgiens pédiatriques ont demandé une échographie préopératoire plus souvent que les urologues pédiatriques (25,3 % c. 3,7 %, p < 0,001). En présence de cryptorchidie palpable, même si les 2 groupes ont utilisé une approche ouverte, les urologues pédiatriques ont préféré l'approche scrotale (55,4 %) et les chirurgiens pédiatriques l'approche inguinale (98,8 %). En présence de cryptorchidie non palpable, les approches laparoscopiques ont été privilégiées par les 2 groupes; toutefois, les urologues pédiatriques ont utilisé une approche Fowler­Stephens en 2 temps plus souvent que les chirurgiens pédiatriques (48,4 % c. 15,8 %, p < 0,001). Conclusion: On a noté une grande variation dans la prise en charge de la cryptorchidie primaire entre les urologues et les chirurgiens pédiatriques avant la publication des lignes directrices. La principale différence entre les 2 groupes concernait le recours à l'échographie préopératoire. L'évaluation future des changements affectant la pratique permettrait de déterminer si les lignes directrices sont un outil efficace pour sa standardisation.

Can MR enterography screen for perianal disease in pediatric inflammatory bowel disease?

BACKGROUND: Pediatric Crohn's disease is associated with perianal disease (PAD). Magnetic resonance enterography (MRE) assesses small bowel involvement in pediatric inflammatory bowel disease (PIBD). Pelvic MRI (P-MRI) is the gold standard for assessing PAD. PURPOSE: To determine if MRE can accurately detect PAD in PIBD, distinguishing perianal fistulae (PAF) from perianal abscesses (PAA), referenced against P-MRI. STUDY TYPE: Retrospective. POPULATION: Seventy-seven PIBD patients, 27 females (mean age 14.1 years), with P-MRI and MRE within 6 months. FIELD STRENGTH/SEQUENCE: 1.5T and 3T; P-MRI: sagittal fat suppressed (FS) T2 fast spin-echo (FSE), coronal short tau inversion recovery, axial T1 FSE, coronal and axial postcontrast FS T1 FSE; MRE: coronal balanced steady-state free-precession (SSFP), coronal cine SSFP, coronal and axial single-shot T2 FS, axial SSFP, coronal ultrafast 3D T1 -weighted gradient echo FS (3D T1 GE), axial diffusion-weighted imaging, coronal and axial postcontrast 3D T1 GE FS. ASSESSMENT: Two radiologists independently, then by consensus, assessed randomized MRI exams, recording PAF number, location, and length; and PAA number, location, length, and volume. Sensitivity analysis used clinical disease as the gold standard, calculated separately for P-MRI and MRE. STATISTICAL TESTS: Comparing MRE and P-MRI consensus data, sensitivity, specificity, positive, and negative predictive values (P/NPV) were calculated. Inter- and intrareader reliability were assessed using kappa statistics. RESULTS: P-MRI and MRE were paired, detecting PAD in 73 patients, PAF in 63, and PAA in 31 P-MRI. MRE sensitivities, specificities, PPV, and NPV were: PAD 82%, 100%, 100%, 23%; PAF 74%, 71%, 92%, 38%; PAA 51%, 85%, 69%, 72%; clinical 82%, 22%, 37%, 69%; clinical P-MRI 96%, 8%, 37%, 80%. MRE interreader agreement for PAD was moderate (kappa = 0.51 [0.29-0.73]), fair for PAF and PAA. DATA CONCLUSION: Using a standard technique, MRE can detect PAD with high specificity and moderate sensitivity in PIBD, missing some PAF and small PAA. LEVEL OF EVIDENCE: 3 Technical Efficacy: Stage 2 J. Magn. Reson. Imaging 2018;47:1638-1645.

Early Posthepatoportoenterostomy Predictors of Native Liver Survival in Biliary Atresia.

OBJECTIVES: Most infants with biliary atresia (BA) require liver transplantation (LT) after hepatoportoenterostomy (HPE), including those who initially clear jaundice. The aim of the present study was to identify clinical and routine laboratory factors in infants with BA post-HPE that predict native liver survival at 2 years. METHODS: A retrospective cohort study was conducted in 217 patients with BA undergoing HPE in Sydney, Australia and Toronto, Canada between January 1986 and July 2009. Univariate and multivariate logistic regression using backwards-stepwise elimination identified variables at 3 months after HPE most associated with 2-year native liver survival. RESULTS: Significant variables (P < 0.05) on univariate analysis included serum total bilirubin (TB) and albumin at 3 months post-HPE, bridging fibrosis or cirrhosis on initial liver biopsy, ascites of <3 months post-HPE, type 3 BA anatomy, age at HPE of >45 days, change in length z scores within 3 months of HPE, and center. On multivariate analysis, TB (P < 0.0001) and albumin (P = 0.02) at 3 months post-HPE, and center (P = 0.0003) were independently associated with native liver survival. Receiver operating characteristic analysis revealed an optimal cut-off value of TB <74 µmol/L (4.3 mg/dL; area under the receiver operating characteristic curve 0.8990) and serum albumin level >35 g/L (3.5 mg/dL; area under the receiver operating characteristic curve 0.7633) to predict 2-year native liver survival. TB and albumin levels 3 months post-HPE defined 3 groups (1: TB ≤74 µmol/L, albumin >35 g/L; 2: TB ≤74 µmol/L, albumin ≤35 g/L; 3: TB >74 µmol/L) with distinct short- and long-term native liver survival rates (log-rank P < 0.001). Length z scores 3 months post-HPE were poorer for group 2 than group 1 (-0.91 vs -0.30, P = 0.0217) with similar rates of coagulopathy. CONCLUSIONS: Serum TB and albumin levels 3 months post-HPE independently predicted native liver survival in BA when controlling for center. Serum albumin level <35 g/L in infants with BA who were no longer jaundiced at 3 months post-HPE was a poor prognostic indicator. Poorer linear growth and absence of significant coagulopathy suggest a role for early aggressive nutritional therapy in this group.

Guidelines for the diagnosis and management of Hirschsprung-associated enterocolitis.

BACKGROUND: Patients with Hirschsprung disease are at risk for Hirschsprung-associated enterocolitis (HAEC), an inflammatory disorder of the bowel that represents the leading cause of serious morbidity and death in these patients. The diagnosis of HAEC is made based on clinical signs and symptoms which are often non-specific, making it difficult to establish a definitive diagnosis in many patients. The purpose of this guideline is to present a rational, expert-based approach to the diagnosis and management of HAEC. METHODS: The American Pediatric Surgical Association Board of Governors established a Hirschsprung Disease Interest Group. Group discussions, literature review, and expert consensus were then used to summarize the current state of knowledge regarding diagnosis, management, and prevention of Hirschsprung-associated enterocolitis (HAEC). RESULTS: Guidelines for the diagnosis of HAEC and its clinical grade, utilizing clinical history, physical examination findings, and radiographic findings, are presented. Treatment guidelines, including patient disposition, diet, antibiotics, rectal irrigations and surgery, are presented. CONCLUSIONS: Clear, standardized definitions of Hirschsprung-associated enterocolitis and its treatment are lacking in the literature. This guideline serves as a first step toward standardization of diagnosis and management. LEVEL OF EVIDENCE: V.

Congenital Diaphragmatic Hernia in a Child With Abdominal Pain and Respiratory Distress.

We report a case of late presentation of congenital diaphragmatic hernia in a child who presented to the emergency department with abdominal pain and respiratory distress. The usual and unusual presentations of congenital diaphragmatic hernia in older children and their possible complications are discussed.

Pneumothorax in a young man with anorexia nervosa.

Primary spontaneous pneumothorax (PSP) is defined as a pneumothorax that occurs in the absence of an external cause in an individual without underlying generalized lung disease. A number of factors are important and should be carefully considered in the pathogenesis of PSP. This is the first case report of a PSP in a severely malnourished adolescent male with anorexia nervosa (AN) who was involved in excessive physical activity. Clinicians caring for adolescents with AN and vigorous exercise need to be aware of the risk factors associated with the development of a PSP. © 2016 Wiley Periodicals, Inc.(Int J Eat Disord 2016; 49:895-898).

Clinical outcomes of splenectomy in children: report of the splenectomy in congenital hemolytic anemia registry.

The outcomes of children with congenital hemolytic anemia (CHA) undergoing total splenectomy (TS) or partial splenectomy (PS) remain unclear. In this study, we collected data from 100 children with CHA who underwent TS or PS from 2005 to 2013 at 16 sites in the Splenectomy in Congenital Hemolytic Anemia (SICHA) consortium using a patient registry. We analyzed demographics and baseline clinical status, operative details, and outcomes at 4, 24, and 52 weeks after surgery. Results were summarized as hematologic outcomes, short-term adverse events (AEs) (≤30 days after surgery), and long-term AEs (31-365 days after surgery). For children with hereditary spherocytosis, after surgery there was an increase in hemoglobin (baseline 10.1 ± 1.8 g/dl, 52 week 12.8 ± 1.6 g/dl; mean ± SD), decrease in reticulocyte and bilirubin as well as control of symptoms. Children with sickle cell disease had control of clinical symptoms after surgery, but had no change in hematologic parameters. There was an 11% rate of short-term AEs and 11% rate of long-term AEs. As we accumulate more subjects and longer follow-up, use of a patient registry should enhance our capacity for clinical trials and engage all stakeholders in the decision-making process.

Rates of Hirschsprung-Associated Enterocolitis Decrease With Increasing Child Opportunity Index.

BACKGROUND: Hirschsprung-associated enterocolitis (HAEC) is the most common cause of morbidity and mortality in patients with Hirschsprung disease (HD). There is a correlation between social determinants of health (SDOH) and outcomes in children with HD. The Child Opportunity Index (COI) is a publicly available dataset that stratifies patients by address into levels of opportunity. We aimed to understand if a relationship exists between COI and HAEC. METHODS: A single-institution, IRB-approved, retrospective cohort study was performed of children with HD. Census tract information was used to obtain COI scores, which were stratified into categories (very low, low, medium, high, very high). Subgroups with and without history of HAEC were compared. RESULTS: The cohort had 100 patients, of which 93 had a COI score. There were 27 patients (29.0%) with HAEC. There were no differences in demographics or clinical factors, including length of aganglionic colon, operative approach, and age at pull-through. As child opportunity score increased from very low to very high, there was a statistically significant decrease in the incidence of HAEC (p = 0.04). CONCLUSION: We demonstrate a significant association between increasing opportunity and decreasing incidence of HAEC. This suggests an opportunity for targeted intervention in populations with low opportunity. LEVEL OF EVIDENCE: III. IRB NUMBER: IRB14-00232.

The Canadian Pediatric Surgery Workforce: A 10-year Prospective Assessment.

BACKGROUND: The Canadian Association of Paediatric Surgeons launched a 10-year prospective assessment of the Canadian pediatric surgery workforce and training environment, beginning in 2013. The results of the first 5 years (2013-2017) were previously published. Here, we present the results of the last 5 years (2018-2022), and the cumulative results of the past decade. METHODS: With IRB approval, a web-based survey was sent to all pediatric surgery division chiefs in Canada each year (2013-2022). The survey gathered workforce data on pediatric surgery practices, as well as data regarding fellowship graduates from Canadian training programs. RESULTS: Complete responses were received from all 18 divisions (100% response rate). Over the decade studied, the number of pediatric surgeons and full-time equivalent positions increased from 73 to 81, and 65 to 82, respectively. Thirty positions were vacated (15 retirement, 6 new Canadian practice, 8 leaving Canada, 1 other), and 38 were filled (20 new Canadian fellowship graduates, 8 Canadian surgeons moving from other sites in Canada, 10 surgeons coming from outside Canada). Seventy-five fellows completed training eligible for North American certification, including 34 Canadians, 31 Americans, and 10 non-North American foreign nationals (9 of whom left North America after training). The proportion of Canadian graduates who desired, but could not find, a Canadian position improved from 44% in the first 5 years to 20% in the second 5 years. CONCLUSIONS: The Canadian pediatric surgery workforce has experienced a modest increase over a decade. A mismatch still exists between Canadian pediatric surgery graduates and attending staff positions, but the situation has improved during the last 5 years. TYPE OF STUDY: Survey.

Duhamel Versus Swenson Pull-Through for Total Colonic Aganglionosis: A Multi-Institutional Study.

BACKGROUND: Total colonic aganglionosis (TCA) is a rare variant of Hirschsprung disease (HD) where the colon and portion of distal ileum lack ganglion cells. Most pediatric use either a straight ileoanal (Swenson or Yancey-Soave) or a short Duhamel pull-through for TCA. There are no large studies comparing these techniques. We aimed to compare short-and medium-term outcomes between these approaches. METHOD: A retrospective review was performed among children with TCA from 2001 to 2019 undergoing a primary Duhamel or Swenson pull-through across three large children's hospitals. Patients undergoing redo and patients with greater than 30 % small bowel aganglionosis were excluded. We gathered data on demographics, operative approach, and outcomes at one, two, and three years. Continuous variables were analyzed with t-tests and categorical variables with Chi square or Fisher's tests. RESULTS: There were 54 patients, with 26 (48 %) undergoing Duhamel and 28 (52 %) undergoing Swenson pull-through. There were no differences in sex, age, medical comorbidities, or operative details, including age at pull-through, laparoscopic vs open, length of involved small bowel, and operative time. Length of stay and post-operative complications were not different. Three years after pull-through, patients undergoing Duhamel had fewer stools per day (1-3 stools 69.6 % vs 14.3 %, p = 0.003) and were less likely to be prescribed fiber supplementation (4.2 % vs 43.8 %, p = 0.003). There were no differences in irrigations, botulinum toxin administration, loperamide, or HD admissions. CONCLUSION: Both Duhamel and straight pull-throughs are safe for treatment of TCA, with acceptable short- and medium-term outcomes. Further studies on patient-reported outcomes are necessary to examine long-term differences. LEVEL OF EVIDENCE: III.

Is activity restriction appropriate for patients with hereditary spherocytosis? A population-based analysis.

Patients with hereditary spherocytosis (HS) are often thought to have an increased risk of blunt splenic injury (BSI) from trauma due to splenomegaly. We aim to quantify this risk. Using a population-based database consisting of all injury-related admissions in Canada from 2001 to 2010, we identified patients with BSI and HS based on the discharge diagnoses. Intercensal population estimates were used to derive rates of BSI. The HS population at risk for BSI was estimated based on population rates of HS obtained from the literature. Rates of BSI in the HS population were estimated and the relative rates of BSI were calculated to compare the populations with and without HS. There were 10,106 patients with BSI over 202,405,788 person-years of observation, yielding an overall rate of BSI in the general population of 5.0 BSI per 100,000 person-years. Of these BSI patients, only two had a history of HS. Population rates of HS in the literature range from 1 in 2,000 to 5,000, corresponding to a low estimate of 2.0 and a high estimate of 4.9 BSI per 100,000 person-years in the HS population. The relative rate of BSI in the population with HS compared to the population without HS ranged from a low of 0.4 (95 % CI 0.1-1.4) to a high of 1.0 (0.1-3.6). The rate of BSI in the HS patient population appears not to differ significantly from those in the general population.

Hirschsprung disease.

PURPOSE OF REVIEW: Hirschsprung disease is relatively common in children. Surgical techniques are available to remove the aganglionic bowel and reconstruct the intestinal tract. Despite many advances, these children may still be difficult to diagnose, and may have ongoing functional problems after surgical correction. RECENT FINDINGS: The genetic basis and cause of Hirschsprung disease are becoming increasingly clearer. Definitive diagnosis is based on rectal biopsy, but more sophisticated techniques are permitting earlier and more accurate diagnosis. Surgical correction has evolved from routine use of a colostomy and open laparotomy to one-stage transanal and laparoscopic approaches. The range of postoperative problems is reviewed so that the practicing pediatrician and primary care physician can effectively care for these children. Future advances may include better genotype-phenotype correlation and development of neuronal stem cell techniques. SUMMARY: Pediatricians and primary care physicians have an important role to play in diagnosing and managing children with Hirschsprung disease.

Diagnosis of postoperative bile leak and accurate localization of the site of leak by gadobenate dimeglumine-enhanced MR cholangiography in a child.

Postoperative bile leaks are difficult to diagnose because signs and symptoms are nonspecific, and definitive diagnosis requires a drainage catheter to be placed that drains frank bile or fluid that turns out bile on laboratory analysis. Hepatobiliary scintigraphy, endoscopic retrograde cholangiopancreatography (ERCP) and percutaneous transhepatic cholangiography (PTC) have been traditionally used to diagnose bile leak. Scintigraphy has poor spatial resolution and cannot localize the site of leak, ERCP may not be possible with altered postoperative anatomy and PTC is invasive and difficult to perform in the absence of dilated ducts. Recently, contrast-enhanced MR cholangiography using hepatobiliary-specific contrast media has been shown to be a noninvasive, safe way to diagnose and localize the site of bile leak in adults. We report a case of postoperative bile leak after choledochal cyst resection in a child in whom diagnosis was confirmed and the site of leak was accurately localized using gadobenate-enhanced MR cholangiography. This case demonstrates the potential of hepatobiliary-specific contrast media to diagnose postoperative bile leak and accurately localize the site of the leak in children noninvasively.

Hirschsprung disease.

Hirschsprung disease (HSCR) is a rare congenital intestinal disease that occurs in 1 in 5,000 live births. HSCR is characterized by the absence of ganglion cells in the myenteric and submucosal plexuses of the intestine. Most patients present during the neonatal period with the first meconium passage delayed beyond 24 h, abdominal distension and vomiting. Syndromes associated with HSCR include trisomy 21, Mowat-Wilson syndrome, congenital central hypoventilation syndrome, Shah-Waardenburg syndrome and cartilage-hair hypoplasia. Multiple putative genes are involved in familial and isolated HSCR, of which the most common are the RET proto-oncogene and EDNRB. Diagnosis consists of visualization of a transition zone on contrast enema and confirmation via rectal biopsy. HSCR is typically managed by surgical removal of the aganglionic bowel and reconstruction of the intestinal tract by connecting the normally innervated bowel down to the anus while preserving normal sphincter function. Several procedures, namely Swenson, Soave and Duhamel procedures, can be undertaken and may include a laparoscopically assisted approach. Short-term and long-term comorbidities include persistent obstructive symptoms, enterocolitis and soiling. Continued research and innovation to better understand disease mechanisms holds promise for developing novel techniques for diagnosis and therapy, and improving outcomes in patients.