Long-term outcomes in newborn surgery.

This article describes the common methods to study long-term outcomes in patients who have undergone major surgery in newborn period. It also sums up today's knowledge on the long-term outcome of some classic newborn surgical conditions. The analysis of long-term outcomes is important to pediatric surgeons. Knowledge of long-term outcome can guide the patient's management and principles of the follow-up throughout the patient's childhood. It also aims to give the parents of the patient a realistic picture on the development of their child. Recent data have shown that many patients who have undergone major surgery during early childhood have significant functional aberrations at adult age. Some of these have a profound influence on the quality of life of these patients.

Intra-articular venous malformation of the knee in children: magnetic resonance imaging findings and significance of synovial involvement.

BACKGROUND: Intra-articular venous malformations of the knee are an uncommon cause of unilateral knee pain in children. Timely diagnosis is important because lesions with intrasynovial involvement can lead to joint space hemorrhage and secondary cartilage damage. OBJECTIVE: To describe our tertiary center's experience of diagnostics and typical magnetic resonance imaging (MRI) findings. MATERIALS AND METHODS: A retrospective review of all patients ≤16 years of age managed for intra-articular venous malformations of the knee at our institution between 2002 and 2018. RESULTS: Of 14 patients (8 male), the mean age at presentation was 6 years (range: 0-14 years). The most common clinical findings were unilateral knee pain (93%), joint swelling (79%), quadriceps atrophy (50%) and a limited range of motion (29%). Cutaneous manifestations were present in four patients (29%). Contrast-enhanced MRI was available in all cases. After initial MRI, a vascular anomaly etiology had been identified in 11 cases (79%), and correctly reported as a venous malformation in 6 (55%). Three patients received entirely different diagnoses (arthritis, tumor or pigmented villonodular synovitis). Three of seven patients with intrasynovial lesions had established chondropathy at diagnosis. Two patients with lesions of the suprapatellar fat pad had intrasynovial involvement that was not visualised on MRI. CONCLUSION: Although MRI usually permits the diagnosis, clinical awareness of these lesions is important for optimal imaging, accurate interpretation and timely diagnosis. Involvement of the intrasynovial cavity carries a risk of hemarthrosis and progressive chondropathy that may be underestimated by MRI.

Intestinal Microbiota in Hirschsprung Disease.

OBJECTIVES: The aim of the study was to characterize the microbiota profiles of patients with Hirschsprung disease (HD) and to evaluate this in relation to postoperative bowel function and the incidence of Hirschsprung-associated enterocolitis (HAEC). METHODS: All patients operated on for HD at our center between 1987 and 2011 were invited to answer questionnaires on bowel function and to participate in a clinical follow-up for laboratory investigations, including fecal DNA extraction, fecal calprotectin (FC), and brush border lactase (LCT) genotyping. The microbiota compositions of patients with HD were compared with those of healthy controls aged between 2 and 7 years. RESULTS: The microbiota composition of eligible patients with HD (n = 34; median age 12 [range, 3-25] years) differed from the healthy controls (n = 141), showing decreased overall microbial richness (P < 0.005). Seventy-seven percent had experienced HAEC. Normal maturation of the intestinal flora was not observed, but patients had a significantly increased abundance of Proteobacteria among other taxa (P < 0.005) resulting in a reduced carbohydrate degradation potential, as predicted by the taxonomic composition. Genetic lactase deficiency was present in 17% and did not correlate with bowel symptoms. No patients reported active HAEC at the time of sampling and FC was within the normal range in all samples. CONCLUSIONS: Patients with HD and HAEC had a significantly altered intestinal microbiome compared to healthy individuals, characterized by a lack of richness and pathologic expansions of taxa, particularly Enterobacteria and Bacilli. Further evaluation is needed to identify whether these observations are intrinsic to HD or secondary to the recurrent use of antibiotics during early childhood.

Congenital diaphragmatic hernia: a scientometric analysis of the global research activity and collaborative networks.

Despite a growing interest to clinicians and scientists, there is no comprehensive study that examines the global research activity on congenital diaphragmatic hernia (CDH). A search strategy for the Web of Science™ database was designed to identify scientific CDH publications. Research output of countries, institutions, individual authors, and collaborative networks was analyzed. Semi-qualitative research measures including citation rate and h-index were assessed. Choropleth mapping and network diagrams were employed to visualize results. A total of 3669 publications were found, originating from 76 countries. The largest number was published by the USA (n = 1250), the UK (n = 279), and Canada (n = 215). The USA combined the highest number of cooperation articles (n = 152), followed by Belgium (n = 115) and the Netherlands (n = 93). The most productive collaborative networks were established between UK/Belgium (n = 53), Belgium/Spain (n = 47), and UK/Spain (n = 34). Canadian publications received the highest average citation rate (22.8), whereas the USA had the highest country-specific h-index (72). Eighty-five (2.3%) articles were published by international multicenter consortiums and national research networks. The most productive institutions and authors were based in North America and Europe. Over the past decades, CDH research has increasingly become multidisciplinary and numerous innovative therapeutic strategies were introduced. CDH-related research has constantly been progressing, involving today many disciplines with main research endeavors concentrating in a few high-income countries. Recent advances in prenatal interventions and regenerative medicine therapy hold the promise of improving CDH outcome in the 21st century. International collaborations and translational research should be strengthened to allow further evolution in this field.

Bowel Function and Quality of Life After Transanal Endorectal Pull-through for Hirschsprung Disease: Controlled Outcomes up to Adulthood.

OBJECTIVE: The aim of this study was to define controlled outcomes up to adulthood for bowel function and quality of life (QoL) after transanal endorectal pull-through (TEPT) for Hirschsprung disease (HD). SUMMARY OF BACKGROUND DATA: Although TEPT is the surgical standard for HD, controlled long-term follow-up studies evaluating bowel function and QoL are lacking. METHODS: Patients aged ≥4 years operated for HD with TEPT between 1987 and 2011 answered detailed questionnaires on bowel function and QoL [Pediatric Quality of Life Inventory (PedsQL, age <18 yrs) or Gastrointestinal Quality of Life Index (GIQLI) and SF-36]. Patients were compared with 3 age- and gender-matched controls each randomly selected from the general population. RESULTS: Seventy-nine patients (64%) responded (median age 15, range 4-32 years; 86% rectosigmoid aganglionosis). Compared with controls, patients reported impairment of all aspects of fecal control (P < 0.05), except constipation. In cross-section, 75% of patients were socially continent (vs 98% of controls; P < 0.001). Soiling, fecal accidents, rectal sensation, and ability to withhold defecation improved with age to levels comparable to controls by adulthood (P = NS), but stooling frequency remained higher in 44% of patients (P < 0.05 vs controls). PedsQL domains in childhood were equal to controls (P = NS), except for proxy-reports of sadness/depression. Adults exhibited lower emotional scores, limitation of personal, and sexual relationships (P < 0.05). CONCLUSION: Compared with matched peers, significant impairment of fecal control prevails after TEPT in HD patients during childhood, but symptoms diminish with age. Although overall QoL appeared comparable to controls, impairment of emotional and sexual domains may prevail in adulthood.

Sexual Function, Fertility and Quality of Life after Modern Treatment of Anorectal Malformations.

PURPOSE: Sexual dysfunction and impaired quality of life due to fecal incontinence are common after classic operations for anorectal malformations. We hypothesized that modern repairs may result in improved outcomes. MATERIALS AND METHODS: Following ethical approval for this single institution cross-sectional study, all patients 16 years or older treated for rectourethral, vestibular or perineal fistula from 1983 onward were sent detailed postal questionnaires on sexual function and quality of life. Each respondent was age and gender matched to 3 controls randomly selected from the general population. Penoscrotal/gynecologic abnormalities were obtained from the records. RESULTS: A total of 41 patients (62%) with a median age of 22 years participated in the study. Of the patients 20 were males with rectourethral fistula (prostatic in 60%), 10 were females with vestibular/perineal fistula and 11 were males with low malformations. Although experience of sexual relationships and orgasmic function were reported in comparable proportions to controls, age at coital debut was significantly delayed in all groups of patients (p ≤0.046). Erectile function was preserved after sagittal repair but absent ejaculations or azoospermia affected 3 males with rectourethral fistula (15%). Penoscrotal/gynecologic abnormalities affected 12% of patients. Overall quality of life scores were comparable to controls but a trend was found for lower scores on emotional items in males with rectourethral fistula (p = 0.06) and for a negative effect on sexual life in females (p = 0.03). CONCLUSIONS: While erectile and orgasmic function appear preserved after sagittal repair, further evaluation of fertility issues in males with rectourethral fistula is indicated. Larger multicenter studies are needed to confirm our findings.

Endoscopic Surveillance After Repair of Oesophageal Atresia: Longitudinal Study in 209 Patients.

AIM: After repair of oesophageal atresia (OA), the need for endoscopic follow-up (EFU) remains unclear. To end this, we assessed the trends of oesophageal mucosal changes in successive follow-up biopsies. METHODS: EFU records of 264 patients including histological grades of oesophagitis (from 0 to III), gastric (GM) or intestinal (IM) metaplasia and dysplasia (mild to severe) at 1, 3, 5 10, 15, and >15 years after repair of OA were reviewed. RESULTS: Included were 209 patients with 616 biopsies. A total of 60 patients had undergone antireflux surgery and 24 had long-gap OA (LG). Median follow-up was 12 (range 1-17) years with 3 (1-6) endoscopies per patient. Highest grade of oesophagitis was Gr 0 (no oesophagitis) in 47%, Gr I in 37%, and Gr II or III in 16%. Metaplasia, GM (n = 31), IM (n = 4), occurred in 17% of patients and reached 15% prevalence by 15 years. Dysplasia and cancer were not found. From 1 to 15 years after repair grade of histological oesophagitis often fluctuated between Gr 0 and Gr I, but further progression was unlikely, hazard ratio = 0.2-3.4 (95% confidence interval 0.0-29), P = 0.06-0.87. LG and antireflux surgery predicted early detection of metaplasia (P < 0.001). Only 9% of patients with metaplasia and 32% with Gr II oesophagitis were symptomatic. A total of 6 (3%) patients had a symptomatic anastomotic stenosis at 1 year. CONCLUSIONS: EFU revealed frequent oesophagitis and metaplasia, but no dysplasia or cancer. Routine endoscopic surveillance had limited benefit and seems unnecessary during childhood after repair of OA.

Bowel functional outcomes in females with perineal or vestibular fistula treated with anterior sagittal anorectoplasty: controlled results into adulthood.

BACKGROUND: Anterior sagittal anorectoplasty is a standardized operative treatment for females with congenital rectoperineal or vestibular fistula. The controlled, long-term outcomes require characterization. OBJECTIVE: The aim of this study was to define the bowel functional outcomes following anterior sagittal anorectoplasty in relation to age- and sex-matched controls. DESIGN: This cross-sectional study was conducted at a single institution. PATIENTS: All females treated for congenital perineal or vestibular fistula with anterior sagittal anorectoplasty between 1983 and 2006 were invited to answer a detailed, previously validated questionnaire on bowel function. Each patient was matched to 3 controls who had answered identical questionnaires. Ethical approval was obtained. Social continence was defined as soiling or fecal accidents <1/week and no requirement for changes of underwear or protective aids. SETTING: This study was conducted at the Hospital for Children and Adolescents, University of Helsinki, Finland. INTERVENTIONS: No interventions were performed. MAIN OUTCOME MEASURES: The primary outcomes measured were the prevalence of problems with rectal sensation, voluntary bowel control, soiling, fecal accidents, constipation, and social problems. Bowel function score (out of 20 items) was considered, as well as the age at completion of toilet training for stool. RESULTS: Of 34 respondents (79%; median age, 13 (4-28) years), all had voluntary bowel movements. Problems withholding defecation, soiling, and fecal accidents were significantly more common among patients than controls (p ≤ 0.001). Eighty-five percent of patients and 100% of controls (p = 0.001) were socially continent; 41% of patients and 76% of controls were totally continent (p = 0.0003). Constipation tended to decline with age (from 59% to 25%; p = 0.16). The bowel functional outcome was good in 68% of patients, satisfactory in 26% of patients, and poor in 6% of patients. Diapers for stool had been discontinued at the same median age as controls. LIMITATIONS: Number of patients in comparative analysis of symptoms by age group. CONCLUSIONS: After anterior sagittal anorectoplasty for perineal or vestibular fistula, 2 of 3 of patients are likely to achieve bowel control comparable to normal in the long term, and the vast majority will be socially continent. The effective treatment of constipation is essential.

Surgical rehabilitation of short and dysmotile intestine in children and adults.

AIMS: This is a descriptive study aiming to compare outcomes of intestinal rehabilitation surgery among pediatric and adult intestinal failure (IF) patients with either primary intestinal motility disorders or short bowel syndrome (SBS) treated by our nationwide program. METHODS: Medical records of IF patients (n = 31, 71% children) having undergone autologous intestinal reconstructions (AIR) (n = 25), intestinal transplantation (ITx) (n = 5), or being listed for ITx (n = 2) between 1994 and 2014 were reviewed. RESULTS: At surgery, median age was 3.4 (interquartile range, 1.0-22.1) in SBS (n = 22) and 16.5 (3.2-26.7) years in dysmotility patients (n = 9) who received median 60% and 83% of energy requirement parenterally, respectively. Median small bowel length was shorter in SBS than dysmotility patients (34 versus 157 cm, p < 0.001). Following AIR, none of the dysmotility patients achieved permanent intestinal autonomy, whereas 68% of SBS patients weaned off parenteral nutrition (PN) (p = 0.022) and none required listing for ITx. Five dysmotility patients who underwent ITx achieved intestinal autonomy. Regarding both AIR and ITx procedures, no significant difference in PN weaning was observed between the two subgroups. At last follow-up, 3.3 (0.6-8.0) years postoperatively, median plasma bilirubin was 6 (4-16) µmol/l, while liver biopsy showed fibrosis (Metavir stage 1-2) in 50% and cholestasis in 8%. Proportion of PN energy requirement had reduced significantly (p = 0.043) among PN-dependent SBS (n = 7) but not among dysmotility patients (n = 5). Overall survival was 90%. CONCLUSION: AIR surgery was beneficial among selected SBS patients, whereas in intestinal dysmotility disorders, permanent PN weaning was only achieved by ITx.

Bowel function and lower urinary tract symptoms in males with low anorectal malformations: an update of controlled, long-term outcomes.

PURPOSE: This study aimed to define the controlled outcomes into adulthood for bowel function and lower urinary tract symptoms (LUTS) for males treated for low ARMs with individualized, minimally invasive approaches that preserve the native continence mechanisms as far as possible. METHODS: This is a single-institution, cross-sectional study. All males treated for low ARMs with cutback anoplasty, incision of anocutaneous membrane or dilatations based on the exact type of each malformation between 1983 and 2006 were invited to answer a detailed questionnaire on bowel function and LUTS. Each patient was matched to three controls representing the normal population. Ethical approval was obtained. RESULTS: Amongst 46 respondents (67%; median age 12.3 (5-29) years), overall fecal control was comparable to controls (p = NS). All patients had voluntary bowel movements; 98% of patients and 97% of controls were socially continent (p = NS); 67% of patients and 64% of controls were totally continent (p = NS). Constipation amongst patients (33 vs 3% in controls; p < 0.0001) declined significantly with age. Outcomes by bowel function score were good in 85%, satisfactory in 15% and poor in 0%. Prevalence of LUTS and age at completion of toilet training were comparable to controls (p = NS). CONCLUSIONS: Our results support the adequacy and appropriateness of treating males with low ARMs with individualized, minimally invasive perineal procedures. Long-term functional outcomes comparable to controls are achieved in the majority, provided that constipation is effectively addressed.

Pediatric urology patients with megaureter may have a risk of contracting Crohn disease.

Crohn disease (CD) may develop at any age, but is most typically diagnosed in late adolescence and early adulthood. To the best of our knowledge, there are no reports showing that congenital urological abnormalities may be associated to CD. We describe 5 pediatric patients who had ureteral/urethral abnormalities in their infancy and developed CD during subsequent years. The occurrence of CD in these patients constitutes a novel potential association.

An interdisciplinary specialist team leads to improved diagnostics and treatment for paediatric patients with vascular anomalies.

AIM: Patients with vascular anomalies are often misdiagnosed, leading to delayed or improper treatment. The aim of this study was to evaluate the impact of an interdisciplinary team on the diagnosis and treatment of paediatric patients with vascular anomalies. METHODS: We reviewed the paediatric patients evaluated by our interdisciplinary team between 2002 and 2012, analysing the referral diagnosis, final diagnosis, patient age, sex, clinical history, laboratory tests, imaging studies and treatments. RESULTS: Of the 480 patients who were evaluated, 435 (90.6%) had a vascular anomaly: 30.7% of all patients had a tumour and 55.2% had a malformation. Haemangiomas comprised 93.2% of all tumours, while malformations included capillary (9.8%), lymphatic (30.1%), venous (36.8%), arteriovenous (3.8%) and combined slow-flow (7.9%) malformations. Tumours were initially diagnosed correctly in 89.2% of the patients, but only 38.0% of the malformations were diagnosed correctly. Improper treatment was given to 1.4%, due to incorrect diagnoses. CONCLUSION: This study showed that haemangiomas were likely to be diagnosed correctly, but other tumours and vascular malformations were likely to be misdiagnosed. Misdiagnosis seldom led to improper treatment, but probably led to delayed treatment in many cases. The interdisciplinary approach led to improved diagnostics and treatment.

Persistent abnormal liver fibrosis after weaning off parenteral nutrition in pediatric intestinal failure.

UNLABELLED: The aim of this study was to evaluate the long-term effects of pediatric intestinal failure (IF) on liver histology. Altogether, 38 IF patients (median age: 7.2 years; range, 0.2-27) underwent liver biopsy, gastroscopy, abdominal ultrasound, and laboratory tests. Sixteen patients were on parenteral nutrition (PN) after 74 PN months (range, 2.5-204). Twenty-two had weaned off PN 8.8 years (range, 0.3-27) earlier, after 35 PN months (range, 0.7-250). Fifteen transplant donor livers served as controls. Abnormal liver histology was found in 94% of patients on PN and 77% of patients weaned off PN (P = 0.370). During PN, liver histology weighted with cholestasis (38% of patients on PN versus 0% of patients weaned off PN; P = 0.003) and portal inflammation (38% versus 9%; P = 0.050) were found. Fibrosis (88% versus 64%; P = 0.143; Metavir stage: 1.6 [range, 0-4] versus 1.1 [range, 0-2]; P = 0.089) and steatosis (50% versus 45%; P = 1.000) were equally common during and after weaning off PN. Plasma alanine aminotransferase (78 U/L [range, 19-204] versus 34 [range, 9-129]; P = 0.009) and conjugated bilirubin (43 µmol/L [range, 1-215] versus 4 [range, 1-23]; P = 0.037) were significantly higher during than after weaning off PN. Esophageal varices were encountered in 1 patient after weaning off PN. Metavir stage was associated with small bowel length (r = -0.486; P = 0.002) and number of septic episodes (r = 0.480; P = 0.002). In a multivariate analysis, age-adjusted small bowel length (ß = -0.533; P = 0.001), portal inflammation (ß = 0.291; P = 0.030), and absence of an ileocecal valve (ß = 0.267; P = 0.048) were predictive for fibrosis stage. CONCLUSION: Despite resolution of cholestasis and portal inflammation, significant liver fibrosis and steatosis persist after weaning off PN. Extensive small intestinal resection was the major predictor for liver fibrosis stage.

Rectopexy for paediatric rectal prolapse: good outcomes but not without postoperative problems.

PURPOSE: Rectopexy is a simple treatment of persisting complete rectal prolapse (RP) or related functional disorders in children. The results of rectopexy have been encouraging with few complications. We describe the postoperative complications and outcome of rectopexy in our institution from 2002 to 2013. METHODS: Ethical committee accepted the study. Hospital records of 27 successive patients (16 males), median age 7.2 (range 2.8-17) years, who underwent rectopexy (25 laparoscopic, 2 open) were reviewed. Indication for rectopexy included RP (n = 24), solitary rectal ulcer with enterocele (n = 2) and rectocele (n = 1). Nine patients (39 %) were healthy. In the remaining 14 patients, RP was secondary to anorectal malformation (n = 2), bladder exstrophy (n = 1), sacrococcygeal teratoma (n = 1) and myelomeningocele (n = 1) or associated with mental retardation (n = 8) and Asperger's syndrome (n = 1). Five (18 %) patients had constipation. Unexpected postoperative events and complications were rated by Clavien-Dindo classification (Grades I-V). RESULTS: Seventeen (61 %) patients had postoperative complications (Grade I n = 5, II n = 2 and III n = 7). Readmission was required in 11 (41 %) and reoperation, endoscopy or other surgical procedure in 9 (33 %) patients. Complications included severe faecal obstruction (n = 2), constipation (n = 3), faecal soiling (n = 1) urinary retention (n = 2), enuresis (n = 1), infection (n = 2), residual mucosal prolapse (n = 5), discomfort at defecation (n = 1) and recurrent RP (n = 2). Reoperations included sigmoid resection with re-rectopexy (n = 1), resection of mucosal prolapse (n = 1), suprapubic urinary catheter (n = 2), evacuation of faecal impaction (n = 2), colonoscopy (n = 3), appendicostomy for antegrade continence enema (n = 1). Mental retardation or behavioural disorder increased the risk of postoperative faecal obstruction and constipation RR = 84 (95 % CI 4.3-1600), p = 0.0035. After median follow-up of 4.1 (range 0.6-11) years RP or related condition was cured in 26 patients. Constipation and faecal soiling require management in a total of seven patients. CONCLUSIONS: Long-term results of rectopexy were good. Postoperative complications from mild to moderate grade were unexpectedly frequent. Preoperative neurobehavioural disorder and constipation increase the risk of postoperative problems and should be mentioned in patient counselling.

Lower urinary tract symptoms from childhood to adulthood: a population based study of 594 Finnish individuals 4 to 26 years old.

PURPOSE: We evaluated voiding habits and lower urinary tract symptoms by age and gender in a large population of individuals from childhood to adulthood. MATERIALS AND METHODS: We studied a cross-sectional sample of 594 individuals 4 to 26 years old randomly selected from the population register of Finland. Participants anonymously answered a detailed postal questionnaire on lower urinary tract symptoms. Parents assisted respondents younger than 16 years. Results were analyzed by age group (4 to 7, 8 to 12, 13 to 17 and 18 to 26 years) and gender. A p value of less than 0.05 was considered statistically significant. RESULTS: The prevalence of urge incontinence significantly decreased with age (45% in respondents 4 to 7 years vs 10% in respondents 13 to 17 years, p <0.05). Urinary tract infections and urge and stress incontinence were more common in females (16% to 32%) than in males (2% to 4%) older than 12 years (p <0.05). The occurrence of some type of minor daytime urinary incontinence was reported by approximately a fourth of the study population, with a significant decline in prevalence between ages 4 to 7 years and 8 to 12 years (p <0.05). Minor urinary incontinence was significantly more common in females older than 12 years. Frequent urinary incontinence affected only 4% of respondents, most of whom were younger than 12 years. CONCLUSIONS: Bladder control and urinary function exhibit considerable variation with age and gender. Due to the imperfections in bladder control in the general population, the evaluation of urinary tract disorders and outcomes of surgery in children and adolescents should be conducted with reference to control data according to age and gender.

Evaluation of bowel function and fecal continence in 594 Finnish individuals aged 4 to 26 years.

BACKGROUND: Limited information exists on what constitutes normal bowel function in children and adolescents. This information is essential for determining outcomes of surgery for anorectal malformations and other colonic disorders. OBJECTIVE: The aim of this study was to define bowel function and fecal continence in a large sample of individuals aged 4 to 26 years. DESIGN: This investigation is a cross-sectional cohort study. SETTINGS: Five hundred ninety-four individuals aged 4 to 26 years were randomly selected from the population register of Finland. Participants anonymously answered a previously validated 7-item postal questionnaire on bowel function. MAIN OUTCOME MEASURES: The primary outcomes measured were bowel function score, fecal continence, stooling frequency, constipation, and social problems in relation to bowel function. RESULTS: Recognition of the need to defecate was well established by age 4, but the ability to hold back defecation improved with age. Fecal accidents decreased from 11% in preschool children to 2% by 13 to 17 years (p = 0.02). Fecal staining of underwear was common (33%); the prevalence of soiling decreased with age. Stooling frequency was normal in 92%. Constipation was more common in females. Problems restricting social life in relation to bowel function were rare (0.5%). CONCLUSIONS: The fine-tuning of fecal continence continues to develop during childhood. Minor soiling is common in healthy individuals. Because of the changes with age, the evaluation of functional outcomes in patients who have undergone surgical treatment of anorectal malformations or other colonic disorders should include comparison with data from age-matched controls.

Inflammatory bowel disease manifesting after surgical treatment for Hirschsprung disease.

Eight children developed chronic inflammatory bowel disease (IBD) 4 to 21 years after surgery for Hirschsprung disease. Three had trisomy 21 and 6 experienced chronic or recurrent enterocolitis. Four had a family history of IBD. Clinical presentation included chronic diarrhea, hematochezia, abscess, and fistula formation. Three required surgery for fistula, stricture, and small bowel obstruction and the other 5 were managed medically. Recognition of this condition may be important in the long-term follow-up of children with Hirschsprung disease, and patients who have carried a diagnosis of chronic enterocolitis may warrant further investigation looking for evidence of IBD.

Lower urinary tract symptoms in adults treated for posterior urethral valves in childhood: matched cohort study.

PURPOSE: We compared the prevalence and bother of lower urinary tract symptoms in adults treated for posterior urethral valves in childhood and population based controls. MATERIALS AND METHODS: Questionnaires were mailed to patients 18 years and older treated at our institution for posterior urethral valves. Of the 124 patients 68 (64.2%) participated, 18 were unavailable and 38 did not participate. Age and sex matched controls were randomly identified from a population based study (response proportion 62.4%). Danish Prostatic Symptom Score was used to assess occurrence (never/rarely/often/always) and bother (none/small/moderate/major) of 12 different lower urinary tract symptoms. Chi-square test was used for the analyses stratified by patient/control status, with the 4-point ordinal scale as the outcome. RESULTS: Median age of 68 patients with posterior urethral valves and 272 controls (ratio 1:4) was 38.5 years (range 18 to 57). Overall, at least 1 moderate or severe lower urinary tract symptom was reported by 32.4% of patients with posterior urethral valves and 15.8% of controls (p=0.002). Mild hesitancy, weak stream, incomplete emptying and straining were reported twice as often by patients with posterior urethral valves as by controls (p<0.05). Prevalence of any urgency incontinence (14.7% vs 4.8%, p=0.014) and any stress incontinence (11.8% vs 3.0%, p=0.005) was increased at least 3-fold in patients with posterior urethral valves compared to controls. Furthermore, prevalence of bother from several lower urinary tract symptoms was increased approximately 2-fold in patients with posterior urethral valves compared to controls (p<0.05). However, most patients and controls reported no or small bother. CONCLUSIONS: In adulthood the occurrence and bother of most lower urinary tract symptoms are increased approximately 2-fold in patients with posterior urethral valves compared to the general population. However, in this cohort of young and middle-aged men most symptoms are mild.

Effects of long-term parenteral nutrition on serum lipids, plant sterols, cholesterol metabolism, and liver histology in pediatric intestinal failure.

BACKGROUND AND OBJECTIVE: Plant sterols (PS) in parenteral nutrition (PN) may contribute to intestinal failure-associated liver disease. We investigated interrelations between serum PS, liver function and histology, cholesterol metabolism, and characteristics of PN. PATIENTS AND METHODS: Eleven patients with intestinal failure (mean age 6.3 years) receiving long-term PN were studied prospectively (mean 254 days) and underwent repeated measurements of serum lipids, noncholesterol sterols, including PS, and liver enzymes. PS contents of PN were analyzed. Liver biopsy was obtained in 8 patients. Twenty healthy children (mean age 5.7 years) served as controls. RESULTS: Median percentage of parenteral energy of total daily energy (PN%) was 48%, including 0.9 g · kg(-1) · day(-1) of lipids. Respective amounts of PN sitosterol, campesterol, avenasterol, and stigmasterol were 683, 71, 57, and 45 µg · kg(-1) · day(-1). Median serum concentrations of sitosterol (48 vs 7.5 µmol/L, P < 0.001), avenasterol (2.9 vs 1.9, P < 0.01), stigmasterol (1.9 vs 1.2, P < 0.005), but not that of campesterol (9.8 vs 12, P = 0.22), were increased among patients in relation to controls, and correlated with PN% (r = 0.81-0.88, P < 0.005), but not with PN fat. Serum cholesterol precursors were higher in patients than in controls. Serum liver enzymes remained close to normal range. Glutamyl transferase correlated with serum PS (r = 0.61-0.62, P < 0.05). Liver fibrosis in 5 patients reflected increased serum PS (r = 0.55-0.60, P = 0.16-0.12). CONCLUSIONS: Serum PS moderately increase during olive oil-based PN, and correlate positively with PN% and glutamyl transferase. Despite well-preserved liver function, histology often revealed significant liver damage.

Long-term results of esophageal atresia: Helsinki experience and review of literature.

Esophageal atresia (EA) affects one in 2,840 newborns, and over half have associated anomalies that typically affect the midline. After EA repair in infancy, gastroesophageal reflux (GER) and esophageal dysmotility and respiratory problems are common. Significant esophageal morbidity associated with EA extends into adulthood. Surgical complications, increasing age, and impaired esophageal motility predict the development of epithelial metaplasia after repair of EA. To date, worldwide, six cases of esophageal cancer have been reported in young adults treated for EA. According to our data, the statistical risk for esophageal cancer is not higher than 500-fold that of the general population. However, the overall cancer incidence among adults with repaired EA does not differ from that of the general population. Adults with repaired EA have had significantly more respiratory symptoms and infections, as well as more asthma and allergies than does the general population. Nearly half the patients have bronchial hyperresponsiveness. Thoracotomy-induced rib fusion and gastroesophageal reflux-associated columnar epithelial metaplasia are the most significant risk factors for the restrictive ventilatory defect that occurs in over half the patients. Over half the patients with repaired EA are likely to develop scoliosis. Risk for scoliosis is 13-fold after repair of EA in relation to that of the general population. Nearly half of the patients have had vertebral anomalies predominating in the cervical spine, and of these, most were vertebral fusions. The natural history of spinal deformities seems, however, rather benign, with spinal surgery rarely indicated.