Antti Koivusalo

Recovery after open versus laparoscopic pyloromyotomy for pyloric stenosis: a double-blind multicentre randomised controlled trial

BACKGROUND A laparoscopic approach to pyloromyotomy for infantile pyloric stenosis has gained popularity but its effectiveness remains unproven. We aimed to compare outcomes after open or laparoscopic pyloromyotomy for the treatment of pyloric stenosis. METHODS We did a multicentre international, double-blind, randomised, controlled trial between June, 2004, and May, 2007, across six tertiary paediatric surgical centres. 180 infants were randomly assigned to open (n=93) or laparoscopic pyloromyotomy (n=87) with minimisation for age, weight, gestational age at birth, bicarbonate at initial presentation, feeding type, preoperative duration of symptoms, and trial centre. Infants with a diagnosis of pyloric stenosis were eligible. Primary outcomes were time to achieve full enteral feed and duration of postoperative recovery. We aimed to recruit 200 infants (100 per group); however, the data monitoring and ethics committee recommended halting the trial before full recruitment because of significant treatment benefit in one group at interim analysis. Participants, parents, and nursing staff were unaware of treatment. Data were analysed on an intention-to-treat basis with regression analysis. The trial is registered with ClinicalTrials.gov, number NCT00144924. FINDINGS Time to achieve full enteral feeding in the open pyloromyotomy group was (median [IQR]) 23.9 h (16.0-41.0) versus 18.5 h (12.3-24.0; p=0.002) in the laparoscopic group; postoperative length of stay was 43.8 h (25.3-55.6) versus 33.6 h (22.9-48.1; p=0.027). Postoperative vomiting, and intra-operative and postoperative complications were similar between the two groups. INTERPRETATION Both open and laparoscopic pyloromyotomy are safe procedures for the management of pyloric stenosis. However, laparoscopy has advantages over open pyloromyotomy, and we recommend its use in centres with suitable laparoscopic experience.

Esophageal Morbidity and Function in Adults With Repaired Esophageal Atresia With Tracheoesophageal Fistula A Population-Based Long-term Follow-up

Objective:We assessed esophageal morbidity and relationships between surgical complications, symptoms, endoscopic findings, immunohistochemistry, and esophageal motility in adults with repaired esophageal atresia (EA). Summary of Background Data:There exist no previous population-based long-term follow-up studies on EA. Methods:Participants were interviewed, and they underwent esophageal endoscopy and manometry. Matched control subjects (n = 287) served as controls. Results:A total of 101 (42%) individuals representative of the entire study population participated at a mean age of 36 years (range, 21–57). Symptomatic gastroesophageal reflux had occurred in 34% and dysphagia in 85% of the patients and in 8% and 2% of the controls (P < 0.001 for both). Endoscopic findings included hiatal hernia (28%), Barrett′s esophagus (11%), esophagitis (8%), and anastomotic stricture (8%). Immunohistochemistry revealed esophagitis in 25%, and CDX2-positive columnar epithelial metaplasia in 21%, with additional goblet cells and MUC2 positivity in 6%. Gastroesophageal reflux and dysphagia were equally common in individuals with normal histology, esophagitis, or epithelial metaplasia. Manometry demonstrated nonpropagating peristalsis in 80% of the patients, and low distal wave amplitudes of the esophagus in all the changes being significantly worse in those with epithelial metaplasia (P ≤ 0.022 metaplasia vs. esophagitis/normal). Anastomotic complications (odds ratio [OR]: 8.6–24, 95% confidence interval [CI]: 1.7–260, P = 0.011–0.008), age (OR: 20, 95% CI: 1.3–310, P = 0.034), low distal esophageal body pressure (OR: 2.6, 95% CI: 0.7–10, P = 0.002), and defective esophageal peristalsis (OR: 2.2, 95% CI: 0.4–11, P = 0.014) predicted development of epithelial metaplasia. Conclusions:Significant esophageal morbidity associated with EA extends into adulthood. Surgical complications, increasing age, and impaired esophageal motility predict development of epithelial metaplasia after repair of EA.

Bowel function and gastrointestinal quality of life among adults operated for Hirschsprung disease during childhood: a population-based study.

Objective: To assess bowel function and gastrointestinal quality of life among adults with operated Hirschsprungs disease (HD). Summary Background Data: Outcomes of HD extending to adulthood are unclear; bowel function and quality of life may deteriorate by aging. Methods: Bowel function and gastrointestinal quality of life were cross-sectionally assessed in a population-based manner among adults operated for HD during childhood between 1950 and 1986. Patients were interviewed during their outpatient visit. Controls matched for age and sex were randomly chosen from the Population Register Centre of Finland. Results: Ninety-two (64%) patients representative for the entire study population responded. The mean age of patients (79% male) was 43 (interquartile range [IQR], 35–48) years. Most (78%) had undergone Duhamel operation, and 94% had aganglionosis confined to the rectosigmoid. The mean overall bowel function score was decreased among patients (17.1 ± 2.8 vs 19.1 ± 1.2; P < 0.0001). They reported increased incidence of inability to hold back defecation (40% vs 17%), fecal soiling (48% vs 22%), constipation (30% vs 9%) and social problems related to bowel function (29% vs 11%; P < 0.05 for all). Gastrointestinal quality of life was only marginally lower among patients (121 ± 15.3 vs 125 ± 13.1; P = 0.0578) mainly because of significantly lower scores in questions assessing disease-specific factors such as bowel function and continence. Age was the only predictor of poor bowel function (OR 1.07, 95% CI 1.00–1.14, P = 0.049), which weakly predicted gastrointestinal quality of life (OR 0.81, 95% CI 0.66–1.01, P = 0.055). Conclusions: Although bowel function deteriorates with increasing age after operated HD, it is associated with only slightly decreased gastrointestinal quality of life.

Long-term Outcomes of Restorative Proctocolectomy in Children With Ulcerative Colitis

OBJECTIVE. Long-term outcomes of restorative proctocolectomy for pediatric-onset ulcerative colitis are unclear. METHODS. Questionnaires on health outcomes and quality of life were mailed to patients with childhood-onset ulcerative colitis who had undergone proctocolectomy with ileoanal anastomosis in 2 university hospitals between 1985 and 2005. Investigators not involved in the surgical management of the patients approached participants. Matched control children were randomly chosen from the Population Register Centre of Finland. RESULTS. Fifty-two (66%) patients and 117 (37%) controls responded. After a mean follow-up of 10 years, at least 1 surgical complication had occurred in 39 (75%) patients, and 28 (54%) had undergone reoperation. Only 1 failure of ileoanal anastomosis occurred. Ulcerative colitis had been reclassified as Crohn disease in 6 (12%) patients. Pouchitis occurred in 37 (73%) patients. The median stool frequency was 5 for day and 1 for night, but 46% used medication to control stool frequency. Nighttime soiling was reported by 56% of the patients. The mean overall quality-of-life score, the mean BMI (22 kg/m2 for both), and the number of subjects (aged >20 years) with offspring (14% vs 15%) was similar to the population-based controls. CONCLUSIONS. Stool frequency after restorative proctocolectomy in children with ulcerative colitis is stable and comparable to those of adult patients. Although nighttime incontinence is common, general health status and overall quality of life are comparable to the normal population.

Modern outcomes of oesophageal atresia: Single centre experience over the last twenty years

AIM OF THE STUDY The aim of this study was assessment of the contemporary outcomes of oesophageal atresia (OA) in a national paediatric surgical centre. PATIENTS AND METHODS A review of the hospital records of all patients who underwent repair of oesophageal atresia (OA) in our institute between 1991 and 2011 was performed. RESULTS The study included 130 consecutive infants with OA: types A (n=4, 3%), B (n=2, 2%), C (n=110, 85%), D (n=5, 4%), and E (n=9, 7%). Median follow-up was 8.8 (range 0.1-21) years. Twenty-nine (22%) infants had cardiac and 76 (58%) other anomalies, and seventeen (13%) had a long-gap OA. The final repair was primary (n =113, 87%) or delayed (n=3) end-to-end anastomosis, oesophageal replacement (n=8) (6%) with gastric tube (n=4) or with jejunum interposition (n=4), and closure of the trachea-oesophageal fistula (Type E, n=9). Oesophageal continuity was achieved in all patients. Overall mortality was 3/130 (2%) and caused by gastric perforation (n=1), prolonged apnoeic spell (n =1), and food asphyxiation (n=1). Oral feeds were achieved in 121 (94%) children. Eight (6%) children remain dependent on feeding ostomy. Long-gap OA was a major predictor of post-repair complications. CONCLUSION The modern outcome for infants with OA is characterized by an extremely low hospital mortality and satisfactory oesophageal function, enabling full oral feeds in the vast majority of children.

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

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. (Hepatology 2013;58:729–738)

National centralization of biliary atresia care to an assigned multidisciplinary team provides high-quality outcomes

Abstract Background and aims. Effects of caseload and organization of care on outcomes of biliary atresia (BA) remain unclear. We compared outcomes before and after national centralization of BA treatment in Finland with a population of 5.4 million people and 60,000 live births/year. Methods. All children born in Finland from 1987 to 2010 with BA were included. Complete patient identification was ascertained from the national Register of Congenital Malformations. Hospital records were reviewed for confirmation of the diagnosis, treatment, and follow-up data. Clearance of jaundice (serum bilirubin ≤20 μmol/l) and survival modalities were compared before and after centralization from five centers to Helsinki. Results. The incidence of BA was 1 in 20,100 live births. A total of 72 BA patients of whom 64 had undergone surgery for BA were identified. After centralization, the median caseload per center increased from 0 (range, 0–3) to 4 (2–5) patients/year (p < 0.001), clearance of jaundice rate increased from 27% to 75% (p = 0.001), 2-year jaundice-free native liver survival from 25% to 75% (p = 0.002), transplant-free survival from 27% to 75% (p = 0.005), and overall survival from 64% to 92% (p = 0.082). Baseline patient characteristics including type of BA and age at portoenterostomy remained unaltered. In a logistic regression analysis including treatment era, operating center, BA splenic malformation syndrome, and age at portoenterostomy as variables, only treatment in Helsinki after centralization predicted clearance of jaundice (odds ratio 4.2; 95% confidence interval 1.05–16.5; p = 0.043). Conclusions. In small countries, BA treatment should be centralized to appointed multidisciplinary teams allowing high quality results with a median of four cases/year.

Outcomes in pediatric patients undergoing straight vs J pouch ileoanal anastomosis: a multicenter analysis

BACKGROUND Outcomes remain controversial for patients undergoing straight (SIAA) vs J pouch (JPAA) ileoanal anastomosis, particularly in children where fewer such cases are performed. Our 3 centers have had extensive experience with both techniques. Thus, we had the unique opportunity to compare outcomes within the same centers. METHODS We retrospectively analyzed 250 children after proctocolectomy with either SIAA or JPAA, for the first 3 years after pull-through. A functional stooling score was developed to further assess outcomes. Data were analyzed using chi(2) tests and generalized linear mixed models for repeated measures. RESULTS Two hundred three patients had sufficient data for complete analysis (42% males; mean surgery age, 15 +/- 7years). Surgical indications were ulcerative colitis (168) and familial adenomatoid polyposis (35). Surgical procedures included SIAA (112) and JPAA (91). Daytime and nighttime stooling frequencies were significantly higher (P < .013) for SIAA patients at 1 to 24 months after pull-through; however, stooling frequencies began approximating each other by this time. Symptomatic pouchitis (compared to enteritis after SIAA) was significantly higher in JPAA patients (odds ratio, 4.5; confidence interval, 2.32-8.72). Frequency of pouchitis declined with time. There was no significant difference in the incidence of surgical complications between the 2 groups. Finally, continence rates were strikingly good in both groups compared to previously reported series. CONCLUSION Straight ileoanal anastomosis and JPAA are associated with considerable morbidity; SIAA has higher stool frequency and JPAA has increased pouchitis. Over time, we found that problems improved, and functional stooling scores became similar. JPAA had consistently lower stool frequency and better continence rates; however, these differences were small and may have minimal clinical significance. In addition, such differences need to be balanced against the high rate of pouchitis with JPAA. Continence was excellent regardless of the technique.

Long-term controlled outcomes after autologous intestinal reconstruction surgery in treatment of severe short bowel syndrome

AIM The aim of this study was to analyze outcomes of severe short bowel syndrome (SBS) treated with autologous intestinal reconstruction (AIR) surgery to facilitate independence of parenteral nutrition (PN). METHODS PN dependence, growth, nutritional status, liver function, and survival were comparatively assessed in SBS children treated with (n=10) or without (n=18) AIR surgery. RESULTS Median follow-up was 9.2 (4.9-14) years. Residual small intestinal length was 28 cm at birth. A total of 13 AIR procedures were performed at median age of 16 (11-41) months: serial transverse enteroplasty (STEP; n=8), longitudinal lengthening and tailoring (LILT; n=2), and tapering enteroplasty (n=3). Following STEP and LILT, length of the remaining small intestine increased by 19 (15-38) cm or 43% (38%-64%). Two children required repeat STEP due to recurrence of symptoms and bowel re-dilatation. Median duration of PN was 12 (11-17) months before and 14 (0-19) months after AIR. Eight children remain off PN 6.9 (3.6-9.7) years after surgery, and one with ultra short bowel (12 cm) receives 12% of energy as PN - all with reassuring growth and nutritional status and normal liver function. Actuarial PN dependence, including those who died on PN, was similar (P=0.19) with or without AIR surgery being 30% and 20% at four years, respectively. Complications of AIR surgery (lymphocele, bowel obstruction, and staple line bleeding) resolved without operative interventions. Survival was 90% with and 83% without AIR procedures. CONCLUSIONS AIR surgery can provide long-term survival, independence of PN, and satisfactory general health in selected children with severe SBS.

Gastroesophageal Reflux in Children With a Congenital Abdominal Wall Defect

BACKGROUND/PURPOSE Gastroesophageal reflux (GER) is considered common in patients with congenital abdominal wall defects (CAWD). The aim of this study was to find out the frequency of GER in children with CAWD and, in particular, whether there is difference between patients with omphalocele and gastroschisis. METHODS Forty-two children, 19 with gastroschisis and 23 with omphalocele were examined for GER at the median age of 12.0 months (range, 1 to 132). Esophagoduodenoscopy with biopsies was performed on all patients. Eighteen patients underwent 18-hour esophageal pH-monitoring. RESULTS GER was detected in 13 patients. All but one patient of the 13 had either macroscopic or microscopic esophagitis. One patient had pathological pH monitoring only. In children with omphalocele, the incidence of GER was 43% (10 of 23), whereas in gastroschisis patients the incidence was 16% (3 of 19), (P value, not significant). The median age of omphalocele patients with GER was significantly lower (7 months) than the median age of those without GER (72 months; P = .01). In patients with gastroschisis age made no difference. Six of 32 patients (19%) with primary fascial closure (small defects) had GER, whereas 7 of 10 patients (70%) in which primary fascial closure was impossible (large defects) had GER (P < .01). CONCLUSION CAWD patients, especially those with omphalocele and a large defect have a high incidence of GER complicated by esophagitis during the first few years of life.