Mikko P. Pakarinen

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.

Imperforate anus: long- and short-term outcome

Anorectal malformations are common anomalies observed in neonates. Survival of these babies is currently achieved in most cases and improvements in operative technique, patient care, and better follow-up have led to improved functional results. A new, simplified classification system (Krickenbeck classification) and method of functional assessment has led to an improved understanding of these anomalies and has allowed for a better comparison of outcomes. Following successful anatomical repair and appropriate programs of bowel care, socially acceptable continence can be achieved in a majority of patients, especially those with an intact sacrum.

Parenteral plant sterols and intestinal failure-associated liver disease in neonates.

Objectives: We prospectively evaluated incidence of prolonged (>28 days) parenteral nutrition (PN), associated complications, and significance of parenteral plant sterols (PS) in neonatal intestinal failure–associated liver disease (IFALD) compared with children. Methods: We recruited 28 neonates (mean age 50 days, range 28–126) and 11 children (6.9 y, 2.1–16.6) in all of Finland. Patients underwent repeated measurements of serum cholesterol, noncholesterol sterols, including PS, cholestanol and cholesterol precursors, and liver biochemistry during and 1 month after discontinuation of PN. Healthy matched neonates (n = 10) and children (n = 22) served as controls. Results: IFALD occurred more frequently among neonates (63%) than children (27%; P < 0.05). Ratios of serum PS, including stigmasterol, sitosterol, avenasterol, and campesterol, and total PS were increased among neonates compared with healthy controls and children on PN by 2- to 22- and 2- to 5-fold (P < 0.005), respectively. Neonates with IFALD had significantly higher ratios of serum PS and cholestanol compared with neonates without IFALD (P < 0.05). Total duration of PN associated with serum cholestanol, stigmasterol, avenasterol, alanine aminotransferase, and aspartate aminotransferase (r = 0.472–0.636, P < 0.05). Cholestanol and individual serum PS, excluding campesterol, reflected direct bilirubin (r = 0.529–0.688, P < 0.05). IFALD persisted after discontinuation of PN in 25% of neonates with 4.2- and 2.2-times higher ratios of serum stigmasterol and cholestanol compared with neonates without IFALD (P < 0.05). Conclusions: Frequent occurrence of IFALD among neonates on PN displays an association to duration of PN and markedly increased serum PS, especially stigmasterol, in comparison to healthy neonates and children on PN. Striking accumulation of parenteral PS may contribute to IFALD among neonates.

Outcome of esophageal atresia beyond childhood

Survivors of esophageal atresia are reaching their adulthood in large numbers for the first time enabling assessment of true long-term outcome among this group of patients. This review summarizes the current knowledge on the subject focusing on late symptoms and complications, esophageal pathology and pulmonary function. Relationships between esophageal dysmotility, gastroesophageal reflux, esophagitis and epithelial metaplastic changes including esophageal cancer are outlined. In addition to pertinent literature, institutional experience, and follow-up of patients with esophageal atresia for more than 60 years is included.

Operative treatment of fractures in children is increasing. A population-based study from Finland.

BACKGROUND Epidemiological data on the incidence of surgical treatment of pediatric fractures are sparse. Our aim was to determine the incidence of in-hospital-treated fractures and of the surgical treatment of these fractures in children and adolescents. METHODS National Discharge Register data on pediatric fractures (in patients younger than the age of eighteen years) treated in the hospital in Finland between 1997 and 2006 were evaluated. RESULTS During the ten-year follow-up period, the incidence (per 100,000 persons) of fractures leading to hospitalization increased by 13.5% (from 319 in 1997 to 362 in 2006; p < 0.001). This change resulted mainly from an increase in the incidence of hospital-treated upper-extremity fractures (23% increase; from 189 in 1997 to 232 in 2006). The incidence of primary fracture surgery increased by 20% (from 237 in 1997 to 284 in 2006; p < 0.001). The incidences of surgery for upper-extremity, lower-extremity, and axial fractures increased by 28%, 3.9%, and 10.7%, respectively. Within the upper-extremity-fracture group, the incremental increase was mainly due to an increase in forearm fracture surgery (62% increase; from fifty-five in 1997 to eighty-nine in 2006) (p < 0.001). CONCLUSIONS Operative treatment of childrens fractures has increased markedly during the last ten years. Evidence-based medical and economic data supporting this change in practice are sparse.

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.

Intestinal adaptation after massive proximal small-bowel resection in the pig

BACKGROUND Small-intestinal adaptation to resection has been extensively studied in rats. The present study investigates morphology, crypt cell proliferation, and disaccharidase activities of the remaining small intestine and colon after 75% proximal resection of porcine small intestine. METHODS Specimens were obtained from the proximal jejunum, middle and distal ileum, and proximal colon preoperatively (n = 5) and 14 weeks after small-bowel transection (n = 5) or resection (n = 5). Proliferation was analyzed immunohistochemically with the Ki-67 antigen MIB-1. Disaccharidase activities were determined in accordance with the method of Dahlqvist. RESULTS In addition to macroscopic enlargement, resection markedly increased the villi and crypts of the remaining small bowel. Crypt cell proliferation decreased with advancing age after transection but remained at the preoperative level after resection. Specific, but not total, activities of maltase and sucrase in the mid-ileum decreased after resection. CONCLUSION Small-intestinal adaptation in the pig involves macroscopic enlargement and a prompt increase in villus size, which is associated with high crypt cell proliferation.

Long-term outcomes of Hirschsprung's disease

Despite significant developments in the understanding of the pathologic anatomy and physiology of Hirschsprungs disease (HD), the results of surgical therapy remain far from perfect. The functional defects and psychosocial difficulties that occur commonly in children with HD are passed on to adulthood in a significant proportion of patients. Recent prospective and adequately controlled cross-sectional studies reveal that constipation and fecal soiling are common late sequelae in adulthood. HD patients show uniformly lower scores of overall bowel function than healthy control subjects. Functional outcome and quality of life may be interrelated and deteriorate with increasing age. Despite these shortcomings, at adult age, most of the HD patients appear to be able to function as normal members of the society in terms of psychosocial, occupational, and recreational activities. Patients are at risk of developing related conditions, such as cancer, that require planning of specific screening programs. Many of the long-term problems that these patients experience are specific to HD. The follow-up of HD patients should be performed by medical personnel familiar with the disease, preferably in a specialized referral center, and the follow-up should continue beyond childhood.