Functional Outcomes After Surgery for Total Colonic, Long-Segment, Versus Rectosigmoid Segment Hirschsprung Disease.

OBJECTIVES: Knowledge on long-term outcomes in patients with Hirschsprung disease is progressing. Nevertheless, differences in outcomes according to aganglionic lengths are unclear. We compared long-term bowel function and generic quality of life in Hirschsprung patients with total colonic or long-segment versus rectosigmoid aganglionosis. METHODS: In this nationwide, cross-sectional study participants with proven Hirschsprung disease received the Defecation and Fecal Continence questionnaire, and the Child Health Questionnaire Child Form-87, or the WHO Quality of Life-100. We excluded deceased patients, patients who were younger than 8 years, lived abroad, had a permanent enterostomy, or were intellectually impaired. RESULTS: The study population (n = 334) was operated for rectosigmoid (83.9%), long-segment (8.7%), or total colonic aganglionosis (7.5%). Fecal incontinence in general was not significantly different between the three groups, but liquid fecal incontinence was significantly associated with total colonic aganglionosis (odds ratio [OR] = 6.00, 95% confidence interval [CI] 2.07-17.38, P = 0.001). Regarding constipation, patients with total colonic or long-segment aganglionosis were less likely to suffer from constipation than the rectosigmoid group (OR = 0.21, 95% CI, 0.05-0.91, P = 0.038 and OR = 0.11, 95% CI, 0.01-0.83, P = 0.032). Quality of life was comparable between the three groups, except for a lower physical score in children with total colonic aganglionosis (P = 0.016). CONCLUSIONS: Over time Hirschsprung patients with total colonic or long-segment aganglionosis do not suffer from worse fecal incontinence in general. A difference in stool consistency may underlie the association between liquid fecal incontinence and total colonic aganglionosis and constipation in patients with rectosigmoid aganglionosis. Despite these differences, generic quality of life is comparable on reaching adulthood.

Did Age at Surgery Influence Outcome in Patients With Hirschsprung Disease? A Nationwide Cohort Study in the Netherlands.

OBJECTIVES: Hirschsprung disease (HD) requires surgical resection of affected bowel, but the current evidence is inconclusive regarding the optimal age for resection. The aim of this study was to assess whether age at resection of the aganglionic segment is a determinant for surgical outcomes. METHODS: A cross-sectional cohort study was done including all consecutive patients with HD between 1957 and 2015, aged 8 years or older (n = 830), who were treated in 1 of the 6 pediatric surgical centers in the Netherlands. Outcome measures were mortality, postoperative complications, stoma rate and redo surgery rate, retrieved from the medical records. Additionally, constipation and fecal incontinence rate in long term were assessed with the Defecation and Continence Questionnaire (DeFeC and P-DeFeC). RESULTS: The medical records of 830 patients were reviewed, and 346 of the 619 eligible patients responded to the follow-up questionnaires (56%). There was a small increase in the risk of a permanent stoma [odds ratio (OR) 1.01 (95% confidence interval {CI}: 1.00-1.02); P = 0.019] and a temporary stoma [OR 1.01 (95% CI: 1.00-1.01); P = 0.022] with increasing age at surgery, regardless of the length of the aganglionic segment and operation technique. Both adjusted and unadjusted for operation technique, length of disease, and temporary stoma, age at surgery was not associated with the probability and the severity of constipation and fecal incontinence in long term. CONCLUSIONS: In this study, we found no evidence that the age at surgery influences surgical outcomes, thus no optimal timing for surgery for HD could be determined.

Quality of Life in Parents of Children With Biliary Atresia.

OBJECTIVES: The aim of the study was to determine quality of life (QoL), stress, and anxiety levels in parents of children with biliary atresia (BA), and to assess factors associated with parental QoL. METHODS: Parents of children (6-16 years) with BA were included in this cross-sectional study. We used validated questionnaires to assess parental QoL, stress, and anxiety levels. We compared the results with reference data from the general population and determined associated factors using generalized linear mixed model analysis. Results are given as mean ±â€ŠSD or median [min-max]. RESULTS: We included 61 parents of 39 children (aged 11 ±â€Š3 years). Thirty-one children (79%) had undergone a liver transplantation (LTx). Parents reported reduced family activities (88 [8-100] vs 95 [30-100], P = 0.002) and more emotional worry (83 [17-100] vs 92 [95-100], P < 0.001) compared with reference data, but a stronger family cohesion (85 [30-100] vs 60 [30-100], P = 0.05). Scores on parental QoL, anxiety and stress were similar to reference data. Fathers (16.0 [11-19]) and mothers (15.4 ±â€Š1.4) scored higher on the psychological domain compared with reference data (vs 14.7 ±â€Š2.2, P < 0.01). There was no significant difference in QoL of parents with children with native liver or those who had undergone LTx. Older age and high anxiety trait in parents were adversely associated with physical QoL. Household income below &OV0556;35 000/year and high anxiety trait were adversely associated with environmental QoL. CONCLUSIONS: QoL in parents of school-aged children with BA appears to be unaffected. Parents with high-anxiety personality trait, older age, and low household income are at increased risk of impaired QoL.

Does Mechanical Bowel Preparation Reduce the Risk of Developing Infectious Complications in Pediatric Colorectal Surgery? A Systematic Review and Meta-Analysis.

OBJECTIVES: To evaluate whether the application of mechanical bowel preparation (MBP) before colorectal surgery reduces the risk of developing infectious complications in children. STUDY DESIGN: In this systematic review and meta-analysis, PubMed, Embase, and the Cochrane Library were systematically searched to identify all articles comparing pediatric patients receiving MBP with pediatric patients not receiving MBP before colorectal surgery. Results are presented with weighted risk differences based on the number of events and sample size per study. RESULTS: Six original studies were included comparing MBP (n = 810) and no MBP (n = 1167). The overall risk of developing infectious complications was 10.1% in patients with MBP, compared with 9.1% in patients without MBP, resulting in a nonsignificant risk difference of -0.03% (95% CI, -0.09% to 0.03%). Concerning the number of wound infections and anastomotic leaks, we found nonsignificant risk differences of -0.03% (95% CI, -0.08% to 0.02%) and 0.01% (95% CI, -0.01% to 0.02%), respectively. CONCLUSION: Based on the current literature, there is insufficient evidence to indicate that the use of MBP leads to a significant difference in the risk of developing infectious complications in pediatric colorectal surgery.

The Diagnostic Accuracy of Serum Alpha-Fetoprotein Levels in Follow-up for Recurrence of Sacrococcygeal Teratoma; a Nationwide Review of SCT Cases in the Netherlands.

BACKGROUND: Serum alpha-fetoprotein (AFP) is often used as tumour marker for recurrent sacrococcygeal teratoma (SCT). We aimed to assess the normal dynamics of serum AFP levels after initial resection and diagnostic accuracy of serum AFP levels the follow-up for recurrence in SCT. METHODS: This retrospective study included 57 patients treated for SCT in the six pediatric surgical centers in the Netherlands from 1980 to 2018. MAIN RESULTS: 57 patients were included in the study of whom 19 children developed 20 recurrences at a median of 14.0 months after initial resection. No significant difference was found in serum AFP level dynamics between the recurrence and non-recurrence group after initial resection (p = 0.950). Serum AFP levels did not significantly increase before recurrence (p = 0.106) compared to serum AFP levels of children without recurrence at the same time. However, serum AFP levels did significantly increase in malignant recurrences (n = 7) (p = 0.03) compared to patients without recurrence. A cut-off value of 55 µg/L was found to be predictive for recurrent SCT with an Area Under the Curve (AUC) of 0.636 with sensitivity of 50% and specificity of 100%. CONCLUSION: Dynamics of serum AFP levels are not different between patients with and without recurrence after initial resection of SCT. Serum AFP levels are not predictive for mature or immature recurrent SCT and normal AFP levels do not rule out recurrent SCT. However, serum AFP levels exceeding 55 µg/L can indicate recurrent SCT, especially malignant recurrences.

Parental wellbeing after diagnosing a child with biliary atresia: A prospective cohort study.

PURPOSE: To determine anxiety, stress, and quality of life (QoL) in parents of children who are diagnosed with biliary atresia (BA). METHODS: Parents of BA patients (0-3 years) completed validated questionnaires at three time points: at first hospitalization (T0); 1-2 months post diagnosis (T1); and 2-3 years post diagnosis (T2). Results are presented in medians (min-max). RESULTS: We included 52 parents (age 31 [24-51 y], 31 females) of 30 BA patients. In fathers, neither anxiety nor stress levels significantly differed from reference values. Mothers reported significantly higher anxiety levels compared to reference values (T0: 48 vs 35, p = 0.001; T1: 43 vs 35, p = 0.03; T2: 37 vs 35, p = 0.04), which significantly decreased over time (-23% between T0 and T2: p = 0.04). Stress in mothers was significantly higher at T1 than at T2 (+35%, p = 0.02), but was not significantly different from reference values at each time point (T0: 17 vs 14, p = 0.07; T1: 18 vs 14, p = 0.09; T2: 13 vs 14, p = 0.52).The overall QoL in mothers and fathers was rather unaffected. CONCLUSIONS: Particularly mothers of infants diagnosed with BA report high anxiety levels up to three years after diagnosis. The overall QoL of parents is rather unaffected after diagnosing BA in their child. LEVEL OF EVIDENCE: Level 2.

Familial Experience With Hirschsprung's Disease Improves the Patient's Ability to Cope.

Introduction: Familial occurrence of Hirschsprung's disease may have a positive effect on patients' ability to cope with the disease. The aim was to compare long-term bowel function and generic quality of life between patients with familial and non-familial Hirschsprung's disease. Methods: This was a nationwide, cross-sectional study in which we included all 830 Hirschsprung patients of 8 years and older who had undergone surgery between 1957 and 2015. We excluded patients with a permanent stoma, intellectual disability, or an unknown or foreign address. We requested patients to complete the validated pediatric or adult Defecation and Fecal Continence questionnaire and the Child Health Questionnaire Child Form-87, or the World Health Organization Quality of Life-100 Assessment Instrument. Results: We analyzed 336 Hirschsprung patients, 15.8% of whom were familial cases and 84.2% were non-familial cases. After adjusting for aganglionic length, sex, and age, patients with familial Hirschsprung's disease were twice more likely to suffer from constipation (OR = 2.47, 95% CI, 1.21-5.05, p = 0.013). The quality of life of the pediatric patients was comparable, but in adult patients the energy/fatigue, thinking/learning/concentration, and work capacity facets showed better scores in the familial patients with Hirschsprung's disease of the rectosigmoid (p = 0.029, p = 0.024, p = 0.036, respectively). Conclusions: Different facets of generic quality of life are better in adult patients with familial Hirschsprung's disease of the rectosigmoid. It seems that familial experience with the disease influences patients' coping abilities positively.

Thoracoscopic Repair of Esophageal Atresia.

Background: Esophageal atresia (EA) is a rare congenital malformation of the esophagus. Surgical treatment is required to restore the continuity of the esophagus. This can be performed through thoracotomy. However, an increasing number of hospitals is performing minimal invasive surgery (MIS). In this article, we describe the technique of thoracoscopic repair of EA in neonates in more detail and show the outcome of a patient cohort operated by young pediatric surgeons in training. Methods: Between 2014 and 2019 correction was performed in 64 EA type C patients at the UMC Utrecht, Wilhelmina Children's Hospital, mainly by young pediatric surgeons in training. Results: All patients were corrected through MIS, 3 days after birth. The median operation duration was 181 (127-334) minutes. Nasogastric tube feeding was started on the first postoperative day, and oral feeding 6 days postop. Postoperative complications included leakage (14.1%), stenosis (51.1%), and recurrent tracheoesophageal fistula (7.8%). Conclusion: Thoracoscopic repair of EA can be performed safely, with good outcome and all the benefits of MIS. However, it remains a challenging procedure and should be performed only in pediatric centers with a vast experience in MIS, especially when training young pediatric surgeons. These centers must have access to a multidisciplinary team of neonatologists, pediatric anesthesiologists, surgeons, and ENT specialists to ensure the best possible care in hemodynamic, respiratory, and cerebral monitoring and gastrointestinal and developmental outcome.

The Dutch Incidence of Infantile Hypertrophic Pyloric Stenosis and the Influence of Seasons.

INTRODUCTION: Studies report contradicting results on the incidence of infantile hypertrophic pyloric stenosis (IHPS) and its association with seasons. We aim to assess the IHPS incidence in the Netherlands and to determine whether seasonal variation is present in a nationwide cohort. MATERIALS AND METHODS: All infants with IHPS hospitalized in the Netherlands between 2007 and 2017 were included in this retrospective cohort study. Incidence rates per 1,000 livebirths (LB) were calculated using total number of LB during the matched month, season, or year, respectively. Seasonal variation based on month of birth and month of surgery was analyzed using linear mixed model and one-way ANOVA, respectively. RESULTS: A total of 2,479 infants were included, of which the majority was male (75.9%). Median (interquartile range) age at surgery was 34 (18) days. The average IHPS incidence rate was 1.28 per 1,000 LB (variation: 1.09-1.47 per 1,000 LB). We did not find a conclusive trend over time in IHPS incidence. Differences in incidence between season of birth and season of surgery were not significant (p = 0.677 and p = 0.206, respectively). CONCLUSION: We found an average IHPS incidence of 1.28 per 1,000 LB in the Netherlands. Our results showed no changing trend in incidence and no seasonal variation.

Health-Related Quality of Life and Psychosocial Morbidity in Anorectal Malformation and Hirschsprung's Disease.

Anorectal malformation (ARM) and Hirschsprung's disease (HD) are the most common congenital colorectal anomalies. Despite advances in surgical techniques and improvements in postoperative clinical care, short- and long-term physical and psychosocial morbidity of these patients remains high. This review outlines the current literature on the physical and psychosocial aspects of health-related quality of life (HrQoL) and its confounders in patients with ARM/HD. Moreover, important coping strategies are summarized to further improve HrQoL of ARM/HD patients and their families.

Health-Related Quality of Life in Biliary Atresia Patients with Native Liver or Transplantation.

INTRODUCTION: We aimed to assess health-related quality of life (HrQoL) in biliary atresia (BA) patients, based on original data and a literature review, and to determine factors associated with their HrQoL. MATERIALS AND METHODS: We reviewed available studies describing HrQoL in BA patients. We assessed HrQoL in Dutch BA patients (6-16 years) using the validated Child Health Questionnaire. We compared HrQoL scores in BA patients with healthy peers and with children who had undergone major surgery in infancy or children with chronic conditions. We determined the relationship between specific patient-related factors and HrQoL. RESULTS: Literature data indicated that HrQoL in children with BA is lower than in healthy peers. In Dutch BA patients (n = 38; age 10 ± 3 years), parent-proxy physical HrQoL (48 ± 11) was significantly lower compared with two reference groups of healthy peers (59 ± 4 and 56 ± 6, respectively, each p < 0.001), and lower than in children with attention deficit hyperactivity disorder (60 ± 5), asthma (54 ± 6), attending a cardiology clinic (52 ± n/r), congenital diaphragmatic hernia (53 ± 7) or D-transposition of the great arteries (54 ± 6; all p < 0.05). Psychosocial HrQoL (50 ± 9) was lower than in healthy peers (54 ± 6, p = 0.02, and 53 ± 6, p = 0.07) and children with asthma (54 ± 6, p = 0.02), and largely comparable to children with other chronic conditions. Parent-proxy physical HrQoL was adversely related to adverse medical event in the past year, special education, and motor impairments; psychosocial HrQoL was adversely related to behavioral problems. CONCLUSION: Children with BA are at risk of impaired HrQoL. Special attention is warranted for children with adverse medical events and special education.

The fat embolism syndrome as a cause of paraplegia.

The fat embolism syndrome is a well-known complication in trauma patients. We describe a rare case of traumatic fat embolism that leads to paraplegia. A 19-year-old male motorcycle accident victim was presented to our hospital. After stabilization and trauma survey, he was diagnosed with bilateral femur fractures, a spleen laceration and a tear in the inferior vena cava, for which damage control surgery was performed. Post-operatively, the patient became paraplegic and developed a fluctuating consciousness, respiratory distress and petechiae. Fat embolism syndrome was considered as the most plausible cause of the paraplegia. The fat embolism syndrome is seen in approximately 1% of trauma patients, mostly those with bilateral fractures of the femur. Prevention of the syndrome depends on early stabilization of fractures. However, even with optimal care, this syndrome can still occur and may have dramatic consequences, as we demonstrate in this case.

Esophageal Atresia and Upper Airway Pathology.

Esophageal atresia is an anomaly with frequently occurring sequelae requiring lifelong management and follow-up. Because of the complex issues that can be encountered, patients with esophageal atresia preferably should be managed in centers of expertise that have the ability to deal with all types of anomalies and sequelae and can perform rigorous lifelong follow-up. Tracheomalacia is an often-occurring concurrent anomaly that may cause acute life-threatening events and may warrant immediate management. In the past, major thoracotomies were necessary to carry out the aortopexy. Nowadays, aortopexy and posterior tracheopexy can both be performed thoracoscopically with quick recovery.

Quality of Life and Anxiety in Parents of Children with an Anorectal Malformation or Hirschsprung Disease: The First Year after Diagnosis.

INTRODUCTION: In 2012, we started the KLANKbord-study. A quality of life (QoL) study that follows patients with an anorectal malformation (ARM) or Hirschsprung disease (HD) and their parents from diagnosis till the age of 18 years. We hypothesized that the diagnosis of ARM or HD initially has a negative influence on QoL and anxiety levels of parents, but that this influence will diminish over time. The aim of this study is to see whether QoL and anxiety levels of parents change within the first year after the diagnosis. METHODS: Parents of all children born with ARM or HD, were eligible for this study. Within 3 months after the diagnosis ARM or HD, parents received a set of validated QoL questionnaires (measurement 1). Measurement 2 was 12 months after the first questionnaire. MAIN RESULTS: During measurement 1 mothers (n = 20) scored significantly higher on the social (p value, 0.01; 95% confidence interval [CI], 0.3946-3.1528) and environmental domain (p value, 0.01; 95% CI, 0.4449-2.2851) of the World Health Organization Quality of Life-BREF (WHOQOL-BREF) compared with the known reference values. Fathers (n = 19) scored significantly higher on the physical (p value, 0.01; 95% CI, 0.2964-1.8072), psychological (p value, 0.001; 95% CI, 0.7697-2.4757), and environmental domain (p value, 0.003; 95% CI, 0.5586-2.4214) than the reference values. Comparison of all domains of the WHOQOL-BREF for mothers and fathers between measurements did not show a significant difference. Anxiety levels of mothers were lower during measurement 2 compared with measurement 1. Anxiety levels of fathers were higher during measurement 2 compared with measurement 1. These differences are not significant. Anxiety levels of mothers were significantly higher than anxiety levels of fathers during measurement 1 (p value, 0.002; 95% CI, 0.808-2,956). During measurement 2 this difference in anxiety of mothers versus fathers did not exist (p value, 0.373; 95% CI, -1.157 to 2.922). CONCLUSION: A negative influence on the QoL of parents having a child with ARM or HD, compared with the reference population was not seen in this population. QoL did not change significantly during the first year for both fathers and mothers. Anxiety levels of mothers did decline during this first year. The number of parents included in this study is still small, which might influence our results. Therefore, we will continue the KLANKbord-study indefinitely.

Qualitative analysis of studies concerning quality of life in children and adults with anorectal malformations.

INTRODUCTION: Anorectal malformations are relatively common congenital anomalies in pediatric surgery. After definitive surgery constipation, soiling, and fecal incontinence are frequently seen problems. Quality of life (QoL) can be influenced by these problems. In the last decades QoL has become an important aspect in the treatment and follow-up of patients with anorectal malformations. This has resulted in various reports concerning QoL. In order to deduce whether the drawn conclusions in the different studies are correct and can be used to adjust standard care for patients with ARM, a qualitative analysis of the studies was performed. MATERIAL AND METHODS: A literature study was performed in PubMed, Psychinfo, Web of Science, and the Cochrane Library (240 hits). Thirty articles were used, following application of our inclusion criteria and in-depth analysis of the articles. A methodological qualitative analysis was also performed and QoL outcome assessed. RESULTS: Six authors (20.0%) used validated QoL questionnaires. Four articles were longitudinal and had more than one measure moment. Eleven studies (36.7%) used only non-validated questionnaires, and eight studies (26.6%) used only validated questionnaires. Nineteen studies correlated fecal continence to QoL, and seven studies established no correlation. Three of these seven studies used validated QoL questionnaires. All twelve studies, which did establish a correlation, used non-validated QoL questionnaires. CONCLUSIONS: Approximately 83% of the studies had not used validated QoL questionnaires. Further, conclusions concerning QoL were often based on functional outcomes, for example fecal incontinence. So far, longitudinal high quality research on QoL in this group has not been achieved.

Historical Treatment Results of Pelvic Ring Fractures: A 12-year Cohort Study.

BACKGROUND: Since the Academic Medical Center Amsterdam was appointed as a level-1 trauma center in July 1997, the number of polytrauma patients who were presented has increased. This stimulated us to perform a retrospective analysis on the treatment results of patients with a pelvic ring fracture and to evaluate our treatment strategies. MATERIALS AND METHODS: A chart review of all patients with a partially stable fracture (Tile/AO type B) or an unstable fracture (Tile/AO type C) was performed. All patients presented between 1 January 1990 and 31 December 2001 were included. Two historical groups (1990-1997 and 1998-2001) were formed. General demographics, treatment method, complications, re-operations, length of hospital stay and anatomic results were recorded for all patients. RESULTS: Fifty-two patients were included in group 1 and 65 patients in group 2. There was a lower mortality in group 2. The B-fractures were treated either conservatively (group 1 83.3% vs. group 2 73.8%), by external fixation (16.7 vs. 9.5%) or by ORIF (0 vs. 16.7%). C-fractures were treated by ORIF in 32.1 versus 82.6%, by external fixation in 28.6 versus 4.4% and conservatively 39.3 versus 13.0%, respectively. Fracture healing with less than 10 mm displacement was achieved in 58.3 versus 78.6% for the B-fractures, while this was achieved in 42.9 versus 73.9% in the C-fractures. Group 2 showed significantly fewer complications. CONCLUSION: Evaluating two consecutive patient groups shows an increase in the number of fractures. A more aggressive surgical treatment has lead to lower mortality, improved anatomical reduction, and lower rate of complications.