Imaging in pediatric appendicitis is key to a low normal appendix percentage: a national audit on the outcome of appendectomy for appendicitis in children.

PURPOSE: A laparoscopic approach for emergency appendectomy is increasingly used, in pediatric patients as well. The objective of this study is to audit the current state of diagnostic work-up, surgical techniques and its outcome in children with acute appendicitis. METHODS: A prospective consecutive observational cohort study was carried out in a 2-month study period. All patients under 18 years that were operated for suspected acute appendicitis were included. Primary outcome was the infectious complication rate after open and laparoscopic approach; secondary outcomes were preoperative use of imaging and post-operative predictive value of imaging, normal appendix rate and children with a postoperative ileus. RESULTS: A total of 541 children were operated for suspected acute appendicitis in 62 Dutch hospitals. Preoperative imaging was used in 98.9% of children. The normal appendix rate was 3.1%. In 523 children an appendectomy was performed. Laparoscopy was used in 61% of the patients and conversion rate was 1.7%. Complicated appendicitis was diagnosed in 29.4% of children. Overall 30-day complication rate was 11.9% and similar after open and laparoscopic. No difference was found in superficial surgical site infections, nor in intra-abdominal abscesses between the open and laparoscopic approach. Complicated appendicitis is an independent risk factor for infectious complications. CONCLUSION: The laparoscopic approach is most frequently used, except for young children. Superficial surgical site infections are more frequent after open surgery only in patients with complicated appendicitis. The normal appendix rate is low, most likely because of routine preoperative imaging.

[Murphy's Law]. / Appendicitis, appendectomie en de wet van Murphy.

Non-surgical treatment of acute phlegmonous appendicitis has been receiving increasing attention in recent years, representing a reversal of policy. The appendectomy came into vogue at the beginning of the 20th century. It is true that prompt surgical intervention in all patients with appendicitis or an indication thereof almost guarantees success, but it gradually came to be forgotten that this intervention was not always necessary. In this article we will document the historical development of this disease and its treatment, and return to the original reports made by pathologist Reginald Fitz and the internist William Osler to show that their opinion was not black and white. Many surgeons in the Netherlands were also initially more restrained. In the course of time recognition of the natural progression of the disease vanished, until new developments placed this more sharply in the limelight.

The impact of disease severity, age and surgical approach on the outcome of acute appendicitis in children.

PURPOSE: Although a national guideline has been implemented, the optimal approach for appendectomy in children remains subject of debate in the Netherlands. Opponents of laparoscopy raise their concerns regarding its use in complex appendicitis as it is reported to be associated with an increased incidence of intra-abdominal abscesses. The aim of this study was to evaluate the outcome of surgical approaches in both simple and complex appendicitis in paediatric patients. METHODS: A 10-year retrospective cohort study was performed (2001-2010) in paediatric patients treated for suspected acute appendicitis. Patients were divided into either simple or complex appendicitis and into different age groups. Primary outcome parameters were complication rate (intra-abdominal abscess (IAA), superficial surgical site infection (SSI) and readmission) and hospital stay. RESULTS: In total, 878 patients have been treated (median age 12, range 0-17 years). Two-thirds of the patients younger than 6 years had complex appendicitis, compared to one quarter in the group aged 13-18. In the complex appendicitis group, LA was associated with more IAA and early readmissions. In the simple appendicitis group, the complication rate was comparable between the two approaches. Significantly more IAAs were seen after LA in the youngest age group. CONCLUSION: This study demonstrates the unfavourable outcome of LA in the youngest age group and in patients with complex appendicitis. Therefore, we advise to treat these patients with an open approach.

Uptake of (18)F-fluoro-2-deoxyglucose in the Healthy Testes of Young Men as Assessed by Positron Emission Tomography/Computed Tomography; Including the Inter- and Intra-observer Variation.

Knowledge of the physiological testicular accumulation of (18)F-fluoro-2-deoxyglucose (FDG) is essential in order to discriminate between normal and pathological findings. In this study, the (18)F-FDG-uptake in healthy testes of young men was assessed using positron emission tomography/computed tomography (PET/CT)-scans. A total of 40 testes of 20 men with a mean age of 26.5 ± 3.9 years were evaluated. (18)F-FDG-uptake was expressed as the standardized uptake value (SUV). Testicular volume was measured on CT and PET. All scans were assessed by three researchers, one of whom assessed every scan twice. Laterality indices and inter- and intra-observer variation were evaluated. Correlation between the SUVmax and SUVpeak, between SUVmean and SUVpeak and between age and SUVpeak were assessed. Testes showed an average SUVmax of 3.42 ± 0.61, SUVpeak of 3.06 ± 0.54 and SUVmean of 2.44 ± 0.44. The average testicular volume on CT was 23.0 ± 6.4 ml, whereas on PET it was 18.0 ± 5.1 ml. Laterality indices were calculated of 0.077 ± 0.065 (SUVmax), 0.074 ± 0.066 (SUVpeak), 0.072 ± 0.063 (SUVmean), 0.245 ± 0.259 (CT), and 0.200 ± 0.188 (PET), respectively. Inter- and intra-observer reliability were found to be perfect for the SUVs (intraclass correlation coefficient [ICC] 0.992-1.0), but poor for testicular volumes (ICC 0.854-0.902). Testicular (18)F-FDG uptake in young men can be measured accurately on PET/CT and shows high symmetry. Consequently, (18)F-FDG PET/CT has the potential to become a useful instrument in the evaluation of the functioning of the individual testis.

Is complete surgical resection of stage 4 neuroblastoma a prerequisite for optimal survival or may >95 % tumour resection suffice?

Numerous studies have shown that for optimal survival in localized International Neuroblastoma Staging System stage 1-3 neuroblastoma, complete tumour resection (CR, macroscopic total tumour removal) is usually mandatory. In contrast, it is conceivable that in stage 4 disseminated disease, less extensive surgery [gross total resection (GTR), >95 % tumour removal] may suffice. This review shows substantial survival benefit in studies reporting on stage 4 patients undergoing CR, but also in studies reporting on patients undergoing GTR. Comparison between these studies is severely hampered by treatment heterogeneity. We found only four studies that explicitly compared survival between patients undergoing either CR or GTR. Two of these studies showed favourable results for patients treated with CR, while the other two did not show differences in survival.

Course of life into adulthood of patients with biliary atresia: the achievement of developmental milestones in a nationwide cohort.

PURPOSE: To investigate the course of life of young adults diagnosed with biliary atresia (BA) in infancy by comparing patients who did and did not underwent transplantation with an age-matched Dutch reference group. METHODS: All patients from the Dutch BA registry, aged >18 years, were invited to complete the course of life questionnaire. RESULTS: Forty patients participated (response = 74%). Twenty-five had not undergone transplantation; 15 had undergone orthotopic liver transplantation. One significant between-group difference was found, namely in substance use and gambling. BA patients who underwent transplantation reported less use than the reference group (p = .01, moderate effect size). Additional moderate effect sizes were found for differences in psychosexual and social development and antisocial behavior. Patients who underwent transplantation had lower scores than one or both other groups. CONCLUSIONS: Development of BA survivors who did not undergo transplantation seems not delayed, whereas that of transplanted patients does seem somewhat delayed. However, patients who underwent transplantation display less risk behavior. Larger samples are necessary to confirm these findings.

Open versus laparoscopic pyloromyotomy for hypertrophic pyloric stenosis: a systematic review and meta-analysis focusing on major complications.

BACKGROUND: There is an ongoing debate about whether laparoscopic pyloromyotomy (LP) or open pyloromyotomy (OP) is the best option for treating hypertrophic pyloric stenosis (HPS). The aim of this study was to compare the results of both surgical strategies by means of a systematic review and meta-analysis of the available literature. METHODS: A systematic search for randomized clinical trials (RCTs) comparing OP and LP was conducted. Studies were reviewed independently for quality, inclusion and exclusion criteria, and outcomes. Primary outcome was major postoperative complications (i.e., incomplete pyloromyotomy, perforation, and need for reoperation). Secondary outcomes were time to full feed, postoperative hospital stay, and any other postoperative complications. RESULTS: Four RCTs with a total of 502 patients (OP 255, LP 247) fulfilled the inclusion criteria and were analyzed in this review. These trials showed an absolute incidence of major postoperative complications of 4.9% in the LP group. Meta-analysis showed that LP did not lead to significantly more major postoperative complications (ARR 3%, 95% CI -3 to 8%) than OP. The mean difference in time to full feed was significant (2.27 h, 95% CI -4.26 to -0.29 h) and the mean difference in postoperative hospital stay tended to be shorter (2.41 h, 95% CI -6.10 to 1.28 h), both in favor of LP. CONCLUSION: So far, the major postoperative complication rate after LP for HPS is not substantially higher than after OP. Because time to full feed and postoperative hospital stay are at best a few hours shorter after LP than after OP, the laparoscopic technique might be acknowledged as the standard of care if the major postoperative complication rate is low. Hence, this laparoscopic procedure should preferably be performed in centers with pediatric surgeons with expertise in this procedure.

Bilateral proximal tibia fracture.

A bilateral fracture of the proximal tibia is rare in children. We describe a girl with a bilateral fracture just distal of the epiphyseal plate after minimal trauma.

Issues in the management of simple and complex meconium ileus.

INTRODUCTION: Various surgical methods are used to treat meconium ileus (MI), including resection with enterostomy (RES), primary anastomosis (RPA), and purse-string enterotomy with intra-operative lavage (PSI). The aim of this study is to discuss the surgical treatment of MI, based on our experience. PATIENTS AND METHODS: Of the 41 MI patients treated at our institution between 1984 and 2007, 18 had simple MI and 23 had complex MI. These groups were analyzed according to treatment modality, concentrating on length of hospital stay, complications [peritonitis, septicemia, adhesive small bowel obstruction (ASBO), and malabsorption/diarrhea], need for additional surgical procedures, mortality. RESULTS: Of the 18 patients with simple MI, 7 (39%) were successfully treated with diluted Gastrografin® enema. The remaining 11 patients were treated surgically: two underwent RPA, of whom one died; five had RES, of whom one developed ASBO; four underwent PSI, of whom two developed peritonitis. In the complex MI group, 14 patients underwent RPA, with peritonitis occurring in three (one died); nine underwent RES, of whom two developed ASBO. CONCLUSION: In patients with simple MI, conservative treatment with diluted Gastrografin® enema is an effective initial treatment in our hands. In case of failure, RES is advisable. Patients with complex MI are candidates for RES. RPA and PSI seem to have higher complication rates.

Pulmonary function impairment after trachea-esophageal fistula: a minor role for gastro-esophageal reflux disease.

BACKGROUND: Long-term impairment of pulmonary function in trachea-esophageal fistula (TEF) patients is, at least in part, commonly ascribed to gastro-esophageal reflux disease (GERD). The objective of this study was to examine the independent effects of the underlying condition and GERD on cardiopulmonary function. METHODS: Cardiopulmonary function of TEF patients, who had (severe) GERD (s-GERD) requiring antireflux surgery (TEF + GERD, n = 11) and TEF patients who did not have s-GERD (group TEF-GERD, n = 20) were compared with control patients who had isolated s-GERD requiring antireflux surgery (group GERD, n = 13). All patients performed spirometry, lung volume measurements, measurement of diffusion capacity and maximal cardiopulmonary exercise testing (CPET). RESULTS: Mean age of the participants was 13.8 ± 2.7 (group TEF + GERD). 13.2 ± 2.9 (group TEF-GERD), and 14.7 ± 1.5 years (group GERD). FVC and TLC were significantly lower in patients with TEF (with and without s-GERD) when compared to patients with isolated s-GERD. Most pulmonary function parameters were similarly affected in both TEF groups, but FEV(1) was lower in the TEF + GERD group than in the TEF-GERD group. Cardiopulmonary exercise parameters were similar in all groups. CONCLUSIONS: TEF patients had restrictive lung function impairment when compared to patients with isolated s-GERD. This difference may be due to several causes, including thoracotomy. FEV(1) was lower in TEF + GERD when compared to TEF-GERD indicating that GERD may affect large airway function in TEF patients. Other differences between TEF patients with and without s-GERD were not significant, suggesting only a minor role for GERD.

Presacral masses in children: presentation, aetiology and risk of malignancy.

AIM: The risk of malignant changes in presacral tumours in children was investigated in relation to age at diagnosis, type of presentation and origin of the tumour. METHOD: A retrospective review was carried out in 17 patients surgically treated for congenital presacral masses over a 22-year period. RESULTS: Constipation was the main symptom in 14 (82%) of 17 patients. The lesions were evident on digital examination in 14 patients. Mature teratoma (n = 9, 64%) was the most common lesion, including three malignancies. Currarino syndrome was diagnosed in 10 (71%) patients. Two unclassified variant HLXB9 gene mutations were found in five (29%) patients who underwent genetic testing. CONCLUSION: Congenital presacral tumours in children were mostly mature teratomas, either as sacrococcygeal teratomas or as part of the Currarino syndrome. The risk of malignancy in patients older than 1 year necessitates early surgical resection.

Challenges and outcome of Wilms' tumour management in a resource-constrained setting.

BACKGROUND: To review the results of Wilms' tumour patients in a tertiary referral hospital in a developing country and to find ways of improving long-term survival. PATIENTS AND METHODS: Between January 1998 and May 2004, 40 patients with Wilms' tumour (WT) were admitted to Queen Elizabeth Central Hospital. Their files were reviewed and general physical condition on admission, pre-operative investigations, management and outcome were noted. RESULTS: The mean age of presentation was 4.2 years with an equal distribution between the sexes. The mean BMI was 15 kg/m2 and more than 80% of the patients were either mildly (PCV <33%) or severely anaemic (PCV <24%). All patients presented with abdominal distension. Half of them had additional complaints including abdominal pain, haematuria, dyspnoea, oedema and or weight loss. Thirty-nine out of the forty patients received pre-operative chemotherapy. Of the 36 patients that underwent surgery, 32 underwent total nephrectomy, one a partial nephrectomy, and in three the tumour was irresectable. There were no intra-operative tumour ruptures. Only 15% of the patients completed their post-operative course of chemotherapy. The 1-year survival lies between 25% and 53%. Fifteen of the 36 patients operated were known to have a recurrence. CONCLUSION: The patients presented in an advanced stage of the disease. Survival rates are disappointing and recurrence rates are high. Some improvement in outcome may be expected with the implementation of more aggressive treatment protocols but early diagnosis, completion of treatment regimens are needed. Pro-active follow-up is essential to measure outcome.

Clinical and genetic characteristics of meconium ileus in newborns with and without cystic fibrosis.

The present study compares the clinical presentation and diagnostic features of meconium ileus (MI) in newborns with and without cystic fibrosis (CF). A retrospective study of 43 patients treated in the Pediatric Surgical Center of Amsterdam was performed. Twenty-three of the patients (53.5%) were diagnosed as having CF. Complex MI was significantly more frequent in patients without CF, and these patients had lower gestational ages and birth weights than patients with CF. All of the patients with complex MI had homozygous DF508 mutations, whereas the patients with simple MI also had other mutations. None of the patients with other mutations had complex MI. Therefore, we conclude that the clinical entity of MI represents a spectrum of underlying pathologies.

Management of trichobezoar: case report and literature review.

Trichobezoars (hair ball) are usually located in the stomach, but may extend through the pylorus into the duodenum and small bowel (Rapunzel syndrome). They are almost always associated with trichotillomania and trichophagia or other psychiatric disorders. In the literature several treatment options are proposed, including removal by conventional laparotomy, laparoscopy and endoscopy. We present our experience with four patients and provide a review of the recent literature. According to our experience and in line with the published results, conventional laparotomy is still the treatment of choice. In addition, psychiatric consultation is necessary to prevent relapses.

Learning curves for pediatric laparoscopy: how many operations are enough? The Amsterdam experience with laparoscopic pyloromyotomy.

BACKGROUND: Few studies on the surgical outcomes of open (OP) versus laparoscopic pyloromyotomy (LP) in the treatment of hypertrophic pyloric stenosis have been published. The question arises as to how many laparoscopic procedures are required for a surgeon to pass the learning curve and which technique is best in terms of postoperative complications. This study aimed to evaluate and quantify the learning curve for the laparoscopic technique at the authors' center. A second goal of this study was to evaluate the pre- and postoperative data of OP versus LP for infantile hypertrophic pyloric stenosis. METHODS: A retrospective analysis was performed for 229 patients with infantile hypertrophic pyloric stenosis. Between January 2002 and September 2008, 158 infants underwent OP and 71 infants had LP. RESULTS: The median operating time between the OP (33 min) and LP (40 min) groups was significantly different. The median hospital stay after surgery was 3 days for the OP patients and 2 days for the LP patients (p = 0.002). The postoperative complication rates were not significantly different between the OP (21.5%) and LP (21.1%) groups (p = 0.947). Complications were experienced by 31.5% of the first 35 LP patients. This rate decreased to 11.4% during the next 35 LP procedures (p = 0.041). Two perforations and three conversions occurred in the first LP group, compared with one perforation in the second LP group. CONCLUSION: The number of complications decreased significantly between the first and second groups of the LP patients, with the second group of LP patients quantifying the learning curve. Not only was the complication rate lower in the second LP group, but severe complications also were decreased. This indicates that the learning curve for LP in the current series involved 35 procedures.

Health-related quality of life and its determinants in children and adolescents born with oesophageal atresia.

AIM: Following surgical correction in the neonatal period, patients born with oesophageal atresia have significant co-morbidity, particularly in childhood. This study evaluates health-related quality of life and its determinants such as concomitant anomalies and the presence of respiratory and/or gastro-intestinal symptoms 6-18 years after repair of oesophageal atresia. METHODS: Parents of 24 patients with oesophageal atresia completed the child health questionnaire for parents and 37 patients completed the child form. Gastro-intestinal symptoms were assessed by a validated standardized reflux questionnaire. Results were compared with a healthy reference population. RESULTS: Parents as well as patients themselves scored significantly lower on the domain general health perception. According to parents, general health perception was negatively affected by age at follow-up and concomitant anomalies. Patients reported that reflux symptoms reduced general health perception. CONCLUSION: In this first study describing health-related quality of life in children and adolescents born with oesophageal atresia, we demonstrated that general health remains impaired because of a high incidence of concomitant anomalies and gastrointestinal symptoms in patients with oesophageal atresia when compared with the healthy reference population.

Patient compliance in the treatment of Burkitt's lymphoma in rural Zambia: a retrospective study on 80 Burkitt's lymphoma patients in Katete, Zambia.

BACKGROUND: In African settings the treatment results of Burkitt's lymphoma (BL) seem to be less favourable compared with Western settings. The aim of this retrospective study was to analyse some factors that affect the treatment of BL. PATIENTS AND METHODS: Over a 16 year period, data were extracted of 80 patients. RESULTS: Complete remission 5%, very good partial response 35%, partial response 16%, no response 10%, data missing 34%. Of all patients, 56% did have a positive response to treatment. However, 51% of this subgroup of patients did not finish treatment. There was no difference in completion of treatment between patients living in Katete district finishing treatment vs. living outside Katete district (respectively 25% vs. 32%, P = 0.7148). CONCLUSION: There is potential for higher cure rates for BL in tropical settings if full effort is put into compliance since a majority of patients, even while having a good prognosis, abandon treatment. Large distance to hospital makes no difference in completing the chemotherapy course.

Psychological outcome and quality of life in children born with congenital diaphragmatic hernia.

OBJECTIVE: To assess psychological and social functioning and health related quality of life and its early determinants in children born with congenital diaphragmatic hernia (CDH). DESIGN: Cross-sectional follow-up study. SETTING: Outpatient clinic of a tertiary care hospital. PARTICIPANTS: 33 CDH survivors aged 6-16 years. MAIN EXPOSURE: Patients who developed CDH associated respiratory distress within 24 h after birth. MAIN OUTCOME MEASURE: Psychological and social functioning assessed with the Wechsler Intelligence Scale for Children (WISC-R), Bourdon-Vos test, Beery Developmental Test of Visual Motor Integration, Child Behavior Checklist (CBCL) and Teacher Report Form (TRF), and health related quality of life assessed with the Child Health Questionnaire (CHQ) and Health Utilities Index (HUI). RESULTS: Normal mean (SD) total IQ (100.0 (13.2)) and normal visual-motor integration, but significantly lower results for sustained attention (Bourdon-Vos test, 38.8 (11.2) points) were found. Learning difficulties were reported by 30% of parents. Eight children had scores in the clinical range on the CBCL and/or TRF, indicating clinically significant behavioural problems. Except for the CHQ scale General Health, health status was not different from the reference population. No significant correlations between test results and severity of CDH were found, except for an association of general health and physical functioning with length of hospital stay. CONCLUSION: CDH patients are at risk for subtle cognitive and behavioural problems, probably not related to CDH severity. Perception of general health is reduced compared to the reference population, indicating that CDH survivors and their parents believe their health is poor and likely to get worse.