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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

Pulmonary function and exercise capacity in survivors of congenital diaphragmatic hernia.

Congenital diaphragmatic hernia (CDH) is associated with pulmonary hypoplasia and pulmonary hypertension. The objective of this study was to assess pulmonary function and exercise capacity and its early determinants in children and adolescents born with high-risk CDH (CDH-associated respiratory distress within the first 24 h) and to explore the relationship of these findings with CDH severity. Of 159 patients born with high-risk CDH, 84 survived. Of the 69 eligible patients, 53 children (mean+/-SD age 11.9+/-3.5 yrs) underwent spirometry, lung volume measurements and maximal cardiopulmonary exercise testing (CPET). Results of the pulmonary function tests were compared with those from a healthy control group matched for sex, age and height. CDH survivors had a significantly lower forced expiratory volume in 1 s (FEV(1)), forced vital capacity (FVC), FEV(1)/FVC, maximum mid-expiratory flow and peak expiratory flow when compared with healthy controls. The residual volume/total lung capacity ratio was significantly higher. Linear regression analysis showed that gastro-oesophageal reflux disease was an independent determinant of reduced FEV(1) and FVC. CPET results were normal in those tested. High-risk CDH survivors have mild to moderate pulmonary function abnormalities when compared with a healthy matched control group, which may be related to gastro-oesophageal reflux disease in early life. Exercise capacity and gas exchange parameters were normal in those tested, indicating that the majority of patients do not have physical impairment.

Paraneoplastic gastro-intestinal anti-Hu syndrome in neuroblastoma.

The anti-Hu syndrome is a well-known paraneoplastic syndrome and may be rarely seen in patients with neuroblastoma. However, it is relatively unknown that anti-Hu antibodies can cause gastro-intestinal signs and symptoms. We report on a child with neuroblastoma who presented with gastro-intestinal disturbances as a result of the anti-Hu syndrome and summaries two similar case reports reported in literature. Neuroblastoma patients with gastro-intestinal disturbances, ranging from constipation to a paralytic ileus, might suffer from the gastro-intestinal anti-Hu syndrome. The causative antibodies can be determined to diagnose or exclude this syndrome, and successful treatment is possible.

[Undescended testis: current views and advice for treatment]. / De niet-scrotale testis: huidige inzichten en advies voor behandeling.

--Undescended testis (UDT) is one of the most common urogenital abnormalities in boys. --UDT is defined as a testis which cannot be brought into a stable scrotal position. --At present, congenital and acquired forms of UDT are recognised. Congenital UDT is defined as a UDT which has never descended from birth. Acquired UDT is defined as a UDT which has been fully descended in the past. --Congenital UDT should be treated surgically between 6 to 12 months of age. --The treatment of acquired UDT is still disputed. As yet, awaiting spontaneous descent at early puberty seems to be the most rational treatment. --In the Netherlands, the high number of late orchidopexies is due to surgery for acquired UDT. To reduce this high number, the guidelines of the first development conference on 'non-scrotal testis' dating back to 1986 should be revised on several points.

Urinary gonadotropin measurements in neonates: a valuable non-invasive method.

BACKGROUND: To measure low neonatal gonadotropin levels, a sensitive non-invasive method is optimal. The aim of the current study was to validate the Architect i2000SR, an automated immunoassay analyser for the measurement of gonadotropins in unextracted neonatal urine samples against serum gonadotropin levels as a gold standard. METHODS: Blood and urine were sampled from 30 approximately six-week-old male and female neonates undergoing elective paediatric surgery. Follicle stimulating hormone (FSH) and luteinizing hormone (LH) were measured and the urine results were corrected for creatinine. RESULTS: The agreement between neonatal serum and urinary FSH was 0.904 (3-5 h between samples) and 0.704 (18-20 h). For LH, the correlation coefficients were 0.785 and 0.507, respectively. CONCLUSION: We conclude that gonadotropins can be reliably measured using the Architect on randomly voided, non-extracted urine samples collected from neonates by an adhesive device. Urinary gonadotropin levels are a proper reflection of the serum levels.

Long-term outcome of elastic stable intramedullary fixation (ESIF) of femoral fractures in children.

In 1993 a prospective data collection of all femoral fracture in children below 16 years of age was initiated in 4 hospitals in the Netherlands. Follow-up was at least two years, with the aim to evaluate leg length discrepancies (LLD). Over a period of 10 years, we included 136 patients < 16 years of age with femoral fractures. Seventy-one patients who were treated with an ESIF-(Elastic Stable Intramedullary Fixation) nail were studied: 44 boys and 27 girls, between the ages of 3-16 years, 23 of which had multiple injuries. Five patients had a Ist or IInd degree open fracture. The average length of stay (LOS) of children with an isolated fracture, was 10 days, and in case of multiple injuries: 12 days. Intra-operative complications were: a broken drill in 2 and an open reduction in 6 cases. Two patients with superficial wound infection were successfully treated with antibiotics. Seven patients had knee complaints of the nails, of which 3 had actually been displaced. Three other patients showed displacement of the nails at X-ray, but had no complaints. At six months follow-up, at the time of removal of the nails, 10 patients had a leg length discrepancy (LLD) exceeding 10 mm. Three children had a clinically significant rotational deformity at this time. Two and a half years after injury 6 patients showed a persistent LLD of more than 10 mm. In all 6 the fractured limb was longer. At ten years of follow-up the LLD persisted in 5 patients. One patient had a persistent clinically significant rotational deformity of more than 10 degrees . The advantages of ESIF-nails are: reduction of hospital stay and rapid mobilisation. The disadvantages: two operations (for insertion and removal) with a risk of complication. Long-term follow-up, preferably till growth stops, is necessary to conclude whether persistent lengthening of the fractured limb remains a problem.

[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.

Pre-operative diagnostic biopsy and surgery in paediatric liver tumours--the Amsterdam experience.

AIM: To report 24 years of pre-treatment biopsy and surgical experience in primary liver tumours in children. METHODS: Between 1979 and 2003, 53 children presented with a primary liver tumour of whom 48 who underwent surgical resection were evaluated (two died, two were unresectable, and one was transplanted). Biopsy data, per- and post-operative complications, mortality, and survival were retrospectively reviewed. RESULTS: Benign tumours were diagnosed in eight patients. Surgical resection for a malignant tumour was performed in 40 patients (26 hepatoblastomas (HB), eight hepatocellular carcinomas (HCC) (four had fibrolamellar HCC), three rhabdomyosarcomas (RMS), one neuroblastoma, one non-hodgkin lymphoma (NHL), and one teratoma). Primary resection was performed in one HB, and four HCCs. The cumulative survival without evidence of disease was 73% for HB (median 7 years) and 88% for HCC (median 3.5 years). CONCLUSION: The treatment results are comparable with those of larger international series except for HCC. The existing diagnostic pitfalls in differentiating between the various liver malignancies justify the use of a diagnostic biopsy.