The Management of Post-appendectomy Abscess in Children; A Historical Cohort Study and Update of the Literature.

Introduction: Recent studies have shown that specific cases of post-appendectomy abscess (PAA) in children could be treated conservatively. However, due to the lack of high-quality evidence, choice of treatment still depends on preferences of the treating surgeon, leading to heterogeneity in clinical practice. Therefore, we aimed to provide an update of recent literature on the management of PAA in children and subsequently evaluate the outcomes of a large multicenter cohort of children treated for PAA. Methods: A literature search was performed in Pubmed and Embase, selecting all randomized controlled trials, prospective and retrospective cohort studies, and case series published from 2014 and onward and reporting on children (<18 years) treated for a PAA. Subsequently, a historical cohort study was performed, including all children (<18 years) treated for a radiologically confirmed PAA between 2014 and 2021 in a tertiary referral center and two large peripheral centers. Medical charts were reviewed to compare non-invasive (i.e., antibiotics) and invasive (i.e., drainage procedures) treatment strategies. Primary outcome was the success rate of treatment, defined as no need for further interventions related to PAA or its complications. Results: The search yielded 1,991 articles, of which three were included. Treatment success ranged between 69-88% and 56-100% for non-invasive and invasive strategies, respectively. Our multicenter cohort study included 70 children with a PAA, of which 29 (41%) were treated non-invasively and 41 (59%) invasively. In the non-invasive group, treatment was effective in 21 patients (72%) compared to 25 patients (61%) in the invasive group. Non-invasive treatment was effective in 100% of unifocal small (<3 cm) and 80% of unifocal medium size PAA (3-6 cm), but not effective for multiple abscesses. Conclusion: Non-invasive treatment of especially unifocal small and medium size (<6 cm) PAA in children seems to be safe and effective. Based on these results, a standardized treatment protocol was developed. Prospective validation of this step-up approach-based treatment protocol is recommended.

AKT Hyperphosphorylation and T Cell Exhaustion in Down Syndrome.

Down syndrome (DS) is associated with increased susceptibility to infections, auto-immunity, immunodeficiency and haematological malignancies. The exact underlying immunological pathophysiology is still unclear. The immunophenotype and clinical characteristics of DS resemble those of Activated PI3K Delta Syndrome (APDS), in which the PI3K/AKT/mTOR pathway is overactivated. We hypothesized that T cell exhaustion and the hyperactivation of the AKT signalling pathway is also present in immune cells of children with DS. In this observational non-interventional cohort study we collected blood samples of children with DS (n=22) and healthy age-matched controls (n=21) for flowcytometric immunophenotyping, phospho-flow AKT analysis and exhaustion analysis of T cells. The median age was 5 years (range 1-12y). Total T and NK cells were similar for both groups, but absolute values and transitional B cells, naive memory B cells and naive CD4+ and CD8+ T cells were lower in DS. pAKT and AKT were increased for CD3+ and CD4+ T cells and CD20+ B cells in children with DS. Total AKT was also increased in CD8+ T cells. Children with DS showed increased expression of inhibitory markers Programmed cell dealth-1 (PD-1), CD244 and CD160 on CD8+ T cells and increased PD-1 and CD244+ expression on CD4+ T cells, suggesting T cell exhaustion. Children with DS show increased pAKT and AKT and increased T cell exhaustion, which might contribute to their increased susceptibility to infections, auto immunity and haematological malignancies.

Interventions with Music in PECTus excavatum treatment (IMPECT trial): a study protocol for a randomised controlled trial investigating the clinical effects of perioperative music interventions.

INTRODUCTION: Pectus excavatum repair is associated with substantial postoperative pain, despite the use of epidural analgesia and other analgesic regimens. Perioperative recorded music interventions have been shown to alleviate pain and anxiety in adults, but evidence for children and adolescents is still lacking. This study protocol describes a randomised controlled trial that evaluates the effects of recorded music interventions on postoperative pain relief in children and adolescents after pectus excavatum repair. METHODS: A multicentre randomised controlled trial was set up comparing the effects of perioperative recorded music interventions in addition to standard care with those of standard care only in patients undergoing a Nuss procedure for pectus excavatum repair. One hundred and seventy subjects (12-18 years of age) will be included in three centres in the Netherlands. Patient inclusion has started in November 2018, and is ongoing. The primary outcome is self-reported perceived pain measured on the visual analogue scale. Secondary outcomes are anxiety level, analgesics consumption, vital parameters such as heart rate, blood pressure and respiratory rate, length of hospital stay, postoperative complications, quality of life and cost-effectiveness. ETHICS AND DISSEMINATION: This study is being conducted in accordance with the Declaration of Helsinki. The Medical Ethics Review Board of Erasmus University Medical Centre Rotterdam, The Netherlands, has approved this protocol. Results will be disseminated via peer-reviewed scientific journals and conference presentations. TRIAL REGISTRATION NUMBER: NL6863.

The Outcome of the Single Step Questionnaire in Pectus Excavatum Patients is Phase Dependent.

INTRODUCTION: Pectus excavatum (PE) is the most common chest wall deformity. Patients with PE may have cosmetic complaints, restricted physical capabilities, or both and may seek surgical correction. One method to assess satisfaction after surgery is the single step questionnaire (SSQ). Although the developers state that the SSQ produces a stabile score and only needs to be used once, we hypothesized that the score may depend on point in time after surgery. MATERIALS AND METHODS: One hundred and eight patients from a longitudinal cohort of patients undergoing a Nuss bar placement for PE were selected. Mean age was 16.0 years (range: 12-29). SSQ was completed at 6 weeks, 6 months, 1 year, and 2 years postoperatively. Mean and median scores per question and total scores were calculated on each measurement moment. Overall scores were tested using the Friedman test. RESULTS: There were significant differences in overall SSQ scores (p < 0.009) throughout the postoperative period, especially between 6 weeks and 6 months (p = 0.006). Scores on general health, exercise capacity, impact on social life, pain during hospital stay, and after discharge changed also significant in the first 2 years after Nuss bar placement. CONCLUSION: There were significant differences in total SSQ score depending on the time of application postoperatively. However, the most clinical relevant difference was between 6 weeks and 6 months. Assessment of the overall satisfaction postoperative with the SSQ questionnaire should not be done with a single measurement but rather at different postoperative time intervals before and after 6 months postoperatively.

Early cost-utility estimation of the surgical correction of pectus excavatum with the Nuss bar.

OBJECTIVES: The surgical correction of pectus excavatum (PE) with a Nuss bar provides satisfactory outcomes, but its cost-effectiveness is yet unproven. We prospectively analysed early outcomes and costs for Nuss bar placement. METHODS: Fifty-four patients aged 16 years or older (6 females and 48 males; mean age, 17.9 years; range 16.0-29.4 years) with a PE filled out a Short Form-36 Health Survey (SF-6D) preoperatively and 1 year after a Nuss procedure. Costs included professional fees and fees for the operating room, materials and hospital care. Changes in the responses to the SF-36 or its domains were compared using the Wilcoxon signed rank test and the utility test results were calculated preoperatively and postoperatively from the SF-6D. The quality-adjusted life years (QALYs) were calculated from the results of these tests. RESULTS: Significant improvements in physical functioning, social functioning, mental health and health transition (all P < 0.05) were noted. The other SF-36 subgroups showed improvement; however, the improvement was not significant. The SF-6D utility showed improvement from 0.76 preoperatively to 0.79 at the 1-year follow-up (P = 0.096). The mean direct costs were €8805. The 1-year discounted QALY gain was 0.03. The estimated cost-utility ratio was €293 500 per QALY gained. CONCLUSIONS: Despite a significant improvement in many domains of the SF-36, the results of the SF-6D cost-utility analysis showed only a small improvement in cost-effectiveness (> €80 000/QALY) for patients with PE 1 year after Nuss bar placement. Based on this discrepancy, general health outcome measurements as the basis for cost-utility analysis in patients with PE may not be the best way forward.

Sports activity in adolescents in the Netherlands with a pectus excavatum; the impact of surgery.

PURPOSE: Pectus excavatum (PE) is the most common chest wall deformity in adolescents. The main complaint is cosmetic, but many patients also complain about exertional dyspnea. This may lead to the patient seeking surgery of the thoracic wall deformity (TWD). The assumption is that both, appearance and physical complaints will have a negative effect on being able or wanting to engage in sport activity. METHODS: In December 2011 a prospective registration of sport activity in pectus excavatum patients started. Sport activity was assessed using questionnaires (CHQ, SF-36 and PEEQ). Measurements were taken before corrective surgery (preoperatively) and 12 months postoperatively. RESULTS: 127 patients have been included. The number of patients who were active in sports preoperatively and after 12 months remained steady. The type of sport activity, individual sport or team sport showed no significant change. The CHQ showed that physical activity caused fewer complaints (p < 0.001). The PEEQ showed a decrease in difficulties with sports activity performance after 12 months (p < 0.001). CONCLUSIONS: Twelve months after surgical correction of PE there was no significant increase in the number of patients performing sport activities. However there was a significant decrease of complaints or difficulties during sport compared to preoperatively. LEVEL OF EVIDENCE: Level IV.

Long-term testicular volume after orchiopexy at diagnosis of acquired undescended testis.

PURPOSE: We studied long-term outcomes of orchiopexy at diagnosis of acquired undescended testes using ultrasound to determine testicular volume. MATERIALS AND METHODS: Patients who had undergone orchiopexy for acquired undescended testis at diagnosis were recruited to assess testicular volume. Testis volume was measured by ultrasound and compared with recently developed normative values for testicular size. For young adults (older than 18 years) volumes were grouped and compared to normative values reported in the literature. In all unilateral cases testicular volume was compared with its counterpart. RESULTS: A total of 155 patients 5.1 to 26.6 years old (181 acquired undescended testes) were included in the study. Mean ± SD followup was 6.6 ± 3.8 years (range 1.4 to 15.5). For all patients 18 years old or younger (125 patients, 143 testes) operated testis volume was 0.1 to 12.7 ml (mean ± SD 2.5 ± 2.9), which was significantly smaller than the normative values (50th percentile) for the same age (p <0.001). Mean ± SD testis volume in young adults (38 testes) was 8.1 ± 3.7 ml, compared to a mean volume of 13.4 ml reported in the literature (p <0.001). In unilateral cases the mean volume of the testes fixed by orchiopexy differed significantly from their counterparts (3.4 ± 3.3 ml vs 4.6 ± 4.6 ml, p <0.001). CONCLUSIONS: The long-term volumes at diagnosis of acquired undescended testes after orchiopexy were significantly less than the normative values at all ages. In unilateral cases the volumes were also significantly less compared to the contralateral testes.

[Intestinal duplication cyst in children: laparoscopically assisted resection is preferred]. / Duplicatiecyste van de darm bij kinderen.

A 5-year-old boy was seen for a second opinion as he had episodes of severe abdominal pain and a variably distended abdomen. Ultrasonography showed a suspected duplication cyst of the terminal ileum. A laparoscopically assisted resection was performed. Histology confirmed the diagnosis of a duplication cyst. A few months later a 10-year-old boy presented with a history of abdominal pain, diarrhoea and vomiting. Abdominal ultrasonography showed an intussusception halfway up the transverse colon where a cyst was seen with a fluid level. The intussusception was reduced during laparoscopy and resection of the duplication cyst of the ileum was performed. Although intestinal duplication cysts are rare (prevalence 1:4500), it is important to know they exist. Laparoscopically assisted resection is an elegant way of treating duplication cysts in children.

[Serious gastrointestinal complications due to accidental ingestion of magnetic toy parts]. / Ernstige gastro-intestinale complicaties door inslikken van magnetisch speelgoed.

A 3-year-old boy presented at the emergency room with abdominal pain, nausea and non-bilious vomiting. Physical examination revealed moderate symptoms of dehydration, a tender and slightly extended but soft abdomen and active peristalsis. With the working diagnosis of viral gastroenteritis the boy was admitted to the hospital for rehydration therapy by nasogastric tube. After a few days an abdominal X-ray was taken because of bilious vomiting and persistent absence of defecation. Connected foreign bodies were seen in the small intestine. These appeared to be 3 magnetic toy parts. Laparotomy showed that all symptoms were due to a volvulus of the small intestine around a fistula caused by entrapment of the intestinal wall between attracted magnets. Magnetic construction toys can be bought in toy stores in the Netherlands. Vigilance should be exercised with magnetic toys, because ingestion of multiple magnets can cause potentially life-threatening bowel complications.

[A girl with a swelling on her head]. / Een meisje met een zwelling op haar hoofd.

A 6-year-old girl had an eosinophilic granuloma on the parietal skull due to Langerhans cell histiocytosis.