Bracing of Pectus Carinatum in Children: Current Practices.

BACKGROUND: Although effective, compressive orthotic bracing (COB) in children with pectus carinatum is still not standardized. This study has aimed to analyze current practices amongst members of the Chest Wall International Group (CWIG). METHODS: A web-based questionnaire was mailed to all CWIG members at 208 departments. It included 30 questions regarding diagnostic work-up, age for COB indication, type of COB used, daily wearing time, treatment duration, complications, and recurrence rate. RESULTS: Members from 44 departments have responded (institutional response rate 21.2%). A total of 93% consider COB as the first-line treatment for PC. A conventional COB (CC) is used in 59%, and the dynamic compression system (FMF) in 41%. The overall compliance rate is >80%. A total of 67% of responders consider COB to be indicated in patients <10 years. The actual wearing time is significantly shorter than the physician-recommended time (p < 0.01). FMF patients experience a significantly faster response than CC patients (p < 0.01). No recurrence of PC has been noted in 34%; recurrence rates of 10-30% have been noted in 61%. CONCLUSIONS: COB is the first-line treatment for PC with a high compliance rate. During puberty, the recurrence rate is high. Treatment standardization and follow-up until the end of puberty are recommended to enhance COB effectiveness.

Bladder Neoplasia in Pediatric Patients-A Single-Center Experience Including a Case Series.

Objective: Bladder lesions like urothelial carcinoma are rare in the first two decades of life. A biopsy of the bladder or urinary cytological examination is seldom required. Gross painless hematuria is the most relevant clinical syndrome. Methods: A retrospective analysis of surgical pathology records collected between 1984 and 2014 at our institution was performed in a search for cases of urothelial neoplasms originating within the urinary bladder in pediatric patients. Diagnoses were confirmed based on pathologic examination using the 2004 World Health Organization (WHO) classification system. We selected keywords such as bladder neoplasia, bladder lesion, urothelial neoplasia, rhabdomyosarcoma, and children. In addition, we describe clinical presentation and diagnostic procedures as well as treatment and follow-up of two patients. A review of the literature was performed to analyze recommendations concerning diagnostic staging, treatment, and follow-up examinations as well as surveillance of urothelial tumors in the pediatric population. Results: Screening the pathology database of the Institute of Medical Genetics and Pathology of the University Hospital Basel between 1988 and 2014 yielded 287 samples involving the urinary bladder, 110 autopsies, 135 biopsies, and 42 cytology specimens. Of these, most samples originated from malformations and inflammation. Only five were tumors: two were urothelial tumors and three were rhabdomyosarcomas. The majority of specimens comprised resections of the diverticula or distal ureter. Our case reports include two patients with a urothelial tumor. Among the urothelial tumors, one was a papillary urothelial neoplasm of low malignant potential (PUNLMP). Painless hematuria was the directing clinical symptom. The tumor was investigated by FISH, and a 9p21 deletion was found. The second tumor-like lesion was a fibroepithelial polyp arising from the bladder neck. Conclusions: Bladder tumors in children are rare and mostly consist of urothelial and mesenchymal neoplasms. Rhabdomyosarcoma is the most common malignant bladder tumor in childhood. Similar to adult urothelial neoplasms, the loss of 9p21 is also implicated in urothelial neoplasms in childhood. Despite an increasing number of case reports and small series published within the last 2 decades, general treatment protocols including recommendations for staging, tumor markers, and follow-up examinations are still not yet available for this tumor entity in the pediatric population.

Pectus Excavatum: Consensus and Controversies in Clinical Practice.

BACKGROUND: Pectus excavatum is the most common congenital anterior chest wall deformity. Currently, a wide variety of diagnostic protocols and criteria for corrective surgery are being used. Their use is predominantly based on local preferences and experience. To date, no guideline is available, introducing heterogeneity of care as observed in current daily practice. The aim of this study was to evaluate consensus and controversies regarding the diagnostic protocol, indications for surgical correction, and postoperative evaluation of pectus excavatum. METHODS: The study consisted of 3 consecutive survey rounds evaluating agreement on different statements regarding pectus excavatum care. Consensus was achieved if at least 70% of participants provided a concurring opinion. RESULTS: All 3 rounds were completed by 57 participants (18% response rate). Consensus was achieved on 18 of 62 statements (29%). Regarding the diagnostic protocol, participants agreed to routinely include conventional photography. In the presence of cardiac impairment, electrocardiography and echocardiography were indicated. Upon suspicion of pulmonary impairment, spirometry was recommended. In addition, consensus was reached on the indications for corrective surgery, including symptomatic pectus excavatum and progression. Participants moreover agreed that a plain chest radiograph must be acquired directly after surgery, whereas conventional photography and physical examination should both be part of routine postoperative follow-up. CONCLUSIONS: Through a multiround survey, international consensus was formed on multiple topics to aid standardization of pectus excavatum care.

Current Concepts in Endoscopic Bladder Neck Injection: Combined Antegrade and Retrograde Endoscopic Injection of the Bladder Neck in Children with Neurogenic Bladder.

INTRODUCTION: Urinary incontinence is common in patients with neurogenic bladder, and efficient management is an ongoing challenge. Besides open surgical procedures like bladder neck reconstruction, artificial sphincter implantation, or sling procedures, endoscopic bladder neck injections of bulking agents enable minimally invasive access with promising results. Several studies report on the effect of antegrade vs. retrograde endoscopic injection techniques. We report our preliminary experience of combined antegrade and retrograde endoscopic injection of the bladder neck in children with neurogenic bladder, in selected cases combined with intravesical Botox® injection. METHODS: With the patient in lithotomy position, antegrade urethrocystoscopy was performed using a 9.5 Fr cystoscope. In parallel, percutaneous suprapubic bladder access introducing a second 9.5 Fr. cystoscope was accomplished. Four quadrant Dx/H injections were performed, with the two surgeons guiding each other by parallel endoscopy to the optimal localization for injection. In selected patients, the procedure was completed with transurethral intravesical Botox® injection. RESULTS: A total of 6 children underwent the combined procedure (2/6 patients including intravesical Botox® injection). The mean follow-up was 15 months (range 3 to 48). 5 Patients experienced a significant improvement of urinary incontinence, however one patient demonstrated complete failure. CONCLUSIONS: Even if we present only preliminary results with a limited number of patients, we present a minimally invasive technique with encouraging results. In carefully selected patients, combined antegrade and retrograde endoscopic injection of the bladder neck is a useful tool to treat urinary incontinence.

Current Development of Minimally Invasive Repair of Pectus Excavatum (MIRPE).

For decades, open surgical repair was the only available method to treat congenital and acquired chest wall deformities (CWDs). In 1998, D. Nuss described a minimally invasive procedure for surgical repair of Pectus excavatum (PE). Today, the Nuss procedure is performed with increasing frequency worldwide and considered as the "gold standard". After its introduction, the method experienced numerous modifications such as routine thoracoscopy and/or sternal elevation, increasing safety of the procedure. Placement of multiple bars and/or the so called cross-bar technique were introduced to correct complex CWDs. Standardized pain management, the introduction of cryo-analgesia and a standardized postoperative physiotherapy program including deep breathing exercises facilitate the establishment of an enhanced recovery after surgery (ERAS) process. However, the widespread use of the minimally invasive repair of pectus excavatum (MIRPE) procedure has been associated with a significant number of serious complications. Furthermore, several studies report near-fatal complications, not only during bar placement, but also during bar removal. This review focuses upon the most relevant modifications, including recent published surgical techniques of MIRPE, in order to describe current developments in the field.

Dysfunctional Voiding and Incontinence Scoring System for Children and Adolescents: A Tool to Predict Clinical Course and Outcome.

INTRODUCTION: Lower urinary tract symptoms (LUTS) in children are common. In 2005, Akbal et al published the dysfunctional voiding and incontinence scoring system (DVAISS) to assess patient's symptoms. Our study aimed to determine the value of this scoring system for predicting the severity and clinical course of voiding abnormalities in children and adolescents. MATERIAL AND METHODS: Patients' symptoms were scored using the DVAISS in children and adolescents with LUTS presenting for the first time to our pediatric urology department between January 2010 and December 2015. We correlated the calculated score with voiding volume, clinical course, and outcome. RESULTS: A total of 168 patients (113 boys, 55 girls; age 5-18 years) with isolated LUTS were included. In 53 patients (group 1), the DVAISS score was less than or equal to 8.5 and in the other 115 patients (group 2), the score was greater than 8.5 suggestive for relevant voiding abnormalities. Patients in group 1 showed a significantly higher average voiding volume (200 vs. 110 mL, p = 0.001). The median time for symptom resolution was significantly higher in group 2 than group 1 (14 vs. 8 months; p = 0.018). The severity of LUTS could be determined by these parameters. CONCLUSION: Based on the DVAISS, a prediction of the clinical course and approximate treatment duration is possible. Therefore, the DVAISS is useful to assess LUTS in children and is also a valuable tool in rating the severity of the disease. It is also a quite precise predictor of the time needed to resolve the symptoms.

Pectus bar removal - why, when, where and how.

OBJECTIVE: Despite its less invasive nature, the widespread use of the minimally invasive repair of pectus excavatum (MIRPE) procedure has been associated with a significant number of serious complications. On the other hand, Pectus bar removal (PBR) is often considered a simple procedure and often scheduled in an outpatient setting. However, several studies report near-fatal complications not only during bar placement, but also during bar removal. The aim of our study was to clarify why a pectus bar should be removed, timing for removal, where PBR should be performed, and overall setup for safe removal. METHODS: A comprehensive review was performed in accordance with PRISMA guidelines, searching for articles published since 1998 in English. "Pectus bar removal AND (near-fatal) complications" were the applied terms. Inclusion criteria were articles reporting on the focus of PBR after MIRPE. Eligible study designs included (retrospective) case study series, case report and reviews. Full-text articles in which the technique in general was described were omitted. RESULTS: Recently published results of an online survey raised awareness about type and number of possible complications during PBR. Furthermore, our comprehensive literature review identified only a few, but serious complications during PBR. CONCLUSIONS: PBR has a high safety profile but in rare cases may be associated with major complications such as life-threatening hemorrhage from various thoracic sources. This risk is higher in patients with a history of complex MIPRE. In an effort to decrease these complications we recommend bilateral opening of surgical incisions, unbending the bar and meticulous mobilization of the bar. To manage these complications if they occur, we recommend removal in a hospital setting with adequate resources and personal including cardiac surgeons. If the postoperative course is uneventful discharge on the same day is reasonable.

Chromosomal Heterogeneity of the G-401 Rhabdoid Tumor Cell Line: Unusual Partial 7p Trisomy.

Rhabdoid tumor is a very aggressive and hardly curable pediatric malignancy. It commonly starts in the kidneys but also can occur in the brain, liver, and other organs. The treatment of this tumor usually involves a combination of surgery, radiation, and chemotherapy. Because this tumor is rare, there is still limited experience with a defined standard of care. Cytogenetic analysis is an important routine method to monitor chromosomal aberrations. We have analyzed metaphases of the G-401 rhabdoid tumor cell line. In these cells we have observed metaphases with derivative chromosome 12 arising from partial trisomy 7p. With increasing passage number the numbers of metaphases having this derivative chromosome 12 were found to be higher. In passage number 2 only one metaphase had this pathological chromosome 12. By passage number 10 and passage number 15 about 25 and 95% of this derivative chromosome 12 were found, respectively. We were able to subclone G-401 cells by limiting dilutions and successfully separated cells having apparently normal karyotypes from cells having derivative chromosome 12. Using the cell proliferation assay we showed that clones possessing the derivative chromosome 12 grew more rapidly than clones with normal chromosomes. The cell cycle analysis confirmed this observation. Overall, in this study we describe for the first time a 7p triplication in a rare rhabdoid tumor of kidney. Both types of clones described in this study could be used as a preclinical model to study the involvement of partial chromosome 7 alterations in the development of rhabdoid tumors.

Sternal elevation techniques during the minimally invasive repair of pectus excavatum.

The aim of the review was to evaluate the routine use of sternal elevation techniques (SETs) during minimally invasive repair of pectus excavatum (MIRPE, the Nuss procedure). We performed a review of the literature between January 1998 and September 2018 with focus on different methods of SET during MIRPE. Reported effects and side effects were evaluated and compared with our own experience concerning the routine use of the vacuum bell for sternal elevation during MIRPE during the last 13 years. SET is more often used in adult patients than in adolescents. SET improves visualization and safety of MIRPE. Advancement of the pectus introducer, retrosternal dissection and placement of the pectus bar are easier. The risk of cardial and/or pericardial lesion is reduced significantly. Different types of retractors, a crane combined with a wire and/or customized hooks are reported to be used as SET. Furthermore, routine use of a subxiphoid incision is reported. However, more technical equipment, and in some SETs additional incisions are necessary. In contrast, no additional skin incision is necessary for the vacuum bell. The routine intraoperative use of the vacuum bell was safe and effective in 131 patients. It facilitates the retrosternal dissection and the insertion of the pectus bar like other SETs. Besides a temporary mild hematoma, no relevant side effect was observed. In conclusion, an increasing number of authors report on the routine use of SET during MIRPE to improve safety of the procedure. We recommend the routine intraoperative use of the vacuum bell during MIRPE.

Standardized Haller and Asymmetry Index Combined for a More Accurate Assessment of Pectus Excavatum.

BACKGROUND: The measurement of the Haller index (HI) is not standardized, and HI does not consider the asymmetry of pectus excavatum. The aim of this study was to determine the most appropriate level for measuring the HI and to introduce the asymmetry index (AI) in order to respect the aspect of asymmetry. METHODS: Preoperative computer tomography scans of 43 patients with pectus excavatum were retrospectively analyzed by measuring both the HI and the AI at 3 different levels: (I) sternomanubrial junction; (II) caudal end of corpus sterni; and (III) tip of the xiphoid. The control group comprised 33 patients without chest wall deformity and a HI < 3.25. For each patient HI and AI were calculated according to a standardized protocol. A McNemar test was used for statistical analyses. RESULTS: The sensitivity of the HI was highest when measured at level II (p < 0.388), and the AI exhibited the highest sensitivity at level I. When combining both indices, the sensitivity of assessing pectus excavatum significantly increased compared with the use of HI at level II alone (p < 0.002). CONCLUSIONS: The measurement of the HI at level II represents the most valid standardized parameter for assessment of the severity of pectus excavatum. Asymmetry, on the other hand, is best assessed by the AI measured at level I. The combination of the standardized HI and AI not only provides a much more accurate description of pectus excavatum, but also improves the comparability of pectus excavatum patients in general.

Treatment of isolated sternal fracture with a vacuum bell in an 8-year-old boy.

Isolated sternal fractures are very rare in children. Pain management is the method of choice, but surgery may be indicated in selected cases with dislocated fractures. However, the indication for open reduction of sternal fractures is discussed controversially. To the best of our knowledge, we present the first report of a child with displaced isolated sternal fracture who was successfully treated by conservative means using a vacuum bell.

Monitoring the effectiveness of the vacuum bell during pectus excavatum treatment: Technical innovation.

BACKGROUND: The vacuum bell (VB) is a valid and the only non-invasive treatment for pectus excavatum (PE). To elevate the sternum the patient himself creates a differential negative pressure inside the VB using a hand pump. A distance and differential pressure measuring device (DPMD) enables us for the first time to assess objectively those parameters. METHODS: After approval by the institutional review board, 53 patients recruited from our outpatient clinic were included in this retrospective study and distributed into three groups (group 1 aged 6 to 10years; group 2 aged 11 to 15years; group 3 aged 16 to 20years). Sternum elevation and differential negative pressure inside the VB compared to atmospheric pressure were assessed with the DPMD, a device developed by engineers at the University of Applied Sciences, Northwestern Switzerland. Pressure-elevation curves were recorded during VB therapy. For statistical comparison of the groups, analysis of variance was used. Post-hoc analysis was performed using the Tukey-Kramer test. A p-value of less than 0.05 was considered to be statistically significant. RESULTS: The VB therapy was monitored in 53 children (39 males, 14 females) aged from 6 to 20years (average, 14years). Relationships were established between the differential negative pressure inside the vacuum bell, the elevation of the sternum, and the patient's age. The younger the patient, the lower is the differential negative pressure difference required to obtain a complete elevation of the sternum. Patient's age, weight, the pectus depth, the differential negative pressure inside the VB, and the elevation of the sternum were correlated. When comparing the depth 25 of the pectus excavatum to the patient's age, a statistically significant difference was verified between the groups 3 and 1 (p=0.0291) and 3 and 2 (p=0.0489). The older the patient, the deeper is the pectus excavatum. However, no statistically significant difference between the groups was found when comparing the sternum elevation to the patient's age (p=0.4574) and the elevation to pressure ratio to the patient's age (p=0.8048). The sternum elevation and the elevation to pressure ratio are independent of the patient's age. CONCLUSIONS: DPMD supplies objective data of the elevation of the sternum and the related pressure inside the VB during its application. Correlation between the patient's age, the elevation of the sternum and the pressure inside the VB were demonstrated, but additional data are needed to better understand their relationship and their impact in the treatment of PE by VB. STUDY TYPE: Diagnostic Study. LEVEL OF EVIDENCE: IV.

Pectus excavatum repair after sternotomy: the Chest Wall International Group experience with substernal Nuss bars.

OBJECTIVES: Patients with pectus excavatum (PE) after prior sternotomy for cardiac surgery present unique challenges for repair of PE. Open repairs have been recommended because of concerns about sternal adhesions and cardiac injury. We report a multi-institutional experience with repair utilizing substernal Nuss bars in this patient population. METHODS: Surgeons from the Chest Wall International Group were queried for experience and retrospective data on PE repair using sub-sternal Nuss bars in patients with a history of median sternotomy for cardiac surgery (November 2000 to August 2015). A descriptive analysis was performed. RESULTS: Data for 75 patients were available from 14 centres. The median age at PE repair was 9.5 years (interquartile range 10.9), and the median Haller index was 3.9 (interquartile range 1.43); 56% of the patients were men. The median time to PE repair was 6.4 years (interquartile range 7.886) after prior cardiac surgery. Twelve patients (16%) required resternotomy before support bar placement: 7 pre-emptively and 5 emergently. Sternal elevation before bar placement was used in 34 patients (45%) and thoracoscopy in 67 patients (89%). Standby with cardiopulmonary bypass was available at 9 centres (64%). Inadvertent cardiac injury occurred in 5 cases (7%) without mortality. CONCLUSIONS: Over a broad range of institutions, substernal Nuss bars were used in PE repair for patients with a history of sternotomy for cardiac surgery. Several technique modifications were reported and may have facilitated repair. Cardiac injury occurred in 7% of cases, and appropriate resources should be available in the event of complications. Prophylactic resternotomy was reported at a minority of centres.

Vacuum bell therapy.

BACKGROUND: For specific therapy to correct pectus excavatum (PE), conservative treatment with the vacuum bell (VB) was introduced more than 10 years ago in addition to surgical repair. Preliminary results using the VB were encouraging. We report on our 13-year experience with the VB treatment including the intraoperative use during the Nuss procedure and present some technical innovations. METHODS: A VB with a patient-activated hand pump is used to create a vacuum at the anterior chest wall. Three different sizes of vacuum bells, as well as a model fitted for young women, exist. The appropriate size is selected according to the individual patient's age and ventral surface. The device should be used at home for a minimum of 30 minutes (twice a day), and may be used up to a maximum of several hours daily. The intensity of the applied negative pressure can be evaluated with an integrated pressure gauge during follow-up visits. A prototype of an electronic model enables us to measure the correlation between the applied negative pressure and the elevation of the anterior chest wall. RESULTS: Since 2003, approx. 450 patients between 2 to 61 years of age started the VB therapy. Age and gender specific differences, depth of PE, symmetry or asymmetry, and concomitant malformations such as scoliosis and/or kyphosis influence the clinical course and success of VB therapy. According to our experience, we see three different groups of patients. Immediate elevation of the sternum was confirmed thoracoscopically during the Nuss procedure in every patient. CONCLUSIONS: The VB therapy has been established as an alternative therapeutic option in selected patients suffering from PE. The initial results up to now are encouraging, but long-term results comprising more than 15 years are so far lacking, and further evaluation and follow-up studies are necessary.

Pulmonary function in pectus excavatum patients before repair with the Nuss procedure.

OBJECTIVES: Whether the origin of symptoms in pectus excavatum patients (Pex) is related to reduced pulmonary function or impaired cardiovascular performance is debatable. However, pulmonary function testing (PFT) is still part of the evaluation prior to surgical repair in Pex. The purpose of this study was to corroborate our hypothesis that the majority of Pex that qualified for surgery present preoperatively with normal or close to normal PFT. METHODS: After institutional review board approval, preoperative PFT data of Pex who underwent surgical repair were analyzed retrospectively: total lung capacity (TLC), vital capacity (VC), functional residual capacity, forced expiratory volume in 1 second (FEV1) and maximal expiratory flow at 25% of FVC (MEF25). RESULTS: 82 patients aged from 9 to 27 years (average, 15 years) underwent PFT. A restrictive pattern (VC<80%) was observed in 45%, an obstructive pattern (FEV1 < 75 %) in 35%, and a normal total lung capacity in 62% of the Pex. No significant correlation was noted between the increasing severity of the Haller index and the PFT. CONCLUSION: Adolescent Pex without relevant respiratory symptoms have nearly a normal lung function. We suggest to skip PFT from the routine preoperative assessment in asymptomatic Pex.

Non-surgical treatment of pectus excavatum.

BACKGROUND: Previously used procedures to correct pectus excavatum (PE) were largely based on surgical techniques like Ravitch procedure or the minimally invasive Nuss technique. Conservative treatment with the vacuum bell (VB) to elevate the funnel in patients with PE, represents a potential alternative to surgery in selected patients. METHODS: A suction cup is used to create a vacuum at the anterior chest wall. Three different sizes as well as a model fitted for young women of VB exist which are selected according to the individual patients age. A patient-activated hand pump is used to create a vacuum at the anterior chest wall. When creating the vacuum, the lift of the sternum is obvious and remains for a different time period. The device should be used for a minimum of 30 minutes (2/day), and may be used up to a maximum of several hours daily. We have an IRB approval for it. Since this paper was conducted as a retrospective study, we did not have to have informed consent of every patient. RESULTS: CT-scans showed that the device lifted the sternum and ribs immediately. In addition, this was confirmed thoracoscopically during the MIRPE procedure. Preliminary results published within the last 10 years proved to be successful. CONCLUSIONS: The VB has been established as an alternative therapeutic option in selected patients suffering from PE. The initial results proved to be dramatic, but long-term results comprising more than 15 years are so far lacking, and further evaluation and follow-up studies are necessary.

Neurogenic appendicopathy: clinical, macroscopic, and histopathological presentation in pediatric patients.

BACKGROUND: The proliferation of nerve fibers in the appendix, in association with an increase of the neuropeptides such as vasoactive intestinal peptide and substance P characterizes neurogenic appendicopathy (NA), which may mimic the symptoms of acute appendicitis (AA). It may be difficult to differentiate this little known distinct pathological entity clinically from AA. The aim of this study is to describe the epidemiology, clinical signs, and histological description of NA in pediatric patients. PATIENTS AND METHODS: After institutional review board approval, all appendiceal specimens from children who underwent appendectomy between February 2006 and December 2008 were analyzed. Analysis included age, gender, clinical symptoms, blood tests, intraoperative macroscopic assessment of the appendix, and the histopathological type of NA. NA was diagnosed by hematoxylin-eosin staining and /or S-100 immunochemistry and then classified into different histopathological groups. RESULTS: Of the 385 appendix specimens examined, 29 (7.5%) met the histopathological criteria of NA. The median duration of abdominal pain was 2 days (range, 1 to 7). The surgeon characterized the appendix as follows: not inflamed in 5 (17.2%), acute in 17 (58.6%), phlegmonous in 4 (13.8%), and perforated in 3 patients (10.4%). Histopathological diagnosis of NA classified the specimen as axial neuroma in 2 (7%), mucosal in 12 (41%), and submucosal in 15 cases (52%). CONCLUSION: NA is a relatively common diagnosis in children. NA must be considered in patients with recurrent right lower quadrant pain or signs of AA, even if intraoperative findings are normal. For these patients, we recommend appendectomy and consideration of a subsequent histopathological workup.