The vacuum bell for conservative treatment of pectus excavatum: the Basle experience

Objective: Surgical repair of pectus excavatum (PE) in childhood is a well-established procedure. Previously used operative techniques to correct PE were largely based on the Ravitch technique. Since about 10years, the minimally invasive repair (MIRPE) by Nuss is well established. Conservative treatment with the vacuum bell 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 of vacuum bell exist which are selected according to the individual patients age. 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 30min (twice/day), and may be used up to a maximum of several hours daily. Results: One hundred and thirty-three patients (110 males, 23 females) aged from 3 to 61years (median 16.21years) used the vacuum bell for 1 to a maximum of 36months. Computed tomographic scans showed that the device lifted the sternum and ribs immediately. In addition, this was confirmed thoracoscopically during the MIRPE procedure. One hundred and five patients showed a permanent lift of the sternum for more than 1cm after 3months of daily application. Thirteen patients stopped the application and underwent MIRPE. Relevant side effects were not noted. Conclusion: The vacuum bell has proved to be an alternative therapeutic option in selected patients suffering from PE. The initial results proved to be dramatic, but long-term results are so far lacking, and further evaluation and follow-up studies are necessar

The vacuum bell for treatment of pectus excavatum: an alternative to surgical correction?

Objective: Pectus excavatum (PE) is the most common chest wall malformation and one of the most frequent major congenital anomalies. The surgical repair of PE in childhood is a well-established procedure. Previously used operative techniques to correct PE were largely based on the Ravitch technique. Today, the minimally invasive repair (MIRPE) by Nuss is well established. Conservative treatment with the vacuum bell to elevate the funnel in patients with PE represents a potential alternative. Methods: A suction cup is used to create a vacuum at the anterior chest wall. A patient-activated hand pump is used to reduce the pressure up to 15% below atmospheric pressure. Three different sizes of vacuum bell exist which are selected according to the individual patients age. 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 min (2 per day), and may be used up to a maximum of several hours daily. Presently, a 12-15-month course of treatment is recommended. In addition, the device was used intraoperatively during the MIRPE procedure to enlarge the retrosternal space to ensure safer passage of the introducer in a few patients. Results: Thirty-four patients (31 males, 3 females), aged 6-52 years (median 17.8 years) used the vacuum bell for 1 to maximum 18 months (median 10.4 months). Follow-up included photography and clinical examination every 3 months. Computed tomographic scans showed that the device lifted the sternum and ribs immediately. In addition, this was confirmed thoracoscopically during the MIRPE procedure. After 3 months, an elevation of more than 1.5 cm was documented in 27 patients (79%). After 12 months, the sternum was lifted to a normal level in five patients (14.7%). Relevant side effects were not noted. Conclusions: The vacuum bell has proved to be an alternative therapeutic option in selected patients with PE. The initial results proved to be dramatic, but long-term results are so far lacking, and further evaluation and follow-up studies are necessary. In addition, the method may assist the surgeon during the MIRPE procedur

Mechanical Complication with Broviac Repair Kit in a 4-Year-Old Boy with MEN 2a

Background. Mechanical complications in the use of indwelling central venous catheters (CVCs) such as the Broviac catheter (BC) include kinking, occlusion, dislocation or leaking. We report on a mechanical complication after using a repair kit for the BC. Method. A 4-year old boy, suffering from multiple endocrine neoplasia type 2a (MEN 2a), intestinal aganglionosis (Hirschsprung's disease), and short bowel syndrome, required a BC for home parenteral nutrition. Result. Due to recurrent leakage of the BC, 5 subsequent repairs were necessary within seven months. During one repair a metallic tube belonging to the repair kit was found to have migrated proximally to the skin entrance level within the BC and requiring surgical removal. Conclusion. To our knowledge, this is the first report focusing on such a serious complication using a BC and its repair kit. The proximal migration of this metallic tube constitutes a distinct theoretical risk of endothoracic foreign body embolization

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

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

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

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

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