Tratamiento del pectus excavatum con campana de succión durante la pubertad / Treatment of pectus excavatum with vacuum bell during puberty

Objetivo: Evaluar la eficacia de la campana de succión durante lapubertad, según las horas diarias de uso y la duración del tratamiento. Material y métodos: Se evaluaron retrospectivamente los pacientestratados con campana de succión durante la pubertad en el periodo 2010-2021. Se recogieron diferentes variables, incluyendo el hundimientoinicial y final, el hundimiento corregido expresado en centímetros y enporcentaje con respecto al hundimiento inicial, las horas diarias de uso,la duración del tratamiento y las complicaciones. Se categorizaron lospacientes en grupos según las horas diarias de uso (≤ 3 horas; 4-5 horas;≥ 6 horas) y la duración del tratamiento (6-12 meses; 13-24 meses; 25-36meses; > 36 meses), y se analizaron estadísticamente. Resultados: Se estudiaron un total de 50 pacientes; 41 varones y9 mujeres, con una edad media de 12,5 años (rango 10-14 años). Nose observaron diferencias significativas entre los diferentes grupos enrelación con el hundimiento inicial, el índice torácico y el hundimientofinal. El hundimiento corregido aumentó en relación con las horas diariasde uso, con diferencias significativas. Las complicaciones fueron leves,3 pacientes abandonaron el seguimiento y 5 pacientes de los 25 quefinalizaron el tratamiento, alcanzaron una buena corrección. Conclusiones: Para aumentar la eficacia del tratamiento, el tiempode uso de la campana de succión durante la pubertad debería alcanzarlas 6 horas diarias. Este método es bien tolerado, presenta leves com-plicaciones y puede ser una alternativa a la cirugía en algunos casos.(AU)

Objective: To assess the efficacy of the vacuum bell during puberty,according to the daily hours of use and treatment duration.Materials and methods: A retrospective analysis of patients treatedwith vacuum bell during puberty in the 2010-2021 period was carried out. Several variables were collected, including baseline and finalsinking, repaired sinking expressed in cm and as a percentage frombaseline sinking, daily hours of use, treatment duration, and complications. Patients were categorized into groups according to the daily hoursof use (≤ 3 hours; 4-5 hours; ≥ 6 hours) and treatment duration (6-12months; 13-24 months; 25-36 months; > 36 months), and they werestatistically analyzed.Results: A total of 50 patients – 41 male and 9 female – were studied, with a mean age of 12.5 years (range: 10-14 years). No significantdifferences among groups were observed in terms of baseline sinking,thoracic index, and final sinking. Repaired sinking did increase withthe daily hours of use, with significant differences. Complications weremild. 3 patients withdrew from follow-up, and 5 out of the 25 patientswho completed treatment achieved a good repair. Conclusions: To increase treatment efficacy, the vacuum bell shouldbe used for 6 hours/day during puberty. This method is well-tolerated,causes mild complications, and may be an alternative to surgery insome cases.(AU)

Impact of Cryoablation on Pectus Excavatum Repair in Pediatric Patients.

BACKGROUND: Minimally invasive repair of pectus excavatum (MIRPE) involves placement of a transthoracic, retrosternal support bar under thoracoscopic guidance. Despite its minimally invasive technical approach, postoperative pain is a significant morbidity that often results in increased length of stay. Multi-modal pain control strategies have been used in the past with limited success. Recently, the use of intraoperative intercostal nerve cryoablation (CA) has been added. In the present study, we aim to evaluate the effects of CA on postoperative pain control, opioid requirements, and perioperative outcomes. STUDY DESIGN: A single-center, retrospective chart review of all patients (less than 18 years old) who underwent MIRPE from 2009 to 2020 was performed. CA was started in June 2018. Data collection included demographics, preoperative characteristics, intraoperative findings, and postoperative outcomes. We hypothesized that CA would be associated with improved pain scores, lower doses of total inpatient opioid requirement, and shorter length of stay (LOS). RESULTS: One hundred sixty-one patients met inclusion criteria: 75 underwent intraoperative CA and 86 underwent MIRPE without CA (NCA group). CA significantly decreased median LOS from 4 days in NCA to 2 days; the use of CA was the only significant predictor of LOS on linear regression. CA was also associated with decreased total PCA, intravenous opioid, and oral opioid dosages. There was no difference in inpatient pain scores and a slight increase in mean procedure time. However, CA was associated with significantly decreased postoperative complications. CONCLUSIONS: The use of cryoablation during MIRPE significantly decreases LOS, perioperative opioid requirements, and postoperative complications, with a minimal increase in operative time. Cryoablation is an effective pain control modality in the surgical management of chest wall deformities in children.

Novel Biodegradable 3D-Printed Analgesics-Eluting-Nanofibers Incorporated Nuss Bars for Therapy of Pectus Excavatum.

A novel hybrid biodegradable Nuss bar model was developed to surgically correct the pectus excavatum and reduce the associated pain during treatment. The scheme consisted of a three-dimensional (3D) printed biodegradable polylactide (PLA) Nuss bar as the surgical implant and electrospun polylactide-polyglycolide (PLGA) nanofibers loaded with lidocaine and ketorolac as the analgesic agents. The degradation rate and mechanical properties of the PLA Nuss bars were characterized after submersion in a buffered mixture for different time periods. In addition, the in vivo biocompatibility of the integrated PLA Nuss bars/analgesic-loaded PLGA nanofibers was assessed using a rabbit chest wall model. The outcomes of this work suggest that integration of PLA Nuss bar and PLGA/analgesic nanofibers could successfully enhance the results of pectus excavatum treatment in the animal model. The histological analysis also demonstrated good biocompatibility of the PLA Nuss bars with animal tissues. Eventually, the 3D printed biodegradable Nuss bars may have a potential role in pectus excavatum treatment in humans.

Successful use of non-invasive positive pressure ventilation in a patient with the severe form of X-linked myotubular myopathy.

The severity of X-linked myotubular myopathy (XLMTM) ranges from mild to severe, depending on the level of ventilatory support required. Patients with the severe form of XLMTM usually die within the first year of life due to respiratory failure. Most survivors need tracheostomies, and there has only been one report about the use of non-invasive positive pressure ventilation (NPPV) in patients with the severe form of XLMTM because of the severity of the associated respiratory failure. We successfully applied NPPV with high-span positive inspiratory pressure (PIP) in a patient with the severe form of XLMTM, who also had secondary pectus excavatum. About a year after the initiation of NPPV with high-span PIP, the patient's pectus excavatum had improved. As the patient's pectus excavatum improved, his respiratory disturbance was ameliorated, and the frequency of respiratory infections gradually decreased. NPPV might be the first-choice respiratory management strategy for patients with XLMTM.

[Adrenocortical function in children with connective tissue syndromes and funnel chest deformation]. / Funktsional'noe sostoianie kory nadpochechnikov u detei s soedinitel'notkannymi sindromami i voronkoobraznoi deformatsiei grudnoi kletki.

Measurement of the content of C-21-corticosteroids in the diurnal urine by thin-layer chromatography has demonstrated that in children with isolated and syndromal forms of funnel-like chest deformation, grades II and III, adrenocortical function is decreased. In preschool children, such a decrease is accompanied by proneness to the tense biosynthesis of glucocorticoids. Children with congenital hereditary connective syndromes manifest adrenocortical dysfunction, most characteristic of Marfan's syndrome, and, to a less degree, of Ehlers-Danlos syndrome and unclassified complex of developmental defects with the Marfan-like phenotype. In view of this fact, the children with Marfan's, Ehlers-Danlos syndromes and with the unclassified complex of developmental defects with the Marfan-like phenotype and children with funnel-like chest deformation, grades II and III, should be given glucocorticoid hormones in stressful conditions (severe diseases, traumas, operations).

[Effect of therapy with beta-adrenoblockers and vitamin complexes on indices of oxyproline excretion in various hereditary connective tissue diseases]. / Vliianie terapii beta-adrenoblokatorom i kompleksom vitaminov na pokazateli ékskretsii oksiprolina pri nekotorykh nasledstvennykh bolezniakh soedinitel'noi tkani.

Excretion of hydroxyproline with urine was studied in 16 children (5-14 years old) with Marphan-Like syndrome and Marphan, Ehlers-Dunlos and Larson syndromes after therapy involving propranolol and a complex of vitamins (ascorbic acid, riboflavin and pyridoxine) and recommended on the basis of echocardiographic analyses. The therapeutic course appears to cause quantitative and qualitative correction of collagen and apparently of elastin fibrilles development. Depending on initial patterns of hydroxyproline excretion and the syndrome form the correction could be complete or partial, while positive effect of the treatment was stable or provisional. The data obtained suggest that the complex treatment developed might be applied as a preoperative therapy of the patients with Marphan-like syndrome as well as with syndromes of Marphan and Ehlers-Dunlos before thoracoplastics caused by hereditary chest deformation and by impairments of cardiovascular system.