Suture fixation of traumatic rib fracture flail segment with Klebsiella osteomyelitis.

We report the case of a 71-year-old male who initially presented with urosepsis and was found to have a rib fracture of his right 6th rib with a flail segment and an associated abscess. Given the concern for infection, surgical rib fixation with titanium plating was not pursued during the washout of his abscess and instead, he successfully underwent rib fracture stabilization with bilateral suture transfixation. He was continued on a prolonged course of antibiotics for Klebsiella pneumonia osteomyelitis and was discharged uneventfully with optimal pain control and adequate respiratory effort.

Spinal Muscular Atrophy (SMA) Mortality Despite Novel Medications: Case Reports.

ABSTRACT: Despite new effective medications, patients with SMA types 1-3 can continue to have inadequate cough flows to prevent episodes of acute respiratory failure. Ventilator unweanable intubated patients are thought to require tracheostomy tubes. As a result, potentially beneficial medications may be discontinued and patients die despite receiving these medications. Three cases are presented of medically treated, physically strengthening children, with SMA type 1. All three subsequently died or underwent tracheotomy. However, there is no evidence of extubation attempts to noninvasive ventilatory support settings or optimal mechanical insufflation-exsufflation despite this option being described to be over 98% successful for extubating unweanable medically untreated children with SMA1.

An initiative to assess and improve the resources and patient care processes used among Chest Wall Injury Society collaborative centers study (CWIS-CC2).

BACKGROUND: Over the last two decades, the acute management of rib fractures has changed significantly. In 2021, the Chest Wall injury Society (CWIS) began recognizing centers that epitomize their mission as CWIS Collaborative Centers. The primary aim of this study was to determine the resources, surgical expertise, access to care, and institutional support that are present among centers. METHODS: A survey was performed including all CWIS Collaborative Centers evaluating the resources available at their hospital for the treatment of patients with chest wall injury. Data about each chest wall injury center care process, availability of resources, institutional support, research support, and educational offerings were recorded. RESULTS: Data were collected from 20 trauma centers resulting in an 80% response rate. These trauma centers were made up of 5 international and 15 US-based trauma centers. Eighty percent (16 of 20) have dedicated care team members for the evaluation and management of rib fractures. Twenty-five percent (5 of 20) have a dedicated rib fracture service with a separate call schedule. Staffing for chest wall injury clinics consists of a multidisciplinary team: with attending surgeons in all clinics, 80% (8 of 10) with advanced practice providers and 70% (7 of 10) with care coordinators. Forty percent (8 of 20) of centers have dedicated rib fracture research support, and 35% (7 of 20) have surgical stabilization of rib fracture (SSRF)-related grants. Forty percent (8 of 20) of centers have marketing support, and 30% (8 of 20) have a web page support to bring awareness to their center. At these trauma centers, a median of 4 (1-9) surgeons perform SSRFs. In the majority of trauma centers, the trauma surgeons perform SSRF. CONCLUSION: Considerable similarities and differences exist within these CWIS collaborative centers. These differences in resources are hypothesis generating in determining the optimal chest wall injury center. These findings may generate several patient care and team process questions to optimize patient care, patient experience, provider satisfaction, research productivity, education, and outreach. LEVEL OF EVIDENCE: Therapeutic/Care Management; Level V.

Spinal Muscular Atrophy Type 1 Survival Without New Pharmacotherapies: Two Treatment Paradigms.

OBJECTIVES: The aims of the study are to present noninvasive respiratory management outcomes using continuous noninvasive ventilatory support and mechanical in-exsufflation from infancy for spinal muscular atrophy type 1 and to consider bearing on new medical therapies. DESIGN: Noninvasive ventilatory support was begun for consecutively referred symptomatic infants with spinal muscular atrophy type 1 from 1 to 10 mos of age. Intercurrent episodes of respiratory failure were managed by intubation then extubation to continuous noninvasive ventilatory support and mechanical in-exsufflation despite failing ventilator weaning and extubation attempts. Intubations, tracheotomies, and survival were monitored. RESULTS: Of 153 patients with spinal muscular atrophy 1 consecutively referred since 1995, 37 became continuous noninvasive ventilatory support dependent, almost half before 10 yrs of age. Of the 37, 18 required continuous noninvasive ventilatory support for a mean 18.6 ± 3.3 yrs to a mean 25.3 (range, 18-30) yrs of age, dependent from as young as 4 mos of age with 0 to 40 ml of vital capacity. One of the 18 died from COVID-19 acute respiratory distress syndrome at age 24 after 23 yrs of continuous noninvasive ventilatory support. Extubation success rate of 85% per attempt (150/176) resulted in only one undergoing tracheotomy. CONCLUSIONS: Medical treatments begun during the first 6 wks of age convert spinal muscular atrophy 1 into spinal muscular atrophy 2 or 3 but cough flows remain inadequate to avoid many pneumonias that, once resolved by a treatment paradigm of extubation to continuous noninvasive ventilatory support and mechanical in-exsufflation, eliminates need to resort to tracheotomies.

Hardware failure for anterior chest wall and costal cartilage injuries: A single-center experience.

BACKGROUND: Often missed in blunt chest wall injury, costal cartilage injuries can cause chest wall instability, refractory pain, and deformity. Notably, there is only a small amount of evidence regarding hardware performance when applied to costal cartilage. In a prior multicenter study, hardware failure rate was found to be approximately 3% following surgical stabilization of rib fractures (SSRFs) for all fracture locations. The aim of the current study was to evaluate hardware performance for costal cartilage injuries. METHODS: All patients undergoing SSRF performed at our institution from 2016 to 2022 were queried, including both acute and chronic injuries. Both radiographic and clinical follow-up were retrospectively reviewed to evaluate for hardware failure defined as plate fracture, malposition, or screw migration following cartilage fixation. RESULTS: After screening 359 patients, 43 were included for analysis. Mean age was 64 years, and 67% of patients were male. Median number of fractures per patient was 7 with 60% of patients sustaining a flail chest injury pattern. Median total plates per operation was 6 and median costal cartilage plates was 3. In total, 144 plates were applied to the costal cartilage for the group. Mean follow-up was as follows: clinical 88 days, two-view plain radiography 164 days, and chest computed tomography 184 days. Hardware failure was observed in 3 of 144 plates (2.1%), in three separate patients. Two cases were asymptomatic and did not require intervention. One patient required revisional operation in the acute setting. CONCLUSION: In our institution, hardware failure for costal cartilage fractures was observed to be 2.1%. This aligns with prior reports of hardware failure during SSRF for all injury locations. Surgical stabilization of anterior and cartilaginous chest wall injury with appropriate plate contouring and fixation technique appears to provide adequate stabilization with a relatively low rate of hardware malfunction. LEVEL OF EVIDENCE: Therapeutic/Care Management; Level V.

Use of Low-Cost Task Trainer for Emergency Department Thoracotomy Training in General Surgery Residency Program.

OBJECTIVE: Emergency department thoracotomy (EDT) is an uncommon but potentially lifesaving procedure that warrants familiarity with anatomy, instruments, and indications necessary for completion. To address this need, we developed a low-cost EDT trainer. The primary objective of this study was to compare the effectiveness of a low-cost EDT trainer to teach emergency department thoracotomy with a discussion-based teaching session. Secondary objective was to study the face validity of the low-cost EDT trainer. DESIGN: A prospective 2-phase randomized control study was conducted. Participants were randomly divided into two groups. In phase one, baseline medical knowledge for both groups was assessed using a multiple-choice question pretest. In Group 1, each participant was taught EDT using a one-on-one discussion with a trauma surgeon, whereas Group 2 used the EDT trainer and debriefing for training. In phase 2 (1 month later), all participants completed a knowledge retention test and performed a videoed EDT using our EDT trainer, the video recordings were later reviewed by content experts blinded to the study participants using a checklist with a maximum score of 22. The participants also completed a reaction survey at the end of phase 2 of the study. SETTING: OhioHealth Riverside Methodist Hospital, an urban tertiary care academic hospital in Columbus, Ohio. PARTICIPANTS: Nine senior surgery residents from training years 3 to 5. RESULTS: The mean score for the performance of the procedure for the simulation-based (Group 2) was significantly higher than that of the discussion-based (Group 1) (Rater 1: 21.2 ± 0.8 vs. 19.0 ± 2.0, p = 0.05, Rater 2: 20.4 ± 1.5 vs. 18.3±1.0, p = 0.04). Group 2 also was quicker than Group 1 in deciding to start the procedure by approximately 56 seconds. When comparing the mean pretest knowledge score to the mean knowledge retention score 30 days after training, the discussion-based group improved from 58.33% to 81.25% (p = 0.01); the simulation-trained group's scores remained at 68.33%. All the participants agreed or strongly agreed that the simulator provided a realistic opportunity to perform EDT and improved their confidence. CONCLUSIONS: The results of this pilot study support our hypothesis that using a low-cost EDT trainer effectively improves general surgery residents' confidence and procedural skills scores in a simulated environment. Further training with low-cost simulators may provide surgical residents with deliberate practice opportunities and improve performance when learning low-frequency procedures.

Mechanical Insufflation-Exsufflation Implementation and Management, Aided by Graphics Analysis.

Mechanical insufflation-exsufflation (MIE) facilitates airway clearance to mitigate respiratory infection, decompensation, and ultimately the need for intubation and placement of a tracheostomy tube. Despite widespread adoption as a respiratory support intervention for motor neuron disease, muscular dystrophy, spinal cord injury, and other diseases associated with ventilatory pump failure and ineffective cough peak flow, there is debate in the clinical community about how to optimize settings when MIE is implemented. This article will demonstrate the clinical utility of MIE graphics in titrating the initial MIE settings, guiding upper airway and lung protective strategies and providing insight to clinicians for ongoing clinical management.

Intermittent abdominal pressure ventilation management in neuromuscular diseases: a Delphi panel Consensus.

BACKGROUND: Intermittent abdominal pressure ventilator (IAPV) use started in the 1930s for ventilatory assistance with muscular dystrophy patients. Later, the device was perfected and expanded for other neuromuscular disorders (NMD). In recent years, the morbidity and mortality tracheotomies and trach tubes related renewed the interest around IAPV. However, there are no guidelines for its use. This study aimed to establish a consensus among physicians involved in its practice to provide IAPV suggestions for the treatment of patients with NMD. METHOD: A 3-step modified Delphi method was used to establish consensus. Fourteen respiratory physicians and one psychiatrist with strong experience in IAPV use and/or who published manuscripts on the topic participated in the panel. A systematic review of the literature was carried out according to the PRISMA to identify existing evidence on IAPV for patients with neuromuscular disorders. RESULTS: In the first round, 34 statements were circulated. Panel members marked 'agree' or 'disagree' for each statement and provided comments. The agreement was reached after the second voting session for all 34 statements. CONCLUSIONS: Panel members agreed and IAPV indications, parameter settings (including procedure protocol), potential limitations, contraindications, complications, monitoring, and follow-up are described. This is the first expert consensus on IAPV.

In- and out-of-hospital outcomes following surgical stabilization of rib fractures in 80 years and older: A single-institution experience.

BACKGROUND: Surgical stabilization of rib fractures (SSRF) has demonstrated benefit in patients with flail chest and multiple displaced fractures. There is mounting evidence for SSRF following chest wall injury (CWI) for the geriatric trauma population. A recent multi-center retrospective study highlighted a mortality benefit even for those patients aged 80 years and older. The objective of this investigation was to review our institutional experience with both in- and out-of-hospital outcomes within this patient population following SSRF. METHODS: A retrospective review of patients 80 years and older was performed at a high-volume level 2 trauma center from 2017 to 2021. SSRF volume is routinely >60 cases per year. Perioperative, inpatient, and outpatient data were collected as available. Primary outcomes were inpatient and 90-day mortality. Secondary outcomes included discharge on narcotics and freedom from narcotics at 30 days. RESULTS: 50 patients were included for review. Mean age was 86 years and mechanism of injury was most often fall. 28 of 50 (56%) patients had flail chest (radiographic). Mean number of ribs fixated was 4.7 and time to surgery 2.5 days. Inpatient mortality was 3/50 (6%), 90-day mortality was 9/50 (18%) of which three were attributable primarily to CWI (6/50, 12%). Of patients with follow-up of 1 year and beyond, 27/28 were alive (96%). With respect to narcotic consumption, 45% (21/47) were discharged on narcotics with 90% (28/31; N limited by missing data) being narcotic-free at 30 days. CONCLUSION: In this high-risk patient population, inpatient mortality was comparably low to prior reports, though 90-day mortality was doubled when incorporating CWI-related deaths. Narcotic use was seen in the minority of patients upon discharge, and most progressed to being narcotic-free at 30 days post-hospitalization. Inpatient outcomes alone may not adequately define both the benefit and risk of SSRF performed in patients 80 years and older.

Mechanical Insufflation-Exsufflation: The Rest of the Story.

Mechanical insufflation-exsufflation has been reported to decrease pneumonia rates by about 90% for patients with Duchenne muscular dystrophy now living into their 40s and 50s without tracheotomy tubes. It greatly reduces respiratory complications and hospitalization rates to less than one per 10 patient-years for advanced spinal muscular atrophy type 1, through 25-30 years of age. It is most successful from the point at which small children become able to cooperate with it, generally from 3 to 5 years of age. However, since the 1950s, successful use to extubate and decannulate ventilator "unweanable" patients with little to no measurable vital capacity without resorting to tracheostomy has always been at pressures of 50-60 cm H2O via oronasal interfaces and at 60-70 cm H2O via airway tubes when present. It must usually also be used in conjunction with up to continuous noninvasive positive pressure ventilatory support. Centers that use these effectively have eliminated need to resort to tracheotomies for people with muscular dystrophies and spinal muscular atrophies, including unmedicated patients with spinal muscular atrophy type 1. Barotrauma has been rare despite dependence on it and noninvasive ventilatory support. Despite this, noninvasive respiratory management continues to be widely underutilized.

High-Level Spinal Cord Injury and the Failure of US Acute Rehabilitation: An Analysis and Commentary.

ABSTRACT: Acute and long-term morbidity and mortality rates have not changed in the United States for people with high-level spinal cord injury in 40 yrs, neither has the conventional invasive respiratory management for these patients. This is despite a 2006 challenge to institutions for a paradigm shift to avoid or decannulate patients of tracheostomy tubes. Centers in Portugal, Japan, Mexico, and South Korea decannulate high-level patients to up to continuous noninvasive ventilatory support and use mechanical insufflation exsufflation, as we have done and reported since 1990, but there has been no such paradigm shift in US rehabilitation institutions. The quality of life and financial consequences of this are discussed. An example of decannulation of a relatively easy case, after failure to do so during 3 mos of acute rehabilitation, is presented to encourage institutions to begin to learn and apply noninvasive management before decannulating more severe patients with little to no ventilator free breathing ability.

Surgical stabilization of severe chest wall injury following cardiopulmonary resuscitation.

BACKGROUND: Cardiopulmonary resuscitation (CPR) contributes to significant chest wall injury similar to blunt trauma. With benefits realized for surgical stabilization of rib fractures (SSRFs) for flail injuries and severely displaced fractures following trauma, SSRF for chest wall injury following CPR could be advantageous, provided good functional and neurologic outlook. Experience is limited. We present a review of patients treated with SSRF at our institution following CPR. METHODS: A retrospective analysis of patients undergoing SSRF following CPR was performed between 2019 and 2020. Perioperative inpatient data were collected with outpatient follow-up as able. RESULTS: Five patients underwent SSRF over the course of the 2-year interval. All patients required invasive ventilation preoperatively or had impending respiratory. Mean age was 59 ± 12 years, with all patients being male. Inciting events for cardiac arrest included respiratory, ventricular tachycardia, ventricular fibrillation, pulseless electrical activity, and anaphylaxis. Time to operation was 6.6 ± 3 days. Four patients demonstrated anterior flail injury pattern with or without sternal fracture, with one patient having multiple severely displaced fractures. Surgical stabilization of rib fracture was performed appropriately to restore chest wall stability. Mean intensive care unit length of stay was 9.8 ± 6.4 days and overall hospital length of stay 24.6 ± 13.2 days. Median postoperative ventilation was 2 days (range, 1-15 days) with two patients developing pneumonia and one requiring tracheostomy. There were no mortalities at 30 days. One patient expired in hospice after a prolonged hospitalization. Disposition destination was variable. No hardware complications were noted on outpatient follow-up, and all surviving patients were home. CONCLUSION: Chest wall injuries are incurred frequently following CPR. Surgical stabilization of these injuries can be considered to promote ventilator liberation and rehabilitation. Careful patient selection is paramount, with surgery offered to those with reversible causes of arrest and good functional and neurologic outcome. Experience is early, with further investigation needed. LEVEL OF EVIDENCE: Therapeutic, Level V.

Amyotrophic Lateral Sclerosis and Noninvasive Positive Pressure Ventilatory Support: "Nasal Noninvasive Ventilation" or "Noninvasive Ventilatory Support"?

ABSTRACT: Many studies suggest a brief statistical benefit on survival and quality of life by using nasal noninvasive ventilation for patients with amyotrophic lateral sclerosis and other neuromuscular conditions. Indeed, nasal noninvasive ventilation has become synonymous with continuous positive airway pressure and lo-span bilevel positive airway pressure. Nasal noninvasive ventilation, however, may not normalize CO2 levels and continuous positive airway pressure and O2 exacerbate hypercapnia and often lead to CO2 narcosis, intubation, and ultimately tracheostomy or palliative care death. However, a third option can be to offer up to continuous noninvasive ventilatory support and extubation to it. Noninvasive ventilatory support can be effective for full, definitive ventilatory support, even for people with no measurable vital capacity, and has maintained classic amyotrophic lateral sclerosis patients for up to 12 yrs without resort to tracheotomies. Nineteen centers have reported 335 amyotrophic lateral sclerosis patients using continuous noninvasive ventilatory support instead of tracheostomy mechanical ventilation for an average of 14 mos (6 mos to 14 yrs). The noninvasive ventilatory support must also be used in conjunction with mechanical insufflation-exsufflation to clear airway debris and normalize or renormalize ambient air oxyhemoglobin saturation, both to avoid intubation and to facilitate extubation. People with amyotrophic lateral sclerosis satisfying specific criteria, even when continuously dependent on tracheostomy mechanical ventilation, can be decannulated and placed on continuous noninvasive ventilatory support with mechanical insufflation-exsufflation.

The Intermittent Abdominal Pressure Ventilator as an Alternative Modality of Noninvasive Ventilatory Support: A Narrative Review.

ABSTRACT: Noninvasive ventilation has become the initial treatment for symptomatic ventilatory pump failure but, when used at ventilatory support settings, can be an alternative to tracheostomy mechanical ventilation. The intermittent abdominal pressure ventilator, a corset with an internal air sack inflated by a ventilator, allows to increase tidal volumes through the raising of the diaphragm and its consequent passive descent. It has been used for daytime support for more than 70 yrs, but its knowledge among clinicians is scarce. A narrative review was performed by searching PubMed, Medline, and the Cochrane Database of Systematic Reviews using the terms "IAPV" or "pneumobelt." One hundred forty patients were cited using the intermittent abdominal pressure ventilator from 1946 until it went off the market in the 1970s, although many continued to use it. There was only one publication on its use from 2003 to 2017, but three publications from 2017 through 2021. It has been used for full diurnal ventilatory support by some patients for more than 50 yrs and has even been used throughout the labor of a mother with no ventilator-free breathing ability. The intermittent abdominal pressure ventilator is a ventilatory support alternative for patients with ventilatory pump failure. It can be effective and well tolerated and maintain quality of life without facial interfaces. More widespread application is warranted.

Mechanical Insufflation Exsufflation, Syringomyelia, and Headache.

ABSTRACT: Mechanical insufflation exsufflation creates cough flows to clear central airways secretions for patients with ineffective cough flows. At times, patients with even potentially effective spontaneous cough flows can have pain that prevents effective coughing. We describe a patient with Arnold-Chiari syndrome, syringomyelia, and hydrocephalus who had nine episodes of pneumonia through the age of 9 yrs, and cough associated headaches, who upon using mechanical insufflation exsufflation had no subsequent pneumonias for at least the next 17 yrs and no headaches when using it for coughing.

Decanulación en Paciente Pediátrica con Enfermedad Neuromuscular: reporte de caso

RESUMEN La enfermedad de multiminicores es un trastorno neuromuscular hereditario caracterizado por la presencia de múltiples 'cores' en biopsia muscular y características clínicas de una miopatía congénita. El presente caso trata de una paciente de 10 años de edad, con diagnóstico de enfermedad neuromuscular multiminicores, traqueostomizada desde los 7 años de edad por destete fallido y debilidad muscular. La paciente fue derivada al Departamento de Rehabilitación Cardio-respiratoria del hospital de Clínicas de la Facultad de Ciencias Médicas de la Universidad Nacional de Asunción, presentando en su primera evaluación dependencia crónica de oxígeno (por más de 12 meses), tos débil y no funcional con flujo pico tosido e inferior a 160 L/m, insuficiencia respiratoria crónica e hipercapnia (53 mmHg de CO2ET). Durante su seguimiento en nuestro departamento fueron seguidas las pautas de Rehabilitación Respiratoria contempladas en el Proyecto de evaluación, tratamiento y seguimiento de pacientes con Enfermedades Neuromusculares, y aprobado por el Consejo Superior de la Facultad de Ciencias Médicas de la Universidad Nacional de Asunción, dichas pautas están fundamentadas en los estudios y publicaciones científicas del Dr. John Bach (Rudgers University, Newart, Nueva Jersey-EEUU) y su equipo colaborador del Grupo Iberoamericano de Cuidados Respiratorios en Enfermedades Neuromusculares. Como resultado final del tratamiento y seguimiento aplicado por más de un año la paciente fue decanulada exitosamente, en un consultorio para pacientes ambulatorios, sin descompensaciones hemodinámicas, con una excelente tolerancia y sin requerimiento de internaciones hospitalarias.

ABSTRACT Multiminicores disease is a hereditary neuromuscular disorder characterized by the presence of multiple 'nuclei' on muscle biopsy and clinical features of a congenital myopathy. The present case concerns a 10-year-old patient, diagnosed with multiminicores disease, tracheostomized since she was seven due to failed weaning and muscle weakness. The patient was referred to the Department of Cardio-respiratory Rehabilitation of the Clínicas Hospital from the National University of Asunción, presenting in her first evaluation chronic oxygen dependence (for more than 12 months), weak and non-functional cough with cough peak flow less than 160 L / m, chronic respiratory failure and hypercapnia (52 mmHg CO2ET). We did the follow-up in order to the Respiratory Rehabilitation guidelines contemplated in the Project for the evaluation, treatment and follow-up of patients with Neuromuscular Diseases, which was approved by the Superior Council of the Medical Sciences School from the National University of Asunción, these guidelines are based on scientific studies and publications done by Dr. John Bach (Rudgers University, Newart, New Jersey-USA) and his collaborating team from the Ibero-American Group for Respiratory Care in Neuromuscular Diseases. As a result, the patient was successfully decannulated, in an outpatient clinic, without hemodynamic decompensations, with excellent tolerance and without the requirement of hospital admissions.

Recomendaciones para el manejo respiratorio de los pacientes con atrofia muscular espinal / Recommendations for respiratory management of patients with spinal muscular atrophia / Recomendações para a manipulação respiratória de pacientes com atrofia muscular espinhal

Resumen: La atrofia muscular espinal (AME) es una enfermedad del asta anterior de la médula espinal, genéticamente determinada y causada por síntesis insuficiente de la proteína de supervivencia de la motoneurona. La debilidad muscular lleva a una disminución progresiva de la capacidad vital y de flujos medibles durante la tos. La intensidad y precocidad de la expresión motora se vincula con los grados de afectación de los grupos musculares respiratorios, determinando la meseta en la capacidad vital y progresión a la insuficiencia ventilatoria, como también el compromiso de los músculos inervados bulbares. Las formas clínicas más severas de AME, en especial aquellas con presentaciones más tempranas y respiración paradojal, tienen capacidades vitales y flujos pico tosidos menores. La evaluación secuencial de estos parámetros es esencial para el pronóstico funcional y vital de estos pacientes. La subclasificación de AME tipo 1 y 2 se relaciona con momentos deseables para la realización de cuidados respiratorios no invasivos en la infancia temprana y en la edad escolar, que mejoran la sobrevida y calidad de vida. Este documento sintetiza dichas recomendaciones con especial referencia a intervenciones guiadas por etapas que incluyan apilamiento de aire (air stacking), protocolos de tos asistida y soporte ventilatorio no invasivo con alta intensidad de presiones de soporte, incluso en aquellos pacientes con pérdida de la autonomía respiratoria, minimizando el riesgo de traqueotomía. La no consideración de estas recomendaciones en la valoración regular de los pacientes resta la oferta de tratamientos oportunos.

Summary: Spinal Muscular Atrophy (SMA) is a disease of the anterior horn of the spinal cord, genetically determined, and caused by deficiency of survival motor neuron (SMN) protein. Muscle weakness leads to a progressive decrease in vital capacity and to diminished cough flows. Respiratory morbidity and mortality are a function of respiratory and bulbar-innervated muscle impairment. It can be measured by the sequential evaluation of vital capacity to determine the life time maximum (plateau) and its subsequent rate of decline, progressing to ventilatory failure. Bulbar-innervated muscle impairment can also be monitored and measured by spirometry. The more severe clinical forms of SMA, especially those with earlier onsets and paradoxical breathing, have lower vital capacities and cough peak flows. The sequential assessment of these parameters is key for the vital and functional prognosis of these patients. SMA sub-classification types 1 and 2 of SMA involve appropriate times for non-invasive respiratory interventions in early childhood and school age and improve afterlife and quality of life. This document summarizes these recommendations, as a function of SMA type, with special reference to interventions that include air stacking, manually and mechanically assisted coughing protocols and noninvasive ventilatory support techniques, even for patients who have no ventilator-free breathing ability to minimize or eliminate the need to resort to tracheotomy. Failure to properly evaluate these patients regularly reduces their survival and chances to avoid invasive airway tubes.

Resumo: A Atrofia Muscular Espinhal (SMA) é uma doença do corno anterior da medula espinhal, geneticamente determinada e causada pela síntese insuficiente da proteína de sobrevivência dos neurônios motores. A fraqueza muscular leva a uma diminuição progressiva da capacidade vital e fluxos mensuráveis durante a tosse. A intensidade e a precocidade da expressão motora estão relacionadas aos graus de envolvimento dos grupos musculares respiratórios, determinando o platô da capacidade vital e a progressão para insuficiência ventilatória, bem como o envolvimento dos músculos inervados do bulbar. As formas clínicas mais graves de SMA, especialmente aquelas com apresentações anteriores e respiração paradoxal, têm capacidades vitais mais baixas e fluxos de tosse mais baixos. A avaliação sequencial desses parâmetros é essencial para o prognóstico funcional e vital desses pacientes. A subclassificação de SMA tipo 1 e 2 está relacionada aos momentos desejáveis para cuidados respiratórios não invasivos na primeira infância e idade escolar, que melhoram a sobrevida e a qualidade de vida. Este documento resume essas recomendações com referência especial às intervenções guiadas por etapas que incluem empilhamento de ar, protocolos de tosse assistida e suporte ventilatório não invasivo com suporte pressórico de alta intensidade, mesmo em pacientes com perda de autonomia respiratória, minimizando o risco de traqueostomia. A não consideração dessas recomendações na avaliação regular dos pacientes reduz a oferta de tratamentos oportunos.