Difficulty Weaning Research Articles

Comprehensive approach to weaning in difficult-to-wean infantile and juvenile-onset glycogen-storage disease type II patients: a case series

BackgroundGlycogen storage disease type II (GSD II) is caused by acid alpha-glucosidase (GAA) deficiency. Both infantile-onset and juvenile-onset GSD II lead to proximal muscle weakness and respiratory insufficiency and require mechanical ventilation. However, GSD II is also independently associated with delayed weaning from mechanical ventilation. This study aimed to describe a comprehensive approach including sequential invasive-noninvasive mechanical ventilation weaning and enzyme replacement therapy (ERT) in patients with weaning difficulties.Case presentationWe studied six difficult-to-wean GSD II (three juvenile-onset, three infantile-onset) patients at the First Affiliated Hospital, Sun Yat-sen University from October 2015 to December 2017. Difficulty in weaning was defined as follows: the need for more than three spontaneous breathing trials or more than 1 week to achieve successful weaning. All patients received comprehensive treatment including sequential invasive-noninvasive mechanical ventilation weaning, ERT and general treatment. Recombinant human acid alpha-glucosidase enzyme therapy (20 mg/kg every 14 days) was used after diagnosis, and Patients 1–6 received ERT for 15.5, 4.5, 2, 2.5, 17, and 2 months, respectively. The therapeutic effect of the comprehensive treatment was observed.The patients’ respiratory function and limb muscle strength improved after each ERT session. Patients who successfully completed a spontaneous breathing trial could proceed to extubation, and then start non-invasive ventilation. The patients’ age range at initial mechanical ventilation was 3–47 (median 26.5) months, duration of invasive ventilation was 1–36 (median 2.75) months, and duration of noninvasive ventilation was 0–0.6 (median 0.05) month. The patients’ nutritional status improved after enhanced nutritional support. Patients 2, 3, and 5 were successfully weaned off the ventilator. Patient 1 underwent tracheal intubation after six weaning failures, and Patients 4 and 6 died after therapy was abandoned by their parents.Discussion and conclusionsMale sex, GSD II type, and the presence of malnutrition and neurological impairment may predict poor respiratory outcomes. The above-described comprehensive sequential invasive-noninvasive mechanical ventilation weaning strategy may increase the success rate of weaning from mechanical ventilation.

Norwood Procedure—Difficulty in Weaning From Cardiopulmonary Bypass and Implications for Outcomes

Difficulty weaning from cardiopulmonary bypass (CPB) or the need to return to CPB (collectively D-CPB) may occur after the Norwood procedure. We sought to evaluate the relationship between D-CBP and survival. This was a retrospective chart review of all patients undergoing a Norwood procedure at our institution during the interval 2005-2017. Primary outcome was survival for the Norwood procedure. Secondary outcomes included various measures of morbidity. Successful wean from CBP (S-CPB) was defined as no need to return to full-flow CPB during the initial definitive wean or after separation from CPB; otherwise, the classification was difficulty with wean (D-CBP). Successful rescue in the D-CPB group was defined as not requiring extracorporeal life support either in the operating room or within the first 3 postoperative days. Of the 196 patients in the cohort, 49 were D-CPB. Survival for S-CPB was 92.5% (136/147) vs 71.4% (35/49) for D-CPB (P = 0.001). Major morbidity occurred in 29.9% (44/147) in S-CPB vs 69.4% (34/49) in D-CPB (P < 0.001). With multivariable analysis, D-CPB was significantly associated with mortality (odds ratio = 8.09; confidence interval 2.72-24.05; P < 0.001). Successful rescue occurred in 30 of 49 patients in the D-CPB group and demonstrated survival similar to the S-CPB group. In the Norwood patient, D-CPB is an important intraoperative event and prognostic factor for mortality and morbidity. Successful rescue appears to ameliorate the impact of D-CPB on survival.

Entrenamiento muscular respiratorio en un paciente neurocrítico con ventilacion mecanica prolongada

La ventilación mecánica se instaura con frecuencia en los pacientes que ingresan a unidades de cuidados intensivos. A pesar de que su uso en la mayoría de los casos es transitorio, del 32% al 41% de los pacientes neurocríticos la requieren por periodos prologandos debido a diversas condiciones que dificultan el destete. En circunstancias en las que la causa de su prolongación es la debilidad muscular, tiene importancia el entrenamiento muscular inspiratorio, el cual puede contribuir al incremento de la fuerza muscular y facilitar el destete. El objetivo del presente reporte es describir el caso de un paciente de 65 años con hemorragia subaracnoidea Fisher III, ventilación mecánica prolongada, y dificultad para el destete en quien se implementó una estrategia de entrenamiento muscular respiratorio.

Diaphragmatic paralysis in young children: A literature review.

Diaphragmatic paralysis (DP) is a rare cause of respiratory distress in young children. In the first years of life, the main cause is phrenic nerve injury after cardiothoracic surgery or obstetrical trauma. DP usually presents as respiratory distress. Asymmetrical thorax elevation, difficulty weaning from mechanical ventilation, pulmonary atelectasis, and repeated pulmonary infections are other suggestive signs or complications. DP is usually suspected on chest X-ray showing abnormal hemidiaphragm elevation. Although fluoroscopy was considered the gold standard for DP confirmation, it has gradually been replaced by ultrasound, which can be done at the bedside. Some electrophysiological tools may be useful for a better characterization of phrenic nerve injury and chance of recovery. The management of DP is mainly based on clinical severity. In mild asymptomatic cases, DP may only require close monitoring. In more severe cases, adequate ventilatory support and/or surgical diaphragmatic plication may be needed. Electrophysiological tools may help clinicians assess the ideal timing for diaphragmatic plication.

Impaired diaphragm resistance vessel vasodilation with prolonged mechanical ventilation

Mechanical ventilation (MV) is a life-saving intervention, yet with prolonged MV (i.e., ≥6 h) there are time-dependent reductions in diaphragm blood flow and an impaired hyperemic response of unknown origin. Female Sprague-Dawley rats (4-8 mo, n = 118) were randomized into two groups; spontaneous breathing (SB) and 6-h (prolonged) MV. After MV or SB, vasodilation (flow-induced, endothelium-dependent and -independent agonists) and constriction (myogenic and α-adrenergic) responses were measured in first-order (1A) diaphragm resistance arterioles in vitro, and endothelial nitric oxide synthase (eNOS) mRNA expression was quantified. Following prolonged MV, there was a significant reduction in diaphragm arteriolar flow-induced (SB, 34.7 ± 3.8% vs. MV, 22.6 ± 2.0%; P ≤ 0.05), endothelium-dependent (via acetylcholine; SB, 64.3 ± 2.1% vs. MV, 36.4 ± 2.3%; P ≤ 0.05) and -independent (via sodium nitroprusside; SB, 65.0 ± 3.1% vs. MV, 46.0 ± 4.6%; P ≤ 0.05) vasodilation. Compared with SB, there was reduced eNOS mRNA expression (P ≤ 0.05). Prolonged MV diminished phenylephrine-induced vasoconstriction (SB, 37.3 ± 6.7% vs. MV, 19.0 ± 1.9%; P ≤ 0.05) but did not alter myogenic or passive pressure responses. The severe reductions in diaphragmatic blood flow at rest and during contractions, with prolonged MV, are associated with diaphragm vascular dysfunction which occurs through both endothelium-dependent and endothelium-independent mechanisms.NEW & NOTEWORTHY Following prolonged mechanical ventilation, vascular alterations occur through both endothelium-dependent and -independent pathways. This is the first study, to our knowledge, demonstrating that diaphragm arteriolar dysfunction occurs consequent to prolonged mechanical ventilation and likely contributes to the severe reductions in diaphragmatic blood flow and weaning difficulties.

Risk Factors of Ventilator-Associated Pneumonia in Critically III Patients.

Ventilator-associated pneumonia (VAP), a hospital acquired pneumonia that occurs more than 48 h after mechanical ventilation, is a common complication of mechanical ventilation with a high mortality rate. VAP can cause patients to have difficulty weaning off the ventilator and to stay in the hospital longer, which results in a huge financial burden to patients and a huge demand for medical resources. Several strategies, such as drugs including chlorhexidine, β-lactam antibiotics and probiotics, have been used to prevent VAP in clinic. The incidence and the mortality rate of VAP have been decreased with the development of preventative strategies in the past decades, but VAP remains one of the most common causes of nosocomial infections and death in the intensive care unit. Current challenges in the management of VAP involved the lack of a gold standard for diagnosis, the absence of effective preventative strategies, and the rise in antibiotic resistance. Therefore, in order to reduce the incidence of VAP and improve the outcome of patients with mechanical ventilation, it is necessary to clarify the risk factors of VAP for clinical prevention and control of VAP. This paper reviews the international risk factors of VAP occurrence reported in recent years, including patient characteristics, increased mechanical ventilation time and prolonged length of hospital stay, disorders of consciousness, burns, comorbidities, prior antibiotic therapy, invasive operations, gene polymorphisms, and mentions the corresponding preventive measures. Each factor is not only an independent risk factor of VAP, but also has an influence on each other. A better understanding of risk factors for VAP is helpful for predicting the occurrence of VAP, improving the prevention and control of VAP, and reducing the morbidity and mortality rates of patients with VAP.

Inability of Diaphragm Ultrasound to Predict Extubation Failure: A Multicenter Study

Diaphragmatic dysfunction may promote weaning difficulties in patients who are mechanically ventilated. The goal of this study was to assess whether diaphragm dysfunction detected by ultrasound prior to extubation could predict extubation failure in the ICU. This multicenter prospective study included patients at high risk of reintubation: those aged > 65 years, with underlying cardiac or respiratory disease, or intubated > 7days. All patients had successfully undergone a spontaneous breathing trial. Diaphragmatic function was assessed by ultrasound prior to extubation while breathing spontaneously on a T-piece. Bilateral diaphragmatic excursion and apposition thickening fraction were measured, and diaphragmatic dysfunction was defined as excursion< 10mm or thickening< 30%. Cough strength was clinically assessed by physiotherapists. Extubation failure was defined as reintubation or death within the 7days following extubation. Over a 20-month period, 191 at-risk patients were studied. Among them, 33 (17%) were considered extubation failures. The proportion of patients with diaphragmatic dysfunction was similar between those whose extubation succeeded and those whose extubation failed: 46%vs51%using excursion (P= .55), and 71%vs68%using thickening (P= .73), respectively. Values of excursion and thickening did not differ between the success and the failure groups: at right, excursion was 14 ± 7mm vs11 ± 8 (P= .13), and thickening was 29 ± 29%vs38 ± 48%(P= .83), respectively. Extubation failure rates were 7%, 22%, and 46%in patients with effective, moderate, and ineffective cough (P< .01). Ineffective cough was the only variable independently associated with extubation failure. Diaphragmatic dysfunction assessed by ultrasound was not associated with an increased risk of extubation failure.

Right Ventricular Failure Following Acute Type A Aortic Dissection: An Alternative Strategy

Coronary malperfusion is a complication of aortic dissection, and can lead to ventricular failure. We report 2 cases of right ventricular (RV) failure, following aortic dissection, successfully bridged to recovery using right ventricular assist devices (RVADs). The first was a 54 year-old man, who had difficulty weaning from bypass due to a stunned RV following aortic root replacement. The second was a 34 year-old woman, who after replacement of the ascending aorta, suffered episodes of ventricular fibrillation (VF), and RV failure. Both had an RVAD implanted, allowing eventual recovery. We recommend more widespread use of VADs in such cases.

Diaphragmatic parameters by ultrasonography for predicting weaning outcomes

BackgroundDiaphragmatic dysfunction remains the main cause of weaning difficulty or failure. Ultrasonographic measurement of diaphragmatic function can be used to predict the outcomes of weaning from mechanical ventilation. Our primary objective was to investigate the performance of various sonographic parameters of diaphragmatic function for predicting the success of weaning from mechanical ventilation.MethodsWe prospectively enrolled 68 adult patients requiring mechanical ventilation who were admitted to the intensive care unit from June 2013 to November 2013. The diaphragmatic inspiratory excursion, time to peak inspiratory amplitude of the diaphragm (TPIAdia), diaphragmatic thickness (DT), DT difference (DTD), and diaphragm thickening fraction (TFdi) were determined by bedside ultrasonography performed at the end of a spontaneous breathing trial. A receiver operating characteristic curve was used for analysis.ResultsIn total, 62 patients were analyzed. The mean TPIAdia was significantly higher in the weaning success group (right, 1.27 ± 0.38 s; left, 1.14 ± 0.37 s) than in the weaning failure group (right, 0.97 ± 0.43 s; left, 0.85 ± 0.39 s) (P < 0.05). The sensitivity, specificity, positive predictive value, and negative predictive value of a TPIAdia of > 0.8 s in predicting weaning success were 92, 46, 89, and 56%, respectively. The diaphragmatic inspiratory excursion, DTD, and TFdi were associated with reintubation within 48 h. The P values were 0.047, 0.021, and 0.028, and the areas under the receiver operating characteristic curve were 0.716, 0.805, and 0.784, respectively.ConclusionAmong diaphragmatic parameters, TPIAdia exhibits good performance in predicting the success of weaning from mechanical ventilation. This study demonstrated a trend toward successful use of TPIAdia rather than diaphragmatic inspiratory excursion as a predictor of weaning from mechanical ventilation.

Difficulty in weaning and cardiopulmonary rehabilitation in inoperable severe mitral regurgitation: a case report

Objective: Severe mitral valve insufficiency may lead to heart failure and associated hemodynamic repercussions, such as congestion and weaning-induced pulmonary oedema. This slows weaning from mechanical ventilation, increases the length of the hospital stay, and worsens the patient’s prognosis. Case report: The present report describes one strategy used for cardiopulmonary rehabilitation and to help wean a patient from mechanical ventilation, with prohibitive comorbidities that contra-indicate surgical mitral valve repair, using respiratory and peripheral muscle training associated with positive pressure ventilation. Discussion: The severe dysfunction of the mitral valve was considered to be surgically inoperable by the surgeon team. However, the weaning and rehabilitation challenges in this patient with severe chronic obstructive pulmonary disease, even with weaning-induced pulmonary edema occurrence, were overcome. The strategy to rehabilitate respiratory muscle force during positive pressure ventilation, concomitantly to a cardiopulmonary rehabilitation program, improved peripheral muscle strength and functionality, reducing energetic demand, allowing the weaning from mechanical ventilation. Conclusion: Despite the severity of the disease, with the adopted strategy it was possible to perform weaning of the mechanical ventilation, allowing the patient to survive with his relatives.

Vitamin A Protects the Preterm Lamb Diaphragm Against Adverse Effects of Mechanical Ventilation.

Background: Preterm infants are deficient in vitamin A, which is essential for growth and development of the diaphragm. Preterm infants often require mechanical ventilation (MV) for respiratory distress. In adults, MV is associated with the development of ventilation-induced diaphragm dysfunction and difficulty weaning from the ventilator. We assessed the impact of MV on the preterm diaphragm and the protective effect of vitamin A during MV.Methods: Preterm lambs delivered operatively at ∼131 days gestation (full gestation: 150 days) received respiratory support by synchronized intermittent mandatory ventilation for 3 days. Lambs in the treated group received daily (24 h) enteral doses of 2500 IU/kg/day vitamin A combined with 250 IU/kg/day retinoic acid (VARA) during MV, while MV control lambs received saline. Unventilated fetal reference lambs were euthanized at birth, without being allowed to breathe. The fetal diaphragm was collected to quantify mRNA levels of myosin heavy chain (MHC) isoforms, atrophy genes, antioxidant genes, and pro-inflammatory genes; to determine ubiquitin proteasome pathway activity; to measure the abundance of protein carbonyl, and to investigate metabolic signaling.Results: Postnatal MV significantly decreased expression level of the neonatal MHC gene but increased expression level of MHC IIx mRNA level (p < 0.05). Proteasome activity increased after 3 days MV, accompanied by increased MuRF1 mRNA level and accumulated protein carbonyl abundance. VARA supplementation decreased proteasome activity and FOXO1 signaling, down-regulated MuRF1 expression, and reduced reactive oxidant production.Conclusion: These findings suggest that 3 days of MV results in abnormal myofibrillar composition, activation of the proteolytic pathway, and oxidative injury of diaphragms in mechanically ventilated preterm lambs. Daily enteral VARA protects the preterm diaphragm from these adverse effects.

Can inspiratory muscle training improve weaning outcomes in difficult to wean patients? A protocol for a randomised controlled trial (IMweanT study)

IntroductionRespiratory muscle dysfunction has been associated with failure to wean from mechanical ventilation. It has therefore been hypothesised that these patients might benefit from inspiratory muscle training (IMT). Evidence, however,...

Evaluation of Ventilator-induced Diaphragmatic Dysfunction by Diaphragmatic Excursion During Spontaneous Breathing Trials

Introduction: Ventilator-induced diaphragmatic dysfunction (VIDD) leads to difficulties in weaning. Diaphragmatic excursion assessment by ultrasonography is a feasible bedside assessment of the diaphragm in the ICU. Our primary aim was to identify the presence of VIDD using US in patients undergoing Spontaneous breathing trials (SBT). Our secondary aim was to assess the impact of VIDD impact on weaning outcome. Methods: This study was conducted in the Critical Care Department of Cairo University Hospital between March 2014 and March 2015. All consecutive subjects who required MV for ≥ 72 h and were ready for SBT were prospectively recruited. Exclusion criteria: Any history of aminoglycoside use, paralytics, central or neuromuscular disease, chemotherapy, cachexia, severe electrolyte imbalance or intra-abdominal pressure (IAP)>7 mmHg Thirty minutes from the start of SBT, each hemi-diaphragm was evaluated by M-mode sonography with the patient in the supine position. Five measurements were recorded and averaged. Ventilator Induced diaphragmatic dysfunction (VIDD) was diagnosed if diaphragmatic excursion (DE) was 0.05). Successful weaning was present in [18/26 (69%) vs. 13/24 (54.2%), p=0.06] and weaning time was shorter [29 ± 18 vs. 43 ± 28 h, p=0.02] in the NDD group versus the VIDD group respectively. The median DE was higher in successfully weaned vs. failed weaning subjects [14.4 (1.9-40) vs. 9.2 (6.6-35.1), p=0.01]. The receiver operator characteristic curves (ROC) showed a cut-off for weaning DE 14 mm for right hemi-diaphragm with an area under the curve (AUC) 0.8. Conclusions: VIDD is present in nearly half of our mechanically ventilated patients ≥ 72 h. VIDD is associated with lower DE and longer weaning time. Diaphragmatic excursion may serve as a valuable tool for predicting weaning outcome as traditional volumetric respiratory indices.

Comparison of Lung Ultrasound-based Weaning Indices with Rapid Shallow Breathing Index: Are They Helpful?

Background and Aims:The diaphragm is considered the main respiratory muscle and difficulty in weaning can occur because of impaired diaphragmatic function. Hence, monitoring diaphragmatic function is important. The aim of this study is to assess the ability of various lung ultrasound (US) indices and the rapid shallow breathing index (RSBI) to predict the outcome of the weaning process and compare them with RSBI.Materials and Methods:This was a prospective study conducted on patients admitted to critical care unit at a tertiary care hospital in north India from February 2017 to June 2017. Patients were put to spontaneous breathing trial (SBT) when they met the weaning criteria. Initial US was done immediately after putting the patient on SBT to check anatomy of the diaphragm and rule out patients according to exclusion criteria. This was followed by complete lung US (LUS) after 20 min of SBT.Results:The RSBI performed better than all other parameters, with an area under the curve (AUC) of 0.996. The sensitivity and specificity is 100%. Only comparable to RSBI is the speed of diaphragmic contraction (DC) which has AUC of 0.93. All other parameters had an AUC <0.8. Moreover, the DC and LUS score are strongly positively correlated with RSBI, whereas diaphragmic excursion and diaphragmic thickness fraction (DTF %) are weakly correlated.Conclusion:In Intensive Care Unit, RSBI is the best clinical tool for weaning, and DC is found to be the best parameter for weaning among the US-based weaning parameters. It can even be a substitute for RSBI, in today's world of real-time monitoring methods.

The effects of one-lung ventilation mode on lung function in elderly patients undergoing esophageal cancer surgery.

The objective of the present study was to explore the effects of different one-lung ventilation (OLV) modes on lung function in elderly patients undergoing esophageal cancer surgery. A total of 180 consecutive elderly patients (ASA Grades I–II, with OLV indications) undergoing elective surgery were recruited in the study. Patients were randomly divided into 4 groups (n = 45). In Group A, patients received low tidal volume (VT < 8 mL/kg) + pressure controlled ventilation (PCV), low tidal volume (VT < 8 mL/kg) + volume-controlled ventilation (VCV) in Group B, high tidal volume (VT ≥ 8 mL/kg) + PCV in Group C and high tidal volume (VT ≥ 8 mL/kg) + VCV in Group D. Two-lung ventilation involved routine tidal volume (8–10 mL/kg) at a frequency of 12 to 18 times/min, and VCV mode. Clinical efficacy among 4 groups was compared. The partial pressure of end-tidal carbon dioxide (PetCO2) did not significantly differ among 4 groups (all P > .05), and the oxygenation index and SO2 in Group A were significantly higher than in the other groups (P < .05). The PetCO2, peak airway pressure (Ppeak), platform airway pressure (Pplat), and mean airway pressure (Pmean) in Group A were significantly lower than those in the other groups (all P < .05). However, airway resistance (Raw) among 4 groups did not significantly differ (all P > .05). The incidence of pulmonary infection, anastomotic fistula, ventilator-induced lung injury, lung dysfunction, difficulty weaning from mechanical ventilation, and multiple organ dysfunction in Groups A and B were lower than that in Groups C and D (all P < .05). The expression levels of IL-6, tumor necrosis factor-α, and C-reactive protein in lavage fluid in Group A were significantly lower than those in the other groups (all P < .05). OLV with low tidal volume (VT < 8 mL/kg) + PCV (5 cmH2O PEEP) improved lung function and mitigated inflammatory responses in elderly patients undergoing esophageal cancer surgery.

Mechanical ventilation. Part II: Basic principles and function of ventilators.

Mechanical ventilation is the process of supporting respiration by manual or mechanical means. When normal breathing is inefficient or has stopped, mechanical ventilation is life-saving and should be applied at once. The ventilator increases the patient's ventilation by inflating the lungs with oxygen or a mixture of air and oxygen. Ventilators play an important role in the anaesthetic management of patients, as well as in the treatment of patients in the ICU. However, there are differences between the anaesthetic ventilators and the ventilators in ICU. The main indication for mechanical ventilation is difficulty in ventilation and/or oxygenation of the patient because of any respiratory or other disease. The aims of mechanical ventilation are to supply adequate oxygen to patients with a limited vital capacity, to treat ventilatory failure, to reduce dyspnoea and to facilitate rest of fatigued breathing muscles. Depression of the central nervous system function is a pre-requirement for mechanical ventilation. Some times, opioids or muscle relaxants can be used in order to depress patient's breathing. Mechanical ventilation can be applied using many different modes: assisted ventilation, controlled ventilation, continuous positive pressure ventilation, intermittent positive pressure ventilation and jet ventilation. Furthermore, there are different types of automatic ventilators built to provide positive pressure ventilation in anaesthetized or heavily sedated or comatose patients: manual ventilators (Ambu-bag), volumecontrolled ventilators with pressure cycling, volume-controlled ventilators with time cycling and pressure-controlled ventilators. In veterinary practice, the ventilator should be portable, compact and easy to operate. The controls on most anaesthetic ventilators include settings for tidal volume, inspiratory time, inspiratory pressure, respiratory rate and inspiration: expiration (I:E) ratio. The initial settings should be between 10-20 ml/kg for tidal volume, 12-30 cmH2 0 for the inspiratory pressure and 8-15 breaths/min for the respiratory rate. Mechanical ventilation is a very important part of treatment in the ICU, but many problems may arise during application of mechanical ventilation in critically ill patients. All connections should be checked in advance and periodically for mechanical problems like leaks. Moreover, complications like lung injury, pneumonia, pneumothorax, myopathy and respiratory failure can occur during the course of mechanical ventilation causing difficulty in weaning.

Dificuldades na introdução de alimentos complementares ao aleitamento materno em bebês não disfágicos: efeitos da atuação fonoaudiológica

Introdução: No período de transição do aleitamento materno para alimento pastoso e/ou sólido, tal modificação pode não ser bem aceita pela mãe e/ou pelo bebê e ocasionar problemas de alimentação e/ou de linguagem subsequentes. Objetivo: descrever os efeitos da atuação fonoaudiológica diante das dificuldades na introdução de alimentos complementares ao aleitamento materno em bebês não disfágicos numa abordagem biopsíquica. Método: Estudo realizado em instituição de saúde filantrópica, de acordo com critérios éticos estabelecidos para pesquisas com seres humanos. Casuística: Cinco díades mãe/bebê, bebês de ambos os gêneros, na faixa etária entre 5 e 8 meses, com dificuldades no desmame e sem diagnóstico de disfagia orofaríngea neurogênica ou mecânica. A amostragem foi obtida por conveniência. Procedimento: Foram realizados quatro encontros, sendo três visitas domiciliares, nos quais foram aplicados três instrumentos de avaliação, adaptados a partir da literatura. Após a ação fonoaudiológica foi reaplicado o último, para efeitos de comparação pré e pós. Resultados: Após a atuação fonoaudiológica, houve eliminação de episódios de engasgos, as refeições se tornaram mais prazerosas para a díade mãe/bebê e os bebês passaram a aceitar melhor os alimentos. Os resultados revelaram diferença estatisticamente significativa entre os obtidos pré e pós atuação fonoaudiológica: média inicial era de 16,0 pontos (dp=1,0) e final 21,2 pontos (dp=0,8/ p= 0,001), (p=0,001). Conclusão: A atuação fonoaudiológica numa abordagem biopsíquica promoveu benefícios nos casos estudados.

Bedside Ultrasound in the Diagnosis and Treatment of Children with Respiratory Difficulty Following Cardiac Surgery.

Many children frequently fail extubation or require a prolonged period of positive pressure ventilation support after cardiac surgery. Pediatric cardiac Intensive Care Unit (PCICU) ultrasound (US) stands as a simple, basic bedside tool that can be performed by trained intensivists for the diagnosis with immediate implication on therapeutic decisions in multiple scenarios that physicians may face in PCICU. Bedside US is widely available, convenient, and inexpensive. This educational article based mainly on our own experience is highlighting the role of US in detecting the most common causes of respiratory weaning difficulties and extubation failure in postoperative cardiac children through proposed illustration and algorithm.

P.123 - Scoliosis is an inescapable comorbidity in SMA type II. A single center experience

Scoliosis is the most debilitating and unresolved problem in SMA type II patients. We present data of progression of scoliosis and treatment in a cohort of 20 SMA type II patients followed in the last fifteen years (2002–2017) in our Neuromuscular Unit. Medical records were collected prospectively. In all patients brace was prescribed since Cobb angle in sitting position was ≥20°or in case of marked kyphosis. All patients underwent systematical X-Ray, functional and pulmonary assessments. Seventeen of 20 patients (age range 2–20 y) have scoliotic curve. Mean age at scoliosis development was 3,4 y (range 2–7 y) and Cobb angle at diagnosis varied from 5° to 35°. Nine patients underwent scoliosis surgery. Six patients had Growing-rods or VEPTR at mean age of 8,1 y (range 6,2- 10y). Five patients (two of them received at first growing-rods) underwent definitive spine fusion at a mean age of 12.2 y (range 10-14y). Postoperative complications were observed in 3 patients: urinary infection, difficulty in extubation and weaning from mechanical ventilation and infective complication of the surgical wound. Despite the use of brace, scoliosis progressed in all cases and, as observed in our series, since the age of 6 years patients develop severe rotoscoliosis that, following the current standards of care, requires surgery. These findings have to be carefully considered in the developing of potentially effective therapy intrathecally administered.

Ventilator-Induced Diaphragmatic Dysfunction: Diagnosis and Role of Pharmacological Agents.

The use of controlled mechanical ventilation results in a major reduction of diaphragmatic contractile force together with atrophy of diaphragm muscle fibers, which is a condition known as ventilator-induced diaphragmatic dysfunction. Ventilator-induced diaphragmatic dysfunction is one of the major contributors to weaning difficulties and even increased mortality. This review summarizes the current data on the pathogenesis and diagnosis of ventilator-induced diaphragmatic dysfunction, and it outlines the use of ultrasonography for diaphragm evaluation. In addition, current pharmacologic agents used to mitigate ventilator-induced diaphragmatic dysfunction are described, with a particular emphasis on the therapeutic potential of theophylline in patients with ventilator-induced diaphragmatic dysfunction-associated weaning difficulties.