Bilateral diaphragmatic paralysis and Lyme neuroborreliosis. Ten years of follow-up.

Borrelia burgdorferi infection (Lyme disease) is one of the few identifiable causes of neuralgic amyotrophy. Bilateral diaphragmatic paralysis is considered rare in borreliosis, and the pattern of long-term recovery of diaphragm function is also uncertain. Transdiaphragmatic pressure is the gold standard for diagnosis of bilateral diaphragmatic paralysis, a study that has been reported on few occasions. We present a case of neuralgic amyotrophy associated with Borrelia infection and bilateral diaphragmatic paralysis that provides a detailed follow-up of the spirometric evolution, of the maximum static pressures in the mouth and of transdiaphragmatic pressure from the onset of symptoms and the long term. This case allows us to know one of the possible evolutionary profiles of diaphragmatic dysfunction in neuralgic amyotrophy due to borreliosis.

La infección por Borrelia burgdorferi (enfermedad de Lyme) es una de las pocas causas identificables de amiotrofia neurálgica. La parálisis diafragmática bilateral es considerada rara en la borreliosis y el patrón de recuperación a largo plazo de la función del diafragma también es incierto. La presión transdiafragmática es el patrón de oro para el diagnóstico de parálisis diafragmática bilateral, un estudio que ha sido informado en pocas ocasiones. Se presenta un caso de amiotrofia neurálgica asociado a infección por Borrelia y parálisis diafragmática bilateral, que aporta un seguimiento detallado de la evolución espirométrica, de las presiones estáticas máximas en la boca y de la presión transdiafragmática desde el inicio de los síntomas y a largo plazo. Este caso permite conocer uno de los posibles perfiles evolutivos de la disfunción diafragmática en la amiotrofia neurálgica por borreliosis.

Bilateral diaphragmatic paralysis and Lyme neuroborreliosis. Ten-years of follow-up / Parálisis diafragmática bilateral y neuroborreliosis de Lyme. Diez años de seguimiento

Abstract Borrelia burgdorferi infection (Lyme disease) is one of the few identifiable causes of neuralgic amyotrophy (AN). Bilateral diaphragmatic paralysis is considered rare in borreliosis, and the pattern of long-term recovery of diaphragm function is also uncertain. Transdiaphragmatic pressure is the gold standard for diagnosing bilateral diaphragmatic paralysis, a study that has been reported on a few occasions. We pres ent a case of AN associated with borrelia infection and bilateral diaphragmatic paralysis that provides a detailed follow-up of the spirometric evolution, the maximum static pressures in the mouth, and the transdiaphragmatic pressure from the onset of symptoms and in the long term. This case allows us to know one of the possible evolutionary profiles of diaphragmatic dysfunction in AN due to borreliosis.

Resumen La infección por Borrelia burgdorferi (enfermedad de Lyme) es una de las pocas causas identificables de amiotrofia neurálgica. La parálisis diafragmática bilateral es considerada rara en la borreliosis y el patrón de recuperación a largo plazo de la función del diafragma también es incierto. La presión transdiafragmática es el patrón de oro para el diagnóstico de parálisis diafragmática bilateral, un estudio que ha sido informado en pocas ocasiones. Se presenta un caso de amiotrofia neurálgica asociado a infección por Borrelia y parálisis diafrag mática bilateral, que aporta un seguimiento detallado de la evolución espirométrica, de las presiones estáticas máximas en la boca y de la presión transdiafragmática desde el inicio de los síntomas y a largo plazo. Este caso permite conocer uno de los posibles perfiles evolutivos de la disfunción diafragmática en la amiotrofia neurálgica por borreliosis.

Diaphragm Ultrasound in Weaning From Mechanical Ventilation.

A 65-year-old man was admitted to the ICU for septic shock due to pneumonia. He remained on mechanical ventilation for 96 hours. His shock resolved, and he no longer required IV vasopressor therapy. His vital signs included a BP of 105/70 mm Hg, heart rate 85 beats/min, respiratory rate 22 breaths/min, and oxygen saturation 95%. His ventilator settings were volume control/assist control with a positive end-expiratory pressure of 5 and an Fio2 set to 40%. On these setting his blood gas showed an Pao2 of 75 mm Hg. He was following simple commands and had minimal tracheobronchial secretions. He was placed on a spontaneous breathing trial with a spontaneous mode of ventilation and pressure support of 7/5. He remained hemodynamically stable and showed no distress through the procedure, so he was extubated to 6 L oxygen by nasal cannula. Eighteen hours later, the patient was found to have increased work of breathing, with use of accessory respiratory muscles. A blood gas showed an elevated level of CO2, so the patient was reintubated. After intubation, the patient again appeared comfortable on minimal ventilator settings. Chest radiography before reintubation showed no new parenchymal process, but an elevated left diaphragm. After a thorough workup, it was determined that diaphragmatic weakness was the most likely reason for respiratory failure. The team questioned whether there was a way to have detected this before extubation.

Clinical and laboratory diagnosis of congenital Zika virus syndrome and diaphragmatic unilateral palsy: case report / Diagnostico clínico e laboratorial do Zika vírus congênito e paralisia diafragmática unilateral: o relato de um caso

Abstract Introduction: several birth defects associated to congenital Zika virus infection have been reported, although the clinical features have not been fully characterized. Description: this is the first case report on unilateral diaphragmatic paralysis diagnosed on a neonate with congenital Zika confirmed by the examination of the amniotic fluid through polymerase chain reaction (ZIKV RT-PCR) and the examination of cerebrospinal fluid by serological test (IgM ZIKV-ELISA) after birth. The main manifestations detected by intrauterine ultrasound were: microcephaly, ventriculomegaly, intracranial calcifications, enlarged cisterna magna, increased amniotic fluid index and fetal akinesia syndrome. The newborn had acute respiratory failure in the first hours of life, requiring mechanical ventila-tion. The X- ray of the chest showed unilateral diaphragmatic paralysis and cardiomegaly. Discussion: diaphragmatic palsy in congenital Zika has not been previously reported, the etiopathogenic mechanisms of this event in congenital Zika virus needs to be elucidated.

Resumo Introdução: apesar de vários defeitos de nascimento associados à infecção congênita pelo Zika vírus terem sido descritos, o quadro clínico ainda não foi completamente caracterizado. Descrição: este é o primeiro relato de caso de paralisia diafragmática unilateral em um neonato com diagnóstico confirmado de Zika congênita pelo exame do líquido amniótico utilizando a reação da polimerase em cadeia (ZIKV PCR-RT) e pelo exame sorológico do líquido cefaloraquidiano (ZIKV IgM-ELISA), após o nascimento. As principais manifestações detectadas pela ultrassonografia intraútero no período gestacional foram: microcefalia, ventriculomegalia, calcificações intracranianas, cisterna magna alargada, aumento do índice de liquido amniótico e síndrome da acinesia fetal. O recém-nascido apresentou falência respiratória aguda nas primarias horas de vida, necessitando de ventilação mecânica. A radiografia de tórax realizada mostrou paralisia diafragmática unilateral e cardiomegalia. Discussão: a paralisia diafragmática na Zika congênita não havia sido previamente relatada, havendo a necessidade de investigação dos mecanismos etiopatogênicos dessa manifestação na infecção congênita pelo Zika vírus.

Is polysomnographic examination necessary for subjects with diaphragm pathologies?

OBJECTIVES: While respiratory distress is accepted as the only indication for diaphragmatic plication surgery, sleep disorders have been underestimated. In this study, we aimed to detect the sleep disorders that accompany diaphragm pathologies. Specifically, the association of obstructive sleep apnea syndrome with diaphragm eventration and diaphragm paralysis was evaluated. METHODS: This study was performed in Süreyyapasa Chest Diseases and Thoracic Surgery Training and Research Hospital between 2014-2016. All patients had symptoms of obstructive sleep apnea (snoring and/or cessation of breath during sleep and/or daytime sleepiness) and underwent diaphragmatic plication via video-assisted mini-thoracotomy. Additionally, all patients underwent pre- and postoperative full-night polysomnography. Pre- and postoperative clinical findings, polysomnography results, Epworth sleepiness scale scores and pulmonary function test results were compared. RESULTS: Twelve patients (7 males) with a mean age of 48 (range, 27-60) years and a mean body mass index of 25 (range, 20-30) kg/m2 were included in the study. Preoperative polysomnography showed obstructive sleep apnea syndrome in 9 of the 12 patients (75%), while 3 of the patients (25%) were regarded as normal. Postoperatively, patient complaints, apnea hypopnea indices, Epworth sleepiness scale scores and pulmonary function test results all demonstrated remarkable improvement. CONCLUSION: All patients suffering from diaphragm pathologies with symptoms should undergo polysomnography, and patients diagnosed with obstructive sleep apnea syndrome should be operated on. In this way, long-term comorbidities of sleep disorders may be prevented.

Is polysomnographic examination necessary for subjects with diaphragm pathologies?

OBJECTIVES: While respiratory distress is accepted as the only indication for diaphragmatic plication surgery, sleep disorders have been underestimated. In this study, we aimed to detect the sleep disorders that accompany diaphragm pathologies. Specifically, the association of obstructive sleep apnea syndrome with diaphragm eventration and diaphragm paralysis was evaluated. METHODS: This study was performed in Süreyyapasa Chest Diseases and Thoracic Surgery Training and Research Hospital between 2014-2016. All patients had symptoms of obstructive sleep apnea (snoring and/or cessation of breath during sleep and/or daytime sleepiness) and underwent diaphragmatic plication via video-assisted mini-thoracotomy. Additionally, all patients underwent pre- and postoperative full-night polysomnography. Pre- and postoperative clinical findings, polysomnography results, Epworth sleepiness scale scores and pulmonary function test results were compared. RESULTS: Twelve patients (7 males) with a mean age of 48 (range, 27-60) years and a mean body mass index of 25 (range, 20-30) kg/m2 were included in the study. Preoperative polysomnography showed obstructive sleep apnea syndrome in 9 of the 12 patients (75%), while 3 of the patients (25%) were regarded as normal. Postoperatively, patient complaints, apnea hypopnea indices, Epworth sleepiness scale scores and pulmonary function test results all demonstrated remarkable improvement. CONCLUSION: All patients suffering from diaphragm pathologies with symptoms should undergo polysomnography, and patients diagnosed with obstructive sleep apnea syndrome should be operated on. In this way, long-term comorbidities of sleep disorders may be prevented.

Evaluation of Respiratory Muscle Strength and Pulmonary Function in Patients with Charcot-Marie-Tooth Disease Type 2.

The aim of this study was to evaluate the pulmonary condition in a large family with Charcot-Marie-Tooth disease type 2 (CMT2). Eighteen participants diagnosed with CMT2 and 20 healthy individuals were evaluated by spirometry and maximal expiratory and maximal inspiratory pressures (MEP and MIP, respectively). Clinical disability was measured with CMT neuropathy score (CMTNS; range 0-36). One control group (CG) comprising 20 individuals, matched for age, sex and body mass index, were used for comparison. Eight patients were female (44.5%) and 10 patients were male (55.5%); mean age was 31.8 years (range 11-79) and CMTNS range was 6-26. Differences between CMT2 and CG in the spirometry and respiratory muscle strength were statistically significant for all dimensions. There were significant correlations between CMTNS and MIP (Pearson = -0.581) and MEP (Pearson = -0.5090). The results of this study show that patients with CMT, in spite of not showing clinical signs of advanced respiratory impairment, may present subclinical respiratory changes. The respiratory comprise in the CMT disease can be silent and insidious without presenting characteristic clinical signals.

Evaluación de los músculos respiratorios en la parálisis diafragmática bilateral / Assessment of respiratory muscle strength in bilateral diaphragmatic paralysis

Measurement of respiratory muscle strength is useful in order to detect respiratory muscle weakness and to quantify its severity. Apropos of a patient with bilateral diaphragmatic paralysis, we review the clinical manifestations and methods for assessing the strength of the respiratory muscles. In patients with severe respiratory muscle weakness, vital capacity and total lung capacity are reduced but are a non-specific and relatively insensitive measure. Conventionally, inspiratory and expiratory muscle strength has been assessed by maximal inspiratory and expiratory mouth pressures sustained for one second (PIMax and PEMax). The sniffmanoeuvre is natural and probably easier to perform. Sniff pressures are more reproducible and useful measure of diaphragmatic strength. However, the PIMax-PEMax and sniff manoeuvres are volition dependent, and submaximal efforts are most likely to occur in patients who are ill or breathless. Non-volitional tests include measurements of twitch esophageal, gastric and transdiaphragmatic pressure during bilateral electrical and magnetic phrenic nerve stimulation. Electrical phrenic nerve stimulation is technically difficult and is also uncomfortable and painful. Magnetic phrenic nerve stimulation is less painful and transdiaphragmatic pressure is reproducible in normal subjects. Systematic clinical evaluation and additional laboratory tests allow the diagnosis in most patients with respiratory muscle weakness.

La evaluación de la fuerza de los músculos respiratorios permite diagnosticar y cuantificar la gravedad de la debilidad muscular en diferentes enfermedades. A propósito de un paciente con parálisis diafragmática bilateral, hemos revisado el cuadro clínico y los procedimientos diagnósticos para evaluar la fuerza de los músculos respiratorios. En los pacientes con debilidad muscular respiratoria severa, disminuye la capacidad vital y la capacidad pulmonar total, pero es una medida inespecífica y relativamente insensible. Tradicionalmente, la fuerza muscular respiratoria es evaluada midiendo la presión inspiratoria y espiratoria máximas en la boca sostenidas durante un segundo (PIMax y PEMax). La medición de la presión inspiratoria máxima en la nariz (SNIP) es una maniobra natural, más simple de medir y más reproducible, siendo útil en la evaluación de la fuerza diafragmática. Sin embargo, estas técnicas no invasivas son operador dependiente, por lo tanto, esfuerzos submáximos es más probable que ocurran en pacientes graves o con disnea. Las mediciones de las presiones esofágica, gástrica y transdiafragmática mediante estimulación eléctrica o magnética del nervio frénico no son dependientes de la voluntad y son más confiables. Sin embargo, la estimulación eléctrica del nervio frénico es técnicamente difícil y puede ser incómoda y dolorosa. La estimulación magnética del nervio frénico es menos dolorosa y la medición de la presión transdiafragmática es reproducible en sujetos normales. La evaluación clínica sistemática y los exámenes de laboratorio complementarios permiten establecer el diagnóstico en la mayoría de los pacientes con debilidad de los músculos respiratorios.

Evaluación por imágenes del diafragma en el niño / Image evaluation of diaphragm in children

The anatomy, embriology andfunctions of the neonate 's diaphragm, as well as its anatomical and functional oddities were reviewed. And, in a deeper way, imaging studies were also reviewed; these have an important role in functional and anatomic evaluation of the diaphragm, each one with its own advantages and limitations. Chest X-rays allow an anatomic two dimensional evaluation of the diaphragm and constitutes the first approach in the study of diaphragm pathology in children; hence, the normal anatomy and the most common pathological signs are reviewed. Digestive tube studies using contrast media still are the best choice for diagnosis of hiatal hernia and of herniation through the foramen of Morgagni, where the colon is ascended. Ultrasound use is highlighted for the evaluation of diaphragmatic motility, as well as some of its advantages over fluoroscopy, which is and has been the method of choice in the diagnosis of diaphragmatic paralysis in children. Multiplanar images are the most complete method for the anatomic evaluation of the diaphragm, since they show its spatial orientation and allow the detailed evaluation of those pahologies where the anatomy is altered, such as diaphragmatic hernias, trauma and tumors.

Revisamos la embriología, anatomía y funciones del diafragma, sus particularidades anatómicas y funcionales en los neonatos y, en forma más profunda, los estudios por imágenes, que en la actualidad tienen un importante rol en su evaluación anatómica y funcional, cada uno de ellos con sus ventajas y limitaciones. La radiografía de tóraxpermite una evaluación anatómica en dos planos del diafragma y constituye la primera aproximación en el estudio de la patología del diafragma en los niños, por lo que se describe la anatomía normal y los signos de las patologías más frecuentes. Los estudios contrastados del tubo digestivo siguen siendo de elección para el estudio de las hernias hiatales y hernias de Morgagni en donde está ascendido el colon. Destacamos el uso del ultrasonido en la evaluación de la motilidad diafragmática, y algunas de sus ventajas sobre la fluoroscopia, que es y ha sido el método de elección en el diagnóstico de la parálisis diafragmática en los niños. Las imágenes multiplanares son un método más completo en la evaluación anatómica del diafragma, muestran mejor su orientación espacial y permiten la evaluación detallada de las patologías donde la anatomía está alterada, como hernias diafragmáticas congénitas, trauma y tumores.

Paresia diafragmática bilateral idiopática / Idiopathic bilateral diaphragmatic paresis

Relata-se o caso de um paciente com dispnéia intensa ao se deitar, em que foram excluídas doenças pulmonares, neuromusculares ou cardíacas, cuja investigação revelou paresia diafragmática bilateral. Um sinal chave para o diagnóstico foi a evidência de respiração paradoxal com o doente em decúbito supino. Havia piora da oxigenação e da capacidade vital forçada com a mudança da posição ortostática para supina. A fluoroscopia ortostática foi normal. A pressão inspiratória máxima estava muito reduzida. A estimulação elétrica transcutânea do diafragma foi normal, e a eletroestimulação do nervo frênico mostrou ausência de resposta, permitindo o diagnóstico de paresia bilateral do diafragma.

We report the case of a patient with severe dyspnea upon reclining. Lung disease, neuromuscular disorders and heart disease were ruled out. However, during the course of the investigation, bilateral diaphragmatic paresis was discovered. A key sign leading to the diagnosis was evidence of paradoxical respiration in the dorsal decubitus position. When the patient was moved from the orthostatic position to the dorsal decubitus position, oxygenation and forced vital capacity worsened. The orthostatic fluoroscopy was normal. Maximal inspiratory pressure was severely reduced. The responses to transcutaneous electric stimulation of the diaphragm were normal. However, electric stimulation of the phrenic nerve produced no response, leading to the diagnosis of bilateral diaphragmatic paresis.

Idiopathic bilateral diaphragmatic paresis.

We report the case of a patient with severe dyspnea upon reclining. Lung disease, neuromuscular disorders and heart disease were ruled out. However, during the course of the investigation, bilateral diaphragmatic paresis was discovered. A key sign leading to the diagnosis was evidence of paradoxical respiration in the dorsal decubitus position. When the patient was moved from the orthostatic position to the dorsal decubitus position, oxygenation and forced vital capacity worsened. The orthostatic fluoroscopy was normal. Maximal inspiratory pressure was severely reduced. The responses to transcutaneous electric stimulation of the diaphragm were normal. However, electric stimulation of the phrenic nerve produced no response, leading to the diagnosis of bilateral diaphragmatic paresis.

Tres casos de parálisis diafragmática: utilidad del estudio electromiográfico / Three cases of diaphragmatic paralysis: the utility of electromyographic studies

La parálisis diafragmática (PD) es poco frecuente en pediatría y su adecuado manejo es importante por el potencial compromiso respiratorio. La PD es sospechada por la radiografía de tórax y confirmada por la ultrasonografía. Ultimamente la electromiografía (EMG) ha mostrado utilidad en la evaluación funcional del diafragma y del nervio frénico (NF), en especial a lo que se refiere a su pronóstico y reversibilidad. Objetivo: Reportar la utilidad de la EMG en 3 pacientes con PD. Pacientes y Método: Caso 1, lactante con PD bilateral secundaria a enfermedad neuromuscular adquirida y distrés respiratorio, la EMG mostró denervación y se indicó ventilación permanente. Caso 2: lactante con PD derecha secundaria a cirugía del plexo braquial, el seguimiento electromiográfico permitió tratamiento conservador. Caso 3: preescolar portadora de cardiopatia compleja, PD bilateral post cirugía cardiaca y dependencia de ventilación mecánica (VM), se realizó plicatura diafragmática bilateral, presentó recidiva, necesitó nueva plicatura izquierda por amplitud muy disminuida en la respuesta diafragmática al estudiar conducción de NF. Conclusión: La EMG determinó la disfunción del NF y permitió una conducta conservadora en estos pacientes evitando la cirugía. Sugerimos que la EMG es una herramienta útil en el estudio de pacientes con PD y en la determinación de su conducta terapéutica.

[Hemidiaphragmatic paralysis as a manifestation of familial brachial plexus neuropathy]. / Parálisis hemidiafragmática como manifestación de la plexopatía braquial familiar.

We present a patient with familial brachial plexus neuropathy (FBPN) who is in the second generation of a family found to have this disease. The patient suffered phrenic paralysis, which has only been previously described in association with FBPN in 4 similar cases, all of which also involved the left phrenic nerve. The side of phrenic paralysis's was unrelated to the side where brachial plexus lesions occurred. We believe that paralysis of the diaphragm, although rare, can be considered a sign of FBPN and that therefore this disease should be included in the differential diagnosis of such paralysis. This is particularly so whenever family or personal history suggest that the peripheral nervous system may be involved, compromising the brachial plexus, or whenever facial or digital dysmorphia is present.

[Diaphragmatic paralysis due to a lesion of the phrenic nerve secondary to venesection at the neck for parenteral feeding]. / Parálisis diafragmática por lesión del nervio frénico secundaria a venodisección en cuello para alimentación parenteral.

It is presented the clinical record of a female newborn of 39 weeks of gestational age, with birth weight of 4600 g, with diaphragmatic palsy eight days after birth. Were realized diaphragmatic plicature and the clinical course was without any complication. Were reviewed the causes of diaphragmatic palsy and was described the relationship, with vein cannulation for parenteral nutrition.

Paralisia bilateral do diafragma no pós-operatório imediato de cirúrgia cardíaca / Immediate post-operative bilateral diaphragmatic paralysis after cardiac surgery

Homem de 56 anos, submetido a implante de prótese metálica em posiçäo aórtica, e a enxerto de safena para artéria coronária direita, evoluiu no pós-operatório imediato com insuficiência respiratória de difícil reconhecimento etiopatogênico, finalmente relacionada à paralisia bilateral do diafragma, diagnóstico confirmado através do exame ultrasonográfico de tórax. O paciente permaneceu sob assistência ventilatória com pressäo positiva por 75 dias, quando foi introduzido ventilador de pressäo negativa (VPN), tipo colete, permitindo o fechamento da traqueostomia e recuperaçäo da respiraçäo espontânea. Alta hospitalar no 90§ dia, com orientaçäo do uso de VPN no período noturon. Após 40 dias, o paciente retornou as suas atividades profissionais, sem qualquer apoio à respiraçäo

A 56 years old man was submitted to an aortic replacement with mechanical prosthesis and to saphenous vein graft to the right coronary artery. He developed respiratory insufficiency after surgery which was finally related to bilateral diaphragmatic paralysis whose diagnosis was confirmed through thorax ultrassonography. The patient remained under ventilatory assitance with positive pressure for 75 days. The introduction of thorax ventilatory negative pressure (VNP), jacket type, allowed the traqueostomy closure. The patient gradually recovered his spontaneous breathing and was discharged after 90 days with instructions to use VNP at night. He returned to his normal professional activities, without auxiliary respiratory support, 40 days after his discharge.

Paralisia diafragmática unilateral reversível associada a envenenamento loxoscélico sistêmico / Reversible unilateral diaphragmatic paralysis associated with systemic Loxosceles poisoning

Relata-se o caso de um paciente vítima de envenenamento loxoscélico associado a paralisia diafragmática direita reversível. O diagnóstico de envenenamento loxoscélico baseou-se nas informaçöes prestadas pelo paciente de que havia encontrado uma aranha marrom em sua cama no dia seguinte à picada e no quadro clínico típico deste tipo de envenenamento: lesäo cutânea necrótica acompanhada de erupçäo escarlatiniforme e comprometimento sistêmico sob a forma de insuficiência renal aguda, distúrbios da coagulaçäo sangüínea, hemólise intravascular e hemoglobinúria. Estas alteraçöes regrediram completamente com o tratamento conservador. O diagnóstico da paralisia diafragmática baseou-se na elevaçäo da hemicúpula diafragmática direita na radiografia de tórax em inspiraçäo forçada e em sua completa imobilidade no exame radioscópico. A paralisia frênica näo existia na radiografia realizada previamente ao acidente e desapareceu completamente trinta dias após o mesmo, o que permitiu associá-la à toxocidade do veneno loxoscélico ou a outras manifestaçöes sistêmicas produzidas por ele