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with previous diagnosis of morbid obesity &#40;BMI of 50<span class="elsevierStyleHsp" style=""></span>kg&#47;m<span class="elsevierStyleSup">2</span>&#41;&#44; arterial hypertension&#44; hypertensive heart disease&#44; hypothyroidism and depression was first admitted to our department in 2007 with a one-month history of progressive exertional dyspnoea&#44; paroxysmal nocturnal dyspnoea and lower limb oedema&#44; with no other symptoms&#46; There was no history of recent trauma or surgery&#46;</p><p id="par0015" class="elsevierStylePara elsevierViewall">On admission&#44; she was started on non-invasive ventilation &#40;NIV&#41; for hypercapnic respiratory failure &#40;pH 7&#46;35&#44; PaCO<span class="elsevierStyleInf">2</span> 72&#46;4 and PaO<span class="elsevierStyleInf">2</span> 40&#46;2<span class="elsevierStyleHsp" style=""></span>mmHg&#41;&#46; The initial chest radiographs showed an hypotransparency on the right pulmonary lower lobe&#46; The chest CT scan identified a large Morgagni hernia on the right side&#44; 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At that time her lung function test showed a moderately severe obstructive pattern &#40;FVC 1&#46;05<span class="elsevierStyleHsp" style=""></span>mL &#40;67&#37;&#41;&#59; FEV<span class="elsevierStyleInf">1</span> 0&#46;64<span class="elsevierStyleHsp" style=""></span>mL &#40;52&#37;&#41;&#59; FEV<span class="elsevierStyleInf">1</span>&#47;FVC ratio 61&#46;14&#37;&#59; TLC 2&#46;87<span class="elsevierStyleHsp" style=""></span>mL &#40;82&#37;&#41;&#59; RV 1&#46;76<span class="elsevierStyleHsp" style=""></span>mL &#40;106&#37;&#41;&#59; ITGV 2&#46;50 &#40;112&#37;&#41;&#41;&#46; A new CT scan showed increased volume of the Morgagni hernia&#44; which contained part of the transverse colon&#44; all the ascending colon&#44; loops of ileum and distal jejunum&#44; with the insinuation of gastric antrum&#44; leading to passive atelectasis of the middle lobe and right lower lobe and deviation of the mediastinal structures to the left hemithorax&#46;</p><p id="par0020" class="elsevierStylePara elsevierViewall">At the end of the year 2013&#44; 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FEV<span class="elsevierStyleInf">1</span>&#47;FVC ratio 74&#46;75&#59; TLC 4&#46;47<span class="elsevierStyleHsp" style=""></span>mL &#40;127&#37;&#41;&#59; RV 2&#46;01<span class="elsevierStyleHsp" style=""></span>mL &#40;117&#37;&#41;&#59; ITGV 2&#46;45<span class="elsevierStyleHsp" style=""></span>mL &#40;110&#37;&#41;&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0025" class="elsevierStylePara elsevierViewall">In 2014&#44; she did a polysomnography which showed severe obstructive sleep apnea with a respiratory disturbance index &#40;RDI&#41; of 39&#46;3&#47;H and time oxygen saturation &#60;90&#37; &#40;T90&#41; of 19&#46;9&#37;&#46; The patient was started on long-term positive airway pressure treatment with good adherence&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">In our literature review&#44; we could only find ten reported cases of MH with respiratory failure in adults&#46;<a class="elsevierStyleCrossRefs" href="#bib0065"><span class="elsevierStyleSup">5&#44;6</span></a> It is a rare aetiology for respiratory failure&#46; It is usually asymptomatic or it presents with non-specific chronic symptoms&#46; Because of these characteristics&#44; the diagnosis of MH it is not often considered when changes in chest radiographs are present&#46; Sometimes the typical radiological images of intra-thoracic gas-filled loops of the bowel are not present and it can appear as an opacity mimicking pneumonia&#44; intrathoracic tumour&#44; atelectasis&#44; pericardial cyst or simulate a pneumothorax&#46;<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">3&#44;4</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">Hypoventilation is a result of lung parenchyma compression by the abdominal viscera and diaphragm impairment&#44; which causes a restrictive ventilatory defect&#46;<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">7</span></a> Oxygenation decreases due to alveolar collapse in the atelectatic lung&#46;<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">7</span></a> These changes in pulmonary mechanics and respiratory muscle performance tend to have worse outcomes in obese patients as they already breathe at abnormally low lung volumes&#46; NIV is a useful tool as it improves alveolar recruitment of the affected area&#44; while simultaneously increasing the functional residual capacity and minute ventilation&#46;<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">7</span></a> Nevertheless&#44; NIV may also lead to deterioration of MH and consequently of respiratory status by causing aerophagia and abdominal distension&#46;<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">5</span></a> Another possible mechanism is by increasing intra-abdominal pressure when using high positive end-expiratory pressure&#46;<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">7</span></a> We think that in our patient the volume increase of the MH since 2007&#44; when she was started on NIV&#44; can potentially be explained by these mechanisms&#44; complicated by the severe obesity&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">On the other hand&#44; there is no consensus or guidelines on surgical timing in non-acute cases&#46; Although the majority of these hernias are asymptomatic&#44; repair is recommended to avoid future complications<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">2&#44;3</span></a> and may improve lung function<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">6</span></a> as we were able to demonstrate&#46; Surgical intervention may also reverse chronic respiratory failure as Tone et al&#46;<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">5</span></a> report and we also found in this case&#46; It is important to stress that the first lung function tests were carried out less than a month before surgery and that the patient regained weight after the surgical intervention&#46; Another aspect to be mentioned is that our laboratory uses the ERS Quanjer 1993 lung function reference values<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">8</span></a> which were validated to patients with a minimum height of 154<span class="elsevierStyleHsp" style=""></span>cm tall&#46; Since our patient height is only 141<span class="elsevierStyleHsp" style=""></span>cm tall&#44; her measured values as percent of predicted are globally overestimated and we should focus on the absolute volumes&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">This case demonstrates the benefit of surgical reduction of this type of hernia&#44; even in cases of chronic respiratory failure and additional causes for increased surgical risk&#46;</p></span>"
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Scientific letter
Morgagni Hernia as a Reversible Cause of Hypercapnic Respiratory Failure
Hernia diafragmática de Morgagni como causa reversible de insuficiencia respiratoria hipercápnica
Patrícia Dionísio
Corresponding author
patriciadionisio1@gmail.com

Corresponding author.
, Susana Moreira, Rita Pinto Basto, Paula Pinto
Pulmonology Department, Centro Hospitalar Lisboa Norte, Portugal
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">The three basic types of congenital diaphragmatic hernia &#40;CDH&#41; include hiatus hernia&#44; posterolateral Bochdalek hernia and anterior Morgagni hernia &#40;MH&#41;&#44; the last being the rarest type &#40;2&#8211;3&#37; of all cases&#41;&#46;<a class="elsevierStyleCrossRefs" href="#bib0045"><span class="elsevierStyleSup">1&#44;2</span></a> The diagnosis of MH in adults is very rare and usually is incidental or presents with non-specific chronic respiratory or gastrointestinal symptoms&#46;<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">2&#44;3</span></a> It can also be present with acute bowel obstruction or intestinal strangulation&#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">3</span></a> Late-presenting CDH is often difficult to diagnose&#44; and delays in treatment are common&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">4</span></a> Few cases have been described in the literature&#46;</p><p id="par0010" class="elsevierStylePara elsevierViewall">A 64-year-old non-smoker caucasian woman&#44; with previous diagnosis of morbid obesity &#40;BMI of 50<span class="elsevierStyleHsp" style=""></span>kg&#47;m<span class="elsevierStyleSup">2</span>&#41;&#44; arterial hypertension&#44; hypertensive heart disease&#44; hypothyroidism and depression was first admitted to our department in 2007 with a one-month history of progressive exertional dyspnoea&#44; paroxysmal nocturnal dyspnoea and lower limb oedema&#44; with no other symptoms&#46; There was no history of recent trauma or surgery&#46;</p><p id="par0015" class="elsevierStylePara elsevierViewall">On admission&#44; she was started on non-invasive ventilation &#40;NIV&#41; for hypercapnic respiratory failure &#40;pH 7&#46;35&#44; PaCO<span class="elsevierStyleInf">2</span> 72&#46;4 and PaO<span class="elsevierStyleInf">2</span> 40&#46;2<span class="elsevierStyleHsp" style=""></span>mmHg&#41;&#46; The initial chest radiographs showed an hypotransparency on the right pulmonary lower lobe&#46; The chest CT scan identified a large Morgagni hernia on the right side&#44; with the compromise of the right lung volume and contralateral mediastinal deviation&#44; as well as signs of pulmonary hypertension&#46; The echocardiogram confirmed mild pulmonary hypertension &#40;PSAP 49<span class="elsevierStyleHsp" style=""></span>mmHg&#41;&#46; She was referred to Cardiothoracic Surgery but was refused because of her surgery-related risk&#46; She was discharged on long-term supplemental oxygen therapy and domiciliary NIV&#46; Between 2008 and 2012 she was admitted three times for decompensated hypercapnic respiratory failure and for oxygen and ventilatory parameters adjustment&#46; Diaphragmatic hernia was also bigger on the chest radiographs&#46; In 2013 she was admitted five times in the context of emesis and gastroparesis&#46; Despite a significant weight loss &#40;BMI 50 to 41<span class="elsevierStyleHsp" style=""></span>kg&#47;m<span class="elsevierStyleSup">2</span>&#41;&#44; she maintained the need for both long-term supplemental oxygen therapy and NIV&#46; At that time her lung function test showed a moderately severe obstructive pattern &#40;FVC 1&#46;05<span class="elsevierStyleHsp" style=""></span>mL &#40;67&#37;&#41;&#59; FEV<span class="elsevierStyleInf">1</span> 0&#46;64<span class="elsevierStyleHsp" style=""></span>mL &#40;52&#37;&#41;&#59; FEV<span class="elsevierStyleInf">1</span>&#47;FVC ratio 61&#46;14&#37;&#59; TLC 2&#46;87<span class="elsevierStyleHsp" style=""></span>mL &#40;82&#37;&#41;&#59; RV 1&#46;76<span class="elsevierStyleHsp" style=""></span>mL &#40;106&#37;&#41;&#59; ITGV 2&#46;50 &#40;112&#37;&#41;&#41;&#46; A new CT scan showed increased volume of the Morgagni hernia&#44; which contained part of the transverse colon&#44; all the ascending colon&#44; loops of ileum and distal jejunum&#44; with the insinuation of gastric antrum&#44; leading to passive atelectasis of the middle lobe and right lower lobe and deviation of the mediastinal structures to the left hemithorax&#46;</p><p id="par0020" class="elsevierStylePara elsevierViewall">At the end of the year 2013&#44; she was submitted to reduction and repair of the hernia and gastropexy&#44; with a favourable expansion of the right lung &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41; and gradual resolution of global respiratory failure&#46; Despite regaining weight &#40;BMI 43<span class="elsevierStyleHsp" style=""></span>kg&#47;m<span class="elsevierStyleSup">2</span>&#41; it was possible to discontinue domiciliary NIV and supplemental oxygen therapy &#40;pH 7&#46;39&#44; PaCO<span class="elsevierStyleInf">2</span> 47&#46;8 and PaO<span class="elsevierStyleInf">2</span> 75&#46;0<span class="elsevierStyleHsp" style=""></span>mmHg in room air&#41;&#46; The echocardiogram showed a reduction in PSAP from 49 to 39<span class="elsevierStyleHsp" style=""></span>mmHg&#46; There was a remarkable improvement in her lung volumes&#44; that are now within the normal range &#40;FVC 2&#46;29<span class="elsevierStyleHsp" style=""></span>mL &#40;157&#37;&#41;&#59; FEV<span class="elsevierStyleInf">1</span> 1&#46;71<span class="elsevierStyleHsp" style=""></span>mL &#40;150&#37;&#41;&#59; FEV<span class="elsevierStyleInf">1</span>&#47;FVC ratio 74&#46;75&#59; TLC 4&#46;47<span class="elsevierStyleHsp" style=""></span>mL &#40;127&#37;&#41;&#59; RV 2&#46;01<span class="elsevierStyleHsp" style=""></span>mL &#40;117&#37;&#41;&#59; ITGV 2&#46;45<span class="elsevierStyleHsp" style=""></span>mL &#40;110&#37;&#41;&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0025" class="elsevierStylePara elsevierViewall">In 2014&#44; she did a polysomnography which showed severe obstructive sleep apnea with a respiratory disturbance index &#40;RDI&#41; of 39&#46;3&#47;H and time oxygen saturation &#60;90&#37; &#40;T90&#41; of 19&#46;9&#37;&#46; The patient was started on long-term positive airway pressure treatment with good adherence&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">In our literature review&#44; we could only find ten reported cases of MH with respiratory failure in adults&#46;<a class="elsevierStyleCrossRefs" href="#bib0065"><span class="elsevierStyleSup">5&#44;6</span></a> It is a rare aetiology for respiratory failure&#46; It is usually asymptomatic or it presents with non-specific chronic symptoms&#46; Because of these characteristics&#44; the diagnosis of MH it is not often considered when changes in chest radiographs are present&#46; Sometimes the typical radiological images of intra-thoracic gas-filled loops of the bowel are not present and it can appear as an opacity mimicking pneumonia&#44; intrathoracic tumour&#44; atelectasis&#44; pericardial cyst or simulate a pneumothorax&#46;<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">3&#44;4</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">Hypoventilation is a result of lung parenchyma compression by the abdominal viscera and diaphragm impairment&#44; which causes a restrictive ventilatory defect&#46;<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">7</span></a> Oxygenation decreases due to alveolar collapse in the atelectatic lung&#46;<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">7</span></a> These changes in pulmonary mechanics and respiratory muscle performance tend to have worse outcomes in obese patients as they already breathe at abnormally low lung volumes&#46; NIV is a useful tool as it improves alveolar recruitment of the affected area&#44; while simultaneously increasing the functional residual capacity and minute ventilation&#46;<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">7</span></a> Nevertheless&#44; NIV may also lead to deterioration of MH and consequently of respiratory status by causing aerophagia and abdominal distension&#46;<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">5</span></a> Another possible mechanism is by increasing intra-abdominal pressure when using high positive end-expiratory pressure&#46;<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">7</span></a> We think that in our patient the volume increase of the MH since 2007&#44; when she was started on NIV&#44; can potentially be explained by these mechanisms&#44; complicated by the severe obesity&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">On the other hand&#44; there is no consensus or guidelines on surgical timing in non-acute cases&#46; Although the majority of these hernias are asymptomatic&#44; repair is recommended to avoid future complications<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">2&#44;3</span></a> and may improve lung function<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">6</span></a> as we were able to demonstrate&#46; Surgical intervention may also reverse chronic respiratory failure as Tone et al&#46;<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">5</span></a> report and we also found in this case&#46; It is important to stress that the first lung function tests were carried out less than a month before surgery and that the patient regained weight after the surgical intervention&#46; Another aspect to be mentioned is that our laboratory uses the ERS Quanjer 1993 lung function reference values<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">8</span></a> which were validated to patients with a minimum height of 154<span class="elsevierStyleHsp" style=""></span>cm tall&#46; Since our patient height is only 141<span class="elsevierStyleHsp" style=""></span>cm tall&#44; her measured values as percent of predicted are globally overestimated and we should focus on the absolute volumes&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">This case demonstrates the benefit of surgical reduction of this type of hernia&#44; even in cases of chronic respiratory failure and additional causes for increased surgical risk&#46;</p></span>"
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ISSN: 03002896
Original language: English
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