Infecciones respiratorias recurrentes en paciente escolar, asociadas a Síndrome de Mounier-Khün: reporte de caso / Recurrent respiratory infections associated with Mounier-Khün Syndrome in a school patient: clinical case report / Infecções respiratórias recorrentes associadas à Síndrome de Mounier-Khün num paciente escolar: relato do caso

El síndrome de Mounier Kühn es una patología infrecuente de la vía aérea, caracterizada por una dilatación anormal de tráquea y bronquios. Se debe sospechar ante la presencia de infecciones broncopulmonares recurrentes e irritación traqueobronquial. El diagnóstico se lleva a cabo a través de la medición del diámetro traqueal en tres segmentos de su anatomía, a través de tomografía torácica. Se presenta el caso de un paciente masculino escolar de 10 años de edad, con antecedentes de infecciones respiratorias recurrentes, hospitalizado por un cuadro neumónico; los hallazgos reportados en la tomografía de tórax corresponden a traqueobroncomegalia además de incremento del diámetro esofágico. El tratamiento de esta entidad es sintomático con medidas de sostén y fisioterapia pulmonar.

Mounier Kühn syndrome is a rare airway pathology characterized by abnormal dilatation of the trachea and bronchi. It should be suspected in the presence of recurrent bronchopulmonary infections and tracheobronchial irritation. The diagnosis is made by measuring the tracheal diameter in three segments of its anatomy, through thoracic tomography. We present the case of a 10 year-old male school boy with a history of recurrent respiratory infections, hospitalized for a pneumonic condition; the findings reported in the thoracic tomography correspond to a tracheobronchomegaly in addition to an increase of the esophageal diameter. The treatment of this entity is symptomatic with supportive measures and pulmonary physical therapy.

A síndrome de Mounier Kühn é uma patologia incomumdas vias aéreas, caracterizada por dilatação anormal da traqueia e brônquios. Devese suspeitar na presença de infecções broncopulmonares recorrentes e irritação traqueobrônquica. O diagnóstico é realizado através da medida do diâmetro traqueal em três segmentos de sua anatomia, através da tomografia de tórax. Apresentamos o caso de um paciente escolar de 10 anos de idade, do sexo masculino, com história de infecções respiratórias de repetição, internado por sintomas pneumônicos; os achados relatados na tomografia de tórax correspondem a uma traqueobroncomegalia, além de aumento do diâmetro esofágico. O tratamento dessa entidade é sintomático com medidas de suporte e fisioterapia pulmonar.

Thoracic Computed Tomography Scan and Bronchoscopy Appearance of Mounier-Kuhn Syndrome: A Case Report.

Mounier-Kuhn syndrome (MKS) is a rare congenital disease with an autosomal recessive inheritance pattern, characterized by an enlargement of the trachea and bronchi. MKS is secondary to a thinning of the muscular mucosa and atrophy of the longitudinal muscle and elastic fibers of the tracheobronchial tree. As a consequence, tracheal diverticulosis and dilatations in the posterior membranous wall appear, along with bronchiectasis that tend to be cystic in appearance. Overall, there is an impairment of mucocilliary clearance, with an ineffective cough, which predisposes the patient to recurrent lower respiratory tract infections. Clinical manifestations vary from asymptomatic to respiratory failure and death, most patients being diagnosed between the third and fourth decades of life. It is an often undiagnosed disease, with a diagnostic algorithm that includes the use of radiological techniques, alone or in combination with bronchoscopy. Specific diagnostic criteria have been developed, based on patients' tracheal and main bronchi diameter on chest X-ray and thoracic computed tomography scan. We present the case of a 45-year-old African American man who presented with a history of multiples episodes of pneumonia that required management in the intensive care unit, on whom MKS was diagnosed.

[Evaluation with chest CT scan of CPAP as a treatment for tracheobronchomalacia in the frame of a Mounier-Kuhn syndrome]. / Évaluation par tomodensitométrie thoracique d'un traitement de la trachéobronchomalacie par pression positive continue dans le cadre d'un syndrome de Mounier­Kuhn.

INTRODUCTION: Tracheobronchomegaly disease is often associated with a tracheobronchomalacia which is responsible for recurrent lower respiratory tract infections. Currently there is no evidence to support any specific treatment for the condition. CASE REPORT: We report the case of a 79 years old patient presenting with tracheobronchomegaly in the context of Mounier-Kuhn syndrome complicated by a tracheobronchomalacia responsible for her symptomatology. The diagnosis of tracheobronchomalacia had been confirmed by high-resolution chest computed tomography (CT) with expiratory slices and virtual bronchoscopy. Treatment with continuous positive airway pressure (CPAP) was proposed, and we confirmed its efficacy using high-resolution chest CT, which showed a decrease in tracheobronchial collapse and a reduction in air trapping. CONCLUSIONS: Three-dimensional virtual bronchoscopy is an interesting tool and a noninvasive method to diagnose tracheobronchomegaly for patients who are at a high anesthetic risk. It is also possible to use it demonstrate the effect of CPAP in tracheobronchomalacia.

Early manifestation of ARDS in COVID-19 infection in a 51- year-old man affected by Mounier-Kuhn syndrome.

A 51-year-old man known to be affected by Mounier-Kuhn syndrome (MKS). presented to Accident & Emergecy (A&E) with fever, dyspnea and deterioration of his chronic coughs. Increased diameters of his trachea (39 mm), right (30 mm) and left (26 mm) main bronchi were revealed by chest computerized tomography (CT) scan. CT scan showed also ground-glass opacities (GGO) and bronchiectasis in the mid and lower zones of both lungs. COIVD-19 infection was eventually confirmed by RT-PCR. A severe form of COVID-19 could occur even in the early stages of the disease in presence of underlying co-morbidities including MKS, which increases the susceptibility to more recurrent and severe respiratory infections.

The effect of ankaferd blood stopper used for massive haemoptysis in a patient with mounier-kuhn syndrome: A case report.

Mounier-Kuhn Syndrome (MKS) is a rare disorder derived from the muscular and elastic tissue defects of the trachea and the main bronchial walls, characterized by tracheobronchomegaly. Patients may present with complaints of cough, phlegm, dyspnoea and haemoptysis. Haemoptysis may be minor and mixed with phlegm or it may be massive. Establishment of airway patency is a priority in the management of massive haemoptysis. Cold saline solution, diluted adrenaline or tranexamic acid may be administered via the endobronchial route to stop haemorrhage while establishing the airway patency. Ankaferd Blood Stopper (ABS) has a haemostatic property and can be locally administered to the airway. In this report, we aim to highlight the effects of ABS administered via an endobronchial route for emergency palliation of a patient with MKS presenting with massive haemoptysis.

Volume Measurement in the Diagnosis of Mounier Kuhn Syndrome and an Unknown Accompanying Pathology: Pulmonary Artery Enlargement.

BACKGROUND: Mounier Kuhn Syndrome (MKS) is a rare congenital anomaly characterized by abnormal dilatation of the trachea and main bronchi. The aim of this study is to discuss tracheal volume measurement in MKS, and the pathologies accompanying MKS, especially pulmonary artery enlargement. MATERIALS AND METHODS: 38 patients, 18 of whom were diagnosed with MKS and 20 as control group, were included in the study. Trachea volume and pulmonary artery diameter were measured through thorax-computed tomography (CT) images of the patients. Accompanying pathologies were recorded. RESULTS: In the measurements done through the CT scans, the trachea volume was found to be 25.45 cm3 in the control group and 44.17 cm3 in the patient group. The most frequent accompanying pathologies were tracheal diverticulum, bronchiectasis and pulmonary artery enlargement. CONCLUSION: In patients with MKS, there is a significant difference in volume calculation as in trachea diameter. Though bronchiectasis and tracheal diverticulum are known as pathologies most frequently accompanying MKS, to the knowledge of the researchers, pulmonary artery enlargement due to the increase in pulmonary truncus diameter was first emphasized in this article.

Mounier-Kuhn syndrome (tracheobronchomegaly): An analysis of eleven cases.

BACKGROUND AND AIM: Mounier-Kuhn syndrome (MKS) is a congenital disorder characterized by tracheobronchomegaly resulting from the absence of elastic fibers in the trachea and main bronchi or atrophy and thinning of the smooth muscle layer. In this syndrome, dead space associated with tracheobronchomegaly increases and discharge of secretions decreases because of ineffective coughing. The most common complications are recurrent lower respiratory tract infections and bronchiectasis. We examined the clinical characteristics, radiological features, and related complications of patients with MKS. METHODS: The cases were obtained between September 2007 and November 2015. Computed tomography scans of the chest were used to diagnose tracheobronchomegaly. RESULTS: All cases (a total of 11) were males with a mean age of 63 ± 13 (range, 38-80) years. The mean diameter of the trachea was 31.53 ± 2.99 mm; the mean transverse diameter was 31.69 ± 3.10 mm and the mean sagittal diameter was 31.36 ± 3.01 mm. Complaints at the time of presentation included chronic cough, purulent sputum, dyspnea, and hemoptysis. There were recurrent pulmonary infections in seven cases, bronchiectasis in six, and tracheal diverticulum in four at the time of diagnosis. CONCLUSIONS: In this article, 11 cases with various rarely seen complications are presented and evaluated in the light of current literature. We recommend that if chronic cough, recurrent pulmonary infections, and bronchiectasis seen in a patient, MKS should be kept in mind.

Ehlers-Danlos Syndrome Type IVB and Tracheobronchomegaly.

Tracheobronchomegaly (TBM) is a rare disease of unknown etiology, characterized by dilatation of the trachea and main bronchi. Despite its rarity, TBM has been reported to accompany several conditions, particularly Ehlers-Danlos syndrome. Herein, we report on a 15-year-old patient who was diagnosed with TBM on the basis of radiologic and endoscopic findings and with Ehlers-Danlos syndrome type IVB presenting with marfanoid features, severe kyphoscoliosis, increased skin elasticity, and ongoing hypotonia since the neonatal period.

Dural ectasia associated with Mounier-Kuhn syndrome: a novel association in the context of spinal stenosis.

We present the case of a patient undergoing lumbar spine decompression for stenosis with a history of Mounier-Kuhn syndrome. The patient presented with axial lumbar spine pain over 6 months with progressive radicular pain to the left L3 dermatome. MRI confirmed spinal stenosis at L3/4 level with associated dural ectasia. The patient had an uneventful spinal decompression with resolution of radicular symptoms and axial spine pain. Dural ectasia poses a significant risk when operating on the lumbosacral spine. Larger cerebrospinal fluid volumes and a capacious dural canal can result in anaesthetic and orthopaedic complications such as inadequate spinal anaesthesia, complicated epidural analgesia, intraoperative dural tears and difficult pedicle screw insertion due to narrow pedicles. This is the first case in the literature detailing the association between dural ectasia and Mounier-Kuhn syndrome. We recommend adequate spinal imaging in patients with Mounier-Kuhn syndrome to exclude dural ectasia prior to undergoing lumbosacral spinal procedures.

Respiratory Conditions Associated with Tracheobronchomegaly (Mounier-Kuhn Syndrome): A Study of Seventeen Cases.

BACKGROUND: Mounier-Kuhn syndrome (MKS) is a rare disorder characterized by enlargement of the trachea and main bronchi and associated with recurrent respiratory tract infections. OBJECTIVE: This multicenter, retrospective study was carried out to describe respiratory conditions associated with tracheobronchomegaly. METHODS: Nine institutions involved in the 'Groupe d'Endoscopie de Langue Française' (GELF) participated in this study. A standard form was used to record patient characteristics, treatments and follow-up from medical charts. RESULTS: Seventeen patients, 53% male, aged 58 ± 18 years at diagnosis were included. Recurrent infections revealed MKS in 88% of cases. Main comorbid conditions were diffuse bronchiectasis in 88% of patients and tracheobronchomalacia in 67% of cases. The exacerbation rate was 1.5 exacerbations/patient/year. The main non-respiratory morbid condition was gastroesophageal reflux disease in 29% of cases. Interventional bronchoscopy was performed in seven patients (41%), consisting of laser (n = 2) and tracheal stenting (n = 5). Complications related to stents were observed in 80% of cases with a mean stent duration of 8 months. Four deaths, including three due to respiratory causes, occurred during follow-up. CONCLUSIONS: This is the largest series of MKS reported in the literature, showing that bronchiectasis and tracheobronchomalacia are the main associated morbid conditions that constitute a challenge for treatment.

Mounier-Kuhn syndrome: a systematic analysis of 128 cases published within last 25 years.

BACKGROUND AND AIMS: Mounier-Kuhn syndrome is a rare disease with abnormal enlargement of major airways, but epidemiological studies are lacking, and currently the most available data about it come from case reports, making it difficult to collate changes in a particular patient to those in previously published cases. The aim of this work is to systematically review cases published in the last 25 years and to use descriptive statistics to summarize the patient demographic and clinical information therein in order to acquire details about patient clinical characteristics. METHODS: Cases published in world literature between 1987 and 2013 were sought and reviewed according to PRISMA guidelines. Cases were included only if patient's age was at least 18 years, and a computed tomography scan with tracheal measurements was available. RESULTS: An 8:1 male predominance was found in 89 identified reports (128 cases). Mean age was 53.9 years, and average tracheal diameter was 36.1 mm. No correlation between increasing age and increasing tracheal diameter was found. Bronchiectasis, tracheal diverticulosis and tracheobronchial dyskinesia were common (49.2%, 33.6% and 28.9%, respectively). Cough, dyspnea and recurrent respiratory infections (71.1%, 51.6% and 50.8%, respectively) were the most common complaints. CONCLUSIONS: The data confirm that syndrome mostly manifests with nonspecific respiratory symptoms and is significantly more common in males. Importantly, there was no connection between age and airway diameter, a fact that could mean that the enlargement does not progress over time, and its severity depends on some other yet undetermined factors.

An unusual cause of recurrent spontaneous pneumothorax: the Mounier-Kuhn syndrome.

We present a case of 63-year-old man who was referred to the emergency department with a right-sided pneumothorax. He had a history of spontaneous pneumothorax for 2 times. The chest computed tomographic scan showed tracheobronchomegaly with an increase in the diameter of the trachea and right and left main bronchus. Fiberoptic bronchoscopy revealed enlarged trachea and both main bronchus with diverticulas. These findings are consistent with a diagnosis of Mounier-Kuhn syndrome. Mounier-Kuhn syndrome is a rare clinical and radiologic condition. It is characterized by a tracheal and bronchial dilation. Diagnosis is made by computed tomography and bronchoscopy. Mounier-Kuhn syndrome should be kept in mind in the differential diagnosis of recurrent spontaneous pneumothorax.