2023-06-22 11:44:54
Updated Thursday, June 22, 2023 – 11:44
They identify this narrowing of the airway in three related women: two sisters and the daughter of one of them.
Image of the Regional University Hospital of Mlaga Jess Domnguez
The Pneumology service of the Hospital Regional Universitario de Mlaga has detected the first case of subglottic tracheal stenosis family idiopathic described in Spain, when identifying this airway narrowing in three related women.
It is a pathology whose cause may be caused by infections, prolonged intubation, trauma or systemic inflammatory diseases. However, a small number of cases are of unknown origin.
This disease consists of a narrowing of the laryngeal lumen at the level of the cricoid cartilage. This is the lowest of the laryngeal cartilages and lies between the cricoid cartilage and the first tracheal rings. Idiopathic subglottic stenosis It is more common in women between the ages of 20-50. and the familial predisposition of this entity is unknown, with few cases reported in the literature, the Board has indicated in a statement.
In this sense, the head of the Pneumology service, Antonio Dorado, has indicated that its detection originated from identifying this pathology in a 55-year-old patient with no history medical surgery of interest, referred due to symptoms of dyspnea -respiratory difficulty- with minimal efforts and without symptoms of gastroesophageal disease.
In the CT of the neck it was observed 4.50 mm tracheal stenosis. Respiratory function tests were then performed, in which fixed airflow obstruction morphology was observed, and conventional bronchoscopy was also performed, in which subglottic stenosis was visualized.
In the second case, Three years later, another patient, the sister of the previous one, who is 53 years old, is treated. and, like the other patient, with no medical history of surgery and referred for 2 years of symptoms of dyspnea on moderate exertion. Similarly, in the tests carried out, a 6-mm tracheal stenosis was observed; with the same respiratory function tests, and subglottic stenosis was also observed.
In the third case, the daughter of one of them, 39 years oldreferred due to symptoms of dyspnea on minimal exertion of one year of evolution, after the pertinent tests was detected in a 5.56 mm tracheal stenosis; with the same respiratory function tests and with subglottic stenosis.
“Idiopathic subglottic stenosis It is a rare disease, with an unknown incidence.. The origin of this stenosis is still unknown, although several studies have been carried out on the action of estrogens in the path of development of this disease, but without finding evidence to support it, as occurs with gastroesophageal reflux, where some authors observed clinical improvement in these patients after starting antireflux treatment. Even so, our series did not present symptoms of reflux,” said Dorado.
Treatment
The three patients were treated by the Bronchoscopy team of the Hospital Regional Universitario de Mlaga. In the first case, the most complex, after balloon tracheopneumoplasty with partial improvement but with recurrence, she was operated on by Thoracic Surgery and sent to the Tracheal Surgery Unit of the Virgen del Roco University Hospital in Seville.
In the second, the patient has been followed up and, in the third, endoscopic treatment with balloon tracheopneumoplasty was decided with clinical improvement and satisfactory subsequent follow-up.
“Conventional treatment includes endoscopic measures, such as resection by thermal ablation -bronchosler, electrocuchilla, etc.- or balloon tracheopneumoplasty, but it presents a high recurrence rate, around 87% at five years, so the alternative would be surgery, more invasivebut with a lower recurrence rate, around 10-40% at 8 years”, added the pulmonologist Francisco Pez.
next to the doctors Antonio Dorado y Francis Fishthe pulmonologist has participated in the study hope salcedo which, after reviewing the medical literature in Spanish, detects that it is the “first case of familial idiopathic subglottic stenosis described in Spain, since the few cases that exist in the literature, even in twin sisters, were reported internationally”.
“All these cases open the door to a possible genetic predisposition for the development of this idiopathic stenosis in adulthoodwhich could be a working hypothesis in future research”, he added. The study and detection of this case has been published in the Elsevier journal ‘Clinical Medicine’.
According to the criteria of The Trust Project
Know more
#case #familial #idiopathic #tracheal #subglottic #stenosis #Spain #detected #Malaga