Page 41 - ECBIP 2021_Program

MODERATED e-POSTER SESSION 01:
Central Airway management
PP03
Loeys-Dietz syndrome (LDS): It is a rare and newly described autosomal dominant ,connective tissue disease in which skeletal, ocular and cardiovascular systems are affected, classified under marfan-like disorders.Heterozygous mutations in transforming growth factor beta receptor1 and 2 (TGFR1,TGFR2) genes are the cause of the disease.The syndrome is typically characterized by hypertelorism, cleft palate or bifid uvula, arterial / aortic aneurysm, and / or arterial tortuosity.
Case study: This case is presented as a contribution to the literature, since the association of trachea-bronchomegaly detected incidentally in LDS may be a part of this syndrome.12-year-old male patient with lds applied to pediatric pulmonology with frequent illness. In the physical examination of the patient; His vital signs were stable and respiratory sounds were normal. Phenotypically, there was a long face and limb length (marfanoid appearance?). He was operated for cleft palate and craniosynostosis.Aortic and pulmonary root dilatation was detected on echocardiography.There was a p.Ser241Leu (c.722C> T) heterozygous mutation in the 4th exon of the TGFR1 gene of the patient. Computed tomography of the patient showed that the diameter of the trachea was larger than normal.Trachea transverse diameter measured at a distance of 10 mm from the carina: 14.7 mm, anteroposterior diameter: 13.6 mm, right main bronchus diameter: 9.9 mm, left main bronchus diameter: 11.3 mm. In 5 male patients of the same age who had thorax bt for different reasons, mean trachea transverse diameter: 10.4 mm (12.6-8.3), anteroposterior diameter: 10.3mm (11.9-8.8), right main bronchus diameter: 8.3 mm (11.4-6.2), left main bronchus diameter: 6.2 mm (7.4-5.4).Bronchoscopy was performed due to recurrent lung infection. Bronchial and trachea diameters were observed to be larger than normal.
As a result: Tracheomegaly has been reported in connective tissue diseases such as Marfan syndrome and Mounier- Kuhn Syndrome, this case is presented since no association of tracheobronchomegaly has been reported before in LDS.
PP04
Introduction: Dieulafoy’s lesion is a rare disease characterised by tortuous and dilated arteries in the submucosa.Though commonly encountered in the gastrointestinal system, the incidence of Bronchial Dieulafoy is rare and commonly presents with hemoptysis. The etiology of this disease is unknown. Often misdiagnosed as endobronchial tumor nodule on bronchoscopy, biopsy of the lesion evokes the risk of triggering massive hemoptysis, which can be fatal. We report a case with recurrent hemoptysis, diagnosed as bronchial dieulafoy.
Case details: A 51 years old female presented with two episodes of massive hemoptysis. The patient had experienced frequent but less severe episodes of hemoptysis for 2 years prior to this. CT thorax did not show any significant abnormality except for ground glass opacities in the right lower lobe. Flexible bronchoscopy showed a small nodular lesion with a smooth surface in the right lower lobe, between RB7 and basal segments. The lesion started to bleed on minimal contact with the bronchoscope. As submucosal tumor infiltration needs to be ruled out, we decided to biopsy the lesion. Anticipating bleed, a bronchial blocker was placed in the right lower lobe and biopsy was taken. Immediately after taking the biopsy, there was a massive spurt of bleeding from the lesion which was controlled with the bronchial blocker and intravenous tranexamic acid.A Digital subtraction angiography was performed,which showed a tortuous right bronchial artery. Bronchial artery embolization was done straight away, following which there are no further episodes of hemoptysis. The histopathological examination of the biopsy showed a normal bronchial mucosa.The patient is being followed up and planned for lobectomy if hemoptysis recurs.
Conclusion: Bronchial dieulafoy is a rare vascular anomaly and should always be included in the differential diagnosis of massive and recurrent hemoptysis. There is no consensus regarding the treatment of Bronchial Dieulafoy and the commonly used methods includes conservative treatment, bronchial artery embolization, surgical resection and various bronchoscopic approaches. Biopsy of this lesion may result in fatal hemoptysis making it a potential disaster for the bronchoscopist.
A CASE REPORT WITH LOEYS DIETZ SYNDROME AND TRACHEA-BRONCOMEGALI ASSOCIATION
Sule Ayas1, Sevgi Pekcan1, Necdet Poyraz2, Mustafa Genceli1, BüSra Sultan Kibar1,
Aslı Imran Yılmaz1, Gökçen Ünal1
1 Department of Pediatric Pulmonology, Meram Faculty of Medicine, Necmettin Erbakan Üniıversty, Konya,Turkey 2 Department of Radiology, Meram Faculty of Medicine, Necmettin Erbakan Üniıversty, Konya,Turkey
BRONCHIAL DIEULAFOY – AN IMPENDING SOURCE OF FATAL HEMOPTYSIS
M.D. Varunn1, Gonuguntla Harikishan2, Deva Ganapathy Thiyagesh2, Samia Syeda2, Preeti Belgundi2
1 PSG Institute of Medical Science and Research, Coimbatore, India
2 Yashoda Hospital, Secundrabad, India
Figure 1: Dieulafoy lesion in the right lower lobe, between RB7 and basal segments.
Figure 2: Digital Subtraction Angiography showing Right Intercostal artery and tortuous Bronchial artery.
ECBIP 2021 41
6th European Congress
for Bronchology and Interventional Pulmonology
OCTOBER ECBIP 15 - 17
ATHENS - GREECE 2021
Book of Abstracts