RARE CASE OF PRIMARY TRACHEAL SCHWANNOMA - Paediatric central airway tumour debulking. скачать в хорошем качестве

RARE CASE OF PRIMARY TRACHEAL SCHWANNOMA - Paediatric central airway tumour debulking.

#paediatricbronchoscopy #drtinkujoseph Presentation: 9 years old child with history of breathlessness, cough and episodic wheezing was managed as a case of bronchial asthma at multiple hospitals for past one year. Gradually her condition started to deteriorate and was admitted at a tertiary care hospital in view of stridor. Soon she was initiated on mechanical ventilator support in view of worsening type 1 respiratory failure. Co morbidities: Hyperthyroidism Radiology: Initial chest x-ray done at the time of hospital admission was normal. CT chest and neck was done once patient got ventilated, which showed soft tissue density lesion with heterogeneous area (1.2x1.2x1.2), with poorly defined posterior margin is seen projecting into tracheal lumen, at a distance of 0.96 cm above carina. Collapse consolidation of left lower lobe. Chest x-ray done 24 hours was ventilation showed near total collapse of left lung. Airway Intervention: Flexible bronchoscopy done immediately after intubating the child showed a large growth at the lower trachea level arising from lateral wall (0.5cm) above the carina causing critical airway narrowing (95% of the tracheal lumen & left main bronchus was occluded). Tracheal tumour resection and reconstruction surgery was suggested initially and due to high risk of morbidity/mortality associated with the procedure patients’ relatives deferred for it. Subsequently patient was shifted to our hospital for rigid bronchoscopy guided debulking of the lesion. In view of progressive hypoxemia on ventilator support, she was shifted to operation theatre for an emergency debulking procedure. Child was intubated with a 6.5mm rigid bronchoscope initially. Airway was secured and VV ECMO was kept as a back up during the procedure. The lesion was initially ablated using 1.9mm size cryo probe (30-40seconds) at multiple sites of the lesion to reduce the chances of a potential major bleed during debulking procedure. 3 F fogarty balloon was also placed adjacent to the lesion to attain haemostasis after debulking. The entire lesion was grasped initially using a wire snare electrocautery and was debulked subsequently. The debulked tissue was removed immediately using flexible cryo probe (Cryoadhesion technique). The base of the lesion was ablated using blunt probe electrocautery and cryo. Adequate haemostasis was achieved post procedure. The entire tracheal lumen was recanalized. Impacted mucous in the left main bronchus, which caused collapse of the left lung was cleared. Child was weaned off from ventilator after 2 hours and was shifted out of ICU within 24 hours. Complications: No immediate/delayed post procedure complications. Post procedure: Histopathological examination of the debulked tissue was suggestive of primary tracheal schwannoma. Whole body PET scan done after few days of procedure ruled out any active pathology at the debulked site and other locations. Learning points: • Not all coughs& wheezes in children are Asthma. • Think about the possible alternate pathologies when your patient is not responding to treatment. • Suspect a central airway obstruction when your patient develops stridor • Early bronchoscopic evaluation helps to clinch the diagnosis in such instances. • Debulking of the central airway lesions in paediatric age group should be done only by an experienced interventional pulmonologist, with adequate back facilities like availability of a paediatric thoracic surgeon, critical care support, perfusionist and a dedicated ECMO team to manage all potential complications associated with the procedure. • Primary tracheal schwannomas are rare and comprise only 1% of all neoplasms. Less than 50 cases only have been reported in the world till date. Most often these are benign lesions. Bronchoscopic surveillance is mandatory for a few years to rule out recurrence.