CONGENITAL TRACHEAL STENOSIS PRESENTING IN THE NEONATAL PERIOD J Reiter, C Springer, E Erez Israel Society of Pediatric Pulmonolgy Jerusalem, September 2 nd, 2015
Topics Case Presentation Surgical Intervention Prof. Erez Post-op Course Discussion
Case Presentation Chief complaint One month old male from Nablus referred to Hadassah for suspected subglottic stenosis History of present illness Born 35 weeks GA, BW 3.3Kg Emergency C-section d/t maternal hemorrhage Diagnosed w/congenital pneumonia: Treated w/ antibiotics Intubated & ventilated 9 days Lt Pneumothorax Extubation dyspnea & stridor unresponsive to steroids / nebs
Case Presentation cont. Background Parents Arab Muslims, non consanguineous 8 healthy siblings No Family history of lung or cardiac disease Physical examination Vitals (RA): Wt 3.350 Kg, HR 148/min, RR 50/min, O2 sat 97%, BP 71/44 mmhg Intermittent stridor at rest Lungs - good bilat air entry, transmitted sounds, no generalized crackles or wheezing Heart S1S2, normal rhythm, no murmurs Abd soft, non distended, non tender, no HSM Extremities - warm, well-perfused, none edematous Skin no rash
Case Presentation cont. CXR
Case Presentation cont. Bronchoscopy
Case Presentation cont. Chest CT angiography
Case Presentation cont. Chest CT angiography
Case Presentation cont. Diagnosis Severe distal tracheal & rt main bronchus stenosis w/complete tracheal rings Course Initially stable on RA Dyspnea, increased O 2 requirement, resp acidosis Intubated and ventilated Rt lung atelectasis Presented to CT-surgery Echo - NL
PROF. ELDAD EREZ, CARDIO-THORACIC SURGERY
Slide Tracheoplasty (1)
Slide Tracheoplasty (2)
Slide Tracheoplasty (3)
Slide Tracheoplasty (4)
Slide Tracheoplasty (5)
Slide Tracheoplasty (6)
POST-OP COURSE
Case Presentation cont. Post-op Course Transferred to PICU intubated & ventilated 72h post-op bronch patent airways Extubated Transferred to peds 2 wk post-op bronch Patent airways Granulation tissue
Case Presentation cont. Post-op Course Transferred to PICU intubated & ventilated 72h post-op bronch patent airways Extubated Transferred to peds 2 wk post-op bronch Patent airways Granulation tissue 6 wk post-op bronch LMB obstruction
Case Presentation cont. Post-op Course Transferred to PICU intubated & ventilated 72h post-op bronch patent airways Extubated Transferred to peds 2 wk post-op bronch Patent airways Granulation tissue 6 wk post-op bronch 8 wk post-op rigid bronchoscopy: Polyp on thick stem obstructing the LMB orifice Cauterized and excised, LMB dilated using rigid bronchoscope Pathology granulation tissue Meds chronic / intermittent CCS, PPI
Case Presentation cont. Post-op Course 3 months post-op bronchoscopy (26/8/15)
DISCUSSION: CONGENITAL TRACHEAL STENOSIS (CTS)
Introduction Epidemiology: Rare 1:64,500 (Canada) 1 0.3-1% of laryngo-tracheal stenoses 2 Male > female Symptoms: A/minimally symptomatic - biphasic wheeze, late onset symptoms Early symptomatic (hours old) respiratory difficulty, wheeze, stridor, frequent respiratory infections, recurrent croup, cyanotic spells, or a coarse, barking cough Late symptomatic (onset ~1 year old) - wheezing, exertional SOB, or retractions Diagnosis: Bronchoscopy 1 Herrera et al, 2007 2 Anton-Pacheco et al, 2006
Retrospective study (1991-2004;n=19) All patient w/ respiratory symptoms Mild stridor on exertion to severe distress and apnea Evaluation Diagnostic bronchoscopy CXR, echo, ph-metry/esophagogram ± CT, MRI, bronchography, PFT, angiography Treatment based on: Clinical group Anatomic type (length, diameter) Presentation period Cantrell and Gould classification
Clinical classification
Results associated anomalies
Literature review (1996-2008): 20 reports, 310 patients, 39 managed conservatively Mean age @ presentation 17.14 ± 24.1m M:F 1.69:1 130 associated co-morbidities (56 Lt PA sling) Management: Surgical techniques Ant. esophageal wall, balloon dilatation, costal cartilage tracheoplasty, pericardial patch, resection & anastomosis, tracheal autograft Slide tracheoplasty better results but requires CP-bypass / ECMO Conservative management Mostly mild or inconsistent symptoms/ less than optimal QOL
Long segment CTS, complete rings Clinical group IIIB Parents refused surgery Precious conception Mortality risk (ST - 14%) 3 followed 3-3.5 ys showed tracheal growth Up to 10% of complete tracheal rings will presumably not require surgery 1 1 Rutter et al, 2004
Conclusion Unless CTS presents with life threatening events or failure to thrive, the child might not require surgical intervention observation to allow for the possibility that complete tracheal rings may grow
Granulation Tissue Aaltonen et al. 2014, Helsinki 2/13 patients Multiple dilation procedures Anton-Pacheco et al. 2006, Madrid 1/19 Endoscopic dilation Chung et al. 2015, Seoul, 1/18 Bronchoscopic removal Herrera et al. 2007, Toronto, 0/20 Perioperative dexamethasone, 6wk ICS Yokoi et al. 2013, Kobe
The End