CONGENITAL TRACHEAL STENOSIS PRESENTING IN THE NEONATAL PERIOD

Transcription

1 CONGENITAL TRACHEAL STENOSIS PRESENTING IN THE NEONATAL PERIOD J Reiter, C Springer, E Erez Israel Society of Pediatric Pulmonolgy Jerusalem, September 2 nd, 2015

2 Topics Case Presentation Surgical Intervention Prof. Erez Post-op Course Discussion

3 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

4 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 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

5 Case Presentation cont. CXR

6 Case Presentation cont. Bronchoscopy

7 Case Presentation cont. Chest CT angiography

8 Case Presentation cont. Chest CT angiography

9 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

10 PROF. ELDAD EREZ, CARDIO-THORACIC SURGERY

11 Slide Tracheoplasty (1)

12 Slide Tracheoplasty (2)

13 Slide Tracheoplasty (3)

14 Slide Tracheoplasty (4)

15 Slide Tracheoplasty (5)

16 Slide Tracheoplasty (6)

17 POST-OP COURSE

18 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

19 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

20 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

21 Case Presentation cont. Post-op Course 3 months post-op bronchoscopy (26/8/15)

22 DISCUSSION: CONGENITAL TRACHEAL STENOSIS (CTS)

23 Introduction Epidemiology: Rare 1:64,500 (Canada) % 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, Anton-Pacheco et al, 2006

24 Retrospective study ( ;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

25 Clinical classification

26 Results associated anomalies

27 Literature review ( ): 20 reports, 310 patients, 39 managed conservatively Mean presentation ± 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

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29 Long segment CTS, complete rings Clinical group IIIB Parents refused surgery Precious conception Mortality risk (ST - 14%) 3 followed ys showed tracheal growth Up to 10% of complete tracheal rings will presumably not require surgery 1 1 Rutter et al, 2004

30 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

31 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

32 The End