Sunday, 10 May 2015

Paediatric Stridor: Overview

Please note that the following is a general guideline only. For a full assessment, exclusion of any other underlying cause for your symptoms and an individualised treatment approach, you will need to be seen by a qualified specialist.







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Definition

Stridor – High Pitched abnormal noise due to turbulent airflow
 Stertorsnoring type noise made by naso-/oropharyngeal obstruction


Causes of stridor can be grouped according to anatomical region involved, BUT in reality very difficult to separate the 2 types.Most cases of stridor usually require endoscopy as history and examination 






History

History and examination is done with an understanding of the common causes of stridor which is discussed separately. Its important to determine the severity and to see the child needs urgent endoscopy and intervention. Also the history and examination may give clues as to the likely anatomical site or the cause. 

Full pregnancy history, including..

       ? Neonatal infections


        Prematurity


        APGARs


        Intubation resuscitation




Stridor history, including..


      Timing? – At birth or developed later?


      Timing in the breathing cycle: inspiratory-glottic, Biphasic-tracheal,  Expiratory-pulmonary wheeze) 


    Precipitants
 – Position, if upset, feeding other? 



Examination

Vital signs, 


Oxygen if necessary



Airway



            Stridor- (timing, duration)


            Nasal obstruction


            Recession/ tracheal tug


            Type of cry?


            Retrognathia


            Tongue and oral cavity anomaly?


            Syndromic Facies





Symptoms suggestive of severity-needs urgent admission and endoscopy



            Increased respiratory effort


            Cyanotic spellas, hypoxia  


            Feeding difficulties , associated with cough and gagging (aspiration), loss of weight


            Dyspnoea or tachypnoea




Investigations

If severe symptoms, urgent ENT endoscopy in a paediatric ENT unit. Fiberoptic endoscopy can be done safely in most paediatric ENT units without general anaesthesia.

Other investigations include


      Chest X-ray/ c spine X ray


      MRI (brain, neck, chest) 


      Sleep study (if symptoms suggestive of OSA)

      Laryngotracheobronchoscopy – general anaesthetic, may involve treatment as well




Causes of paediatric stridor

 Several ways of classifyingCongenital-then according to locationAlso according to aetiology 


 Congenital Nasopharyngeal



-      Choanal atresia, stenosis

-      Craniofacial abnormalities 
-       Encephalocele, Meningocele
-       Nasal glioma
-       Haemangioma, lymphangioma
-       Lingual thyroid
-       Thyroglossal cyst




Congenital Laryngeal-       


        Laryngomalacia
-       Posterior laryngeal cleft
-       Laryngeal cysts, vallecula cysts
-       Webs
-       Stenosis
-       Arytenoid fixation
-       Vocal cord paralysis
-       Haemangiomas and lymphangiomas



 Congenital Tracheal-       


         Stenosis
-       Atresia
-       Abnormal cartilaginous rings
-       Tracheobronchomalacia
-       Vascular compression
-       Haemangiomas and lymphangiomas



 Traumatic

-       Always think about foreign bodies-       

        Thermal or chemical injuries
-       Haematomas
-       Intubation trauma (IATROGENIC)
-       Traumatic fracture of larynx
-       Damage to recurrent laryngeal nerve



 Infectious

-      Adenotonsillar hypertrophy
-       Parapharyngeal abscess
-       Peritonsillar abscess
-       Retropharyngeal abscess
-       Lymphadenopathy
-       Ludwig’s angina
-       Epiglottitis
-       Croup and other forms of tracheitis



 Neoplastic









Paediatric Stridor: Specific Conditions 


1 Laryngomalacia


Definition

Abnormal flaccidity of the neonatal larynx which leads to collapse during inspiration

Epidemiology

most common cause of stridor in infants 
M>F, 2:1

Aetiology

? cartilaginous structures are weak,
? neuromuscular "immaturity" exists within the supraglottis leading to laryngeal GORD and Laryngomalacia: Unclear if its a cause or an effect!! 

Symptoms

Newborn typically will develop intermittent, inspiratory, 2 weeks of life
Spontaneous resolution of stridor is 9 months of age, and 75% will have no stridor by 18 months of age
Infrequently-severe, resulting in feeding difficulties, failure to thrive, apnea, pectus excavatum, or cyanosis
In these severe cases surgical intervention is recommended to prevent cardiac failure
Isolated finding in the otherwise healthy infant or may be associated with other neurologic disorders such as cerebral palsy
Associated GORD

Signs

respiratory distress
Flexi if possible may be diagnostic
17 % have a second lesion hence if severe need LBO
Lat Neck XR

Treatment

90% medical

Observation/ higher calorie feeding if not gaining weight
Fatten up: add fat cream etc to feeds start solids earlier even at 8 weeks
Rx GORD-Ranitidien (safe in infancy)n2-4 mg/ kg per dose tds po or IV
Omeprazole (safe in infancy < 1 yr not established)-about 2/3 mg / kg per day

Surgical Rx

Laryngoscopy bronchoscopy  for assessment and treatment surgical -rarely needed
Emergency tracheostomy Rare







2 Sub-Glottic Stenosis 


Definition


narrowing of a new born < 4mm
narrowing of a preterm < 3.5 mm


Epidemiology
M:F= 2:1
3rd most common congenital anomaly of pediatric airway


Aetiology

1 Congenital: Due to incomplete re canalization of the laryngo-tracheal tube
2 Acquired
      -ETT-trauma
     -Infection/ inflammmatory/ reflux (all unlikely in the infant)


Clinical
1 Stridor
       inspiratory or biphasic 
      mild stenosis may be asymptomatic until an URTI -recurrent croup croup
2 Dysphonia
3 associated with other anomaly-10% of laryngomalacia has SG stenosis/ Down syndrome

Treatment 

Rx reflux
Steroids if oedema
Surgery
Endoscopic: Dilatation / laser/ cold steel devision and dilate-local kenocort or Mit mic C
Open Procedures 




Haemangioma


Definition
Hamartomatous vascular neoplasms endothelial hyperplasia


Epidemiology 
Most common tumour in infancy, 2% can become apparent up to 10%
F:M  3:1
Increased incidence with maternal age, multiple gestations and with C villous sampling/ prem babies


Etiology and pathogenisis
Some familial


Classification
Congenital (rare)
      • congenital-rare, fully formed at birth
      • RICH & NICH types
Infantile (common)-grows then involutes. Infantile type has GLUT 1 receptor and can be treated with B blockers


Clinical
Most (Infantile type) grow -6 months (proliferative phase)
stabilise for 1 yr (stable phase)
slowly involute most gone by the age of 3 years (involution phase)

Laryngeal symptoms
     first 6 months of life with inspiratory or biphasic stridor
     recurrent croup -responds temporarily to oral steroids
     diagnosis is made at the time of direct laryngoscopy

Extra laryngeal symptoms
    1/2 have other cutaneous haemangiomas



Investigations
L&B: Biopsy- GLUT1 glucose transporter protein (infantile is GLUT1 positive, congenital-ve)
MRI brain / H&N 



Treatment 
1 Steroids
2 Propranalol if Gute 1 positive


2 Surgery-Laser-
KTP pulse dye laser






Laryngeal Web 

Definition
a membrane in the larynx due to incomplete re-canalization of the larynx

Epidemiology
rare, 75% glottic level, mostly anterior webs


Aetiology
Failure of recanalisation of the larynx during embryonic development-10th week
Assoicated with CATCH22/ VCF syndrome ( Ch 22 q 11)


Clinical
No sy , hoarse/ apnea
Syndrome features


Examination
Flexible scope


Investigation
Lat Neck XR-sail sign
Flexi scope
L&B
All undergo genetics- CATCH 22, Cardiac evaluation


Treatment
Mild can observe
Rest Surgery






Bilateral Vocal Cord Palsy 


EPIDEMIOLOGY
another common cause of neonatal stridor

PATHOPHYSIOLOGY
Idiopathic
Birth trauma/ Hypoxia/ intubation trauma 
CNS
peripheral neurologic diseases
Thoracic-diseases or iatrogenic


CLINICAL
stridor-inspiratory or biphasic, with a high-pitched musical quality
weak cry


EXAMINATION  
flexible laryngoscopy: paediatric ENT unit
vocal fold fixation vs neurogenic vocal -direct laryngoscopy is performed under GA and the glottis palpated


INVESTIGATIONS
laryngeal EMG → fixation vs paralysis
MRI - ? Arnold-Chiari malformation or other intracranial cause
If idiopathic congenital bilateral vocal cord paralysis is present → genetics consultation 


TREATMENT Unilateral
Unilateral-Non surgical
Manage airway patency 
    usually not an issue
Manage aspiration & feeding, speech pathology, barium sallow 


TREATMENT Bilateral vocal cord paralysis
Surgery unless stable







Recurrent Respiratory Papillomatosis 

Definition

The most common benign neoplasm of the larynx caused by the HPV virus

Epidemiology

Juvenile< 5yrs of age
Incidence 4/100,000
M=F
Adults20-40s
M:F  3:2
Incidence-2/100,000
more in poor 
Overall prevalence of HPV in women nearly 33% and the most common STD


Aetiology

Vertical transmission children, 
adults, sexual or reactivation


Presenting symptoms

3 yrs age mean
Sy of recurrent croup dysphonia, barking type cough, 
Failure to thrive
Stridor
Dysphagia
Chest infections


Treatment

Vaccination


Gardisil- Quadrivalent vaccine against HPV 6, 11, 16, and 18 or placebo to evaluate prevention of high-grade cervical lesions

Expected reduced prevalence of the Virus in vaccinated women hence likely lower vertical transmission

HPV vaccine may play a role in the prevention of RRP in children and newborns. 

Surgery

Goal is to get a balance between going too often and letting tumor burden increase

Microdebrider
Laser



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