דלקות ריאה חוזרות דר. דוד שוסיוב היחידה למחלות ריאה בילדים בית חולים הדסה ירושלים * חשוב ! בבחינה

Definition Recurrent pneumonia is defined as at least 2 pneumonia episodes in a 1-year period or at least 3 during a lifetime. * חשוב ! בבחינה

Recurrent Pneumonia Recurrent pneumonia constitutes a threat to the integrity and normal development of the respiratory system Every possible effort should be performed to make the correct diagnosis and treatment

Recurrent Pneumonia localized foreign body aspiration congenital anomalies asthma mucoid impaction diffuse Cystic Fibrosis (CF) Immunodeficiency Primary Ciliary Dyskinesia (PCD) Aspiration syndromes Vasculitis * * חשוב ! בבחינה

Recurrent Pneumonia localized foreign body aspiration congenital anomalies asthma mucoid impaction

Foreign Body Aspiration in Children Aspiration of a foreign body to the airway can endanger life immediately, or in the long term due to chronic lung infection and bronchiectasis the main goals of the treatment of foreign bodies are: ◦prevention ◦early diagnosis ◦Intervention

Foreign Body Aspiration in Children The diagnosis is some times very difficult as there is often no: ◦ leading history ◦typical physical examination ◦radiographic findings. *

Rigid Bronchoscopy

Foreign Body Aspiration in Children 8 months old infant developed a sudden cough while crawling on the floor. examination revealed diminished breath sounds over the right lung

Foreign Body Aspiration in Children A 14 months old girl choked during eating chicken soup on examination she was tachypneic and dispneic. breaths sounds were normal over both lungs chest radiograph was normal fluoroscopy was interpreted as normal

Due to her critical condition, rigid bronchoscopy was performed demonstrating a hollow chicken bone in the left main bronchus Foreign Body Aspiration in Children

Revision of the fluoroscopy film revealed the chicken bone located in the left main bronchus

Foreign Body Aspiration in Children A 3 year old girl was transferred from another hospital due to a severe asthma attack which did not respond to high doses of IV steroids and inhaled bronchodilators Examination revealed normal breaths sound while lying supine, however in the seating position she developed severe respiratory distress

Foreign Body Aspiration in Children Rigid bronchoscopy demonstrated a swollen watermelon seed sliding along the trachea

Inflammatory pseudo-polyp A 3 years old boy was referred to evaluate recurrent RLL pneumonia Bronchoscopy revealed a polypoid mass obstructing the Rt. main bronchus

Inflammatory pseudo-polyp After removing the polypoid mass, a small piece of almond was seen

Rigid bronchoscopies performed for suspected foreign body aspiration in children - results of 173 procedures

Rigid bronchoscopies performed for suspected foreign body aspiration in children *

Rigid bronchoscopies performed for suspected foreign body aspiration in children - history

Rigid bronchoscopies performed for suspected foreign body aspiration physical examination

Rigid bronchoscopies performed for suspected foreign body aspiration in children - Chest X ray

Rigid bronchoscopies performed for suspected foreign body aspiration in children -Yield of 86 fluoroscopies P=0.034

Indications for Bronchoscopy for Suspected Foreign Aspiration The prediction of foreign body aspiration is best achieved by: 1. history 2. physical examination 3. chest radiography *

Recurrent Pneumonia localized foreign body aspiration congenital anomalies asthma mucoid impaction

Recurrent Pneumonia Congenital anomalies distorted structure ◦sequestration ◦cystic adenomatoid malformation (CCAM) airway obstruction Stenosis Malacia external compression vascular rings, slings cysts

Cong Cystic Adenomatoid Malformation - CCAM

Pulmonary sequestration Angiography with systemic circulation

Bronchomalacia - LMB

Bronchial stenosis

Normal aortic arch ?Double aortic arch

Recurrent Pneumonia localized foreign body aspiration congenital anomalies asthma mucoid impaction

Asthma Right middle lobe syndrome * !

Recurrent Pneumonia localized foreign body aspiration congenital anomalies asthma mucoid impaction

Mucoid impaction : bronchial cast eosinophils dehydrated secertions Associated with : Post heart surgery * cystic fibrosis asthma allergic bronchopulmonary aspergillosis (ABPA)

Recurrent Pneumonia diffuse cystic fibrosis immunodeficiencies primary ciliary dyskinesia aspiration syndromes

Recurrent Pneumonia diffuse cystic fibrosis immunodeficiencies primary ciliary dyskinesia aspiration syndromes

Immunodeficiencies Agammaglobulinemia - x linked IgG subclass deficiency Complement deficiency Neutrophil abnormalities: ◦Schwachman-Diamond syndrome ◦Chronic granulomatous diseae (CGD) ◦Chediac-Higashi syndrome ◦Job’s syndrome ( תסמונת איוב ) ◦cyclic neutropenia ◦drug induced neutropenia

Recurrent Pneumonia diffuse cystic fibrosis immunodeficiencies primary ciliary dyskinesia (PCD) !! aspiration syndromes

Primary ciliary dyskinesia chronic sinusitis chronic and rec. otitis media recurrent pneumonia & bronchiectasis situs inversus (50%) male infertility (50%) Females some problems Low Nasal Nitric Oxide (NO)

PCD maxillary sinusitis

PCD maxillary sinusitis

Normal cilium

PCD

dysorientation of central tubules

Recurrent Pneumonia diffuse cystic fibrosis immunodeficiencies primary ciliary dyskinesia aspiration syndromes

Aspiration from above choanal stenosiscleft palate macroglossialaryngotracheal cleft micrognathiaTEF-H type vascular ring congenital anomalies

Tracheo-esophageal fistula

Aspiration from above

Familial Dysautonomia (FD) Riley-Day Syndrome Aspiration ◦Eating and drinking ◦GE reflux and vomiting (FD crisis) ◦Dysautonomia- Fever, high and low blood pressure etc.

Case Presentation,CP aspiration from above

Gastro-Esophageal Reflux and Aspiration one of the major causes for lung disease in infants and children diagnosis is often delayed due to: ◦unawareness ◦low sensitivity and specificity of diagnostic procedures

Gastro-Esophageal Reflux Disease (GERD) symptoms respiratory chronic cough wheezing recurrent pneumonia apnea stridor hoarseness gastrointestinal Vomiting irritability dysphagia anemia failure to thrive

Gastro-Esophageal Reflux barium swallow

Gastro-Esophageal Reflux 24 hr esophageal pH monitoring

Gastric scintigram (milk scan)

Recurrent Pneumonia diagnosis history physical findings ◦clubbing ◦burrel chest ◦cracels Chest x-ray Bronchoscopy HRCT Bronchography (?) Sweat Test Immunoglobulins IgE PFT with challenge Nitric Oxide (exhaled and Nasal) Arterial Blood Gas SaO2 * ! ?

* ! Clubbing - התאלות

Recurrent pneumonia LLL

Bronchiectasis - CT

Bronchiectasis - bronchography

Flexible bronchoscopy

Rigid bronchoscopy

PCP CMV Aspergillus fumigatus mucor

Cndida albicans

BAL – lipid laden alveolar macrophages *

BAL – Hemosiderin laden macrophages * ! Pulmonary hemorrhage Pulmonary Hemosiderosis Primary and seconday Hypoxia Crackles (crepitation) Pulm Infiltration in CXR (ground glass appearance) Decreased Hb during exacerbations Higher DLCO in PFT

Charcoal tracheal instillation

Recurrent pneumonia treatment Identify and treat primary cause Physiotherapy and postural drainage Hypertonic Saline Inhalation – to increase mucociliary clearance DNAase inhalation (Pulmozyme) Antibiotics (PO, IV, Inhalations) Surgery

תודה רבה ברוכים הבאים למשחק האמיתי