Cyanosis resident survival guide (pediatrics)

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Usman Ali Akbar, M.B.B.S.[2]

Synonyms and keywords: Cyanosis approach in children, Cyanosis workup pediatrics, Cyanosis management in newborn, Approach to blue discoloration of skin in infants, Hypoxemia approach in children, Hypoxia approach in children

Cyanosis resident survival guide (pediatrics) Microchapters
Overview
Causes
FIRE
Diagnosis
Treatment
Do's
Don'ts

Overview

This section provides a short and straight to the point overview of the disease or symptom. The first sentence of the overview must contain the name of the disease.

Causes

Life-Threatening Causes	Pulmonary Causes	Congenital Cardiac Conditions	Hematological Causes	Peripheral Cyanosis
Decreased inspired FiO2 Smoke inhalation Exposure to toxic gases	Asthma Bronchiolitis Bronchopulmonary dysplasia Cystic fibrosis Empyema Hyaline membrane disease Pneumonia	Atrioventricular canal defect Epstein anomaly Hypoplastic left heart syndrome Pulmonary atresia Pulmonic stenosis Tetralogy of Fallot Total anomalous pulmonary venous drainage Transposition of the great vessels Truncus arteriosus	Methemoglobinemia Polycythemia	Acrocyanosis Cold exposure Shock Vasomotor instability Hypothermia Arterial or venous obstruction
Upper airway obstruction Tracheitis Croup Epiglottitis Foreign body aspiration	Pulmonary vascular disorders Pulmonary edema Pulmonary embolism Pulmonary hemorrhage Pulmonary hypertension
	Other Causes Flail chest Hemothorax Open pneumothorax Pneumothorax

FIRE: Focused Initial Rapid Evaluation

Patients presenting to the emergency department with cyanosis and respiratory distress require emergency supplementation of O2, use of pulse-oximetry and airway, breathing, and circulation support.

• A Focused Initial Rapid Evaluation (FIRE) should be performed to identify the patients in need of immediate intervention].

Patient present with cyanosis

No Respiratory Distress

Respiratory Distress

Peripheral Cyanosis

Central

Differential Cyanosis

No Obstruction

Obstruction

Reassurance & Warming

Hypoxia Test

Cardiac Evaulation

Hyperoxia Test

Give O2 and Positive Pressure Ventilation

PaO2<100

PaO2 100-150

PaO2 <100

PaO2 100-150

PaO2 >150

ENT Evaluation

Cardiac Evaluation

Persistent Pulmonary hypertension of newborn ,Cardiac Evaluation

Cardiac Cause

Persistent Pulmonary hypertension of newborn

Respiratory management, Give O2 and positive pressure Ventilation

Complete Diagnostic Approach

Shown below is an algorithm summarizing the diagnosis of [[disease name]] according the the [...] guidelines.

Patient presents with cyanosis

History Age: Certain conditions are more common in neonates than the older children such as cyanotic heart conditions and polycythemia Presence/Absence of Fever: A history of fever shows the presence of existing infection History of Trauma : Chest wall trauma can cause central cyanosis. Exposure to toxic gases: Exposure to certain gases and smoke can cause cyanosis. Nitrates containing food can also cause methemoglobinemia. Medication Induced Cyanosis: Certain medications such as amiodarone can be a cause of cyanosis. Co-existing Pulmonary Pathology: Conditions such as asthma or bronchopulmonary dysplasia can result in cyanosis. History of Congenital Heart Disease: Central cyanosis can be caused by a number of cyanotic congenital cardiac conditions and may result in shock. History of Neurological Disease:Respiratory depression due to drug or toxin ingestion, CNS lesions, a history of seizures, breath-holding spells in infants, and neuromuscular disease should be asked and rule out while pursuing causes of cyanosis.

Pysical Examination Fever :An intrinsic pulmonary pathology such as pneumonia can cause fever and cyanosis in children. Pulmonary Examination : Pulmonary examination may elicit flaring, grunting, retractions and respiratory distress. Tachypnea is an important finding in patient with respiratory cause of cyanosis. An upper airway obstruction can cause stridor. Pulmonary edema can cause rales or crackles.Clear lung sounds may be associated with cardiac conditions which cause cyanosis. Injury to lung may present with abnormal chest wall movement, sucking chest wound, ecchymosis on chest wall, tracheal deviation, subcutaneous crepitus and abnormal breathing sounds. Cardiac Examination: Look for cardiac murmur.A loud or single second heart sound can be present in cyanotic cardiac conditions or pulmonary hypertension. Skin Examination : Cold exposure can cause peripheral vasoconstriction causing cyanosis whereas central cyanosis due to methemoglobinemia may present with gray appearing skin.

Diagnostic Studies CBC with differential : An elevated white blood cell may indicate infection. Arterial Blood Gases: PaO2>150 mmHg may indicate Pulmonary Parenchymal Disease PaO<150 and Normal PCO2 shows the presence of Intra or Extra-Pulmonary Right to Left Shunts. PaO2>150 mmHg and elevated PCO2 may cause central hyperventilation. PaO2 <150 mm Hg, usually <50 mmHg and normal PCO2 may include transposition physiology. Normal PaO2 and PCO2 may be due to hemoglobin disorders. Hematocrit : Polycythemia or elevated hematocrit may be present in plethoric children. Chest X-ray:Chest X-ray may show findings in pulmonary pathology. Egg-on-end appearance and pulmonary venous congestion may be present in transposition of great arteries. ECG : It is helpful in congenital cardiac conditions. This can be sometimes augmented with echocardiography to specifically identify cardiac pathology. Methemglobinemia serum level: It is measured in cyanotic patient switch normal PaO2 with excluded cardiac pathology. Difference between calculated oxygen saturation on ABGs analysis and direct measurement by co-oximetry may indicate methemoglobinemia. Differential Saturation(pre-ductal vs post-ductal): Its is absent in pulmonary parenchymal disease and present if there is right to left shunt at ductus arteriosus.Post ductal differential saturation is present in transposition of great arteries.

Treatment

Shown below is an algorithm summarizing the treatment of [[disease name]] according the the [...] guidelines.

Treatment Depends upon the etiology of cyanosis.

Respiratory Compromise

No Respiratory Compromise

*An adequate airway should be established and supplemental oxygen is given. Continuous positive airway pressure (CPAP) or intubation for positive pressure ventilation can be done for infants with respiratory distress and carbon dioxide retention. If there is airway obstruction prone positioning or oral airway is established to relieve cyanosis.

Depending upon etiology Sepsis : Broad-spectrum antibiotics should be initiated such as ampicillin and gentamicin. Blood cultures should be obtained to identify the causative agent. Neonatal Hypoglycemia : Adequate blood glucose should be maintained in range of >45 to 50 mg/dl Cyanotic Congenital Cardiac Conditions should be approached with proper pediatric consultation. Prostaglandin E1 should be infused at 0.01-0.05mcg/kg/min.

TGA, TAPVR ,Truncus arteriosus

TOF

Ebstein anomaly

Hypoplastic left heart syndrome

Sepsis, shock, low cardiac output state, cold exposure, metabolic disorder, polycythemia

Eisenmenger syndrome with pulmonary hypertension

Methemoglobinemia

Infusion of Prostaglandin, Diuretic therapy,surgery

Infusion of Prostaglandin for keeping patency of ductus arteriosus, infusion of vasodilator for reduced systemic resistance, mechanical ventilation in shock state and imposing hypercapnia and alveolar hypoxia for increased pulmonary resistance

Tricuspid valve repair

Hypoplastic left heart syndrome

Treatment of underlying disorder

Phosphodiesterase-5 inhibitor (sildenafil, tadalafil), Endothelin receptor antagonist (bosentan,macitentan, ambrisentan)

Infusion of Methylenblue,dextrose,N-acetyl cystein

Do's

• Quickly think about hypoplastic left heart syndrome In infants with sudden onset of shock , collapse and severe academia in the first week of life, as well as neonate sepsis and metabolic disorders.
• In ebstein anomaly repair of tricuspid valve indicates if there is :Cyanosis, Right-side heart failure, Poor functional capacity, Paradoxical emboli.
• In differential cyanosis if oxygen saturation of right arm is more than legs and improves with O2 supplemental therapy, think about severe coarctation of aorta, aortic arch interruption, primary pulmonary hypertension.
• In the presence of central cyanosis + hemolytic anemia (jundic,heinze body,fragment RBC)+ renal failure consider about methemoglobinemia.
• Quickly correct dehydration and any distress in infants with cyanotic tet spell in Tetralogy of Fallot to maintain pulmonary blood flow through atretic pulmonary artery and reducing right to left shunt through VSD.

• Think about paradoxical embolism and do a Brain CT scan in the precence of new neurologic symptoms in cyanotic congenital heart disease because of passing the emboli from right to left shunt and hyperviscosity leading to thrombosis.

Don'ts

• Cyanotic congenital heart diseases that pulmonary congestion is independent on patent ductus arteriosus(PDA) do not worsen with dehydration include :
• Transposition of great arteries(TGA)
• Truncus arteriosus(TA)
• Total anomalous pulmonary venous connection(TAPVR)