Cyanosis resident survival guide (pediatrics)

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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

Cyanosis can be defined as bluish discoloration of skin and mucosa and can also be a manifestation of oxygen desaturation of arterial or capillary blood. Cyanosis ,hypoxemia and hypoxia should be differentiated and can occur independently. The causes of cyanosis in newborn range from congenital cardiac conditions to life threatening conditions such as exposure to toxic gases or infections leading to sepsis. The management of cyanosis depends upon etiology and emergent cases with respiratory distress need rapid evaluation and response with immediate establishment of airway access and oxygen support.

Causes

  • The causes of cyanosis are diverse and are listed below.[1][2]
Life-Threatening Causes Pulmonary Causes Congenital Cardiac Conditions Hematological Causes Peripheral Cyanosis
Decreased inspired FiO2
Upper airway obstruction

Pulmonary vascular disorders

Other Causes

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.[3]
 
 
 
 
 
 
 
 
 
Patient present with cyanosis
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
No Respiratory Distress
 
 
 
 
 
 
 
 
 
 
 
 
Respiratory Distress
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Peripheral Cyanosis
 
Central cyanosis
 
Differential Cyanosis
 
 
 
No Obstruction
 
 
 
Obstruction
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Reassurance & Warming
 
Hypoxia Test
 
Cardiac Evaulation
 
 
 
Hypoxia 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 cyanosis in newborn according the the American Academy of Neonatology guidelines.

 
 
 
Patient presents with cyanosis
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
History
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Pysical Examination
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
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 vessels.
 
 

Treatment

Shown below is an algorithm summarizing the treatment of cyanosis.[3][4]

 
 
 
 
 
 
 
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 [5]
     
    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[6]
     
    Hypoplastic left heart syndrome
     
    Treatment of underlying disorder
     
     
    Phosphodiesterase-5 inhibitor (sildenafil, tadalafil), Endothelin receptor antagonist (bosentan,macitentan, ambrisentan)[7]
     
     
    Infusion of Methylenblue,dextrose,N-acetyl cystein
     
     

    Do's

    Don'ts

    References

    1. McMullen, SM; Patrick, W (2013). "Cyanosis". The American journal of medicine. 126 (3): 210–2. doi:10.1016/j.amjmed.2012.11.004. ISSN 0002-9343. PMID 23410559.
    2. Sasidharan, Ponthenkandath (2004). "An approach to diagnosis and management of cyanosis and tachypnea in term infants". Pediatric clinics of North America. Elsevier BV. 51 (4): 999–1021. doi:10.1016/j.pcl.2004.03.010. ISSN 0031-3955. PMID 15275985.
    3. 3.0 3.1 Dasgupta, Soham; Bhargava, Vidit; Huff, Monica; Jiwani, Amyn K.; Aly, Ashraf M. (2016-09-30). "Evaluation of The Cyanotic Newborn: Part I—A Neonatologist's Perspective". NeoReviews. American Academy of Pediatrics (AAP). 17 (10): e598–e604. doi:10.1542/neo.17-10-e598. ISSN 1526-9906.
    4. "Congenital Heart Disease". NCBI Bookshelf. 2010-07-22. Retrieved 2020-10-13.
    5. Rao, P. Syamasundar (2013). "Consensus on Timing of Intervention for Common Congenital Heart Diseases: Part II - Cyanotic Heart Defects". The Indian Journal of Pediatrics. 80 (8): 663–674. doi:10.1007/s12098-013-1039-2. ISSN 0019-5456.
    6. 6.0 6.1 Holst KA, Connolly HM, Dearani JA (2019). "Ebstein's Anomaly". Methodist Debakey Cardiovasc J. 15 (2): 138–144. doi:10.14797/mdcj-15-2-138. PMC 6668741 Check |pmc= value (help). PMID 31384377.
    7. de Campos F, Benvenuti LA (2017). "Eisenmenger syndrome". Autops Case Rep. 7 (1): 5–7. doi:10.4322/acr.2017.006. PMC 5436914. PMID 28536680. Vancouver style error: initials (help)
    8. Gobergs R, Salputra E, Lubaua I (2016). "Hypoplastic left heart syndrome: a review". Acta Med Litu. 23 (2): 86–98. doi:10.6001/actamedica.v23i2.3325. PMC 5088741. PMID 28356795.
    9. O’Brien, Patricia; Marshall, Audrey C. (2014). "Tetralogy of Fallot". Circulation. 130 (4). doi:10.1161/CIRCULATIONAHA.113.005547. ISSN 0009-7322.
    10. . doi:10.1161/STROKEAHA.116.012882Stroke. Missing or empty |title= (help)