Cyanosis resident survival guide (pediatrics): Difference between revisions

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| [[File:Siren.gif|30px|link=Cyanosis resident survival guide (pediatrics)]]|| <br> || <br>
| [[Cyanosis resident survival guide (pediatrics)|'''Resident'''<br>'''Survival'''<br>'''Guide''']]
|}


{{CMG}} {{AE}}  
{{CMG}} {{AE}} {{Usman Ali Akbar}}


{{SK}} 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
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==Overview==
==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.
[[Cyanosis]] can be defined as [[Bluish discoloration of the skin|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 a [[newborn]] range from [[congenital]] [[cardiac]] [[conditions]] to life-threatening [[conditions]] such as [[Exposure assessment|exposure]] to [[toxic]] [[gases]] or [[infections]] leading to [[sepsis]]. The management of [[cyanosis]] depends upon the [[etiology]] and [[Emergency|emergent]] cases with [[respiratory distress]] need rapid evaluation and [[Response variable|response]] with immediate establishment of [[Airway|airway access]] and [[oxygen]] [[support]].


==Causes==
==Causes==


*
*The causes of [[cyanosis]] are diverse and are listed below.<ref name="McMullen Patrick 2013 pp. 210–2">{{cite journal | last=McMullen | first=SM | last2=Patrick | first2=W | title=Cyanosis. | journal=The American journal of medicine | volume=126 | issue=3 | year=2013 | issn=0002-9343 | pmid=23410559 | doi=10.1016/j.amjmed.2012.11.004 | pages=210–2}}</ref><ref name="Sasidharan 2004 pp. 999–1021">{{cite journal | last=Sasidharan | first=Ponthenkandath | title=An approach to diagnosis and management of cyanosis and tachypnea in term infants | journal=Pediatric clinics of North America | publisher=Elsevier BV | volume=51 | issue=4 | year=2004 | issn=0031-3955 | pmid=15275985 | doi=10.1016/j.pcl.2004.03.010 | pages=999–1021}}</ref>


{| class="wikitable"
{| class="wikitable"
|+
|+
!style="background:#4479BA; color: #FFFFFF;" align="center" + |Life-Threatening Causes  
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Life-Threatening Causes
!style="background:#4479BA; color: #FFFFFF;" align="center" + |Pulmonary Causes
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Pulmonary Causes
!style="background:#4479BA; color: #FFFFFF;" align="center" + |Congenital Cardiac Conditions
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Congenital Cardiac Conditions
!style="background:#4479BA; color: #FFFFFF;" align="center" + |Hematological Causes
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Hematological Causes
!style="background:#4479BA; color: #FFFFFF;" align="center" + |Peripheral Cyanosis
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Peripheral Cyanosis
|-
|-
|'''Decreased inspired FiO2'''
|'''Decreased inspired FiO2'''
*[[Smoke inhalation]]
*[[Smoke inhalation]]
*Exposure to toxic gases
*[[Exposure effect|Exposure]] to [[toxic]] [[gases]]
|
|
*[[Asthma]]
*[[Asthma]]
Line 48: Line 54:
*[[Hyaline membrane disease]]
*[[Hyaline membrane disease]]
*[[Pneumonia]]
*[[Pneumonia]]
|
| rowspan="3" |
*[[Atrioventricular canal defect (patient information)|Atrioventricular canal defect]]
*[[Atrioventricular canal defect (patient information)|Atrioventricular canal defect]]
*[[Epstein's syndrome|Epstein anomaly]]
*[[Epstein's syndrome|Epstein anomaly]]
Line 58: Line 64:
*[[Transposition of the great vessels]]
*[[Transposition of the great vessels]]
*[[Truncus arteriosus]]
*[[Truncus arteriosus]]
|
| rowspan="3" |
*[[Methemoglobinemia]]
*[[Methemoglobinemia]]
*[[Polycythemia]]
*[[Polycythemia]]
|
| rowspan="3" |
*[[Acrocyanosis]]
*[[Acrocyanosis]]
*Cold exposure
*[[Cold exposure]]
*[[Shock]]
*[[Shock]]
*Vasomotor instability
*[[Vasomotor|Vasomotor instability]]
*Hypothermia
*[[Hypothermia]]
*Arterial or venous obstruction  
*[[Arterial obstruction|Arterial]] or [[Venous|venous obstruction]]  
|-
|-
|'''Upper airway obstruction'''
| rowspan="2" |'''Upper airway obstruction'''
*[[Tracheitis]]
*[[Tracheitis]]
*[[Croup]]
*[[Croup]]
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*[[Foreign body aspiration]] <br />
*[[Foreign body aspiration]] <br />
|
|
|'''Pulmonary vascular disorders'''
'''Pulmonary vascular disorders'''
 
*[[Pulmonary edema]]
*[[Pulmonary edema]]
*[[Pulmonary embolism]]
*[[Pulmonary embolism]]
*[[Pulmonary hemorrhage]]
*[[Pulmonary hemorrhage]]
*[[Pulmonary hypertension]]
*[[Pulmonary hypertension]]
|
|
|-
|-
|
|'''Other Causes'''
|
|External compression-tumor etc.
 
*[[Flail chest]]
*[[Flail chest]]
*[[Hemothorax]]
*[[Hemothorax]]
*[[Pneumothorax|Open pneumothorax]]
*[[Pneumothorax|Open pneumothorax]]
*[[Pneumothorax]]
*[[Pneumothorax]]
|
|
|}
|}


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==FIRE: Focused Initial Rapid Evaluation==
==FIRE: Focused Initial Rapid Evaluation==
*[[Patients]] presenting to the [[emergency department]] with [[cyanosis]] and [[respiratory distress]] require [[emergency]] [[Supplements|supplementation]] of [[oxygen]], use of [[pulse oximetry]] and [[airway]], [[breathing]], and [[Circulation|circulation support]].
*A Focused Initial Rapid Evaluation (FIRE) should be performed to identify the [[patients]] in need of immediate [[intervention]].<ref name="Dasgupta Bhargava Huff Jiwani pp. e598–e604">{{cite journal | last=Dasgupta | first=Soham | last2=Bhargava | first2=Vidit | last3=Huff | first3=Monica | last4=Jiwani | first4=Amyn K. | last5=Aly | first5=Ashraf M. | title=Evaluation of The Cyanotic Newborn: Part I—A Neonatologist’s Perspective | journal=NeoReviews | publisher=American Academy of Pediatrics (AAP) | volume=17 | issue=10 | date=2016-09-30 | issn=1526-9906 | doi=10.1542/neo.17-10-e598 | pages=e598–e604}}</ref>
{{Family tree/start}}
{{Family tree | | | | | | | | | | A01 | | | |A01= [[Patient]] presents with [[cyanosis]]}}
{{Family tree | | | |,|-|-|-|-|-|-|^|-|-|-|-|-|-|-|.| }}
{{Family tree |boxstyle=background: #FA8072; color: #F8F8FF;| | | C01 | | | | | | | | | | | | | C02 |C01= No [[Respiratory distress|Respiratory Distress]] | C02= [[Respiratory distress|Respiratory Distress]]}}
{{familytree | |,|-|-|+|-|-|-|.| | | | | | | |,|-|^|-|-|.| }}
{{familytree |boxstyle=background: #FA8072; color: #F8F8FF;| F01 | | F02 | | F03 | | | | F04 | | | | F05 |F01=[[Peripheral cyanosis|Peripheral Cyanosis]]|F02=[[Central cyanosis]]|F03= [[Differential Cyanosis]]|F04=No [[Obstruction]]|F05=[[Obstruction]]}}
{{familytree | |!| | | |!| | | |!| | | | | |!| | | | | |!| |}}
{{familytree |boxstyle=background: #FA8072; color: #F8F8FF;| F01 | | F02 | |F03 | | | | F04 | | | |  F05 |F01=[[Reassurance]] and Warming|F02= [[Hypoxia]] Test |F03= [[Cardiac]] Evaulation |F04= [[Hypoxia]] Test |F05= Give [[oxygen]] and [[Positive pressure ventilation|Positive Pressure Ventilation]]}}
{{Family tree | | |,|-|^|-|.| | | |,|-|-|-|+|-|-|-|.| | |!| }}
{{familytree | | F01 | | F02 | |F03 | |F04 | |F05 | |F06 |F01=PaO2<100|F02=PaO2 100-150|F03= PaO2 <100 |F04= PaO2 100-150 |F05= PaO2 >150 |F06=ENT Evaluation }}
{{familytree | |!| | | |!| | | |!| | | | | |!| | |!| |}}
{{familytree | | F01 | | F02 | |F03 | |F04 | |F05 |F01=[[Cardiac]] Evaluation|F02=
Persistent [[pulmonary hypertension]] of [[newborn]], [[Cardiac]] Evaluation
|F03= [[Cardiac]] [[Cause]]|F04=Persistent [[pulmonary hypertension]] of [[newborn]] |F05=
[[Respiratory]] management,
Give [[oxygen]] and [[positive pressure ventilation|positive pressure Ventilation]]
}}
{{Family tree/end}}


==Complete Diagnostic Approach==
==Complete Diagnostic Approach==
Shown below is an algorithm summarizing the diagnosis of <nowiki>[[disease name]]</nowiki> according the the [...] guidelines.
 
*Shown below is an algorithm summarizing the [[diagnosis]] of [[cyanosis in newborns]] according to the American Academy of [[Neonatology]] guidelines:
{{familytree/start |summary=PE diagnosis Algorithm.}}
{{familytree/start |summary=PE diagnosis Algorithm.}}
{{familytree | | | | A01 | | | A01= }}
{{familytree | | | | A01 | | | A01= [[Patient]] presents with [[cyanosis]]}}
{{familytree | | | | |!| | | | }}
{{familytree | | | | |!| | | | }}
{{familytree | | | | B01 | | | B01= }}
{{familytree | | | | B01 | | | B01=  
{{familytree | | |,|-|^|-|.| | }}
<big>'''History'''</big>  <br>
{{familytree | | C01 | | C02 | C01= | C02= }}
*'''[[Age]]: '''Certain [[conditions]] are more common in [[neonates]] as compared to older [[children]] such as [[cyanotic]] [[heart]] [[conditions]] and [[polycythemia]].<br>
*'''Presence/Absence of [[Fever]]: '''A history of [[fever]] shows the presence of existing [[infection]].<br>
*'''History of [[Trauma]]: '''[[Chest wall]] [[trauma]] can cause [[central cyanosis]].<br>
*'''Exposure to [[toxic]] [[gases]]: '''Exposure to certain [[gases]] and [[smoke]] can cause [[cyanosis]]. [[Nitrates]] containing [[food]] can also cause [[methemoglobinemia]].<br>
*'''[[Medication]] Induced [[Cyanosis]]: '''Certain [[medications]] such as [[amiodarone]] can be a cause of [[cyanosis]].<br>
*'''Co-existing [[Pulmonary]] [[Pathology]]: '''[[Conditions]] such as [[asthma]] or [[bronchopulmonary dysplasia]] can result in [[cyanosis]].<br>
*'''History of [[Congenital Heart Disease]]: '''[[Central cyanosis]] can be caused by a number of [[cyanotic]] [[congenital]] [[cardiac]] [[conditions]] and may result in [[shock]].<br>
*'''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]].<br>
}}
{{familytree | | | | |!| | | | }}
{{familytree | | | | B01 | | | B01=<big>'''[[Physical Examination]]'''</big>  <br>
*'''[[Fever]]: '''An [[intrinsic]] [[pulmonary]] [[pathology]] such as [[pneumonia]] can cause [[fever]] and [[cyanosis]] in [[children]].<br>
*'''[[Pulmonary]] Examination: '''[[Pulmonary]] examination may elicit [[flaring]], [[grunting]], [[retractions]], and [[respiratory distress]]. [[Tachypnea]] is an important finding in [[patients]] with the [[respiratory]] [[causes]] 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]], [[crepitus|subcutaneous crepitus]] and [[abnormal]] [[breathing sounds]].<br>
*'''[[Cardiac]] Examination: '''Look for [[cardiac]] [[murmur]]. A loud or single [[second heart sound]] can be present in [[cyanotic cardiac]] [[conditions]] or [[pulmonary hypertension]].<br>
*'''[[Skin]] Examination: '''[[Cold]] exposure can cause [[peripheral]] [[vasoconstriction]] causing [[cyanosis]] whereas [[central cyanosis]] due to [[methemoglobinemia]] may present with [[gray]] appearing [[skin]].<br>
}}
{{familytree | | | | |!| | | | }}
{{familytree | | | | B01 | | | B01=<big>'''[[Diagnostic]] Studies'''</big>  <br>
*'''[[CBC]] with differential: '''An elevated [[white blood cell]] may indicate [[infection]].<br>
*'''Arterial Blood Gases:'''
*PaO2>150 mmHg may indicate [[Pulmonary]] [[Parenchymal]] [[Disease]].<br>
*PaO<150 and Normal PCO2 shows the presence of Intra or Extra-Pulmonary [[Shunt|Right to Left Shunts]].<br>
*PaO2>150 mmHg and elevated PCO2 may cause [[hyperventilation|central hyperventilation]].<br>
*PaO2 <150 mm Hg, usually <50 mmHg and normal PCO2 may include [[transposition]] [[physiology]].<br>
*Normal PaO2 and PCO2 may be due to [[hemoglobin]] [[disorders]].<br>
*'''[[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 congestion|pulmonary venous congestion]] may be present in the [[transposition of great arteries]].<br>
*'''[[ECG]]: '''It is helpful in [[congenital]] [[cardiac]] [[conditions]]. This can be sometimes augmented with [[echocardiography]] to specifically identify [[cardiac]] [[pathology]].<br>
*'''[[Methemoglobinemia]] [[serum]] level: '''It is measured in a [[cyanotic]] [[patient]] having [[normal]] PaO2 with excluded [[cardiac]] [[pathology]]. Difference between calculated [[oxygen saturation]] on ABGs [[analysis]] and direct measurement by co-oximetry may indicate [[methemoglobinemia]].<br>
*'''Differential [[Saturation]] (pre-ductal vs post-ductal): '''It 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 the [[transposition of great vessels]].<br>
}}


{{familytree/end}}
{{familytree/end}}


==Treatment==
==Treatment==
Shown below is an algorithm summarizing the treatment of <nowiki>[[disease name]]</nowiki> according the the [...] guidelines.
*Shown below is an algorithm summarizing the [[treatment]] of [[cyanosis]]:<ref name="Dasgupta Bhargava Huff Jiwani pp. e598–e604">{{cite journal | last=Dasgupta | first=Soham | last2=Bhargava | first2=Vidit | last3=Huff | first3=Monica | last4=Jiwani | first4=Amyn K. | last5=Aly | first5=Ashraf M. | title=Evaluation of The Cyanotic Newborn: Part I—A Neonatologist’s Perspective | journal=NeoReviews | publisher=American Academy of Pediatrics (AAP) | volume=17 | issue=10 | date=2016-09-30 | issn=1526-9906 | doi=10.1542/neo.17-10-e598 | pages=e598–e604}}</ref><ref name="NCBI Bookshelf 2010">{{cite web | title=Congenital Heart Disease | website=NCBI Bookshelf | date=2010-07-22 | url=https://www.ncbi.nlm.nih.gov/books/NBK209965/ | access-date=2020-10-13}}</ref>
{{familytree/start |summary=PE diagnosis Algorithm.}}
{{familytree/start |summary=PE diagnosis Algorithm.}}
{{familytree | | | | | | | | A01 |A01= }}  
{{familytree | | | | | | | | A01 |A01= [[Treatment]] depends upon the [[etiology]] of [[cyanosis]]}}  
{{familytree | | | | |,|-|-|-|^|-|-|-|-|.| | | }}
{{familytree | | | | |,|-|-|-|^|-|-|-|-|.| | | }}
{{familytree | | | B01 | | | | | | | | B02 | | |B01= |B02= }}
{{familytree | | | B01 | | | | | | | | B02 | | |B01=[[Respiratory]] Compromise |B02=No [[Respiratory]] Compromise }}
{{familytree | | | |!| | | | | | | | | |!| }}
{{familytree | | | |!| | | | | | | | | |!| }}
{{familytree | | | C01 | | | | | | | | |!| |C01= }}
{{familytree | | | C01 | | | | | | | |C02| |C01=
{{familytree | |,|-|^|.| | | | | | | | |!| }}
*An adequate [[airway]] should be established and supplemental [[oxygen]] is given.<br>
{{familytree | D01 | | D02 | | | | | | D03 |D01= |D02= |D03= }}
*[[Continuous positive airway pressure]] (CPAP) or [[intubation]] for [[positive pressure ventilation]] can be done for [[infants]] with [[respiratory distress]] and [[carbon dioxide]] retention.<br>
{{familytree | |!| | | | | | | | | |,|-|^|.| }}
*If there is an [[airway]] [[obstruction]], [[prone]] positioning or [[oral airway]] is established to relieve [[cyanosis]].<br>
{{familytree | E01 | | | | | | | E02 | | | E03 |E01= |E02= |E03= }}
| C02= Depending upon [[etiology]]
{{familytree | | | | | | | | | | |!| | | | |!| }}
*'''[[Sepsis]]: '''[[Broad-spectrum antibiotics]] should be initiated such as [[ampicillin]] and [[gentamicin]].
{{familytree | | | | | | | | | | F01 | | | F02 |F01= |F02= }}
[[Blood cultures]] should be obtained to identify the [[causative agent]].<br>
*'''[[Neonatal]] [[Hypoglycemia]]''': Adequate [[blood glucose]] should be maintained in range of >45 to 50 mg/dl.<br>
*'''[[Cyanotic]] [[Congenital]] [[Cardiac]] [[Conditions]]''' should be approached with proper [[pediatric]] [[consultation]].<br>
*[[Prostaglandin]] E1 should be infused at 0.01-0.05mcg/kg/min.<br>}}
{{familytree | | | | | | | | | | | | | |!| }}
{{familytree | | |,|-|-|v|-|-|-|v|-|-|-|+|-|-|-|-|v|-|-|-|-|-|v|-|-|-|-|.| }}
{{familytree |D01| |D02| | |D03| |D04| | |D05| |D06| | |D07| |D01=[[TGA]], [[TAPVR ]],[[Truncus arteriosus]]|D02= [[TOF]]|D03=[[Ebstein anomaly]]  |D04=[[Hypoplastic left heart syndrome]] |D05=[[Sepsis]], [[shock]], low [[cardiac output]] state, [[cold exposure]], [[metabolic disorder]], [[polycythemia]] |D06=[[Eisenmenger syndrome]] with [[pulmonary hypertension]] |D07=[[Methemoglobinemia]]}}
{{familytree | |!| | | |!| | | |!| | | |!| | | | |!| | | | |!| | | | | |!||}}
{{familytree |D01| |D02| | |D03| |D04| |D05| | |D06| | |D07| | |D01= [[Infusion]] of [[prostaglandin]], [[diuretic]] [[therapy]], and [[surgery]].<ref name="Rao2013">{{cite journal|last1=Rao|first1=P. Syamasundar|title=Consensus on Timing of Intervention for Common Congenital Heart Diseases: Part II - Cyanotic Heart Defects|journal=The Indian Journal of Pediatrics|volume=80|issue=8|year=2013|pages=663–674|issn=0019-5456|doi=10.1007/s12098-013-1039-2}}</ref>|D02=[[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]]|D03=[[Tricuspid valve]] repair<ref name="pmid31384377">{{cite journal |vauthors=Holst KA, Connolly HM, Dearani JA |title=Ebstein's Anomaly |journal=Methodist Debakey Cardiovasc J |volume=15 |issue=2 |pages=138–144 |date=2019 |pmid=31384377 |pmc=6668741 |doi=10.14797/mdcj-15-2-138 |url=}}</ref>|D04=[[Hypoplastic left heart syndrome]] |D05=[[Treatment]] of underlying [[disorder]]|D06=[[Phosphodiesterase-5 inhibitor]] ([[sildenafil]], [[tadalafil]]). [[Endothelin receptor antagonist]] ([[ bosentan]],[[ macitentan]], [[ambrisentan]]).<ref name="pmid28536680">{{cite journal |vauthors=de Campos FPF, Benvenuti LA |title=Eisenmenger syndrome |journal=Autops Case Rep |volume=7 |issue=1 |pages=5–7 |date=2017 |pmid=28536680 |pmc=5436914 |doi=10.4322/acr.2017.006 |url=}}</ref>|D07=[[Infusion]] of [[methylene blue]], [[dextrose]], N-acetyl cysteine}}
{{familytree/end}}
{{familytree/end}}


==Do's==
==Do's==


*The content in this section is in bullet points.
*[[Hypoplastic left heart syndrome]] in [[infants]] should be considered with [[Symptoms and Signs|signs and symptoms]] such as:<ref name="pmid28356795">{{cite journal |vauthors=Gobergs R, Salputra E, Lubaua I |title=Hypoplastic left heart syndrome: a review |journal=Acta Med Litu |volume=23 |issue=2 |pages=86–98 |date=2016 |pmid=28356795 |pmc=5088741 |doi=10.6001/actamedica.v23i2.3325 |url=}}</ref>
**Sudden onset of [[shock]]
**[[Collapse]]
**Severe [[academia]] in the first week of life
**Neonatal [[sepsis]]
**[[Metabolic disorders]]
*In [[Ebstein anomaly]], repair of [[tricuspid valve]] is indicated if the following criteria is met:<ref name="pmid31384377">{{cite journal |vauthors=Holst KA, Connolly HM, Dearani JA |title=Ebstein's Anomaly |journal=Methodist Debakey Cardiovasc J |volume=15 |issue=2 |pages=138–144 |date=2019 |pmid=31384377 |pmc=6668741 |doi=10.14797/mdcj-15-2-138 |url=}}</ref>
**[[Cyanosis]]
**[[Right-side heart failure]]
**Poor [[functional capacity]]
**[[Paradoxical emboli]]
*In differential [[cyanosis]], if [[oxygen saturation]] of [[right arm]] is more than that of [[legs]] and improves with [[Oxygen|oxygen supplemental]] [[therapy]], then think about the following:
**Severe [[coarctation of aorta]]
**[[Aortic arch interruption]]
**[[Primary pulmonary hypertension]]
*In the presence of [[central cyanosis]] + [[hemolytic anemia]] ([[jaundice]], [[heinz body]], [[fragment RBC]]) + [[renal failure]], consider [[diagnosis]] of [[methemoglobinemia]] and [[Treatment|treat]] accordingly.
*[[Hydration]] is important in [[Tetralogy of Fallot|tetralogy of fallot]] to maintain [[pulmonary blood flow]] through [[Atresia|atretic]] [[pulmonary artery]] and reducing right to left [[shunt]] through [[VSD]].<ref name="O’BrienMarshall2014">{{cite journal|last1=O’Brien|first1=Patricia|last2=Marshall|first2=Audrey C.|title=Tetralogy of Fallot|journal=Circulation|volume=130|issue=4|year=2014|issn=0009-7322|doi=10.1161/CIRCULATIONAHA.113.005547}}</ref>
*If [[paradoxical embolism]] is suspected, proceed with a [[Brain CT scan]] in the presence of new [[neurological]] [[symptoms]] in a [[patient]] with [[cyanotic]] [[congenital heart disease]]. It occurs due to passing of [[emboli]] from right to left [[shunt]] and [[hyperviscosity]] ultimately leading to [[thrombosis]].<ref>{{cite journal|doi=10.1161/STROKEAHA.116.012882Stroke}}</ref>


==Don'ts==
==Don'ts==


*The content in this section is in bullet points.
*There are certain [[cyanotic congenital heart diseases]] that are dependent on [[patent ductus arteriosus]] ([[PDA]]). Don't give [[indomethacin]] to [[patients]] with the following ductal dependent [[Congenital Abnormalities|congenital conditions]]:
**[[Hypoplastic left heart syndrome case study one|Hypoplastic left heart syndrome]]
**Critical [[Aortic stenosis]]
**[[Interrupted aortic arch]]
**[[Transposition of the great vessels|Transposition of the great arteries]]
**[[Pulmonary stenosis|Critical Pulmonary stenosis]]


==References==
==References==
{{Reflist|2}}
{{Reflist|2}}


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Latest revision as of 21:39, 1 March 2021



Resident
Survival
Guide

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 a 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 the etiology and emergent cases with respiratory distress need rapid evaluation and response with immediate establishment of airway access and oxygen support.

Causes

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

  • A Focused Initial Rapid Evaluation (FIRE) should be performed to identify the patients in need of immediate intervention.[3]
 
 
 
 
 
 
 
 
 
Patient presents with cyanosis
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
No Respiratory Distress
 
 
 
 
 
 
 
 
 
 
 
 
Respiratory Distress
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Peripheral Cyanosis
 
Central cyanosis
 
Differential Cyanosis
 
 
 
No Obstruction
 
 
 
Obstruction
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Reassurance and Warming
 
Hypoxia Test
 
Cardiac Evaulation
 
 
 
Hypoxia Test
 
 
 
Give oxygen 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 oxygen and positive pressure Ventilation

Complete Diagnostic Approach

 
 
 
Patient presents with cyanosis
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
History
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Physical Examination
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Diagnostic Studies
 
 

Treatment

 
 
 
 
 
 
 
Treatment depends upon the etiology of cyanosis
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Respiratory Compromise
 
 
 
 
 
 
 
No Respiratory Compromise
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
*An adequate airway should be established and supplemental oxygen is given.
 
 
 
 
 
 
 
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.

     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
    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, and 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 methylene blue, dextrose, N-acetyl cysteine
     
     

    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)