CYANOSIS is defined as the bluish discolouration of the skin and mucous membrane due to presence of increased amount of reduced

hemoglobin (>5 gm/dl) or of certain abnormal hemoglobins in the capillary blood.



The term cyanosis is derived from the greek word KYANOS which means GREENISH BLUE.

INFLUENCE OF HEMOGLOBIN LEVEL ON CYANOSIS: Hb in venules is about 2 g/dl  Oxygen saturation 80% - cyanosis occurs  Polycythemia- cyanosis occurs in higher oxygen saturation  Anemia- cyanosis occurs in lower saturation level
 Normal reduced

TYPES OF CYANOSIS 1) Peripheral cyanosis 2) Central cyanosis

PERIPHERAL CYANOSIS:  Cyanosis associated with normal arterial oxygen saturation is called peripheral cyanosis.  In peripheral cyanosis, there is increased extraction of Oxygen by peripheral tissue MECHANISM OF PERIPHERAL CYANOSIS a) Reduced cardiac output b) Peripheral vasoconstriction c) Sluggish circulation in the extremities

CAUSES OF PERIPHERAL CYANOSIS a) Exposure to cold air or cold water b) Hypothermia c) Congestive cardiac failure d) Hypoglycemia e) Shock or peripheral circulatory failure due to any cause f) Sepsis g) Frost bite h) Polycythemia

SITES TO BE LOOKED FOR 1. Tip of the nose 2. Ear lobules 3. Tip of fingers and toes 4. Nail bed of fingers and toes 5. Palms and soles ATYPICAL SITES FOR EXAMINATION OF CYANOSIS 1. Nasal mucous membrane examined by nasal septum 2. Rectal mucous membrane seen by proctoscopy 3. Retina examined by opthalmoscopy

CENTRAL CYANOSIS


Cyanosis secondary to desaturation of arterial blood is called central cyanosis usually recognized when arterial oxygen saturation is 85% or lower

 It is

it may not be detected until saturation is 75% in dark skinned persons

MECHANISM OF CENTRAL CYANOSIS 1) R L shunting at the intracardiac, great vessel, or intrapulmonary level 2) Alveolar hypoventilation 3) Ventilation perfusion mismatch 4) Inadequate transport of O2 by Hb 5) Hemoglobinopathies ( including Methemoglobinemia ) that limits O2 transport

CARDIAC CAUSES
Cyanotic heart Cyanotic heart dis with pul. dis with pul Blood flow blood flow TOF Tricuspid atresia Critical pulmonary stenosis/ atresia Double outlet right ventricle with PS Single ventricle with PS TGA TAPVC Left ventricular Persistent outflow pulmonary obstruction hypertension of newborn Severe aortic stenosis Hypoplastic left heart flow Severe COA Acute cardiac failure due to any cause

RESPIRATORY CAUSE
Upper airway obstruction Congenital malformation Angioneurotic oedema Epiglotitis Diptheria Retropharyngeal abscess Foreign body Macroglossia Respiratory failure Pneumothorax Severe pneumonia Bronchiolitis Bronchial asthma Lobar collapse Cystic fibrosis Pulmonary oedema Pumonary hemorrhage

CNS CAUSES Cyanosis occurs due to a) Apnea b) Hypoventilation or c) Paralysis of respiratory muscles
CNS causes Encephalitis Meningitis Status epilepticus ICH Paralysis of respiratory muscles- Paralytic Poliomyelitis Gullian Barre syndrome Cogenital Myopathies

METABOLIC CAUSES:
Metabolic causes Methhemoglobinemia Sulfhemoglobinemia Carboxyhemoglobinemia

SITES TO BE EXAMINED
Sites to be examined Tongue Inner aspect of lips Mucous membrane of gums, soft palate and cheeks PLUS sites mentioned in peripheral cyanosis

HOW TO DIFFERENTIATE CYANOSIS FROM BRUISING / ECCHYMOSIS  Apply pressure over the skin  Skin will blanch with cyanosis but not with ecchymosis If you are allowed to examine ONLY ONE SITE IN CYANOSIS; what to do? - look for cyanosis at the tip of the tongue, REASONS Its colour is not affected by race or ethnic background  Circulation is not sluggish as in peripheral parts of the body  If the tip of the tongue is blue, cyanosis must be central but if the finger tips are blue, it may be either of central or peripheral in type.

ENTEROGENOUS CYANOSIS / PIGMENT CYANOSIS When cyanosis is produced due to the presence of excess sulphemoglobin (>0.5 gm/dl) or methemoglobin (>1.5 gm/dl) in blood, it is called enterogenous cyanosis


The causes are- methemoglobinemia sulphemoglobinemia

METHEMOGLOBINEMIA It is an altered state of Hb in which the ferrous form of haeme is oxidized to the ferric form thus making the haeme moeity unable to bind to oxygen


Blood is chocolate brown in colour The chocolate brown colour of blood in Methemoglobinia does not change colour with addition of oxygen whereas dark blood due to Oxyhemoglobinemia will turn red with oxygen

CAUSES
Acquired Aniline dyes Congenital Cytochrome b5 reductase deficiency (NADPH deficiency) responsible for 95% of methemoglobin reduction NADPH deficiency of HMP shunt

Bnzene Chloroquine Dapsone Local anaesthetic agents Nitrates

 

A MetHb level of 15% is associated with visible cyanosis A MetHb level of 70% is lethal

SULPHAEMOGLOBINEMIA  A sulphur atom is incorporated into Hb molecule  When hydrogen sulphide (H2S) or sulphide is combined with Hb, the Hb becomes incapable of carrying oxygen CAUSES - usually drug induced
Associated drugs Sulfonamides Acetanilid Phenacetin Phenazopyridine Trinitrotoulene Nitrates

DIFFERENTIAL CYANOSIS Bluish discolouration of the lower but not the upper extremity and head, seen in PDA MECHANISM  Patients with large PDA develop progressive pulmonary vascular disease  There is pressure overload of RV  As soon as pulmonary pressure exceeds aortic pressure, shunt reversal (R L) occurs  The upper extremity remains pink because the brachiocephalic trunk, left common carotid trunk and left subclavian trunk is given off proximal to PDA

REVERSE DIFFERENTIAL CYANOSIS

 

Bluish discolouration of the upper but not the lower extremity Seen when TGA is combined with pulmonary hypertension and a patent ductus

MECHANISM

In this setting, the desaturated blood enters the ascending aorta from RV and saturated LV blood enters the descending aorta via the patent ductus

ACROCYANOSIS
   

Bluish colour of the digits seen in neonates and infants Form of peripheral cyanosis Reflects sluggish blood flow in the digits No clinical significance when associated with circulatory shock

CIRCUMORAL CYANOSIS
  

Bluish skin colour around the mouth Form of peripheral cyanosis Seen in healthy child with fair skin because of sluggish capillary blood flow in association with vasoconstriction Isolated circumoral cyanosis is of no concern unless it occurs as a result of low cardiac output

HARLEQUIN CONDITION
 Unique

condition in which one quadrant or one half of the body may become cyanotic or pale while the rest of the body remains pink.  However hands and feet remains warm  Exact reason is not known  Thought to be because of vasomotor instability

ORTHOCYANOSIS
  

Cyanosis only in upright position Due to hypoxia occurring in upright position As a result of pulmonary A-V malformation

CENTRAL + PERIPHERAL CYANOSIS

 

Cardiogenic shock with pulmonary oedema CCF due to LVF

HISTORY TAKING IN THE APPROACH TO CYANOSIS 1) MATERNAL HISTORY Important features of maternal history predisposing to pediatric disorders include:
Pregnancy history Gestational diabetes Oligohydromnios Lithium intake (1st trimester) PIH Advanced maternal age Polyhydromnios Delivery of previous sibling with RDS Associated cuase TTNB, RDS, Hypoglycemia, TGA Pulmonary hypoplasia Ebstein anomaly IUGR, Polycythemia, Hypoglycemia Trisomy 21 TEF / Oesophageal atresia Surfactant proteinB deficincy Group B Streptococcal pneumonia

MATERNAL HISTORY contd.

Labour history PROM, Fever, Chorioamnitis Sedatives, Anaesthetics C- section Asphyxia Preterm delivery Breech delivery Passage of meconium in-utero

Associated cause Sepsis Respiratory depression, apnoea TTNB, RDS, Persistent pulmonary hypertension of newborn Cerebral oedema Metabolic acidosis RDS Erb s palsy with phrenic nerve palsy MAS

2) ONSET

Immediate onset after birth TTNB RDS Pneumothorax MAS Congenital diaphragmatic hernia Cyanotic CHD

Onset hours after birth Aspiration TEF

Onset of cyanosis after breast feeding B/L Choanal Atresia

3) AGE  CHD or Polycythemia are more common in neonate  Methemoglobinemia is more likely in young infants esp. those with gastroenteritis or oxidant toxin exposure 4) FEVER Fever may be present in children with  Infection  Causes secondary to upper airway obstruction (eg. Croup)  Lower airway disease (eg. Pneumonia ) or  Shock (eg. Septic shock)

5) TRAUMA Chest wall or upper airway trauma may cause central cyanosis due to  Lung injury or  Upper airway obstruction 6) EXPOSURE TO DRUGS / SMOKE  Smoke inhalation -Cyanosis due to inspired Oxygen  Drugs( eg. Methemoglobinemia, Sulphemoglobinemia)  Nitrates  Aniline dyes  Naphthalene etc.

7) PRIOR LUNG DISEASE Exacerbation of preexisting lung disease is a common cause of respiratory distress and central cyanosis in children. Eg.
 

Asthma, Bronchopulmonary dysplasia

8) CONGENITAL HEART DISEASE Cyanotic CHD may explain profound central cyanosis in an otherwise well appearing child or underlying pulmonary oedema or shock in the ill appearing child. 9) MEDICATIONS
Amiodarone Blue skin colour  Colloidal Silver - Argyria, a gray tinting of the skin


PHYSICAL EXAMINATION IN THE APPROACH TO CYANOSIS 1) The effects of local heat and breathing Oxygen on the different types of cyanosis areCauses of cyanosis 1) PERIPHERAL 2) CENTRAL a) pulmonary b) polycythemia c) R L shunt 3) ENTEROGENOUS Remains Remains Remains Remains Abolished Abolished Remains Remains Local heat Abolished Breathing Oxygen for 10 mins Remains

2) CLINICAL FINDINGS OF CENTRAL CYANOSIS

Clinical findings Respiration CNS depression Shallow Irregular Poor Disappears on -stimulation - oxygen administration Normal Pulmonary disease Tachypnoea Retraction Grunt Normal Improves / Abolishes on oxygen administration Crackles &/ or Decreased breath sound Cardiac disease Tachypnoea without grunt

Muscle tone Cyanosis

Normal Not much affected

Auscultation

Murmur may be present

3) STRIDOR + SUPRASTERNAL RETRACTION It identifies upper airway obstruction

Important upper airway obstruction in pediatric clinical practice1) Foreign body 2) Croup 3) Epiglottitis 4) Laryngotracheomalacia 5) Glossoptosis with micrognathia (Pierre Robin syndrome)

4) ABDOMINAL EXAMINATION
 Scaphoid

abdomen in congential diaphragmatic hernia  Hepatomagaly can co exist with pulmonary congestion in TAPVR  Tender hepatomegaly in CCF  Absence of bowel sounds indicates paralytic ileus which may be seen in Sepsis  Gangrenous bowel, or  Peritonitis

5) TRAUMATIC LUNG INJURY May have multiple findings including abnormal chest wall movement ( eg. a flail segment)  open, sucking chest wound  chest wall ecchymosis or abrasion  subcutaneous air with crepitations  tracheal deviation  focal tenderness on palpation over ribs, sternum, or scapula 6) Central cyanosis with slate gray appearance of the skin is characteristics of Methemoglobinemia.

7) PULSE OXIMETRY  Accurate, reliable and non invasive  It however does not indicate patients ventilation  2 principles involved- Differential light absorption by Hb and OxyHb - Identification of pulsatile component of signal  In severe cyanosis with respiratory distress both preductal and postductal O2 saturation should be monitored to detect the gradient across the Ductus arteriosus  For this purpose, pulse oximeter probes should be placed over the right hand and a lower extremity

LIMITATIONS a) Bright overhead lighting, shivering and motion artifacts may give pulsatile waveforms and saturation values when there is no pulse b) False reading in Methemoglobinemia and Sulphemoglobinemia c) Vasoconstriction and hypothermia cause reduced tissue perfusion and failure to generate a signal

INVESTIGATION IN THE APPROACH TO CYANOSIS 1) HYPEROXIA TEST  Clinical tool to differentiate between cardiac and pulmonary etiologies of cyanosis.  Indicated if pulse oximeter reading is < 85% in both room air and 100% oxygen  Steps of hyperoxia test -the arterial PO2 is measured -O2 should be administered through a plastic hood for atleast 10 mins so that alveolar O2 is replaced completely with 100% O2 - the arterial PO2 is measured again and compared

INTERPRETATION
 Arterial O2

concentration rises above 100 mm Hg indicates pulmonary disease  Rise in arterial O2 concentration does not exceed 100 mmHg and the rise is not more than 10-30 mmHg indicates significant intracardiac R L shunt  Arterial PO2 from right radial artery is 10-15 mmHg higher than that from an umbilical artery catheter -indicates R L ductal shunt

POINTS TO BE NOTED WHILE DOING HYPEROXIA TEST



The response of PO2 to 100% O2 inhalation should be interpreted in the light of clinical picture, especially the degree of pulmonary pathology seen on CXR infants with Cyanotic CHD with a large pulmonary blood flow viz TAPVR may have a rise in arterial PO2 to 100 mmHg or higher

 Some

HYPEROXIA contd.
 Infants

with massive intrapulmonary shunt from lung disease ( but with a normal heart ) may not show rise in arterial PO2 to 100 mmHg Arterial blood samples for measurement of PO2 should be obtained from the upper part of body viz radial, brachial, or temporal arteries to avoid false low values caused by a R L ductal shunt.

2) ABG  Cyanotic patients with abnormalities in respiration, control of breathing or ventilate have a decreased partial pressure of O2 (PaO2) in arterial blood


Helps in determining the oxygenation, ventilation and acid base status Arterial PaO2 In neonates, O2 saturation is not a good indicator to confirm central cyanosis as there is increased affinity of fetal Hb

ABG contd.


Increased PaCO2 Indicates Pulmonary / CNS disorders Decreased pH- Indicates sepsis, shock, severe hypoxemia Methemoglobinemia- Oxygen saturation is decreased but PaO2 is normal

3) CHEST X-RAY
 Integral

part of the initial assessment of a child with cyanosis

 It

should be ordered to evaluate lung fields for consolidation, infiltration and increased vasulature pulmonary oedema cardiac sillhoute and mediastinum may suggest congenital heart disease

 The

The following table shows the important CXR findings that results in cyanosis
Disease TOF X- ray findings Boot shaped heart Concave pulmonary segment Oligemic lung fields Egg shaped heart/Egg on string appearance Slight cardiomegaly pulmonary vascular markings Snowman appearance with linear pulmonary segment Extreme cardiomegaly Reticular granularity Air bronchogram Decreased lung volume Good lung volume with perihilar markings along with fissural fluid in the right horizontal fissure

D-TGA

TAPVR Ebstein anomaly RDS

TTNB

X-RAY FINDINGS contd.

Disease Pneumothorax X-ray findings Mediastinal shift towards normal side Hypertranslucent lung field Lung markings absent, Ribs horizontal, Flat diaphragm Mediastinal shift towards normal side Homogenous opacity on effected side Obliteration of costophrenic angles Trachea pulled to the affected side Non homogenous opacities on affected side Normal costophrenic angles Overcrowding of ribs No mediastinal shift Non homogenous opacity on affected side Costophrenic angles are clear Normal ribs

Pleural effusion

Collapse of lung

Pneumonic consolidation

X RAY FINDINGS contd.

Disease Pulmonary oedema

X- ray findings Fluffy infiltrates or hazy lung fields Associated cardiomegaly Diagnostic with an indwelling orogastric tube curled in upper oesophgeal pouch

Tracheooesophageal fistula/ Oesophageal atresia

Congenital More common in left side Diaphragmatic hernia Intestinal gas pattern in left hemithorax Mediastinal shift to right side

4) ECG In cyanotic patients with abnormal findings on cardiac examination, ECG can be very helpful in solidifying a diagnosis of heart disease COMMON ELECTROCARDIOGRAPHIC MANIFESTATION OF CHD
Congenital defect Anomalous pulmonary venous return a) Total b) Partial ASD a) Primum type Superior QRS axis RBBB or RVH First degree AV block (>50%) RAD, RVH or RBBB First degree AV block (10%) ECG findings

RAD, RVH &RAH Mild RVH or RBBB

b) Secundum type

ECG FINDINGS contd.

Congenital defect Ebstein anomaly ECG findings RAH, RBBB First degree AV block WPW pre-excitation No RVH Normal LVH, LAH () BVH, LAH RVH or BVH LVH RVH or BVH RAD RVH, with or without strain RVH, RAH BVH, RAH or BVH

PDA a) Small shunt b) Moderate shunt c) Large shunt d) Eissenmenger s syndrome Pulmonary abscess with hypoplastic RV Eissenmenger s syndrome TOF D TGA (complete transposition) a) Intact ventricular septum b) VSD &/or PS

ECG FINDINGS contd.

Congenital defect VSD a) Small shunt b) Moderate shunt c) Large shunt d) Eissenmenger s syndrome ECG findings Normal LVH, LAH () BVH,LAH RVH

COA a) Age < 6 months b) Older children

RBBB or RVH LVH, normal, RBBB

5) ECHOCARDIOGRAPHY Extremely useful, safe and non invasive that can be used for the diagnosis and management of cardiac disease  TYPES a) M mode echocardiography b) 2 D echocardiography c) Doppler echocardiography M MODE ECHOCARDIOGRAPHY


M mode echocardiography is obtained with ultrasonographic transducer placed along the left sternal border and directed towards the part of heart examined

 Although

2 D echocardiography has largely replaced the M mode echocardiography in the diagnosis of heart disease, the M mode echocardiography retains many important applications such asa) measurement of the dimension of cardiac chambers and ventricles, thickness of ventricular septa and free wall b) LV systolic function c) Study of the motion of cardiac valves and interventricular septum d) Detection of pericardial fluid

2 D ECHOCARDIOGRAPHY It is performed by directing the plane of transducer along a number of cross-sectional planes through the heart and great vessels


A routine 2D echocardiography is obtained from four transducer locationsa) parasternal b) apical c) subcostal d) suprasternal notch

DOPPLER ECHOCARDIOGRAPHY  Combines the study of cardiac structure and blood flow profile


Two commonly used techniquesa) Pulsed Wave - Pulsed wave emits a short burst of ultrasound and doppler echo receiver listens for returning information b) Continuous Wave- Continuous wave emits a central ultrasound beam with one crystal and another crystal continuously receives returning information

OTHER LABORATORY TESTS

a) Complete Blood Count -

or WBC- Sepsis Hb > 22 gm/dl indicates Polycythemia b) Hematocrit - > 65% indicates Polycythemia c ) Hb electrophoresis and Spectroscopy for diagnosis of Methemoglobinemia and Sulphemoglobinemia

CONCLUSION
 Cyanosis

is frequently seen in children especially newborns  Major cause- cardiopulmonary causes,  There are several other conditions that may present with similar symptoms.  A systematic approach to diagnosis is required  history,  physical examination,  work up (eg. CXR, ECG, Echocardiography, Hyperoxia test)  laboratory tests including blood glucose, CBC, and septic work up, as indicated is required

ETIOLOGY AND EVALUATION OF CYANOSIS IN CHILDREN

ALOGARITHMIC APPROACH TO PERIPHERAL CYANOSIS

ALOGARTHMIC APPROACH TO CENTRAL CYANOSIS