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MEDICAL SCIENCES
Bezhnar Vitalina-Olena Zahoruiko Veronika Tkachynska Yuliana Chorna Liudmyla Tarnavska S.I., Shakhova O.O., Department of Pediatrics and Children's Infectious Diseases Bukovinian State Medical Univercity, Chernivtsi, Ukraine DOI: 10.24412/2520-6990-2023-26185-3-4
DIFFERENTIAL DIAGNOSIS OF CYANOSIS IN CHILDREN
Resume. A number of scientific sources and studies were analyzed to explain which diseases cause the symptom of cyanosis. To understand the whole picture, we analyzed the current literature, which reveals all the pathologies in which we can observe cyanosis, both peripheral and central.
Key words: cyanosis, children, peripheral and central
Introduction. In more than 60% of patients, bouts of respiratory arrest are accompanied by cyanosis. The child usually cries briefly, becomes silent, stops breathing, and turns cyanotic. This can lead to loss of consciousness and provoke a seizure, even though the episode can resolve on its own [9-11]. Cyanosis is often accompanied by not only pulmonary but also cardiac pathologies. Tetralogy of Fallot is a congenital anomaly that leads to pulmonary stenosis, interventricular defect, biventricular aortic origin, and right ventricular hypertrophy. This is the most common heart defect in children, accompanied by cyanosis, accounting for 7 to 10 percent of congenital defects [12].
Cyanosis is a condition that occurs with certain he-modynamic disorders, manifested by a bluish color of the skin and mucous membranes. There are many reasons that can lead to this symptom. In order to better perform differential diagnosis, we first need to understand the pathophysiology and atophysiology of cyanosis. The next step is to consider the most common syndromes that are accompanied by cyanosis and which should be taken into account at the diagnostic stage.
The aim of the study is to systematize knowledge about the most common nosologies accompanied by cyanosis for better differentiation of diseases.
Materials and methods. A number of scientific sources and studies were analyzed to explain which diseases cause the symptom of cyanosis. To understand the whole picture, we analyzed the current literature, which reveals all the pathologies in which we can observe cyanosis, both peripheral and central.
Pathophysiology. The answer to the question of why cyanosis occurs is very simple. It is because in certain pathologies, the amount of oxygen that binds to hemoglobin is low. According to the literature, we know that oxygen in the blood is carried in two aggregate states: approximately 2% with plasma, and the remaining 98% is bound to hemoglobin [1, 2, 3]. Therefore, it follows that cyanosis occurs in situations where oxygen does not reach the peripheral tissues in sufficient quantities.
Oxygen may not be supplied to hemoglobin in the proper amount for certain reasons, but it may not be
supplied to hemoglobin in sufficient quantity if the concentration of hemoglobin itself in the blood is low. When the hemoglobin level is low, we may not see cyanosis, which makes it difficult to diagnose blood disorders early.
Differential diagnosis. For the first time in a child's life, cyanosis can be detected immediately after the birth of a child by examining and examining the newborn on the Apgar scale. According to the "skin color" criterion, cyanosis is rated at 0 points, acrocya-nosis - 1 point.
Cyanosis is a bluish color of the skin and mucous membranes caused by an increase in the concentration of reduced hemoglobin (>1.9-3.1 mmol/L) in the blood. It is known that peripheral and central forms of cyanosis are quite well recognized. Peripheral cyanosis, in other words, acrocyanosis in newborns is considered a benign discoloration of the skin of the extremities, which disappears over time. Central cyanosis is a serious pathological sign and includes discoloration of the lips and mucous membranes of the oral cavity, especially the tongue [4].
Acrocyanosis can be seen even in healthy new-borns, usually noticeably around the mouth, on the arms and legs. It is believed to be a benign condition caused by vasomotor changes as a result of the transition from intrauterine to extrauterine life. Differentiation of acrocyanosis from pathological causes of peripheral cyanosis (e.g., septic shock) is based on its clinical manifestation. Sometimes this picture can be observed in healthy infants immediately after delivery, and usually this condition does not last longer than 2448 hours after birth [5].
In peripheral cyanosis, the concentration of deox-ygenated hemoglobin on the venous side of the capillary bed increases due to increased tissue oxygen extraction, while the systemic oxygen saturation of arterial blood is normal. Peripheral cyanosis may be associated with peripheral vasoconstriction. As a rule, it affects the distal extremities, and sometimes the periorbital or periorbital areas [6]. The extremities may be cool or clammy. In newborns with isolated peripheral cyanosis, the mucous membranes remain pink, which
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MEDICAL SCIENCES / «<g©LL©(MUM~J©U®MaL» #26H®I)), 2023
distinguishes it from central cyanosis, in which the mucous membranes are cyanotic. It can be benign (e.g., acrocyanosis) or pathological. Causes include exposure to cold, sepsis, shock, venous obstruction, and polycythemia. Some of these causes may be associated with both central cyanosis and peripheral vasoconstriction [7].
The difference between central cyanosis and peripheral cyanosis is that there is a reduced oxygen saturation of the arterial circulation. Newborn infants usually have central cyanosis up to 5-10 minutes after birth, as oxygen saturation increases to 85-95 percent by 10 minutes of age [8]. Persistent central cyanosis is always a pathology and requires immediate evaluation and treatment [7].
Having considered the types of cyanosis, we can begin to consider the pathological conditions that lead to central cyanosis. After analyzing the literature [4], we can distinguish four groups of pathological conditions and nosologies accompanied by cyanosis, which occurs due to insufficient oxygen consumption by the lungs, insufficient pulmonary blood flow, abnormal pulmonary blood flow, and other cases with normal pO2 .
Let's consider the disorders that are accompanied by insufficient oxygen consumption by the lungs. First, respiratory failure (respiratory distress syndrome, pneumonia, meconium aspiration syndrome, pneumothorax, congenital diaphragmatic hernia). Secondly, upper airway obstruction (Pierre-Robin syndrome, tumors of the vascular ring, tumors of the neck or face). The third group of pathological conditions is hypoventilation (apnea, perinatal asphyxia, sepsis, metabolic disorders, congenital anomalies of the central nervous system or muscles, neonatal botulism).
The following pathological conditions lead to insufficient pulmonary blood flow Increased pulmonary vascular resistance (permanent pulmonary hypertension of known etiology or idiopathic, complete abnormal pulmonary venous return with subsequent obstruction, cor tiatriatum sinistrum) congenital heart disease (Fallot's tetrad, pulmonary atresia or critical pulmonary stenosis with intact interventricular septum, tricuspid valve atresia, Ebstein's anomaly, protruding Eustachian valve).
Next, our attention is drawn to the transposition of the great arteries, complete anomalous pulmonary venous insult, cor triatrium dexter, pulmonary arteriove-nous malformation, which lead to anomalous pulmonary blood flow.
Cases with a normal pO2 are methemoglobinemia and polycythemia.
Conclusions. Cyanosis in children can occur under any circumstances. Sometimes this symptom is a cause for concern, sometimes we can only observe this symptom in the dynamics, and sometimes we even recognize that this condition may be transient, and therefore we must reassure parents and assure them that it will pass with time. The best way to differentiate cyanosis is to study the mechanism of its occurrence. With this knowledge, we can choose the right treatment tactics for patients as soon as possible to avoid complications.
References:
1. Adeyinka A, Kondamudi NP. Cyanosis. [Updated 2023 Aug 12]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK482247/
2. Lundsgaard C. Studies on cyanosis : ii. secondary causes of cyanosis. J Exp Med. 1919 Sep 01;30(3):271-93.
3. Lundsgaard C. Studies on cyanosis : i. primary causes of cyanosis. J Exp Med. 1919 Sep 01;30(3):259-69.
4. Izraelit A, Ten V, Krishnamurthy G, Ratner V. Neonatal cyanosis: diagnostic and management challenges. ISRN Pediatr. 2011;2011:175931. doi: 10.5402/2011/175931. Epub 2010 Dec 29. PMID: 22482063; PMCID: PMC3317242.
5. Reproduced with permission from Janelle Aby, MD. Copyright © 2016 Stanford University Well Newborn Nursery. All rights reserved.
6. Cyanosis of the newborn infant. Recognition and clinical evaluation. Lees MH J Pediatr. 1970;77(3):484.
7. Eric C Eichenwald, Joseph A Garcia-Prats, Carrie Armsby, MD, MPH. Approach to cyanosis in the newborn. Literature review current through: Aug 2023. This topic last updated: Jul 13, 2022.
8. Textbook of Neonatal Resuscitation, 6th ed, American Academy of Pediatrics, 2011.
9. Lombroso CT, Lerman P. Breathholding spells (cyanotic and pallid infantile syncope). Pediatrics. 1967 Apr;39(4):563-81.
10. Leung AKC, Leung AAM, Wong AHC, Hon KL. Breath-Holding Spells in Pediatrics: A Narrative Review of the Current Evidence. Curr Pediatr Rev. 2019;15(1):22-29.
11. Goldman RD. Breath-holding spells in infants. Can Fam Physician. 2015 Feb;61(2):149-50.
12. Diaz-Frias J, Guillaume M. Tetralogy of Fal-lot. https://www.ncbi.nlm.nih.gov/books/NBK513288/
