72: Poor Perfusion

You receive a report that an infant “doesn't look good” or looks “mottled.” Other descriptions may include a “washed-out appearance” or “poor perfusion.”

1. What is the age of the infant? Hypoplastic left heart syndrome may cause poor perfusion and a mottled appearance. It may be seen at days 1–21 of life (more commonly at day 2 or 3). In an infant <3 days old, sepsis may be a cause. Associated risk factors for sepsis are premature rupture of membranes, maternal infection, and fever.

2. What are the vital signs? If the temperature is lower than normal, cold stress or hypothermia associated with sepsis may be present. Hypotension may cause poor perfusion (see normal blood pressure values in Table 65–1 and Appendix C). Decreased urine output (<2 mL/kg/h) may indicate depleted intravascular volume or shock.

3. Is the liver enlarged? Are metabolic acidosis, poor peripheral pulse rate, and a gallop present? These problems are signs of failure of the left side of the heart (eg, hypoplastic left heart syndrome). Poor perfusion occurs because of reduced blood flow to the skin.

4. If mechanical ventilation is being used, are chest movements adequate and are blood gas levels improving? Inadequate ventilation can result in poor perfusion. Pneumothorax may also be a cause.

5. Are congenital anomalies present? Persistent cutis marmorata (see Section III.A.12) may be seen in Cornelia de Lange syndrome and in trisomy 18 and 21. Chromosome 22q11 deletion syndrome can present with abnormal vascular tone with hypotension. Cornelia de Lange syndrome consists of multiple congenital anomalies: a distinctive facial appearance, pre- and postnatal growth deficiency, feeding problems, psychomotor delay, behavioral problems, and malformations that mainly involve the upper extremities.

1. More common causes

   1. Sepsis.

   2. Cold stress/hypothermia. In general, a skin temperature <36.5°C.

   3. Hypotension usually with shock.

   4. Hypoventilation.

   5. Pneumothorax.

   6. Hypoglycemia can mimic hypoxemia, and poor perfusion can be seen.

   7. Polycythemia with hyperviscosity. Infants have sluggish capillary refill and poor peripheral perfusion.

   8. Acute hemorrhagic anemia due to acute blood loss can present with symptoms of hypovolemia including poor perfusion, hypotension, tachycardia, and pallor. A decrease in peripheral perfusion occurs with a 10% loss of blood volume.

   9. Necrotizing enterocolitis (NEC).

   10. Left-sided obstructive heart disease. Newborns with critical left-sided obstructive lesions (ductal-dependent systemic circulation) generally appear normal at birth, and when the ductus arteriosus begins to close, they have cardiac failure with systemic hypoperfusion (poor perfusion with cold, clammy, mottled skin), poor peripheral pulses, increasing metabolic acidosis, and shock. Cyanosis may not be seen until later. One study showed that the majority of infants presented with shock, approximately one-third presented with heart failure, and a small percentage presented with profound cyanosis. These diseases include hypoplastic left heart syndrome (HLHS), critical aortic stenosis (AS), coarctation of the aorta (COA) (with or without septal defect), and interrupted aortic arch (IAA). When the ductus closes, infants with COA and IAA have hypoperfusion of the lower half of the body, and infants with AS and HLHS have hypoperfusion of the entire systemic circulation.

   11. Infant of substance-abusing mother. Mottling can be a sign of neonatal abstinence.

   12. Cutis marmorata. A marbling, red/blue, lacy, reticulated mottling pattern on the skin. It may occur in healthy infants especially when exposed to cold stress (immature neurologic and vascular system). It may also indicate poor perfusion in septic infants. Persistent mottling (persistent cutis marmorata) can occur in hypothyroidism, central nervous system dysfunction, and some congenital syndromes (Cornelia de Lange, Edward [trisomy 18] and Down [trisomy 21]). Cutis marmorata telangiectatica congenita is a rare vascular malformation (see Chapter 6). See Plate 1.

2. Less common causes

   1. Enteroviral/viral or fungal infection. Presents with poor perfusion and overwhelming sepsis.

   2. Periventricular hemorrhage/intraventricular hemorrhage (PVH/IVH). Presentation varies but can present with extreme signs, including sudden onset of poor perfusion, pallor, and hypotonia.

   3. Subgaleal hemorrhage (rare). Most commonly associated with a vacuum evacuation or forceps delivery. It progresses after birth and can have a massive amount of blood.

   4. Inborn errors of metabolism. Present with a history of deterioration with poor perfusion. Organic acidemia, urea cycle defects, and certain disorders of amino acid metabolism can present with poor perfusion, lethargy, and other symptoms.

   5. Seizures.

   6. Hematologic. Bleeding disorders.

   7. Adrenal problems. Congenital adrenal hyperplasia, Addison disease, adrenal hemorrhage.

   8. Renovascular hypertension. Presents with apnea, irritability, and mottling of the skin.

   9. Gastrointestinal problems. NEC, perforation, volvulus.

   10. Systemic air embolism. Symptoms are sudden and include mottling of the skin with pallor.

   11. Chronic pain. Infants experiencing chronic pain can exhibit decreased and poor perfusion with cool extremities.

1. Physical examination. Note the temperature and vital signs and look for signs of sepsis. The cardiovascular and pulmonary examinations are important because they may suggest cardiac problems or pneumothorax. Does the infant have an S3 gallop with or without a cardiac murmur (left-sided obstructive ductal-dependent heart lesion)? Signs of trisomy 18 include micrognathia and overlapping digits. Signs of trisomy 21 include a single palmar transverse crease and epicanthal folds. Look for scalp swelling to rule out subgaleal hemorrhage. Abdominal distension can be seen with gastrointestinal problems.

2. Laboratory studies

   1. Complete blood count (CBC) to evaluate for sepsis or decreased/increased hematocrit. To evaluate for polycythemia or blood loss.

   2. Blood gas. These studies reveal inadequate ventilation or the presence of acidosis, which may be seen in sepsis or NEC. Persistent metabolic acidosis can be seen in subgaleal hemorrhage.

   3. Blood glucose levels. To rule out hypoglycemia.

   4. Cultures. If sepsis is suspected, a complete workup should be considered, especially if antibiotics are to be started. This workup includes cultures of blood, urine, and spinal fluid (if indicated). If enteroviral infection is suspected, send for viral cultures.

   5. Polymerase chain reaction. Studies of stool and cerebrospinal fluid and nasopharyngeal or throat swab for enterovirus and other viruses.

   6. Inborn errors of metabolism. See Chapter 101 for appropriate tests.

3. Imaging and other studies

   1. Transillumination of the chest (see Chapter 27, Section III.B and Chapter 40) can be performed quickly to help determine whether or not a pneumothorax is present.

   2. Chest radiograph if pneumonia, pneumothorax, congenital heart lesion, or hypoventilation is suspected. In left-sided heart lesions, the radiograph shows cardiomegaly with pulmonary venous congestion (except in hypoplastic left heart syndrome, in which the size of the heart may be normal). If a view taken during lung expansion shows that the lungs are down only to the sixth rib or less, hypoventilation should be considered. With hyperventilation, lung expansion is down to the ninth or tenth rib. See Figure 11–15 for radiograph of pneumonia. See Figure 11–20 for radiograph showing a pneumothorax.

   3. Abdominal radiograph if NEC is suspected. See Figure 11–23 for a radiograph showing pneumatosis intestinalis seen in NEC. Air can be seen with a perforation. Obstruction can be seen with malrotation with volvulus.

   4. Echocardiography should be performed if a congenital heart lesion is suspected. In hypoplastic left heart syndrome, a large right ventricle and a small left ventricle are seen on the echocardiogram, and there is failure to visualize the mitral or aortic valve. In aortic stenosis, the echocardiogram reveals a deformed aortic valve. In coarctation of the aorta, it reveals decreased aortic diameter. In venous air embolism, one can see acute obstruction of the right ventricle outflow tract.

   5. Head ultrasound to rule out intraventricular bleed. Optimal imaging for subgaleal hemorrhage is by computed tomography (CT) or magnetic resonance imaging.

   6. Karyotyping or molecular genetic testing if trisomy 18 or 21 or a deletion is suspected. Cornelia de Lange syndrome has mutations in the NIPBL and SMC3 genes.

   7. CT of the head to look for intracranial air bubbles if systemic air embolism suspected.

1. Immediate plan. An initial quick workup should be performed. While checking vital signs and quickly examining the patient, order a STAT blood gas and a chest radiograph. Initiate oxygen supplementation and transilluminate the chest if a pneumothorax is suspected. Send a STAT CBC and differential and blood culture.

2. Specific plans

   1. Sepsis. Full cultures and empirical antibiotic therapy may be started at the discretion of the physician.

   2. Cold stress. Gradual rewarming is necessary, usually at a rate of ≤1°C/h. It can be accomplished by means of a radiant warmer or incubator or a heating pad. (See Chapter 7.)

   3. Hypotension or shock. If the blood pressure is low because of depleted intravascular volume, give crystalloid (normal saline), 10 mL/kg intravenously for 5–10 minutes. (See Chapter 65.)

   4. Hypoventilation. If suspected, it may be necessary to increase the pressure being given by the ventilator. The amount of pressure must be decided on an individual basis. One method is to increase the pressure by 2–4 cm H2O and then obtain blood gas levels in 20 minutes. Another method is to use bag-and-mask ventilation, observing the manometer to determine the amount of pressure needed to move the chest. (See Chapter 46.)

   5. Pneumothorax. See Chapter 70.

   6. Hypoglycemia. See Chapter 62.

   7. Polycythemia. See Chapters 71 and 122.

   8. Anemia secondary to acute blood loss. See Chapter 82.

   9. NEC. See Chapter 113.

   10. Left-sided obstructive heart lesions. Initial stabilization with respiratory support (endotracheal intubation and mechanical ventilation if poor respiratory effort and hypoxemia), volume resuscitation, inotropic support with dopamine for low cardiac output, and correction of metabolic acidosis. Immediate cardiac consultation. PGE1 is considered before diagnosis is confirmed if ductal-dependent systemic blood flow is suspected. The infant should be stabilized and transferred to a pediatric cardiac center. Surgery is usually indicated in all these patients. For a full discussion of cardiac abnormalities, see Chapter 89.

   11. Cutis marmorata. If this condition is secondary to cold stress, treat the patient as described in Section V.B.2. If the condition persists, consider formal genetic testing for various syndromes noted. Thyroid studies will be necessary if hypothyroidism is suspected. If CNS dysfunction is suspected, this should be evaluated further.

   12. Periventricular hemorrhage/intraventricular hemorrhage (PVH/IVH). Initial supportive care (maintain blood pressure, stabilize blood gases, transfuse if necessary, treat for seizures, etc.). After stabilization, close follow-up is required. Serial lumbar punctures may be necessary. (See also Chapter 104.)

   13. Subgaleal hemorrhage. Early recognition, appropriate resuscitation, supportive care as in volume replacement, blood transfusion, and coagulation factors if necessary. Pressure wrapping of the head is controversial.

   14. Inborn errors of metabolism. See Chapter 101.

   15. Seizures. See Chapter 129.

   16. Hematologic problems. Blood transfusions and diagnosing and treating the specific bleeding disorder are necessary.

   17. Adrenal insufficiency. Blood volume replacement and steroid therapy are usually necessary.

   18. Renovascular hypertension. Usually treated with aggressive medical management.

   19. Intestinal problems. See Chapters 113 and 131.

   20. Enteroviral infections. Supportive management. See Chapter 92. Systemic air embolism. Supportive cardiac and respiratory care. One hundred percent oxygen therapy, hyperbaric oxygen.

   21. Chronic pain. See Chapter 14.