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  1. Problem. 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."

  2. Immediate questions

      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.

      1. 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 Appendix C). Decreased urine output (<2 mL/kg/h) may indicate depleted intravascular volume or shock.

      1. Is the liver enlarged? Is there 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.

      1. 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.

      1. Are congenital anomalies present? Persistent cutis marmorata (see definition later) may be seen in Cornelia de Lange syndrome and in trisomies 18 and 21. Chromosome 22q11 deletion syndrome can present with abnormal vascular tone with hypotension; 75% of these infants have congenital heart defects. 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.

  3. Differential diagnosis

      1. More common causes

          1. Sepsis.

          1. Cold stress, in general, a skin temperature <36.5°C.

          1. Hypotension, usually with shock.

          1. Hypoventilation.

          1. Pneumothorax.

          1. Necrotizing enterocolitis (NEC).

          1. Left-sided heart lesions such as hypoplastic left heart syndrome, coarctation of the aorta, and aortic stenosis.

          1. Cutis marmorata, a marbling pattern of the skin (the infant appears poorly perfused). May occur in a healthy infant, especially when exposed to cold stress and with other congenital syndromes (Cornelia de Lange syndrome, trisomies 18 [Edwards syndrome] and 21 [Down syndrome]). Persistent mottling can also be seen in hypothyroidism and (CNS) dysfunction.

      1. Less common causes

          1. Enteroviral infection presents as overwhelming sepsis.

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

          1. Subgaleal hemorrhage. Case report of an infant presenting with poor perfusion.

          1. Inborn errors of metabolism.

          1. Seizures.

          1. Hematologic. Bleeding disorders, polycythemia.

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

          1. Renovascular hypertension.

          1. Intestinal problems.

  4. Database

      1. Physical examination. Note the temperature and vital signs. Look for signs of sepsis. The cardiovascular and pulmonary examinations are important because they may suggest cardiac problems or pneumothorax. Signs of trisomy 18 include micrognathia and overlapping digits. Signs of trisomy 21 include a single palmar transverse crease and epicanthal folds.

      1. Laboratory studies

          1. Complete blood count with differential to evaluate for sepsis or decreased hematocrit.

          1. Blood gas levels. These studies reveal inadequate ventilation or the presence of acidosis, which may be seen in sepsis or NEC.

          1. Cultures. If sepsis ...

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