A newborn boy is evaluated on arrival in the nursery. He was born 3 hours ago by spontaneous vaginal delivery at 40 weeks gestation after an uncomplicated pregnancy. Apgar scores were 9 at 1 minute and 10 at 5 minutes after birth. His mother has no significant medical history and does not take any medications. Temperature is 36.7 C (98 F), pulse is 124/min, and respirations are 26/min. He is awake, alert, and active with a strong cry. His head is normocephalic and atraumatic with an open flat anterior fontanelle. Pupils are equal and reactive to light and accommodation, and red reflexes are present bilaterally. The nares are patent, there is no cleft lip or palate, and the pharynx is not erythematous. Clavicles are intact without crepitus. S1 and S2 are normal without murmurs, and lungs are clear to auscultation bilaterally. The abdomen is soft, nontender, and nondistended with normal bowel sounds and no organomegaly. Femoral pulses are normal and symmetrical. All extremities move spontaneously and there is no edema. There are no rashes, skin lesions, or jaundice, but the complexion appears ruddy with generalized erythema. Blood samples obtained from the heel through simple prick method reveal a hematocrit of 70%. Repeat testing revealed a hematocrit of 65%. The patient is re-evaluated in the nursery and appears comfortable, with no tachycardia or respiratory distress. He feeds slowly but has no emesis, and voids spontaneously after his first feed. A decision is made to observe the child without intervention. Approximately 12 hours later, he appears irritable and lethargic. The infant has refused all subsequent feeds and has had decreased urine output. Nursery staff observed an episode of apnea. Temperature is 37.4 C (99.3 F), pulse is 170/min, and respirations are 60/min. Physical examination shows a drowsy, hypotonic, poorly responsive newborn. The fontanelle is open and flat. Pupils are equal and reactive with normal red reflexes. There is no rhinorrhea. Oral mucosa is moist. S1 and S2 are present without murmurs. On pulmonary examination there are no wheezes or rales, but there are intercostal retractions. The skin is erythematous and ruddy, and there is cyanosis of the fingertips. There are no focal neurologic deficits. Laboratory results from peripheral blood draw are as follows:
The patient is transferred to the neonatal intensive care unit, and treatment with intravenous fluids and glucose is initiated. His symptoms fail to improve, and his hematocrit after several hours is 71%. What is the most appropriate next step in management of this patient?

Question

A newborn boy is evaluated on arrival in the nursery.  He was born 3 hours ago by spontaneous vaginal delivery at 40 weeks gestation after an uncomplicated pregnancy.  Apgar scores were 9 at 1 minute and 10 at 5 minutes after birth.  His mother has no significant medical history and does not take any medications.  Temperature is 36.7 C (98 F), pulse is 124/min, and respirations are 26/min.  He is awake, alert, and active with a strong cry.  His head is normocephalic and atraumatic with an open flat anterior fontanelle.  Pupils are equal and reactive to light and accommodation, and red reflexes are present bilaterally.  The nares are patent, there is no cleft lip or palate, and the pharynx is not erythematous.  Clavicles are intact without crepitus.  S1 and S2 are normal without murmurs, and lungs are clear to auscultation bilaterally.  The abdomen is soft, nontender, and nondistended with normal bowel sounds and no organomegaly.  Femoral pulses are normal and symmetrical.  All extremities move spontaneously and there is no edema.  There are no rashes, skin lesions, or jaundice, but the complexion appears ruddy with generalized erythema.  Blood samples obtained from the heel through simple prick method reveal a hematocrit of 70%. Repeat testing revealed a hematocrit of 65%.  The patient is re-evaluated in the nursery and appears comfortable, with no tachycardia or respiratory distress.  He feeds slowly but has no emesis, and voids spontaneously after his first feed.  A decision is made to observe the child without intervention.  Approximately 12 hours later, he appears irritable and lethargic.  The infant has refused all subsequent feeds and has had decreased urine output.  Nursery staff observed an episode of apnea.  Temperature is 37.4 C (99.3 F), pulse is 170/min, and respirations are 60/min.  Physical examination shows a drowsy, hypotonic, poorly responsive newborn.  The fontanelle is open and flat.  Pupils are equal and reactive with normal red reflexes.  There is no rhinorrhea.  Oral mucosa is moist.  S1 and S2 are present without murmurs.  On pulmonary examination there are no wheezes or rales, but there are intercostal retractions.  The skin is erythematous and ruddy, and there is cyanosis of the fingertips.  There are no focal neurologic deficits.  Laboratory results from peripheral blood draw are as follows:
 The patient is transferred to the neonatal intensive care unit, and treatment with intravenous fluids and glucose is initiated.  His symptoms fail to improve, and his hematocrit after several hours is 71%.  What is the most appropriate next step in management of this patient?

Quizplus · Accepted Answer

Explanation: Although the majority of newborns with polycythemia are asymptomatic, some affected patients will develop hyperviscosity, which causes complications such as hypoperfusion and tissue hypoxia. This manifests clinically in signs such as plethora, lethargy, irritability, drowsiness, poor feeding, abdominal distension, and hypotonia. Respiratory compromise may occur, resulting in apnea or cyanosis. Metabolic derangements such as hypoglycemia and hyperbilirubinemia are common laboratory abnormalities. Asymptomatic patients can be observed clinically, as the condition usually resolves within the first 24 hours of life; however, those with clinical symptoms require treatment. Initial treatment consists of hydration and correction of hypoglycemia. If first-line treatment is ineffective, an alternate management strategy is partial exchange transfusion. This procedure consists of withdrawing blood from the infant and infusing normal saline. There are risks associated with this procedure, so it is not the first-line option; however, it improves short-term symptoms of hyperviscosity and is therefore a reasonable next step if more conservative management fails. (Choices A and C) Apnea, tachypnea, retractions, and cyanosis could suggest cardiac or pulmonary pathology. However, the patient has laboratory evidence of polycythemia and his cardiopulmonary examination demonstrated clear lungs and normal heart sounds. Therefore, although chest x-ray and echocardiography might be performed later as part of a complete workup, the most important next step is to correct the underlying hematologic abnormality. (Choice B) The patient is irritable and lethargic, but he does not have any focal neurologic deficits, and CT scan would expose his developing brain to a significant amount of radiation. In addition, he has evidence of polycythemia, which is known to cause neurologic symptoms. (Choice D) Apnea, tachypnea, and hypoglycemia can be markers of neonatal sepsis. However, this patient does not have other laboratory findings suggestive of sepsis (eg, neutropenia, leukocytosis, thrombocytopenia), and he has the classical clinical picture and laboratory findings of symptomatic neonatal polycythemia, so correction of the polycythemia is a more appropriate step. Educational objective: Neonatal polycythemia is frequently asymptomatic but can be a life-threatening disease that causes apnea, hyperviscosity, hypoglycemia, hyperbilirubinemia, and respiratory compromise. Treatment consists of adequate hydration and correction of metabolic derangements (eg, hypoglycemia). Partial exchange transfusion is an option if patients fail to improve with conservative management; however, there are associated risks with the procedure. References: Management of polycythemia in neonates. (http://www.ncbi.nlm.nih.gov/pubmed/20725868)

A Newborn Boy Is Evaluated on Arrival in the Nursery