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Question:

A 6-hour-old boy is evaluated in the newborn nursery due to scalp swelling.  The patient was born at term via cesarean delivery to a 21-year-old primigravida.  The pregnancy was unremarkable, and the mother's only medication was a prenatal multivitamin.  Delivery was complicated by 18 hours of active labor followed by arrest of descent, necessitating cesarean delivery.  Family history is notable for a maternal uncle with severe hemophilia A.  Temperature is 37.3 C (99.1 F) and pulse is 190/min.  The patient appears pale.  Examination of the scalp shows diffuse, fluctuant edema extending from the right ear across the vertex to just above the left ear.  Laboratory results are as follows:

Complete blood count
     Hemoglobin9 g/dL
     Platelets192,000/mm3
     Leukocytes10,000/mm3
PT13 sec
PTT38 sec

Which of the following is the most likely cause of this patient's clinical presentation?

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Explanation:

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This newborn has diffuse scalp swelling and signs of blood loss (eg, pallor, anemia, tachycardia), findings concerning for a subgaleal hemorrhage.

Subgaleal hemorrhage is a rare but potentially fatal neonatal injury caused by the rupture of emissary veins upon scalp traction during delivery.  These sheared veins, which connect the dural sinuses and the scalp, can cause massive blood accumulation between the periosteum and galea aponeurotica.  Although subgaleal hemorrhage occurs more frequently with vacuum-assisted deliveries, it can also develop with spontaneous vaginal or cesarean delivery, as seen in this patient.

Examination shows diffuse, fluctuant scalp swelling that extends beyond the suture lines and potentially beyond the skull to the neck.  The swelling shifts with movement and expands over 2-3 days.  Prompt diagnosis is critical because progressive bleeding can lead to hypovolemic shock, disseminated intravascular coagulation, and death.  Treatment is generally supportive (eg, volume resuscitation, correction of coagulopathy).

(Choice A)  Vitamin K, which is low in newborns due to poor placental transfer, is an essential cofactor in the carboxylation of proteins that activate coagulation factors.  Infants who do not receive the routine intramuscular vitamin K injection at birth are at risk for bleeding between weeks 1 and 4 of life.  Early-onset (<24 hr) bleeding due to vitamin K deficiency can occur with use of certain prenatal medications (eg, anticonvulsants), not used by this patient's mother, that limit vitamin K action.

(Choice B)  Factor VIII deficiency (ie, hemophilia A) is an X-linked bleeding disorder that typically presents in infancy with easy bleeding (eg, hemarthrosis, intracranial hemorrhage) and a prolonged PTT.  Although this patient has a positive family history, his PTT is normal, making this diagnosis unlikely.

(Choice C)  Disseminated intravascular coagulation is characterized by massive coagulation factor consumption and presents with bleeding (eg, venipuncture site oozing).  Laboratory evaluation reveals thrombocytopenia and prolonged PT and PTT.  This patient's platelet count, PT, and PTT are normal.

(Choice E)  Cephalohematoma is characterized by bleeding between the skull and periosteum due to subperiosteal vessel rupture.  Unlike in this patient, examination shows a firm, nonfluctuant swelling that does not cross suture lines or lead to significant blood loss.

Educational objective:
Subgaleal hemorrhage is caused by the shearing of veins between the dural sinuses and scalp due to scalp traction during delivery.  Blood accumulates between the periosteum and galea aponeurotica, causing diffuse, fluctuant scalp swelling.  Rapid hemorrhage expansion can lead to hypovolemic shock, disseminated intravascular coagulation, and death.