P0485 - When You're Not OK Without Vitamin K

Introduction:
A previously healthy, unvaccinated, 3-month-old Amish female presented to the emergency department (ED) with one day history of altered mental status and new onset seizures. Parents described 10 episodes of vomiting one day prior, in addition to a five minute episode of full body stiffening and jerking movements at home on day of presentation.

Case Description:
Upon arrival to the ED, the child was obtunded. She was posturing bilateral upper extremities with tonic-clonic movements and had a tense, bulging fontanelle. An irregular shallow breathing pattern was noted while the remainder of her exam was otherwise unremarkable. Initial labs demonstrated normocytic anemia (Hbg 7.5), leukocytosis (WBC 31K), and thrombocytosis (platelets 1332K). Coagulation studies were abnormal with APTT > 200 sec, PT > 120 sec, INR > 15.5. MRI showed a large right frontal intracranial hemorrhage (ICH) with intraventricular hemorrhage (IVH) along with evidence of obstructive hydrocephalus and intracranial hypertension. Subsequently, she was admitted to the PICU after emergent hemorrhage evacuation and external ventricular drain placement. She required multiple packed RBC and FFP transfusions. Most importantly, she was treated with vitamin K injections. Coagulation studies improved drastically with vitamin K repletion. Ultimate diagnosis was ICH with IVH secondary to Late Vitamin K Deficiency Bleeding (VKDB).

Discussion:
Vitamin K Deficiency Bleeding (VKDB) presents in 3 typical ways: early, classic, and late. "Early" VKDB is bleeding in the first 24 hours of life, typically due to Vitamin K deficiency in the mother or medications that block action of vitamin K. "Classic" VKDB occurs within 1 week of life and usually consists of cutaneous, mucosal, gastrointestinal, or circumcision site bleeding. "Late" VKDB is bleeding that occurs between day 8 and 6 months, and is almost exclusively in breastfed infants often presenting with intracranial hemorrhage. In 1961, the AAP first recommended intramuscular (IM) vitamin K prophylaxis immediately after birth of all newborns to prevent VKDB. VKDB is uncommon in the US, with few reported cases restricted to deliveries which prophylaxis was not offered or was refused. Our patient, who was from an Amish community, was delivered by a midwife and no vitamin K was offered; eventually leading to patient's late VKDB. Without prophylaxis, the incidence of late VKDB ranges from 4.4 to 7.2 per 100,000 infants. The relative risk for developing late VKDB has been estimated at 81 times greater among infants without prophylactic IM vitamin K.

Conclusion:
It is essential to recognize the presentation of the 3 types of vitamin K deficiency bleeding, early, classic and late, and understand that prompt vitamin K repletion can quickly stabilize patients. This case stresses the importance of intramuscular vitamin K prophylaxis in infants to lessen risk of predictable preventable morbidities such as intracranial hemorrhage and subsequent seizures.