Vitamin K deficiency bleeding (VKDB) in infancy is an acquired coagulopathy secondary to reduction of vitamin K (VK)-dependent coagulation factors (II, VII, IX, X), below hemostatic levels; 30-60 % of cases are associated with intracranial haemorrhage [4
]. In a bleeding infant a prolonged PT together with a normal fibrinogen level and platelet count is almost diagnostic of VKDB; rapid correction of the PT and/or cessation of bleeding after VK administration are confirmative [5
]. A normal PT for age excludes the diagnosis of VKDB [6
Our case seems to fall in the category of late VKDB. Late VKDB begins on or after day 8, most often between weeks 2 and 8 and rarely after 3 months [4
]. In co-operative studies the upper age limit was set arbitrarily at the end of week 12 [8
]; but infants presenting with VKDB between weeks 13 and 26 should also be reported [9
]. Our case presented at 18 weeks of age.
Late VKDB occurs almost exclusively in breast-fed infants, more often in boys than girls [4
]; these infants often have history of diarrhea lasting for more than one week or antibiotic consumption within a week of the onset of the presenting bleeding which is commonly an intracranial one [10
]. Its incidence ranges widely; for reasons which may include racial variation, maternal diet, and different VK prophylaxis regimens and compliance.
The child discussed in this report did not receive a prophylactic vitamin K intramuscular injection after birth. Child birth occurred at home under the care of insufficiently educated midwife. Here we point that the obstetric and neonatal care along with the compliance of prophylactic measures in Pakistan does not seem to be satisfactory. Emphasis upon initial prophylactic vitamin K supplementation and an additional intramuscular dose or oral supplementation of vitamin K especially for exclusively breast-fed infants may reduce this catastrophic problem [11
APTT was also prolonged to 120 seconds in this child. This suggested coexisting deficiency of other clotting factors including factor V, factor X and factor 1. However plasma assays for these factors were not performed due to cost issues, especially when APTT level returned to normal after administration of vitamin K. We wanted to perform these assays later, but the child was lost for follow-up despite repeated requests and calls.
The late onset of VKBD can point to the presence of some secondary cause of the deficiency. Such possible causes include: chronic diarrhea, hepatitis, celiac disease, alpha1-antitrypsin deficiency and cystic fibrosis. These causes were clinically ruled out due the absence of suggestive symptom including diarrhea and jaundice.
The initial symptoms in a child with alpha1-antitrypsin deficiency include cough, sputum production, and wheezing. The child may have history of treatment with multiple courses of antibiotics and evaluation for sinusitis.
In cystic fibrosis, the first feature to be noticed by the parents, in a baby having the disease, may be salty skin when kissed. Other features appear as the disease progressively involves the pulmonary and the digestive systems. The child may have chronic diarrhea and failure to gain weight or grow, due to malabsorption resulting from the blockage of pancreatic ducts. Frequent chronic coughing and difficulty in breathing results from respiratory tract blockage with thick mucous.
In early reports on cases of VKBD, intracranial bleeding (ICH) was observed in 65-100%, [12
] but in more recent reports in 30-60% [4
]. Other common sites being; gastrointestinal mucosa& cutaneous [3
]. Late VKBD has been also reported to manifest as nodular cutaneous purpura [13
]. However, no cases of orbital bleeding are reported to occur as a manifestation of VKDB.
Most of the cases of non-traumatic orbital hemorrhages are subperiosteal. A subperiosteal orbital hemorrhage detected on computed tomography is usually superior in site, and results in no visual compromise. These features allow conservative management [14
]. Orbital hematomas rarely cause visual deterioration, but reportedly an irreversible one, unless treated with early surgical decompression [15
In the case of this report immediate medical management of bleeding diathesis was lifesaving. However, conservative management alone was found to be ineffective to treat the intraorbital, large, organized hematoma. Surgical intervention was required to restore visual function or prevent further deterioration.