Irritable and listless infant!!!!!!!!!

[unseennude]

Parents bring their full-term female infant, aged 5 weeks, to the emergency department. The mother states that the baby had been doing well until a few days ago, when she started to become fussy and irritable, with vomiting and swelling around her right eye. She was feeding poorly yesterday and has been "tired" for the last several hours.

On physical examination, the infant is irritable and listless. She has mild swelling with ecchymosis around the right eye with an additional area of ecchymosis on the right knee. Her anterior fontanel is bulging, and her right pupil is dilated and unresponsive. Nonenhanced head CT is ordered, but during preparation for scanning, the patient develops shallow breathing with hypoxia, with an oxygen saturation of 80% on room air. This condition progresses to apnea and slowing of her heart rate to 60 beats per minute. Atropine is administered, and the patient is intubated.

Head CT is performed (see Image).



The patient's laboratory investigations show the following values: on CBC, WBC of 20.6 X 109/L (20,600/mm3), hemoglobin (Hb) 89 g/L (8.9 g/dL), hematocrit (Hct) 0.273 (27.3%), platelets 516 X 109/L (516 X 109/mL); BUN 5 mmol/L (14 mg/dL), creatinine 17.7 mmol/L (0.2 mg/dL), aspartate aminotransferase (AST) 121 U/L, alanine aminotransferase (ALT) 149 U/L, total protein 76 g/L (7.6 g/dL), albumin 46 g/L (4.6 g/dL); prothrombin time (PT) >50 s and activated partial thromboplastin time (aPTT) = 90.6 s.

What is your diagnosis?

Hint:The patient was a full-term female infant delivered at home and breastfed

[Oak]

I thinks its a case of Vitamin K Definciency.

[Soul]

Yes..subdural hematoma in this 5 wk old infant with prolonged PT n PTT suggests bleeding. But how to say vit K def? may be there is some bleeding disorder running in family. or congenital clotting factor deficiency?
As her ALT, AST are also elevated, so vit K deficiency due to liver disease?

[CSF]

sub dural hematoma is indicating towards the spontaneous bleeding which might be due to lack of coagulation factors as babies are aprticulary prone to deficiency of coagulation factors either lack of vit k or congenital deificiency of clotting factors like II or V or X .... whats the anser unseennude

[Oak]

Initially i though there would be some kind of infection because scenario depicts the signs of meningism but as plaletelets is normal, PTT and PT are prolong with subduarl hematoma and most of all ecchymosis which indicates the spontaneous bleeding thus, the cause is vit k deificiency. Becasue vit k deficiency is more common in home delivery.

I would be dead sure if unseennude could have told that delivery occured at home because if delivery occur at hospital child usually get a single dose of Vit K.

CT shows extensive infarct and subdural hematoma there is significant midline shifting in the uppr CT I think becuase of this changes might have result in signs of irritation.

So, its a case of vit k deficiency. See if it improves with a Vit K infusion, if not then as CSF said think about congenital deficiency of clotting factos.

But, I would go for vit k deficiency. whats the answer unseennude ?

[unseennude]

All of your differential diagnosis is correct. ( Vit K related Bleeding disorder and inherited disorder of clotting factor)
Thank you very much guys for your answer.

Bleeding related to vitamin K deficiency: Nonenhanced CT scan shows an acute right-sided subdural hematoma (SDH) (see Image 1) associated with extensive infarction involving the entire right cerebral hemisphere and the medial portion of the left frontal lobe. Extensive subfalcine herniation changes are present, with entrapment of the left foramen of Monro and enlargement of the left lateral ventricle. The sutures are separated due to the acute increase in intracranial pressure.

Given the prolonged PT of >50 s and the aPTT of 90.6 s, a bleeding disorder was determined to be the etiology of the patient's SDH. The differential diagnosis included vitamin K deficiency bleeding (VKDB) or an inherited deficiency of factor II, V, or X. To prepare for evacuation of her SDH, the patient was given vitamin K 2 mg intramuscularly (IM) and fresh frozen plasma (FFP) 20 mL/kg over 2 h. Surgical evacuation of the SDH was completed without complication except for excessive oozing for which intraoperative recombinant activated factor VII (Novo-7) 1200 mg was administered, with good effect. Repeat assessment of her PT and aPTT resulted in values of 8.3 and 24.6 s, respectively. Her coagulation parameters remained normal for the subsequent duration of the patient's hospitalization. On admission, the patient's factor II, VII, and X levels were <10% and her factor V level was 89% (within normal limits), and a diagnosis of VKDB was confirmed.

Vitamin K is central to the coagulation cascade, and its absence or consumption can result in bleeding. Vitamin K activates clotting factors II, VII, IX, and X, which are collectively known as the prothrombin complex, as well as anticoagulant proteins C and S. In mild or early stages of vitamin K deficiency, only the PT is substantially prolonged because of the short half-life of factor VII. However, in severe or prolonged deficiency, both PT and aPTT are markedly elevated because of the deficiency of factors II, IX, and X. In infants, vitamin K deficiency should be confirmed by the resolution of the bleeding symptoms and by the correction of PT and aPTT with the administration of vitamin K and by ruling out other causes of bleeding, such as inherited deficiency of common pathway factors (II, V, or X), liver disease, and disseminated intravascular coagulation (DIC). Assays of factors II, V, VII, and X help in differentiating congenital factor deficiency from vitamin K deficiency.

Newborns are particularly at risk for vitamin K deficiency because of poor placental transmissibility (30:1 maternal-infant gradient), absent hepatic menaquinones (vitamin K2 produced by gut bacteria), and low levels of vitamin K in breast milk (relatively low compared with supplemented infant formula). The deficiency leads to unexpected bleeding in otherwise healthy neonates if adequate vitamin K prophylaxis is not administered at birth.

Therapy for VKDB depends on the severity of the bleeding. Minor bleeding can effectively be corrected with IM or subcutaneous administration of vitamin K. Intravenous administration is not recommended because of a risk of anaphylactoid reactions. The PT and aPTT normalize 4-8 hours after the administration of vitamin K. In cases of severe or life-threatening bleeding (eg, ICH), the patient should receive FFP 10-15 mL/kg and/or prothrombin complex concentrate or recombinant factor preparations (eg, Novo-7) in addition to vitamin K.

The neurologic condition of the infant in this case improved rapidly after surgery. She was able to breastfeed well within 24 hours, and brain imaging studies obtained on days 2 and 8 after surgery showed no recurrence of the SDH (see Image 2 for CT obtained 2 days after surgery). Her neurologic status greatly improved by day 13, and she was discharged home with vitamin K supplementation 2 mg by mouth once a week until 6 months of age. The infant was born at home and therefore did not receive vitamin K prophylaxis at birth or any time during the 5 weeks before her presentation.

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