Bleeding Disorders in the Neonate

Summary
Introduction
Clinical presentation
Differential diagnosis
Laboratory investigations
Interpretation of Lab investigations in a bleeding neonate
Management

Summary

history, physical examination and determining whether the neonate is 'well' or 'sick' is helpful in assessment of a bleeding neonate
simple and widely available lab tests are useful in investigating a bleeding neonate
treatment depends on the cause of bleeding and clinical condition of the neonate
'treat the baby not the numbers'
consult a Paediatric Haematologist (ph (03) 9345 5522) if in doubt

Introduction

Neonates are susceptible to bleeding for various reasons

immaturity of the haemostatic system because of quantitative and qualitative deficiency of coagulation factors
maternal disease and drugs
birth trauma
other conditions eg sepsis and asphyxia

Clinical presentation

Bleeding in neonates may present with

oozing from the umbilicus or stump
cephalhaematoma
bruising more than that anticipated after delivery
bleeding from peripheral venipuncture or procedure sites
bleeding into scalp
bleeding following circumcision
petechiae
intracranial haemorrhage
bleeding from mucous membranes
unexplained anaemia and hypotension

A detailed history and complete examination is essential in the assessment of a bleeding neonate.

Particular points in the history include

maternal diseases such as ITP, preeclampsia and diabetes
maternal exposure to drugs such as aspirin, anticonvulsants, rifampicin and isoniazid
family history of bleeding disorders
previous affected siblings
confiration of Vitamin K administration

Physical examination will determine whether the neonate is 'well' or 'sick', which is very useful as the differential diagnosis is very different in the two circumstances.

Differential diagnosis

Causes of bleeding in a 'well' neonate

immune thrombocytopenia (alloimmune or autoimmune (maternal ITP))
vitamin K deficiency
inherited coagulation factor deficiencies such as haemophilia
bleeding from anatomic lesions such as a haemangioma, A-V malformation

Causes of bleeding in a 'sick' neonate

DIC - usually associated with sepsis, asphyxia, severe RDS or NEC
consumption thrombocytopenia without depletion of coagulation factors
liver failure

Bleeding at a single site is more likely to have an anatomic or structural component.

Major bleeding from any primary cause may induce a secondary DIC, which may mask the original pathology.

Laboratory investigations

first line tests include
- platelet count
- APTT
- PT
- fibrinogen
- d-dimer

The results should be interpreted in the context of normal laboratory values for gestation and postnatal age.

Interpretation of Lab investigations in a bleeding neonate

Isolated prolonged APTT, consider
- factors XII, IX, XI and VIII deficiencies
- heparin
- early DIC
Isolated prolonged PT, consider
- early DIC
- liver disease
- vitamin K deficiency
- factor VII deficiency
Combined prolonged APTT and PT+/- low fibrinogen, consider
- DIC
- liver disease
- vitamin K deficiency
- rarely inherited factor deficiency e.g. prothrombin deficiency
Normal APTT, PT, platelet count, fibrinogen, consider
- factor XIII deficiency
- platelet function defect
- A-V malformation
- severe neutropenia (bleeding from umbilical stump)

Von Willebrand Disease rarely presents in the newborn period.

Factor XII deficiency causes a prolonged APTT, but no clinical bleeding.

Management

Management depends on

the cause of bleeding and
clinical condition of the neonate

DIC

treat the primary condition
blood products i.e. platelets and FFP are used on clinical grounds
- 10-15 ml/ kg of FFP can be given to correct coagulation abnormality
- if platelets <50 and actively bleeding, transfuse platelets

Vitamin K deficiency

IV vitamin K1, 1mg is usually effective within hours
also give FFP 10-15 mls/kg to immediately increase the levels of clotting factors

Thrombocytopenia - Refer to Thrombocytopenia

Inherited factor deficiency

initial therapy with FFP after blood taken for specific factor assays
specific factor replacement when diagnosis known