Definition

Haemorrhagic diseases of the newborn refer to a group of conditions characterized by abnormal bleeding or haemorrhage in newborn infants.

This syndrome is defined by spontaneous internal or external bleeding in neonates. This is a result of decreased activity of several clotting factors and diminished platelet function.

This is an uncommon condition in which the baby bleeds from some of its internal organs. It occurs due to deficiency of the vitamin K dependent clotting factors. Its decline in incidence is due to the routine administration of vitamin K at birth.

Mostly the organs of the alimentary tract are affected.

Bleeding that usually occurs between the 2nd and 5th day of the baby’s life, the condition is known as HYPOTHROMBINEMIA.

Bleeding may occur from various sites in newborns, categorized as follows:

Common Sites of Bleeding

Common Sites: Alimentary Tract

• Intestines: Dark black blood may be passed in the stools via the rectum, known as MELENA, which is the most common presentation.
• Stomach: Fresh, unaltered blood or brown-colored fluid may be vomited, termed HAEMATEMESIS.
• Umbilicus: Fresh blood may be observed.

Less Common Sites

• Urinary Tract: Blood may be passed in the urine through the urethra, referred to as HAEMATURIA.
• Genital Tract: Bleeding through the vagina, typically due to estrogen hormone effects (pseudo menstruation).
• Respiratory Tract: Bleeding from the lungs, known as HAEMOPTYSIS.
• Brain Ventricles: Bleeding from these sites.
• Peritoneal Cavity: Bleeding into the abdominal cavity, which is a serious condition.

Causes of Haemorrhagic Diseases

(A) Abnormalities of Clotting Factors

• Vitamin K-dependent factor deficiencies: Deficiencies of factors II, VII, IX, and X are most common. This usually manifests between days 2 and 5 of life and is more frequent in preterm and breastfed infants. Protein C deficiency can also contribute.
• Drug-induced effects: Maternal ingestion of certain medications during pregnancy, including phenytoin, warfarin compounds, rifampicin, and salicylates, can interfere with vitamin K metabolism and function.

(B) Abnormalities in Clotting

• Disseminated Intravascular Coagulation (DIC): This complex disorder involves inappropriate activation of the clotting cascade, leading to simultaneous clotting and bleeding. Underlying causes include infection (sepsis), hypoxia (lack of oxygen), shock, and necrotizing enterocolitis (NEC).

(C) Platelet Problems

• Qualitative defects (thrombasthenia): Impaired platelet function.
• Quantitative defects (thrombocytopenia): Low platelet count, reducing the blood’s ability to form clots. Causes include immune thrombocytopenic purpura (ITP), infections, and inherited disorders.

(D) Inherited Abnormalities of Blood Coagulation

Inherited disorders affecting the production of specific clotting factors (e.g., haemophilia A, haemophilia B, von Willebrand disease).

• Haemophilia A: Decreased levels of Factor VIII.
• Hemophilia B (Christmas disease): Deficiency of Factor IX.
• Von Willebrand Disease (VWD): Reduced levels and functional activity of Von Willebrand factor (VWF).

(E) Trauma

• Obstetric trauma: Can result in cephalhematoma (bleeding between the skull and periosteum), visceral injuries (liver, spleen rupture), umbilical cord rupture, or slippage of the cord ligature.

(F) Other Factors

• Liver dysfunction: Impaired liver function affects the synthesis of clotting factors, leading to bleeding. Causes include sepsis, hypoxia, and inherited metabolic disorders.
• Vitamin K deficiency: Particularly prevalent in breastfed infants due to limited stores at birth.

Others include:

• Birth Trauma: Trauma during childbirth, especially in cases of prolonged labor or difficult deliveries, can lead to bleeding in various parts of the body.
• Infections: Certain infections contracted during pregnancy or after birth, such as sepsis or congenital cytomegalovirus (CMV) infection, can cause hemorrhagic complications in newborns.
• Gastrointestinal Conditions: Conditions affecting the gastrointestinal tract, such as necrotizing enterocolitis (NEC) or gastric ulcers, may lead to gastrointestinal bleeding in newborns.
• Respiratory Issues: Respiratory conditions like respiratory distress syndrome (RDS) or pneumonia can sometimes result in bleeding from the respiratory tract.
• Congenital Abnormalities: Structural abnormalities or malformations in organs or blood vessels, such as congenital heart defects or vascular malformations, may predispose newborns to bleeding disorders.
• Maternal Factors: Certain maternal conditions or exposures during pregnancy, such as maternal drug use, alcohol consumption, or maternal hypertension, can increase the risk of hemorrhagic diseases in newborns.
• Sepsis: Infection leads to DIC and other clotting abnormalities.
• Hypoxia: Reduced oxygen levels can damage the liver and other organs involved in coagulation.
• Vascular Malformations: Abnormal blood vessels can lead to easy bruising and bleeding.
• Acquired Disorders: Conditions such as leukemia or other malignancies can affect the blood’s ability to clot.
• Maternal Medications: Some medications taken during pregnancy can affect the baby’s clotting factors (e.g., certain anticonvulsants).
• Inherited Metabolic Disorders: Conditions affecting metabolism can indirectly influence clotting.

Early Warning Signs

Prolonged bleeding from: a. Injection site. b. After circumcision, as seen in certain cultural practices. c. Oozing from a firmly ligatured cord.

• Bruising or petechiae (small red or purple spots) on the skin.
• Excessive bleeding from minor cuts or abrasions.
• Persistent bleeding from the umbilical stump beyond the usual period.

Late Warning Signs

• Passing blood in stool (melena), which can be distinguished from meconium by observing the colour change when placed in water.
• Vomiting blood (hematemesis).
• Signs of internal haemorrhage, especially in severe cases with excessive vomiting.
• Paleness of the face and lips (pallor).
• Lethargy or limpness in the cot.
• Signs of shock, such as rapid heartbeat, rapid breathing, and cold or clammy skin.
• Swelling or bulging of the fontanelle (soft spot on the baby’s head), indicating increased intracranial pressure.

Assessment/Diagnosis/Investigations

• Detailed History: Including maternal history (medications, illnesses), birth history (trauma, asphyxia), and infant’s symptoms (bleeding sites, severity).
• Physical Examination: Assess for bruising, petechiae (pinpoint haemorrhages), purpura (larger haemorrhages), bleeding from the umbilical cord or other sites.
• Laboratory Tests:
  • Complete blood count (CBC): To evaluate platelet count, haemoglobin, and hematocrit. Significant bleeding is often associated with platelet counts below 20,000–30,000/mm³.
  • Blood smear: To assess platelet morphology and presence of other abnormalities.
  • Reticulocyte count: To assess bone marrow response.
  • Prothrombin time (PT): Measures the extrinsic coagulation pathway.
  • Partial thromboplastin time (PTT): Measures the intrinsic coagulation pathway.
  • Fibrinogen levels: Assess the level of this essential clotting protein.
  • D-dimer test: Elevated levels suggest DIC (normal level < 0.5 µg/mL).
  • Specific coagulation factor assays: To identify specific factor deficiencies (e.g., Factor VIII, IX, VWF).
  • Von Willebrand panels: For suspected VWD.

Management

Presence of streaks of blood in the vomitus or stools of a neonate should put the midwife/nurse on the alert.

A. Maternity Centre

Transfer the baby to hospital without delay because 30mls of blood from a baby is the same as 500mls lost in an adult.

While waiting for transport do the following: a. Keep the baby lying in a warm cot. b. Provide warmth and maintain hydration: Prevent hypothermia and dehydration which worsen bleeding. c. Examine the mother’s breasts for cracked nipples. If nipples are cracked, the baby must not continue breastfeeding. d. Gentle handling: Minimize trauma to prevent further bleeding. e. Re-assure the mother and treat the cracked nipples. f. If no cause is found do the following:

• Give infant chloral hydrate 30mgs (4mls) orally.
• Give injection of Vitamin K 0.5-1 mg intramuscularly depending on the maturity of the baby.
• Observe the general condition of the baby and record your findings. Stop breastfeeding as suckling increases bleeding.
• Write a detailed note to take to the hospital.
• Continue to reassure the mother. g. Support vital signs: Monitor heart rate, respiratory rate, blood pressure, oxygen saturation. h. Refer immediately: Any significant bleeding (intracranial hemorrhage suspected, significant blood loss), inability to control bleeding with Vitamin K, or suspicion of other underlying causes necessitate urgent referral to a higher-level facility.

B. Hospital

• Inform the Dr.
• Intramuscular injection of Vitamin K 1mg.
• Mist chloral hydrate 30 mgs of phenobarbital 7.5-8 mg 6 hourly.
• The baby may be fed in the absence of haematemesis.
• In haematemesis it is advisable to withhold feeds and give the baby subcutaneous or intravenous fluids.
• Urgent grouping and cross matching plus estimation of haemoglobin are done.
• Intravenous infusion drip may be put up.
• In severe cases the baby is transfused with fresh blood.
• A close observation is kept on the baby noting the color, pulse and bleeding site.
• Vitamin C is given to repair worn out cells.

Specific Management

Treatment depends heavily on the underlying cause: a. Vitamin K₁ (Aquamephyton): 1 mg IV or IM, potentially repeated weekly. b. Fresh Frozen Plasma (FFP): 10 mL/kg IV, potentially repeated. Replaces clotting factors immediately. c. Platelet Concentrates: To raise platelet count from 50,000/mm³ to 100,000/mm³. d. Fresh Whole Blood: 10 mL/kg or more may be used for single transfusions in cases of significant blood loss. e. Clotting Factor Concentrates: Administered for known deficiencies (e.g., Factor VIII, IX, VWF) to control bleeding. f. Treatment of Specific Problems: Address underlying conditions such as sepsis or DIC with appropriate therapies.

Management of Cord Bleeding

• Slipping ligature: Religation is necessary.
• Bleeding after cord separation: Hemostasis can be achieved through pressure or ligature after securing bleeding points with artery forceps.

Even minimal blood loss can be critical; blood transfusion may be required.

Prevention

All premature babies and any suspected cases of internal haemorrhage must be given injection Vitamin K ¼-1 mg intramuscularly.

1. Ensure Proper Handling: Handle neonates with care, avoiding any unnecessary trauma to the baby’s body, especially the abdomen and head, which can lead to bleeding issues.
2. Early Detection: Train healthcare professionals and caregivers to recognize early signs of bleeding disorders in neonates, such as prolonged bleeding from injection sites or circumcision wounds.
3. Maternal Health: Provide proper antenatal care to pregnant women to identify any maternal conditions that may increase the risk of bleeding disorders in newborns.
4. Vitamin K Supplementation: Administer prophylactic Vitamin K to newborns shortly after birth to prevent Vitamin K deficiency bleeding, a common cause of bleeding disorders in neonates.
5. Avoidance of Medications: Educate healthcare providers and caregivers about the potential risks of medications that can increase the risk of bleeding disorders in neonates, such as certain anticoagulants or drugs that interfere with platelet function.
6. Optimal Cord Care: Ensure proper cord care practices, including keeping the umbilical cord clean and dry to prevent umbilical infections, which can lead to bleeding complications.
7. Prompt Medical Attention: Encourage caregivers to seek immediate medical attention if they notice any signs of bleeding in the neonate, such as blood in the stool or vomit, so that appropriate medical intervention can be initiated promptly.