Severe aortic thrombosis in a newborn diagnosed at birth
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《美国医学杂志》
Department of Cardiology, All India Institute of Medical Sciences, Ansari nagar, New Delhi-110029, India
Aortic thrombosis in the neonate is a rare entity with high mortality. Prognosis is determined by the severity of aortic thrombosis, time to diagnosis and the treatment regimen used.[1] We report a newborn, who presented with evidence of aortic thrombosis at birth.
A 36-week preterm female baby was born to a 22-year-old gravida 2, para 1 mother, delivered by lower segment cesarean section in a private hospital. The antenatal course was complicated by oligohydramnios and intrauterine growth retardation. The baby at birth weighed 2.26 kg and was noted to have bilateral pale lower limbs. She had absent pulses in the lower limbs bilaterally and her left arm mean arterial pressure was 49 mm Hg. Doppler examination revealed an occlusive thrombus in descending aorta at its bifurcation. She was treated with a loading dose of intravenous urokinase 4000 U/Kg and heparin infusion 20 U/Kg/hour for 12 hours. The patient's condition improved during the second day and she was referred to our centre.
At admission general condition of baby was stable, her pulse was 122 beats per minute, respiration was 32 breaths per minute and oxygen saturation was 96 % in room air in upper limbs and 88% in lower limbs. She had pink bilateral lower limbs but still absent bilateral femoral and other lower limb pulses. She had blood pressures of 78/52 mmHg in right arm and 36/16 mmHg in right leg. Computed tomography angiogram demonstrated an infrarenal aortic thrombus with reformation of bilateral external iliac arteries Figure1. There was no history of umbilical artery catheterization or maternal diabetes. Family history of thrombosis was absent. Two-dimensional echocardiography revealed a structurally normal heart. Laboratory investigations including complete hemogram, aspartate aminotransferase, alanine aminotransferase and serum electrolytes were within the normal range. The septic screen was negative. Coagulation profile revealed prothrombin time 11s (control: 12s), international normalized ratio (INR) 1.1, activated partial thromboplastin time 32s (control: 64s) and D-dimer 1.27 mg/mL (normal, <0.5mg/mL). Detailed evaluation for hypercoagulable state revealed no abnormalities. Assays for Protein C and Protein S were normal; and negative for antiphospholipid antibodies and homocysteine.
Baby was discharged on oral warfarin on day 11 of life. She is on follow up and doing well. The lower limb pulses are still absent but there is no evidence of limb ischemia.
Several cases of aortic thrombosis have been reported in English literature, none has been reported in Indian literature to the best of our knowledge. Most of the described cases of aortic thrombosis are associated with the catheterization of an umbilical artery. The other risk factors at this age are perinatal asphyxia, maternal diabetes, sepsis, polycythemia, thrombophilia, dehydration and congenital heart disease.[1], [2] We considered oligohydramnios as a probable cause of aortic thrombosis in this case after ruling out other causes.
All infants with aortic thrombosis should be considered for treatment regardless of severity, as this is potentially life-threatening disease. Therapeutic options include unfractionated heparin, low molecular weight heparin, fibrinolysis and surgery. Degree of vascular compromise resulting from aortic obstruction, patient's clinical condition and the underlying cause of thrombosis should guide the choice of treatment. In stable patients without severe vascular compromise, use of unfractionated heparin or low molecular weight heparin may be adequate.[2] If aortic obstruction is complete and the vascular compromise is severe, fibrinolysis or surgery should be considered. Streptokinase has been used successfully[3] but found to be less effective than other fibrinolytic agents, and is associated with significant side effects. Therefore, streptokinase is not recommended for use in neonates[2]. Fibrinolytic treatment with urokinase or tissue plasminogen activator is safe and a better alternative; can be implemented initially, or added if progression of symptoms is observed with use of anticoagulation alone.[4],[5] When there is no response to fibrinolytic treatment or if there are any contraindications to medical treatment, surgical thrombectomy is indicated. The significant mortality and high morbidity involved with surgery prohibits this mode of treatment to be the primary approach for all infants with aortic thrombosis.[6] Randomized controlled trials are needed to determine whether unfractioned heparin, low molecular weight heparin, fibrinolysis or surgery is the optimal treatment for severe aortic thrombosis.
References
1. Nowak-Gottl U, Von Kries R, Gobel U. Neonatal symptomatic thromboembolism in Germany: two year survey. Arch Dis Child Fetal Neonatal Ed 1997; 76: F163-167.
2. Klinger G, Hellmann J, Daneman A. Severe aortic thrombosis in the neonate -successful treatment with low-molecular-weight heparin: two case reports and review of the literature. Am J Perinatol 2000; 17 : 151-158.
3. Kirk CR, Bhrolchain CN, Qureshi SA. Streptokinase for aortic thrombosis. Arch Dis Child 1988; 63: 1086-1087.
4. Hustead VA, Wicklund BM. Treatment of neonatal aortic thrombosis with urokinase. Am J Pediatr Hematol Oncol 1990; 12: 336-339.
5. Kennedy LA, Drummond WH, Knight ME, Millsaps MM, Williams JL. Successful treatment of neonatal aortic thrombosis with tissue plasminogen activator. J Pediatr 1990; 116: 798-801.
6. McFaul RC, Keane JF, Nowicki ER, Castaneda AR. Aortic thrombosis in the neonate. J Thorac Cardiovasc Surg 1981; 81 : 334-337.(Sahu Jitendra K, Saxena A)
Aortic thrombosis in the neonate is a rare entity with high mortality. Prognosis is determined by the severity of aortic thrombosis, time to diagnosis and the treatment regimen used.[1] We report a newborn, who presented with evidence of aortic thrombosis at birth.
A 36-week preterm female baby was born to a 22-year-old gravida 2, para 1 mother, delivered by lower segment cesarean section in a private hospital. The antenatal course was complicated by oligohydramnios and intrauterine growth retardation. The baby at birth weighed 2.26 kg and was noted to have bilateral pale lower limbs. She had absent pulses in the lower limbs bilaterally and her left arm mean arterial pressure was 49 mm Hg. Doppler examination revealed an occlusive thrombus in descending aorta at its bifurcation. She was treated with a loading dose of intravenous urokinase 4000 U/Kg and heparin infusion 20 U/Kg/hour for 12 hours. The patient's condition improved during the second day and she was referred to our centre.
At admission general condition of baby was stable, her pulse was 122 beats per minute, respiration was 32 breaths per minute and oxygen saturation was 96 % in room air in upper limbs and 88% in lower limbs. She had pink bilateral lower limbs but still absent bilateral femoral and other lower limb pulses. She had blood pressures of 78/52 mmHg in right arm and 36/16 mmHg in right leg. Computed tomography angiogram demonstrated an infrarenal aortic thrombus with reformation of bilateral external iliac arteries Figure1. There was no history of umbilical artery catheterization or maternal diabetes. Family history of thrombosis was absent. Two-dimensional echocardiography revealed a structurally normal heart. Laboratory investigations including complete hemogram, aspartate aminotransferase, alanine aminotransferase and serum electrolytes were within the normal range. The septic screen was negative. Coagulation profile revealed prothrombin time 11s (control: 12s), international normalized ratio (INR) 1.1, activated partial thromboplastin time 32s (control: 64s) and D-dimer 1.27 mg/mL (normal, <0.5mg/mL). Detailed evaluation for hypercoagulable state revealed no abnormalities. Assays for Protein C and Protein S were normal; and negative for antiphospholipid antibodies and homocysteine.
Baby was discharged on oral warfarin on day 11 of life. She is on follow up and doing well. The lower limb pulses are still absent but there is no evidence of limb ischemia.
Several cases of aortic thrombosis have been reported in English literature, none has been reported in Indian literature to the best of our knowledge. Most of the described cases of aortic thrombosis are associated with the catheterization of an umbilical artery. The other risk factors at this age are perinatal asphyxia, maternal diabetes, sepsis, polycythemia, thrombophilia, dehydration and congenital heart disease.[1], [2] We considered oligohydramnios as a probable cause of aortic thrombosis in this case after ruling out other causes.
All infants with aortic thrombosis should be considered for treatment regardless of severity, as this is potentially life-threatening disease. Therapeutic options include unfractionated heparin, low molecular weight heparin, fibrinolysis and surgery. Degree of vascular compromise resulting from aortic obstruction, patient's clinical condition and the underlying cause of thrombosis should guide the choice of treatment. In stable patients without severe vascular compromise, use of unfractionated heparin or low molecular weight heparin may be adequate.[2] If aortic obstruction is complete and the vascular compromise is severe, fibrinolysis or surgery should be considered. Streptokinase has been used successfully[3] but found to be less effective than other fibrinolytic agents, and is associated with significant side effects. Therefore, streptokinase is not recommended for use in neonates[2]. Fibrinolytic treatment with urokinase or tissue plasminogen activator is safe and a better alternative; can be implemented initially, or added if progression of symptoms is observed with use of anticoagulation alone.[4],[5] When there is no response to fibrinolytic treatment or if there are any contraindications to medical treatment, surgical thrombectomy is indicated. The significant mortality and high morbidity involved with surgery prohibits this mode of treatment to be the primary approach for all infants with aortic thrombosis.[6] Randomized controlled trials are needed to determine whether unfractioned heparin, low molecular weight heparin, fibrinolysis or surgery is the optimal treatment for severe aortic thrombosis.
References
1. Nowak-Gottl U, Von Kries R, Gobel U. Neonatal symptomatic thromboembolism in Germany: two year survey. Arch Dis Child Fetal Neonatal Ed 1997; 76: F163-167.
2. Klinger G, Hellmann J, Daneman A. Severe aortic thrombosis in the neonate -successful treatment with low-molecular-weight heparin: two case reports and review of the literature. Am J Perinatol 2000; 17 : 151-158.
3. Kirk CR, Bhrolchain CN, Qureshi SA. Streptokinase for aortic thrombosis. Arch Dis Child 1988; 63: 1086-1087.
4. Hustead VA, Wicklund BM. Treatment of neonatal aortic thrombosis with urokinase. Am J Pediatr Hematol Oncol 1990; 12: 336-339.
5. Kennedy LA, Drummond WH, Knight ME, Millsaps MM, Williams JL. Successful treatment of neonatal aortic thrombosis with tissue plasminogen activator. J Pediatr 1990; 116: 798-801.
6. McFaul RC, Keane JF, Nowicki ER, Castaneda AR. Aortic thrombosis in the neonate. J Thorac Cardiovasc Surg 1981; 81 : 334-337.(Sahu Jitendra K, Saxena A)