Dear Editor,

We read with interest the article by Beattie et al. in a recent issue of Archives. [1] They reported a clinical course of a neonate initially presenting with oozing, rectal bleeding and thrombocytopenia on the 2nd day of life. Inferior vena cava (IVC) thrombosis was detected on the 5th day. A first cranial ultrasound (US) was performed on the same day and showed a left middle cerebral artery (MCA) territory infarct; echocardiogram revealed left to right shunt across the patent foramen oval (PFO). On the 7th day a subclavian steal syndrome was also diagnosed.

This report highlights the thrombo-embolic genesis of neonatal stroke and, for the first time, describes the insurgence of subclavian steal syndrome in a newborn infant.

We would like to offer some comments about this interesting case.

Embolization through PFO is supposed to be the cause of left MCA infarct, but this cannot be surely demonstrated. The neonate has always been asymptomatic and the diagnosis of stroke was made on the 5th day of life, when a cerebral US scan was performed for the first time. As a matter of fact, although the baby had been presenting with thrombocytopenia and bleeding since the 2nd day of life, cranial US was done only when an IVC thrombosis was detected.

From this clinical course it is very difficult to ascertain the origin of the stroke: could the thrombocytopenia be due to a thrombus creation in the middle cerebral artery?. Only performing a cerebral US scan before the diagnosis of deep venous thrombosis could have verified this possibility.

Given that, it is not possible to surely ascribe the stroke aetiology to the paradoxical embolism, rather than to a MCA thrombosis: we do consider as mandatory to evaluate with cranial US all neonates with thrombocytopenia and not only those with venous thrombosis.

Moreover, we think that the cause of thrombosis in such a case should be better clarified. Since the mother was positive for lupus anticoagulant (LAC), the baby correctly underwent the study for LAC. Nevertheless, in neonates with thrombosis a complete screening panel for thrombophilia has been recommended [2]. It includes, in two levels, several tests including prothrombin or factor V mutation and not only the dosage of LAC or anticardiolipin antibodies of maternal origin. [2] Risk for thrombosis in neonates from mothers with antiphospholipid syndrome (APLA) is well known and these pregnancies should be carefully monitored and managed. [3,4]

Thromboses may involve not only the venous district, as cited by Beattie et al., [1] but also the cerebral arterial district. In fact, ten cases of neonatal arterial thrombosis (of which 6 cases affecting the cerebral arterial territory) related to maternal APLA have been published. [5] An European Register for foeto-neonatal thrombosis in these babies has been recently opened and we are involved in data collection. [5] A better definition and data collection of such cases is warranted.

References:

[1] Beattie LM, Butler SJ, Goudie DE. Pathways of neonatal stroke and subclavian steal syndrome. Arch Dis Child Fetal Neonatal Ed 2006; 91: F204 -F207. doi: 10.1136/adc.2005.079830

[2] Manco-Johnson M, Nuss R. Neonatal thrombosis disorders. Neoreviews 2000; 10: e201-e205.

[3] Caruso A, De Carolis S, Di Simone N. Antiphospholipid antibodies in obstetrics: new complexities and sites of action. Hum Reprod Update 1999; 2: 267-276.

[4] De Carolis S, Ferrazzani S, De Stefano V, et al. Inherited thrombophilia: treatment during pregnancy. Fetal Diagn Ther. 2006; 21:281- 6.

[5] Boffa MC, Aurousseau MH, Lachassinne E, et al. European register of babies born to mothers with antiphospholipid syndrome. Lupus 2004; 13: 713 -7.