신생아에서의 뇌정맥동 혈전증의 임상 특징 및 예후

Abstract

"- ABSTRACT -

Clinical Characteristics and Prognosis of Cerebral Sinovenous Thrombosis in Neonate

Yun Ju Lim

Department of Medical Sciences

The Graduate School, Ajou University

(Supervised by Professor, Moon Sung Park)

Objective: Cerebral Sinovenous Thrombosis (CSVT) is a rare disorder in pediatric patients with a high risk of an adverse neurodevelopmental outcome. It has been reported that the neonatal incidence is approximately 0.41 per 1000 live births, but the value might be underestimated because of unspecific clinical presentation and difficulties in radiological diagnosis in neonatal patients. Early diagnosis and treatment of CSVT in neonatal patient is critical to achieve better neurodevelopmental outcomes. With recent advance in radiologic technologies, earlier diagnosis makes better survival rate and outcome of the disease. In this study, we describe clinical presentations,sequential neuroimaging finding and outcomes in Korean neonates of CSVT.

Subjects and methods: We found ten neonates with CSVT admitted to the neonatal intensive care unit at Ajou university hospital from January 1996 to February 2008 and reviewed their medical records retrospectively. Initial diagnosis was made by MRI / MR venography and follow up studies were done at 1 week to 2 months interval. Neurodevelopmental outcome was assessed by BSID-II at 5 months to 6 years of age.

Result: All ten patients were admitted between 1 to 5 days after birth and their mean gestational age and mean birth weight were 37.9±2.2 weeks and 2.9±0.7 kg respectively. The main clinical features were prolonged lethargy, hypotonia, and seizure.Laboratory tests were not remarkable including coagulation tests. The locations of thrombosis were seven superior sagittal sinus, three right transverse sinus, two left transverse sinus, and one deep straight sinus according to initial brain MRI and MR venogram. Patients were managed conservatively except three neonates received intravenous heparin injection for 3 to 5 days (75 IU/kg, bolus) without any complications. Among ten cases, one died of severe occipital hemorrhageand one was lost during follow up. Within 4 weeks from initial study MRI / MR venogram were carried out for eight patients and no thrombosis were detected. The neurodevelopmental outcomes were assessed with Bayley standard developmental examination. Except one epileptic patient, 7 patients showed delayed motor and cognitive development.

Conclusion: Although our result could be overestimated due to the nature of retrospective study, most of patients with CSVT had neurologic and/or developmental sequels. But considering the severity of the disease, we may suggest that early intervention and active management could be needed for earlier diagnosis and better outcome.

"