MRI in Neonatal Dural Sinus Thrombosis raphy (CT), and most recently magnetic resonance imaging ( M R I ) . W e r e p o r t a t e r m n e w b o r n w i t h seizures a n d a Rami Grossman, MD*, Gerald Novak, MD*, Mahendra Patel, MD*, Joseph Maytal, MD*, Jose Ferreira, MD*, and Lydia Eviatar, MD* This 10-day-old female infant was the 3,629 gm product of an uncomplicated 42-week gestation to a 38-year-old G6,P3 mother who had 3 prior first trimester spontaneous abortions. There were no immediate postnatal complications. On the eighth day of life, the infant developed loose stools. The next day, 2 brief (<1 min) episodes of right arm twitching were observed. On the day of admission, another brief episode of twitching of the fight ann, face, and leg occurred, followed by 20 min of lethargy. Another 1 min episode occurred in the emergency room. There was no history of trauma. The family history was remarkable for a 71/2-year-old brother who developed staphylococcal sepsis and necrotizing enterocolitis on the second day of life. On physical examination, the patient was alert, pink, active, and afebrile. The weight was 3,742 gm and head circumference 36.75 cm (95th percentile). The anterior fontanel was open and flat. She was well hydrated. The funduscopic examination, muscle tone, deep tendon reflexes, and Moro reflex all were normal. Initial complete blood count, hematocrit, serum electrolytes, serum glucose, bilirubin, calcium, and magnesium all were within normal limits. Lumbar puncture yielded grossly bloody cerebrospinal fluid with 28,000 erythrocytes/mm3 and 22 leukocytes/mm3 (53% segmented neutrophils, 47% monocytes); the supernatant was xanthochromic. Serum glucose concentration was 50 mg/dl and total protein 111 mg/dl. No organisms were observed with Gram staining and cultures were negative. PT, PTI', AT-III, fibrinogen, protein-C, and protein-S levels were within normal limits. EEG was normal. Maternal anticardiolipin antibodies were negative. Unenhanced cranial CT revealed a hyperdense internal cerebral vein, vein of Galen, straight sinus, and superior sagittal sinus without any CT evidence of subarachnoid hemorrhage or venous infarction (Figs 1A,1B). MRI confirmed the CT findings; hyperintense signal appeared in the same venous structures on Tl-weighted images. These findings were persistent on the coronal Tl-weighted MRI and T2-weighted images. No evidence of any parenchymal pathology existed (Figs 1C,1D). On the eighth day of admission a repeat MRI demonstrated partial resolution of the DST. By the second day after admission, the patient was active and feeding well. She was discharged in good condition 8 days after admission. A repeat MRI at 30 days of age was entirely normal (Fig 2). The physical examination, including head circumference, was normal as well. The patient was examined in clinic 1 year later and was neurologically normal and had a normal head circumference. Dural sinus thrombosis in the newborn period is a rare but underrecognized condition which may cause seizures, macrocephaly, lethargy, and respiratory depression. A 10-day-old term infant with no pre- or perinatal risk factors for thrombosis presented with seizures and was found to have dural sinus thrombosis on computed tomography and magnetic resonance imaging (MRI). One week later, MRI revealed partial resolution and 3 weeks later disclosed a complete resolution of the thrombosis. Clinicians should consider the diagnosis of neonatal dural sinus thrombosis in infants presenting with seizures and/or increased intracranial pressure even in the absence of risk factors or when the cranial computed tomography is normal. M R I is the most sensitive diagnostic tool to establish the diagnosis and permit a noninvasive follow-up, contributing to our understanding of the natural history, associated pathology, and prognosis of this condition. G r o s s m a n R, N o v a k G, Patel M , M a y t a l J, F e r r e i r a J, E v i a t a r L. M R I in n e o n a t a l d u r a l s i n u s t h r o m b o s i s . Pediatr Neurol 1993;9:235-8. Introduction D u r a l sinus t h r o m b o s i s ( D S T ) r e p r e s e n t s a rare b u t u n d e r r e c o g n i z e d c a u s e o f seizures in t e r m infants. B e f o r e 1980, it w a s d e s c r i b e d o n l y in a u t o p s y studies o f a s p h y x i a t e d n e w b o r n s [1,2]. L i t e r a t u r e r e v i e w r e v e a l e d o n l y a f e w r e c e n t c a s e reports d e s c r i b i n g sinus t h r o m b o s i s in n e w b o r n s [3-16]. D i f f e r e n t d i a g n o s t i c p r o c e d u r e s h a v e b e e n s u g g e s t e d as c o n f i r m a t o r y , i n c l u d i n g c a r o t i d a n g i o g raphy, digital s u b t r a c t i o n a n g i o g r a p h y , c o m p u t e d t o m o g - From the *Division of Pediatric Neurology; Schneider Children's Hospital; and *Department of Radiology; Long Island Jewish Medical Center;Long Island Campus for the Albert Einstein College of Medicine; New Hyde Park, New York. s u b a r a c h n o i d h e m o r r h a g e d u e to DST, a n d r e v i e w prev i o u s r e p o r t s o f n e o n a t a l D S T a n d the d i a g n o s t i c m o d a l ities used. Case Report Discussion D S T h a s b e e n d e s c r i b e d i n c h i l d r e n as a c o m p l i c a t i o n of sinusitis, meningitis, dehydration, hypercoagulable states, n e o p l a s m s , i n t r a c r a n i a l surgery, a n d h o m o c y s t i n uria [14]. F r o m t h e first p a t h o l o g i c s t u d y b y B a i l y a n d H a s s in 1931 [15] t h r o u g h t h e early 1980s, t h e r e w e r e n o clinical Communications should be addressed to: Dr. Grossman; Division of Pediatric Neurology, Room 267; Schneider Children's Hospital; Long Island Jewish Medical Center; New Hyde Park, NY 11042. Received August 28, 1992; accepted November 22, 1992. Grossman et al: Dural Sinus Thrombosis 235 Figure 1. (A,B) Unenhanced axial CT scans. (C,D) Midline sagittal Tl-weighted MR1 scans without gadolinium. Demonstrating hyperdense (1) internal cerebral vein, (2) vein of Galen, (3) straight sinus, and (4) superior sagittal sinus on CT and hyperintensity of the same venous structures on MRI due to extensive midline dural venous sinus and deep venous thrombosis. reports of neonatal DST, in spite of its frequent occurrence (about 40%) in some autopsy studies of asphyxiated newborns [1,2]. In the last 4 years more than 30 patients with neonatal DST have been reported, presenting with a variety of signs, including macrocrania, bulging anterior fontanet, irritability, and respiratory depression. Most of these patients had a very good outcome, correlating mainly with the Apgar score at birth, and the severity of the underlying disorders. All reported patients with neonatal DST in all languages, are summarized in Table 1, describing the clinical presentation and modalities used to make the diagnosis. Shevell and Silver reviewed the largest s e r i e s - 1 7 patients [3]. These patients were identified by a retrospective review of unenhanced CT scans, which revealed a Figure 2. (A,B) Midline sagittal Tl-weighted MRI scans (30 days of life). Complete resolution of venous sinus and deep venous thrombosis. 236 PEDIATRICNEUROLOGY Vol.9 No. 3 Table I. Literature review of infants with neonatal DST Reference No. of Patients Imaging Study Diagnostic for DST AngioSone~ CT MRI gram gram Presentation Patronas and Duda [5] 1 with vomiting, diarrhea, seizures; 1 with fever, vomiting, diarrhea, lethargy Schubigerand Schubiger [9] Seizures Marciniac and Wilson [7] Seizures, protein c deficiency Hanigan and Rossi [11] Seizures - + Wong and LeMesurier [6] 2 Seizures + + Konishi and Kuriama [4] 3 Seizures +2 -1 +1 Edwards and Kuharik [13] 2 Meconium aspiration, respiratory distress, hypotonia, hypotension Shevell and Silver [3] 17 15 with seizures, 2 with bulging fontanels and macrocrania + - Baram and Butler [10] Irritability Hanigan and Tracy [ 12] 27 weeks gestation with RDS and other complications, multiple trauma during delivery and hydrocephalus, grade III, IVH, traumatic subarachuoid hemorrhage and jitteriness + + + Davies and Slavotivek [8] 1 Respiratory depression 4-* Rivkin and Anderson [16] 7 Seizures x3 Lethargy x4 +6 -1 Present patient 1 Seizures +** Totals: 44 29 presented with seizures; 15 with different presentations 5 with normal or nondiagnostic CT scans + +7 * Autopsy revealed a much more extensive lesion. ** MRI revealed a much more extensive lesion. Abbreviations: 1VH = Intraventricular hemorrhage RDS = Respiratory distress syndrome dense sagittal sinus with concomitant small ventricles. Two were confirmed by angiography and nuclear flow scan. No etiology was apparent in 14 patients and 14 of the 17 infants recovered fully. MRI was not used. Other authors have reported cases in which the CT findings of DST were confirmed angiographically [4,6] or at autopsy [8]. Only 4 reports of MRI in the diagnosis of neonatal DST have been published. Hanigan and Tracy reported 5 infants with neonatal DST [12], 1 of whom had been reported previously [11]. The others presented with a very complicated neonatal course. All were diagnosed by MRI, following head sonograms revealing various abnormalities, including ventricular dilatation, "enlarged choroid plexus," cystic lesions, or periventricular leukomalacia. In 1 patient, cranial CT demonstrated a small midline hemorrhage. All of these patients had follow-up MRI scans within 6 months disclosing resolution of the thrombotic process. Baram and Butler described an asphyxiated newborn with maternal fever and meconium aspiration who Grossman et ah Dural Sinus Thrombosis 237 presented with irritability and jitteriness, but without true seizures [10]; cranial C T was normal, but M R I d e m o n strated a thrombosis o f the right transverse sinus. Rivkin and A n d e r s o n reported 7 neonates presenting with focal seizures or lethargy [16]. In one of them, cranial C T was nondiagnostic and in all the others, M R I demonstrated the pathology m u c h more extensively. Literature r e v i e w and our patient demonstrated an important role for M R I in the diagnosis and f o l l o w - u p of newborns with DST. Our patient is the first in w h o m the diagnosis and c o m p l e t e resolution of D S T o v e r 3 w e e k s was demonstrated by MRI. M R I clearly visualizes the D S T in patients in w h o m cranial C T is normal, nondiagnostic, or does not demonstrate the full extent of the lesion. M R I avoids the risks of carotid angiography and d o c u m e n t s associated cerebral pathology as well. D S T should be considered m u c h more frequently in neonates with seizures, unexplained irritability, macrocephaly, or bulging fontanel, especially when cranial C T demonstrates subarachnoid bleeding. The fact that 31 of 44 reported infants with neonatal D S T have been reported in the last 4 years indicates that clinicians are m o r e aware o f this diagnosis and m o r e sensitive d i a g n o s t i c m o d a l i t i e s are available. D S T in an otherwise well child has an excellent prognosis [3,6,11, 16]. T h i s d i a g n o s i s , t h e r e f o r e , is i m p o r t a n t for reassurance of parents and exclusion o f other conditions causing neonatal seizures. We believe that m o r e frequent use o f M R I in patients w i t h u n d i a g n o s e d neonatal seizures, macrocrania, and bulging fontanel or a b n o r m a l cranial sonograms [12] will reveal a m u c h larger infant population with the diagnosis o f DST. 238 PEDIATRIC NEUROLOGY Vol. 9 No. 3 References [1] Mac Gregor AR. Pathology of infancy and childhood. London: Livingston, 1960;28-54. [2] Towbin A. Central nervous system damage in human fetus and newborn infant. Am J Dis Child 1970;I 19:529-42. [3] Shevell MI, Silver K. Neonatal dural sinus thrombosis. Pediatr Neurol 1989;5:161-4. [4] Konishi Y, Kuriama M. Superior sagittal sinus thrombosis in neonates. Pediatr Neurol 1987;3:222-5. [5] Patronas NJ, Duda E. Superior sagittal sinus thrombosis diagnosed by computed tomography. Surg Neurol 1981;15:11-4. [6] Wong VK, LeMesurier J. Cerebral venous thrombosis as a cause of neonatal seizures. Pediatr Neurol 1987;3:235-7. [7] Marciniac E, Wilson HD. Neonatal purpura fulminans: A genetic disorder related to the absence of protein C in the blood. Blood 1985, 65:15-20. [81 Davies RE Slavotivek JE Calcified cerebral sinus thrombosis in infancy- CT appearances with pathological correlation. Pediatr Radiol 1989;20:101-3. [9] Shubiger G, Shubiger O. Thrombose des sinus sagittal superior beim neugeborenen-Diagnose durch computertomographie. Helv Pediatr Acta 1982;37:137-9. [10] Bar'am TZ, Butler IJ. Transverse sinus thrombosis in newborns: Clinical and magnetic resonance imaging findings. Ann Neurol 1988;24: 792-4. [11] Hanigan WC, Rossi LJ. MRI of cerebral vein thrombosis in infancy: A case report. Neurology 1986;36:1354-6. [12] Hanigan WC, Tracy PT. Neonatal cerebral venous thrombosis. Pediatr Neurosci 1988;14:177-83. [13] Edwards MK, Kuharik MA. Sonographic demonstration of cerebral sinus thrombosis. AJNR 1987;8:1153-5. [14] Schwab FJ, Peyster RG. CT of cerebral venous sinus thrombosis in a child with homocystinuria. Pediatr Radiol 1987;17:244-5. [15] Baily OT, Hass GM. Dural sinus thrombosis in early life: The clinical manifestations and extent of brain injury in acute sinus thrombosis. J Pediatr 1931;11:755-71. [16] Rivkin MJ, Anderson ML. Neonatal idiopathic cerebral venous thrombosis: An unrecognized cause of transient seizures or lethargy. Ann Neurol 1992;32:51-6.