Case Report Thrombosed Dural Sinus Malformation in a Fetus: A Case Report Hiroko Asai, MD, Toshio Okamoto, MD, PhD, Etsushi Tsuchida, MD, Fumikatsu Nohara, MD, Ken Nagaya, MD, PhD, Hiroshi Azuma, MD, PhD From the Asahikawa Medical University, Departmemt of Pediatrics, 2-1-1-1 Midorigaoka-Higashi, Asahikawa, Hokkaido, Japan.
ABSTRACT BACKGROUND AND PURPOSE
A thrombosed dural sinus malformation (DSM) is a rare condition, the clinical features of which have not yet been completely characterized. Here, we describe the clinical course of a patient with a thrombosed DSM and discuss the outcomes in live birth cases from a review of the literature. CASE DESCRIPTION
An ultrasonography examination of a 32-year-old woman at 25 weeks’ gestation indicated a fetal posterior fossa mass. The size of the intracranial mass remained constant during the second trimester and was observed to decrease from 33 weeks of gestation. A postnatal diagnosis of thrombosis in the dural sinus was established by magnetic resonance imaging and venography. No brain damage or hydrocephalus was noted. Although the circumference of the infant’s head was enlarged at birth, her neurological outcome was normal at 1 year of age.
Keywords: Thrombosed dural sinus malformation, outcome, fetus. Acceptance: Received February 12, 2013, and in revised form November 8, 2013. Accepted for publication November 19, 2013. Correspondence: Address correspondence to Hiroko Asai. E-mail: [email protected]
asahikawa-med.ac.jp. J Neuroimaging 2014;24:603-606. DOI: 10.1111/jon.12099
Although normal cranial circumference is reportedly an essential factor for a favorable prognosis, the patient in this report with a cranial circumference at + 2.0 SD (35.6 cm) had a favorable prognosis. Further studies focused on improving clinical diagnostic accuracy in this rare entity will facilitate appropriate counseling.
Introduction Antenatal thrombosed dural sinus malformation (DSM), also known as dural sinus thrombosis, fetal dural arteriovenous fistula (AVF), or giant dural sinus ectasia, is a rare condition. Although the number of reported cases of antenatal thrombosed DSM have been increasing, the outcome of this condition remains unclear. Elucidating further knowledge on the clinical course of thrombosed DSM may improve management of this condition. Here, we report a case of thrombosed DSM detected in a fetus who had a favorable outcome and present a review of the literature.
Case Report A 32-year-old woman was referred to our institution at 26 weeks of gestation following the detection of a fetal intracranial mass in the posterior fossa. An ultrasonography examination performed at the referring hospital showed a small hypoechoic focus in the mass (Fig 1A). Fetal magnetic resonance imaging (MRI) performed at 26 weeks of gestation revealed a supratentorial meningeal mass, measuring 35 × 39 × 54 mm. T2weighted imaging indicated a large, slightly hypointense posterior fossa mass with a focal area of increased hypointensity. The mass compressed the cerebrum and cerebellum, but no
hydrocephalus was detected (Figs 1B, C). On the basis of these findings, we made a preliminary diagnosis of fetal brain tumor, such as teratoma. Additional ultrasonography examinations revealed a gradual decrease in the size of the mass from 33 weeks of gestation. An ultrasonographic examination, performed at 35 weeks of gestation, indicated that the mass had decreased in size to 23 × 32 mm. The cranial circumference ranged between + 1 and + 2SD throughout fetal life. There were no signs of cardiac failure. A female infant was born through normal spontaneous vaginal delivery at 38 weeks of gestation. The 1- and 5-min Apgar scores were 8 and 9, respectively. Her birth weight was 3135 g (+.3 SD), and cranial circumference was 35.6 cm (+2.0 SD) and there was no evidence of bulging of the anterior fontanelle. The coagulation profile of the infant revealed a normal platelet count, prothrombin time, activated partial thromboplastin time, fibrinogen level, protein C level, and protein S level. MRI performed at 4 days of age indicated a significant decrease in the size of the mass compared with that at 26 weeks of gestation (Fig 2B). T2-weighted imaging revealed a hyperintense mass within the dural sinus (Figs 2B, C). No ventriculomegaly or infarction was noted. Magnetic resonance angiography and venography indicated sinus patency and absence of an arteriovenous shunt (Fig 2D). These findings were consistent with a
◦ 2014 by the American Society of Neuroimaging C
Fig 1. Prenatal imaging studies. (A) Axial ultrasonography at 25 weeks of gestation showing a 35 ×40 mm cystic mass. There is a hyperechoic area within the cystic mass. Coronal (B) and sagittal (C) T2-weighted images at 26 weeks of gestation showing an iso-hypointense mass in the dural sinus with a concentric ring of hypointensity. The mass is compressing the cerebrum and cerebellum (arrow).
Fig 2. Postnatal imaging studies. (A) Computed tomography, day 1. Apparent decrease in size of the mass, with mineralization in the dural sinus. Postnatal MRI (B, C) and MR venography (D), day 4. T1-weighted coronal (B) and sagittal (C) images showing decreased thrombosis in the dural sinus. The dural venous sinus remains patent (D) (arrow).
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Table 1. Reported Cases of Thrombosed Dural Sinus Malformation with Live Birth Reference
Gicquel et al (2000) Visentin et al (2001)
Regression No change
Emamian et al (2002) Clode et al (2005) Rossi et al (2006)
None Borderline ventoriculomegaly None
No change NR
Jung et al (2006) Van Keirsbilck et al (2007) Laurichesse Delmas et al (2008)
Favorable (16 months) Death (intraoperative hemorrhage) Favorable (1 year)
No Embolization for AVF
Favorable (18 months) Favorable (5 months)
Favorable (7 months) Favorable (3 months)
None Intracranial venous hypertension (postnatal) None Isolated ischemic lesion (postnatal) None
Favorable (24 months)
Regression Regression No change
VD CS VD
None Portosystemic shunt macrocephaly
No No No
Favorable (12 months) Favorable (24 months) Death at 4 months (respiratory failure) Favorable (16 months)
Favorable (28 months)
Death at 5 months (hydrocephalus) Favorable (5 months)
Regression Regression Regression NR Regression Enlarged (prenatal) Regression (after treatment) Regression Regression NR
NR NR NR NR NR NR
Major hydrocephalus Major hydrocephalus Limited frontal parenchymal necrosis None None None None None hydrocephalus
NR VD CS
No change Regression Regression
CS VD CS
Pandey et al (2012)
Simsek et al (2012) Haan et al (2008)
Spampinato et al (2008) Merzoug et al (2008)
Legendre et al (2009) Jenny et al (2010)
Byrd et al (2011)
None None Macrocephaly, increased intracranial pressure, DIC Respiratory distress, cardiac failure, macrocephaly, DIC None None Schizencephaly/ polymicrogyria Macrocephaly Hemorrhage into DSM, DIC (day2) Congestive heart failure (1 month) none Macrocephaly, cardiac failure, haemorrhagic infarction, hydrocephalus
No No No No No Embolization at 5 months
Favorable (7 months) Favorable (10 months) Favorable at birth Favorable (1 month) Favorable (6 months) Favorable (3 years)
No No Puncture of the posterior collection
Favorable (12 months) Favorable (4 months) Mild hemiparesis (18 months)
Craniotomy for evacuate the hematoma
Mild left hemiparesis (1 year) Favorable (5 years)
No No No
Favorable (1 year) Favorable (6 years) Developmental delay
Favorable (12 months)
Favorable (10 months) Death at day 4 (irreversible circulatory insufficiency)
Note: CS = caesarian section; DIC = disseminated intravascular coagulation; NR = not reported; VD = vaginal delivery.
Asai et al: Thrombosed Dural Sinus Malformation in a Fetus
diagnosis of thrombosed DSM. The cranial circumference decreased to within the normal range by 1 month of age. Clinical follow-up of the patient at 1 year of age showed normal neurological development.
Discussion In this report, we present a rare case of thrombosed DSM in a fetus with a favorable clinical outcome. Since 2000, there have been 31 reported cases of live births with thrombosed DSM. Clinical data on these patients are summarized in Table 1.1–15 The clinical course of live birth cases with thrombosed DSM is variable, ranging from death and neurological complications to normal neural development. However, many patients have a favorable outcome, as indicated in Table 1. The predictors of favorable outcome include sufficient collateral venous drainage of the brain; no congenital structural abnormalities; and absence of brain insult, infarction, ventricular hemorrhage, and fetal cardiac failure.1,2 In particular, normal cranial circumference and decreasing thrombus size are essential factors for a favorable prognosis.1 Conversely, increasing size of thrombus and imaging evidence of associated infarction or intracranial hemorrhage are associated with a relatively poor prognosis.1,3 The method of delivery appears unrelated to clinical outcome in infants with thrombosed DSM. Our patient had a normal developmental assessment at 1 year of age despite a cranial circumference at + 2.0 SD (35.6 cm) at birth. Our patient had no hydrocephalus or any other cerebral abnormalities. In addition, there was no sign of increased intracranial pressure, and cranial circumference decreased to within the normal range soon after birth. We believe that the increased cranial circumference in the present patient may be a result of thrombosis, since other reports have described a persistent cranial enlargement even after the regression of thrombosis.15 Five patients who exhibited macrocephaly have been previously reported.1,7,8,15 Two patients with AVF died due to cardiac decompensation and respiratory failure, and 2 other cases with a bulging fontanelle experienced mild hemiparesis.7 These findings, together with those of the patient case who had favorable prognosis, suggest that macrocephaly alone may not be a negative prognostic factor. The effect of surgical treatment is controversial. Of the 31 reported patients, 6 underwent surgical intervention including embolization for AVF, surgical derivation for major hydrocephalus, craniotomy for evacuation of the hematoma, and decompression.4–7 Patients treated by embolization or surgical derivation had a favorable prognosis,4,6 while those who underwent craniotomy or decompression had an un-
favorable prognosis.7 Infants with hydrocephalus or circulatory insufficiency due to AVF died in the absence of surgical treatment.1,4,8 On the basis of our experience and literature review, we conclude that pregnancy termination should not be necessarily recommended in cases of fetal thrombosed DSM since the prognosis is favorable in many of these infants. More accurate prediction of clinical outcome may be possible as additional case reports are collected and studied.
References 1. Laurichesse Delmas H, Winer N, et al. Prenatal diagnosis of thrombosis of the dural sinuses: report of six cases, review of the literature and suggested management. Ultrasound Obstet Gynecol 2008;32:188198. 2. Byrd SE, Abramowicz JS, Kent P, et al. Fetal MR imaging of posterior intracranial dural sinus thrombosis: a report of three cases with variable outcomes. Pediatr Radiol 2012;42:536-543. 3. Simsek Y, Oztanir N, Sigirci A , et al. Spontaneous resolution of fetal dural sinus thrombosis with term delivery of a live infant. Ultrasound Obstet Gynecol 2012;40:614-615. 4. Merzoug V, Flunker S, Drissi C, et al. Dural sinus malformation (DSM) in fetuses. Diagnostic value of prenatal MRI and follow-up. Eur Radiol 2008;18:692-699. 5. Visentin A, Falco P, Pilu G, et al. Prenatal diagnosis of thrombosis of the dural sinuses with real-time color Doppler ultrasound. Ultrasound Obstet Gynecol 2001;17:322-325. 6. Rossi A, De Biasio P, Scarso E, et al. Prenatal MR imaging of dural sinus malformation: a case report. Prenat Diagn 2006;26:11-16. 7. Jenny B, Zerah M, Swift D, et al. Giant dural venous sinus ectasia in neonates. J Neurosurg Pediatr 2010;5:523-528. 8. De Haan TR, Padberg RD, Hagebeuk EEO, et al. A case of neonatal dural sinus malformation: clinical symptoms, imaging and neuropathological investigations. Eur J Paediatr Neurol 2008;12:41-45. 9. Gicquel JM, Potier A, Sitruk S, et al. Normal outcome after prenatal diagnosis of thrombosis of the torcular Herophili. Prenat Diagn 2000;20:824-827. 10. Emamian SA, Bulas DI, Vezina GL, et al. Fetal MRI evaluation of an intracranial mass: in utero evolution of hemorrhage. Pediatr Radiol 2002;32:593-597. 11. Clode N, Gouveia R, Grac¸a LM. Prenatal diagnosis of thrombosis of dural sinuses. Int J Gynaecol Obstet 2005;91:172-174. 12. Jung E, Won HS, Kim SK, et al. Spontaneous resolution of prenatally diagnosed dural sinus thrombosis: a case report. Ultrasound Obstet Gynecol 2006;27:562-565. 13. Van Keirsblick J, Naulaers G, Cannie M, et al. Prenatal diagnosis of a dural sinus thrombosis with favorable outcome. Prenat Diagn 2007;27:1056-1058. 14. Legendre G, Picone O, Levaillant JM, et al. Prenatal diagnosis of a spontaneous dural sinus thrombosis. Prenat Diagn 2009;29:808-813. 15. Pandey V, Dummula K, Parimi P. Antenatal thrombosis of torcular herophili presenting with anemia, consumption coagulopathy and high-output cardiac failure in a preterm infant. J Perinatol 2012;32:728-730.
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