PRENATAL DIAGNOSIS
Prenat Diagn 2009; 29: 808–813. Published online 5 May 2009 in Wiley InterScience (www.interscience.wiley.com) DOI: 10.1002/pd.2277
RESEARCH LETTER
Prenatal diagnosis of a spontaneous dural sinus thrombosis G. Legendre1, O. Picone1,2,3 * , J. M. Levaillant1,2,3 , J. Delavaucoupet4 , A. Ozanne5 , R. Frydman1,2,3 and M. V. Senat1,3,6 1
AP-HP, Service de Gyn´ecologie–Obst´etrique, Hˆopital Antoine B´ecl`ere, Clamart, F-92140, France INSERM, U 782, Clamart, F-92140, France 3 Universit´e Paris Sud, UMR-S0782, Clamart, F-92140, France 4 AP-HP, Service de Radiologie, Hˆopital Antoine B´ecl`ere, Clamart, F-92140, France 5 AP-HP, Service de Neuroradiologie Diagnostique et Th´erapeutique, Centre de Ref´erence des Maladies Neurovasculaires Malformatives de l’Enfant, Hˆopital, Bicˆetre, Le Kremlin Bicˆetre, F-94276, France 6 Service d’Epid´emiologie, D´emographie et Sciences Sociales, INSERM U822, Le Kremlin-Bicˆetre, F-94276, France 2
KEY WORDS: prenatal diagnosis; dural sinus thrombosis; fetal ultrasound; fetal 3D/4D ultrasound; fetal MRI; genetic counselling
We present the case of a 31-year-old woman, gravida 1, who was referred at 22 weeks’ of gestation to our center to explore abnormalities revealed by her routine second-trimester ultrasound examination. The patient had an unremarkable medical and obstetrical history. The couple was not consanguineous. The first screening ultrasound scan, at 13 weeks, was normal. At 14 weeks, genetic serum screen for Down syndrome yielded normal results placing the fetus at low risk for chromosomal abnormalities. A routine second-trimester ultrasound revealed an abnormal intracranial mass. The ultrasound scan performed at our center at 23 weeks revealed a 9-mm hyperechoic area within a hypoechoic area triangular in shape, about 4 cm2 in the posterior intracranial fossa. Within the hypoechoic area, color Doppler showed high-velocity flow compatible with an arteriovenous shunt (Figure 1). The rest of the ultrasonographic fetal survey was normal. This suggested the presence of a dural sinus malformation (DSM). Amniocentesis revealed a normal 46, XX karyotype. Monthly follow-up ultrasound scans showed an increase in size of the hyperechoic area, which remained confined to within the DSM. We concluded that it corresponded to a spontaneous thrombosis of the DSM. Two fetal MRI examinations at 28 and 32 weeks of gestation confirmed the diagnosis of an evolving DSM. The malformation decreased in size at the torcular (confluence of sinuses) and at the posterior superior sagittal sinus. It appeared as a spontaneous hyper intense signal on the T1-weighted sequence and as a hypo intense signal on the T2-weighted sequence. At 35 weeks, the DSM was no longer detectable on ultrasound. *Correspondence to: O. Picone, Service de Gyn´ecologie Obst´etrique et M´edecine de la Reproduction, Hˆopital Antoine B´ecl`ere, 157, Rue de la Porte de Trivaux, 92141 Clamart cedex, France. E-mail:
[email protected]
Copyright 2009 John Wiley & Sons, Ltd.
As vaginal delivery was not contraindicated, the patient was allowed to deliver vaginally, after spontaneous labor, of a healthy 2780-g girl at a gestational age of 39.4 weeks. MRI one month after birth found that the remodeling of the dural sinus was satisfactory and the cerebral parenchyma was normal. Four months after birth, the baby’s psychomotor development appeared normal. DSM is caused by the failure of the dural sinuses to mature, with the persistence of giant venous lakes forming an aneurysmal structure collecting both superficial and deep cerebral veins. The development of arteriovenous shunts in the wall of the DSM is a secondary event. The velocity of these shunts is usually low and they rarely cause systemic hemodynamic complications, although they can disrupt venous drainage and thus lead to venous thrombosis and hydrocephaly (Barbosa et al., 2003). Spontaneous thrombosis of the venous spaces is part of the natural history of DSM. The thrombosis may lead to the maturation of the sinuses (healing) or it can extend to the normal cerebral venous drainage and cause an infarction. In the latter case, evidently, the prognosis is poor (Barbosa et al., 2003). Until the 1980s, DSM thrombosis was reported mainly in children, and all reported cases were diagnosed in postmortem series after births involving asphyxia. Those postnatal cases focused on an association with trauma or infection (Visentin et al., 2001). Later studies pointed to hypercoagulation caused by polycythemia or thrombophilia as possible aggravating factors (Visentin et al., 2001). Prenatal diagnosis of DSM is rare, with 30 cases reported in the literature. The diagnosis is usually based on 2-dimensional (2D) ultrasound findings supported by Doppler and MRI (Jung et al., 2006; Rossi et al., 2006; Merzoug et al., 2008; Spampinato et al., 2008). The association of a hyperechoic area surrounded by a hypoechoic triangular area in the posterior fossa is suggestive of DSM at 2D ultrasound, with the hyperechoic area corresponding to the thrombosis within the DSM. An enlargement of the interhemispheric space Received: 5 January 2009 Revised: 2 March 2009 Accepted: 2 March 2009 Published online: 5 May 2009
809
PRENATAL DIAGNOSIS OF A SPONTANEOUS DURAL SINUS THROMBOSIS
A
B ∗
* C
D
∗
∗
Figure 1—(A) 2D and (B) 3D ultrasound images (coronal plane) of the fetal head at 23 weeks of gestation showing a hyperechogenic clot (∗ ) surrounded by a hypoechogenic triangular-based area corresponding to a dilated longitudinal superior sinus (↓). Color Doppler shows a high-speed vessel compatible with an arterio-venous shunt. (C) Prenatal MRI image (sagittal plane—T1-weighted sequences) at 28 weeks of gestation showing a hyperintense signal in T1-weighted-image and (D) a hypointense in T2-weighted-image corresponding to the thrombus (∗ ). The mass is located in the dural space and centered on the torcular area; the tentorium and the cerebellum are displaced
corresponding to the expansion of superior sagittal sinus (as occurred in our case) is also common. The absence of blood flow at color Doppler confirms the presence of a thrombus (Visentin et al., 2001). On MRI analysis, the DSM appears as a wellcircumscribed mass with a heterogeneous hypointense signal on T2-weighted images and a hyperintense signal in T1-weighted images, corresponding to the thrombus. MRI can evaluate the evolution and maturation of sinuses (Merzoug et al., 2008). It is also the technique of choice in the postpartum period to confirm the diagnosis, assess prognosis and guide treatment. MR angiography allows to demonstrate the absence or reduction of blood flow in the occluded sinus and thereby replaces angiography (Barbosa et al., 2003). 3D ultrasound has not been evaluated yet in the diagnosis of DSM. DSM must be distinguished from other malformations of the posterior brain fossa, such as tumors, arachnoid cysts and vein of Galen malformations (Jung et al., 2006). Ultrasound with color Doppler imaging can easily differentiate DSM from other vascular malformations Copyright 2009 John Wiley & Sons, Ltd.
such as a vein of Galen malformation, and from cystic lesions such as arachnoid cysts or Dandy–Walker syndrome (Visentin et al., 2001). An aneurysm of the vein of Galen appears as an oblong image above and behind the thalami, with a turbulent flow and sometimes hydramnios (Jung et al., 2006). MRI can also differentiate DSM from parenchymal lesions in the posterior fossa, like teratomas, which are the principal cerebral tumors and are characterized by heterogeneous images on MRI (Jung et al., 2006; Rossi et al., 2006). The constant hyperintense signal on T2-weighted sequences of cystic lesions is useful to distinguish them from vascular malformations, in which vascular flow leads to hypointense signals at T2-weighted sequences (Rossi et al., 2006). Outcomes of the antenatally diagnosed cases of thrombosis of DSM include three neonatal deaths, two with mental retardation, and 19 (63%) with satisfactory development (including one with partial necrosis of the frontal lobe) (Table 1). (Gicquel et al., 2000; Visentin et al., 2001; Emamian et al., 2002; Komiyama et al., 2004; Prenat Diagn 2009; 29: 808–813. DOI: 10.1002/pd
Copyright 2009 John Wiley & Sons, Ltd.
Cardiomegaly
Intracranial hypertension
24
34
Merzoug et al. (2008) case no.◦ 3
Jung et al. (2006) Merzoug et al. (2008) case no.◦ 1 Merzoug et al. (2008) case no.◦ 2
Komiyama et al. (2004) Case no.◦ 1 Komiyama et al. (2004) Case no.◦ 2 Rossi et al. (2006)
Torcular
Torcular
34
Torcular
31
28
Posterior sinus
Torcular
23
35
22
Longitudinal — superior sinus Torcular + — right transverse sinus Torcular Cardiomegaly systemic shunt
24
Major hydrocephalus
Major hydrocephalus
—
—
ventriculomegaly
Emamian et al. (2002) Clode et al. (2005)
Torcular
28
—
—
Visentin et al. (2001)
Torcular
Torcular
21
—
Associated signs
22
Torcular
22
Location of thrombosis
Visentin et al. (2001)
Gicquel et al. (2000) Visentin et al. (2001)
GA at diagnosis
Prenatal trauma or invasive tests
NR
NR
NR
NR
Amniocentesis (16 SA)
NR
NR
NR
NR
Amniocentesis
—
Amniocentesis
Amniocentesis (15 WG)
Table 1—Cases of dural sinus malformation diagnosed antenatally
NR
NR
NR
Normal
NR
NR
NR
NR
NR
NR
NR
NR
Normal
M
F
F
F
M
M
M
NR
NR
M
?
F
F
Thrombophilia and coagulation Fetal test gender
=
(Postnatal)
—
—
—
= (T2/T3)
Evolution
Death (3 days)
—
Mental retardation
Mental retardation
NR
NR
Hydrocephalus (derivation) Good (7 months) Death (5 months)
Good (1 month) endovascular treatment VB (40 WG) Good (7 month) TOP (34 WG) —
CS (37 WG)
CS (36 WG)
CS (38 WG)
VB (36 WG) Good (12 month) CS Good (18 month)
CS (38 WG)
TOP
TOP
—
Long-term follow-up
VB (40 WG) Good
Outcome of pregnancy
—
Thrombus of torcular with normal brain —
—
—
—
—
—
Thrombus of torcular with normal brain Thrombus of torcular with normal brain Thrombus of torcular with massive intraoperative hemorrhage —
—
Anatomopathology
810 G. LEGENDRE ET AL.
Prenat Diagn 2009; 29: 808–813. DOI: 10.1002/pd
Copyright 2009 John Wiley & Sons, Ltd.
Merzoug et al. (2008) case no.◦ 12 Laurichesse Delmas et al. (2008) case no.◦ 1 Laurichesse Delmas et al. (2008) case no.◦ 2 Laurichesse Delmas et al. (2008) case no.◦ 3
Merzoug et al. (2008) case no.◦ 4 Merzoug et al. (2008) case no.◦ 5 Merzoug et al. (2008) case no.◦ 6 Merzoug et al. (2008) case no.◦ 7 Merzoug et al. (2008) case no.◦ 8 Merzoug et al. (2008) case no.◦ 9 Merzoug et al. (2008) case no.◦ 10 Merzoug et al., 2008 case no.◦ 11
Torcular
Torcular
Torcular
Torcular + longitudinal superior sinus
Torcular
Torcular
22
22
28
28
32
25
Torcular
Torcular
31
24
Torcular
24
Torcular
Torcular
25
24
Torcular
Location of thrombosis
32
GA at diagnosis
Table 1—(Continued )
—
—
—
Polyhydramnios
Minor hydrocephalus
—
Polyhydramnios
—
—
—
Frontal parenchymal necrosis —
Associated signs
—
—
—
NR
NR
NR
NR
NR
NR
NR
NR
NR
Prenatal trauma or invasive tests
Normal
Normal
NR
NR
NR
NR
NR
NR
NR
NR
NR
NR
Thrombophilia and coagulation test
F
F
F
M
M
F
M
M
F
F
?
F
Fetal gender
(Postnatal after treatment)
Evolution
VB
VB (41 WG)
TOP (26 WG)
TOP (33 WG)
NR
NR
NR
NR
NR
NR
TOP (26 WG)
NR
Outcome of pregnancy
Good (12 month)
Good (24 months)
—
Endovascular treatment Good (36 months) —
Good (6 months)
Good (12 months)
Good (0 month)
Good (10 months)
Good (7 months)
—
Good (4 months)
Long-term follow-up
—
—
Thrombus of torcular with normal brain Thrombus of torcular with normal brain
—
—
—
—
—
Thrombus of torcular with normal brain —
—
Anatomopathology
PRENATAL DIAGNOSIS OF A SPONTANEOUS DURAL SINUS THROMBOSIS
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Prenat Diagn 2009; 29: 808–813. DOI: 10.1002/pd
Copyright 2009 John Wiley & Sons, Ltd.
Torcular + longitudinal superior sinus
Torcular
Torcular + longitudinal superior sinus Torcular + longitudinal superior sinus
22
31
22
Laurichesse Delmas et al. (2008) case no.◦ 5 Laurichesse Delmas et al. (2008) case no.◦ 6 Spampinato et al. (2008) —
—
BDP = 90e` me p; EFW = 10e` me p
Ascites + pericardial effusion + tricuspid regurgitation + BDP >95e` me p Portosystemic shunt + polyhydramnios
Associated signs
Amniocentesis (25 WG)
NR
—
—
—
Prenatal trauma or invasive tests
—
NR
Normal
Normal
Normal
F
NR
M
F
M
Thrombophilia and coagulation Fetal test gender
(Postnatal)
Evolution
NR, not reported; M, male; F, female; CS, Cesarean section; VB, vaginal birth; TOP, termination of pregnancy; WG, weeks of gestation.
22
Posterior sinus + complex mass
32
Laurichesse Delmas et al. (2008) case no.◦ 4
Present case
Location of thrombosis
GA at diagnosis
Table 1—(Continued )
Good (24 months)
—
Long-term follow-up
— —
VB (38 WG) Good (4 months)
—
—
Thrombus of torcular and hemangioma
Anatomopathology
VB (38 WG) Good (16 months)
VB (36 WG) Death (4.5 months)
CS (38 WG)
TOP (33 WG)
Outcome of pregnancy
812 G. LEGENDRE ET AL.
Prenat Diagn 2009; 29: 808–813. DOI: 10.1002/pd
813
PRENATAL DIAGNOSIS OF A SPONTANEOUS DURAL SINUS THROMBOSIS
Clode et al., 2005; Jung et al., 2006; Rossi et al., 2006; Merzoug et al., 2008; Spampinato et al., 2008; Laurichesse Delmas et al., 2008). These results suggest a better prognosis than that reported in pediatric series, in which outcome was unfavorable in more than 70% of cases (Barbosa et al., 2003). It is likely that the time of diagnosis results in a bias, as the forms that persist after birth are probably more severe. Although follow-up in our case has lasted only four months, the prognosis of spontaneous thrombosis of the superior sagittal sinus and torcular can be good. Many case reports describe a decrease in thrombus size both prenatally and postnatally. Only in one case did the size of the thrombus increase and this led to termination of pregnancy (Merzoug et al., 2008). Extension of the thrombosis to the torcular herophili and the lack of drainage of either the superficial or deep veins into the cavernous sinus are suspected to be the factors of poor prognosis (Barbosa et al., 2003). The prenatal counselling is extremely difficult. A poor prognosis seems more frequent in cases complicated by intracerebral hemorrhage or infarction, in boys compared with girls (Laurichesse Delmas et al., 2008). Prematurity also seems to be an aggravating factor (Barbosa et al., 2003). DSM has no genetic support and it is typically a sporadic condition of unknown etiology (Barbosa et al., 2003). In conclusion, diagnosis of DSM should be considered when the image of the median cerebrum and posterior fossa is abnormal. Serial monitoring with ultrasound scans allows to follow the course of the thrombosis and to document the absence of associated cerebral abnormalities. Spontaneous regression of the DSM in the antenatal period seems indicative of good prognosis. The ultrasound examination should also look for signs of cardiac decompensation. MRI analysis of the fetal brain provides important diagnostic information. While recent prenatal cases report a better prognosis than the older neonatal series, counselling of the couple when DSM is diagnosed remains extremely difficult. The physician should explain the prognostic uncertainty.
Copyright 2009 John Wiley & Sons, Ltd.
Vaginal delivery does not seem to be contraindicated in cases with spontaneous regression of the DSM. If instrumental extraction is necessary, vacuum extraction should probably be avoided, as its use may be associated with cephalohematomas (Simonson et al., 2007). In the postnatal period, MRI may be performed to guide possible endovascular treatment. REFERENCES Barbosa M, Mahadevan J, Weon YC, et al. 2003. Dural sinus malformation (DSM) with giant lakes, in neonates and infants. Review of 30 consecutive cases. Interv Neuroradiol 9: 407–424. Clode N, Cardoso C, Tavares J, Albuquerque M, Silva JL, Gra¸ca LM. 2005. Prenatal diagnosis of thrombosis of the dural sinuses. Int J Gynaecol Obstet 91(2): 172–174. Emamian SA, Bulas DI, Vezina GL, Dobovsli E, Cogan P. 2002. Fetal MRI evaluation of an intracranial mass∼: in utero evolution of hemorrhage. Pediatr Radiol 32: 593–597. Gicquel JM, Potier A, Sitrik S, Girard N. 2000. Normal outcome after prenatal diagnosis of thrombosis of the torcular Herophili. Prenat Diagn 20: 824–827. Jung E, Won HS, Kim SK, et al. 2006. Spontaneous resolution of prenatally diagnosed dural sinus thrombosis: a case report. Ultrasound Obstet Gynecol 27: 562–565. Komiyama M, Ishiguro T, Kitano S, Sakamoto H, Nakamura H. 2004. Serial antenatal sonographic observation of cerebral dural sinus malformation. Am J Neuroradiol 25: 1446–1448. Laurichesse Delmas H, Winer N, Gallot D, et al. 2008. Prenatal diagnosis of thrombosis of the dural sinuses: report of six cases, review of the literature and suggested management. Ultrasound Obstet Gynecol 32: 188–198. Merzoug V, Flunker S, Drissi C, et al. 2008. Dural sinus malformation in fœtus. Diagnostic and value or prenatal MRI and follow-up. Eur Radiol 18: 692–699. Rossi A, De Biasio P, Scarso E, et al. 2006. Prenatal MR imaging of dural sinus malformation: a case report. Prenat Diagn 26(1): 11–16. Simonson C, Barlow P, Dehennin N, et al. 2007. Neonatal complications of vacuum-assisted delivery. Obstet Gynecol 109(3): 626–633. Spampinato MV, Hardin V, Davis M, Chang E, Rumboldt Z. 2008. Thrombosed fetal dural sinus malformation diagnosed with magnetic resonance imaging. Obstet Gynecol 111: 569–572. Visentin A, Falco P, Pilu G, et al. 2001. Prenatal diagnosis of thrombosis of the dural sinuses with real-time color Doppler ultrasound. Ultrasound Obstet Gynecol 17: 322–325.
Prenat Diagn 2009; 29: 808–813. DOI: 10.1002/pd
