Article Text

Other full case
Cerebral venous sinus thrombosis presenting in pregnancy and puerperium
  1. Ganesh Dangal1,
  2. Lok Bahadur Thapa2
  1. 1
    Gynaecology and Obstetrics, Kathmandu Model Hospital, Exhibition Road, PO Box 6064, Kathmandu, Nepal
  2. 2
    Emergency Department, Kathmandu Model Hospital, Exhibition Road, PO Box 6064, Kathmandu, Nepal
  1. Ganesh Dangal, gareshma{at}hotmail.com

Summary

Cerebral venous sinus thrombosis (CVST) during pregnancy and puerperium is an uncommon diagnosis. CVST can present with wide range of symptoms and signs, and most of the time it is left undiagnosed. Here, three cases of CVST in early pregnancy and puerperium in young women who presented with seizure attacks, altered sensorium and severe headache, respectively, are reported. Subsequent imaging with MRI and magnetic resonance venography (MRV) showed CVST in the first two cases, whereas in the third case it showed a venous infarction. All were treated with low molecular weight heparin followed by warfarin. They all made excellent recoveries and are in regular follow-up.

Statistics from Altmetric.com

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.

BACKGROUND

Cerebral venous sinus thrombosis (CVST) in pregnancy and puerperium is quite unusual. CVST presents with various symptoms and signs such as seizure attacks, headache and rarely with neurological deficits such as paresis and coma. There should be a high index of suspicion in order to diagnose this disorder. Most often CVST is left undiagnosed and could lead to life-threatening conditions. Yet its management is simple with excellent prognosis. Thus, timely intervention and correct diagnosis could save lives and prevent debilitating consequences.

CASES

Case 1

A 24-year-old woman (para 2) in her 9th postpartum day presented with headache, vomiting and abnormal movements of whole body for 1 day. She had multiple episodes of abnormal movements with loss of consciousness, tongue bite and clenching of teeth. Her medical history was unremarkable, with no hospitalisation other than normal delivery 4 years ago and induced abortion 2 years ago. The patient did not have a history of using any medication or contraceptive pills. On physical examination, the patient appeared fully alert and oriented. Her vitals were normal and there was no neurological deficit. Other laboratory investigations including complete blood count and metabolic profile were within normal limits. She had seizure attacks after the admission and was controlled by phenytion. CT and MRI scans were normal and a magnetic resonance venography (MRV) scan was consistent with left dural venous sinus thrombosis (fig 1).

Figure 1

Case 1 coronal view of MRI venogram demonstrating lack of flow in the left transverse sinus.

Case 2

A 22-year-old para 1 woman in her 6th postpartum day following caesarean section was admitted with altered sensorium associated with vacant look, abnormal movements of the body and frothing from the mouth for 1 day. There was no history of epilepsy. The patient did not have a history of using contraceptive pills. On examination she was fully oriented and her vital signs were normal. There was no neurological deficit. The seizure was controlled with diazepam and phenytoin. The routine laboratory investigations were all within normal ranges. CT scan was normal and a MRV scan showed left transverse venous sinus thrombosis with cortical venous sinus thrombosis (fig 2).

Figure 2

Case 2 coronal view of MRI venogram demonstrating lack of flow in the left transverse sinus.

Case 3

A 27-year-old woman (gravida 2, para 0+1) with 10 weeks of pregnancy presented with severe headache starting suddenly a day ago. She stated that it was the “worst headache of her life” and the pain had been persistent and worsening despite use of analgesics. The patient had associated vomiting but no fever, visual disturbances and seizures. Her medical history was unremarkable with no hospitalisation other than induced abortion 4 years ago and she had been using oral contraceptive pills since then. On examination she was fully oriented with her surroundings. Her vitals were normal and there were no meningeal signs or neurological deficits. Her routine laboratory investigations were all normal. A CT scan of the brain was normal whereas the MRI showed venous infarction on the right temporal side (fig 3) and MRV was normal. Subsequently her pregnancy was terminated and the treatment started.

Figure 3

Case 3 MRI showing venous infarction on the right temporal region.

TREATMENT

All the cases were treated with low molecular weight heparin followed by warfarin.

OUTCOME AND FOLLOW-UP

For all patients the hospital stay was uneventful and they made excellent recoveries.

DISCUSSION

Cerebral venous sinus thrombosis has a variety of clinical presentations ranging from severe headache to deep coma. The most common presentation includes headache (97%) followed by seizure attacks (47%) and paresis (43%).1 The incidence is increasing to 7 per 1 000 000 as newer and more advanced imaging modalities emerge.2 Women are more commonly affected than men, with a ratio of 1.29:1.3 It commonly presents in women of 25–35 years of age and it occurs more often during puerperium than during pregnancy.4 There is no race predilection, and the associated mortality is reported to be 7%.5 More than 100 causes of cerebral venous sinus thrombosis have been recorded in the scientific literature. However, even with extensive investigation, no cause is identified in 20% to 25% of patients.6 The most common pathogenesis includes hypercoagulable states such as pregnancy and puerperium and the use of oral contraceptives. Women using oral contraceptive have an increased risk of CVST by approximately 20%.7 Largely inherited prothrombotic tendencies such as factor V Leidan mutation, protein S and C and anti-thrombin III deficiencies are important causes, accounting for perhaps 10% to 15% of cases, and infective causes related to middle ear, facial infection or penetrating head trauma probably occur less commonly due to modern aggressive antibiotic treatment.8

Neuroimaging remains the main cornerstone for the diagnosis. A CT scan is a useful initial examination technique to rule out other acute cerebral disorders and to show venous infarcts or haemorrhages, but its results can be entirely normal. The most sensitive examination technique and imaging modality of choice is MRI in combination with MRV.9 The combination of abnormal signal in a sinus and a corresponding absence of flow in MRV confirmed the diagnosis, but expert radiological judgement is required to avoid diagnostic and technical pitfalls.10 Treatment options for cerebral venous sinus thrombosis include anticoagulation, thrombolytic therapy and in some cases surgical thrombectomy. Anticoagulation therapy with lower molecular weight heparin is the treatment of choice followed by the oral warfarinisation. Prognosis is quite variable. The outcome can result from total recovery to death, however prospective studies have reported independent survival rate of approximately 80%.11 The risk of recurrent cerebral venous sinus thrombosis in future pregnancies and puerperium is low.12

In conclusion, CVST may present with extremely varied symptoms and signs. Diagnosis can be confirmed by MRI in most cases. It is a potentially life-threatening condition if it is undiagnosed, but remains a treatable disorder and it should be considered in every woman with neurological symptoms in pregnancy and/or puerperium.

LEARNING POINTS

  • Cerebral venous sinus thrombosis (CVST) is an uncommon disorder in pregnancy and puerperium, so a high index of suspicion needs to be present in order to diagnose it.

  • MRI + magnetic resonance venography (MRV) scans can confirm the diagnosis.

  • The mainstay of treatment is heparinisation.

Acknowledgments

We thank Dr Bharat Pradhan, executive director, Kathmandu Model Hospital for allowing us to publish this article.

REFERENCES

Footnotes

  • Competing interests: None.

  • Patient consent: Patient/guardian consent was obtained for publication.