Multiple cerebral venous sinus thrombosis: Case report
E. Mogere, MBChB, Resident, Department of Surgery, The Aga Khan University Hospital, Nairobi, Kenya
and D.O. Olunya, FRCS (Neurosurg), Consultant Neurosurgeon and Senior Lecturer, Department of
Surgery, The Aga Khan University Hospital
Dr. E. Mogere, P.O. Box 30270-00100, Nairobi, Kenya, Email: firstname.lastname@example.org
Cerebral venous thrombosis (CVT) is an uncommon clinical problem and can be characterised by nonspecific and common symptoms of headaches and vomiting due to the intracranial hypertension. Alternate diagnoses are entertained especially when procoagulant factors are not elicited. We present a 35 year old female, parity 2+0, gravida 3 at 8 weeks gestation who presented with headache, vomiting, photophobia and neck stiffness. The diagnostic workup and radiological findings confirmed multiple venous sinus thromboses. The case is discussed in the light of diagnostic challenge, treatment pathways and how an understanding of the basic sciences explains its clinical presentation.
The diagnosis of cerebral venous sinus thrombosis should be considered in a young patient presenting with an unusual headache in the absence the usual vascular risk factors.
Cerebral venous thrombosis is an uncommon disease that presents in a non-specific pattern. Ninety percent of patients report a headache, 50% have some type of neurological deficit and convulsions occur in 40% of cases (1). This nonspecific presentation leads to misdiagnosis, delayed diagnosis or medical misadventures. Average delay from onset of symptoms to diagnosis is 7 days (2).
The patient profile is varied, defining clinical characteristics few and aetiological relationships diverse. Documented series indicate association of sinus thrombosis with presence of prothrombotic factors (3), genetic factors including protein C and S deficiency, pregnancy, nephrotic syndrome as well as dehydration and cancer (4).
A clinician’s high index of suspicion remains the vanguard of making an accurate and timely diagnosis. We report a case of a young female who presented with non-specific symptoms and signs and managed for multiple cerebral venous sinus thrombosis.
A. N., a 35 year old female patient, at parity 2+0, gravida 3 at 8 weeks gestation presented to our hospital with 6 days history of a severe global throbbing headache of sudden onset that was associated with vomiting, photophobia and neck stiffness. She had no antecedent trauma, fever nor any history of hypertension. Her obstetric and gynecological history was unremarkable, without any use of oral contraceptives. She was a married housewife who neither smoked nor consumed any alcohol. On general examination, A.N. was restless but had no conjuctival pallor, scleral icterus, oral thrush or lymphadenopathy. Her vital signs were normal (brachial blood pressure of 106/65 mmHg, temperature 370C) except for a tachycardia of 106 beats/min (good volume, regular non-collapsing pulse).
She was fully conscious with normal higher functions and pupils that were 3 mm in diameter and responsive to light, both directly and consensually. Positive findings included photophobia, a left sided abducent nerve palsy and bilateral papilloedema at fundoscopy. Her neck was stiff with negative Kerning’s and Brudzinki’s signs while the spine, motor, sensory and cerebellar examinations were unremarkable. The systemic findings were noncontributory. An impression of a subarachnoid hemorrhage with differential diagnosis of meningitis and an intracranial space occupying lesion were entertained.
Laboratory investigations included a haemogram (Hb of 13.7g/dL, WBCs of 8.2×109 /L with a neutrophil count of 70%) and electrolytes (K+ 2.8 mmol/L). The rest of the biochemistry, and the coagulation profile were unremarkable. The opening pressures were high and the CSF bloody at lumbar puncture. An MRI was then performed that revealed hyperintense signals in the superior sagittal sinus (SSS), straight, right transverse and sigmoid sinuses-changes due to thrombosis (Figure 1).
The thrombi were multiple with some being contiguous and others discrete. A thrombophilia screen ordered after this showed a normal profile (Table 1).
Click to view table 1
Her subsequent management entailed anticoagulation with high dose enoxaparin and treatment with clonazepam, analgesics and potassium supplementation. The patient had an uneventful recovery and was discharged home on the 7th day. She will complete one year of anticoagulation.
Most of A.N.’s major venous sinuses were involved with the exception of the inferior sagital. The superior saggittal sinus usually drains into the confluence of sinuses then into the straight and finally the sigmoid sinus. The combination of both continuous and discrete thrombi point to either a systemic aetiology or a combination of both local and systemic causes. Some anatomic features may explain some of the local factors contributing to thrombosis and propagation. Sinuses are valveless endothelial lined channels formed between the two layers of the dura. Endothelial disruptions in these low pressure channels (contributing to stasis) may initiate or propagate thrombosis (5). The cerebral veins join the sinuses obliquely generally against the bloodstream and create regions of turbulent flow. These features may point to prothrombotic zones and explain thrombus propagation in sinuses in proximity. Hence a systemic hypercoagulable state in combination with local features may account for the varied pattern of thromboses.
This patient had no other procoagulant factor other than pregnancy. High circulating estrogen levels in pregnancy are accepted to be the cause of this hypercoagulable state. In general, aetiological relationships of cerebral sinus thrombosis are varied. About 85% of patients are noted to have a procoagulant factor (3) which may include protein C and S genetic deficiencies, pregnancy, nephrotic syndrome as well as dehydration and cancer (4). Her age and physiological status did not fall into the other thrombophilia risk patterns.
Close to 90% of patients report a headache with 50% having some type of neurological deficit while convulsions occur in