A 21-year-old lady, gravida one para zero, presented at 15 weeks’ gestation with a 1-week history of persistent nausea, vomiting, neck stiffness and photophobia. She also reported a 6-week history of intermittent frontal headache. The headache was dull in nature, occurred daily and constantly for the 4 days prior to admission, worsened on standing and was relieved on lying flat. She reported that symptoms of nausea, vomiting, neck stiffness and photophobia occurred in association with the headache for 1 week (International Classification of Headache Disorders II 7.2.3). There was no history of previous recurrent headaches, head/spinal trauma or intervention. It was also noted that there was no history of headaches or connective tissue disorders within her family. Her neurological examination, including funduscopy, was essentially normal. Her blood pressure on admission was 124/68. D-dimer level was within the normal range. A diagnosis of hyperemesis gravidarum was made but, due to the prominent headache, neuroimaging was performed. The MR venogram suggested thrombosis of the right transverse venous sinus ( and ). Thus, intravenous heparin was started. However, her headaches remained intractable and a neurological consultation was requested. A MRI brain scan demonstrated cerebellar tonsillar descent with ectopia and flattening of the pontine profile (). Gadolinium was not administered due to her early stage of pregnancy so presence of pachymeningeal enhancement could not be determined. The previous suggestion of venous sinus thrombosis was then believed to be an artefact after further opinions were obtained. Due to the prominent orthostatic component of her headache, consistent with a low CSF pressure, a lumbar puncture was performed. The opening pressure was <2 cm H2O with sluggish and minimal CSF flow. She was diagnosed with SIH. The heparin was discontinued and she was treated with two autologous epidural blood patches, 72 h apart, which alleviated her symptoms. At follow-up 4 weeks later she remained asymptomatic and at term delivered a healthy boy via spontaneous vaginal delivery. No further headache occurred until she became pregnant 5 years later.
Time of flight MR venogram image illustrating a filling defect in the right transverse sinus.
Time of flight MR venogram saggital image illustrating a filling defect in the proximal right transverse sinus.
T1 weighted MRI saggital image illustrating cerebellar tonsillar ectopia.
The second presentation of this, then 26-year-old, lady, gravida two para one, was at 16 weeks’ gestation. She presented with a 10-day history of frontal headache with associated nausea and photophobia. The headache radiated towards the posterior aspect of the neck, occurred intermittently on a daily basis, worsened on standing and towards evening time and improved on lying recumbent. The headache became progressively more severe in the week prior to admission. There was no associated vomiting (International Classification of Headache Disorders II 7.2.3). There was no previous history of head or spinal trauma. Examination, including funduscopy, was unremarkable. Her blood pressure on admission was 116/64. Her D-dimer level was within the normal range.
She was initially treated with 3500 units of subcutaneous tinzaparin once a day to treat the possibility of cerebral venous sinus thrombosis while conservative measures, including bed rest, intravenous fluids, oral analgesics and increased caffeine intake, were used to treat the possibility of intracranial hypotension.
A MRI brain scan was performed, which revealed no abnormalities. In addition, normal venous sinuses were visualised on MR venography (). A MRI brain scan with gadolinium enhancement was not performed due to her early stage of pregnancy. A lumbar puncture was performed revealing a low opening pressure of 4 cm H2O. She was again diagnosed with SIH. She failed to improve on conservative management so an autologous epidural blood patch procedure was performed with injection of 25 ml of autologous blood into the epidural space between the second and third lumbar vertebrae. This provided symptomatic and complete relief of headache.
Time of flight MR venogram illustrating normal venous sinuses.