Remember the Veins

History and Physical

A 13-month-old girl was evaluated in the emergency department for gastroenteritis and dehydration and discharged after 48 hours. Twenty days later, she returned with vomiting, irritability, and loss of ambulation. Family history was unremarkable. General examination showed a sick and irritable infant. Neurological exam showed right greater than left esotropia and generalized hypotonia with hyperreflexia. The anterior fontanelle was bulging.

Diagnostic Workup

Head CTV ( Fig. 25.1 ) and brain MRI ( Fig. 25.2 ) showed superior sagittal sinus thrombosis. Blood count showed microcytosis with a hemoglobin level of 7.5 g/dl. Lumbar puncture showed normal pressures and CSF composition. The patient received anticoagulation for 5 months, with clear clinical improvement and normalization of the venous angiogram at 4 months ( Fig. 25.3 ). She was also started on iron supplementation for her microcytic anemia.

A head C T scan shows a round defect in the posterior midline region marked by an arrow. The illustration shows an axial C T scan of the head. An arrow points to a round low density area at the inner margin of the skull in the posterior midline. Cerebral gyri and sulci appear on both sides of the midline. — Fig. 25.1

A sagittal brain M R I scan shows an arrow that points to a bright area near the superior sagittal sinus. The illustration shows a sagittal M R I scan of the head. An arrow points to a bright segment along the upper midline of the brain. The scan shows the cerebral cortex with visible gyri, the corpus callosum that arches above the ventricles, the cerebellum that lies behind the brainstem, and the nasal and oral structures in the lower left. — Fig. 25.2

A sagittal magnetic resonance venography scan shows a dense midline vascular structure with surrounding cerebral vessels. The view shows a sagittal magnetic resonance venography scan of the head. A thick midline vascular structure extends from the front to the back of the cranial cavity. Curved vessels radiate outward from central segments, with several large veins that arches through the middle and lower brain regions. Smaller vessels form a network throughout the cerebrum. — Fig. 25.3

Clinical Differential Diagnosis

Central nervous system infection can present with neurologic deterioration but should have associated meningeal signs, fever, and/or CSF abnormalities.

Idiopathic intracranial hypertension can produce false localizing signs (irritability, vomiting, cranial nerve VI paralysis). Lumbar puncture should show high CSF pressures, and papilledema may be seen at fundoscopy.

Brain tumors and other intracranial masses can produce secondary intracranial hypertension with mass effect and herniation in severe cases.

Imaging Differential Diagnosis

Lemierre syndrome involves septic thrombophlebitis secondary to head and neck infections. The inciting infection should be identified, and the neck veins are often involved first ( Fig. 25.4 ).

An axial cervical C T scan shows an area marked by an arrow on the left side. The view presents an axial cervical C T scan with contrast. An arrow on the left side points to a distinct round region near the lateral edge of the neck. The scan shows multiple anatomical structures that includes vertebrae, airways, soft tissue, and bone. Teeth appear in the upper part, and a large ring shaped structure appears centrally. Several symmetrical high density structures are visible on both sides. — Fig. 25.4

On a noncontrast examination, posttraumatic subdural hematomas can appear hyperdense and track eccentrically along the dural reflections and sinuses ( Fig. 25.5 ).