Swan Kang, Shirin Hamed-Azzam, Fergus Robertson, Indran Davagnanam, David H. VerityRead More
Sinus pericranii (SP) is a rare trans-osseous communication between the intracranial and extracranial venous drainage systems, and has only rarely been reported with signs in the superior eyelid.
In this report, we present a case of SP with venous drainage outflow located in the medial aspect of the upper eyelid.
A 50-year-old Caucasian woman presented with a 5-year history of a painless gradually enlarging left-upper-lid lump. There was no history of neurological deficit or periocular or head trauma, and apart from a history of hypercholesterolemia and a stable left iris nevus for 20 years, she was otherwise entirely well.
Examination revealed a subtly pulsatile fullness to the medial upper eyelid with engorged subcutaneous venous channels in the lateral half of the eyelid (see Fig. 1 and Video 1, Supplemental Digital Content [available online], which demonstrates prominent left-upper-lid vessels with a biphasic pulsatile pattern). However, there was no associated ptosis, and the signs did not change with the Valsalva manoeuvre. Her visual acuity and colour appreciation were normal, as were her pupil reactions to light, and ocular ductions. Finally, her retinal vessels in the left eye were mildly dilated.
Ultrasonography revealed grossly dilated nasal upper lid vessels that were compressible and hypo-echoic. Magnetic resonance imaging with time-of-flight angiography revealed a subtle midline frontal transcranial deficit conducting a vascular channel (see Figs. 2 and 3). This communicates with a prominent vessel, which descends over the forehead to dilated left preseptal draining veins. Digital subtraction angiography (DSA) confirmed a sinus pericranii draining the cerebral veins of the front third of the brain and communicating with left preseptal veins and thereafter the left facial vein (see Fig. 4, Fig. 5, Fig. 6).
Therapeutic options, including monitoring and surgical occlusion of SP, were considered. Although considered to be an accessory SP, a significant drainage area was noted on DSA, and thus occlusion of the venous connection was considered potentially unsafe, with possible compromise to frontal lobe drainage with risks of cerebral hemorrhage or infarction. Thus, and in the absence of recent progression, or functional or major aesthetic compromise, a period of active monitoring was agreed.
The report adhered to the ethics principles outlined in the Declaration of Helsinki as amended in 2013. A written consent was obtained and is on file.
SP is an unusual venous anomaly characterized by communication of scalp veins with an underlying dural sinus. The condition was first described by Stromeyer in 1850.
SP typically presents in childhood with a nonpulsatile reducible soft tissue mass that increases in size with increased venous pressure.
SP is usually asymptomatic and most commonly occurs in the midline or the frontal region, and communicates with the superior sagittal sinus.
Venous outflow patterns associated with SP are classified as either dominant or accessory,and although most SPs occur as solitary lesions, up to 20% of patients are reported to have associated venous malformations elsewhere.
In some cases of SP with accessory venous outflow, surgical excision or endovascular embolization have been performed to improve cosmesis, to prevent hemorrhage, or to minimize the risk of air embolism.
SP is either congenital or acquired (possibly after trauma), with the former being differentiated from the latter by the presence of an endothelial lining.
Those presenting as a congenital extradural developmental venous anomaly are thought to be formed by transient venous hypertension during embryogenesis,
whereas in traumatic cases, injury to the emissary vein or dural sinuses is thought to lead to abnormal communications between the epicranial and dural venous systems.
Most cases of congenital SP present in children or young adults with equal gender distribution, whereas in traumatic cases there is a male preponderance.
Although patients with SP are often asymptomatic, headache, vertigo, dizziness, and pulsatile tinnitus are sometimes reported.
This anomaly is rare, and its natural history is unclear. In a previously reported case series of 21 patients, only 2 cases of untreated accessory SP resolved spontaneously, and the outcomes of dominant SP—which is almost always managed conservatively—depended on the underlying clinical and comorbid neurological conditions.
Evaluation of SP requires a thorough neurovascular imaging assessment, with investigations including colour Doppler ultrasonography, computed tomography, and magnetic resonance imaging. DSA is the gold-standard imaging modality as it clearly defines the lesional anatomy and the extent of the dependent cerebral venous territory.
There is no consensus or guideline on the optimal management strategy for this condition, and the therapeutic decision is individually tailored. Dominant SP, where the anomaly forms the major outflow for intracranial venous drainage, is best managed conservatively with life-time surveillance to monitor complications such as cerebral venous hypertension or hemorrhage.
Accessory SP can be treated with surgical or endovascular interventions depending on the presence of refractory symptoms and/or psychological impact caused by disfigurement. SP can be treated with endovascular or percutaneous image-guided embolization techniques or with open surgical ligation.
There have been only 4 cases reported in the literature of the SP involving the eyelid, and few cases in adults and pulsatile. The case reported herein is both exceptionally rare and atypical in a number of ways: (i) it involved the upper eyelid; (ii) there was subtle (transmitted) pulsatility and no enlargement with Valsalva manoeuvre; (iii) the patient presented in the fifth decade of life. Although rare, physicians should consider SP in the differential diagnosis of a vascular lid anomaly. Appropriate comprehensive neuroimaging should include catheter angiogram where diagnostic uncertainty persists. Surgery carries significant potential risk of air embolus, venous hypertension with consequent cerebral venous hemorrhage or infarction.