Sinus Pericranii Associated With Craniosynostosis

Abstract

Sinus pericranii is a vascular tumor of the head having communication with the dural venous system. The tumor enlarges with increased intracranial pressure when the patient is in the lateral recumbent position. Since Stromeyer used the term "sinus pericranii" in his report in 1850, approximately 170 cases have been reported. However, relatively few cases of sinus pericranii have been reported in association with craniosynostosis or by plastic surgeons. In this study, we report seven cases of sinus pericranii associated with craniosynostosis. There were two patients with Apert syndrome, two with Crouzon syndrome, two with oxycephaly, and one with trigonocephaly. The sites of occurrence were the parietal region in six patients and the frontal region in one patient. In either type of case, the tumor was clinically a soft subcutaneous mass of the head. The clinical characteristics of the tumor were its disappearance when the patient was in a sitting position and its appearance when the patient was in a recumbent position or crying. The patients were preoperatively diagnosed with sinus pericranii from imaging test findings, such as from magnetic resonance imaging and aforementioned clinical findings. In all cases, treatment for sinus pericranii was performed during the surgery for craniosynostosis. For patients with lesions in either site, we did not perform craniotomy, and we used a minimally invasive method of tumor excision and resection of the sites of communication using electrocoagulation. Perioperatively, heavy hemorrhage was not observed, and, postoperatively, no recurrence has been observed in any patient. In this study, we examine the etiology, diagnosis, and treatment of sinus pericranii, in particular for patients with craniosynostosis.

Sinus pericranii: diagnosis and management in 21 pediatric patients

“…Single-stage surgery for both conditions has been previously described, 30 although in many cases SP can be secondary to venous hypertension related to craniosynostosis and therefore may improve, or even disappear, after surgical treatment of craniosynostosis, as we found in 1 case. In such cases, we suggest that craniosynostosis should be treated first, and closure of the residual SP may be contemplated at a later stage, if required.…”

“…34,35,40 Sinus pericranii can be isolated (i.e., primitive) or associated with other malformations (i.e., secondary), including craniosynostosis or intracranial venous abnormalities such as dural sinus hypoplasia. 30,50 The pathogenesis of SP is still unclear; however, its frequent association with intra-cranial developmental venous anomalies (DVAs) and other venous anomalies suggests a congenital predisposition. It has been postulated that transient intracranial venous hypertension in the late embryonic period could lead to the development of venous anomalies, including SP, causing partial regression of bony afferents to the primitive dural sinus.…”

Sinus pericranii presenting with macrocephaly and mental retardation

“…[5] Sinus pericranii may be associated with various other anomalies, like systemic angiomas and craniosynostosis. [67] It can be diagnosed by clinical examination and radiological imaging. Usefulness of MR venography and computed tomography angiography has already been described to confidently diagnose sinus pericranii and exclude other mimicking causes of scalp swelling.…”

Sinus pericranii: long-term outcome in a 10-year-old boy with a review of literature

“…Sinus pericranii (SP) was initially described by Hecker in 1845 and the term sinus pericranii was later coined by Stromeyer 1. Due to its rarity, pathogenesis and natural history remains unclear.…”