|HOW I DO IT
|Year : 2018 | Volume
| Issue : 2 | Page : 33-37
Sinus Pericranii: An Igims Experience
Keashav Mohan Jha1, Sudhir Kumar1, Om Prakash Gupta2, Samrendra Kumar Singh3
1 Professor, Department of Neurosurgery, IGIMS, Patna, India
2 Associate Professor, Department of Neurosurgery, IGIMS, Patna, India
3 Assistant Professor, Department of Neurosurgery, IGIMS, Patna, India
|Date of Web Publication||10-Dec-2020|
Om Prakash Gupta
Associate Professor, Dept. of Neurosurgery, IGIMS, Patna, Bihar
Source of Support: None, Conflict of Interest: None
Sinus pericranii (SP) is a rare, generally benign venous varix in extra cranial location that is connected to intracranial venous system. In this study, we will share our experience with five cases of sinus pericranii operated at our institute along with considerations of preoperative surgical risk. In this study, two cases of Sinus pericranii with associated craniosynostosis were not included. Out of 5 patients in this study, 4 patients were of congenital origin and another with history of trauma. The study population consisted of three male and two female. There were three children with age of 11, 18 and 36 months. Other two were young adults (one male and other female). One child was referred to us in hemodynamically unstable condition after wrongly diagnosed and operated as epidermoid cyst. All five sinus pericranii patients were operated with excellent results. We will share our experience with these five cases followed by a review of literature.
Keywords: Sinus Pericranii, Venous anomaly of cranium, Diagnosis of Pericranii, Surgical treatment of sinus pericranii
|How to cite this article:|
Jha KM, Kumar S, Gupta OP, Singh SK. Sinus Pericranii: An Igims Experience. J Indira Gandhi Inst Med Sci 2018;4:33-7
| Introduction:|| |
Our reason for presenting this paper is the rarity of this unusual and interesting anomaly on the one hand, and on the other hand the fact that we had seen seven cases including two associated with craniosynostosis. These two patients associated with craniosynostosis were not included in this study. All other five cases were operated with excellent results.
In normal situation, there is no connection between the intracranial and extra cranial venous system. Sinus pericranii is a rare venous varix in extracranial location connected to the intracranial venous system.
This condition was first described and operated by Percival Pott in 1760. Hecker in 1845 for the first time published a case report as ‘varix spurious circumscriptus venae diploicae frontalis’. In 1850 Stromeyer described this anomaly and proposed the term sinus pericranii. It is found beneath or in the periosteum of the cranial vault and connected with an intracranial sinus through anomalous diploic veins of congenital or acquired origin,. Sinus pericranii presents mostly in children as small circumscribed swelling of the scalp in vicinity of sagittal or transverse sinus,,. Some case will come for treatment in young adult, though swelling was present from childhood, as in one of our case. Most of sinus pericranii are of congenital origin. Trauma can lead to sinus pericranii as seen in one of our case.
It is now appropriate to reconsider this disorder, as advances in angiographic techniques have clarified the pathogenesis and categorization of this disease more precisely.
In this paper authors discuss their seven years experience with sinus pericranii at a single centre. They also tried to clarify the role of surgery based on their experience.
| Methods:|| |
Between January 2011 to December 2017, eleven patients with cystic vascular swelling in the region of venous sinuses and suspected to be Sinus pericranii were admitted and investigated in our department. Apart from routine investigation, CT Scan, MR angiogram and Digital subtraction angiogram was done. Seven of them were diagnosed as sinus pericranii. Among them, two were associated with craniosynostosis and were not included in this study. All other five cases were operated. These cases were followed up at the interval of six months in our outpatient clinic to rule out recurrence. Summary of all cases are provided in [Table 1]. two cases are described in detail.
| Illustrative Cases:|| |
Eighteen month old boy was presented with soft midline swelling in the right posterior frontal region. The child had normal delivery. The physical and mental development for child had been normal. There was no significant history of trauma. Parents noticed swelling, when child was 6 month old and this gradually increased in size. The lesion was 2.5cmX 2.5cm in recumbent position with well demarcated boundaries. The lesion was soft and fluctuant and did not move over cranium. Skin overlying the lesion was movable. There was associated hemangioma on the face.
Plain X ray of skull was normal. On CT scan of brain an isodense lesion was seen which homogenously enhanced with contrast. Abnormal communication between the lesion and intracranial venous sinuses could not be demonstrated.
Operation: A paravertebral incision was made lateral the lesion, taking care not to injure the vascular lesion. It was adherent to skull and looked like a multi loculated cyst. Numerous scalp veins including three large feeders to sinus pericranii from sagittal sinus was identified, coagulated and ligated. The lesion was removed in enblock, with meticulous micro vascular technique. Bone wax was used to control diploic bleeding. It was dissected up to skull defect through which lesion communicated with the sagittal sinus. After disconnection of sinus pericranii from the sagittal sinus, pulsation from the malformation disappeared. After completion of surgery, absence of residual lesion was confirmed by Valsalva maneuver and puffing patient in Trendlenberg position
Macroscopically, the specimen was a collection of spongy tissue and was loculated. The inner surface contained multiple small holes corresponding to connection between the lesion and intracranial venous system. Histopatholgical examination showed plenty of fibrous connective tissue and irregular sinuses lined by endothelium. At places large veins of irregular thickness of was seen. At other places there was proliferation of small vessels which lacked smooth muscles. Postoperative period was uneventful and patient was discharged home after 48 hours. Patient was regularly followed up for six years and has no recurrence
3 year old male child was referred to us in moribund condition from another medical college in the city with history of torrential uncontrolled bleeding during an operation of the cystic swelling in the scalp. The child had a cystic swelling in midline in posterior frontal region since the age of one year. It had recent increase in size. The child was seen at their General surgery OPD and was diagnosed to have epidermoid cyst. Plain X-ray of skull was normal. Routine hemogram and other routine investigations were normal. CT scan or MRI was not done. Patient was taken for surgery (excision of epidermoid cyst) as an outpatient procedure. During surgery, inadvertently the wall of the lesion was incised and it started bleeding torrentially. Bleeding was controlled by packing and tight bandage was sent to us as there was no neurosurgical facility in that hospital..
We received patient in hemodynamically unstable condition with very feeble pulse and unrecordable blood pressure. Two units of blood were transfused before patient was shifted to OT. Another two units were arranged before it was decided to open bandage and explore the lesion.
Under general anesthesia patient was positioned supine with head end elevated. Previous dressing and packing was opened. The lesion was in frontal region at midline. There was incision on the wall of the lesion and it was packed tightly with gauze. During surgery, bleeding from incised wound was controlled by continuous finger pressure of the assistant. A semicircular incision was made lateral to the lesion with base toward midline. The wall of the lesion was identified laterally. It was located beneath periosteum. The lesion was meticulously dissected from underlying skull surface and peripheral periosteum from all side. During this process many tiny venous communications between the lesion and underlying bones were coagulated and cut. Bone wax was used to control bleeding from large diploic emissary veins. After this bleeding from the incised wound was controlled and the lesion could be excised completely. Postoperative period was uneventful. Patient required one unit of blood during surgery. Patient was not transfused in post operative period. Repeat CT scan of the child after 48 hours was normal and was discharged from hospital after one week. Histopatholgical examination of the lesion was reported as vascular anomaly consistent with sinus pericranii. At follow up after two years, patient was doing well with no recurrence of lesion.
[Table 1] Summary of Five Operated Cases of Sinus pericranii (Clinical features radiological findings, treatment and outcomes)
| Discussion:|| |
There are few published small series and individual case reports dealing with Sinus pericranii. Till date, about 150 cases of sinus pericranii are reported in literature. Due to rarity of this condition, there is no agreement on diagnosis, classification and management of this condition. Majority of cases are not classified based on angiography and with respect to dominance. It is now; appropriate to reconsider this disorder, as new radiological techniques have clearly defined the role of surgery. In this article, we will review our seven year experience at a single centre (IGIMS). Two cases out of seven were associated with craniosynostosis and were excluded from the study. Rest five cases were operated with good results.
Since the description by Hecker (1845) and Stromeyer (1850), many nomenclatures and classifications of this lesion had been reported (1, 2, 3 and 4). Fevre and Modec proposed the first classification system for Sinus pericranii in 1936.
Gerlach et al in 1967, defined it as a varix and classified into three types based on the size of venous communications between intracranial and extracranial part of this lesion. Newton TH and Troost BT described this communication as large, tortuous and thinned wall vascular Channels.
Volkmann (1950) classified it into "true sinus pericranii" and "pseudo sinus pericranii" based on whether it filled with raised Intra Cranial Pressure and disappeared with compression.
Mastins' classification based on etiology into congenital, spontaneous and traumatic is widely adopted at present,. Congenital pathogenesis was first proposed by Muller. Spontaneous origin is due to development of varix as a result of some chronic disease of skull leading to pressure erosion of the skull,. Fractures of skull, injury to an emissary vein or direct sinus injury have been contemplated as etiology of traumatic sinus pericranii.,,,. For the first time, Hahn (1928) described histopathology of sinus pericranii. He did not find histological difference between spontaneous and traumatic sinus pericranii.
Commonly, sinus pericranii is present in midline along the superior sagittal sinus. It is mostly in frontal and parietal region,,,. Rarely, it is in a lateral location,,.
The exact location in relation to periosteum is controversial. Ohata et al reported that lesion was beneath periosteum in 60% of their cases.
Most authors feel that Sinus pericranii is a clinically benign condition and raise only cosmetic concern. There had been reports where sinus pericranii was wrongly diagnosed as epidermoid cyst of skull,. This series also included one case where the sinus pericranii was diagnosed as epidermoid cyst and was operated without proper precautions and investigations leading to disaster.
In Pre CT era it was diagnosed by conventional angiography and Sinography,,,. CT scan allows direct visualization of bony defect. Osteolytic lesions can be defined well using bone windows. The lesion is slightly hyper dense compared to brain and subcutaneous tissue and will enhance with contrast. The enhancement will depend on the vascularity of lesion,,. MRI with or without MR angiography provides a definitive diagnosis of Sinus pericranii and its pattern of drainage into dural sinus. MR imaging will show signal void. Multiplanar MR shows the relationship of the lesion with the adjacent sinus,,. Sadler, et al suggested that during this procedure signal would increase after the injecting intravenous gadolinium.
DSA is gold standard in the diagnosis of sinus pericranii. It shows cranial circulation in venous phase including sinus pericranii and classify into dominant and accessory pattern. Gondalfo et al explained that dominant sinus pericranii are untreatable as it serves as a major venous outflow channel to intracranial content. In comparison, accessory sinus pericranii can be safely operated because only a small portion venous of outflow from brain traverses it. This classification of sinus pericranii into dominant and accessory, has been important criterion for determining whether this lesion can be safely operated,.
The sinus pericranii had been reported to be associated with vascular malformation, hemangioma of cerebellum, or retina as part of von HippelLindau syndrome, blue nevus syndrome, venous cavernoma of scalp, hemangioma of tongue and craniosynostosis,,,.
Spontaneous regression of sinus pericranii had been reported in literature,. Most cases had been operated because of cosmetic reasons. Sinus pericranii can present with hemorrhage, infection, air embolism, intracranial hypertension and sinus thrombosis. In one of our cases, patient was wrongly diagnosed, leading to incision of the lesion with disastrous result. This led clinician including us to pursue an aggressive intervention,,,.
| Conclusion:|| |
Sinus pericranii is a challenging diagnosis for clinician. This rare vascular anomaly which is seldom symptomatic and is often misdiagnosed. Sinus pericranii is a clinically benign condition and is operated for cosmetic reasons. There are reports, where sinus pericranii presented with hemorrhage, infection, air embolism, intracranial hypertension and sinus thrombosis. One of patient was wrongly diagnosed, leading to incision of the lesion with disastrous result. Neuro imaging has very important role in the diagnosis and classification of this uncommon condition. If operation is contemplated, detailed analysis of drainage pattern should be done and it should be avoided in case of dominant Sinus pericranii.
|Figure 1: Case 1 One and half year old boy admitted with soft tissue swelling in left posterior frontal region near midline. Parents noticed swelling when child was 6 month old. This swelling gradually increased in size.|
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|Figure 2: Case 1 Post contrast CT scan showed connection of the lesion to superior sagittal sinus.|
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|Figure 3: Case 2 24 year female presented with soft swelling in midline frontal region since birth. Initially swelling was very small, which had increased in size and had extreme pain in forehead and scalp for last one year. Swelling was absent in sitting or standing in upright position. It will appear only when patient will bend, cough or sneeze.|
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|Figure 4: Case 2 Microscopic examination of the wall of the lesion showed thick fibrous connective tissue with large sinuses lined by endothelial cells and small non muscular vessels. (Hematoxylin& Eosin × 80 original magnification). |
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|Figure 5: Case 3 Micro photograph of the wall of excised sinus pericranii showing the variable sized thin walled vessels lined by endothelium along with fibrocollagenous ssue and absence of muscular layer.(Hematoxylin and Eosin × 40 original magnifica on)|
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|Figure 6: Case 4 A 11 month girl child presented with a pinkish red mass of the size of 2cm× 2 cm in the midline in posterior frontal region. This has been present since birth. The mass varied in size with Valsalva maneuver or dependency. An underlying bony defect was palpable (due to non fusion of bones)|
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]