- Department of Neurosurgery, Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, State University of New York, New York, USA
- Department of Neurosurgery, Women and Children's Hospital of Buffalo, Buffalo, New York, USA
Renée M. Reynolds
Department of Neurosurgery, Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, State University of New York, New York, USA
Department of Neurosurgery, Women and Children's Hospital of Buffalo, Buffalo, New York, USA
DOI:10.4103/2152-7806.181827Copyright: © 2016 Surgical Neurology International This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.
How to cite this article: Shakir HJ, McBride P, Renée M. Reynolds. Dural-based infantile hemangioma of the posterior fossa: Case report. Surg Neurol Int 05-May-2016;7:52
How to cite this URL: Shakir HJ, McBride P, Renée M. Reynolds. Dural-based infantile hemangioma of the posterior fossa: Case report. Surg Neurol Int 05-May-2016;7:52. Available from: http://surgicalneurologyint.com/surgicalint_articles/dural%e2%80%91based-infantile-hemangioma-of-the-posterior-fossa-case-report/
Background:The authors present the unique case of a dural-based, infantile hemangioma located in the posterior fossa of a 15-day-old infant.
Case Description:The patient presented with hydrocephalus. The lesion was identified by magnetic resonance imaging and was subsequently resected. Diagnosis of the lesion was confirmed with immunohistochemistry staining. The patient's hospital course was complicated by transverse sinus thrombosis and a cerebrospinal fluid leak that were treated with anticoagulation therapy and ventriculoperitoneal shunt placement, respectively.
Conclusion:Although hemangiomas are benign entities, our patient's lesion was in the posterior fossa causing compression and hydrocephalus that necessitated resection. We encourage others to consider the possibility of hemangioma in the differential diagnosis of dural-based posterior fossa lesions in infants.
Keywords: Glucose transporter 1, infant, intracranial hemangioma, neonatal ultrasonography, posterior fossa
The most prevalent benign neoplasm of infancy is the infantile hemangioma.[
The patient is a 15-day-old dizygotic twin girl born via uncomplicated vaginal delivery. She was referred to our institution as a result of findings of ventriculomegaly noted on prenatal screening ultrasonography. The cause of the hydrocephalus was unknown at that time. On examination, the patient had a soft but full anterior fontanelle, along with dilated scalp veins. The head circumference was greater than the 97th percentile for gestational age. No focal neurologic deficits were appreciated on examination. Postnatal cranial ultrasonography revealed progressive hydrocephalus as well as a midline echogenic mass in the posterior fossa with mass effect on the cerebellum. Magnetic resonance imaging (MRI) was obtained, which revealed a very large dural-based extraaxial mass along the dorsal aspect of the posterior fossa [
Preoperative images. (a) Sagittal T2 image demonstrating a heterogeneous posterior fossa lesion with compression of the cerebellum and enlargement of the supratentorial ventricles. (b) Contrast-enhanced axial T1 image of the posterior fossa lesion demonstrating avid contrast enhancement. (c) Axial T2 MRI showing significant heterogeneity within the lesion with effacement of the fourth ventricle
Owing to the compressive nature of the lesion and the progressive hydrocephalus, the infant was taken to the operating room for resection of the posterior fossa lesion. Prior to the resection, an external ventricular drain (EVD) was placed in the occipital horn of the right lateral ventricle with care to avoid the egress of cerebral spinal fluid until the tumor was resected. A suboccipital craniectomy and C1 laminectomy were performed. The lesion appeared extremely vascular and clearly emanated from the dura without intraaxial involvement. Because of the patient's size and the low circulating blood volume, minimizing blood loss was imperative. Blood products were on standby. Care was taken not to breach the venous system as the mass was circumferentially dissected away from the surrounding tentorium and brain parenchyma. Cavitron ultrasonic aspiration and bipolar cautery were used to reduce the blood supply to the tumor externally while it was debulked internally. Following resection of the lesion, a large dural defect remained. A dural graft was used to close the defect with the additional support of a large piece of non-suturable DuraGen (Integra Life Sciences, Plainsboro, New Jersey) and Tisseel (Baxter Healthcare, Deerfield, Illinois) because the paper thin nature of the patient's dura made watertight closure challenging.
Because of our patient's small body habitus (2.7 kg), minimizing hemorrhage and maintaining intravascular volume were of utmost importance. In sum, throughout the duration of the case, our patient received 160 ml of lactated Ringer's solution, 90 ml of packed red blood cells, 50 cc of fresh frozen plasma, 40 ml of platelets, and 10 ml of cryoprecipitate. Despite efforts taken to reduce volume loss, the estimated blood loss was 150 ml, whereas the patient's estimated total circulating blood volume was only 230 ml and urine output was 50 ml.
Postoperative imaging revealed gross total resection of the mass and significant decompression of the brainstem and cerebellum [
The pathology report of the lesion showed positive staining of 20% for MIB-1, a marker for actively proliferating cells. Stains for glucose transporter 1 (GLUT1) and CD31 were also positive, indicating that the lesion was an infantile hemangioma.[
At the time of the most recent office evaluation 4 months since her operation and 6 weeks from shunt placement, the patient had completed her course of anticoagulation therapy with follow-up imaging demonstrating patency of all venous sinuses. There has been no recurrence of the mass. She remains on antiepileptic medications for seizure prophylaxis but has not had seizure activity. The patient continues to meet her developmental milestones in subsequent outpatient follow up visits.
One percent of infantile hemangiomas involve the central nervous system, and approximately half of these are intracranial.[
Despite the benign nature of intracranial infantile hemangioma, mass effect from these lesions can cause considerable and permanent damage to the developing brain. Therefore, intervention is required, especially in the face of hydrocephalus, seizures, or focal neurologic deficits. Because our patient had progressing hydrocephalus with enlarged head circumference and effacement of the fourth ventricle with mass effect on the cerebellum, we did not believe a trial of steroids or propranolol would be a reasonable management option, especially because the diagnosis had yet to be confirmed. However, if the lesion had been found incidentally in a less dangerous location and the diagnosis was biopsy proven, conservative management with observation and corticosteroids may be considered because cases of regression after medical therapy have been reported.[
The postoperative transverse sinus thrombosis seen in our patient was likely due to direct surgical manipulation and retraction of the brain parenchyma and venous structures. Volume loss during the procedure may also have been a contributing factor. Our patient did not experience any other signs or symptoms of hypercoagulability, nor is there any reported association of infantile hemangiomas with a hypercoagulable state. In addition, the postoperative timing of the thrombosis and its close proximity to the surgical site lead us to believe it was a sequela of the surgical procedure rather than of the disease process itself.
Although hemangiomas overall are common in infants, the unique nature of our case report stems from its location in the posterior fossa and dural attachment. To our knowledge, this has not been described in the literature. The diagnosis was suspected by classic MRI features and was confirmed by histopathology staining including GLUT1 and CD31. Although hemangiomas are benign entities, our patient's lesion was in the posterior fossa causing compression and hydrocephalus that necessitated resection. We encourage others to consider the possibility of hemangioma in the differential diagnosis of dural-based posterior fossa lesions in infants.
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Conflicts of interest
There are no conflicts of interest.
We thank Paul H Dressel, BFA for preparation of the illustrations and Debra J Zimmer for editorial assistance. The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.
Conception and design: Shakir. Acquisition of data: All authors. Analysis and interpretation of data: All authors. Drafting the manuscript: Shakir, McBride. Critically revising the manuscript: All authors. Reviewed submitted version of manuscript: All authors.
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