- Vivian L Smith Department of Neurosurgery, University of Texas Medical School at Houston, Houston TX, USA
- Department of Pathology, University of Texas Medical School at Houston, Houston TX, USA
Vivian L Smith Department of Neurosurgery, University of Texas Medical School at Houston, Houston TX, USA
DOI:10.4103/2152-7806.113357Copyright: © 2013 Esquenazi Y This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
How to cite this article: Esquenazi Y, Kerr K, Bhattacharjee MB, Tandon N. Traumatic rupture of an intracranial dermoid cyst: Case report and literature review. Surg Neurol Int 12-Jun-2013;4:80
How to cite this URL: Esquenazi Y, Kerr K, Bhattacharjee MB, Tandon N. Traumatic rupture of an intracranial dermoid cyst: Case report and literature review. Surg Neurol Int 12-Jun-2013;4:80. Available from: http://sni.wpengine.com/surgicalint_articles/traumatic-rupture-of-an-intracranial-dermoid-cyst-case-report-and-literature-review/
Background:Dermoid tumors are benign congenital cystic lesions that usually present with local mass effect. Very rarely, they present as spontaneous ruptures. Traumatic rupture of these dermoid cysts is an extremely rare event and only a handful of such cases have been ever reported.
Case Description:A 47-year-old female presented to our hospital with a ruptured intracranial dermoid cyst following a mild head injury. The ruptured cyst contents were disseminated into the subarachnoid and intraventricular compartments, resulting in an obstructive hydrocephalus. After medical stabilization, she underwent gross total resection of the cyst using combined transsylvian, transcortical-transventricular, and sub-frontal approaches. A ventriculo-peritoneal shunt was eventually also needed.
Conclusion:Traumatic rupture of an intracranial dermoid cyst is an extremely rare event and this is only the fourth such case reported in the literature. We presume that this rupture occurs due to sudden shifts in the cyst sac, which is adherent to some partially mobile intracranial contents. Although computed tomography (CT) is often adequate in making a diagnosis of this entity, magnetic resonance imaging (MRI) provides complete characterization of the extent of lipid dissemination, and is essential for operative planning. Intravenous steroids at presentation are helpful in managing the aseptic meningitis associated with rupture. Complete surgical resection is the goal, but must be weighed against the risk for injury to nearby vital structures. Hydrocephalus should be managed promptly, and patients should be monitored for it closely in the perioperative period. Even though the recurrence rate with subtotal resection is extremely rare, follow up should be done routinely.
Keywords: Aseptic meningitis, communicating hydrocephalus, dermoid cyst, intracranial rupture
Dermoid cysts, also known as dermoid tumors, are rare benign congenital cystic masses of embryological origin that represent 0.04-0.6% of all intracranial tumors.[
A 47-year-old healthy female struck the back of her head on a shelf in a closet at her home and suffered a transient loss of consciousness. She had a headache immediately thereafter and this progressively worsened over the next 2 days. She also developed nausea and vomiting and photophobia over this time interval. On physical examination at initial presentation to our hospital, she was found to have nuchal rigidity, but otherwise her neurological examination was unremarkable. A noncontrast computer tomography (CT) scan revealed a heterogeneous very low attenuation lesion extending from the floor of the anterior cranial fossa superiorly along the falx, with diffuse scattered areas of low-attenuation throughout the subarachnoid space and lateral ventricles. These areas of low attenuation resembled the appearance of pneumocephalus. Magnetic resonance imaging (MRI) scans revealed a large multi-lobulated lesion extending along the floor of the left anterior and middle cranial fossae that was hyperintense on T1 weighted sequences at all locations except at the skull base attachment at the planum sphenoidale, where it was hypointense. Droplets of T1 hyperintense material were present throughout the subarachnoid space, including the sylvian fissures bilaterally, the quadrigeminal cistern, the ambient cisterns, and sulci over the frontal convexity. Fat-fluid levels were present throughout the ventricular system, with evidence of obstructive hydrocephalus [Figures
Preoperative sagittal and coronal T1 MRI of the brain (a and b) shows a large T1 hyperintense multilobulated lesion extending along the floor of the left anterior and middle cranial fossa, with areas of T1 hypointense signal around the component in the left anterior cranial fossa. Locules of T1 hyperintense material are present throughout the subarachnoid spaces along the sylvian fissures bilaterally, extending into the frontal sulci, parasagittal frontal lobes, tectum, and into the posterior fossa. Fat-fluid levels are present within the ventricular system, with evidence of obstructive hydrocephalus
The patient was immediately started on high dose intravenous (IV) steroids and monitored in the intensive care unit. Two days after admission, her symptoms of headaches and nausea had resolved. The patient was taken to the operating room on hospital day three. The tumor was removed via a left fronto-temporal craniotomy and orbital osteotomy with combined transsylvian, sub-frontal, and transcortical-transventricular approaches. The sylvian fissure was opened widely, and large amounts of fat globules as well as some hair fragments were found within the fissure. The ventricular contents of the ruptured cyst were approached through a small corticectomy in the middle frontal gyrus. The frontal horn of the lateral ventricle was entered, where a large amount of fatty material expressed itself under pressure. Sebaceous material and hair were removed from a contained cyst that was dissected away from the surrounding ependyma. The sub-frontal exposure allowed access to the remainder of the cyst at the skull base, where it was dissected away from the optic nerve and the carotid artery in a piecemeal fashion and away from the dura of the skull base. During the dissection, and at the conclusion of the case, the ventricular system and subarachnoid space were thoroughly irrigated with saline solution.
Once a gross total removal of the cyst was achieved, communication between the basal-frontal and the intraventricular approaches was accomplished and a septostomy was performed. A ventricular catheter was placed and externalized to allow residual fat droplets and sebaceous material in the ventricular system a path to exit the cranium [
Two weeks after discharge the patient was seen in clinic with worsening headaches and a subgaleal fluid accumulation. Repeat head CT showed evidence of dilatation of the ventricular system consistent with communicating hydrocephalus. The patient underwent placement of a right frontal ventriculo-peritoneal shunt.
Dermoid cysts comprise 0.04-0.6% of all intracranial tumors[
Patients with spontaneous ruptured intracranial dermoid cysts present with a variety of neurologic symptoms such as headaches, seizures, nausea, visual loss, facial numbness, neck pain, meningeal signs, and altered consciousness. Some of these patients will report progressive neurologic symptoms prior to the acute rupture due to the mass effect of the growing cyst. These symptoms depend on the location of the cyst, but can include worsening headache due to hydrocephalus, and progressive visual loss from optic chiasm compression.[
CT scans of patients with dermoid cysts reveal well-circumscribed, homogenously hypodense lesions due to the predominant lipid content of the tumor. Heterogenous appearing dermoid tumors (like in this case) occur when there is a greater admixture of hair, calcification and epidermal debris. Hypodense areas found in the subarachnoid space and ventricles represent lipid droplets that have disseminated after initial rupture. Dermoid cysts do not enhance with the administration of contrast, and no edema is seen.[
In patients with large dermoid cysts with previously reported neurologic symptoms, surgical resection is recommended.[
In our case, as in others,[
Although recurrence after subtotal resection is extremely rare, close follow up is recommended. In the four reported cases of recurrence in the literature, times to presentation after initial resection were 5, 12, 13, and 66 months.[
Traumatic rupture of an intracranial dermoid cyst is an extremely rare event. We report the fourth such case in the literature, and outline a course of effective evaluation and management. Although CT is often adequate in making a diagnosis of this entity, MRI is superior for complete characterization of the extent of lipid dissemination, and for operative planning. IV steroids at the time of presentation decrease the symptoms of aseptic meningitis associated with rupture. Complete surgical resection is optimal, but must be weighed against the risk for injury to nearby vital structures. Hydrocephalus should be managed promptly, and monitored for closely in the perioperative period. The recurrence rate even following a subtotal resection is extremely low, but incompletely resected cysts should be followed routinely.
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