Radiologically atypical congenital posterior fossa dermoid cyst presenting late in life

doi:10.1016/j.jocn.2007.03.023

Journal of Clinical Neuroscience

Volume 15, Issue 7, July 2008, Pages 835-838

https://doi.org/10.1016/j.jocn.2007.03.023 Get rights and content

Abstract

We report the case of a 74-year-old woman who presented with deterioration in gait, short-term memory loss and urinary incontinence. She had a past history of excision of a cervical dermal sinus tract at the age of 5 years. CT scan in 2004 revealed ventriculomegaly and an extremely hypodense ovoid structure lying in the midline low posterior fossa with calcification anteriorly. On MRI, the lesion was hypointense on T₁-weighted and hyperintense on T₂-weighted images, with incomplete suppression on fluid-attenuated inversion-recovery images and marked restriction on diffusion weighted images. Cerebrospinal fluid isotope study revealed non-communicating hydrocephalus. Posterior fossa crainectomy and removal of the lesion was undertaken. Pathological study revealed a dermoid cyst. Post-operatively, her hydrocephalus persisted and a ventriculo-atrial shunt was inserted with excellent functional recovery.

Introduction

A congenital spinal dermal sinus is present in approximately 1 in 2500 live births and supposedly results from failure of the neuroectoderm to separate from the cutaneous layer during neurulation.¹ Cervical dermal sinus tracts account for only 1% of all dermal sinus tracts.² Epidermoid and dermoid cysts may form at any point along a dermal sinus. Intracranial dermoid cysts are rare. We report the unusual case of a 74-year-old woman presenting with obstructive hydrocephalus caused by a dermoid cyst of the posterior fossa with a past history of excision of a cervical dermal sinus tract at age 5 years. To our knowledge, this is the oldest patient presenting with an intracranial dermoid cyst in the literature.

Section snippets

Case report

A 74-year-old woman who had a history of progressive gait deterioration, becoming wheelchair-bound, short term memory loss and urinary incontinence, was referred on 4 January 2005. CT scan in 2004 revealed ventriculomegaly and an extremely hypodense ovoid structure lying in the midline lower posterior fossa with calcification anteriorly (Fig. 1). On MRI from 1999, 2000 and 2004, the lesion was hypointense, heterogenous on T₁-weighted (Fig. 2a) and hyperintense on T₂-weighted images (Fig. 2b).

Discussion

Intracranial dermoid cysts account for 0.04% to 0.7% of primary intracranial tumors.³ Dermoid cysts with dermal sinus tracts are most often found in infants, children, or young adolescents. They may present as a cutaneous fistula or a skin dimple in infants.[4], [5] They can present with meningitis with subdural or intracerebral absess. Dermoid cysts without a dermal sinus tract can present late, but usually in the third decade of life, with a long history of vague symptoms, most commonly

Acknowledgement

We would like to thank Professor Michael Gonzales, Director of Pathology, at the Royal Melbourne Hospital, for the pathology details.

References (9)

K.C. Wang et al.
Spinal congenital dermal sinus – experience of 5 cases over a period of 10 years
J Korean Med Sci
(1993)
L.L. Ackerman et al.
Cervical and thoracic dermal sinus tracts. A case series and review of the literature
Pediatr Neurosurg
(2002)
G. Rubin et al.
Craniocerebral epidermoids and dermoids. A review of 44 cases
Acta Neurochir
(1989)
W.C. Shen et al.
Dermal sinus with dermoid cyst in the upper cervical spine: case note
Neuroradiology
(2000)

There are more references available in the full text version of this article.

Cited by (14)

Infected intradural dermoid cyst without dermal sinus tract mimicking brain abscess: A case report
2020, International Journal of Surgery Case Reports
Citation Excerpt :
Nevertheless, due to the credo of medicine "Primum non-nocere" meaning "First, do no harm" and benign nature of dermoid cyst, adequate gross total resection with meticulous hemostasis the residual capsule firmly adhering eloquent areas and major vessels was appropriate. Moreover, the risk of recurrence and neurological deficit might be reduced by carefully bipolar coagulation [9,10]. In the case of the infected dermoid cyst, we irrigated meticulously the operative field with saline and povidone-iodine solution.
Infection is a rare complication of intradural dermoid cyst. We reported an infected intradural dermoid cyst without dermal sinus tract mimicking brain abscess.
A 4-year-old boy with no medical history complained of a palpable mass on his head. On examination, the occipital palpable mass was firm and immobile, had no redness, swelling, and pain. The preoperative MRI showed a well-defined, ring-enhancing lesion. White blood count was $12.8 \times 10^{9}$ cells/liter. We have sought no other infection sites. We encountered intraoperatively the pus from the infected mass invading subcutaneous layer and skull bone. We excised completely the tumor and carefully coagulated the residual capsule invading superior sagittal sinus. Histopathological examination was infected dermoid cyst. The infection agent was Staphylococcus aureus. The patient was received systemic antibiotic therapy for 21 days following oral antibiotics for 1 month. He was discharged with no complications.
The diagnosis of infected dermoid cyst was often based on MRI images and especially dermal sinus tract. However, in the absence of a dermal sinus tract, preoperative diagnosing an infected dermoid cyst might be very challenging. The ideal treatment of the dermoid cyst was total resection of the cyst with the epithelial lining. Nevertheless, due to the benign nature of dermoid cyst, adequate gross total resection with meticulous hemostasis the residual capsule firmly adhering eloquent areas and major vessels was more appropriate.
Diagnosing infected dermoid cyst with no dermal sinus might be challenging. Systemic antibiotic therapy after gross total resection was an effective treatment.
Infected Intradural Dermoid Cyst with Complete Dermal Sinus of Posterior Fossa
2018, World Neurosurgery
Citation Excerpt :
Nevertheless, it is not necessary to attempt complete removal of the cyst wall if it firmly adheres to the brain structures. Cauterization is likely to prevent recurrence and reduce the risk of neurologic impairment.6 Extreme caution is advised when the dermal sinus is found to penetrate the occipital region, as a connection between the dermal sinus, dermoid cyst, and cranial venous confluence is possible and an unexpected penetration is associated with rapid and fatal exsanguinations.
Intracranial dermoid cysts are benign embryologic tumors. They occur most often in children and young adults. Infected intradural dermoid cyst with complete dermal sinus of posterior fossa is rare. The main feature of this dermal sinus is the continuity from the intradural cyst through the occipital bone to the skin.
We report 2 cases of infected intradural dermoid cyst with complete dermal sinus of posterior fossa in children, with special attention to the clinical and anatomical features of this rare combination of anomalies.
A 5-year-old boy presented with a midline cystic lesion responsible for triventricular hydrocephalus revealed by a cerebellar syndrome and a dermal sinus. The second case was a 36-month-old girl who had an occipital cystic lesion that was limited by a pseudo-wall with diffusion restriction revealed by a double dermal sinus. We inserted an external shunt to relieve the increased intracranial pressure then performed a suboccipital approach for excision followed by systemic antibiotic therapy. Both cases had satisfactory outcomes.
Infected intradural dermoid cyst with complete dermal sinus of posterior fossa is rare, and a double dermal sinus has not yet published, to our knowledge.
A cystic lesion in the fourth ventricle
2011, Journal of Clinical Neuroscience
A cystic lesion in the fourth ventricle
2011, Journal of Clinical Neuroscience
Rupture of intracranial dermoid cyst with disseminated lipid droplets
2010, Revue Neurologique
Les kystes dermoïdes sont des tumeurs bénignes rares du système nerveux central, d’évolution lente. Ils se développent au cours de la vie embryonnaire, à partir d’une anomalie de fermeture du tube neural et sont essentiellement situés sur l’axe médian du système nerveux central. Ils sont responsables de nombreux symptômes aspécifiques dont les plus fréquents sont les céphalées et les crises convulsives, et peuvent se rompre, surtout de façon spontanée. La rupture est également de symptomatologie variable, allant du caractère totalement asymptomatique jusqu’au décès dans les cas extrêmes.
Nous rapportons deux cas de rupture de kyste dermoïde intracérébral. La rupture du kyste chez un patient, totalement asymptomatique, a été découverte lors d’IRM successives. Chez l’autre patient, la rupture du kyste a été diagnostiquée au cours d’un bilan pour des céphalées quotidiennes. Ces kystes dermoïdes n’ont pas été traités chirurgicalement. Une simple surveillance par IRM a été préconisée devant la bonne évolution spontanée des deux patients.
La chirurgie d’exérèse totale ou sub-totale des kystes dermoïdes n’est pas la seule alternative thérapeutique. Un traitement conservateur avec une surveillance régulière par IRM cérébrale peut être suffisant et doit être discuté si les symptômes de rupture ne mettent pas en jeu le pronostic vital.
Dermoid cysts are rare slow-growing benign tumors of the central nervous system generally diagnosed in the third to fifth decade. They are formed from inclusion of ectodermal elements during neural tube closure, and are mostly located along the cranial or spinal midline axis. They cause many non specific symptoms such as headache and seizures, and may spontaneously rupture spreading fatty droplets into the ventricles and subarachnoid spaces. Rupture of dermoid cysts causes sequelae which may vary from no symptoms to death. In general, subtotal surgical removal is required for ruptured dermoid cysts.
We report two cases of ruptured intracranial dermoid tumor with non-specific clinical presentations. The first rupture was asymptomatic and discovered on brain magnetic resonance imaging (MRI) performed for other purposes. The second case was identified on brain imaging performed because of daily headache. These dermoid cysts were not surgically treated. Surveillance was advised because of the spontaneously favourable outcome observed in both cases.
Surgical removal is not the only treatment of ruptured dermoid cyst. Monitoring with brain MRI can be sufficient if the rupture has no severe clinical impact.
Atypical dermoid cyst of the corpus callosum: a case report
2024, European Review for Medical and Pharmacological Sciences

View all citing articles on Scopus

View full text

Journal of Clinical Neuroscience

Case ReportRadiologically atypical congenital posterior fossa dermoid cyst presenting late in life

Abstract

Introduction

Section snippets

Case report

Discussion

Acknowledgement

Spinal congenital dermal sinus – experience of 5 cases over a period of 10 years

J Korean Med Sci

Cervical and thoracic dermal sinus tracts. A case series and review of the literature

Pediatr Neurosurg

Craniocerebral epidermoids and dermoids. A review of 44 cases

Acta Neurochir

Dermal sinus with dermoid cyst in the upper cervical spine: case note

Neuroradiology

Infected intradural dermoid cyst without dermal sinus tract mimicking brain abscess: A case report

Infected Intradural Dermoid Cyst with Complete Dermal Sinus of Posterior Fossa

A cystic lesion in the fourth ventricle

A cystic lesion in the fourth ventricle

Rupture of intracranial dermoid cyst with disseminated lipid droplets

Atypical dermoid cyst of the corpus callosum: a case report

Case Report
Radiologically atypical congenital posterior fossa dermoid cyst presenting late in life