Ruptured giant dermoid cyst of the cavernous sinus

We report a 27-year-old male with a giant dermoid cyst of the cavernous sinus that extended into the posterior fossa and ruptured, causing meningitis and hydrocephalus. We also discuss the radiological features, surgical challenges and management difficulties of such a cyst. A giant dermoid cyst of the cavernous sinus that extends into the posterior fossa and subsequently ruptures has not been previously reported.     

Temporal dermoid cyst with a partial dermal sinus tract

BACKGROUND: Cranial dermal sinuses are rare and occur most frequently in the posterior fossa and along the midline. Likewise, supratentorial dermoid cysts are very uncommon. METHOD: We report a unique case of an adult female patient with both a supratentorial dermoid cyst and an incomplete dermal sinus tract. RESULTS: The patient is a 31-year-old female, who presented with a new onset complex partial seizure. Neuroimaging and surgery revealed a right superior temporal dermoid cyst with an associated dermal sinus tract. Furthermore, the dermal sinus tract was incomplete and had no cutaneous manifestations. CONCLUSION: We present a rare patient with an off midline supratentorial dermoid cyst associated with a uniquely incomplete cranial dermal sinus tract. The dermal sinus tract involved the bone, dura and intradural compartment, without involving the overlying skin. This represents a novel variant in the spectrum of cranial dermal sinus abnormalities.     

Abscedation of posterior fossa dermoid cysts

Dermoid cysts of the posterior fossa are uncommon. When associated with a dermal sinus, these cysts are often diagnosed during early childhood. The main risk of such an association is contamination of the cyst leading to abscedation of the dermoid itself or formation of daughter abscesses within the cerebellar hemisphere. We recently treated a 20-month-old girl who had a congenital dermal sinus leading to an intradural dermoid cyst. In addition to the midline dermoid cyst, computerized tomography revealed an enhancing lesion extending into the adjacent left cerebellar hemisphere. Suboccipital craniectomy was undertaken after 2 days of external ventricular drainage, and the infected dermoid and adjacent cerebellar abscess were excised. Cultures of the operative specimen revealed Corynobacterium aquaticum, Enterobacter sakazakii and Enterobacter cloacae, requiring 6 weeks of intravenous antibiotic therapy consisting of ceftriaxone, penicillin and gentamicin. A diligent literature search revealed only 24 sporadic cases reported over a period of 56 years. All 24 cases were in children (mean age 17 months), and one-third were in infants under the age of 1 year. All but 1 of these patients underwent posterior fossa surgery, with mortality and morbidity rates of 13% and 10%, respectively. Eleven (40%) children had suppuration within the cerebellar parenchyma, while the rest had abscedation of the dermoid cyst alone. Among the cases reviewed S. aureus was the most common agent, occurring with a probability of 64%. Key issues for appropriate management of these benign lesions are discussed.     

Dermoid cyst in the posterior fossa accompanied by Klippel-Feil syndrome

Objects: Dermoid cysts accompanied by Klippel-Feil syndrome are uncommon, and the coincidence of these two rare diseases suggests an interesting idea about the pathogenesis of a dermoid cyst, which the authors consider with reference to an actual case. A posterior fossa dermoid cyst with dermal sinus and Klippel-Feil syndrome are reported in the same patient. A 23-year-old woman was admitted to hospital with progressive headaches. Methods: Cervical radiography demonstrated C4–5 vertebral fusion, and magnetic resonance imaging revealed a large cystic mass in the midline of the posterior fossa. The cystic lesion was totally removed along with the accompanying dermal sinus. It was histopathologically diagnosed as a dermoid cyst. Conclusion: The rare coincidence of a dermoid cyst and Klippel-Feil syndrome suggests the possibility that a disturbance in the mesoderm before the fourth week of gestation might play an important part in the causation of dermoid cyst. Received: 11 February 2000     

Cervical dermal sinus associated with dermoid cyst

We report a rare case of cervical dermal sinus associated with a dermoid cyst in a 10-month-old infant, who presented with a 1-month history of motor weakness of the right upper extremity. Magnetic resonance imaging showed an intradural extramedullary dermoid cyst extending from C3 to C4, which was connected with the skin dimple along the sinus tract. Total excision of the dermoid cyst and the sinus tract with C3-5 laminectomy resulted in good functional recovery.     

Posterior fossa abscesses secondary to dermal sinus associated with dermoid cyst in children

We report on the rare disease of posterior fossa abscesses secondary to dermal sinus associated with dermoid cyst in 4 pediatric cases seen over a 15-year of period. All children had a small dimple or a pinhole on the skin of the occipital region. On admission they had the signs of increased intracranial pressure, while 3 of them still suffered from signs of meningitis resulting from a bacterial infection through the dermal sinus. CT scan and MR images revealed both the route of the dermal sinus and intracranial lesions. Total resection was successfully achieved before various treatments in all patients without nervous sequelae. In conclusion, a posterior fossa dermoid cyst should be considered in all children with an occipital dermal sinus. Cerebellar abscesses will arise once bacterial infection has occurred through the associated dermal sinus. Radiological studies such as CT scans and MR images can help confirm the diagnosis and locate the lesions. Early neurosurgical interventions including external ventricular drainage, external abscess drainage and primary removal should be planned as soon as possible in accord with the children's condition.     

Dermoid tumour of the lateral wall of the cavernous sinus.

Congenital intracranial dermoid tumors are very rare. Supratentorial dermoid cysts have been more frequently reported over the past decade and they are known to have a predilection for the cavernous sinus. Dermoid tumors originating from the cavernous sinus are usually interdural and thus, presentation with ophthalmoplegia is uncommon. They are congenital benign tumors and are believed to originate from ectopic inclusion of epithelial cells during closure of the neural tube during embryonic development. The location of these dermoid lesions in the cavernous sinus and the complexity of the operative procedure for these lesions have been noted by several authors. In this report, we describe the case of a dermoid cyst that was embedded in the lateral wall of the cavernous sinus and review the literature relating to related cavernous dermoid lesions.     

Posterior fossa dermoid cyst with sinus tract and meningitis in a toddler

Intracranial dermoid cysts are rare, but can cause recurrent bacterial or aseptic meningitis, abscesses or mass effect. We describe a toddler who manifested an occipital "lump" for 6 months without further evaluation. He presented with lethargy and meningitis. A posterior fossa dermoid cyst with a sinus tract was detected by imaging studies. He underwent prompt surgical excision of the cyst and the sinus, and received a prolonged course of antibiotics, because anaerobes were isolated from the contents of the cyst and blood. His postoperative course was complicated, and required the placement of a lumboperitoneal shunt. A high index of suspicion for the detection of dermoid cysts is required when a congenital midline skin lesion such as a dimple or a scalp swelling is reported. Prophylactic excision is indicated to prevent serious sequelae.     

Dermoid cyst of the posterior fossa

Intracranial dermoid tumors represent a rare clinical entity accounting for 0.1-0.7% of all intracranial tumors. Their location in the posterior fossa is uncommon. We report a 16-year-old male patient who presented with clinical signs of increased intracranial pressure and cerebellar symptoms. The CT scan revealed a median cystic lesion of the fourth ventricle causing an active triventicular hydrocephalus. The MRI showed a median well shaped cystic lesion, of low signal intensity compared to the CSF, with capsular contrast enhancement. He underwent endoscopic third ventriculostomy before subtotal removal of the lesion. The postoperative course was uneventful, and the histological diagnosis was a dermoid cyst. Through this observation, we aim to discuss the clinical, and radiological aspects of the posterior fossa dermoid cyst, and to review the therapeutic strategies.     

Paradoxical Exacerbation of Symptoms with Obstruction of the Venous Outflow after Gamma Knife Radiosurgery for Treatment of a Dural Arteriovenous Fistula of the Cavernous Sinus

A 59-year-old female presented with progressive right proptosis, chemosis and ocular pain. An imaging work-up including conventional catheter angiography showed a right-sided dural arteriovenous fistula of the cavernous sinus, which drained into the right superior petrosal sinus, right superior ophthalmic vein, and right inferior ophthalmic vein, and cortical venous reflux was seen via the right petrosal vein in the right posterior fossa. After failure of transvenous embolization, the patient underwent Gamma Knife radiosurgery (GKRS). At one month after GKRS, she developed increasing ocular pain and occipital headache. Repeat angiography showed partial obliteration of the fistula and loss of drainage via the superior and inferior ophthalmic veins with severe congestion, resulting in slow flow around the right cerebellar hemisphere. Prompt transarterial embolization relieved the patient''s ocular symptoms and headache. We report on a case of paradoxical exacerbation of symptoms resulting from obstruction of the venous outflow after GKRS for treatment of a dural arteriovenous fistula of the cavernous sinus.     

Intracranial paramedian hourglass-shaped dermoid associated with hereditary steatocystoma multiplex.

This is the first report to describe the coexistence of two rare diseases, intracranial paramedian hourglass-shaped dermoid and steatocystoma multiplex. A 46-year-old female with a history of steatocystoma multiplex, bradydactylies and kyphosis showed oscillopsia, ataxia and hemifacial spasm. MRI findings suggested a giant dermoid cyst extending from the left middle temporal fossa to the cerebellopontine angle, and this was confirmed surgically. We propose the involvement of some genetic factor or pathological process common to both disorders, steatocystoma multiplex and dermoid. From the clinical point of view, patients with cystic skin lesion should also be checked for intracranial lesion.     

Multiple aneurysms associated with bilateral carotid occlusion and venous angioma: surgical management risk-case report.

A unique case of multiple aneurysms associated with bilateral carotid artery occlusion and venous angioma is described. A 42 year old female presented with subarachnoid haemorrhage. Cerebral angiograms demonstrated(1) a ruptured saccular aneurysm in the right posterior cerebral artery,(2) bilateral occlusion of internal carotid arteries,(3) a rete mirabile in the subtemporal fossa fed by left external carotid artery which connected with the internal carotid artery at the cavernous portion where a saccular aneurysm had formed, and(4) a venous angioma in the posterior fossa. The ruptured aneurysm of the posterior cerebral artery was obliterated preserving the anterior choroidal arteries. However, a left hemiparesis developed and CT scan revealed a small low density area in the right posterior limb of the internal capsule postoperatively. A ruptured aneurysm associated with bilateral extracranial carotid occlusion poses a clinical dilemma and treatment of such cases is challenging and difficult. The non-surgical and surgical outcomes of ruptured cerebral aneurysms associated with internal carotid occlusion are reviewed.     

儿童颅内外沟通巨大皮样囊肿合并颅内感染的诊治分析

目的 探讨儿童颅后窝中线部位颅内外沟通巨大皮样囊肿继发颅内感染的诊治方法及治疗效果。方法 回顾性分析2016~2020年收治8例颅后窝中线部位、颅内外沟通巨大皮样囊肿继发颅内感染的临床资料。结果 8例均有头皮窦道、经颅骨及硬脑膜缺损使颅内外皮样囊肿相沟通,并继发颅内感染。首选敏感抗菌素控制感染达标后,开颅手术治疗,囊肿完整切除7例,顺利治愈,术后3周内出院;1例因囊壁炎症性浸润,分离时囊壁破裂,内容物外溢,全切囊肿后,反复温生理盐水冲洗,术后发生无菌性脑膜炎、延迟2个月治愈出院。全部患儿至少随访3个月,未见复发。结论 儿童颅后窝中线部位颅内沟通的皮样囊肿易反复继发颅内感染,应尽早诊断,首选敏感抗菌素控制感染后手术完整切除颅内外沟通的皮样囊肿,可取得良好效果     

Unusual cause of cerebellar abscess: occipital dermal sinus and dermoid cyst

Cerebellar abscess induced by a contiguous dermal sinus is a rare event. In a large series of acute posterior fossa abscedation, otogenic suppuration is the cause in 93% of the cases, while hematogenous infection is infrequent, probably because of the relatively weak bloodflow in this part of the central nervous system. The authors present an occipital dermal sinus and dermoid cyst revealed by a cerebellar abscess. The literature is reviewed briefly, treatment and prophylactic measures are discussed.     

Posterior fossa dermoid in association with Klippel-Feil syndrome--a short report.

A posterior fossa dermoid cyst in association with the Klippel-Feil syndrome, in a 4 year old child is reported. Early diagnosis to prevent complications like neural compression, cyst rupture and staphylococcal meningitis justifies investigation for posterior fossa dermoids in cases of Klippel-Feil syndrome. Their embryological basis is discussed.     

Dermoid cyst mimicking hematoma in the posterior fossa.

Intracranial dermoid cysts are usually reported to be associated with long lasting or waxing-waning symptoms. Computer tomography (CT) scans usually depict such neoplasms as well-defined areas of low density. This report is about a case of a dermoid cyst, the acute clinical features and CT hyperdensity of which mimicked a hematoma in the posterior fossa. The association of acute onset with CT hyperdensity makes this case of dermoid cyst very unusual.     

Radiologically atypical congenital posterior fossa dermoid cyst presenting late in life.

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-eighted 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.     

Cavernous sinus dermoid cyst with unusual parapharyngeal development

We report a case of dermoid cyst arising in the cavernous sinus and parapharyngium areas through the foramen lacerum. A 12-year-old girl was hospitalized in October 1966 for progressive intracranial hypertension. CT before a prior surgical intervention in 1994 showed an intracranial process suggesting a dermoid cyst. CT performed in October 1996 showed a temporal process at the left side measuring 8 cm x 5 cm and well circumscribed with a thin wall. This process extended towards the parapharyngium area through the foramen lacerum. The exocranial part measured 4 cm in diameter. The density of the process strongly suggested a mature dermoid cyst with fatty and calcium components. The histology study confirmed the diagnosis of dermoid cyst. The frequency of intracranial dermoid cysts is 2 to 3 times less than that of epidermoid cysts. This is an uncommon process accounting for 1% of intracranial tumors occurring in children. In the sustentorial spaces, the process occurs more frequently in the parasellar area. To our knowledge, exocranial extension has not been reported to date.     

Primary intracranial squamous cell carcinoma arising in an epidermoid cyst--a case report and review of literature

Primary intracranial squamous cell carcinoma is extremely rare, with most of the cases arising from malignant transformation of an epidermoid or a dermoid cyst. We report here a case of a 45-year-old male patient who presented with 1-month history of intermittent headache and recent onset of altered sensorium. Imaging revealed a midline posterior fossa mass lesion compressing the fourth ventricle and causing hydrocephalus. A provisional diagnosis of dermoid cyst was considered. Histopathological examination revealed a squamous cell carcinoma possibly arising from an underlying epidermoid cyst. This entity is being reported for its rarity.     

上颌窦-翼腭窝-海绵窦手术入路的显微解剖学研究

目的对上颌窦-翼腭窝-海绵窦手术入路相关结构进行显微外科解剖学研究,为手术入路提供解剖学基础.方法利用10例经福尔马林固定的国人成人尸头共20侧,完全模拟该手术入路,对入路相关解剖标志进行了详细地显微解剖、观察、拍摄、测量和统计.结果上颌窦后壁与翼腭窝区结构复杂,有重要的神经和血管经过,是颅前、中窝与鼻腔、窦肿瘤互相蔓延的通道.该入路可较好地显露海绵窦前外侧相关的结构.结论该手术入路是海绵窦入路的一个特殊方法,拓展海绵窦手术入路的路径,为利用颅底硬膜外间隙进行入路提供了依据.