Headache in Ruptured Intracranial Dermoid Cysts.

PURPOSE OF REVIEW: Intracranial dermoid cysts are benign, rare mass-occupying lesions of the central nervous system arising from the neuroectodermal cell lines. While rupture of intracranial dermoid cysts is rare, it can present with a variety of clinical manifestations. To explore the headache manifestations among patients with ruptured intracranial dermoid cysts. To our knowledge, limited studies focusing on headache due to the intracranial dermoid cysts rupture have been published to date. A literature review was done through PubMed/Medline. Articles within the past 10 years were reviewed. Articles in languages other than English were excluded. RECENT FINDINGS: Rupture of intracranial dermoid cysts could have various manifestations including headache, seizure, and meningitis. Depending on the location of the cyst, headache secondary to the rupture of the intracranial dermoid cysts could present in different ways. A detailed systematic literature review of headache presentations due to intracranial dermoid cysts rupture is provided.

Location and restoration of function after cerebellar tumor removal-a longitudinal study of children and adolescents.

Sequelae in children following cerebellar tumor removal surgery are well defined, and predictors for poor recovery include lesions of the cerebellar nuclei and the inferior vermis. Dynamic reorganization is thought to promote functional recovery in particular within the first year after surgery. Yet, the time course and mechanisms of recovery within this critical time frame are elusive and longitudinal studies are missing. Thus, a group of children and adolescents (n = 12, range 6-17 years) were followed longitudinally after cerebellar surgery and compared to age- and gender-matched controls (n = 11). Patients were examined (1) within the first days, (2) 3 months, and (3) 1 year after surgery. Each time behavioral tests of balance and upper limb motor function, ataxia rating, and a MRI scan were performed. Data were used for subsequent lesion-symptom mapping of cerebellar function. Behavioral improvements continued beyond 3 months, but were not complete in all patients after 1 year. At that time, remaining deficits were mild. Within the first 3 months, cerebellar lesion volumes were notably reduced by vanishing edema. Reduction in edema affecting the deep cerebellar nuclei but not reduction of total cerebellar lesion volume was a major predictor of early functional recovery. Persistent impairment in balance and upper limb function was linked to permanent lesions of the inferior vermis and the deep cerebellar nuclei.

Traumatic hemorrhage within a cerebellar dermoid cyst.

Intracranial dermoid cysts with hemorrhage are fairly rare. Herein, we reported a 28-year-old female patient with a cerebellar dermoid cyst, which was found accidently on neuro-imaging after head trauma. MR scanning revealed that the lesion was located within the cerebellar vermis and was measured 3.5cm×3.9cm×3.0cm, with hyper-intensity on T1WI and hypo-intensity on T2WI. However, on CT imaging, it showed hyper-dense signals. It was removed completely via midline sub-occipital approach under surgical microscope. Histological examination proved it was a dermoid cyst with internal hemorrhage. In combination with literature review, we discussed the factors that might be responsible for the hemorrhage within dermoid cysts.

An uncommon etiology for a common problem: hirsutism.

Hirsutism is a common problem affecting women that is usually the result of a benign etiology. However, sudden onset or rapidly progressive hirsutism, especially when accompanied by virilizing signs, is suspicious for androgen-producing neoplasms of the ovaries or adrenals. A 28-year-old female presented with the rapid onset of hirsutism and virilizing signs, accompanied by a markedly elevated serum testosterone. Initial imaging studies demonstrated normal adrenal glands and ovaries. She was later discovered to have a rare steroid-secreting ovarian tumor. This case emphasizes the importance of a high level of suspicion for an androgen-producing neoplasm in the patient with sudden onset or rapid progression of virilizing signs and symptoms.

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.

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.

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.

[Ocular dermoids and microphthalmia in a juvenile sheep]. / Okuläre Dermoide und Mikrophthalmie bei einem Jungschaf.

A 6-month-old Leine sheep was presented because of dermal tissue located on the left eye. During the first examination, the animal was clinically silent, apart from the deformed eye. A corneal and conjunctival dermoid and blindness of the left eye were diagnosed. Over a period of a year, the animal displayed conjunctivitis and inflammation of the affected eye. Furthermore, the sheep did not develop according to its age. During histopathological examination of the euthanized animal, microphthalmia and aphakia of the left eye were found in addition to the dermoids. Dermoids are described in humans and in different domestic animals. They can be combined with other congenital malformations. In sheep, dermoids are rarely diagnosed or reported in the literature.

Intracranial dermoid cyst rupture-related brain ischemia: Case report and hemodynamic study.

RATIONALE: Spontaneous rupture of intracranial dermoid cyst is a rare but serious clinical event that can result in cerebral ischemia. Cerebral vasospasm and vasculitis are considered as potential mechanisms of dermoid cyst rupture-related cerebral ischemia. However, the hemodynamic mechanisms between cerebral ischemia and dermoid cyst rupture are not well known. PATIENT CONCERNS: A 55-year-old, right-handed man was admitted to our hospital with sudden receptive aphasia and right-sided hypoalgesia. Brain magnetic resonance imaging (MRI) revealed a ruptured dermoid cyst and watershed infarcts in the left hemisphere. Then brain magnetic resonance angiography disclosed mild stenosis in the left middle cerebral artery (MCA), and further high-resolution MRI demonstrated it was caused by an unstable atherosclerosis plaque. Transcranial Doppler of the patient showed a decreasing tendency of peak systolic velocity (PSV) of the left MCA at different time points after the stroke (from 290cm/s at day 6 to 120cm/s at day 30), indicating a transient vasospasm. However, the time course of dynamic cerebral autoregulation (dCA) seemed different from the PSV. The patient's dCA reached its lowest point at day 8 and was restored at day 10. The time course of dCA indicated a "called procedure" of a cerebrovascular regulating function to deal with the stimulation in subarachnoid space. DIAGNOSES: A dermoid cyst rupture-related cerebral infarction was diagnosed in this patient. INTERVENTIONS: Aspirin (100 mg/d) and atorvastatin (20 mg/d) were given to the patient. A neurosurgical operation was strongly recommended to minimize the risk of further injury of the ruptured dermoid cyst; however, the patient refused the recommended treatment. OUTCOMES: The neurological deficit of the patient was significantly improved on 30 days follow-up. LESSONS: We found that the spread of cyst contents through the subarachnoid and/or ventricular system can induce a vasospasm. Then, dCA was "called" to deal with the stimulation in the subarachnoid space. Compromised dCA seems to be one of the compensatory of cerebral vasospasm after a dermoid cyst rupture.

Imaging for spine tumors and new applications.

OBJECTIVES: Discuss intramedullary, intradural/extramedullary, and extradural spinal tumors including imaging characteristics with emphasis on MR and advances in treatment. METHODS: Literature and institutional review. RESULTS: Spinal tumors: intramedullary, intradural/extramedullary, and extradural, comprise a wide range of histological tumors with an even wider range of clinical symptoms and prognostic features. They are relatively rare and if left untreated, can cause serious neurological deficits and disability. An accurate diagnosis is therefore crucial in determining prognosis and directing therapy. Magnetic resonance imaging (MRI) has revolutionized the diagnosis of intraspinal tumors, allowing for early detection and improved anatomical localization. Magnetic resonance has also become an integral part in staging of both primary and metastatic neoplasms of the spine for guiding therapy and is an excellent modality for follow-up. Advances in MRI (perfusion and molecular imaging) may help refine and describe these neoplasms for accurate treatment and prognosis in the future. Surveillance protocols and role of magnetic resonance are not well established. CONCLUSIONS: Magnetic resonance plays an integral role in evaluation of spinal tumors with increasing role in staging and treatment.

Full-thickness central corneal grafts in lamellar keratoscleroplasty to treat limbal dermoids.

PURPOSE: To assess the surgical outcomes in patients who underwent lamellar keratoscleroplasty with full-thickness central corneal grafts for limbal dermoids. DESIGN: Retrospective, noncomparative, analysis of a consecutive case series with chart review. PARTICIPANTS: Ten patients (6 male, 4 female; mean age, 12.3+/-5.0 years; range, 5.7-22.4 years) with 10 limbal dermoids in 10 eyes undergoing lamellar keratoscleroplasty with full-thickness central corneal grafts between 1992 and 2003 at National Taiwan University Hospital were studied. METHODS: The medical records and external eye photographs were reviewed. MAIN OUTCOME MEASURES: Best-corrected visual acuity (BCVA), corneal astigmatism and topography, cosmetic outcomes, and surgical complications were measured as outcome indicators. For analysis of the surgical effect on corneal astigmatism, the 9 patients with topographic data available were divided into 2 groups according to their preoperative corneal astigmatism (group 1, > or =6 diopters [D]; group 2, <6 D). RESULTS: The mean earliest recorded BCVA and the latest postoperative BCVA were 6/30 and 6/10, respectively, and the improvement of BCVA after operation combined with amblyopia treatment was 4.9+/-3.6 lines on a Snellen chart. Patients with preoperative astigmatism > or =6.0 D (9.7+/-1.0 D; n = 4) were found to have a marked decrease in astigmatism after surgery (astigmatism change, 5.2+/-1.7 D). Patients with preoperative astigmatism <6.0 D (3.4+/-0.2 D; n = 5) were found to have an insignificant increase in astigmatism after surgery (astigmatism change, 0.0+/-0.6 D). After surgery, significant corneal opacity was found in 1 patient; mild bluish scleral hue was found in 3 patients. Surgical complications included prolonged reepithelialization, interface neovascularization, graft rejection, and steroid glaucoma. CONCLUSIONS: Using full-thickness central corneal grafts in lamellar keratoscleroplasty for limbal dermoids achieved good cosmetic results with limited postoperative complications. Operation may markedly reduce corneal astigmatism in patients with high preoperative corneal astigmatism. Visual prognosis is good in patients undergoing surgery combined with adequate amblyopia treatment.

Long-term survival in a child with a brain stem dermoid cyst.

BACKGROUND: Brain stem dermoid cysts are very rare lesions, and in most, the outcome has been very poor. Because of the dangers of dissecting the cyst wall away from the brain stem parenchyma, some authors have advocated not to attempt a radical resection. METHODS: We describe a child in whom the brain stem dermoid cyst recurred rapidly after a conservative approach. We therefore attempted a radical removal. RESULTS: During surgery, the almost complete resection of the cyst wall was not very difficult, leading to an apparent cure after 4 years. CONCLUSION: In exceptional cases, it may be possible to remove a brain stem dermoid cyst without prohibitive morbidity and with long-term cure.

Primary amenorrhea associated with hirsutism, acanthosis nigricans, dermoid cysts of the ovaries and a new type of insulin resistance.

We describe a 15 1/2 year old presenting with primary amenorrhea, hirsutism, acanthosis nigricans and insulin resistance. Ovarian vein catheterization studies revealed bilateral excess plasma testosterone and androstenedione secretion, and at surgery multiple dermoid cysts of both ovaries were found in association with polycystic ovaries. The suggestion that the dermoid cysts may be causative in the evolution of the polycystic ovarian disease has been made. The mechanism of the insulin resistance appears to be at the post receptor level. The acanthosis nigricans diminished following surgery with normalization of the plasma androgens.

46,XX gonadal dysgenesis with epibulbar dermoid.

Pure gonadal dysgenesis with 46,XX genotype is a rare abnormality with unknown etiology. Although sensorineural deafness has been described with 46,XX gonadal dysgenesis, the majority of reported cases of 46,XX gonadal dysgenesis have no associated physical abnormalities. We report a patient with 46,XX gonadal dysgenesis associated with epibulbar dermoids and preauricular skin tags, the classic ocular and skin manifestations of Goldenhar sequence (oculoauricular vertebral dysplasia). We propose that our patient may represent a new and previously unreported syndrome.

Clinical evolution of cystic teratoma during treatment with combination chemotherapy.

Progressive, cystic tumor enlargement in the abdomen developed in a patient with teratocarcinoma during treatment with systemic chemotherapy. Tumor markers were elevated in the cyst fluid and negative in serum. Further, the patient underwent a successful surgical debulking of large amounts of cystic teratoma.

Effects of pirfenidone and dermoid cyst fluid on adhesion formation.

OBJECTIVE: To evaluate the effects of pirfenidone and human dermoid cyst fluid on adhesion formation in a rat model. DESIGN: A prospective, blinded, controlled study. SETTING: Sprague-Dawley white rats in a conventional laboratory setting. INTERVENTION(S): Rats were divided into four groups according to the type of solution tested (dermoid fluid, 0.05 mg of pirfenidone, normal saline, and a combination of dermoid fluid and pirfenidone). In the first experiment, the solution (0.5 mL) was injected intraperitoneally. In the second experiment, the solution was instilled intraperitoneally after a standardized injury to the right uterine horn. The degree of adhesion formation was evaluated 2 weeks after the initial surgery. RESULT(S): Human dermoid cyst fluid caused adhesion in 4 of 10 intact rats and in 9 of 10 standardized injured rats. Pirfenidone did not decrease adhesion formation. No adhesion was found after injection of saline or pirfenidone in intact rats. The mean +/- SEM adhesion score in standardized injured rats was higher in the dermoid group (4.6 +/- 0.6) than in the pirfenidone group (2.8 +/- 0.7) or the saline group (3.8 +/- 0.5). The mean +/- SEM adhesion score in intact rats was 0.6 +/- 0.3 after dermoid fluid injection and after dermoid instillation in standardized injured rats was 4.6 +/- 0.6. CONCLUSION(S): Human dermoid fluid causes adhesion formation even in intact rats, and the adhesion is worse in the presence of serosal injury. Intraperitoneal pirfenidone does not decrease adhesion formation.

Intraorbital and periorbital tumors in children--value of ultrasound and color Doppler imaging in the differential diagnosis.

PURPOSE: To evaluate the role of ultrasonography (US) and color Doppler imaging (CDI) in the diagnosis of orbital tumors in children. DESIGN: Retrospective nonrandomized interventional case series. METHODS: This study included 42 children with intraorbital and periorbital tumors who were evaluated in our clinic. All children underwent a complete clinical evaluation as well as orbital US and CDI. The children then underwent operation or were followed up, based on the clinical diagnosis and the findings on the imaging modalities. RESULTS: The mean age of the patients at diagnosis was 22.5 months (range 2 weeks-14 years old). Eighteen patients (12 female and 6 male) were diagnosed with hemangioma based on the findings in US and CDI; 16 patients (9 female and 7 male) were diagnosed with dermoid, 5 patients (4 female and 1 male) with lymphangioma, 2 patients with rhabdomyosarcoma, and 1 patient with a subperiostal abscess. Twenty-two patients underwent operation, and the diagnosis was confirmed on pathology. Twenty patients did not undergo operation and continued to be followed up in the clinic (mean follow-up period 38.2 months). The disease course in all patients who did not undergo operation was consistent with the working diagnosis. CONCLUSION: Both US and CDI are useful modalities in the diagnosis of intraorbital and periorbital tumors in the pediatric age group.