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.

[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.

Congenital hairy polyp and autoamputation in an infant with acute otitis media.

Hairy polyp is a rare, benign tumor that frequently localizes in the nasopharynx and oropharynx. The embryogenesis of hairy polyp is not known precisely. The clinical profile can vary depending on the size and location of the tumor. In this report, we present a case with hairy polyp who was admitted with acute otitis media and completely recovered after spontaneous autoamputation.

Intraoperative neurophysiological monitoring in patients undergoing tethered cord surgery after fetal myelomeningocele repair.

OBJECT: Fetal myelomeningocele closure has been shown to be advantageous in a number of areas. In this study, the authors report on neural function in patients who had previously undergone fetal myelomeningocele repair and returned to the authors' institution for further surgery that included intraoperative neurophysiological monitoring. METHODS: The authors retrospectively reviewed data obtained in 6 cases involving patients who underwent fetal myelomeningocele repair and later returned to their institution for spinal cord untethering. (In 4 of the 6 cases, the patients also underwent removal of a dermoid cyst [3 cases] or removal of an epidermoid cyst [1 case] during the untethering procedure.) Records and imaging studies were reviewed to identify the anatomical level of the myelomeningocele as well as the functional status of each patient. Stimulated electromyography (EMG) and transcranial motor evoked potential (tcMEP) recordings obtained during surgery were reviewed to assess the functional integrity of the nerve roots and spinal cord. RESULTS: During reexploration, all patients had reproducible signals at or below their anatomical level on stimulated EMG and tcMEP recordings. Corresponding to these findings, prior to tethering, all patients had antigravity muscle function below their anatomical level. CONCLUSIONS: All 6 patients had lower-extremity function and neurophysiological monitoring recording signals at or below their anatomical level. These cases provide direct evidence of spinal cord and nerve root conductivity and functionality below the anatomical level of the myelomeningocele, further supporting that neurological status improves with fetal repair.

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.

Large congenital corneal dermoid with spontaneous partial regression: the first report.

PURPOSE: To report a spontaneous partial regression of a large congenital corneal dermoid in a newborn. MATERIALS AND METHODS: Review and follow-up of the medical records of a female newborn presenting with a tumor mass of her right eye and no other congenital abnormalities. The child presented on the first day of life with a mushroom-shaped mass of 15 × 15 mm in diameter that involved the most of the cornea leaving a small clear portion superiorly. The mass protruded through the palpebral aperture. A diagnosis of corneal dermoid was made based on clinical presentation of the mass and radiographic and ultrasound findings. Enucleation of the right eye was discussed, but declined by parents. RESULTS: During the first month of life, a spontaneous partial regression of the mass occurred, so that the patient was able to close the lids completely above the mass. This dermoid is the third type according to Mann's classification. Characterized by involvement of the entire anterior segment and lack of lens. CONCLUSIONS: To the best of our knowledge, the case reported is the first documented spontaneous partial regression of a large corneal dermoid. Surgical excision at an early age may be recommended to avoid development of amblyopia and dramatic growth of the tumor. In our case the tumor regressed and surgical excision would have no impact on visual outcome. Future management of type III corneal dermoid needs to consider whether to perform surgery at an early stage or to recommend conservative management.

Symptomatic syringomyelia associated with a dermoid tumor in the posterior fossa.

A 28-year-old female presented with a dermoid tumor in the posterior fossa associated with symptomatic syringomyelia. Magnetic resonance (MR) imaging revealed cervical syringomyelia and tonsillar herniation concomitant with an intradural posterior fossa tumor which was totally removed. The histological diagnosis was dermoid tumor. Her symptoms subsided and MR imaging demonstrated complete resolution of the syrinx 12 months after tumor removal. Benign congenital intracranial tumor may cause tonsillar herniation resulting in symptomatic cervical syringomyelia. However, both tonsillar herniation and cervical syrinx may clinically and radiologically resolve following removal of the intracranial lesion and posterior fossa decompression.

Solid corneal dermoids and subconjunctival lipodermoids: impact of differentiated surgical therapy on the functional long-term outcome.

PURPOSE: The functional long-term outcome after differentiated surgical therapy of solid corneal dermoids and subconjunctival lipodermoids with special regard to determinants for amblyopia will be assessed. METHODS: Forty-six consecutive patients undergoing surgery for solid epibulbar dermoids, subconjunctival lipodermoids, or both were included. Visual acuity, refraction, keratometry, and degree of amblyopia were determined. Surgical therapy for corneal dermoids consisted of lamellar sclerokeratectomy, lamellar keratoplasty, corneoscleroplasty, and lamellar removal with autologous episcleral transplant. Surgical therapy for lipodermoids consisted of excision and reduction of the volume of the tumor. Follow-up examination was performed on average 4.5 years after surgical intervention. RESULTS: A significant correlation between tumor volume and preoperative visual acuity could be observed in patients with solid corneal dermoids not occluding the optical axis. Visual acuity improved significantly from 0.21 +/- 0.4 to 0.35 +/- 0.4 after surgery of corneal dermoids. Most eyes had concomitant hyperopia. Postoperative visual acuity correlated positively with preoperative visual acuity (P = 0.0001). After tumor excision, hyperopia and astigmatism were not reduced significantly on average. Nineteen of 47 patients suffered from amblyopia. Amblyopia was more often observed in patients with preoperative hyperopia > or =2 diopters and astigmatism >2 diopters. Visual acuity, refraction, and astigmatism were not changed significantly by surgery in patients with subconjunctival lipodermoids. CONCLUSIONS: Epibulbar dermoids require differentiated surgical therapy. Amblyopia is a major threat of solid corneal dermoids. The incidence of amblyopia seems to depend on preoperative occlusion of the optical axis and preoperative degree of hyperopia and astigmatism.

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.

Ruptured intracranial dermoid cysts: clinical, radiographic, and surgical features.

OBJECTIVE: Intracranial dermoid cysts are pathologically characterized by a thick, stratified squamous epithelium cyst wall containing dermal elements. Rupture into the subarachnoid spaces and ventricles is extremely rare. We review the clinical, radiographic, and surgical features of eight ruptured dermoid cysts. METHODS: We retrospectively evaluated five surgically treated patients with pathologically proven ruptured dermoid cysts. Clinic notes, operative reports, and neuroimaging, including initial computed tomographic and magnetic resonance imaging scans, were reviewed. Imaging was also available on three outside patients reviewed by members of our radiology department. RESULTS: The most common presentations were headaches (57%) and seizures (42%), followed by hydrocephalus (29%) from intraventricular rupture. These lesions were consistently hypodense on computed tomographic scans and hyperintense on T1-weighted images with minimal to no enhancement after gadolinium administration. Disseminated fat droplets were present in the subarachnoid space in both cerebral hemispheres in all patients, and five patients had intraventricular rupture with fat-fluid levels in the ventricles. Gross to near-total resection of the primary lesion was achieved in all five surgically treated patients treated at our institution. Four patients had remnant tumor capsules adherent to neurovascular structures that were unresectable. Repeat resection was performed for one recurrence; there were no further recurrences during a follow-up period of 2 to 134 months (mean, 65.6 mo). Two patients with preoperative hydrocephalus eventually required ventriculoperitoneal shunting. CONCLUSION: Ruptured intracranial dermoid cysts represent 0.18% of all central nervous system tumors surgically treated in our institution during a 12-year period. The presence of disseminated fat droplets in the subarachnoid space or ventricles on neuroimaging is diagnostic for a ruptured dermoid cyst. Gross total removal is achievable; however, residual tumor capsules adherent to neurovascular structures should be left behind to minimize complications.

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.