A 46-year-old female, known case of adiposis dolorosa since adolescence, noticed painful thickening of scalp in bilateral parieto-occipital areas and vertex 1 year back. Six weeks prior to the presentation to our service, she developed severe occipital headache refractory to drug treatment. She improved after bilateral greater occipital nerve blocks. She was subjected to bilateral greater occipital chemical neurolysis which has given her complete pain relief.
A 20-year-old female presented with episodic occipital headaches and imbalance on walking of 2 years duration. Neurological examination showed hyper-reflexia of all four limbs. Computed tomographic (CT) brain scan showed a hyperdense lesion in premedullary cistern [ Figure 1a]. Magnetic resonance imaging (MRI) of brain revealed a well-defined extra-axial lesion, 13 × 25 × 14 mm in dimensions with marked hyperintensity on T1-weighted images (T1WI) and hypointensity on T2-weighted image (T2WI). The lesion did not show any restriction of diffusion on diffusion-weighted imaging (DWI) and no enhancement on giving gadolinium [ Figure 1b-g]. Gradient echo (GRE) sequence of MR imaging did not show any blooming [ Figure 1h]. Based on the imaging features, a diagnosis of a high protein-containing cystic lesion was postulated. During surgery by suboccipital approach, a thin-walled extra axial cyst containing pearly and pultaceous material with viscous fluid was excised. There was no evidence of calcification. Patient was relieved of her symptoms after the surgery and was well at follow-up of 2 years. On histopathological examination, the cyst wall consisted of lamellated keratinizing squamous epithelium suggestive of epidermoid cyst [ Figure 2a and b].Intracranial epidermoids are rare lesions accounting for 1% of all intracranial tumors. [1] Epidermoid tumors typically have long T1 and T2 relaxation times due to the presence of cholesterol in a solid crystalline state and keratin within the tumor. These are hypointense on T1-weighted, hyperintense on T2-weighted MR image and have no enhancement on post-contrast MR images. Epidermoid cysts are hypodense on CT scan. These typically restrict diffusion on DWI. In our case, the lesion was hyperdense on CT scan, hyperintense on T1-weighted image, hypointense on T2-weighted image, not enhancing by gadolinium and was not restricting diffusion on DWI.
We report a 16-year-old girl who had stunted growth and primary amenorrhea presenting with headache and vomiting. MRI of brain showed posterior fossa dermoid cyst with hydrocephalus and empty sella. Growth hormone (GH), Leutinizing hormone (LH) and Follicular stimulating hormone (FSH) were grossly reduced on endocrinological work-up. She underwent suboccipital midline craniectomy and complete excision of dermoid cyst .She had no additional deficit after the surgery. She had menarche six months after the surgery and developed adequate secondary sexual characters, however her height remains subnormal at follow-up after 2 years as compared to age matched controls. We briefly review the radiological features, pathogenesis and outcome of this underestimated entity.
KeywoRds: Hydrocephalus, Empty sella, Amenorrhea, Dermoid cyst, Brain tumors
ÖZBaşağrısı ve kusmayla gelen ve büyüme geriliği ve primer amenore gösteren 16 yaşında bir kız hastayı bildiriyoruz. Beyin MRG hidrosefali ve boş sella ile birlikte bir posterior fossa dermoid kisti gösterdi. Endokrin incelemede büyüme hormonu (GH), luteinizan hormon (LH) ve foliküler stimüle edici hormon (FSH) önemli ölçüde azalmıştı. Suboksipital orta hat kraniyektomi ve dermoid kistin tam eksizyonu yapıldı. Cerrahiden sonra herhangi bir defisit saptanmadı. Cerrahiden altı ay sonra menarş gelişti ve yeterli sekonder cinsel özellikler ortaya çıktı. Ancak hastanın boyu 2 yıllık takipten sonra yaş açısından eşleşmiş kontrollere göre halen normalin altında kaldı. Bu gereğinden az düşünülen durumun radyolojik özellikleri, patogenezi ve sonucunu kısaca gözden geçiriyoruz.
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