Search

Introduction: Persistent craniopharyngeal canal (PCC) is a rare anomaly of the base of the skull. PCC is defined as a well-corticated osseous canal, extending from the roof of the nasopharynx to the base of the sella over the course of the sphenoid corpus. We reported a PCC case with unique associations. The magnetic resonance imaging findings are discussed.Case presentation: We report a case of a 2-year-old boy with persistent craniopharyngeal canal, bilateral microphtalmia with large colobomatous cyst, and ectopic adenohypophysis with Rathke cleft cyst. He also has ectopic neurohypophysis and optic atrophy.Conclusion: The presence of orbital and optic tract malformations, craniofacial and intracranial anomalies, and tumors can accompany craniopharyngeal canals (CCs). MRI is helpful in the evaluation of PCC and associated anomalies in these patients. [ABSTRACT FROM AUTHOR]

A 3-month-old English Cocker Spaniel and a 6-month-old miniature poodle presented with clinical signs related to an abnormal right eye since birth. In both dogs, the right globe could not be identified and was replaced by a fluctuant intraorbital mass covered by a vascularized mucous membrane. Ultrasonography demonstrated that both masses were cystic structures markedly larger in size than the normal contralateral globes. In both cases, surgical excision revealed a multilobular cyst filling the whole orbital cavity. Histopathologic examination and immunostaining for glial fibrillary acid protein and S100 protein supported a diagnosis of neural cysts associated with ocular dysplasia. The definitive diagnosis was congenital cystic eye and microphthalmos with cyst for the Cocker Spaniel and miniature poodle, respectively. Karyotype was normal in both dogs. Congenital cystic eye and microphthalmos with cyst result from defects in early embryonic life that arise following formation of the optic vesicle and prior to closure of the optic fissure. To the authors’ knowledge neither has been reported in the canine species. They should be considered in the differential diagnosis of orbital cysts in dogs. [ABSTRACT FROM AUTHOR]

There is little in the literature about the clinical spectrum of orbital cysts of childhood and no comprehensive classification has been proposed. The authors propose a classification of orbital cysts of childhood and review their clinical features, pathology, and management. The major categories in the classification include cysts of surface epithelium, teratomatous cysts, neural cysts, secondary cysts, inflammatory cysts, and noncystic lesions with cystic component. Cysts of the surface epithelium are further divided into simple epithelial cyst (epidermal, conjunctival, respiratory, and apocrine gland), and dermoid cyst (epidermal and conjunctival). Epidermal dermoid cyst (dermoid) is by far the most common orbital cystic lesion in children, accounting for over 40% of all orbital lesions of childhood and for 89% of all orbital cystic lesions of childhood that come to biopsy or surgical removal. Neural cysts include those associated with ocular maldevelopment (congenital cystic eye and colobomatous cyst) and those associated with brain and meningeal tissue (cephalocele and optic nerve meningocele). The most important secondary cyst is mucocele that can occur in children with cystic fibrosis. Inflammatory cysts are generally due to parasitic infestations and are more common in tropical areas of the world. Noncystic lesions that can have a cystic component include adenoid cystic carcinoma, rhabdomyosarcoma, lymphangioma, and others. Each type of cyst has rather characteristic, but not pathognomonic, clinical features. Computed tomography and magnetic resonance imaging can help differentiate a cystic lesion from a solid tumor, suggest the type of cyst, and help in planning management. The pathology varies with the cells that line the cyst and with the inflammatory agent. Management varies from local excision to observation, depending on the location and type of cyst. Orbital cysts of childhood can be classified into categories, based mainly on their histopathology. The clinical, radiologic, and histopathologic features can be correlated with the classification in order to better evaluate a child with a cystic lesion in the orbit. [Copyright &y& Elsevier]

Colobomatous cyst of the orbit is a rare congenital cystic malformation associated with ocular maldevelopment. Usually, the cyst is associated with a microphthalmic globe. We present a rare case of a unilateral large colobomatous cyst associated with a normalsized globe, giving the appearance of a double globe on imaging. [ABSTRACT FROM AUTHOR]