Pub Date : 2020-07-01DOI: 10.1097/IOP.0000000000001503
V. Vasanthapuram, Ravi Varma, Nandini Bothra
Ophthalmic Plast Reconstr Surg, Vol. 36, No. 4, 2020 e109 A 3-year-old boy was brought by his parents with complaints of red eyes (Fig. A) since the age of 3 months. With time, progressive increase in the redness of the OS along with increase in the swelling on the left side of scalp was noted. There was no history of seizures. On examination, the vision was 6/6 in OU and extraocular motility was normal. There were no papillae, trantas spots, or limbal thickening; however, dilated conjunctival vessels were present in the temporal quadrant of the OS (Fig. B). The fundus examination was unremarkable, with no evidence of vascular malformation. Systemic examination did not show any vascular lesion. An MRI with contrast of brain showed well-defined heterogeneously enhancing (T2 weighted) hyperintense lesion in left superior ophthalmic fissure and posterior orbit (arrow in Fig. C, D). Similar lesion was present in the left temporal fossa, in the subcutaneous plane in the temporal area of scalp (* in Fig. C). The diploic space of the lesser wing of sphenoid bone and adjacent frontal bone showed asymmetric enhancement (double arrows in Fig. C, D). Prominence of left sphenoparietal sinus and pterygoid venous plexus, multiple venous anomalies in bilateral thalami, and bilateral cerebellar hemispheres were also noted (arrows in Fig. E). Based on the above findings, a diagnosis of cerebrofacial arteriovenous metameric syndrome Type 2 was made. Cerebrofacial arteriovenous metameric syndrome is a rare, nonhereditary condition with vascular malformations in a metameric distribution involving the craniofacial region (brain and face) with subtypes based on the distribution of lesions. Type 2 or “lateral prosencephalic group” involves the occipital lobe, thalamus, optic tract, and retina intracranially and the maxilla as its facial component. An embryonic developmental defect has been proposed to cause the condition. The most common presenting symptom is decreased vision; however, the patient in this study presented with just congestion of the eye and minimal scalp swelling. Other symptoms like mental retardation due to underdevelopment of the brain, seizures due to bleeding, and so on may be seen. The child is under periodic observation to note for progress in the lesion or the symptomatology, if any.
{"title":"Cerebrofacial Arteriovenous Metameric Syndrome Type 2.","authors":"V. Vasanthapuram, Ravi Varma, Nandini Bothra","doi":"10.1097/IOP.0000000000001503","DOIUrl":"https://doi.org/10.1097/IOP.0000000000001503","url":null,"abstract":"Ophthalmic Plast Reconstr Surg, Vol. 36, No. 4, 2020 e109 A 3-year-old boy was brought by his parents with complaints of red eyes (Fig. A) since the age of 3 months. With time, progressive increase in the redness of the OS along with increase in the swelling on the left side of scalp was noted. There was no history of seizures. On examination, the vision was 6/6 in OU and extraocular motility was normal. There were no papillae, trantas spots, or limbal thickening; however, dilated conjunctival vessels were present in the temporal quadrant of the OS (Fig. B). The fundus examination was unremarkable, with no evidence of vascular malformation. Systemic examination did not show any vascular lesion. An MRI with contrast of brain showed well-defined heterogeneously enhancing (T2 weighted) hyperintense lesion in left superior ophthalmic fissure and posterior orbit (arrow in Fig. C, D). Similar lesion was present in the left temporal fossa, in the subcutaneous plane in the temporal area of scalp (* in Fig. C). The diploic space of the lesser wing of sphenoid bone and adjacent frontal bone showed asymmetric enhancement (double arrows in Fig. C, D). Prominence of left sphenoparietal sinus and pterygoid venous plexus, multiple venous anomalies in bilateral thalami, and bilateral cerebellar hemispheres were also noted (arrows in Fig. E). Based on the above findings, a diagnosis of cerebrofacial arteriovenous metameric syndrome Type 2 was made. Cerebrofacial arteriovenous metameric syndrome is a rare, nonhereditary condition with vascular malformations in a metameric distribution involving the craniofacial region (brain and face) with subtypes based on the distribution of lesions. Type 2 or “lateral prosencephalic group” involves the occipital lobe, thalamus, optic tract, and retina intracranially and the maxilla as its facial component. An embryonic developmental defect has been proposed to cause the condition. The most common presenting symptom is decreased vision; however, the patient in this study presented with just congestion of the eye and minimal scalp swelling. Other symptoms like mental retardation due to underdevelopment of the brain, seizures due to bleeding, and so on may be seen. The child is under periodic observation to note for progress in the lesion or the symptomatology, if any.","PeriodicalId":19621,"journal":{"name":"Ophthalmic Plastic & Reconstructive Surgery","volume":"4 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2020-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"89865864","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2020-05-01DOI: 10.1097/iop.0000000000001439
N. Homer, A. Huggins, V. Durairaj
Ophthalmic Plast Reconstr Surg, Vol. 36, No. 3, 2020 e81 A 62-year-old woman presented with an incidentally discovered osteolytic mass within the left greater wing of the sphenoid (Fig. B). The patient had intact visual acuity and full extraocular motility. The 9 × 13 × 7 mm mass extended into the extraconal inferolateral orbit and displaced the lateral rectus muscle, demonstrated on CT axial bone windows (Fig. A). A review of the patient’s CT imaging from 7 years prior showed interval expansion of previously unnoticed bony disruption (Fig. C). A lateral orbitotomy was performed and incisional biopsy revealed meningioma, WHO grade I (Fig. D). Further tumor resection was pursued via a craniotomy in conjunction with neurosurgery. Fewer than 2% of all meningiomas are intraosseous, the majority of which display osteoblastic activity. Historical doctrine has suggested that meningioma-induced bony degradation infers malignant pathology. This case demonstrates rare osteolytic behavior of a histologically benign intraosseous meningioma.
{"title":"Benign Meningioma With Rare Osteolytic Behavior.","authors":"N. Homer, A. Huggins, V. Durairaj","doi":"10.1097/iop.0000000000001439","DOIUrl":"https://doi.org/10.1097/iop.0000000000001439","url":null,"abstract":"Ophthalmic Plast Reconstr Surg, Vol. 36, No. 3, 2020 e81 A 62-year-old woman presented with an incidentally discovered osteolytic mass within the left greater wing of the sphenoid (Fig. B). The patient had intact visual acuity and full extraocular motility. The 9 × 13 × 7 mm mass extended into the extraconal inferolateral orbit and displaced the lateral rectus muscle, demonstrated on CT axial bone windows (Fig. A). A review of the patient’s CT imaging from 7 years prior showed interval expansion of previously unnoticed bony disruption (Fig. C). A lateral orbitotomy was performed and incisional biopsy revealed meningioma, WHO grade I (Fig. D). Further tumor resection was pursued via a craniotomy in conjunction with neurosurgery. Fewer than 2% of all meningiomas are intraosseous, the majority of which display osteoblastic activity. Historical doctrine has suggested that meningioma-induced bony degradation infers malignant pathology. This case demonstrates rare osteolytic behavior of a histologically benign intraosseous meningioma.","PeriodicalId":19621,"journal":{"name":"Ophthalmic Plastic & Reconstructive Surgery","volume":"96 2 Pt 1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2020-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"89513356","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2020-03-01DOI: 10.1097/iop.0000000000001430
Gherusa H M Moré, J. Vieira, P. Akaishi, A. Cruz
e58 Ophthalmic Plast Reconstr Surg, Vol. 36, No. 2, 2020 A prenatal ultrasonographic examination at 35 weeks of gestational age disclosed a right orbital abnormality in a male fetus, which was considered as a sign of neurofibromatosis. At 38 gestational age, a T2W MRI disclosed a large cystic-like lesion with small solid component expanding the right orbital cavity (Fig. 1A). Possible diagnoses included congenital cyst eye, venous-lymphatic malformation, and congenital teratoma. On the first day of life, a second T1W MRI disclosed the same cystic-like lesion with a small solid component (Fig. 1B). Four days later, the child was referred to the hospital for management of the massive right proptosis. A third T1W post contrast MRI showed a heterogeneous mass occupying the whole orbit cavity. (Fig. 1C) An eyelid and conjunctival sparing orbital exenteration was performed (Fig. 2A) The specimen revealed that the normal orbital contents were completely involved by a large tumor (Fig. 2B). The histopathological examination showed that the mass was formed by well-differentiated tissues from 3 germline components such as respiratory epithelium, intestinal mucosa, smooth muscle, bone, cartilage, skin, and glial tissue. Mature teratomas are the most common type of childhood germ cells tumors. They can occur at a variety of locations. Congenital orbital teratomas are relatively uncommon and their antenatal images are quite rare. To the best of the authors’ knowledge, the present case represents a unique radiologic documentation of the rapid transformation of a fetal cystic-like lesion into a heterogenous mass of mature teratoma.
{"title":"Orbital Teratoma: MRI Changes From Fetal Life to Exenteration.","authors":"Gherusa H M Moré, J. Vieira, P. Akaishi, A. Cruz","doi":"10.1097/iop.0000000000001430","DOIUrl":"https://doi.org/10.1097/iop.0000000000001430","url":null,"abstract":"e58 Ophthalmic Plast Reconstr Surg, Vol. 36, No. 2, 2020 A prenatal ultrasonographic examination at 35 weeks of gestational age disclosed a right orbital abnormality in a male fetus, which was considered as a sign of neurofibromatosis. At 38 gestational age, a T2W MRI disclosed a large cystic-like lesion with small solid component expanding the right orbital cavity (Fig. 1A). Possible diagnoses included congenital cyst eye, venous-lymphatic malformation, and congenital teratoma. On the first day of life, a second T1W MRI disclosed the same cystic-like lesion with a small solid component (Fig. 1B). Four days later, the child was referred to the hospital for management of the massive right proptosis. A third T1W post contrast MRI showed a heterogeneous mass occupying the whole orbit cavity. (Fig. 1C) An eyelid and conjunctival sparing orbital exenteration was performed (Fig. 2A) The specimen revealed that the normal orbital contents were completely involved by a large tumor (Fig. 2B). The histopathological examination showed that the mass was formed by well-differentiated tissues from 3 germline components such as respiratory epithelium, intestinal mucosa, smooth muscle, bone, cartilage, skin, and glial tissue. Mature teratomas are the most common type of childhood germ cells tumors. They can occur at a variety of locations. Congenital orbital teratomas are relatively uncommon and their antenatal images are quite rare. To the best of the authors’ knowledge, the present case represents a unique radiologic documentation of the rapid transformation of a fetal cystic-like lesion into a heterogenous mass of mature teratoma.","PeriodicalId":19621,"journal":{"name":"Ophthalmic Plastic & Reconstructive Surgery","volume":"10 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2020-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"80931422","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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