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Purpose: Our study aimed to evaluate the utility of the anterior segment morphometry for objectively assessing anterior segment architectural changes of corneal clouding in the mucopolysaccharidoses (MPS) cohort and to investigate whether these measurements correlate with the slit?lamp findings on the cornea and early diagnosis of glaucoma. Methods: This retrospective study involved 70 eyes of 35 children with cloudy cornea due to MPS variants. Anterior segment architectural alterations were measured using anterior segment imaging and biometry in MPS children and compared with controls. Results: Mean age of the cohort at the time of assessment was 7.9 ± 4.5 years. Males constituted two?thirds of the cohort. Variants of MPS with cloudy cornea were as follows: Type I (62%), Type IV (11%), and Type VI (22%). Morphometric measurements were available in 22 eyes of 11 MPS children and an age?matched healthy control group. There were significant differences between MPS cohort and controls in refraction in Diopters (5.03 ± 0.39 and 0.01 ± 0.04; P < 0.0001), axial length (AXL) in mm (21.39 ± 0.28 and 23.04 ± 0.28; P = 0.0002), average keratometry in Diopters (40.67 ± 0.44 and 42.83 ± 0.44; P < 0.0001), anterior chamber depth (ACD) in mm (2.92 ± 0.07 and 3.65 ± 0.07; P < 0.0001), and intraocular pressure (IOP) in mmHg (25.2 ± 2.0 and 14.1 ± 2.3; P = 0.0003). Secondary glaucoma was observed in 28% of the MPS cohort. Conclusion: The anterior segment morphometry in the cloudy cornea due to MPS provides an objective measurement of anterior segment architectural changes, thus diagnosing early?onset secondary glaucoma. These findings highlight that cloudy cornea due to MPS variants merits close monitoring throughout life
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Background: Previous studies have suggested that the clinical features of vesicoureteral reflux in infants differ from those of older children with regard to the male-to-female ratio and severity of renal parenchymal damage. Aim of the study is to know the profile and pattern of primary vesicoureteral reflux in subjects seeking care at a tertiary care teaching centre of southern India.Methods: This was a hospital based study, conducted at the Department of Paediatrics, Kasturba Medical College located at Manipal during August 2004 - August 2012 over a period of 8 years. All the children in the age group of 1 month to 18 years with Vesicoureteral Reflux, who presented to the study centre during study period, were included in the study.Results: Majority (78.5%) presented before 5 years, youngest age at presentation was 1 month and oldest at 14 year 8 months. Among 93 children studied, 65 were males and 28 were females with male to female ratio of 2.3. The commonest presenting complaint was fever (58%), followed by recurrent Urinary tract infection (UTI) (40.8%), dysuria 32 (34.4%) and reports of documented UTI was available in 23 (24.7%) cases. Four children had hypertension at presentation. Thirteen of them had associated posterior urethral valve. Neurogenic bladder was present in 3 children, 2 had associated Anorectal malformation and one had meningomyelocele.Conclusions: Majority of vesicoureteral reflux cases presented before 5 years of age. There was a preponderance of males. Majority had grade IV and V reflux. The mean age at presentation of vesicoureteral reflux was 3.6 years. The commonest presenting complaint was fever, followed by recurrent UTI.
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A 40‑year‑old male presented with an orbital extension of conjunctival squamous cell carcinoma (SCC). The orbital mass was seen protruding outward from the left palpebral fissure overhanging the lower eyelid, completely obscuring the globe and lower lid. The patient gave a history of excision biopsy, which was histopathologically diagnosed as ocular surface squamous neoplasia. He also gave a history of tumor recurrence, which gradually progressed to assume the form of the presently visible orbital mass. Computed tomography of the orbits showed the mass extending into the left orbit causing superior displacement of the globe. After a negative locoregional and systemic metastatic screening, neoadjuvant intravenous systemic chemotherapy with cisplatin and 5‑fluorouracil were initiated in an attempt to reduce the size of the tumor. Three cycles of tri‑weekly chemotherapy resulted in a significant reduction of the orbital tumor size with the globe and the lower lid being visible, thus making a lid‑sparing orbital exenteration possible. The patient subsequently underwent an orbital exenteration and at 6‑month follow‑up, the patient was free from local and regional disease. To our knowledge, this is the first reported case where systemic neoadjuvant chemotherapy has been used to reduce the size of invasive SCC with orbital extension, thereby permitting a lid‑sparing orbital exenteration.
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Schwannomas are benign, encapsulated, primary neurilemmal tumors composed of proliferating Schwann cells. Schwannomas are commonly seen in the orbit, but are rare on the epibulbar surface. Herein, we report a case of a 12‑year‑old boy who presented to us with a slow‑growing painless subconjunctival mass in the left eye. There was no intraocular extension of the mass and intra‑operatively, the mass could be clearly delineated and was excised off the underlying sclera. Histopathological examination of the mass showed typical features of schwannoma and immunohistochemistry helped to confirm the diagnosis. There was no recurrence of the lesion observed at follow‑up 26 months after surgery. Here, we describe this uncommon tumor and review the available literature. Although rare, an epibulbar schwannoma should be considered in the differential diagnosis of an amelanotic, painless subconjunctival nodular mass. Excision of the lesion is the recommended treatment.