Purpose To study the relationship between the location of orbital and palpebral(eyelid)hemangiomas and ocular problems in children and the interventions needed to prevent or reduce the severity of visual impairment. Methods This study included 12 cases (10 female and 1 male;age range:1 day to 5 months old at first evaluation;mean age 2,5 months)of eyelid and/or orbit hemangiomas gathered over a 6 year period (2005-2011).Ultrasonography and magnetic resonance imaging(MRI)identified these lesions to be:palpebral in 2 cases, palpebral and orbital involvement in 9 cases.Pediatric and ophthalmological evaluation(orthoptic examination,visual acuity determination with Teller Acuity Cards,refractometry and fundus opthalmoscopy)were performed in all cases. Results The mean follow-up was 36 months (range 11-63 months) Treatments for infants and children with hemangiomas obstructing the visual axis included oral steroid administration(9 cases);in 1 case a surgical treatment was associated.4 cases developed amblyopia and 1 case strabismus.Treatment of early amblyopia through selective patching part-time patch occlusion of the uninvolved eye and early prescription of astigmatism correction was performed.10 children achieved normal vision at last evaluation. Conclusion In our study 10 patients treated for visual impairment and available for long-term follow-up showed an excellent functional outcome with no residual amblyopia.In most cases,early intervention to address hemangioma-related visual disturbances can lead to a good functional outcome. Intervention typically involves medical debulking of the hemangioma(using oral steroids)combined with the treatment of early amblyopia through selective patching and/or optical correction with refractive lenses.

Purpose Shaken Baby Syndrome(SBS) presents with acute encephalopathy,subdural and retinal haemorrhages,with inconsistent history,in < 2 years old.Long-term sequelae are still under study.Aim is to present the long-term outcome in a case series of SBS followed-up by a multidisciplinary team. Methods Case series of 14 children(9 M,5 F)with confirmed SBS has been examined and followed-up.Patients underwent fundus evaluation (indirect ophtalmoscopy) and wide-field digital ophthalmic camera (RetCam II) until bleeding reabsorption.Assessment was repeated at follow-up combined with ocular motility evaluation, visual field(BEFIE test),visual acuity(Teller acuity cards),refractometry, cognitive-behavioral evaluations (Griffiths scales,Child Behaviour Check List), and family stress measurement(Parenting Stress Index),segmentation analysis of MRI. Results Mean age at acute episode of SBS: 6.5 months (range 2-20), 9/14 in the first 6 months of life. Mean age at last follow-up evaluation: 32 months (range 8-65). 4 cases were lost to follow-up. At last follow-up evaluation: 4 out of 10 had a decreased visual acuity (cortical visual impairment), 4 out of 10 had visual field deficits, 3 out of 10 had strabismus. None of the cases showed significant refractive errors. Cognitive/behavioural assessment demostrated global delay and impairments in neuromotor ability, speech/language development and attention problems. MRI analysis showed atrophic alterations in several brain areas. Conclusion Multidisciplinary assessment including ophthalmologic examination and neuro behavioural evaluation provides crucial prognostic information in cases of suspected SBS.Functional outcome is largely dependent on brain injury and atrophy.

Purpose To evaluate the ocular findings and the visual outcome in a group of patients with mucopolysaccaridoses(MPS) followed and treated with new therapeutic options. Methods 18 pazients with MPS (9 males and 9 females) were examinated: 3 cases with MPS type I, 4 with MPS type II,7 with MPS type III, 1 with MPS type IV and 3 with MPS type VI. The patients underwent an ophthalmological assessment with : visual acuity (with Teller Acuity Cards, Lea Symbols o letter chart, depending on patient age or degree of cohoperation), slit-lamp examination, fundus ophthalmoscopy, intraocular pressure, refractometry, electroretinography and eye echography. Results The mean follow-up was 48 months (ranged from 3 to 190 months).The mean age at first ophtalmologic evaluation was 6,9 years ( ranged from 1 to 20 years). 8 case presented progressive corneal clouding, 5 case retinal pigmentary degeneration, and 1 papilledema. Visual acuity worsened severely in 3 cases with MPS type I: 1 case from 10/10 to 5/10, 1 case from 5/10 to 1/10; in another case visual acuity decreased to 1/20 and underwent to corneal transplantation with good visual outcome (RE 5/10 and LE 7/10). The other cases showed slightly reduction of visual function. 3 patients had ocular hypertension and were treated with hypotensive eye drops. 8 patients underwent enzyme replacement therapy. Conclusion The mucopolysaccaridoses(MPS) are rare systemic disorders caused by accumulation of glycosaminoglycans. Ophthalmological manifestations are frequent in MPS, particularly in MPS I, II and VI, characterized by corneal clouding, retinal distrophy and blindness.Regular ophthalmic monitoring to determine disease progression and management of complications are necessary as a part of multidisciplinary approach.

Purpose To study long-term outcome of SBS on visual and cognitive functions. Methods Case series of 10 children (7 M, 3 F) with confirmed SBS has been examined and followed-up. All chldren underwent a fundus evaluation by indirect ophtalmoscopy and wide-field digital ophtalmic camera (RetCam II) in the acute phase and until retinal hemorrhage resorption. The assessment was repeated at follow-up combined with ocular motility evaluation, visual field (BEFIE test), visual acuity by preferential looking tecnique (teller acuity cards), refractometry, cognitive-behavioural evaluations (Griffiths scales, Child Behaviour Check List), and family stress measurement (Parenting Stress Index). Results Mean age at acute episode of SBS: 6 months (range 2-20). Mean age at last follow-up evaluation: 27.6 months (range 4.5-41). At last follow-up evaluation: 1 out of 6 had a decreased visual acuity (cortical visual impairment), 3 out of 6 had mild-severe visual field deficits, 3 out of 6 has strabismus. None of the cases showed significant refractive errors. Due to age or severity of impairment and delay in global development, in any of the cases we were able to perform a recognition acuity test by symbols or E-charts. Cognitive and behavioural assessment demonstrated global delay and impaiments in speech/language development and attention problems. Conclusion SBS is characterized by severe long-term sequelae both in visual and cognitive function. Several visual impairments are observed, mainly related to cortial injury of visuo-spatial area. Although follow-up is difficult due to family history, there is strong indication for global assessment until scholar age where other impairments are demonstrated.

Purpose: Describe the long term follow-up of children affected by congenital achromatopsia (CA). CA is a rare recessive inherited vision disorder (1:50000) and includes two clinical categories: complete CA and incomplete CA. Methods: Fourteen children (9 M, 5 F) were followed-up and a rehabilitation individual program was carried out. The assessment included: far and near visual acuity (Teller, Lea Symbols, Snellen Charts),refraction, color vision (Farnsworth, Ishihara, Montessori), contrast sensitivity, visual field, slit lamp examination, fundus ophthalmoscopy, electrophysiologic tests (photopic, scotopic and flicker ERG and VEPs) and genetic examination. Results: Early onset of nystagmus, low vision and photophobia are first clinical manifestations of CA. The differential diagnosis includes Leber Amaurosis, and congenital nystagmus and albinism. Visual acuity in our patients is around 1/10 and it is worse for near. Refractive errors showed a distribution toward hyperopia (50%) and emmetropia (35%). Macular hypoplasia is present in all cases and electrophysiology showed a reduced or absent photopic ERG, while scotopic system appears normal. Conclusions: Due to abnormal or totally absent cone function, which lead to macular hypoplasia, CA shows reduced visual acuity, absent/severely impaired color vision, nystagmus and photophobia. Low vision aids such as telescopic systems and magnification devices along with OCR/ICR software were prescribed after a carefully evaluation. Spectral filters were early prescribed to reduce photophobia and improve vision. The molecular analysis may confirm the diagnosis and give genetic couseling to the family. An early detection of CA is underlined by the importance to carry out a complete and precocious rehabilitative approach.

Among the numerous problems that spina bifida (SB) patients are faced with, impairments to the visual apparatus are often considered late and are not covered extensively in the literature. At the Pediatric Department of the University of Padua, an assessment of the visual function of 59 SB patients between 5 months and 26 years of age (29 male and 30 female) has been carried out by means of an ophthalmologic protocol. As far as the alteration of the exstrinsic ocular mobility is concerned, 44% (26/59) of our patients revealed a manifest squint and only 3% (two patients) suffered from a latent squint. The most frequent type is a convergent squint (80%). The assessment of visual acuity made through Optotype was good in 82% of the cases (unlike what is commonly reported in the literature) and mild in 18%. None of the patients manifested hypovision. Refraction defects were present in 59% (34/59) of the patients. Regular ophthalmologic evaluations from birth or from diagnosis allow ophthalmologic treatments that are tailored to children suffering from SB and also enable them to reach and maintain a good visual standard and to observe the subtle symptoms of endocranial hypertension sooner. An early discovery and correct treatment of visual problems improves cognitive and motive performance as well as the autonomy of SB patients.

Objective. To determine whether demographic, clinical, and laboratory variables at onset of arthritis can predict the development and the severity of anterior uveitis (AU) in oligoarticular juvenile idiopathic arthritis (JIA).Methods. In a retrospective study, a cohort of 366 patients with oligoarticular onset JIA from 3 pediatric rheumatology centers were evaluated. Patients were classified in 3 groups: severe uveitis (SU) with a mean greater than or equal to 2 uveitis relapses/year with complications or need for immunosuppressive therapy; mild uveitis (MU) with a mean less than or equal to 1 uveitis relapse/year with no complications; and no uveitis. Variables that were significant with univariate tests or were clinically relevant for each outcome underwent multivariate logistic regression analysis.Results. There were 316 patients available for analyses: 66 in the SU group, 64 in the MU group, and 186 in the no uveitis group. Multivariate analysis showed the following factors to be significant as predictors of AU onset: low age at onset (OR 0.96), alpha(2)-globulin plasma concentration (OR 1.34) and HLA-A19 (OR 2.87), B22 (OR 4.51) and DR9 (OR 2.33), while HLA-DR1 conferred protection (OR 0.13). This model was not good in predicting which patient would develop uveitis (sensitivity 55%, specificity 26%). Time interval between onset of arthritis and the first AU and elevated alpha(2)-globulin level in the serum were the best predictors of AU severity (OR 1.62 and 0.85, respectively). When applied prospectively, the model revealed good sensitivity (89.2%), specificity (76.1%), and efficiency (86.3%).Conclusion. Clinical and laboratory variables measurable at onset of arthritis can be used to predict severity of the course of AU in oligoarticular JIA, but not its onset. More accurate prediction can shorten or lengthen the intervals between ophthalmologic evaluations and can change the therapeutic approach undertaken on the basis of expected disease severity.

60 patients with myelomeningocele, aged between 24 months and 23 years, have been studied neuro-ophthalmologically. All subjects have been examined trough neurological, neuro-radiological and ophthalmological researches to determin the incidence of oculomotor disorders, the correlation with the typical cerebral anomalies of this patients and the possible influence on the visual-motor and on the intellectual performances. The squint, present in 66,7% of the patients, is above all concomitant and convergent; the nystagmus, observed in 48,3% of the subjects, is mainly horizontal. These disorders are strongly connected with: 1) the presence of hydrocephalus, with or without shunt; 2) the possible complications of the Ventricle-Peritoneal shunt; 3) the caudal dislocation of the cerebellum and of the medulla (Arnold-Chiari 2), The persistence of squint and nystagmus despite the treatment is strictly connected with the cerebral deformities present in these patients (in particular, Chiari 2 and midline anomalies). The absence of visual-motor and intellectual impairment may be due to the early diagnosis and treatment of the refractive errors and/or the squint.

Also in childhood, the orbital cavity is a possible place for different kinds of lesions. The recent contribution of magnetic risonance imaging (MRI) enables a more accurate differential diagnosis of intraorbital pathology. The clinical and neuroradiological data of two new patients affected respectively by intraorbital meningioma and schwannoma are presented. Both patients showed exophthalmos without pain and were treated with surgery. The different etiologies of intraorbital lesions, i.e. vascular, neoplastic, inflammatory, cystic and malformative are analyzed. Sometimes the case history and neuroradiological assessment alone enable a correct diagnosis. More often, however, only surgery reveals the exact nature of the disease. The age of the patient too can represent a criteria for a possible differential diagnosis.

A three-phase Program for blindness and low vision in children and adolescents (O-18 aged) has been supported by the local Government of the Veneto region from September 1995. A visual assessment and a focused questionnaire were proposed. The assessed prevalence of subjects affected by severe visual impairment (level of vision less than 1/10 or blindness) and aged under 18 is equal to 0.35/1000. The 62% of the cases considered shows other associated debilitant pathologies: the 24% of them is handicap, the 12% are chronic diseases, both handicap and chronical diseases in the 64%. Other social findings included information on family behavioural change depending on the visual impairment (50% of the families). The authors suggest that a complete multidisciplinary assessment and support of visually impaired children and their families is required everytime.

Medical and Pediatric OncologyVolume 26, Issue 2 p. 116-124 Multiple central nervous system haemangioblastomas Giulio J. D'Angio MD, Corresponding Author Giulio J. D'Angio MD Paediatric Neuro-Oncology Program, Divisions of Child Neurology, Oncology, Pathology, Ophthalmology, Neuroradiology, and Neurosurgery, Department of Pediatrics, University of Padua, Padua, ItalyDepartment of Radiation Oncology, HUP, 34th & Spruce Sts., Philadelphia, PA 19104Search for more papers by this authorAudrey E. Evans MD, Audrey E. Evans MD Paediatric Neuro-Oncology Program, Divisions of Child Neurology, Oncology, Pathology, Ophthalmology, Neuroradiology, and Neurosurgery, Department of Pediatrics, University of Padua, Padua, ItalySearch for more papers by this authorLuana Padovan MD, Luana Padovan MD Paediatric Neuro-Oncology Program, Divisions of Child Neurology, Oncology, Pathology, Ophthalmology, Neuroradiology, and Neurosurgery, Department of Pediatrics, University of Padua, Padua, ItalySearch for more papers by this authorGiorgio Perilongo MD, Giorgio Perilongo MD Paediatric Neuro-Oncology Program, Divisions of Child Neurology, Oncology, Pathology, Ophthalmology, Neuroradiology, and Neurosurgery, Department of Pediatrics, University of Padua, Padua, ItalySearch for more papers by this authorAntonio Battistella MD, Antonio Battistella MD Paediatric Neuro-Oncology Program, Divisions of Child Neurology, Oncology, Pathology, Ophthalmology, Neuroradiology, and Neurosurgery, Department of Pediatrics, University of Padua, Padua, ItalySearch for more papers by this authorMarina Gardiman MD, Marina Gardiman MD Paediatric Neuro-Oncology Program, Divisions of Child Neurology, Oncology, Pathology, Ophthalmology, Neuroradiology, and Neurosurgery, Department of Pediatrics, University of Padua, Padua, ItalySearch for more papers by this authorLuisa Pinello MD, Luisa Pinello MD Paediatric Neuro-Oncology Program, Divisions of Child Neurology, Oncology, Pathology, Ophthalmology, Neuroradiology, and Neurosurgery, Department of Pediatrics, University of Padua, Padua, ItalySearch for more papers by this authorCarla Carollo MD, Carla Carollo MD Paediatric Neuro-Oncology Program, Divisions of Child Neurology, Oncology, Pathology, Ophthalmology, Neuroradiology, and Neurosurgery, Department of Pediatrics, University of Padua, Padua, ItalySearch for more papers by this authorMario Pellone MD, Mario Pellone MD Paediatric Neuro-Oncology Program, Divisions of Child Neurology, Oncology, Pathology, Ophthalmology, Neuroradiology, and Neurosurgery, Department of Pediatrics, University of Padua, Padua, ItalySearch for more papers by this author Giulio J. D'Angio MD, Corresponding Author Giulio J. D'Angio MD Paediatric Neuro-Oncology Program, Divisions of Child Neurology, Oncology, Pathology, Ophthalmology, Neuroradiology, and Neurosurgery, Department of Pediatrics, University of Padua, Padua, ItalyDepartment of Radiation Oncology, HUP, 34th & Spruce Sts., Philadelphia, PA 19104Search for more papers by this authorAudrey E. Evans MD, Audrey E. Evans MD Paediatric Neuro-Oncology Program, Divisions of Child Neurology, Oncology, Pathology, Ophthalmology, Neuroradiology, and Neurosurgery, Department of Pediatrics, University of Padua, Padua, ItalySearch for more papers by this authorLuana Padovan MD, Luana Padovan MD Paediatric Neuro-Oncology Program, Divisions of Child Neurology, Oncology, Pathology, Ophthalmology, Neuroradiology, and Neurosurgery, Department of Pediatrics, University of Padua, Padua, ItalySearch for more papers by this authorGiorgio Perilongo MD, Giorgio Perilongo MD Paediatric Neuro-Oncology Program, Divisions of Child Neurology, Oncology, Pathology, Ophthalmology, Neuroradiology, and Neurosurgery, Department of Pediatrics, University of Padua, Padua, ItalySearch for more papers by this authorAntonio Battistella MD, Antonio Battistella MD Paediatric Neuro-Oncology Program, Divisions of Child Neurology, Oncology, Pathology, Ophthalmology, Neuroradiology, and Neurosurgery, Department of Pediatrics, University of Padua, Padua, ItalySearch for more papers by this authorMarina Gardiman MD, Marina Gardiman MD Paediatric Neuro-Oncology Program, Divisions of Child Neurology, Oncology, Pathology, Ophthalmology, Neuroradiology, and Neurosurgery, Department of Pediatrics, University of Padua, Padua, ItalySearch for more papers by this authorLuisa Pinello MD, Luisa Pinello MD Paediatric Neuro-Oncology Program, Divisions of Child Neurology, Oncology, Pathology, Ophthalmology, Neuroradiology, and Neurosurgery, Department of Pediatrics, University of Padua, Padua, ItalySearch for more papers by this authorCarla Carollo MD, Carla Carollo MD Paediatric Neuro-Oncology Program, Divisions of Child Neurology, Oncology, Pathology, Ophthalmology, Neuroradiology, and Neurosurgery, Department of Pediatrics, University of Padua, Padua, ItalySearch for more papers by this authorMario Pellone MD, Mario Pellone MD Paediatric Neuro-Oncology Program, Divisions of Child Neurology, Oncology, Pathology, Ophthalmology, Neuroradiology, and Neurosurgery, Department of Pediatrics, University of Padua, Padua, ItalySearch for more papers by this author First published: February 1996 https://doi.org/10.1002/(SICI)1096-911X(199602)26:2<116::AID-MPO9>3.0.CO;2-OCitations: 4AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. 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The authors report their experience in the treatment of 21 children affected by retinoblastoma. There were 14 bilateral and seven unilateral cases of retinoblastoma; mean age at diagnosis was 16 months in males (16 patients) and 30 months in females (five patients). They were all staged according to Howarth et al. (1980) and treatment was correlated to the various stages. The treatment protocol was determined by a group of co-workers at the University of Padova. Among the 35 affected eyes, 26 were enucleated and the others were treated without surgery. One patient died of cerebral metastases and two patients are affected by a second tumor with an unfavorable prognosis for survival. Two other patients are affected by chronic liver disease, probably due to chemotherapy. The authors confirm the importance of early diagnosis and prompt and effective treatment in children affected by retinoblastoma. They also stress that recently developed genetic techniques allow diagnosis of retinoblastoma even before the symptoms appear.