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Development of optic disk drusen in familial pseudopapilloedema—a pediatric case series
Volume 14 Number 1 / February 2010
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that there is a natural slow rate of cupping seen in children, and that there is a subset of children with a faster progression. This rate, projected into adulthood, would result in these children developing glaucomatous optic neuropathy. Conclusions: Prematurity and LBW are associated with increased rate of cupping in children. This association is important in stimulating future studies on this topic.
Discussion:Complications leading to visual loss were infrequent, however, a third of the reported incidences of sight loss were not treated. The majority of respondents favour treatment with cryotherapy over laser photocoagulation. Conclusions: The treatment of globe perforation during strabismus surgery may reduce the risk of sight threatening sequelae. Further prospective study is required to elucidate optimal management strategies.
095 Development of optic disk drusen in familial pseudopapilloedema—a pediatric case series. Harry Petrushkin, Nadeem Ali, Marie Restori, Gillian G. W. Adams Introduction: Children with optic disk swelling but no other features suggesting raised intracranial pressure present a challenging clinical scenario. Optic disk drusen can be a reassuring feature which may point towards pseudopapilloedema but may not always be present. We present children from two families with pseudopapilloedema who developed disk drusen over time. Methods: Five children (2 siblings from one family and 3 siblings from another family) were monitored in the paediatric ophthalmology department for 2-8 years. Age range at first presentation was 1-7 years. All underwent clinical examination, refraction, and serial B-scan ultrasonography of the optic disks and optic nerve sheaths. Three children had CT scans and one had a lumbar puncture. Results: All 5 children had optic disk swelling without other ophthalmoscopic features of papilloedema. Serial B-scan ultrasonography showed no drusen at the first visit, but over time, all children developed small linear drusen at the optic disk. At no point was there optic nerve sheath dilation. CT was avoided in two children because of the ultrasound findings, the absence of symptoms of raised intracranial pressure and normal visual function. CT was performed and reported as normal in three children. Discussion: Pseudopapilloedema can be familial so sibling examination is worthwhile. Reassuring symptomatology and B-scan ultrasonography may avoid the need for neuroimaging. Disk drusen in psuedopapilloedema may develop in later childhood, which highlights the value of serial B-scans.
097 Central tenotomy for small angle deviations. Adele Marie M. Roa, Anya A. Trumler, David B. Granet Introduction: Diplopic patients with a small angle strabismus in the range of 2D-5D are treated most commonly with prisms. This small deviation is not easily corrected surgically. The purpose of this study is to evaluate the outcome of a surgical technique, the central minitenotomy (1) in correcting small angle deviations. Methods: A Retrospective chart review of 7 patients with binocular diplopia who underwent the central mini-tenotomy for small angle deviations. Data gathered include the patient's demographics, subjective complaints, sensorimotor status, pre-operative and postoperative deviation, and complications. Results: The mean age at the time of surgery was 61 (range, 50-72 years old). All 7 patients presented with binocular diplopia. Preoperatively, the range of deviation was 2D to 6D. All needed this deviation to be corrected to eliminate diplopia in pre-operative testing and desired not to wear prism spectacles. Five subjects underwent central tenotomy of 1 inferior rectus and four out of those had an accompanying horizontal deviation that needed correction (horizontal muscle recess or resect procedure) to achieve binocular single vision. One patient had only a central tenotomy of the inferior rectus. Two patients had the procedure on the medial rectus. One had a unilateral central tenotomy but also had a vertical deviation that was corrected by an oblique muscle surgery. The other subject had bilateral medial rectus central tenotomy with symmetric bilateral inferior rectus recession. During early post-operative follow up (5th day), 6 patients measured between orthotropia to 2D of deviation. None of the 6 patients reported diplopia. Five subjects followed up between 3 weeks to 2 months postoperatively and measurements remained stable. There were no complications in any of the 7 subjects. Conclusions: The Central Mini-Tenotomy is a simple effective technique for the treatment of small angle strabismus, a vexing problem with few other surgical solutions.
096 The management of globe perforation during strabismus surgery. Dinesh Rathod, Raina Goyal, Patrick Watts Introduction: Globe perforation is a potentially devastating complication of strabismus surgery. Strabismologists may suspect or encounter a perforation during their careers however the management of such cases remains controversial. This study aims to report practices of strabismus surgeons who have previously either had reason to suspect or encountered a globe perforation. Methods: A survey of all consultant ophthalmologists in the United Kingdom was performed. The questionnaire solicited data on the management and outcomes of perforation if suspected or encountered during strabismus surgery. Results: Five hundred and six of 850 (59.5%) responses were collated. Of these 235 (46.4%) performed strabismus surgery, of whom 58.8% performed fewer than 50, 31.8% performed 50 to 100 and 8.6% performed more than 100 surgeries annually. Ninety-five (40.4%) had suspected globe perforation during strabismus surgery, of whom 83 subsequently carried out dilated fundoscopy, with twelve excluding retinal perforation. Fifty-three detailed treatment with cryopexy (50.9%), retinal laser (30.2%), both (9.4%) or other (3.8%). Three referred to vitreoretinal specialists for treatment. Thirty-three respondents prescribed additional antibiotics. Visual loss occurred in six cases, with two incidences of endophthalmitis, two retinal detachments, a suprachoroidal haemorrhage and one from a chorioretinal scar.
Journal of AAPOS
098 Secondary pseudotumor Cerebri in Patients with Nephropathic Cystinosis. David L. Rogers, Mary Lou McGregor Purpose: To report the association of secondary pseudotumor cerebri (PTC) in patients with nephropathic cystinosis. Methods: A retrospective review of all patients with cystinosis at Nationwide Children's Hospital between July 2007 and July 2009 was performed. Results: A total of 6 patients were identified. Age ranged from 7-22 years. There were 3 males and 3 females. Three patients had secondary PTC, 1 male and 2 females. Their ages ranged from 19 to 22 years. Two of these patients had a B-scan ultrasound of the optic nerve sheath diameter (ONSD) and both were wide measuring .5 mm. Treatment was initiated with oral acetazolamide. All patients were uncontrolled medically and required surgical intervention that included neurosurgical and optic nerve sheath decompression. All patients with secondary PTC were post-renal transplant. Conclusion: We find a high rate of secondary PTC in patients with cystinosis. By chance alone, only 1 in 10 billion patients should have both cystinosis and PTC. These patients have many risk factors
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that there is a natural slow rate of cupping seen in children, and that there is a subset of children with a faster progression. This rate, projected into adulthood, would result in these children developing glaucomatous optic neuropathy. Conclusions: Prematurity and LBW are associated with increased rate of cupping in children. This association is important in stimulating future studies on this topic.
Discussion:Complications leading to visual loss were infrequent, however, a third of the reported incidences of sight loss were not treated. The majority of respondents favour treatment with cryotherapy over laser photocoagulation. Conclusions: The treatment of globe perforation during strabismus surgery may reduce the risk of sight threatening sequelae. Further prospective study is required to elucidate optimal management strategies.
095 Development of optic disk drusen in familial pseudopapilloedema—a pediatric case series. Harry Petrushkin, Nadeem Ali, Marie Restori, Gillian G. W. Adams Introduction: Children with optic disk swelling but no other features suggesting raised intracranial pressure present a challenging clinical scenario. Optic disk drusen can be a reassuring feature which may point towards pseudopapilloedema but may not always be present. We present children from two families with pseudopapilloedema who developed disk drusen over time. Methods: Five children (2 siblings from one family and 3 siblings from another family) were monitored in the paediatric ophthalmology department for 2-8 years. Age range at first presentation was 1-7 years. All underwent clinical examination, refraction, and serial B-scan ultrasonography of the optic disks and optic nerve sheaths. Three children had CT scans and one had a lumbar puncture. Results: All 5 children had optic disk swelling without other ophthalmoscopic features of papilloedema. Serial B-scan ultrasonography showed no drusen at the first visit, but over time, all children developed small linear drusen at the optic disk. At no point was there optic nerve sheath dilation. CT was avoided in two children because of the ultrasound findings, the absence of symptoms of raised intracranial pressure and normal visual function. CT was performed and reported as normal in three children. Discussion: Pseudopapilloedema can be familial so sibling examination is worthwhile. Reassuring symptomatology and B-scan ultrasonography may avoid the need for neuroimaging. Disk drusen in psuedopapilloedema may develop in later childhood, which highlights the value of serial B-scans.
097 Central tenotomy for small angle deviations. Adele Marie M. Roa, Anya A. Trumler, David B. Granet Introduction: Diplopic patients with a small angle strabismus in the range of 2D-5D are treated most commonly with prisms. This small deviation is not easily corrected surgically. The purpose of this study is to evaluate the outcome of a surgical technique, the central minitenotomy (1) in correcting small angle deviations. Methods: A Retrospective chart review of 7 patients with binocular diplopia who underwent the central mini-tenotomy for small angle deviations. Data gathered include the patient's demographics, subjective complaints, sensorimotor status, pre-operative and postoperative deviation, and complications. Results: The mean age at the time of surgery was 61 (range, 50-72 years old). All 7 patients presented with binocular diplopia. Preoperatively, the range of deviation was 2D to 6D. All needed this deviation to be corrected to eliminate diplopia in pre-operative testing and desired not to wear prism spectacles. Five subjects underwent central tenotomy of 1 inferior rectus and four out of those had an accompanying horizontal deviation that needed correction (horizontal muscle recess or resect procedure) to achieve binocular single vision. One patient had only a central tenotomy of the inferior rectus. Two patients had the procedure on the medial rectus. One had a unilateral central tenotomy but also had a vertical deviation that was corrected by an oblique muscle surgery. The other subject had bilateral medial rectus central tenotomy with symmetric bilateral inferior rectus recession. During early post-operative follow up (5th day), 6 patients measured between orthotropia to 2D of deviation. None of the 6 patients reported diplopia. Five subjects followed up between 3 weeks to 2 months postoperatively and measurements remained stable. There were no complications in any of the 7 subjects. Conclusions: The Central Mini-Tenotomy is a simple effective technique for the treatment of small angle strabismus, a vexing problem with few other surgical solutions.
096 The management of globe perforation during strabismus surgery. Dinesh Rathod, Raina Goyal, Patrick Watts Introduction: Globe perforation is a potentially devastating complication of strabismus surgery. Strabismologists may suspect or encounter a perforation during their careers however the management of such cases remains controversial. This study aims to report practices of strabismus surgeons who have previously either had reason to suspect or encountered a globe perforation. Methods: A survey of all consultant ophthalmologists in the United Kingdom was performed. The questionnaire solicited data on the management and outcomes of perforation if suspected or encountered during strabismus surgery. Results: Five hundred and six of 850 (59.5%) responses were collated. Of these 235 (46.4%) performed strabismus surgery, of whom 58.8% performed fewer than 50, 31.8% performed 50 to 100 and 8.6% performed more than 100 surgeries annually. Ninety-five (40.4%) had suspected globe perforation during strabismus surgery, of whom 83 subsequently carried out dilated fundoscopy, with twelve excluding retinal perforation. Fifty-three detailed treatment with cryopexy (50.9%), retinal laser (30.2%), both (9.4%) or other (3.8%). Three referred to vitreoretinal specialists for treatment. Thirty-three respondents prescribed additional antibiotics. Visual loss occurred in six cases, with two incidences of endophthalmitis, two retinal detachments, a suprachoroidal haemorrhage and one from a chorioretinal scar.
Journal of AAPOS
098 Secondary pseudotumor Cerebri in Patients with Nephropathic Cystinosis. David L. Rogers, Mary Lou McGregor Purpose: To report the association of secondary pseudotumor cerebri (PTC) in patients with nephropathic cystinosis. Methods: A retrospective review of all patients with cystinosis at Nationwide Children's Hospital between July 2007 and July 2009 was performed. Results: A total of 6 patients were identified. Age ranged from 7-22 years. There were 3 males and 3 females. Three patients had secondary PTC, 1 male and 2 females. Their ages ranged from 19 to 22 years. Two of these patients had a B-scan ultrasound of the optic nerve sheath diameter (ONSD) and both were wide measuring .5 mm. Treatment was initiated with oral acetazolamide. All patients were uncontrolled medically and required surgical intervention that included neurosurgical and optic nerve sheath decompression. All patients with secondary PTC were post-renal transplant. Conclusion: We find a high rate of secondary PTC in patients with cystinosis. By chance alone, only 1 in 10 billion patients should have both cystinosis and PTC. These patients have many risk factors