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Volume 19 Number 2 / April 2015
A case of familial exudative vitreoretinopathy identified after genetic testing Kyle E. Miller, MD,a Mary J. Willis, MD,b and Scott K. McClatchey, MDc,d,e We report the case of a 21-month-old girl who was found to have familial exudative vitreoretinopathy after genetic testing revealed a genetic deletion at 7q22. She had previously been followed for exotropia; however, fundus examinations in the office were thought to be normal. After the pediatric geneticist identified the link between 7q22 deletions and vitreoretinopathies an examination under anesthesia was performed. Fluorescein angiography during this examination confirmed the presence of avascular areas of the retina.
Case Report
A
21-month-old girl, born at 41 weeks’ gestational age, presented with symptoms of exotropia. Her prenatal course was unremarkable. She was born via C-section because of nonreassuring fetal heart tones and was subsequently intubated and admitted to the neonatal intensive care unit for meconium aspiration. She was discharged after 1 week. She was readmitted within 2 months due to a second episode of pneumonia and global hypotonia. During systemic evaluation for this admission she had microarray genetic testing performed which revealed a gene deletion at the 7q22 location. She was diagnosed with pulmonary stenosis, global delay, low muscle tone, and apnea. She was dependent on gastrostomy feeds. The patient was evaluated at 10 months of age for possible strabismus. She could fix and follow, and cycloplegic refraction was 11.50 13.00 102 in the right eye and 12.00 13.00 094 in the left eye. She was noted to have an exotropia of 45D by Krimsky testing, with spontaneously alternating fixation. Fundus examination was unremarkable; retinal vasculature was normal, and there was no evidence of dilation, tortuosity, or arborization. She was
Author affiliations: aDepartment of Ophthalmology, Naval Medical Center San Diego, San Diego, California; bDepartment of Pediatrics, Naval Medical Center San Diego; c Ophthalmology Department, Naval Medical Center San Diego; dUniformed Services University of Health Sciences, Bethesda, Maryland; eLoma Linda University Medical Center, Loma Linda, California Disclaimer: The views expressed in this article are those of the author and do not necessarily reflect the official policy or position of the Department of the Navy, Department of Defense, nor the U.S. Government. Submitted July 31, 2014. Revision accepted October 3, 2014. Published online March 29, 2015. Correspondence: Kyle E. Miller, MD, NMCSD, Department of Ophthalmology, 34800 Bob Wilson Dr., San Diego, CA 92134 (email: [email protected]). J AAPOS 2015;19:178-180. Copyright Ó 2015 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/$36.00 http://dx.doi.org/10.1016/j.jaapos.2014.10.029
FIG 1. Normal posterior pole showing mild tilting of the disk.
prescribed spectacles; however, a large angle exotropia was still present on follow-up. A dilated fundus examination was again unremarkable except for large optic nerves in both eyes. Given her multiple other medical comorbidities, we elected to monitor her strabismus. She was subsequently evaluated by a pediatric geneticist, who noted the patient’s 7q22 gene deletion and identified the association with familial exudative vitreoretinopathy. Given the new genetic information, the patient underwent a third dilated fundus examination in the office, and the findings were considered normal to the midperiphery (Figure 1). Due to the difficulty of the examination, an examination under anesthesia with fluorescein angiography was performed and revealed areas of nonperfused retina in the periphery (Figure 2). A well-demarcated line was noted between the perfused and avascular retina as well as areas of late leakage at the transition zone (Figure 3). The patient subsequently underwent laser photocoagulation to the avascular zones.
Discussion Familial exudative vitreoretinopathy (FEVR) was described by Criswick and Schepens1 in 1969 as a process that had retinopathy of prematurity–like features in children born at term. They described peripheral avascular zones in the retina that would lead to fibrovascular changes and retinal traction that caused retinal detachments. They noted that the disease had a very unpredictable course and had a propensity to recur. The initial presentation of FEVR can range from small avascular zones of retina to total retinal detachment, with possible asymmetric involvement between the eyes. Given the wide variety of disease, several classification systems have been developed. Early classification systems defined three stages: a peripheral avascular zone (stage 1), fibrovascular mass lesions (stage 2), and retinal holes and tears or anterior segment changes (stage 3).2,3 Benson4 further described these stages (renamed mild, moderate, and severe) in his classification of 39 patients.4 Ranchod and colleagues5 classified a
Journal of AAPOS
Volume 19 Number 2 / April 2015
FIG 2. Peripheral photograph of the right eye. Note the mild irregularity noted in the periphery (arrow).
series of 273 eyes of 145 patients into 5 stages with subcategories, from avascular peripheral zones (stage 1) to total retinal detachment (stage 5). With avascular zones and retinal vessel leakage, our patient would be classified as stage 2A. An important facet of our case was the role genetic testing played in leading to the diagnosis and treatment. The pediatric geneticist informed us of the relationship between the 7q deletion and the loss of the autosomal dominant gene TSPAN12 located in this region. The TSPAN12 gene was shown by Poulter and colleagues10 to be involved in the development of autosomal dominant FEVR in patients who had previously screened negative for NDP, LRP5, or FZD4 mutations. These genes play a role in the Norrin-b-catenin signaling pathway, important in retinal vasculature development with TSPAN12 directly involved in the Norrin-FZD4-LRP5 signaling complex.6 The treatment of FEVR in the early stages is controversial. Benson’s report on 39 patients included 34 eyes with mild disease, of which 23 did not receive treatment. Final visual outcome for these patients was not explicitly reported; however, Benson states that treatment should be for those “at high risk of progression.”4 Pendergast and Trese7 monitored 6 eyes with stage 1 disease over 33 months and found no disease progression. More recently, Pinero and colleauges8 recommended early treatment and reviewed 17 eyes in which 33.3% had stage 1 disease and received laser or cryotherapy treatment. All patients had “stable” visual acuity. Treatment options are advancing as intravitreal antivascular endothelial growth factor therapies are investigated. Pendergast and Trese7 support laser therapy as the base treatment; however, some reports indicate the potential benefit of anti-VEGF therapy. A series of 4 eyes reported that all patients had reduced exudation, although 2 continued to progress to vitreoretinal traction.9 A more recent study examined the use of bevacizumab in 7 patients and found good results in patients who had not yet progressed to retinal detachment.10 Although these treatments show promise, there is much work to be done prior to widespread acceptance. More severe cases of the disease,
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179
FIG 3. Intravenous fluorescein angiogram at 2 minutes showing an area of peripheral nonperfusion (star), with an area of leakage at the avascular zone border consistent with the same area in Figure 2.
involving retinal detachments, require surgical repair, including scleral buckling with possible vitrectomy.7 Given the variety of disease severity at presentation and unpredictable course, the visual outcomes of patients with familial exudative vitreoretinopathy is also variable. Patients who present with neovascularization had visual acuity outcomes ranging from 20/40 to 20/100. Those with retinal detachment ranged from 20/20 to light perception in one series.7 Benson4 reported that patients who presented at less than 3 years of age or had neovascularization on examination trended to worse outcomes. The present case illustrates the importance of multidisciplinary care and communication to manage complex multisystemic disease. With the additional information provided by our geneticist, the patient was subsequently evaluated with fluorescein angiography leading to the prompt recognition and treatment of a potentially blinding disease. References 1. Criswick VG, Schepens CL. Familial exudative vitreoretinopathy. Am J Ophthalmol 1969;68:578-94. 2. Gow J, Oliver GL. Familial exudative vitreoretinopathy: an expanded view. Arch Ophthalmol 1971;86:150-55. 3. Laqua H. Familial exudative vitreoretinopathy. Albrecht Von Graefes Arch Klin Exp Ophthalmol 1980;213:121-33. 4. Benson W. Familial exudative vitreoretinopathy. Trans Am Ophthalmol Soc 1995;93:473-521. 5. Ranchod TM, Ho LY, Drenser KA, Capone A, Trese M. Clinical presentation of familial exudative vitreoretinopathy. Ophthalmology 2011;118:2070-75. 6. Poulter J, Ali M, Gilmour D, Rice A, et al. Mutations in TSPAN12 cause autosomal-dominant familial exudative vitreoretinopathy. Am J Hum Genet 2010;86:248-53. 7. Pendergast SD, Trese MT. Familial exudative vitreoretinopathy: results of surgical management. Ophthalmology 1998;105:1015-23. 8. Pinero AM, Sempere J, Nadal J, Elizalde-Montagut J. Familial exudative vitreoretinopathy: our experience. Arch Soc Esp Oftalmol 2008; 83:703-8. 9. Quiram PA, Drenser KA, Lai MM, Capone A, Trese MT. Treatment of vascularly active familial exudative vitreoretinopathy with pegaptanib sodium (Macugen). Retina 2008;28:S8-12.
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10. Sisk RA, Berrocal AM, Albini TA, Murray TG. Bevacizumab for the treatment of pediatric retinal and choroidal diseases. Ophthalmic Surg Lasers Imaging 2010;41:582-92.
Superior oblique palsy: A complication of endoscopic sinus surgery Jessica S. Lin, MD,a Ting T. Liu, MD,a R. Peter Manes, MD,b and Jennifer A. Galvin, MDa A 34-year-old woman with chronic sinusitis and nasal polyps was treated with bilateral pansinusotomy. Postoperatively, she had a unilateral superior oblique paresis. We suspect that superior oblique damage occurred without involvement of the medial rectus because of inadvertent entry of the endoscopic probe through the superior lamina into the left orbit.
Case Report
A
34-year-old woman presented 1 day after nasal endoscopic surgery with acute diplopia to an outside general ophthalmology office. Four days later she was referred to Yale Eye Center for further evaluation of her postoperative diplopia. Of note, her procedure was a stereotactic computer-assisted navigation with bilateral frontal sinusotomy, sphenoidotomy, total ethmoidectomy, and maxillary antrostomy with tissue removal. Medications included acetaminophen with codeine, fluticasone propionate nasal spray, and prednisone (20 mg daily). There was no prior history of trauma, strabismus, or diplopia before this surgery. On ophthalmological examination, corrected visual acuity was 20/20 in both eyes, ocular motility was 1 deficit in the field of action of the left superior oblique, and both pupils were round and reactive. She was found to have a left hypertropia of 6D in primary gaze and 9D in right gaze. Right head tilt was orthotropic, but there was a left hypertropia of 10D on left head tilt. Stereoacuity was reduced to 400 seconds of arc. Double Maddox rod testing demonstrated 5 of extorsion in the left eye. On prism adaptation test, her diplopia resolved with
FIG 1. Magnetic resonance imaging of the orbits with post-contrast enhancement noted on the 1 mm thin–cut coronal view. The arrow indicates enlargement of the proximal aspect of the left superior oblique muscle, with questionable discontinuity of left superior oblique muscle.
a 6 base-up prism over the right eye. Slit-lamp examination demonstrated ecchymosis over her left medial canthus. Otherwise, the anterior segment examination was within normal limits. At postoperative day 1, MRI of the orbits, with postcontrast enhancement noted on the 1 mm thin-cut coronal view, showed intramuscular edema, inflammation, and enlargement of the proximal aspect of the left superior oblique muscle with questionable discontinuity of left superior oblique muscle (Figure 1). The remaining extraocular muscles appeared intact. The clinical examination and imaging was consistent with an iatrogenic left superior oblique palsy. At 1 month follow-up her left hypertropia in primary gaze decreased to 1D, and double Maddox rod testing showed no extorsion. Her symptoms became minimal, with the only noticeable diplopia in reading position, as her left hypertropia was measured at 14D in downgaze. She was orthotropic on right head tilt and had a left hypertropia of 6D on left head tilt. At 3 months’ follow-up, she had a small right head tilt of \5 and remained orthotropic in primary gaze with no extorsion on double Maddox rod testing. In downgaze, her left hypertropia was noted to be increased, measuring at 18D. She was orthotropic on right head tilt and had left hypertropia of 6D on left head tilt. At both 1 and 3 month follow-up, the ocular versions showed a persistence of a 1 deficit in the field of action of the left superior oblique.
Discussion a
Author affiliations: Department of Ophthalmology and Visual Science, Yale School of Medicine, New Haven, Connecticut; bSection of Otolaryngology, Department of Surgery, Yale School of Medicine Submitted May 29, 2014. Revision accepted October 12, 2014. Published online March 30, 2015. Correspondence: Dr. Jennifer A. Galvin, MD, Yale School of Medicine, Department of Ophthalmology and Visual Science, 40 Temple Street, 3rd Floor, New Haven, CT 06510 (email: [email protected]). J AAPOS 2015;19:180-181. Copyright Ó 2015 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/$36.00 http://dx.doi.org/10.1016/j.jaapos.2014.10.031
Superior oblique palsy after endoscopic surgery has been known to occur in conjunction with damage to the medial or inferior rectus muscle, with the medial rectus being the most commonly affected muscle because it lies closest to the lamina papyracea.1-4 A retrospective review of 15 cases demonstrated superior oblique palsy in 4 cases from nerve damage, damage to or destruction of the muscle itself, or due to mild contusion of the muscle.1 To our knowledge, this is the first reported case of isolated superior oblique palsy following nasal endoscopic surgery. Surgical techniques involving external frontal approaches that do not involve endoscopic means have been reported to cause isolated
Journal of AAPOS
Miller, Willis, and McClatchey
Volume 19 Number 2 / April 2015
A case of familial exudative vitreoretinopathy identified after genetic testing Kyle E. Miller, MD,a Mary J. Willis, MD,b and Scott K. McClatchey, MDc,d,e We report the case of a 21-month-old girl who was found to have familial exudative vitreoretinopathy after genetic testing revealed a genetic deletion at 7q22. She had previously been followed for exotropia; however, fundus examinations in the office were thought to be normal. After the pediatric geneticist identified the link between 7q22 deletions and vitreoretinopathies an examination under anesthesia was performed. Fluorescein angiography during this examination confirmed the presence of avascular areas of the retina.
Case Report
A
21-month-old girl, born at 41 weeks’ gestational age, presented with symptoms of exotropia. Her prenatal course was unremarkable. She was born via C-section because of nonreassuring fetal heart tones and was subsequently intubated and admitted to the neonatal intensive care unit for meconium aspiration. She was discharged after 1 week. She was readmitted within 2 months due to a second episode of pneumonia and global hypotonia. During systemic evaluation for this admission she had microarray genetic testing performed which revealed a gene deletion at the 7q22 location. She was diagnosed with pulmonary stenosis, global delay, low muscle tone, and apnea. She was dependent on gastrostomy feeds. The patient was evaluated at 10 months of age for possible strabismus. She could fix and follow, and cycloplegic refraction was 11.50 13.00 102 in the right eye and 12.00 13.00 094 in the left eye. She was noted to have an exotropia of 45D by Krimsky testing, with spontaneously alternating fixation. Fundus examination was unremarkable; retinal vasculature was normal, and there was no evidence of dilation, tortuosity, or arborization. She was
Author affiliations: aDepartment of Ophthalmology, Naval Medical Center San Diego, San Diego, California; bDepartment of Pediatrics, Naval Medical Center San Diego; c Ophthalmology Department, Naval Medical Center San Diego; dUniformed Services University of Health Sciences, Bethesda, Maryland; eLoma Linda University Medical Center, Loma Linda, California Disclaimer: The views expressed in this article are those of the author and do not necessarily reflect the official policy or position of the Department of the Navy, Department of Defense, nor the U.S. Government. Submitted July 31, 2014. Revision accepted October 3, 2014. Published online March 29, 2015. Correspondence: Kyle E. Miller, MD, NMCSD, Department of Ophthalmology, 34800 Bob Wilson Dr., San Diego, CA 92134 (email: [email protected]). J AAPOS 2015;19:178-180. Copyright Ó 2015 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/$36.00 http://dx.doi.org/10.1016/j.jaapos.2014.10.029
FIG 1. Normal posterior pole showing mild tilting of the disk.
prescribed spectacles; however, a large angle exotropia was still present on follow-up. A dilated fundus examination was again unremarkable except for large optic nerves in both eyes. Given her multiple other medical comorbidities, we elected to monitor her strabismus. She was subsequently evaluated by a pediatric geneticist, who noted the patient’s 7q22 gene deletion and identified the association with familial exudative vitreoretinopathy. Given the new genetic information, the patient underwent a third dilated fundus examination in the office, and the findings were considered normal to the midperiphery (Figure 1). Due to the difficulty of the examination, an examination under anesthesia with fluorescein angiography was performed and revealed areas of nonperfused retina in the periphery (Figure 2). A well-demarcated line was noted between the perfused and avascular retina as well as areas of late leakage at the transition zone (Figure 3). The patient subsequently underwent laser photocoagulation to the avascular zones.
Discussion Familial exudative vitreoretinopathy (FEVR) was described by Criswick and Schepens1 in 1969 as a process that had retinopathy of prematurity–like features in children born at term. They described peripheral avascular zones in the retina that would lead to fibrovascular changes and retinal traction that caused retinal detachments. They noted that the disease had a very unpredictable course and had a propensity to recur. The initial presentation of FEVR can range from small avascular zones of retina to total retinal detachment, with possible asymmetric involvement between the eyes. Given the wide variety of disease, several classification systems have been developed. Early classification systems defined three stages: a peripheral avascular zone (stage 1), fibrovascular mass lesions (stage 2), and retinal holes and tears or anterior segment changes (stage 3).2,3 Benson4 further described these stages (renamed mild, moderate, and severe) in his classification of 39 patients.4 Ranchod and colleagues5 classified a
Journal of AAPOS
Volume 19 Number 2 / April 2015
FIG 2. Peripheral photograph of the right eye. Note the mild irregularity noted in the periphery (arrow).
series of 273 eyes of 145 patients into 5 stages with subcategories, from avascular peripheral zones (stage 1) to total retinal detachment (stage 5). With avascular zones and retinal vessel leakage, our patient would be classified as stage 2A. An important facet of our case was the role genetic testing played in leading to the diagnosis and treatment. The pediatric geneticist informed us of the relationship between the 7q deletion and the loss of the autosomal dominant gene TSPAN12 located in this region. The TSPAN12 gene was shown by Poulter and colleagues10 to be involved in the development of autosomal dominant FEVR in patients who had previously screened negative for NDP, LRP5, or FZD4 mutations. These genes play a role in the Norrin-b-catenin signaling pathway, important in retinal vasculature development with TSPAN12 directly involved in the Norrin-FZD4-LRP5 signaling complex.6 The treatment of FEVR in the early stages is controversial. Benson’s report on 39 patients included 34 eyes with mild disease, of which 23 did not receive treatment. Final visual outcome for these patients was not explicitly reported; however, Benson states that treatment should be for those “at high risk of progression.”4 Pendergast and Trese7 monitored 6 eyes with stage 1 disease over 33 months and found no disease progression. More recently, Pinero and colleauges8 recommended early treatment and reviewed 17 eyes in which 33.3% had stage 1 disease and received laser or cryotherapy treatment. All patients had “stable” visual acuity. Treatment options are advancing as intravitreal antivascular endothelial growth factor therapies are investigated. Pendergast and Trese7 support laser therapy as the base treatment; however, some reports indicate the potential benefit of anti-VEGF therapy. A series of 4 eyes reported that all patients had reduced exudation, although 2 continued to progress to vitreoretinal traction.9 A more recent study examined the use of bevacizumab in 7 patients and found good results in patients who had not yet progressed to retinal detachment.10 Although these treatments show promise, there is much work to be done prior to widespread acceptance. More severe cases of the disease,
Journal of AAPOS
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FIG 3. Intravenous fluorescein angiogram at 2 minutes showing an area of peripheral nonperfusion (star), with an area of leakage at the avascular zone border consistent with the same area in Figure 2.
involving retinal detachments, require surgical repair, including scleral buckling with possible vitrectomy.7 Given the variety of disease severity at presentation and unpredictable course, the visual outcomes of patients with familial exudative vitreoretinopathy is also variable. Patients who present with neovascularization had visual acuity outcomes ranging from 20/40 to 20/100. Those with retinal detachment ranged from 20/20 to light perception in one series.7 Benson4 reported that patients who presented at less than 3 years of age or had neovascularization on examination trended to worse outcomes. The present case illustrates the importance of multidisciplinary care and communication to manage complex multisystemic disease. With the additional information provided by our geneticist, the patient was subsequently evaluated with fluorescein angiography leading to the prompt recognition and treatment of a potentially blinding disease. References 1. Criswick VG, Schepens CL. Familial exudative vitreoretinopathy. Am J Ophthalmol 1969;68:578-94. 2. Gow J, Oliver GL. Familial exudative vitreoretinopathy: an expanded view. Arch Ophthalmol 1971;86:150-55. 3. Laqua H. Familial exudative vitreoretinopathy. Albrecht Von Graefes Arch Klin Exp Ophthalmol 1980;213:121-33. 4. Benson W. Familial exudative vitreoretinopathy. Trans Am Ophthalmol Soc 1995;93:473-521. 5. Ranchod TM, Ho LY, Drenser KA, Capone A, Trese M. Clinical presentation of familial exudative vitreoretinopathy. Ophthalmology 2011;118:2070-75. 6. Poulter J, Ali M, Gilmour D, Rice A, et al. Mutations in TSPAN12 cause autosomal-dominant familial exudative vitreoretinopathy. Am J Hum Genet 2010;86:248-53. 7. Pendergast SD, Trese MT. Familial exudative vitreoretinopathy: results of surgical management. Ophthalmology 1998;105:1015-23. 8. Pinero AM, Sempere J, Nadal J, Elizalde-Montagut J. Familial exudative vitreoretinopathy: our experience. Arch Soc Esp Oftalmol 2008; 83:703-8. 9. Quiram PA, Drenser KA, Lai MM, Capone A, Trese MT. Treatment of vascularly active familial exudative vitreoretinopathy with pegaptanib sodium (Macugen). Retina 2008;28:S8-12.
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10. Sisk RA, Berrocal AM, Albini TA, Murray TG. Bevacizumab for the treatment of pediatric retinal and choroidal diseases. Ophthalmic Surg Lasers Imaging 2010;41:582-92.
Superior oblique palsy: A complication of endoscopic sinus surgery Jessica S. Lin, MD,a Ting T. Liu, MD,a R. Peter Manes, MD,b and Jennifer A. Galvin, MDa A 34-year-old woman with chronic sinusitis and nasal polyps was treated with bilateral pansinusotomy. Postoperatively, she had a unilateral superior oblique paresis. We suspect that superior oblique damage occurred without involvement of the medial rectus because of inadvertent entry of the endoscopic probe through the superior lamina into the left orbit.
Case Report
A
34-year-old woman presented 1 day after nasal endoscopic surgery with acute diplopia to an outside general ophthalmology office. Four days later she was referred to Yale Eye Center for further evaluation of her postoperative diplopia. Of note, her procedure was a stereotactic computer-assisted navigation with bilateral frontal sinusotomy, sphenoidotomy, total ethmoidectomy, and maxillary antrostomy with tissue removal. Medications included acetaminophen with codeine, fluticasone propionate nasal spray, and prednisone (20 mg daily). There was no prior history of trauma, strabismus, or diplopia before this surgery. On ophthalmological examination, corrected visual acuity was 20/20 in both eyes, ocular motility was 1 deficit in the field of action of the left superior oblique, and both pupils were round and reactive. She was found to have a left hypertropia of 6D in primary gaze and 9D in right gaze. Right head tilt was orthotropic, but there was a left hypertropia of 10D on left head tilt. Stereoacuity was reduced to 400 seconds of arc. Double Maddox rod testing demonstrated 5 of extorsion in the left eye. On prism adaptation test, her diplopia resolved with
FIG 1. Magnetic resonance imaging of the orbits with post-contrast enhancement noted on the 1 mm thin–cut coronal view. The arrow indicates enlargement of the proximal aspect of the left superior oblique muscle, with questionable discontinuity of left superior oblique muscle.
a 6 base-up prism over the right eye. Slit-lamp examination demonstrated ecchymosis over her left medial canthus. Otherwise, the anterior segment examination was within normal limits. At postoperative day 1, MRI of the orbits, with postcontrast enhancement noted on the 1 mm thin-cut coronal view, showed intramuscular edema, inflammation, and enlargement of the proximal aspect of the left superior oblique muscle with questionable discontinuity of left superior oblique muscle (Figure 1). The remaining extraocular muscles appeared intact. The clinical examination and imaging was consistent with an iatrogenic left superior oblique palsy. At 1 month follow-up her left hypertropia in primary gaze decreased to 1D, and double Maddox rod testing showed no extorsion. Her symptoms became minimal, with the only noticeable diplopia in reading position, as her left hypertropia was measured at 14D in downgaze. She was orthotropic on right head tilt and had a left hypertropia of 6D on left head tilt. At 3 months’ follow-up, she had a small right head tilt of \5 and remained orthotropic in primary gaze with no extorsion on double Maddox rod testing. In downgaze, her left hypertropia was noted to be increased, measuring at 18D. She was orthotropic on right head tilt and had left hypertropia of 6D on left head tilt. At both 1 and 3 month follow-up, the ocular versions showed a persistence of a 1 deficit in the field of action of the left superior oblique.
Discussion a
Author affiliations: Department of Ophthalmology and Visual Science, Yale School of Medicine, New Haven, Connecticut; bSection of Otolaryngology, Department of Surgery, Yale School of Medicine Submitted May 29, 2014. Revision accepted October 12, 2014. Published online March 30, 2015. Correspondence: Dr. Jennifer A. Galvin, MD, Yale School of Medicine, Department of Ophthalmology and Visual Science, 40 Temple Street, 3rd Floor, New Haven, CT 06510 (email: [email protected]). J AAPOS 2015;19:180-181. Copyright Ó 2015 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/$36.00 http://dx.doi.org/10.1016/j.jaapos.2014.10.031
Superior oblique palsy after endoscopic surgery has been known to occur in conjunction with damage to the medial or inferior rectus muscle, with the medial rectus being the most commonly affected muscle because it lies closest to the lamina papyracea.1-4 A retrospective review of 15 cases demonstrated superior oblique palsy in 4 cases from nerve damage, damage to or destruction of the muscle itself, or due to mild contusion of the muscle.1 To our knowledge, this is the first reported case of isolated superior oblique palsy following nasal endoscopic surgery. Surgical techniques involving external frontal approaches that do not involve endoscopic means have been reported to cause isolated
Journal of AAPOS
