Letters to the Editor ISOLATED OCULOMOTOR PALSY IN A CHILD CAUSED BY AN INTERNAL CAROTID ARTERY ANEURYSM To the Editor: Shah and colleagues1 describe isolated oculomotor nerve palsy arising from an internal carotid artery aneurysm. The authors emphasize that aneurysms need to be included in the differential diagnosis of acquired oculomotor palsy in childhood. We recently examined a child who developed a right oculomotor palsy in conjunction with a large cavernous malformation of the midbrain. At 28 months of age, he presented with isolated mydriasis leading to oculomotor palsy in the right eye. He subsequently developed a left hemiparesis (Weber syndrome) and left facial weakness. Magnetic resonance (MR) imaging showed an intraparenchymal cavernous hemangioma within the midbrain (Figure 1A). Although the appearance of the lesion initially suggested aneurysm it was distinguishable by its intraparenchymal localization and its signal characteristics (Figure 1). Surgical intervention was not recommended because of the high risk of neurologic deficit with resection. Over the following 3 months, his ptosis and ocular motor rotations recovered with no signs of aberrant regeneration. He was left with a mild residual anisocoria and a small intermittent exotropia. Repeat MR imaging showed contraction of the lesion and resolution of the surrounding edema (Figure 1B), and an adjacent developmental venous anomaly. (Figure 1C). This clinical history demonstrates that cavernous malformations of the midbrain should be considered in the differential diagnosis of acquired vascular oculomotor palsies of childhood.2 In children, cavernous malformations of the brainstem tend to be larger in size and show higher rates of recurrence. Cavernous malformations typically manifest as hypointense lesions on T2-weighted sequences and even more pronounced hypointensity on susceptibility sequences (so-called “blooming phenome-
non”).3 Occasionally, developmental venous anomalies (formerly referred to as venous angiomas) with characteristic curvilinear enhancement and branching pattern (so-called Medusa head appearance) are associated with these lesions.3 (Figure 1). Rarely, they may acutely hemorrhage and develop surrounding edema. Unlike aneurysms, they are not associated with flow-related artifact and are located within the brain parenchyma rather than the subarachnoid space. Spontaneous hemorrhage and edema can produce acute neurological signs that may gradually improve without treatment as the hemorrhage and edema subside.4 Rarely, extraparenchymal cavernomas can directly infiltrate the oculomotor nerve and mimic presentation of posterior communicating artery aneurysms.5 Due to the high risk of neurologic injury with surgical resection and sometimes indolent clinical course, brainstem cavernous malformations are often observed after a single clinical event. If the malformation rebleeds, the cavernoma is large or presents near to the surface of the brainstem, or the patient suffers further neurologic deterioration, surgery may be offered at that time. Michael C. Brodsky, MD Departments of Ophthalmology and Neurology Mayo Foundation, Rochester, Minnesota Nicholas M. Wetjen, MD Department of Neurosurgery, Mayo Foundation Rochester, Minnesota Gesina F. Keating, MD Department of Neurology, Mayo Foundation Rochester, Minnesota Kelly K. Koeller, MD Department of Radiology, Mayo Foundation Rochester, Minnesota
FIG 1. Cavernous malformation of the midbrain. A, Axial T2-weighted magnetic resonance (MR) image showing predominantly hypointense but heterogeneous mass of right cerebral peduncle with surrounding hyperintensity, most consistent with edema. B, Follow-up axial T2-weighted MR image showing resolution of surrounding of T2 hyperintensity and contraction in size of mass. C, Follow-up post-contrast axial T1-weighted MR image showing curvilinear enhancement (arrows) in pattern typical for developmental venous anomaly.
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Letters to the Editor References
1. Shah V, Kumar M, Akkayasamy K, Rana K. Isolated oculomotor palsy in a child caused by an internal carotid artery aneurysm. J AAPOS 2013; 17:648-9. 2. Abla AA, Lekovic GP, Garrett M, et al. Cavernous malformations of the brainstem presenting in childhood: Surgical experience in 40 patients. Neurosurgery 2010;67:1589-99. 3. Campeau NG, Lane JI. De Novo development of a lesion with the appearance of a cavernous malformation adjacent to an existing developmental venous anomaly. AJNR Am J Neuroradiol 2005;26:156-9. 4. Flemming KD, Link MJ, Christianson TJH, et al. Prospective hemorrhage risk of intracerebral cavernous malformations. Neurology 2012;78:632-6. 5. Wolfe SQ, Manzano G, Langer DJ, Marcos JJ. Cavernous malformation of the oculomotor nerve mimicking a partially thrombosed posterior communicating artery aneurysm: Report of two cases. Neurosurgery 2011;69:470-74. Supported in part by a grant from Research to Prevent Blindness, Inc. http://dx.doi.org/10.1016/j.jaapos.2014.02.008 J AAPOS 2014;18:515-516. Copyright Ó 2014 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/$36.00 Editor’s note: Shah and colleagues declined to respond to this letter.
CLINICAL EVOLUTION OF BILATERAL BROWN SYNDROME To the Editor: We read with interest the recent Sorrentino and Warman’s recent article concerning the natural history of bilateral Brown syndrome.1 The authors retrospectively reviewed the medical records of patients with bilateral Brown syndrome not treated surgically. At time of first diagnosis, 8 of the 9 patients presented with orthophoria in primary position; 1 with esotropia of 18D. Only 1 patient had a compensatory head position at diagnosis (a slight chin-up position). At last follow-up, 6 of the patients remained orthophoric in primary position, 1 developed an exotropia of 30 D, and 1 developed a right hyperphoria. The patient initially diagnosed with esotropia had progression of the horizontal strabismus and was treated surgically. Five patients remained with little or no compensatory head position. Brown syndrome, characterized by a limitation of elevation in adduction and positive forced duction testing, is usually unilateral but occurs bilaterally in 10% of all cases. The natural history of congenital Brown syndrome is poorly understood. It seems likely that a significant percentage of patients with unilateral1-3 or bilateral4 Brown syndrome will experience spontaneous improvement or resolution of their motility disturbance without surgical intervention. Due to the rarity of the bilateral involvement and the lack of studies with long-term follow-up, we would like to broaden the available evidence pertaining to the clinical evolution of the bilateral Brown syndrome by relating a small case series.
Volume 18 Number 5 / October 2014 We retrospectively reviewed the medical records of bilateral Brown syndrome evaluated and followed at Ophthalmology Departments of Bambino Ges u IRCCS Children’s Hospital of Rome between 2005 and 2013. A total of 5 cases (3 females) were included. All of them had idiopathic Brown syndrome. The age range at first diagnosis was 1-6 years of age. The mean follow-up period was 5.2 years (range, 1-8 years). At the time of first diagnosis, 2 of the 5 patients were orthophoric in primary position, 2 were esotropic, and 1 presented right hypertropia. Three patients had a compensatory head position at diagnosis (chin-up position). At the most recent follow-up visit, 2 patients (40%) had some improvement in their ocular movements without any intervention. In their most recent control, 4 patients were orthophoric in primary position, the hypertropic patient showed a reduction of the vertical strabismus, and 1 patient remained esotropic. A compensatory head position was present in only 1 patient at the end of follow-up. In this case series, the clinical evolution of the strabismus due to bilateral Brown syndrome seemed to be positive without surgical treatment. We agree with Sorrentino and Warman1 in suggesting careful evaluation of the clinical conditions and adequate follow-up before considering a surgical approach. Most longitudinal series of patients with congenital Brown syndrome have a relatively shortterm follow-up and consequently likely underestimate the true incidence of spontaneous resolution. Luca Buzzonetti, MD Sergio Petroni, MD Paolo Capozzi, MD Ophthalmology Department Bambino Ges u Children’s Hospital IRCCS, Rome, Italy References 1. Sorrentino D, Warman R. Clinical progression of untreated bilateral Brown syndrome. J AAPOS 2014;18:156-8. 2. Dawson E, Barry J, Lee J. Spontaneous resolution in patients with congenital Brown syndrome. J AAPOS 2009;13:116-18. 3. Lambert SR. Late spontaneous resolution of congenital Brown syndrome. J AAPOS 2010;14:373-5. 4. Capasso L, Torre A, Gagliardi V, Magli A. Spontaneous resolution of congenital bilateral Brown’s syndrome. Ophthalmologica 2001;215: 372-5. http://dx.doi.org/10.1016/j.jaapos.2014.06.002 J AAPOS 2014;18:516. Copyright Ó 2014 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/$36.00 Editor’s note: Sorrentino and Warman declined to respond to this letter.
Journal of AAPOS
non”).3 Occasionally, developmental venous anomalies (formerly referred to as venous angiomas) with characteristic curvilinear enhancement and branching pattern (so-called Medusa head appearance) are associated with these lesions.3 (Figure 1). Rarely, they may acutely hemorrhage and develop surrounding edema. Unlike aneurysms, they are not associated with flow-related artifact and are located within the brain parenchyma rather than the subarachnoid space. Spontaneous hemorrhage and edema can produce acute neurological signs that may gradually improve without treatment as the hemorrhage and edema subside.4 Rarely, extraparenchymal cavernomas can directly infiltrate the oculomotor nerve and mimic presentation of posterior communicating artery aneurysms.5 Due to the high risk of neurologic injury with surgical resection and sometimes indolent clinical course, brainstem cavernous malformations are often observed after a single clinical event. If the malformation rebleeds, the cavernoma is large or presents near to the surface of the brainstem, or the patient suffers further neurologic deterioration, surgery may be offered at that time. Michael C. Brodsky, MD Departments of Ophthalmology and Neurology Mayo Foundation, Rochester, Minnesota Nicholas M. Wetjen, MD Department of Neurosurgery, Mayo Foundation Rochester, Minnesota Gesina F. Keating, MD Department of Neurology, Mayo Foundation Rochester, Minnesota Kelly K. Koeller, MD Department of Radiology, Mayo Foundation Rochester, Minnesota
FIG 1. Cavernous malformation of the midbrain. A, Axial T2-weighted magnetic resonance (MR) image showing predominantly hypointense but heterogeneous mass of right cerebral peduncle with surrounding hyperintensity, most consistent with edema. B, Follow-up axial T2-weighted MR image showing resolution of surrounding of T2 hyperintensity and contraction in size of mass. C, Follow-up post-contrast axial T1-weighted MR image showing curvilinear enhancement (arrows) in pattern typical for developmental venous anomaly.
Journal of AAPOS
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Letters to the Editor References
1. Shah V, Kumar M, Akkayasamy K, Rana K. Isolated oculomotor palsy in a child caused by an internal carotid artery aneurysm. J AAPOS 2013; 17:648-9. 2. Abla AA, Lekovic GP, Garrett M, et al. Cavernous malformations of the brainstem presenting in childhood: Surgical experience in 40 patients. Neurosurgery 2010;67:1589-99. 3. Campeau NG, Lane JI. De Novo development of a lesion with the appearance of a cavernous malformation adjacent to an existing developmental venous anomaly. AJNR Am J Neuroradiol 2005;26:156-9. 4. Flemming KD, Link MJ, Christianson TJH, et al. Prospective hemorrhage risk of intracerebral cavernous malformations. Neurology 2012;78:632-6. 5. Wolfe SQ, Manzano G, Langer DJ, Marcos JJ. Cavernous malformation of the oculomotor nerve mimicking a partially thrombosed posterior communicating artery aneurysm: Report of two cases. Neurosurgery 2011;69:470-74. Supported in part by a grant from Research to Prevent Blindness, Inc. http://dx.doi.org/10.1016/j.jaapos.2014.02.008 J AAPOS 2014;18:515-516. Copyright Ó 2014 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/$36.00 Editor’s note: Shah and colleagues declined to respond to this letter.
CLINICAL EVOLUTION OF BILATERAL BROWN SYNDROME To the Editor: We read with interest the recent Sorrentino and Warman’s recent article concerning the natural history of bilateral Brown syndrome.1 The authors retrospectively reviewed the medical records of patients with bilateral Brown syndrome not treated surgically. At time of first diagnosis, 8 of the 9 patients presented with orthophoria in primary position; 1 with esotropia of 18D. Only 1 patient had a compensatory head position at diagnosis (a slight chin-up position). At last follow-up, 6 of the patients remained orthophoric in primary position, 1 developed an exotropia of 30 D, and 1 developed a right hyperphoria. The patient initially diagnosed with esotropia had progression of the horizontal strabismus and was treated surgically. Five patients remained with little or no compensatory head position. Brown syndrome, characterized by a limitation of elevation in adduction and positive forced duction testing, is usually unilateral but occurs bilaterally in 10% of all cases. The natural history of congenital Brown syndrome is poorly understood. It seems likely that a significant percentage of patients with unilateral1-3 or bilateral4 Brown syndrome will experience spontaneous improvement or resolution of their motility disturbance without surgical intervention. Due to the rarity of the bilateral involvement and the lack of studies with long-term follow-up, we would like to broaden the available evidence pertaining to the clinical evolution of the bilateral Brown syndrome by relating a small case series.
Volume 18 Number 5 / October 2014 We retrospectively reviewed the medical records of bilateral Brown syndrome evaluated and followed at Ophthalmology Departments of Bambino Ges u IRCCS Children’s Hospital of Rome between 2005 and 2013. A total of 5 cases (3 females) were included. All of them had idiopathic Brown syndrome. The age range at first diagnosis was 1-6 years of age. The mean follow-up period was 5.2 years (range, 1-8 years). At the time of first diagnosis, 2 of the 5 patients were orthophoric in primary position, 2 were esotropic, and 1 presented right hypertropia. Three patients had a compensatory head position at diagnosis (chin-up position). At the most recent follow-up visit, 2 patients (40%) had some improvement in their ocular movements without any intervention. In their most recent control, 4 patients were orthophoric in primary position, the hypertropic patient showed a reduction of the vertical strabismus, and 1 patient remained esotropic. A compensatory head position was present in only 1 patient at the end of follow-up. In this case series, the clinical evolution of the strabismus due to bilateral Brown syndrome seemed to be positive without surgical treatment. We agree with Sorrentino and Warman1 in suggesting careful evaluation of the clinical conditions and adequate follow-up before considering a surgical approach. Most longitudinal series of patients with congenital Brown syndrome have a relatively shortterm follow-up and consequently likely underestimate the true incidence of spontaneous resolution. Luca Buzzonetti, MD Sergio Petroni, MD Paolo Capozzi, MD Ophthalmology Department Bambino Ges u Children’s Hospital IRCCS, Rome, Italy References 1. Sorrentino D, Warman R. Clinical progression of untreated bilateral Brown syndrome. J AAPOS 2014;18:156-8. 2. Dawson E, Barry J, Lee J. Spontaneous resolution in patients with congenital Brown syndrome. J AAPOS 2009;13:116-18. 3. Lambert SR. Late spontaneous resolution of congenital Brown syndrome. J AAPOS 2010;14:373-5. 4. Capasso L, Torre A, Gagliardi V, Magli A. Spontaneous resolution of congenital bilateral Brown’s syndrome. Ophthalmologica 2001;215: 372-5. http://dx.doi.org/10.1016/j.jaapos.2014.06.002 J AAPOS 2014;18:516. Copyright Ó 2014 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/$36.00 Editor’s note: Sorrentino and Warman declined to respond to this letter.
Journal of AAPOS
