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Accessory fibrotic lateral rectus muscles in exotropic Duane syndrome with severe retraction and upshoot
Video Articles Accessory fibrotic lateral rectus muscles in exotropic Duane syndrome with severe retraction and upshoot Stacy L. Pineles, MD, and Federico G. Velez, MD This article may be viewed at jaapos.org.
Article Summary
A
3.5-year-old boy presented at the University of California Los Angeles–Olive View Hospital with abnormal eye movements present since birth. His parents noticed that his left eye always appeared smaller than his right eye and that he had a right head turn. On examination, the patient demonstrated a 5 right head turn. In forced primary position, he had an exotropia of 6D. He had 2 limitation to adduction and abduction and 13 overelevation in adduction (upshoot) in the left eye. The decision was made to perform surgery consisting of a lateral rectus recession with Y-splitting for presumed exotropic Duane syndrome with a significant upshoot. Preoperative forced duction testing revealed moderate restriction to adduction and no restriction to abduction. The surgical video demonstrates the presence of 2 accessory bands posterior to the lateral rectus muscle. All 3 structures (the lateral rectus and the 2 accessory bands) were recessed to 17 mm from the limbus in a V-shaped configuration (Figures 1 and 2). At postoperative month 1, the patient was orthotropic in primary position, and his upshoot had resolved. His adduction improved to 1 but his abduction worsened to 3. First reported in 1893 by Nussbaum,1 accessory muscles have been well described in disorders such as Duane syndrome and congenital fibrosis of the extraocular muscles.2 They should be suspected in patients with atypical patterns of restrictive strabismus, globe retraction in directions other than adduction, or in patients with severe up- or downshoots. Lueder2 described 3 types of accessory bands in his 2002 review: (1) structures arising from extraocular muscles and inserting in abnormal locations, (2) fibrous
Author affiliations: Jules Stein Eye Institute and Department of Ophthalmology, University of California, Los Angeles Grant support: NIH/NEI K23EY021762 (SLP), Knights Templar Eye Foundation (SLP), Oppenheimer Family Foundation (SLP), Research to Prevent Blindness (all authors). Submitted April 7, 2015. Revision accepted May 30, 2015. Correspondence: Federico G. Velez, MD, Jules Stein Eye Institute, 100 Stein Plaza, David Geffen School of Medicine at UCLA, Los Angles, CA 90095-7002 (email: velez@ Jsei.ucla.edu). Copyright Ó 2015 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/$36.00 http://dx.doi.org/10.1016/j.jaapos.2015.05.023
Journal of AAPOS
FIGURE 1. Final position of lateral rectus (LR) and accessory bands.
bands located beneath the rectus muscles, and (3) discrete anomalous structures in the posterior orbit. In a study of 453 strabismic subjects, Khitri and Demer3 demonstrated anomalous bands in 2.4% of subjects evaluated by magnetic resonance imaging. Histopathological evidence implicates that these bands are made up of extraocular muscle tissue4; however, pathological specimens are rare, because the bands are often difficult to access and not necessarily removed as part of routine treatment. In our case, no pathological specimen was obtained; however, the bands grossly resembled fibrous tissue with scattered muscle fibers. We theorize
FIGURE 2. Multiple separate bands could be seen in the location of the lateral rectus.
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Pineles and Velez
therefore, that they are a combination of Lueder’s first and second types, that is, a fibrous band with some features of extraocular muscle. References 1. Nussbaum M. Vergleichend-anatomische Beitr€age zur Kenntnis der Augenmuskeln. Anat Anz 1893;8:208-10.
Volume 19 Number 6 / December 2015 2. Lueder GT. Anomalous orbital structures resulting in unusual strabismus. Surv Ophthalmol 2002;47:27-35. 3. Khitri MR, Demer JL. Magnetic resonance imaging of tissues compatible with supernumerary extraocular muscles. Am J Ophthalmol 2010; 150:925-31. 4. Knowlton PB, Mawn LA, Atkinson JB, Donahue SP. Strabismus resulting from an anomalous extraocular muscle in Gorlin syndrome. J AAPOS 2014;18:495-8.
Review of A Family Guide to Childhood Glaucoma and Cataracts 2014, by Alex V. Levin with Christopher Fecarotta, edited by Sharon F. Freedman, Andrea Osborn, and Ian Hubling (Shadow Fusion, 2014). This book was developed by the Pediatric Glaucoma and Cataract Family Association, organized in 1993 as a collaboration between Dr. Alex Levin and parents of children with glaucoma at the Hospital for Sick Children, Toronto. The group was expanded in 2000 to include families of children with cataracts. The organization provides information and support to families with children affected by these complicated disorders and facilitates communication between families. A Family Guide is the group’s latest effort. Separated into sections on diagnoses, medical and surgical treatment of cataracts and glaucoma, and associated aspects of care, such as treatment for amblyopia, the book consists entirely of answers to parents’ frequently asked and important questions. Parents of children with complicated diseases generally fall into three categories. A few want minimal information (“Just tell us what to do, Doc”). Most families desire a good explanation, ask some questions, and then proceed with treatment as recommended. On the other end of the spectrum are families who have an intense interest in learning as much as possible about their children’s disorder. They usually ask multiple questions, frequently conduct their own Internet research, and engage in detailed discussions on planned and alternative treatments. This excellent book will prove interesting to those parents in the middle group but will primarily appeal to those in the last group. The book offers detailed information, similar to what might be found in an ophthalmology text for medical professionals unfamiliar with ophthalmology. Underlying anatomy, etiology, surgical treatments for cataracts and glaucoma, and medical treatments for glaucoma are included. Overall, the book represents an outstanding resource for families. A Family Guide is quite comprehensive. There are very good sections on associated problems, including spectacles, contact lenses, and amblyopia treatment. Because of the nature of the question-and-answer format, there is some repetition of information, but the repetition may be beneficial with such complicated issues. One of the few areas under-represented is nystagmus, which is a fairly common finding in children with cataracts and glaucoma. There is an excellent section describing the roles of residents and fellows in training, which provides clarification and reassurance to parents dealing with a frequently changing array of medical caregivers. Levin is an expert on the treatment of infantile cataracts and glaucoma. Yet he recognizes that there are often several acceptable alternative treatments for these disorders and does not impose his views regarding “correct” treatment. Overall, this is an excellent addition to our resources for parents of children with complicated ocular disorders, particularly those who are passionate about learning as much as they can. The authors and sponsoring organization deserve thanks. Gregg T. Lueder, MD Ophthalmology and Visual Sciences, Washington University School of Medicine in St. Louis
Journal of AAPOS
Voiceover transcript Time 0:10 0:34 0:46 0:52 0:58 1:21 1:42 1:48 1:53 2:03 2:15 2:27 2:37
Comment A 3½-year-old boy presented to the clinic with abnormal eye movements. He was found to have a left face turn and a small left exotropia. This video demonstrates a very large left upshot as well as a limitation to abduction and adduction. The decision was made to perform a lateral rectus recession with Y-split. The lateral rectus muscle was isolated and found to be 7 mm from the limbus; however, a stoppage was detected a few millimeters posterior to the insertion, as demonstrated here. An Apt clamp was placed on the muscle and it was disinserted and sutured. Attention was turned to the sclera, where an accessory band was noted and hooked. Care was taken to carefully dissect connective tissue from the insertion of the band. The band was found to be inserted 2 mm posterior to the original insertion. An Apt clamp was placed on the band and it was disinserted and sutured. The two structures could easily be seen to be discrete and separate. Forced duction testing was repeated and there was still felt to be restriction to abduction. A second band was visualized and hooked. An Apt clamp was placed on the band. This band was found to be 4 mm from the original insertion. It was disinserted and sutured. All 3 bands were discrete and separate. Forced duction testing was free. The 3 structures were sutured in a V-shaped pattern 17 mm from the limbus. Forced duction testing was repeated and there was no restriction to abduction or adduction. Postoperatively, the patient had improved adduction and an improvement in his over-elevation in adduction. His abduction decreased from a 1 to a 3. His anomalous head turn also resolved.
Journal of AAPOS
550.e1
Article Summary
A
3.5-year-old boy presented at the University of California Los Angeles–Olive View Hospital with abnormal eye movements present since birth. His parents noticed that his left eye always appeared smaller than his right eye and that he had a right head turn. On examination, the patient demonstrated a 5 right head turn. In forced primary position, he had an exotropia of 6D. He had 2 limitation to adduction and abduction and 13 overelevation in adduction (upshoot) in the left eye. The decision was made to perform surgery consisting of a lateral rectus recession with Y-splitting for presumed exotropic Duane syndrome with a significant upshoot. Preoperative forced duction testing revealed moderate restriction to adduction and no restriction to abduction. The surgical video demonstrates the presence of 2 accessory bands posterior to the lateral rectus muscle. All 3 structures (the lateral rectus and the 2 accessory bands) were recessed to 17 mm from the limbus in a V-shaped configuration (Figures 1 and 2). At postoperative month 1, the patient was orthotropic in primary position, and his upshoot had resolved. His adduction improved to 1 but his abduction worsened to 3. First reported in 1893 by Nussbaum,1 accessory muscles have been well described in disorders such as Duane syndrome and congenital fibrosis of the extraocular muscles.2 They should be suspected in patients with atypical patterns of restrictive strabismus, globe retraction in directions other than adduction, or in patients with severe up- or downshoots. Lueder2 described 3 types of accessory bands in his 2002 review: (1) structures arising from extraocular muscles and inserting in abnormal locations, (2) fibrous
Author affiliations: Jules Stein Eye Institute and Department of Ophthalmology, University of California, Los Angeles Grant support: NIH/NEI K23EY021762 (SLP), Knights Templar Eye Foundation (SLP), Oppenheimer Family Foundation (SLP), Research to Prevent Blindness (all authors). Submitted April 7, 2015. Revision accepted May 30, 2015. Correspondence: Federico G. Velez, MD, Jules Stein Eye Institute, 100 Stein Plaza, David Geffen School of Medicine at UCLA, Los Angles, CA 90095-7002 (email: velez@ Jsei.ucla.edu). Copyright Ó 2015 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/$36.00 http://dx.doi.org/10.1016/j.jaapos.2015.05.023
Journal of AAPOS
FIGURE 1. Final position of lateral rectus (LR) and accessory bands.
bands located beneath the rectus muscles, and (3) discrete anomalous structures in the posterior orbit. In a study of 453 strabismic subjects, Khitri and Demer3 demonstrated anomalous bands in 2.4% of subjects evaluated by magnetic resonance imaging. Histopathological evidence implicates that these bands are made up of extraocular muscle tissue4; however, pathological specimens are rare, because the bands are often difficult to access and not necessarily removed as part of routine treatment. In our case, no pathological specimen was obtained; however, the bands grossly resembled fibrous tissue with scattered muscle fibers. We theorize
FIGURE 2. Multiple separate bands could be seen in the location of the lateral rectus.
549
550
Pineles and Velez
therefore, that they are a combination of Lueder’s first and second types, that is, a fibrous band with some features of extraocular muscle. References 1. Nussbaum M. Vergleichend-anatomische Beitr€age zur Kenntnis der Augenmuskeln. Anat Anz 1893;8:208-10.
Volume 19 Number 6 / December 2015 2. Lueder GT. Anomalous orbital structures resulting in unusual strabismus. Surv Ophthalmol 2002;47:27-35. 3. Khitri MR, Demer JL. Magnetic resonance imaging of tissues compatible with supernumerary extraocular muscles. Am J Ophthalmol 2010; 150:925-31. 4. Knowlton PB, Mawn LA, Atkinson JB, Donahue SP. Strabismus resulting from an anomalous extraocular muscle in Gorlin syndrome. J AAPOS 2014;18:495-8.
Review of A Family Guide to Childhood Glaucoma and Cataracts 2014, by Alex V. Levin with Christopher Fecarotta, edited by Sharon F. Freedman, Andrea Osborn, and Ian Hubling (Shadow Fusion, 2014). This book was developed by the Pediatric Glaucoma and Cataract Family Association, organized in 1993 as a collaboration between Dr. Alex Levin and parents of children with glaucoma at the Hospital for Sick Children, Toronto. The group was expanded in 2000 to include families of children with cataracts. The organization provides information and support to families with children affected by these complicated disorders and facilitates communication between families. A Family Guide is the group’s latest effort. Separated into sections on diagnoses, medical and surgical treatment of cataracts and glaucoma, and associated aspects of care, such as treatment for amblyopia, the book consists entirely of answers to parents’ frequently asked and important questions. Parents of children with complicated diseases generally fall into three categories. A few want minimal information (“Just tell us what to do, Doc”). Most families desire a good explanation, ask some questions, and then proceed with treatment as recommended. On the other end of the spectrum are families who have an intense interest in learning as much as possible about their children’s disorder. They usually ask multiple questions, frequently conduct their own Internet research, and engage in detailed discussions on planned and alternative treatments. This excellent book will prove interesting to those parents in the middle group but will primarily appeal to those in the last group. The book offers detailed information, similar to what might be found in an ophthalmology text for medical professionals unfamiliar with ophthalmology. Underlying anatomy, etiology, surgical treatments for cataracts and glaucoma, and medical treatments for glaucoma are included. Overall, the book represents an outstanding resource for families. A Family Guide is quite comprehensive. There are very good sections on associated problems, including spectacles, contact lenses, and amblyopia treatment. Because of the nature of the question-and-answer format, there is some repetition of information, but the repetition may be beneficial with such complicated issues. One of the few areas under-represented is nystagmus, which is a fairly common finding in children with cataracts and glaucoma. There is an excellent section describing the roles of residents and fellows in training, which provides clarification and reassurance to parents dealing with a frequently changing array of medical caregivers. Levin is an expert on the treatment of infantile cataracts and glaucoma. Yet he recognizes that there are often several acceptable alternative treatments for these disorders and does not impose his views regarding “correct” treatment. Overall, this is an excellent addition to our resources for parents of children with complicated ocular disorders, particularly those who are passionate about learning as much as they can. The authors and sponsoring organization deserve thanks. Gregg T. Lueder, MD Ophthalmology and Visual Sciences, Washington University School of Medicine in St. Louis
Journal of AAPOS
Voiceover transcript Time 0:10 0:34 0:46 0:52 0:58 1:21 1:42 1:48 1:53 2:03 2:15 2:27 2:37
Comment A 3½-year-old boy presented to the clinic with abnormal eye movements. He was found to have a left face turn and a small left exotropia. This video demonstrates a very large left upshot as well as a limitation to abduction and adduction. The decision was made to perform a lateral rectus recession with Y-split. The lateral rectus muscle was isolated and found to be 7 mm from the limbus; however, a stoppage was detected a few millimeters posterior to the insertion, as demonstrated here. An Apt clamp was placed on the muscle and it was disinserted and sutured. Attention was turned to the sclera, where an accessory band was noted and hooked. Care was taken to carefully dissect connective tissue from the insertion of the band. The band was found to be inserted 2 mm posterior to the original insertion. An Apt clamp was placed on the band and it was disinserted and sutured. The two structures could easily be seen to be discrete and separate. Forced duction testing was repeated and there was still felt to be restriction to abduction. A second band was visualized and hooked. An Apt clamp was placed on the band. This band was found to be 4 mm from the original insertion. It was disinserted and sutured. All 3 bands were discrete and separate. Forced duction testing was free. The 3 structures were sutured in a V-shaped pattern 17 mm from the limbus. Forced duction testing was repeated and there was no restriction to abduction or adduction. Postoperatively, the patient had improved adduction and an improvement in his over-elevation in adduction. His abduction decreased from a 1 to a 3. His anomalous head turn also resolved.
Journal of AAPOS
550.e1