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Effects of extraocular muscle surgery in children with monocular blindness and bilateral nystagmus
BACKGROUND: Monocular infantile blindness may be associated with bilateral horizontal nystagmus, a subtype of fusion maldevelopment nystagmus syndrome (FMNS). Patients often adopt a significant anomalous head posture (AHP) towards the fixing eye in order to dampen the nystagmus. This clinical entity...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2014
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4255448/ https://www.ncbi.nlm.nih.gov/pubmed/25413480 http://dx.doi.org/10.1186/1471-2415-14-137 |
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author | Sturm, Veit Hejcmanova, Marketa Landau, Klara |
author_facet | Sturm, Veit Hejcmanova, Marketa Landau, Klara |
author_sort | Sturm, Veit |
collection | PubMed |
description | BACKGROUND: Monocular infantile blindness may be associated with bilateral horizontal nystagmus, a subtype of fusion maldevelopment nystagmus syndrome (FMNS). Patients often adopt a significant anomalous head posture (AHP) towards the fixing eye in order to dampen the nystagmus. This clinical entity has also been reported as unilateral Ciancia syndrome. The aim of the study was to ascertain the clinical features and surgical outcome of patients with FMNS with infantile unilateral visual loss. METHODS: In this retrospective case series, nine consecutive patients with FMNS with infantile unilateral visual loss underwent strabismus surgery to correct an AHP and/or improve ocular alignment. Outcome measures included amount of AHP and deviation at last follow-up. RESULTS: Eye muscle surgery according to the principles of Kestenbaum resulted in a marked reduction or elimination of the AHP. On average, a reduction of AHP of 1.3°/mm was achieved by predominantly performing combined horizontal recess-resect surgery in the intact eye. In cases of existing esotropia (ET) this procedure also markedly reduced the angle of deviation. A dosage calculation of 3 prism diopters/mm was established. CONCLUSIONS: We advocate a tailored surgical approach in FMNS with infantile unilateral visual loss. In typical patients who adopt a significant AHP accompanied by a large ET, we suggest an initial combined recess-resect surgery in the intact eye. This procedure regularly led to a marked reduction of the head turn and ET. In patients without significant strabismus, a full Kestenbaum procedure was successful, while ET in a patient with a minor AHP was corrected by performing a bimedial recession. |
format | Online Article Text |
id | pubmed-4255448 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2014 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-42554482014-12-05 Effects of extraocular muscle surgery in children with monocular blindness and bilateral nystagmus Sturm, Veit Hejcmanova, Marketa Landau, Klara BMC Ophthalmol Research Article BACKGROUND: Monocular infantile blindness may be associated with bilateral horizontal nystagmus, a subtype of fusion maldevelopment nystagmus syndrome (FMNS). Patients often adopt a significant anomalous head posture (AHP) towards the fixing eye in order to dampen the nystagmus. This clinical entity has also been reported as unilateral Ciancia syndrome. The aim of the study was to ascertain the clinical features and surgical outcome of patients with FMNS with infantile unilateral visual loss. METHODS: In this retrospective case series, nine consecutive patients with FMNS with infantile unilateral visual loss underwent strabismus surgery to correct an AHP and/or improve ocular alignment. Outcome measures included amount of AHP and deviation at last follow-up. RESULTS: Eye muscle surgery according to the principles of Kestenbaum resulted in a marked reduction or elimination of the AHP. On average, a reduction of AHP of 1.3°/mm was achieved by predominantly performing combined horizontal recess-resect surgery in the intact eye. In cases of existing esotropia (ET) this procedure also markedly reduced the angle of deviation. A dosage calculation of 3 prism diopters/mm was established. CONCLUSIONS: We advocate a tailored surgical approach in FMNS with infantile unilateral visual loss. In typical patients who adopt a significant AHP accompanied by a large ET, we suggest an initial combined recess-resect surgery in the intact eye. This procedure regularly led to a marked reduction of the head turn and ET. In patients without significant strabismus, a full Kestenbaum procedure was successful, while ET in a patient with a minor AHP was corrected by performing a bimedial recession. BioMed Central 2014-11-20 /pmc/articles/PMC4255448/ /pubmed/25413480 http://dx.doi.org/10.1186/1471-2415-14-137 Text en © Sturm et al.; licensee BioMed Central Ltd. 2014 This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. |
spellingShingle | Research Article Sturm, Veit Hejcmanova, Marketa Landau, Klara Effects of extraocular muscle surgery in children with monocular blindness and bilateral nystagmus |
title | Effects of extraocular muscle surgery in children with monocular blindness and bilateral nystagmus |
title_full | Effects of extraocular muscle surgery in children with monocular blindness and bilateral nystagmus |
title_fullStr | Effects of extraocular muscle surgery in children with monocular blindness and bilateral nystagmus |
title_full_unstemmed | Effects of extraocular muscle surgery in children with monocular blindness and bilateral nystagmus |
title_short | Effects of extraocular muscle surgery in children with monocular blindness and bilateral nystagmus |
title_sort | effects of extraocular muscle surgery in children with monocular blindness and bilateral nystagmus |
topic | Research Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4255448/ https://www.ncbi.nlm.nih.gov/pubmed/25413480 http://dx.doi.org/10.1186/1471-2415-14-137 |
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