PubMed · 13670245
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B T SAYOC. 1959. Blepharoptosis.. https://pubmed.ncbi.nlm.nih.gov/13670245/
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BACKGROUND: Congenital fibrosis of extraocular muscles (CFEOM) is a complex strabismus syndrome that typically occurs in isolation and results from dysfunction of all or part of cranial nerves III (CNIII) and IV (CNIV) and/or the muscles that these nerves innervate. Only a few patients with CFEOM and additional central nervous system malformations have been reported. We describe four additional patients with CFEOM associated with central nervous system (CNS) abnormalities. METHODS: Four patients who presented with congenital restriction of eye movements in association with neurological abnormalities underwent complete ophthalmological examination including electroretinography (ERG) and eye movement recordings. Neurological examinations, neuroradiological studies, muscle histology, chromosomal and genetic linkage analysis were performed. RESULTS: Clinical examination and forced duction testing confirmed that all four patients met criteria for CFEOM; all had congenital restrictive ophthalmoplegia primarily affecting extraocular muscles innervated by the oculomotor nerve. Two brothers had CFEOM and Marcus Gunn jaw winking. In each of the four cases, CFEOM occurred in association with one or several neuroradiological findings, including agenesis of the corpus callosum, colpocephaly, hypoplasia of the cerebellar vermis, expansion of the ventricular system, pachygyria, encephalocele and/or hydrancephaly. CONCLUSIONS: We present four cases of CFEOM in association with CNS malformations that confirm that CFEOM can be part of a more complex neurological dysfunction and provide further support to a neurogenic aetiology for this disorder. We also describe for the first time the coexistence of CFEOM and Marcus Gunn jaw winking in two siblings. This suggests a genetic mechanism. Aberrant innervation supports primary developmental abnormality of cranial nerves in CFEOM.
Many methods have been used to make the soft tissue fixation in endoscope-assisted forehead lifting (EAFL). Since we started doing EAFL, we have employed the fixation with stainless screws and absorbable screws. After noticing that the temporary fixation with metallic screws had some negative effects, we changed to temporary fixation by bioabsorbable (polylactic acid copolymer) screws. The purpose of this study is to transmit our experience and to comment on some points that we believe will improve this method. Our study includes the endoscopic surgeries we have performed over the last four years. Ninety-nine patients were operated on. In 61 of them, absorbable screws were used. We have had very good results using these screws without important complications. We think that they offer a great improvement to endoscopic facial surgery.