Abstract
Purpose
To report the incidence, demographics, and associations of various conditions among patients with pediatric ptosis.
Methods
A retrospective, observational case–control study of patients (birth–18 years; n = 2,408) diagnosed with pediatric ptosis in 1/2003–12/2012. Age- and gender-matched control patients (n = 9,632) were randomly selected from the district members. Medical and socio-demographic information were extracted from electronic medical records.
Results
The average age of diagnosis was 5.6 years, and 1,325 (54 %) were male, with an incidence of 19.9/100,000. Systemic conditions significantly associated with pediatric ptosis include myasthenia gravis, congenital anomalies, deafness, mental retardation, muscular dystrophy, neurological diseases, epilepsy, schizophrenia, and malignancy.
Ophthalmic conditions associated with pediatric ptosis include exotropia, progressive external ophthalmoplegia, hypotropia, esotropia, hyperopia, vertical heterophoria, intermitent esotropia, astigmatism, retinopathy, internuclear ophthalmoplegia, and myopia. Symptoms of diplopia, blurred vision, and aniseikonia were significantly more common.
Conclusion
Pediatric ptosis is associated with various systemic and ophthalmic conditions, and many are diagnosed after the age of 5 years. Clinicians should maintain a high degree of suspicion and thoroughly evaluate all patients with pediatric ptosis to properly assess underlying systemic associations. A better understanding of the patho-physiological association between these factors and pediatric ptosis may help its prevention and treatment.
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References
Griepentrog GJ, Diehl NN, Mohney BG (2011) Incidence and demographics of childhood ptosis. Ophthalmology 118:1180–1183
Berry-Brincat A, Willshaw H (2009) Paediatric blepharoptosis: a 10-year review. Eye (Lond) 23:1554–1559
McMullan TF, Robinson DO, Tyers AG (2006) Towards an understanding of congenital ptosis. Orbit 25:179–184
Lin LK, Uzcategui N, Chang EL (2008) Effect of surgical correction of congenital ptosis on amblyopia. Ophthal Plast Reconstr Surg 24:434–436
Pavone P, Barbagallo M, Parano E, Pavone L, Souayah N, Trifiletti RR (2005) Clinical heterogeneity in familial congenital ptosis: analysis of fourteen cases in one family over five generations. Pediatr Neurol 33:251–254
Demirci H, Frueh BR, Nelson CC (2010) Marcus Gunn jaw-winking synkinesis: clinical features and management. Ophthalmology 117:1447–1452
Harrad RA, Shuttleworth GN (2000) Superior rectus-levator synkinesis: a previously unrecognized cause of failure of ptosis surgery. Ophthalmology 107:1975–1981
Pradhan M, Hart R, Vincent A (2009) Concordance of congenital ptosis in monozygotic twins. Clin Exp Ophthalmol 37:747–748
Vestal KP, Seiff SR, Lahey JM (1990) Congenital ptosis in monozygotic twins. Ophthal Plast Reconstr Surg 6:265–268
Baldwin HC, Manners RM (2002) Congenital blepharoptosis: a literature review of the histology of levator palpebrae superioris muscle. Ophthal Plast Reconstr Surg 18:301–307
Stackhouse JR, Escaravage GK Jr, Dutton JJ (2009) Monozygotic twins with incompletely concordant simple congenital ptosis in a 4-generation pedigree. Ophthal Plast Reconstr Surg 25:493–494
Lee MJ, Oh JY, Choung HK, Kim NJ, Sung MS, Khwarg SI (2009) Frontalis sling operation using silicone rod compared with preserved fascia lata for congenital ptosis a three-year follow-up study. Ophthalmology 116:123–129
Sieb JP, Kraner S, Steinlein OK (2002) Congenital myasthenic syndromes. Semin Pediatr Neurol 9:108–119
VanderPluym J, Vajsar J, Jacob FD, Mah JK, Grenier D, Kolski H (2013) Clinical characteristics of pediatric myasthenia: a surveillance study. Pediatrics 132:e939–e944
Heidary G, Engle EC, Hunter DG (2008) Congenital fibrosis of the extraocular muscles. Semin Ophthalmol 23:3–8
Biousse V, Newman NJ (2000) Third nerve palsies. Semin Neurol 20:55–74
Mirzai H, Baser EF (2006) Congenital Horner’s syndrome and the usefulness of the apraclonidine test in its diagnosis. Indian J Ophthalmol 54:197–199
Gusek-Schneider GC, Martus P (2000) Stimulus deprivation amblyopia in human congenital ptosis: a study of 100 patients. Strabismus 8:261–270
Anderson RL, Baumgartner SA (1980) Amblyopia in ptosis. Arch Ophthalmol 98:1068–1069
Iljin A, Loba A, Omulecki W, Zielinski A (2003) Congenital blepharoptosis: Part I. Evaluation of the results of surgical treatment for congenital blepharoptosis. Acta Chir Plast 45:8–12
Srinagesh V, Simon JW, Meyer DR, Zobal-Ratner J (2011) The association of refractive error, strabismus, and amblyopia with congenital ptosis. J AAPOS 15:541–544
Gusek-Schneider GC (2002) Congenital ptosis: amblyogenic refractive errors, amblyopia, manifest strabismus and stereopsis related to the types of ptosis. Data on 77 patients and review of the literature. Klin Monatsbl Augenheilkd 219:340–348
Merriam WW, Ellis FD, Helveston EM (1980) Congenital blepharoptosis, anisometropia, and amblyopia. Am J Ophthalmol 89:401–407
Skaat A, Fabian D, Spierer A, Rosen N, Rosner M, Ben Simon GJ (2013) Congenital ptosis repair-surgical, cosmetic, and functional outcome: a report of 162 cases. Can J Ophthalmol 48:93–98
Allen CE, Rubin PA (2008) Blepharophimosis-ptosis-epicanthus inversus syndrome (BPES): clinical manifestation and treatment. Int Ophthalmol Clin 48:15–23
Mrabet A, Oueslati S, Gazzah H, Ben Hamida M (1991) Clinical and electrophysiological study of 2 familial cases of Marcus Gunn phenomenon. Rev Neurol (Paris) 147:215–219
Carlow TJ, Depper MH, Orrison WW Jr (1998) MR of extraocular muscles in chronic progressive external ophthalmoplegia. AJNR Am J Neuroradiol 19:95–99
Hageman G, Ippel PF, te Nijenhuis FC (1992) Autosomal dominant congenital Horner’s syndrome in a Dutch family. J Neurol Neurosurg Psychiatry 55:28–30
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The authors have no financial interest in any of the materials used in this study. No conflicting relationship exists for any author.
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Design and conduct of the study (YAN); data collection, management, analysis, and interpretation (SV); preparation, review, and approval of the manuscript (YAN): literature search and critical review. (YAN).
Dr Arie Nemet had full access to all study data, and takes responsibility for the integrity of the data and the accuracy of the data analysis. The manuscript was edited by Faye Schreiber, MS.
Both authors have read and approved the manuscript.
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Nemet, A.Y., Segal, O., Mimouni, M. et al. Associated morbidity of pediatric ptosis — a large, community based case–control study. Graefes Arch Clin Exp Ophthalmol 252, 1509–1514 (2014). https://doi.org/10.1007/s00417-014-2759-3
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DOI: https://doi.org/10.1007/s00417-014-2759-3