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FIG 3. Clinical photo of patient at follow up showing left side ptosis, anisocoria, and mild heterochromia.

established for children, and it is considered acceptable to use adult sizing. Lymph nodes .1 cm in the head and neck are considered enlarged. It is recommended that persistent lymph nodes (present more than 6 weeks) of unclear origin or high suspicion for malignancy should be further evaluated. Ultrasound is the recommended initial mode of imaging, followed, if necessary, by CT and/or MRI.5 Increased patient age (.10 years), larger lymph node size (.2 cm), multiple sites of adenopathy (.2) and supraclavicular location of adenopathy are all associated with a greater risk of malignancy.6 Biopsy should be strongly considered in patients with a combination of these factors or other suspicious findings such as severe systemic symptoms. Childhood rhabdomyosarcoma, a soft tissue malignant tumor of mesenchymal origin, accounts for approximately 3.5% of the cases of cancer among children aged 0-14 years. The incidence is 4.5 per 1 million children and 50% of cases are seen in the first decade of life.7 Rhabdomyosarcoma in infants is rare, estimated at 5% in North America.8 Rhabdomyosarcoma has been reported to cause Horner syndrome by mass effect when located in the head and neck region.2 We conducted extensive imaging including PET scan to evaluate the neck for malignancy. PET scan is not only useful in identifying the anatomical location of tumor but also has high sensitivity and provides information on the malignant potential of the tumor.5,9 In conclusion, an infant presenting with Horner syndrome in the absence of birth trauma or surgical interventions should receive a complete work-up to rule out neuroblastoma. Neck masses compressing the sympathetic plexus should be evaluated by PET scan with possible biopsy to rule out malignancy. Enlarged lymph nodes causing mechanical compression of the sympathetic nerves should be included in differential diagnosis. Lastly, Horner syndrome may persist after resolution of the underlying condition, indicating permanent damage to sympathetic plexus. References 1. Smith SJ, Diehl N, Leavitt JA, Mohney BG. Incidence of pediatric Horner syndrome and the risk of neuroblastoma: a population-based study. Arch Ophthalmol 2010;128:324-9. 2. Jeffery AR, Ellis FJ, Repka MX, Buncic JR. Pediatric Horner syndrome. J AAPOS 1998;2:159-67. 3. Smith SJ, Diehl NN, Smith BD, Mohney BG. Urine catecholamine levels as diagnostic markers for neuroblastoma in a defined population: implications for ophthalmic practice. Eye (Lond) 2010;24:1792-6.

4. Mahoney NR, Liu GT, Menacker SJ, Wilson MC, Hogarty MD, Maris JM. Pediatric Horner syndrome: etiologies and roles of imaging and urine studies to detect neuroblastoma and other responsible mass lesions. Am J Ophthalmol 2006;142:651-9. 5. Nolder AR. Cervical Lymphadenopathy: when to biopsy? Current Opinion in Otolaryngology & Head and Neck Surgery 2013;21: 567-70. 6. Soldes OS, Younger JG, Hirschl RB. Predictors of malignancy in childhood peripheral lymphadenopathy. J Pediatr Surg 1999;34:1447-52. 7. National Cancer Institute: PDQÒ Childhood Rhabdomyosarcoma Treatment. Bethesda, MD: National Cancer Institute. Available at: http://cancer.gov/cancertopics/pdq/treatment/childrhabdomyosarcoma/ HealthProfessional. Accessed 06/16/2014. 8. Pias RC, Ragab AH. Rhabdomyosarcomas in infancy. In: Maurer HM, Ruymann FB, Pochedly CE, eds. Rhabdomyosarcoma and Related Tumors of Children and Adolescents. Boca Raton: CRC Press; 1991:375. 9. Kushner BH, Yeung HW, Larson SM, Kramer K, Cheung NK. Extending positron emission tomography scan utility to high-risk neuroblastoma: fluorine-18 fluorodeoxyglucose positron emission tomography as sole imaging modality in follow-up of patients. J Clin Oncol 2001;19:3397-405.

Nonsurgical treatment of cyclic esotropia Nathalie Voide, MD, Claudine Presset, CO, Georges Klainguti, MD, and Pierre-Franc¸ois Kaeser, MD Cyclic esotropia is characterized by a 24-hour period of straight eye position followed by 24 hours of large-angle esotropia. Possible mechanisms include notably progressive loss of compensation of a latent strabismus. The classic treatment is surgical correction of the angle measured on the days with manifest deviation. We report the first case of cyclic esotropia successfully treated by prismatic correction of the latent strabismus present on “straight” days.

Case Report

A

nisometropic right eye amblyopia was diagnosed in a healthy 6-year-old girl. Initial visual acuity was 20/50 in the right eye and 20/20 in the left eye left eye. A 4D distance and 2D near esophoria was present. Lang stereotest was positive and stereoacuity measured using TNO stereotest was 60 arcsec. Cycloplegic refraction was 13.75 in the right eye and 12.0 in the left

Author affiliations: Department of Ophthalmology, University of Lausanne, Jules Gonin Eye Hospital, Lausanne, Switzerland Submitted July 1, 2014. Revision accepted November 14, 2014. Published online March 28, 2015. Correspondence: Pierre-Franc¸ ois Kaeser, MD, Jules Gonin Eye Hospital, Avenue de France 15, CH-1004 Lausanne, Switzerland (email: [email protected]). J AAPOS 2015;19:196-198. Copyright Ó 2015 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/$36.00 http://dx.doi.org/10.1016/j.jaapos.2014.11.008

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Volume 19 Number 2 / April 2015 eye. Spectacles were prescribed, and left eye patching was started 6 hours a day. Three months later, the patient complained of horizontal diplopia. A 40D esotropia was present at distance and near, and the patient was referred to Jules Gonin Eye Hospital for further evaluation and possible surgery (Figure 1A,B). Ocular motility, slit-lamp, and fundus examinations were normal. Neurologic examination and magnetic resonance imaging of the brain and orbits were normal. Careful review of the patient’s history revealed that diplopia was present only every other day; a 6D distance and 10D near esophoria was present on the next day (Figure 1C,D). Lang test was positive, and stereoacuity was 60 arcsec at TNO stereotest. Cycloplegic refraction confirmed that the spectacles appropriately corrected the anisometropia. The cyclic nature of the esotropia was confirmed over a period of 1 month, as documented by the child’s parents. The cyclic pattern consisted of a 48-hour cycle throughout the month, with a large esotropia alternating daily with apparently straight ocular alignment. We prescribed a 6D base-out Fresnel press-on prism in front of the right eye to correct the distance esophoria measured on the “straight” days. This resulted in the immediate break of the cyclic rhythm and resolution of the esotropia. One month later, the eyes remained straight with the spectacles and prism, and stereoacuity was normal (Figure 1E,F). The prism was removed and no recurrence of strabismus had occurred at the latest follow-up, 24 months later (Figure 1G,H).

Discussion Cyclic strabismus is rare (1 every 3,000-5,000 strabismus cases). It is characterized by alternating periods of straight eyes and ocular large-angle deviation, most frequently esotropia.1-5 The present case is slightly unusual in that a wellcompensated latent strabismus was present during the “straight” days, with normal stereopsis. This latent strabismus was already present at the first examination, before amblyopia treatment, and thus corresponded to the “basic” binocular state of the patient. The cycle of strabismus in such cases is reported to last from 48 hours to 5 days.1,2 It most frequently occurs in association with acquired nonaccommodative esotropia, but cases associated with accommodative, congenital, or consecutive esotropia have also been reported.2,6 Cyclic strabismus tends to become permanent with time.3 The etiology of cyclic strabismus is unknown. Possible mechanisms include progressive loss of compensation of a latent strabismus, alteration of a central clock mechanism, and incomplete cerebral dominance, with interhemispheric rivalry.1-5 As in the present case, cyclic strabismus can occur during part-time occlusion.6 Adequate optical correction of ametropia does not necessarily prevent cyclic esotropia.6

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FIG 1. Clinical photographs of a 6-year-old girl with circadian esotropia. A 40D esotropia was present on day 1 with and without glasses (A-B), whereas a 6D esophoria was present on day 2 (C-D). The cyclic rhythm was broken and esotropia resolved with placement of a 6D base-out Fresnel press-on prism in front of the right eye (E-F). The prism was removed after 1 month, with no recurrence 24 months later (G-H).

Treatment of cyclic strabismus classically relies on the surgical correction of the deviation present on the strabismus day; results are generally excellent, with no overcorrection on the previously “straight” days.1-5,7 Spectacles allow fusion in rare cases, while miotics and botulinum toxin injection have been reported to be unsuccessful.2-4,7 In the present case, we elected to correct the latent deviation present on the “straight” days in order to relieve any compensation effort and to maximize the binocular balance. This resulted in an immediate break of the cyclic rhythm. A long-term resolution of the strabismus was obtained despite removal of the prism after 1 month. We interpret these results to indicate restoration of compensation and normal binocular function, supporting the theory that cyclic strabismus may be due to progressive loss of compensation, that is, an underlying tendency to strabismus. We recommend that this noninvasive treatment should be attempted first in cyclic strabismus to allow early recompensation of the binocular function in cases with latent strabismus on the “straight” days. In such cases, prism correction should thus be tried as first-line treatment, with surgery performed only in unsuccessful cases.

Literature Search A comprehensive search of PubMed, February 1964 to May 2014, was performed for a combination of the following terms: cyclic, alternate day, circadian, periodic, clock-mechanism strabismus, and esotropia.

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1. Richter CP. Clock-mechanism esotropia in children: alternate-day squint. Johns Hopkins Med J 1968;122:218-23. 2. Helveston EM. Cyclic strabismus. Am Orthopt J 1973;23:48-51. 3. Caputo AR, Greenfield PS. Cyclic esotropia. Ann Ophthalmol 1978; 10:775-8. 4. Metz HS, Jampolsky A. Alternate day esotropia. J Pediatr Ophthalmol Strabismus 1979;16:40-42.

5. Parlato CJ, Nelson LB, Harley RD. Cyclic strabismus. Ann Ophthalmol 1983;15:1126-9. 6. Kee C, Hwang J-M. Accommodative esotropia decompensated to cyclic esotropia in a 6-year-old boy. J AAPOS 2014;18:77-8. 7. Lai YH, Fredrick DR. Alteration of cyclic frequency by botulinum toxin injection in adult onset cyclic esotropia. Br J Ophthalmol 2005; 89:1540-41.

Journal of AAPOS

Nonsurgical treatment of cyclic esotropia.

Cyclic esotropia is characterized by a 24-hour period of straight eye position followed by 24 hours of large-angle esotropia. Possible mechanisms incl...
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