Horner Syndrome from a Pediatric Otolaryngology Perspective.

INTRODUCTION: Horner syndrome is described as the clinical triad of miosis, ptosis, and anhidrosis. In pediatric patients the condition may be congenital or acquired from neoplastic, infectious or traumatic conditions, including birth trauma. Most cases of pediatric Horner syndrome present first to a pediatric ophthalmologist however since the neural pathways involve the cervical sympathetic chain otolaryngologists should understand the pathophysiology to avoid delay in management of potentially malignant cases. OBJECTIVES: To aid otolaryngologists in recognizing and managing pediatric Horner syndrome by describing 3 unique cases from malignant, traumatic and/or congenital causes. METHODS: Case report of 3 pediatric patients with Horner syndrome presenting to our pediatric otolaryngology department. RESULTS: Case #1 is 5-month-old female with ptosis and a left level II 1.5 cm neck mass. Magnetic resonance imaging showed the mass displacing the common carotid artery and excisional biopsy revealed a poorly differentiated neuroblastoma. Case #2 is a 9-year-old female with anisocoria appearing after suffering a severe playground injury. Case #3 is a 3-year-old-male who developed ptosis and anisocoria following re-excision of a recurrent cervical lymphatic malformation. CONCLUSION: Pediatric Horner syndrome may be a benign finding that is easily overlooked but may reflect a serious underlying condition. Otolaryngologists should be aware of the pathophysiology and differential diagnosis, including malignant causes, to appropriately manage patients.

Optical coherence tomography to monitor vigabatrin toxicity in children.

PURPOSE: The antiepileptic drug vigabatrin is known to cause permanent loss of vision. Both visual field testing and electroretinogram are used to detect retinal damage. Adult data on optical coherence tomography (OCT) shows that retinal nerve fiber layer (RNFL) thinning may be an early indicator of vigabatrin-induced retinal toxicity. The purpose of this study was to investigate whether OCT can detect early vigabatrin-induced retinal toxicity in children. METHODS: Pediatric patients (≤18 years of age) requiring vigabatrin for seizure control who were followed at our institution were invited to participate. Patients were examined according to manufacturer guidelines, with most examinations taking place under general anesthesia. RNFL thickness was measured by OCT (Stratus Model 3000, Zeiss) and compared to total cumulative dose of vigabatrin. In most cases, indirect ophthalmoscopy, fundus photography, and electroretinography were also performed. RESULTS: OCT and complete dosing data was available for 19 patients. Patients with tuberous sclerosis (TS, n = 12) received higher cumulative doses (mean, 1463 g) than non-TS patients (mean, 351 g, P = 0.044). RNFL thinning was detected in the nasal (P < 0.01), superior (P < 0.01), and inferior (P < 0.05) quadrants in patients with TS, particularly once cumulative dose exceeded 1500 g. CONCLUSIONS: In our study population of patients with TS, higher cumulative doses of vigabatrin were associated with RNFL thinning in the nasal, superior, and inferior quadrants. These findings were pronounced once cumulative dose exceeded 1500 g. This pattern of RNFL thinning is similar to what has been shown in adult patients taking vigabatrin.

Recurrent disc hemorrhage does not increase the rate of visual field progression.

AIMS: To determine whether recurrent disc hemorrhage (DH) accelerates glaucomatous visual field (VF) loss compared to an isolated, single, detected DH. METHODS: We evaluated the disc photographs of consecutive patients with >/=5 SITA-Standard fields for DH. Group A had patients with a single DH in one eye, and group B had at least one recurrence in the same eye. Automated pointwise linear regression analysis was used to calculate rates of progression. Logistic regression was used to determine ocular or systemic variables associated with DH recurrence after baseline assessment. RESULTS: One hundred and seventeen patients were enrolled (group A = 72, group B = 45). The mean age was 67.1 +/- 10.8 years; most patients were women (65%) of European ancestry (92%) diagnosed with primary open-angle glaucoma (47%). The mean number of VF after the initial DH was 7.9 +/- 2.9, spanning a mean of 4.6 +/- 2.2 years. None of the ocular or systemic characteristics revealed a significant difference between groups. The mean global rate of progression (group A, -0.8 +/- 0.6 vs group B, -0.8 +/- 0.7 dB/year, p = 0.93) and number of eyes reaching a progression endpoint (group A, 70% vs group B, 73%, p = 0.80) did not differ between groups. Recurrent DH eyes showed a tendency to be followed longer, with a greater number of disc photographs, which was not significant in the multivariate analysis. The global rates of progression between groups remained non-significant even after adjusting to follow-up time and number of VF tests (p = 0.69). CONCLUSION: Recurrent DH does not result in a faster rate of VF progression compared to a single detected DH. Eyes with single or recurrent DH have similar risks for future disease progression.