Neuro-ophthalmic Complications in Pediatric Leukemia.

BACKGROUND: Optic neuropathy in childhood leukemia occurs through multiple direct and indirect mechanisms, including leukemic infiltration of the optic nerve, infection, blood dyscrasias, or adverse effects of treatment. We aimed to characterize visual outcomes in pediatric patients with leukemia-associated neuro-ophthalmic manifestations. METHODS: We retrospectively identified patients with leukemia and optic nerve pathology over 13 years by diagnostic billing codes. We collected information on demographics, presentation, treatment course, and visual outcomes directly from medical records. RESULTS: Of the 19 patients who met inclusion criteria, 17 (89.5%) had pseudotumor cerebri and 2 had direct optic nerve infiltration. Causes of increased intracranial pressure included central nervous system infiltration (6 of 17), hyperviscosity/leukemia (2 of 17), venous sinus thrombosis (3 of 17), medication induced (5 of 17), and bacterial meningitis (1 of 17). 47.1% (8 of 17) had papilledema at the time of leukemia diagnosis, and 94.1% (16 of 17) of patients with pseudotumor cerebri were treated with acetazolamide. At presentation, 3 patients had decreased vision secondary to macular ischemia, subhyaloid vitreous hemorrhage, or steroid induced glaucoma. Following treatment of pseudotumor cerebri, binocular visual acuity was ≥20/25 in all patients. One patient with optic nerve infiltration had a final visual acuity of count fingers in the affected eye. CONCLUSIONS: In our chart review, the most common mechanism of neuro-ophthalmic involvement in pediatric leukemia was elevated intracranial pressure from a myriad of causes. Visual outcomes from patients with elevated intracranial pressure were excellent. Understanding the mechanisms by which leukemia can cause optic nerve disease in pediatric patients can facilitate earlier diagnosis and treatment and potentially improve visual outcomes.

Disseminated Tuberculosis in a Healthy Adolescent Female.

Disseminated tuberculosis (TB) is an uncommon sequela of Mycobacterium TB infection in which bacteria disseminate and form colonies outside of the lungs. Most reports of disseminated TB are found in immunocompromised patients, particularly in patients with human immunodeficiency virus (HIV) infection, malnutrition, genetic susceptibility, diabetes mellitus, smoking, and alcohol abuse. Few case reports describe the finding of disseminated TB in immunocompetent patients, especially in healthy adolescents. The purpose of this report is to present a case in which disseminated TB was found in an otherwise healthy adolescent, female patient, and to explore the effects of pubertal hormonal changes on the immune system. Several studies in the pediatric population have suggested that hormonal changes of estrogen and testosterone associated with puberty may influence the pathogenesis of active TB. While the exact pathogenesis of disseminated TB remains unknown, this case highlights the need for heightened awareness of TB among otherwise healthy adolescents, and specifically, the effect puberty has on disease progression.

Postoperative Correction and Drift After Vertical Rectus Muscle Transposition for Total Sixth Cranial Nerve Palsy.

PURPOSE: To determine the magnitude of change between the preoperative and postoperative alignment and amount of postoperative drift for two vertical rectus muscle transpositions (VRTs). METHODS: Retrospective review of medical records of patients with total sixth cranial nerve palsy who underwent VRT procedures. The primary outcome measure was the magnitude of esotropia in prism diopters (PD) at the preoperative and postoperative visits. RESULTS: Twenty-seven patients were included. Sixteen had full tendon transposition with Foster augmentation (FTT+FA) and 11 had partial tendon transposition with resection and simultaneous medial rectus recession (PTT+R+MRR). A larger correction was obtained with PTT+R+MRR (mean ± standard deviation [SD]: 52 ± 19 PD; range: 27 to 87 PD) when compared to FTT+FA (mean: 40 ± 13 PD; range: 15 to 68 PD). At postoperative month 2, a greater esotropic drift was noted in the PTT+R+MRR group (16 PD) than the FTT+FA group (6 PD). Although the difference in the amount of correction was not statistically significant (P = .071), the difference in the amount of drift was statistically significant (P = .009). CONCLUSIONS: There was a trend toward greater correction with PTT+R+MRR than FTT+ FA, but it was not statistically significant. FTT+FA had significantly less postoperative drift than PTT+R+MRR. The results suggest that a small immediate postoperative overcorrection may be desirable in some VRT procedures. [J Pediatr Ophthalmol Strabismus. 2019;56(4):238-242.].

Microcornea, posterior megalolenticonus, persistent fetal vasculature, chorioretinal coloboma (MPPC) syndrome: Case series post vitrectomy.

PURPOSE: MPPC syndrome has been described as a syndrome that presents with chorioretinal coloboma, posterior megalolenticonus, persistent fetal vasculature, and chorioretinal coloboma. The purpose of our study is to report three patients who present with a variation of MPPC syndrome who each underwent pars plana vitrectomy, pars plana lensectomy, and amblyopic management. Clinical characteristics, ancillary test findings, and post-surgical functional results are compared to what is reported in the literature. METHODS: Retrospective review of medical records of patients who presented with microcornea, persistent fetal vasculature, chorioretinal coloboma, and microphthalmia who underwent surgical correction at Bascom Palmer Eye Institute. RESULTS: 3 patients (6 eyes) were included, two males and one female. All patients were born full term, vaginally, and had no family history of genetic abnormalities affecting the eye. All patients had color fundus images, fluorescein angiography (FA) and echography. Four eyes underwent surgery. Following surgical intervention, patients demonstrated improved visual acuity, and improved functional status. CONCLUSION AND IMPORTANCE: Patients with microcornea, PFV, chorioretinal coloboma, and microphthalmos, can benefit from surgical intervention when functional decline or media opacities are noted. Though difficult to assess accurate visual acuity and visual improvement pre-operatively and post-operatively, it is evident that our patients demonstrated improvement in functionality and vision following surgical intervention.

Strabismus surgery outcomes without removal of scleral buckle in patients with previous retinal detachment repair.

PURPOSE: To report the motor and sensory outcomes of strabismus surgery following scleral buckle procedure for retinal detachment (RD) without removal of the scleral buckle. METHODS: The medical records of patients who underwent strabismus surgery without removal of scleral buckle following RD surgical repair at a tertiary referral center between 2002 and 2015 were reviewed retrospectively. Demographic data were recorded, and rates of surgical motor success (defined as horizontal deviation of ≤10Δ and vertical deviation of ≤4Δ) and sensory success (resolution of diplopia) were calculated. RESULTS: A total of 23 patients (mean age, 58.4 ± 24.4 years; 12 males) were included. The average time between the RD surgery and onset of strabismus was 11.05 ± 10.95 months (range, 1-42 months). The strabismus was horizontal in 6 patients, vertical in 2, and combined in 15. Eighteen patients (78%) presented with diplopia. Adjustable sutures were used in 18 patients. Final motor surgical success was achieved in 17 of 23 patients (74%), and diplopia improved in 17 of 18 patients (94%) who had preoperative fusional capability. There was no statistically significant difference in age, number of RD surgeries, macular status, time to strabismus surgery, visual acuity in the worse eye, or magnitude of preoperative horizontal and vertical deviation with regard to motor success rate and with persistence of diplopia postoperatively. CONCLUSIONS: In our study cohort, strabismus surgery without removal of the scleral buckle resulted in motor success and alleviated diplopia in the majority of patients with prior RD repair.