Split-Tendon Medial Transposition of Lateral Rectus for Pediatric Complete Oculomotor Palsy.

BACKGROUND: Split-tendon medial transposition of lateral rectus (STMTLR) for complete oculomotor palsy can correct large angles of exotropia in adults, but outcomes are variable, and complications are frequent. Only a few pediatric cases have been reported, and further insight is needed to assess the child's alignment outcomes and ability for postsurgical gain of function. The aim of our study is to report the outcomes of this surgical procedure in pediatric cases of complete oculomotor palsy. METHODS: A retrospective review of outcomes was conducted on 5 consecutive patients with complete oculomotor palsy treated with STMTLR by a single surgeon (V.S.S.) between 2015 and 2021 at tertiary referral centers. Primary outcome was postoperative horizontal alignment, and secondary outcome was demonstration of gain-of-function activity in the field of action of the paretic medial rectus muscle. RESULTS: Five cases of pediatric complete oculomotor palsy underwent surgical treatment with STMTLR. Subjects averaged 5.3 years old (range 10 months-16 years). Two were female. Etiologies were heterogeneous, and all presented with unilateral (n = 2) or bilateral complete oculomotor palsy with exodeviations ranging from 45 to >120 prism diopters. Two subjects had bilateral disease secondary to military tuberculosis with CNS involvement. A third subject presented iatrogenically with complete bilateral third nerve palsies secondary to removal of a nongerminomatous germ cell tumor (NGGCT) of the pineal gland. The 2 remaining subjects had monocular involvement in their right eye, 1 from compressive neuropathy after a cavernoma midbrain hemorrhage, and 1 from a congenital right oculomotor palsy. All patients were observed to have stable ocular alignment for a period of at least 6 months before surgery. Unilateral STMTLR was performed in all cases except the subject with NGGCT, in which bilateral STMTLR was performed. Measurement of alignment permanence out to 1-3 years postop resulted in an average correction of 40.83 prism diopters (range 37.5-45 prism diopters) per operated eye. Four of 5 subjects regained limited but active adduction eye movements, and the 2 unilateral cases demonstrated improved convergence. None of the subjects experienced significant complications. CONCLUSIONS: STMTLR was a safe and effective approach for the surgical correction of complete pediatric oculomotor palsy in our case series. In addition, pediatric patients may benefit from STMTLR with immediate gain-of-function activity in the transposed lateral rectus muscle, which supports the hypothesis that children have a dynamic and adaptive neuroplasticity of visual target selection that predominates established agonist/antagonist neural signaling.

Nausea and Vomiting as Initial Manifestations of Pediatric NMOSD.

Intractable nausea and vomiting are commonly attributed to gastrointestinal (GI) conditions but can sometimes be a symptom of an underlying central nervous system disease. One potentially overlooked neurologic cause of intractable nausea and vomiting that is refractory to antiemetics is area postrema syndrome (APS). APS is a condition characterized by lesions of the dorsal caudal medulla and is considered a core clinical feature of neuromyelitis optica spectrum disorder (NMOSD). APS is present in up to 30% of patients ultimately diagnosed with NMOSD and can be the first presenting symptom of NMOSD in 12% of patients, as our case illustrates. Importantly, APS is highly responsive to immunotherapy. We present the case of a 14-year-old female with a history of migraines who presented to the emergency department multiple times for persistent nausea, vomiting, and hiccups. Multiple GI diagnoses were considered until she developed additional neurologic symptoms that prompted further workup and revealed the final diagnosis of NMOSD-APS. We posit that NMOSD-APS should be considered in the differential diagnosis for patients with intractable nausea and vomiting, especially in patients with a negative GI workup result and poor response to antiemetics.

Pediatric autoimmune retinopathy and optic neuropathy: a case report and a review of the literature.

Purpose: The purpose of the study was to present a rare case of pediatric bilateral optic neuropathy and retinopathy, which was consistent with a diagnosis of autoimmune retinopathy. We also reviewed the most current literature and phenotypes associated with reported pediatric cases of autoimmune retinopathy. Design: The design of the study was a case report, with a retrospective case series literature review. Subjects: This study incorporated data from six subjects, with one presenting as an original case report and five being identified from the English-language literature published to date. Materials and methods: The materials and methods involved a descriptive analysis of fundus findings, electrophysiologic testing, serum autoantibody testing, optical coherence tomography (OCT), brain MRI scanning, and fluorescein angiography, which were performed where available. Main outcome measures: The study evaluated the clinical presentation and treatment outcomes of all subjects and followed their visual function over time. Results: All six subjects had retinal abnormalities that were documented on imaging, while five out of the six subjects had optic nerve abnormalities. Electrophysiologic testing was performed on three subjects, all of whom recorded abnormal results. An underlying neoplastic disorder was described for four subjects. Serum autoantibody testing results were available for four subjects. The serum testing included using antibodies against a 22-kDa antigen, a 35-kDa optic nerve-derived antigen, a 62-kDa antigen, enolase, recoverin, tubulin, and pyruvate kinase M2. Our subject presented 12 years after resection of a ganglioglioma with asymmetric bilateral vision loss, disc edema in one eye, advanced disc pallor in the fellow eye, and bilateral subtle retinal infiltrates, despite having a normal fluorescein angiogram. OCT demonstrated asymmetric ganglion cell layer thinning, which is consistent with the vision loss. Our subject also had abnormal brain MRI findings of widespread pachymeningeal enhancement, but he had a normal cerebrospinal fluid composition. He was initially treated with high-dose pulse steroids, followed by intravenous immunoglobulin therapy. He experienced partial visual recovery in both eyes. Conclusions: Pediatric autoimmune retinopathy and optic neuropathy are rare diseases that can present with unique signs and symptoms. In pediatric patients who present with symptoms of subacute progressive vision loss with negative inflammatory workups, a history of prior neoplasm, and/or clinical findings of progressive retinopathy or optic neuropathy, an autoimmune process should be considered in the differential.

Pearls & Oy-sters: Leber Hereditary Optic Neuropathy-Plus Masquerading as Neuromyelitis Optica Spectrum Disorder in a 2-Year-Old Child.

"Leber hereditary optic neuropathy (LHON-Plus)" is a phenotype of LHON that is characterized by extraocular neurologic manifestations, which may be the first manifestations of the disease.

A case of presumed monostotic sphenoid fibrous dysplasia and abducens nerve palsy.

Monostotic fibrous dysplasia is a rare osteodystrophy that typically affects adolescents, manifests later in life, and may be associated with subclinical hormonal imbalances. It can lead to serious complications, including visual compromise. We report a case of presumed monostotic sphenoid fibrous dysplasia presenting with transient abducens nerve palsy and esotropia in an otherwise healthy infant. The mechanism of transient, noncompressive abducens nerve palsy is not clearly understood but has been theorized to be secondary to localized inflammation during a cycle of florid bone remodeling that includes cyst formation, microhemorrhage, and resorption.

Unilateral Terson-Like Syndrome in a Patient With a Perinatal Ischemic Stroke.

Terson syndrome typically presents with bilateral hemorrhagic retinopathy associated with acute intracranial bleeding. The authors present a case of neonatal hemispheric ischemic stroke with vasogenic edema and increased intracranial pressure creating a unilateral Terson-like syndrome. Magnetic resonance imaging indicated congenital occlusion of the left internal carotid artery, among other vascular abnormalities. Chronic submacular, peripheral subretinal, and vitreous hemorrhage were observed, suggesting a multilaminar hemorrhagic process resembling Terson syndrome without frank intracranial hemorrhage. The patient underwent successful lens-sparing vitrectomy of the left eye. A unilateral Terson-like syndrome can result from severe cerebral edema following neonatal stroke in the setting of multiple congenital cerebrovascular abnormalities. [Ophthalmic Surg Lasers Imaging Retina. 2020;51:592-595.].