Idiopathic Orbital Pseudotumor Preceding Systemic Inflammatory Disease in Children.

PURPOSE: To describe four pediatric cases in which isolated orbital pseudotumor preceded the development of a systemic inflammatory disease by months to years. METHODS: The medical records of all patients with the clinical diagnosis of orbital pseudotumor seen at the Ocular Oncology Service of Wills Eye Hospital and Northern Virginia Ophthalmology Associates from 2010 to 2015 were reviewed retrospectively, and those associated with systemic inflammatory disease were selected for further study. Data were retrospectively collected from medical record review regarding patient demographics and clinical features, time to development of systemic inflammatory disease, and medical management. RESULTS: In four pediatric patients, isolated orbital pseudotumor preceded the development of a systemic inflammatory disease, including pauciarticular juvenile idiopathic arthritis, eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome), granulomatosis with polyangiitis (Wegener's granulomatosis), and Crohn's disease. CONCLUSIONS: Orbital pseudotumor may be an antecedent to systemic inflammatory disease in children. Because this was a small case series, the authors are not ready to suggest that a full systemic work-up for systemic inflammatory disease is warranted for every patient with orbital pseudotumor. However, close observation and suspicion for systemic inflammatory conditions may be reasonable in children who present with idiopathic orbital pseudotumor. [J Pediatr Ophthalmol Strabismus. 2019;56(6):373-377.].

Considerations on the etiology of congenital Brown syndrome.

PURPOSE OF REVIEW: Brown syndrome is an ocular motility disorder characterized by limited volitional and passive elevation of the eye in adduction. Although originally thought due to abnormalities in the trochlea or tendon sheath (limiting the free movement of the tendon through the trochlea), recent evidence suggests that some cases of congenital Brown syndrome may be related to neurodevelopmental abnormalities of the extraocular muscles (congenital cranial dysinnervation disorders, CCDD). RECENT FINDINGS: CCDD is a term encompassing congenital abnormalities of eye movements caused by congenital innervational abnormalities. The abnormal development of cranial nerve nuclei or abnormalities in cranial nerve axonal transport affects the development of the extraocular muscle(s). Currently, congenital fibrosis of the extraocular muscles, Duane syndrome, Moebius syndrome, Horizontal gaze palsy and progressive scoliosis, and synergistic divergence are included as CCDDs. In addition, congenial ptosis, Jaw Wink ptosis, and congenital superior oblique palsy are also included. Recently, it has been suggested that some cases of congenital Brown syndrome and congenital superior oblique paresis are related, and these entities may be part of the CCDDs spectrum. SUMMARY: Important findings regarding the cause of congenital Brown syndrome will be reviewed.

Possible association of congenital Brown syndrome with congenital cranial dysinnervation disorders.

BACKGROUND: Congenital cranial dysinnervation disorders (CCDDs) are known to arise from abnormal development of individual and multiple cranial nerve nuclei or abnormalities in cranial nerve axonal transport. We report our findings for several patients with Brown syndrome in association with other known abnormalities characteristic of CCDDs. METHODS: The medical records of patients presenting during a 4-year period with congenital Brown syndrome were retrospectively reviewed. Patients with Brown syndrome confirmed by forced ductions were included in the study if the Brown syndrome was associated with either an abnormal development of the superior oblique muscle or superior oblique paresis, ptosis, Duane syndrome, or other known CCDDs. RESULTS: A total of 9 patients with Brown syndrome were identified. Of these, 3 also demonstrated a contralateral superior oblique palsy; 2, a contralateral Duane syndrome; 1, an ipsilateral congenital ptosis; and 3, a moderate to severely hypoplastic ipsilateral superior oblique muscle. CONCLUSIONS: Some patients with congenital Brown syndrome are associated with and possibly in the spectrum of CCDDs. We propose that Brown syndrome may be due to abnormal development of the trochlear nerve, which results in physical changes in the superior oblique muscle-tendon-trochlea complex. This results in a tendon that is either long and lax, absent, or abnormally inserted (ie, superior oblique paresis) or a tendon that is restricted in its movements through the trochlea (Brown syndrome).

Recurrent bleeding following traumatic hyphema due to mild hemophilia B (Christmas disease).

Traumatic hyphemas generally resolve spontaneously. When recurrent bleeding occurs, a coagulopathy should be suspected. We present a unique case of a traumatic hyphema with recurrent bleeding in association with mild hemophilia B (factor IX deficiency).

Linear nondisplaced orbital fractures with muscle entrapment.

PURPOSE: Linear nondisplaced orbital floor fractures with muscle entrapment occur in the pediatric population. These fractures occur with minimal trauma and few external signs of injury. This study reviews the clinical findings, radiologic findings and interpretations, preoperative and postoperative ocular motility, and outcomes in this subset of orbital fracture patients treated with early surgical repair. METHODS: Review of 12 children with linear orbital floor fractures with inferior rectus muscle entrapment, who underwent surgical repair by a single surgeon. RESULTS: All affected eyes demonstrated significant limitation to elevation and, in seven, depression preoperatively. All 12 patients were operated within 4 days of injury. Radiologist interpretation of computed tomographic (CT) findings recognized fracture in 9 of 11 cases in which a dictated report was available. The radiology report correctly identified muscle entrapment in only three cases and was equivocal in three other cases. The ophthalmologist, based on clinical examination and observation of the CT images, correctly identified findings consistent with linear orbital fracture with muscle entrapment in every case. Surgical findings included a nondisplaced linear floor fracture with muscle entrapment. In the early postoperative period limited elevation was present in 10 patients and limited depression was present in 7. Duction deficits and diplopia resolved in 4 days to 5 months. CONCLUSIONS: Despite prompt surgical repair, limited elevation and depression occur in the early postoperative period, possibly due to muscle edema, hemorrhage, and ischemia. Recovery of normal ocular motility may take weeks or months. External signs of injury may be minimal and radiologic interpretation may not recognize fracture or muscle entrapment.

Neonatal candidiasis: ophthalmologic infection.

Endophthalmitis results from hematogenous seeding of the eye in preterm infants with candidiasis. Early systemic therapy decreases the frequency of eye involvement, now only about 6% in infants with systemic candidiasis. Eye disease can occur on the first day, but is more likely with prolonged candidemia. Candida albicans and C tropicalis predominate in newborns. Indirect ophthalmoscopy will identify the characteristic unilateral or bilateral, yellow-white, fluffy, retinal or vitreal balls with frequent hemorrhage or inflammatory vitreous haze. Systemic antifungal therapy alone usually results in good visual outcome, but unresponsive or recurrent lesions are reported. Lens abscesses presenting as cataracts may be seeded before regression of the tunica vasculosa lentis, leaving an infection nidus in a subsequently avascular structure that is poorly reached by antifungal drugs. Lensectomy may be required to clear the visual field and the infection. Candidemia is also implicated in progression of retinopathy of prematurity to stage 3 or beyond, possibly due to induction of cytokines or angiogenic factors.

Management of pediatric cataract and lens opacities.

The diagnosis and surgical management of cataracts in children has benefitted from improvements in microsurgical techniques. Intraocular lens placement has become standard in children 2 years of age and older. Initial experience with intraocular lens implantation in infants found a high rate of surgical complications. However, refinements in surgical techniques may lesson these complications. Posterior capsule opacification is a frequent postoperative problem after pediatric cataract surgery. Posterior continuous curvilinear capsulorhexis with anterior vitrectomy is an effective means to prevent opacification of the visual axis in children.