Gustavo Savino

Phenylephrine eye drops in pediatric patients undergoing ophthalmic surgery: incidence, presentation, and management of complications during general anesthesia

Phenylephrine eye drops are widely used as mydriatic agent to reach the posterior segment of the eye. In literature, many reports suggest a systemic absorption of this agent as a source of severe adverse drug reactions. Hence, we reviewed our experience with topical phenylephrine in ophthalmic surgery.

Atypical Presentation of a Case of Solitary Fibrous Tumor of the Orbit

Purpose: To describe a rare case of solitary fibrous tumor of the orbit in a child, clinically misdiagnosed as arterious hemangioma. Methods: A nine-year-old child presented a hard-elastic palpable mass located in the right inferotemporal orbit. Computed tomography, orbital pulsed-wave Doppler and internal carotid artery angiography consented diagnosis of high-vascularization mass probably related to arterious hemangioma with feeder vessel arising from a branch of ophthalmic artery. The orbital mass was surgically removed. Results: The histological examination showed mesenchimal tumor, CD34 positive, related to solitary fibrous tumor of the orbit. Conclusions: Solitary fibrous tumor of the orbit should be considered in the differential diagnosis of arterious hemangioma.

Prevalence of neurological involvement and malformative/systemic syndromes in A- and V-pattern strabismus.

Purpose: To evaluate the prevalence of neurological involvement and malformative/systemic syndromes in A- and V-patterns with respect to other kinds of infantile concomitant strabismus. Methods: A retrospective comparative study of 14,006 consecutive patients examined in the Pediatric Ophthalmology and Strabismus Unit at the Catholic University of Rome between January 2002 and February 2010 was carried out. A total of 2.72% (385) of patients presented with concomitant constant infantile strabismus associated with A- and V- patterns (study group; mean age 8 years; range: 8 months – 37 years; male 211, female 174). Another 377 consecutive patients affected by infantile concomitant strabismus without A- or V- patterns were used as controls (control group; mean age 9 years, range: 1–34 years; male 194, female 183). All patients underwent a complete ophthalmologic and orthoptic examination with ocular motility evaluation and prism cover test or the Hirschberg test in primary, up and down gaze positions, as well as cycloplegic retinoscopy. The angle of deviation was evaluated at near (33 cm) and distance fixation (6 m) with full correction of refraction. Results: Neurological involvement and malformative/systemic syndromes were observed in 30.4% of the study group and in 19.8% of patients in the control group (P < 0.001). Patients with A-pattern showed a greater prevalence of neurological impairment, hydrocephalus and meningomyelocele, while those affected by V-pattern exhibited a greater prevalence of craniosynostosis and malformative syndromes. Conclusions: Neurological involvement and malformative/systemic syndromes seem to be more evident in patients presenting with A- and V-pattern strabismus.

Amniotic membrane transplantation associated with a corneal patch in a paediatric corneal perforation.

Editor, A 9-year-old girl was admitted to our department because of sudden ocular pain and visual acuity loss caused by a corneal perforation in the right eye. The patient had suffered an episode of herpes simplex virus (HSV) stromal keratitis in the same eye 10 months earlier, which was resolved by the administration of oral acyclovir, topical trifluridine and dexamethasone. The aetiological diagnosis of HSV was based on clinical and indirect serologic immune enzymatic reactivity (indirect fluorescence assay [IFA]), which confirmed the presumed HSV keratitis reinfection. On admission, the patient referred to no history of corneal symptoms or eye trauma since the previous keratitis episode. Visual acuity was counting fingers at 10 cm and ocular examination revealed a 4-mm paracentral ulceration with the iris incarcerated inside (Fig. 1). The anterior chamber was partially collapsed and corneal diffuse stromal inflammation with conjunctival injection was observed. Prophylactic antibiotic and antiinflammatory treatments with tobramycin eyedrops six times per day, cyclopentolate 1% eyedrops once per day and oral amoxicillin suspension twice per day (50 mg ⁄kg ⁄day) were started immediately. Penetrating keratoplasty (PK) under general anaesthesia was scheduled 1 day after admission to the hospital. Viscoelastic materials were injected to deepen the anterior chamber and to prevent peripheral anterior synechiae, via a temporal corneal paracentesis. The original operation plan was changed and a corneal circular human patch (diameter: 3 mm) was anchored with eight 100 nylon interrupted sutures to fill up the perforated area. A large, monolayer amniotic membrane (AM) graft was also put in place, with its epithelial side up to cover and protect the corneal patch (Fig. 2). After surgery, topical tobramycin and artificial tears were administrated four times per day for 8 weeks and oral acyclovir (20 mg ⁄kg ⁄day) was used for 3 months. The patient was followed up for 10 months. The AM dissolved at 1 month and corneal suture removal was performed at 5 months to reduce limbal neovascularization (Fig. 3). At the final ophthalmological examination (at 10 months), corneal transparency as a result of the epithelialization of the corneal graft and the stability of the corneal stromal thickness was observed. The patient’s best corrected visual acuity was 18 ⁄ 20 and no recurrence of HSV corneal disease was observed. Herpes simplex virus stromal keratitis may interfere with vision and predispose to amblyopia in cases of recurrent herpetic infection in childhood. However, only a few case series of HSV keratitis in children have been reported in the literature and the occurrence of corneal perforation is a rare but invalidating complication which may be related to an excessive inflammatory response and recurrence of the disease (Chong et al. 2004). Multilayered AM transplantation or PK may be performed for the

Restrictive Limitation of Sursumduction Caused by an Anomalous Muscular Structure

We report a peculiar case of congenitally restricted sursumduction caused by an anomalous band-like structure within the right orbit which was completely distinct from the extraocular muscles. Imaging (static and dynamic ultrasound studies of the orbit and globe, CT and MRI) revealed the origin of the anomalous structure in Zinn’s ring, its intraconal course and bulbar insertion. The ultrasound characteristics were similar to those of the extraocular muscles and optic nerve, and contractile activity appeared to occur during eye movements. The seemingly muscular nature of this structure and its total autonomy with respect to the normal extraocular muscles distinguish this case from previously reported cases of congenital restrictive strabismus.

Corneal Topographic Changes After Eyelid Ptosis Surgery

Purpose: To evaluate the corneal topography and the topographic changes after ptosis surgery on patients affected by congenital and acquired blepharoptosis. Methods: Twenty eyes of 17 patients affected by acquired and congenital ptosis underwent surgical correction through anterior levator complex tightening. Computerized tomography (Syrius Sistem; CSO) was used to analyze any change in corneal astigmatism (CYL), simulated keratometry, anterior corneal symmetry index front, apical keratometry front, and central corneal thickness. Visual acuity, margin reflex distance, and levator function were also measured. Results: After surgical ptosis repair, corneal topography demonstrated a reduction in average keratometry of 0.15 ± 0.47 diopters (D) and in corneal astigmatism of 0.26 ± 1.12 D. Significant differences were found in apical keratometry front (−1.84 ± 1.76 D) and in best-corrected visual acuity (−0.18 ± 0.06 logMAR) in the postoperative examinations. Central corneal thickness did not show significant differences between preoperative and postoperative examinations. Postoperative topographic maps showed a reduction of symmetry index front (0.10 ± 0.64 D). Conclusions: Eyelid ptosis modifies anterior corneal surface inducing refractive errors and modifying corneal astigmatism in patients, thus affecting the quality of vision. The surgical correction of blepharoptosis induces anterior corneal surface modification, restoring corneal symmetry and regular corneal astigmatism. Postoperative corneal topography showed normal corneal contours.

Mydriasis induced by sub-Tenon's ropivacaine injection in patients undergoing strabismus surgery

PURPOSE To investigate the effects of sub-Tenons capsule ropivacaine injection on pupillary diameter in strabismus surgery. METHODS A total of 16 patients were randomly assigned to 2 groups. The ropivacaine group (R) received sub-Tenons ropivacaine 1% (2.5-3 mL) injections. In the control group (C), general anesthesia was induced and a saline solution (2.5-3 mL) was injected in the sub-Tenons capsule. Pupillary diameter was measured at baseline and 2 and 5 minutes from the injections. The effects of the injections were expressed as a ratio of final (5 minutes) versus basal diameter. RESULTS A significant mydriasis occurred in the ropivacaine group when compared with the control group (Group R vs Group C ratio = 2.32, p < 0.0001) or with unoperated fellow eye (R vs unoperated eye ratio = 2.29, p < 0.0001). CONCLUSIONS Ropivacaine in the sub-Tenons capsule induces a mydriasis probably related to a pharmacological ciliary ganglion block.

Focal dermal hypoplasia (goltz-gorlin syndrome): A new case with a novel variant in the PORCN gene (c.1250T > C:p.F417S) and unusual spinal anomaly

Focal dermal hypoplasia (FDH; Goltz–Gorlin syndrome; OMIM 305600) is a disorder that features involvement of the skin, skeletal system, and eyes. It is caused by loss‐of‐function mutations in the PORCN gene. We report a young girl with FDH, microphthalmos associated with colobomatous orbital cyst, dural ectasia and cystic malformation of the spinal cord, and a de novo variant in PORCN. This association has not been previously reported, and based on these observations the phenotypic spectrum of FDH might be broader than previously appreciated. It would be prudent to alter the suggested surveillance for this rare disorder.

Cavernous venous malformation (cavernous hemangioma) of the orbit: current concepts and a review of the literature.

The cavernous venous malformation of the orbit, previously called cavernous hemangioma, is the most common primary orbital lesion of adults. Cavernous venous malformation occurs more often in women and typically presents in the fourth and fifth decades of life. It is a benign vascular malformation characterized by a well-defined capsule and numerous large vascular channels. The most common sign of cavernous venous malformation is progressive axial proptosis from the preferential involvement of the intraconal orbital space. Optic nerve damage and other signs of orbital pathology may be present, with a variable degree of visual impairment. The combination of ultrasound, computed tomography, and magnetic resonance imaging leads to an accurate diagnosis in the vast majority of cases. Surgical and nonsurgical treatments are required in case of symptomatic lesions, with a characteristic multidisciplinary management influencing optimal outcome. Orbitotomy represents the traditional surgical approach. Recently, the endoscopic transnasal approach to the orbital cavity has gained interest, representing a feasible and safe, less-invasive surgical technique for the management of cavernous venous malformation.

Chronic Lymphocytic Leukemia With Eyelid Involvement Responding to Alemtuzumab

distinct clinicopathologic entity associated with the Kaposi’s sarcoma-associated herpes virus. Blood 88:645-656, 1996 6. Ansari MQ, Dawson DB, Nador R, et al: Primary body cavity-based AIDSrelated lymphomas. Am J Clin Pathol 105:221-229, 1996 7. Boulanger E, Gérard L, Gabarre J, et al: Prognostic factors and outcome of human herpes virus 8–associated primary effusion lymphoma in patients with AIDS. J Clin Oncol 23:4372-4380, 2005 8. Waddington TW, Aboulafia DM: Failure to eradicate AIDS-associated primary effusion lymphoma with high-dose chemotherapy and autologous stem cell reinfusion: Case report and literature review. AIDS Patients and STDs 18:67-73, 2004