Congenital isolated unilateral third nerve palsy in children: the diagnostic contribution of high-resolution MR imaging.

PURPOSE: To describe the neuroanatomical correlates of unilateral congenital isolated oculomotor palsy by means of high-resolution MRI. METHODS: Children with a clinical diagnosis of congenital isolated oculomotr palsy and with a high-resolution MRI acquisition targeted on the orbits and cranial nerves were selected and included in the study. An experienced pediatric neuroradiologist evaluated all the exams, assessing the integrity and morphology of extraocular muscles, oculomotor, trochlear and abducens nerves as well as optic nerves and globes. Clinical data and ophthalmologic evaluations were also collected. RESULTS: Six children (age range: 1-16 years; males: 3) were selected. All patients showed, on the affected side (left:right = 5:1), anomalies of the III nerve and extraocular muscles innervated by the pathological nerve. One patient had complete nerve agenesis, two patients showed a diffuse thinning of the nerve, from the brainstem to the orbit and 3 patients showed a distal thinning of the oculomotor nerve, starting at the level of the cavernous sinus. In all cases atrophy of corresponding muscles was noticed, but the involvement of the affected muscles varied with the nervous pattern of injury. CONCLUSIONS: High-resolution MRI represents a valuable tool for the diagnosis of III nerve anomalies in unilateral congenital IOP, showing different patterns of nerve involvement and muscular atrophy.

Reoperation in esotropic Duane retraction syndrome: Long-term motor outcome of superior rectus transposition.

AIM: The aim was to evaluate the long-term motor outcome of superior rectus transposition procedure in patients affected by unilateral esotropic Duane retraction syndrome with residual esotropia and anomalous head position. METHODS: A retrospective analysis of medical records of patients affected by esotropic Duane retraction syndrome who underwent superior rectus transposition procedure as reoperation for residual esotropia and/or residual anomalous head position. Amount of deviation, anomalous head position, duction limitation, globe retraction, presence of upshoot/downshoot, and vertical deviation were analyzed before and after superior rectus transposition procedure. RESULTS: Twenty patients were selected. All patients underwent unilateral medial rectus recession or bilateral medial rectus recession, for unilateral esotropic Duane retraction syndrome at least 2 years before superior rectus transposition reoperation. Mean age at surgery (superior rectus transposition) was 12 ± 6.8 years, and the follow-up period was 2.7 ± 0.6. Mean deviations at distance and near before surgery were 19.5 ± 5.7 and 15.2 ± 6.8, respectively. Two patients showed upshoot. Head turn was 11.4 ± 5.1°; abduction limitation was -2.6 ± 0.9. After superior rectus transposition, all patients showed an improvement of esotropia at distance and near (8.1 ± 5.7 and 5.1 ± 5.6, respectively; p < 0.05), anomalous head position (5.6 ± 3.9°; p < 0.05), and abduction limitation (-2.3 ± 0.8; p < 0.05). No statistically significant changes occurred in globe retraction. No adduction limitation, vertical deviation, and upshoot/downshoot were present after superior rectus transposition procedure. Results were stable during follow-up. CONCLUSION: Superior rectus transposition procedure is an effective procedure in esotropic Duane retraction syndrome patients who previously undergone unilateral/bilateral medial rectus recession, with residual esotropia and anomalous head position. It allows improvement of esotropia, head turn, and partial recovery of abduction in a significant percentage of patients (30%) with no vertical complications.

Orbital myeloid sarcoma (chloroma): Report of 2 cases and literature review.

PURPOSE: Myeloid sarcoma (MS) of the orbit is an uncommon condition in occurring in children, generally coupled to myeloproliferative neoplasms. OBSERVATIONS: We describe two rare cases of orbital MS in young boys with aggressive local symptoms but without evidence of acute myeloid leukemia (AML), both patients underwent orbitotomy for gross-tumor resection and biopsy. At follow up, there was no evidence of recurrence nor evolution of the myeloproliferative neoplasms clinically and by radiological and laboratory work-up. We also provide a detailed description of the magnetic resonance imaging presentation, with an extensive pathological analysis correlation. CONCLUSIONS AND IMPORTANCE: A comprehensive revision of the literature on isolated orbital MS was carried out with particular emphasis on clues for differential diagnosis and treatment options, stressing the need to consider MS even in the absence of sign and symptoms of an underlying myeloproliferative disorders.

Evaluation of 3D heads-up vitrectomy: outcomes of psychometric skills testing and surgeon satisfaction.

OBJECTIVES: To evaluate the use of a three-dimensional heads-up microscope (3DM) during 25-gauge pars plana vitrectomy (PPV) compared with a traditional ophthalmic microscope (TM) in terms of efficacy, safety, and teaching and learning satisfaction. METHODS: Prospective comparative interventional study. Fifty eyes affected by one of the following diseases: rhegmatogenous or tractional retinal detachment, epiretinal membrane, full-thickness macular hole, vitreous hemorrhage, or dropped lens. The 50 eyes were randomly assigned to one of two groups: group A (25 eyes) underwent 25-gauge PPV with 3DM, and group B (25 eyes) underwent 25-gauge PPV with TM. The main outcome measures were the duration of the operation, intraoperative complications, and surgeon and observer satisfaction. A questionnaire was used to assess surgeon satisfaction according to the following parameters: comfort, visibility, image quality, depth perception, simplicity of use, maneuverability, and teaching. A questionnaire to assess observer satisfaction was completed by 20 observers (surgical residents or ophthalmic surgeons). RESULTS: The degree of satisfaction was higher using 3DM for both surgeons and observers (P < 0.001). The average duration of the operation did not differ significantly between the two methods. No major complications occurred for either method. CONCLUSIONS: PPV with 3DM is more comfortable for the surgeon and poses no substantially greater risk of complications for the patient. The high-definition screen delivers excellent depth perception and better screen parameter control, which results in high-quality surgical performance. 3DM surgery helps to significantly improve teaching and learning intra-operative surgical procedures.

Essential infantile esotropia with inferior oblique hyperfunction: long term follow-up of 6 muscles approach.

AIM: To evaluate long term follow-up (10y) of 6 muscle surgical approach in essential infantile esotropia (EIE). METHODS: A 6 muscle approach to EIE was retrospectively evaluated in patients with inferior oblique (IO) hyperfunction and lateral rectus (LR) pseudoparalysis, who underwent surgery at different ages. Different clinical characters were analyzed pre- and postoperatively, in patients who underwent a 6 muscles approach ≤4 years of age. All patients underwent a multiple muscles approach: bilateral medial recti (MR) recession (4-5 mm), bilateral LR resection (lower than 7 mm) and bilateral IO recession and anteroposition. Of 108 children with preoperative angle ≥+30 prism diopters (PD) and IO hyperfunction were selected from larger cohort of patients (n=213, 103 females and 110 males) after excluding patients with: angle variability, who underwent reoperation and with incomplete follow up. Preoperative assessment and complete orthoptic examination were performed. Follow-up was performed 3mo, 2, 5 and 10y after surgery. Statistical analysis was performes using SAS statistical software package (version 9.1, SAS Institute Inc., Cary, NC, USA). RESULTS: Ten years follow up data analysis showed the following percentage of orthotropic patients: (0 PD): 3mo, 22.2%; 2y, 16.7%; 5y, 25.0% and 10y, 27.8%. A slight, significant (P<0.01), increase of 2y follow up residual deviation was found when compared to 3mo one. Stationary surgical results is reported during time, with a trend of mean residual deviation reduction (P=0.04). CONCLUSION: Our results confirm the reliability of multiple muscles surgical approach in the treatment of patients affected by EIE with OI hyperfunction.

Infantile esotropia: risk factors associated with reoperation.

The aim of this study was to identify clinical and demographic factors associated with misalignment after first surgery performed on children affected by infantile esotropia to evaluate the reoperation rate. A retrospective study was carried out, analyzing data from 525 children who underwent bilateral medial recti recession, bilateral lateral recti resection, and inferior oblique recession and anteroposition by the same surgeon (AM). Postoperative evaluation included assessment of motor alignment at approximately 3 months, 6 months, 1 year, and 5 years. Statistical analysis was performed with a logistical regression model in which the dependent variable was the presence/absence of reoperation. We found that late surgery (after 3 years of age) and a family history of strabismus are associated with a higher risk of reoperation, while some clinical factors, including some classically associated with worst motor outcome as preoperative angle, dissociated vertical deviation, and amblyopia, did not influence the incidence of reoperation in infantile esotropia. Male patients and patients with hyperopia in preoperative examinations have a significantly decreased reoperation rate.

Long-Term Outcomes of Primary Intraocular Lens Implantation for Unilateral Congenital Cataract.

PURPOSE: In congenital cataracts, the need for early intervention is well established to prevent visual deprivation and amblyopia. We evaluated patients with unilateral amblyogenic congenital who had undergone simultaneous cataract removal and intraocular lens implantation. METHODS: We retrospectively reviewed all patients operated on between 1990 and 2010 at the Pediatric Eye Department of the University Federico II of Naples (Italy). Minimum follow-up for inclusion was 48 months. RESULTS: 52 patients (28 females, 24 males, mean age at surgery 9.0 ± 4.5 months, range 2-21 months) were included in this retrospective review. Mean follow-up was 100.7 ± 57.0 months (range, 48-270 months). At last visit, mean BCVA in the operated eyes was 0.65 ± 0.4 (range, 0.04-1.3) LogMAR and 40 patients (76.9%) had strabismus. In patients undergoing surgery at an age >12 months, BCVA was significantly lower (0.92 ± 0.4 LogMAR versus 0.60 ± 0.4 LogMAR, p = 0.01), although development of myopic shift was less frequent (61.5% versus 43.5%, p = 0.03). BCVA at last visit was higher in patients with a first stable pseudophakic SE between +1D and +3D (p = 0.02). CONCLUSIONS: Worse final BCVA, despite less frequent development of myopic shift, was observed when surgery was performed after 12 months of age. A hyperopic correction in first stable pseudophakic SE seems advisable.

Cataract management in juvenile idiopathic arthritis: simultaneous versus secondary intraocular lens implantation.

PURPOSE: To compare primary versus secondary intraocular lens (IOL) implantation after cataract removal in patients with juvenile idiopathic arthritis (JIA). METHODS: Retrospective interventional study. Data were obtained for 40 children (40 eyes) with JIA-associated uveitis operated on for cataract before the age of 16 years between January 1998 and January 2005. RESULTS: Twenty-one patients underwent primary IOL implantation at a mean age of 13.1 ± 2.6 (9-16) years. Mean follow-up was 48.2 ± 5.4 (35-64) months. Nineteen patients underwent cataract removal at a mean age 12.3 ± 2.0 (9-16) years and secondary IOL implantation 13.6 ± 0.3 (11-16) months later. Mean follow-up was 47.2 ± 6.5 (32-64) months. BCVA difference between the two groups was significant at 24-month visit only (p = 0.001). Incidence of secondary glaucoma was significantly lower in the group that underwent secondary IOL implantation (p = 0.01). CONCLUSION: Secondary IOL implantation after cataract removal in children with JIA-related uveitis provided a significantly lower incidence of secondary glaucoma.

Long-term outcome of primary versus secondary intraocular lens implantation after simultaneous removal of bilateral congenital cataract.

BACKGROUND: Long-term outcomes of intraocular lens (IOL) implantation for congenital cataract in children under 2 years old are still undetermined. METHODS: We retrospectively reviewed all cases of bilateral congenital cataract who had undergone simultaneous bilateral cataract removal with posterior capsulotomy and central anterior vitrectomy between 1990 and 2010. Patients randomly underwent primary IOL implantation or secondary IOL implantation after a period of contact lens wear. The two groups were compared for visual outcome and complications during follow-up. RESULTS: Cataract removal and primary IOL implantation was performed in 30 eyes (15 patients; nine males, six females) at a mean age of 6.8 ± 4.2 months. After 79.31 ± 63.4 months, best-corrected visual acuity (BCVA) was 0.53 ± 0.36 EDTRS LogMAR. In 36 eyes (18 patients, 11 males, seven females) the lens was removed at a mean age of 5.42 ± 2.80 months, and after 32.0 ± 6.1 months of contact lens utilization, secondary IOL implantation was performed. After 109.0 ± 33.8 months, BCVA was 0.54 ± 0.4 ETDRS LogMAR. The association between age at surgery and final visual acuity and the difference between the two groups concerning type of cataract at baseline, BCVA and refractive error at last visit, incidence of posterior capsular opacification, glaucoma, strabismus, and nystagmus during follow-up were not significant (p > 0.05). Myopic shift was more frequent in eyes undergone primary IOL implantation (p < 0.001). CONCLUSIONS: Similar visual outcome and complications were observed during long-term follow-up after both primary and secondary IOL implantation following simultaneous bilateral congenital cataract removal with posterior capsulotomy and central anterior vitrectomy.

Bilateral methicillin-resistant Staphylococcus aureus keratitis following hyperopic photorefractive surgery.

To report clinical manifestations of a female patient with bilateral bacterial keratitis following photorefractive keratectomy (PRK). Bilateral PRK was performed for moderate hyperopia. Bandage contact lenses were fitted at the conclusion of the surgery. Bilateral infectious keratitis with hypopion was diagnosed within 4 days after surgery. Smear and culture were obtained and showed the presence of methicillin-resistant Staphylococcus aureus (MRSA). The patient was treated with systemic prednisone and topical antibiotics (vancomycin, tobramycin and netylmicin) and betamethasone. After 1 month corneal leukoma was still present and remained unchanged during the following 7 months. Infectious keratitis is a rare complication of PRK that appears early in the postoperative period. MRSA keratitis may determine long-term visual impairment despite prompt therapeutic intervention.