Simultaneous surgery in bilateral congenital cataract.

PURPOSE: To assess the efficiency of simultaneous surgery in bilateral congenital cataract not only in patients with a high anesthesiologic risk profile. METHODS: A retrospective study was carried out on 40 bilateral congenital cataract patients (80 eyes) who presented to the outpatient department of pediatric ophthalmology of the University Federico II of Naples in the period from 1990 to 2005. All patients had undergone cataract extraction from both eyes in a single surgical session. Visual rehabilitation was achieved in all patients by corneal lenses; successively 25 patients had a secondary intraocular lens implant in the posterior chamber after a period of 2.5-3 years. RESULTS: The mean age at cataract surgery was 7 months (1-17 months). Visual acuity (VA) was assessed in 52 eyes: 8 eyes (15%) presented VA or=20/40. Ocular motility disorders were seen in 28 patients (70%). Eight patients had postoperative complications: the formation of secondary membranes in five patients, and secondary glaucoma in three patients. There were no cases of endophthalmitis. CONCLUSIONS: Simultaneous surgery in bilateral congenital cataract may be taken into consideration not only for patients with a high anesthesiologic risk profile.

Congenital cataract extraction with primary aphakia and secondary intraocular lens implantation in the posterior chamber.

PURPOSE: To evaluate visual outcomes, eye movement abnormalities, and postoperative complications after secondary posterior chamber intraocular lens (IOL) implantation in aphakic children who had initial LAC correction. METHODS: A retrospective study was carried out on 53 patients (94 eyes). Following aphakic surgery and LAC correction the patients received secondary IOL implantation in the posterior chamber. Special attention was paid to factors that may have influenced their visual outcomes, such as eye movement abnormalities, cataract unilaterality, and cataract density. RESULTS: No association was found between age at surgery and the onset of strabismus (p=0.611) or with visual acuity (p=0.086). However, unilaterality and total cataract density were found to have a negative association with poor vision (p<0.001). Strabismus and nystagmus were found to have a statistically significant negative association with visual acuity (p=0.002). Posterior capsule opacification occurred in six eyes of five patients following cataract extraction; IOL dislocation occurred in four eyes after IOL intraocular implantation, and secondary glaucoma occurred in one eye. CONCLUSIONS: IOL implantation before 12 months of age may be useful in unilateral cataract; in bilateral cataract, simultaneous surgical aphakia, LAC correction, and then IOL implantation at 2.5-3 years of age, together with anti-amblyopic therapy when strabismus or asymmetric cataract density occur, are efficient methods to obtain visual recovery. Extraocular muscle surgery may be required to correct strabismus.

Photorefractive keratectomy for myopic anisometropia: a retrospective study on 18 children.

PURPOSE: To evaluate the safety and efficacy of photorefractive keratectomy (PRK) performed under topical anesthesia in children with myopic anisometropia. METHODS: Medium to high unilateral myopia was corrected in 18 patients by PRK. At the time of surgery patients were between 7 and 17 years of age (mean 10 years). All the surgical procedures were performed under topical anesthesia. Pre- and postoperative data regarding visual acuity, eye alignment, and binocular vision were analyzed. RESULTS: The mean correction (SE) obtained with excimer laser was -8.21 D (range: -2.25 to -14.50, SD 3.90). The mean preoperative best spectacle-corrected visual acuity (BSCVA) was 20/70, and the postoperative mean BSCVA was 20/50. A significant difference was observed between the arithmetic mean of the preoperative and postoperative BSCVA (p=0.001). Two of 18 patients improved stereopsis; furthermore, surgery variation strabismus was registered in 33.3% of the patients, following PRK. Patients were followed up for a mean of 39 months. CONCLUSIONS: The treatment of medium to high unilateral myopia with PRK under local anesthesia was found effective and safe in pediatric and adolescent patients. This procedure may improve ocular alignment and stereopsis. Further studies are needed to increase the patient experience and extend follow-up time in order to assess the long-term stability of the RESULTS.

Strabismus in developmental cataract.

PURPOSE: To evaluate the presence of strabismus in patients with developmental cataract rendered pseudophakic and how this influences their visual acuity. METHODS: A retrospective study was carried out on 113 patients with developmental cataract who came under the authors' observation at the outpatient department of the Pediatric Ophthalmology Unit of the University of Federico II of Naples from 1990 to 2005. All patients were followed up for a long period (mean 62 months, range 36-144 months). Age at diagnosis, sex, laterality, age at cataract extraction, morphology, and cataract density were all considered as possible factors associated with strabismus. Visual acuity and ocular motility before and after cataract extraction surgery were especially noted. Statistical evaluation was performed using t-test, Chi-square test, and Fisher exact test. RESULTS: Out of the 113 patients a total of 181 eyes were affected: 68 patients (60%) presented bilateral cataract, 45 patients (40%) monolateral cataract. Strabismus was present in 39 patients (34%) before cataract surgery. Age at cataract diagnosis, age at surgery, sex, and cataract morphology were not found to be statistically associated with strabismus. However, laterality was found to be statistically associated with the onset of strabismus. Cataract density was found to be statistically associated with poor vision. Patients with strabismus presented a non statistically significant lower visual acuity. CONCLUSIONS: Strabismus has a greater incidence in developmental cataract compared to the general population, and can influence visual acuity, especially in monolateral and total cataracts. Intraocular lens implants produced satisfactory visual rehabilitation.

Ocular manifestations in a case of childhood cicatricial pemphigoid.

PURPOSE: Cicatricial pemphigoid is a rare chronic autoimmune disease, characterized by progressive alterations on the skin and mucous membranes. Ocular lesions consists of chronic conjunctivitis, progressive subepithelial fibrosis on the conjunctival fornix together with symblepharon formation, obstruction of the Meibomian ducts, sicca syndrome, and occasionally, entropion associated with consequent trichiasis and corneal ulcers. METHODS: A 9-year-old patient with cicatricial pemphigoid with severe eye involvement came to our observation. A complete anamnesis, ophthalmologic examination, and systemic evaluation, including serum antibody levels evaluation and biopsies of mouth, vulva, parotid, and intestine mucosa, were performed. RESULTS: Ocular examination showed blepharospasm, conjunctival hyperemia, symblepharon, total cicatricial corneal leucoma, severe dryness, trichiasis, and eyelid edema. The results of medical and surgical treatment are reported. CONCLUSIONS: Given the serious clinical picture, possibly due to a late diagnosis, it was not possible to avoid relapse and appearance of new cicatricial adherences for which we considered wait and see the most appropriate approach, protecting the anterior segment of both eyes, sacrificing their function. Further follow-up was not possible as the patient died. An early diagnosis would have had a significant influence on the clinical course and on the response to therapy.

Incidence of ocular pathologies in Italian children with Down syndrome.

PURPOSE: This study identifies the incidence of primary ocular pathologies in a population of Italian children with Down syndrome. METHODS: A total of 157 Italian children with Down syndrome, age between 1 month and 18 years, were screened between February 2005 and October 2006. The ophthalmologic evaluation included a global inspection of orbit and bulbus oculi, evaluation of ocular motility and visual acuity, slit lamp biomicroscopy, cycloplegic skiascopy, tonometry, and indirect ophthalmoscopy. RESULTS: The overall incidence of ocular abnormalities was epicanthal fold (132 patients, 84%), hyperopia (93 patients, 59%), astigmatism (44 patients, 28%), myopia (14 patients, 9%), strabismus (56 patients, 36%, 45 cases of esotropia and 11 cases of exotropia), congenital nasolacrimal duct obstruction (35 patients, 22%), cataract (18 patients, 11%), nystagmus (9 patients, 6%), blepharitis and conjunctivitis (6 patients, 4%), and retinal anomalies (10 patients, 6%). Unlike previous reports in patients with Down syndrome, no congenital glaucoma, keratoconus, or Brushfield spots were observed. CONCLUSIONS: Compared to other studies in patients with Down syndrome, we observed a higher incidence of hyperopia and a lower incidence of myopia. The incidence of nystagmus, blepharitis,and conjunctivitis was less than that reported in other studies, while strabismus, especially exotropia (20%), had a high incidence in our cohort. We also frequently observed obstruction of the nasolacrimal duct (22%), but no keratoconus or glaucoma.

Unusual seizures with a benign course in a case of acute lymphoblastic leukemia of childhood.

We report the unusual seizures in a patient with acute lymphoblastic leukemia. The convulsive disorder began acutely as partial somatomotor status epilepticus and with diffuse EEG slowing. The seizures then became myoclonic-atonic (drop attacks) and an EEG focus appeared on the left paramedian centro-parietal areas, activated by proprioceptive stimuli. Despite the severity of the clinical picture at onset, the seizures showed a benign course, and disappeared on carbamazepine therapy. We think that this epileptic syndrome may have been caused by diffuse iatrogenic encephalopathy, probably related to intrathecal methotrexate therapy.

Upper gastrointestinal tract motility in children with progressive muscular dystrophy.

Gastric emptying was evaluated in 15 children (mean age, 8.0 years) with progressive muscular dystrophy to detect early gastrointestinal smooth muscle involvement; 10 of the children also underwent esophageal manometry. Clinical evidence of skeletal muscle dysfunction was minimal in 14 of the 15 patients; 10 of them had no gastrointestinal symptoms. Gastric emptying studies were performed by using 500 muCi of technetium 99m-sulfur colloid bound to a scrambled egg, and scintigraphic measurements were made continuously for 60 to 90 minutes. Gastric emptying studies and manometric tracings were compared with those from 11 children (mean age, 8.4 years) without gastrointestinal or muscular disorders. Mean (+/- SD) percentage retention of gastric isotope was significantly greater in patients with muscular dystrophy than in control subjects. No differences were found between the two groups in distal esophageal motility or in upper and lower esophageal sphincter pressures or relaxation. Contraction amplitudes in the upper portion of the esophagus, however, were significantly lower in patients with myopathy than in control subjects. These data suggest that dysfunction of smooth muscle of the upper gastrointestinal tract is detectable in children with muscular dystrophy early in the course of the disease, even when gastrointestinal symptoms are absent and skeletal muscle symptoms are minimal.