Stability of silicone band frontalis suspension for the treatment of severe unilateral upper eyelid ptosis in infants.

PURPOSE: To address the efficacy, safety, and stability of frontalis suspension of the upper eyelid with a silicone band for the treatment of severe congenital ptosis in infants. METHODS: Data from 22 pediatric patients undergoing unilateral frontalis suspension with silicone band were retrospectively reviewed with a follow-up ranging from 18 to 30 months. The margin-reflex distance (MRD) and the corneal staining had been evaluated at each pre- and postoperative visit. Data were analyzed by analysis of variance and t test for paired data. RESULTS: MRD was absent before surgery in all cases: it ranged from -1 mm to -4 mm (-2.4+/-0.8 mm). Immediately after surgery, it increased to 2.9+/-0.3 mm, and then progressively reduced by 0.6 mm within the first 3 months (p=0.001); a further reduction of 0.2 mm, occurring between 3 and 12 months after surgery, was not statistically significant. After the 12-month visit, no changes in MRD were found at follow-up for any patient. Corneal staining, which was present in five patients over the first 2 postoperative weeks, recovered without sequelae. Complications occurred in three eyes: overcorrection and corneal ulcer in one case requiring removal of the silicone band, one granuloma, and one extrusion of the silicone band from the upper frontal incision. CONCLUSIONS: During the study period, the frontalis suspension with a silicone band was an effective and safe procedure. MRD values were stable between month 3 and the end of follow-up, although this series does not preclude the possible occurrence of blepharoptosis at longer time intervals.

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.

Mortality from second tumour among long-term survivors of retinoblastoma: a retrospective analysis of the Italian retinoblastoma registry.

Survivors of retinoblastoma (Rb) are at high risk of dying from second malignant tumour. The occurrence of second malignant neoplasm (SMN) and related mortality in a cohort of 1111 cases from the Italian Retinoblastoma Registry was analysed, considering the possible role of both genetic and iatrogenic causes. Rb patients had a greater than 10-fold excess in overall mortality compared with the general population (standardized mortality ratio (SMR) 10.73, 95% CI 9.00-12.80). Their excess risk attributable to cancers other than Rb was 14.93 95% CI 10.38-21.49). Survivors of hereditary Rb had an SMR for all causes of 16.25 (95% CI 13.20-20.00), whereas their SMR for all cancers was 25.72 (95% CI 17.38-38.07). Survivors of unilateral sporadic Rb had an SMR of 4.12 from all cancers (95% CI 1.55-10.98) and a much higher excess for overall mortality (SMR 13.34, 95% CI 10.74-16.56). As expected, survivors of hereditary Rb had higher mortality from cancers of the bone (SMR 391.90, 95% CI 203.90-753.20) and soft tissue (SMR 453.00, 95% CI 203.50-1008.40), small intestine (SMR 1375.50, 95% CI 344.00-5499.70), nasal cavity (SMR 13.71, 95% CI 1.93-97.35) and cancers of the brain and central nervous system (SMR 41.14, 95% CI 13.2-127.55).

Surgical approaches to cavernous haemangiomas of the orbit.

Cavernous haemangiomas are the most common orbital masses and the second most common cause of unilateral proptosis after thyroid ophthalmopathy. We retrospectively analysed 19 patients with retrobulbar cavernous haemangiomas, 9 of whom had lateral orbitotomy to remove retrobulbar cavernous haemangiomas located superior (n=4), inferior (n=2) or lateral (n=3) to the optic nerve. Seven patients had lateral orbitotomy together with an anterior medial approach to gain access to retrobulbar cavernous haemangiomas located medially to the optic nerve in the posterior half of the orbit. An anterior approach was used in 3 patient with an anteriorly located cavernous haemangioma. We describe here the planning of surgical treatment based on the site of the lesion.

Correction of congenital entropion of the lower eyelid: incisional versus rotational surgery.

PURPOSE: When, at birth, the eyelid margin is rolled inward against the globe, the condition is referred to as congenital entropion. Upper eyelid involvement is commonly associated with a tarsal abnormality, while lower eyelid entropion is often associated with epiblepharon. Entropion does not resolve spontaneously, and may cause corneal pathology if untreated. The purpose of this study is to compare the two common techniques for the correction of congenital entropion. METHODS: The authors performed a pilot study of 24 consecutive patients with lower bilateral congenital entropion to compare the results of incisional versus rotational surgery. RESULTS: The rotational procedure was carried out in 14 patients; incisional surgery was performed in 10 patients. Twenty-one patients had good functional and cosmetic results. There were only three case of relapse after 3, 4, and 3 months. CONCLUSIONS: The authors consider both techniques satisfactory, but the procedure of choice, considering the age of the patients and previous studies, remains rotational sutures because of its simplicity, quickness, and low risk of complication.

Normal endothelial cell density range in childhood.

Specular microscopy of the in vivo corneal endothelium of 214 clinically normal eyes in children ranging from 5 to 14 years of age showed a regular mosaic of hexagonal cells. The cell population density of individuals presented some variation, as it doses in older subjects. Quantitative analysis permitted us to determine the normal range of the endothelial cell count at each age. The mean (+/- SD) value ranged from 3591 +/- 399 cells per square millimeter at age 5 years to 2697 +/- 246 cells per square millimeter for the oldest subjects. Our data show a rapid decrease in cell density up to age 10 years. We estimate from our data a decrease in cell density of 13% between ages 5 and 7 years and an additional decrease of 12% by age 10 years.

Atlantoaxial dislocation related to instilling eyedrops in a patient with Down's syndrome.

PURPOSE: To alert ophthalmologists of the risk of hyperextending the neck of patients with Down's syndrome while holding them to instill eyedrops. METHOD: We describe a case of atlantoaxial dislocation related to instilling eyedrops in a 6-year-old girl with Down's syndrome. RESULT: Early recognition of the problem allowed prompt treatment and partial recovery of the patient. CONCLUSION: Examination and clinical maneuvers in a child with Down's syndrome require gentleness and avoidance of hyperextension of the neck to prevent spinal cord injury.

Normal exophthalmometric values in children.

We used a Hertel exophthalmometer to measure the degree of ocular protrusion in 852 subjects who had no history of orbital trauma or disease, endocrine disease, severe myopia, buphthalmos, or craniofacial deformities. The subjects ranged in age from 3 to 10 years. No statistically significant difference was observed between boys and girls or between right and left eyes in each age group. The mean normal protrusion values ranged from 9.11 mm in the 3-year-old group to 11.67 mm in the 10-year-old group. The mean outer orbital margin distance ranged from 78.7 mm in the 3-year-old group to 89.1 mm in the 10-year-old group. The data obtained were tabulated to plot percentile distribution curves of normal exophthalmometric values and outer orbital margin distance in children. No subject had more than 2 mm of asymmetry between the eyes.

Genetic isolate and preserved para-arteriole retinal pigment epithelium.

Preserved para-arteriole retinal pigment epithelium (PPRPE) is an uncommon form of retinitis pigmentosa, with a very peculiar funduscopic appearance. To our knowledge no patient under age ten, affected by PPRPE, has been reported in the literature. The authors present here a seven-year-old boy, whose fundus examination is consistent with the diagnosis of PPRPE. The case report confirms that PPRPE starts early in childhood, and additionally supports the hypothesis of an autosomal recessive inheritance of this condition, since the proband's family lives in a 'genetic isolate'.

Electronystagmographic investigation in X-linked ocular albinism.

Albinism represents the most frequent cause of nystagmus induced by visual sensory disorders. We studied five patients aged between three and five years with ocular albinism with the aim of identifying the characteristics of nystagmic oscillations. The patients underwent complete ophthalmological examination and electronystagmography (ENG). ENG revealed either pendular or jerk waveforms (or both) with the same complex effects of fixation seen in those with idiopathic congenital nystagmus. Three children presented face turn due to reduced severity of nystagmus in lateroversion. Latent nystagmus was not observed.

Macular dysplasia and pigmented paravenous retino-choroidal atrophy.

Pigmented paravenous retino-choroidal atrophy (P P R C A) is a rare retinal disease characterized by bilateral patches of pigment and areas of chorioretinal atrophy distributed along the veins. The authors present a 21-year-old male with pigmented paravenous retinochoroidal atrophy and unilateral macular dysplasia. To their knowledge, this is the second reported case of macular involvement. They believe that such association is not occasional, but may be suggestive of a variable expressivity of the disease.

Balanced translocation (t 2q; 10p) and ocular anomalies. A possible HOX gene defect.

The authors report a child with a phenotype typical of a first branchial arch defect. The patient has a balanced translocation involving chromosome 2. They propose a defect that has occurred during the translocation in a gene mapped to chromosome 2 and belonging to the HOXD family. HOX gene defects can perturb the expression of other genes important for head development.

Unexplained head tilt following surgical treatment of congenital esotropia: a postural manifestation of dissociated vertical divergence.

BACKGROUND: Strabismus surgery for congenital esotropia can be complicated by the development of a postoperative head tilt. PURPOSE: To determine the pathophysiology of acquired head tilting following horizontal realignment of the eyes in children with congenital esotropia. MATERIALS AND METHODS: Retrospective analysis of nine children with congenital esotropia who developed unexplained head tilts following horizontal realignment of the eyes. RESULTS: Shortly after strabismus surgery, each child developed a head tilt in association with asymmetrical dissociated vertical divergence (DVD). Five children maintained a head tilt toward the side of the fixing eye (group 1), which did not serve to control the DVD. Four children maintained a head tilt toward the side of the hyperdeviating eye, which served to control the DVD (group 2). Children in group 2 had earlier horizontal muscle surgery and developed better stereopsis than those in group 1, suggesting that the higher degree of single binocular vision and stereopsis in these children may have led to a compensatory torticollis to control an asymmetrical DVD. CONCLUSIONS: The onset of an unexpected head tilt after congenital esotropia surgery is usually a postural manifestation of asymmetrical DVD. In this setting, a head tilt toward the side of the fixing eye corresponds with a postural manifestation of the underlying central vestibular imbalance that produces DVD, while a head tilt toward the side of the hyperdeviating eye serves to counteract the hyperdeviation and stabilise binocular vision.

Neuron-specific enolase and embryology of the trabecular meshwork of the rat eye: an immunohistochemical study.

Neuron-specific enolase (NSE) is a unique form of the glycolytic enzyme enolase found exclusively in neurons and neuroendocrine tissues. Immunohistochemical techniques in which antineuron-specific enolase antibodies are used have made it possible to map out derivatives of the neural crest in humans. By using affinity-purified antibodies against NSE, we investigated whether the contribution of the neural crest cells to the development of the anterior ocular structures in the rat is similar to that in man. We found that filtration structures in rats show morphologically striking similarities with the analogous region of the human eye. Hence, the rat eye, with certain reservations, is a suitable model for experimental studies on ocular diseases that are characterized by chamber angle anomalies or congenital glaucoma.

Keratoconus and Turner's syndrome.

PURPOSE: To report the association of keratoconus and Turner's syndrome in three patients and to review the ophthalmic manifestations of Turner's syndrome. METHODS: Three patients with keratoconus and Turner's syndrome were identified and reported in a retrospective review. RESULTS: These three cases represent the first series of patients with keratoconus and Turner's syndrome. All three patients underwent penetrating keratoplasty with good visual rehabilitation. None of the patients had other ocular features associated with Turner's syndrome. CONCLUSION: Turner's syndrome is commonly associated with ocular problems. In this series we identify an association of keratoconus with Turner's syndrome. Clearly, a careful ocular examination in this condition with attention to ocular features of Turner's syndrome is important.

Refractive surgery in children.

Refractive surgery techniques, especially those using laser ablation, have revolutionized the treatment of refractive errors. The short-term results have been well studied in adults, but long-term outcomes are not known. No good studies exist to tell us whether the pediatric eye responds the same as the adult eye to these techniques, but there is reason to believe that the pediatric eye will have many differences, both short- and long-term based on other similar surgeries adapted for pediatrics. These techniques have great potential to add to our armamentarium of treatments for frustrating problems such as unilateral high myopia with amblyopia, but they should be used with caution. The patients who are the most attractive to refractive surgeons, namely, teenagers with typical myopia who want to discard their spectacles, are the patients with potentially the most to lose from a procedure with unknown long-term results for a condition easily treated with other modalities. Those who need it most, namely infants and children with high anisometropia who cannot tolerate contact lenses or spectacles, are the least cooperative, the most difficult to treat postoperatively, and the least able to afford expensive treatments not covered by insurance, are therefore the least likely to be offered the procedure, or to have a study designed to evaluate their specific needs and concerns. It behooves ophthalmologists interested in pediatrics to carefully discuss and research the possible indications and theoretical concerns of these powerful techniques in pediatric eyes.

Selective photocoagulation in Coats' disease: ten-year follow-up.

PURPOSE: The diagnostic hallmark of Coats' disease is development of "light bulb" telangiectasis in the retinal periphery, leading to posterior pole intraretinal and subretinal exudation. Even after complete obliteration of all abnormal vessels and resorption of all exudates, follow-up examinations are mandatory for several years. METHODS: We retrospectively analyzed the charts, pictures and/or fluorescein angiographies of a series of 32 consecutive patients, in which the diagnosis of Coats' disease was made. All patients underwent selective photocoagulation of the telangiectasis using a yellow-dye laser. Efficacy of treatment was monitored with drawings and/or fluorescein angiographies. RESULTS: All our patients had unilateral disease, with macular involvement and exudative retinal detachment. Visual acuity improved in one patient, decreased from light perception to blindness in another, and was unchanged in the remainder. CONCLUSIONS: This is the first report of anatomical benefits from treatment with a yellow-dye laser (i.e., selective photocoagulation without cryotherapy or drainage) despite the presence of a severe form of Coats' disease. Our young Coats' patients responded quickly to treatment. With successful obliteration of the abnormal vasculature, exudates began to absorb in about 4 to 8 weeks although, in some cases, 10 to 12 months were needed to obtain resolution of the exudative detachment. Poor visual outcome of 20/100 or worse was common. In Coats' patients, the key issue is continuous control and long-term follow-up.

Ectopia lentis et pupillae with patchy depigmentation of the skin, hair and lashes: a new association.

We describe a case of a four year-old boy, with congenital ectopia lentis et pupillae, who developed patchy unilateral depigmentation of the skin, hair and lashes. The association between ectopia lentis et pupillae and transillumination of the iris is well documented in the literature, but it has never been reported with skin hypopigmentation.

Refractive surgery for unilateral high myopia in children.

PURPOSE: To evaluate the safety and efficacy of refractive surgery in children. METHODS: Pediatric patients with unilateral high myopia who were 9 years of age or older were offered refractive surgery to supplement optical correction. The patients and families were informed that the operation may not improve their best-corrected visual acuity. Photorefractive keratectomy (PRK) or laser in situ keratomileusis (LASIK) was performed on the more myopic eye with the use of topical anesthesia. Cycloplegic refraction, stereopsis, motility, and best-corrected visual acuity were measured before the procedure and at 2 months and 20 months after the procedure. All patients had completed amblyopia therapy before surgery. RESULTS: Fourteen eyes of 14 patients aged 9 to 14 years received refractive surgery. Average age at the time of surgery was 11.9 years (+/-1,6). Average corrected preoperative visual acuity was 20/147 (+/-0.065 in decimals). Average preoperative refraction was -7.96 D (+/-2,16) spherical equivalent. Twenty months after refractive surgery, the uncorrected visual acuity averaged 20/129 (+/-0.08 in decimals) and best-corrected vision averaged 20/121 (+/-0.08 in decimals). Average refraction was -0.46 D (+/-0,58) at 2 months and -0.67 D (+/-0,68) D at 20 months. An average myopic shift in refraction of -0.22 D was found in treated eyes during the 20 months of follow-up; this was not statistically significant (P =.69). Three patients had LASIK and 11 patients had PRK. LASIK patients averaged -0.875 D of myopic shift over 20 months of follow-up. Those with PRK averaged -0.025 D. This difference was not statistically significant (P =.10). The vision of 5 of 14 patients improved 1 or 2 lines after refractive surgery. Two patients who had 20/80 vision preoperatively improved to 20/60. No patients lost any lines of vision. Only 4 patients demonstrated stereopsis preoperatively, and all retained stereopsis postoperatively. No patient gained stereopsis. CONCLUSIONS: LASIK and PRK can be performed safely and effectively in children who are cooperative enough to undergo the procedures with topical anesthesia. Refractive surgery does not improve vision in densely amblyopic eyes but may give modest improvement in those that are mildly amblyopic. No significant complications were encountered aside from a myopic shift over time.

Conservative management of congenital nasolacrimal duct obstruction.

Fifty-nine children 1 to 24 months of age with congenital nasolacrimal duct obstruction (CNDO) were treated with local hydrostatic massage and antibiotic eye drops. Children 1 to 12 months of age showed a cure rate of 93.3%; only two of them underwent nasolacrimal probing. Children 13 to 24 months of age had a cure rate of 79.3%, and six underwent probing. The initial probings were successful in both age groups. Fifty-one children (86.4%) were thus spared nasolacrimal probing.