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Isolated strabismus affects 1-5% of the general population. Most forms of strabismus are multifactorial in origin; although there is probably an inherited component, the genetics of these disorders remain unclear. The congenital fibrosis syndromes (CFS) represent a subset of monogenic isolated strabismic disorders that are characterized by restrictive ophthalmoplegia, and include congenital fibrosis of the extraocular muscles (CFEOM) and Duane syndrome (DURS). Neuropathologic studies indicate that these disorders may result from the maldevelopment of the oculomotor (nIII), trochlear (nIV) and abducens (nVI) cranial nerve nuclei. To date, five CFS loci have been mapped (FEOM1, FEOM2, FEOM3, DURS1 and DURS2), but no genes have been identified. Here, we report three mutations in ARIX (also known as PHOX2A) in four CFEOM2 pedigrees. ARIX encodes a homeodomain transcription factor protein previously shown to be required for nIII/nIV development in mouse and zebrafish. Two of the mutations are predicted to disrupt splicing, whereas the third alters an amino acid within the conserved brachyury-like domain. These findings confirm the hypothesis that CFEOM2 results from the abnormal development of nIII/nIV (ref. 7) and emphasize a critical role for ARIX in the development of these midbrain motor nuclei.

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OBJECTIVE: To describe presenting ophthalmic signs and symptoms in children with parameningeal rhabdomyosarcoma. DESIGN: Retrospective, noncomparative case series. PARTICIPANTS: Twenty-three children with parameningeal rhabdomyosarcoma treated from 1978 through 1998. MAIN OUTCOME MEASURES: Each patient's presenting symptoms and history, the ophthalmic signs at presentation, the location of the tumor on computed tomographic scanning, the pathologic diagnosis after tumor biopsy, and the child's outcome after chemotherapy and radiotherapy. RESULTS: Eight of 23 patients with parameningeal rhabdomyosarcoma had ophthalmologic signs at presentation. All patients had sixth nerve palsies, and four had additional third nerve palsies. Fifth and seventh nerve involvement was seen in three children each, and two manifested additional fourth nerve palsies. In two patients, invasion of the optic canals and posterior orbit resulted in unilateral blindness in one and marked unilateral visual loss in the other. In no patient was the symptomatic history longer than 12 weeks. Seven of eight patients were treated with a combination of surgery, chemotherapy, and radiotherapy. The mean survival time for five patients who died from parameningeal rhabdomyosarcoma associated with ophthalmic signs was 27.2 months. Three patients are currently alive, with a mean survival time of 33.7 months after diagnosis. The survival time of 12 patients with parameningeal rhabdomyosarcoma and without ophthalmologic signs at presentation was better, in that nine are alive, with a mean survival time of 54 months. CONCLUSIONS: Advanced parameningeal rhabdomyosarcoma with skull base invasion may present with ophthalmic signs. These patients sought treatment late and had advanced tumors because their initial signs and symptoms were often attributed to more common ailments. Usually, the onset of the cranial nerve palsy initiated referral to the tertiary care center. The sixth nerve was the most vulnerable. Further invasion of the skull base or cavernous sinus may cause additional third, fourth, and fifth nerve palsies. The optic nerve was involved when the optic canals and orbit were encroached on by the tumor. The prognosis for survival was poor when these tumors were accompanied by ophthalmic signs.

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BACKGROUND: Inadvertent perforation of the globe is a well-recognized complication of extraocular muscle surgery. We evaluated the incidence, risk factors, and sequelae of this complication at our institution. METHODS: Medical records of patients who underwent extraocular muscle surgery at King Khaled Eye Specialist Hospital, Saudi Arabia, between September 1983 and April 1997, were reviewed for the occurrence of globe perforation. We documented preoperative visual acuity and refraction, surgical procedure, how the perforation occurred, and immediate management, as well as the sequelae of the perforation, its management, and final outcome. RESULTS: Recognized perforations occurred in 15 of 4886 procedures, for an overall incidence rate of 3/1000. Perforations were 3 times more common in myopic eyes (>-6.00 D, P =.05) and 2 times more common in eyes with previous extraocular muscle surgery. Perforations occurred during muscle reattachment (5 cases), placement of traction sutures at the limbus (4 cases with transient hyphema), muscle disinsertion (3 cases), and placement of sutures at the muscle insertion before disinsertion (3 cases). One patient had a large scleral laceration with uveal prolapse, necessitating scleral patch graft at the time of surgery, and later had retinal detachment surgery with loss of 2 lines of visual acuity. Endophthalmitis, cataract, glaucoma, and phthisis bulbi were not encountered in our review. CONCLUSION: The current incidence of globe perforation is low and only rarely associated with serious sequelae.

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PURPOSE: To better describe the clinical and neuroimaging spectrum of abnormalities in clinical anophthalmos. METHODS: We performed a retrospective review of all 17 patients admitted to the King Khaled Eye Specialist Hospital with clinical anophthalmos over a 15 year period who had a complete ophthalmological examination documented and received computed tomographic (CT) imaging of the orbits and brain. RESULTS: Patients with clinical anophthalmos had a high incidence of developmental abnormalities involving both eyes (15/17 patients, 88%), the brain (12/17 patients, 71%) and the body (7/12, 58%). The incidence of central nervous system anomalies reached 100% in patients with bilateral small optic nerves on CT scan. CONCLUSIONS: Patients with clinical anophthalmos share a similar constellation of neurological, somatic and neuroradiological abnormalities as patients with microphthalmos, septo-optic dysplasia and clinical optic nerve hypoplasia. This fact may provide insight into developmental abnormalities of the afferent visual system and brain.

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OBJECTIVE: To document clinical features and subsequent management of pyramidal anterior polar cataracts in children. DESIGN: Retrospective, noncomparative case series and clinicopathologic correlation. PARTICIPANTS: Fifteen patients who presented to the pediatric ophthalmology clinic. INTERVENTION: All patients underwent measurement of visual acuity, assessment of ocular motility, examination of the anterior and posterior segments, and cycloplegic refraction. Amblyopia treatment was instituted when appropriate. When visual impairment occurred from cataract progression or amblyopia or both, cataract removal with or without lens implantation was performed. After surgery, correction of refractive error and treatment of amblyopia were instituted. Several pyramidal opacities were retrieved during cataract extraction and examined by light and electron microscopy. MAIN OUTCOME MEASURES: Visual acuity at initial presentation, size of lens opacity before surgery, amblyopia status, most recent visual acuity after cataract extraction, and histologic examination of lens opacity. RESULTS: Nine children had bilateral and six had unilateral pyramidal cataracts (24 eyes). There was no discernible inheritance pattern. Patients were followed for 27 months on average. Twenty of 24 eyes developed cortical opacification that extended significantly beyond the base of the pyramidal lesion. Nineteen eyes required cataract surgery: 10 eyes underwent lensectomy with anterior vitrectomy and 9 had extracapsular cataract extraction, 8 of which had insertion of a posterior chamber intraocular lens. Amblyopia was present or developed in all six patients with unilateral cataract and in eight of nine patients with bilateral cataract. Visual acuity in many eyes remained poor despite amblyopia therapy. The pyramidal opacities consisted of hyperplastic lens epithelium, which exhibited a loss of polarity and was surrounded by a collagenous matrix. CONCLUSIONS: Pyramidal anterior polar cataracts are present at birth and may represent a variant of anterior polar lens opacities. They may be unilateral or, if bilateral, they may be either symmetric or asymmetric. They consist of hyperplastic lens epithelium in a collagenous matrix. Patients with pyramidal cataracts are likely to develop amblyopia. This can result from either unilateral occurrence or asymmetry of bilateral opacities and is often worsened by surrounding cortical opacification. Many patients require cataract surgery. All infants and young children with anterior polar opacities showing this configuration should be followed for cataract progression and amblyopia.

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OBJECTIVE: To review the results of combined trabeculotomy and trabeculectomy as a primary procedure in congenital glaucoma. METHODS: A retrospective review of 100 consecutive eyes in 60 children undergoing surgery from December 30, 1991, to April 17, 1996. Features at initial examination, ie, corneal size and clarity, presence or absence of anterior segment structural abnormalities, and intraocular pressure (IOP), were noted. Data pertaining to perioperative use of mitomycin and the occurrence of complications were collected. After surgery, all patients had IOP, corneal integrity, and any postoperative complication recorded under chloral hydrate sedation. RESULTS: Mean preoperative IOP was 31 mm Hg. Average horizontal corneal diameter was 12.60 mm. Ninety-five eyes had corneal opacification. Twenty-nine eyes had additional anterior segment anomalies, with ectropion uveae (n = 11), Peters anomaly (n = 9), and partial aniridia (n = 7) being the most common. Mitomycin (0.2 or 0.4 mg/mL) was used in 87 eyes. Eleven eyes sustained hyphemas during or just after surgery. Total average follow-up was 304 days. Eyes in which no coexistent anterior segment anomalies were present had a 78% (49 eyes) operative success (IOP, <21 mm Hg); however, in eyes with associated anterior segment anomalies, the success rate was much lower (45% [18 eyes]). The difference in success rates between both groups was statistically significant (P = .03, chi2 test). CONCLUSIONS: Primary combined trabeculotomy and trabeculectomy was a useful initial procedure in uncomplicated congenital glaucoma. This was particularly true where corneal opacification, as in nearly all our eyes, precluded goniotomy, however, where other stigmata of anterior segment dysgenesis coexisted, results were significantly poorer.

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BACKGROUND: In glaucoma associated with Sturge-Weber syndrome (SWS), medical treatment often fails to control intraocular pressure, thus requiring surgical intervention that may result in serious complications. METHODS: Eighteen consecutive patients with SWS were reviewed retrospectively at the King Khaled Eye Specialist Hospital. An intraocular pressure less than 20 mm Hg, plus stable optic nerve cup-to-disc ratio and corneal diameter (or visual fields where appropriate), were parameters chosen to indicate that the glaucoma was being controlled. RESULTS: Glaucoma was found in 15 of 18 patients (22 eyes). The mean follow-up time was 62 months (range, 12 to 148 months). Medical treatment alone was successful in 5 patients (7 eyes); the remainder required surgical intervention. The initial surgical procedures included cyclocryotherapy, YAG laser goniotomy, surgical goniotomy, and trabeculotomy or trabeculectomy. Eight eyes required subsequent surgery, 5 with Molteno or Ahmed implants. Early postoperative choroidal effusion and hemorrhage occurred in 4 eyes and resolved spontaneously. Hemorrhagic choroidal detachment with total retinal detachment developed in 2 patients 3 to 5 months after surgery. In 1 patient a recurrent serous choroidal detachment after suture lysis was associated with total optic atrophy. CONCLUSIONS: Glaucoma in SWS is common. Control of glaucoma was successfully achieved with medical treatment in 7 of 22 eyes of our 15 SWS-glaucoma patients, and we consider it the initial treatment of choice. Of the 15 eyes that required surgery, late postoperative complications resulted in loss of vision as a result of persistent postoperative hypotony in 3 eyes that underwent surgical procedures.

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OBJECTIVE: To determine the safety and efficacy of surgical versus medical management in the treatment of ophthalmic complications of homocystinuria, and also to document ocular complications of homocystinuria other than lens dislocation. DESIGN: Retrospective case series. PARTICIPANTS: Forty-five patients with ophthalmic complications of homocystinuria participated. INTERVENTION: Eighty-four surgical procedures were performed on 40 patients. There were 82 procedures done with the patients under general anesthesia and 2 with the patients under local anesthesia. Medical therapy was attempted initially in all patients with lens dislocation and was the sole therapy used for five patients. MAIN OUTCOME MEASURES: Complications resulting from medical or surgical treatment and final visual acuity were studied. RESULTS: All patients had a history of lens subluxation or dislocation. Fourteen (31%) were receiving dietary treatment at the time of presentation and 29 (64%) were mentally retarded. Eighty-two procedures were performed with the patients under general anesthesia with 2 surgical complications and 1 postsurgical complication. Lens dislocation into the anterior chamber was the most frequent indication for surgery (50%) followed by pupillary block glaucoma (12%). Prophylactic peripheral iridectomy was not successful in preventing lens dislocation into the anterior chamber in five patients. Anesthetic precautions such as stockings to prevent deep venous thrombosis, preoperative hydration, or aspirin were given in 85% of cases. Other common ophthalmic complications found include optic atrophy (23%), iris atrophy (21%), anterior staphylomas (13%), lenticular opacities (9%), and corneal opacities (9%). CONCLUSION: Laser iridectomy was unsuccessful in preventing lens dislocation into the anterior chamber. With appropriate anesthetic precautions and modern microsurgical techniques, the risks associated with the surgical management of ocular complications of homocystinuria are reduced. Surgical treatment should be considered, especially for cases of repeated lens dislocation into the anterior chamber or pupillary block glaucoma. If a conservative, nonsurgical approach is undertaken, these patients must be observed carefully for repeat episodes of lens dislocation.

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PURPOSE: To examine the safety of mitomycin C (MMC) use in pediatric glaucoma surgery. DESIGN: Retrospective interventional case series. PARTICIPANTS: One hundred eighty pediatric glaucoma patients younger than 7 years of age (254 eyes) who underwent glaucoma surgery and were followed for at least 1 year participated. INTERVENTIONS: Surgeries consisted of trabeculectomy or combined trabeculotomy-trabeculectomy with adjunctive use of MMC. MAIN OUTCOME MEASURES: Control of intraocular pressure (IOP) and occurrence of complications were measured. An IOP below 21 mmHg without any additional medical or surgical treatment was considered a success. RESULTS: Primary congenital glaucoma was present in 98% of the patients. Sixty percent had a trabeculectomy, and 40% had a trabeculotomy-trabeculectomy. The combined surgery was performed mostly in infants up to 1 year of age. Complications were cystic bleb (19), bleb leak (4), retinal detachment (3), flat anterior chamber (3), cataract (2), and endophthalmitis (1). Children younger than 2 years of age had fewer complications but higher failure rates. The combined procedure in infants up to 2 years of age was moderately more successful (57%) than trabeculectomy alone (39%). Minimal complications seen in this group were more likely related to young age than to the type of surgery. Complications increased with time after surgery, with most occurring 2 years or more after surgery. CONCLUSIONS: Success of MMC-augmented glaucoma filtering surgery increased with age, as did the complication rate. Serious complications were uncommon in the authors' group of pediatric patients up to now. However, complications related to bleb thinning increased with time after surgery, and additional problems can be anticipated with longer follow-up.

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Evaluation of 4-month-old girl who presented with congenital cataracts revealed heterochromia iridis, fundus hypopigmentation, residual white forelock and sensory neural hearing loss--findings consistent with Waardenburg syndrome type II. Bilateral peripheral iridectomies performed at lensectomy provided tissue for evaluation. Light microscopy revealed fewer melanocytes in the blue iris than in the brown. Electron microscopic examination showed a significant (p = 0.0001) reduction in melanosome size in the blue iris, and the nerve endings contained fewer vesicles. A defect in neural crest cell migration and melanin synthesis may be responsible for the heterochromia iridis seen in Waardenburg syndrome type II.

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AIM: Preseptal and orbital cellulitis are rare presenting features of intraocular retinoblastoma. The objectives of this study were to determine the frequency of retinoblastoma associated cellulitis, as well as to review its clinical and histopathological features. METHODS: The medical records of 292 retinoblastoma patients in the King Khaled Eye Specialist Hospital in Riyadh, Saudi Arabia were reviewed. Those indicating a history of, or presenting with, cellulitis were retrieved and their clinical, radiological, and histopathological variables were assessed. Patients with definite extraocular tumour extension on clinical or radiological examination were excluded. RESULTS: 14 patients were found to have retinoblastoma associated cellulitis (4.8%); nine had bilateral and five had unilateral retinoblastoma. Conjunctival and blood cultures were performed in 10 cases and were negative. 10 children were treated with intravenous steroids, often in conjunction with antibiotics, resulting in a prompt decrease in inflammation. Three other children were treated with antibiotics alone and one received no treatment. Computed tomographic scanning depicted large intraocular tumours occupying between 80% and 100% of the globe in each case. In eight patients, periocular inflammation was radiologically interpreted as possible extraocular extension. In one patient serial computed tomographic scanning showed a reduction in intraocular calcification over time which occurred in the presence of cellulitis, 12 patients underwent enucleation and histopathological examination revealed large necrotic, poorly differentiated tumours associated with uveal involvement and early optic nerve invasion. Focal perilimbal destruction was seen in one patient, and in another peripapillary extrascleral extension was present. 12 patients are alive with a mean follow up of 56.4 months. CONCLUSIONS: Radiological evaluation of scleral integrity may be hindered by periocular inflammatory changes. The orbital cellulitis correlated well with the presence of advanced intraocular retinoblastoma with massive necrosis and anterior chamber involvement. In the majority of patients, cellulitis was not indicative of an extension of retinoblastoma into the orbit. Intravenous steroid treatment reduced orbital inflammation, facilitating examination and subsequent enucleation.

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OBJECTIVE: The purpose of the study was to assess integration of porous polyethylene allografts in 37 anophthalmic sockets of retinoblastoma enucleations. DESIGN: A clinical review. PARTICIPANTS: Thirty-four patients (19 female, 15 male) whose age when diagnosed with retinoblastoma ranged from 1 to 72 months participated. Sixteen patients had unilateral retinoblastoma and 18 had bilateral retinoblastoma. INTERVENTION: Enucleation (3 bilateral, 31 unilateral) with implantation of a porous polyethylene (PP) sphere (16-20 mm in diameter) was performed. Of the 37 spheres, 34 were primary implants and 3 were secondary implants. In the eight patients with postimplantation exposure, the PP implant had to be removed. MAIN OUTCOME MEASURES: Data on eight sockets with exposed PP implant were measured. RESULTS: There were no implant extrusions, but conjunctival dehiscence-exposure occurred in eight patients (21.6%). In 3 of 37 sockets, the volume replacement was not good, and in 5 cases, fornices were too shallow to accommodate a well-fitting prosthesis. In all but one socket, the overall cosmesis was graded subjectively from acceptable (grade 2) to excellent (grade 3). CONCLUSIONS: Although the PP implant is a useful and a less-expensive alternative to hydroxyapatite in the porous matrix implant category, if conjunctival exposure takes place, the implant presents a serious management problem because of its nonresponsiveness to medical and surgical treatments.

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The extraocular fibrosis syndromes are congenital ocular-motility disorders that arise from dysfunction of the oculomotor, trochlear, and abducens nerves and/or the muscles that they innervate. Each is marked by a specific form of restrictive paralytic ophthalmoplegia with or without ptosis. Individuals with the classic form of congenital fibrosis of the extraocular muscles (CFEOM1) are born with bilateral ptosis and a restrictive infraductive external ophthalmoplegia. We previously demonstrated that CFEOM1 is caused by an autosomal dominant locus on chromosome 12 and results from a developmental absence of the superior division of the oculomotor nerve. We now have mapped a variant of CFEOM, exotropic strabismus fixus ("CFEOM2"). Affected individuals are born with bilateral ptosis and restrictive ophthalmoplegia with the globes "frozen" in extreme abduction. This autosomal recessive disorder is present in members of three consanguineous Saudi Arabian families. Genetic analysis of 70 individuals (20 affected individuals) reveals linkage to markers on chromosome 11q13, with a combined LOD score of 12.3 at the single nonrecombinant marker, D11S1314. The 2.5-cM CFEOM2 critical region is flanked by D11S4196/D11S4162 and D11S4184/1369. Two of the three families share a common disease-associated haplotype, suggesting a founder effect for CFEOM2. We hypothesize that CFEOM2 results from an analogous developmental defect to CFEOM1, one that affects both the superior and inferior divisions of the oculomotor nerve and their corresponding alpha motoneurons and extraocular muscles.

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OBJECTIVE/DESIGN: This study aimed to examine and describe three siblings with alacrima, the eldest of whom had associated achalasia and adrenocortical insufficiency. PARTICIPANTS: Three affected siblings and four age-matched control subjects participated. INTERVENTION/MAIN OUTCOME MEASURES: The three children underwent complete ophthalmologic examinations; computed tomographic scanning of brain, orbit, chest, and abdomen; and measurement of serum cortisol. All three were subjected to a short synacthen challenge. Lacrimal gland biopsies were performed on the two younger subjects, and specimens were studied by light and electron microscopy. RESULTS: All three children showed virtually absent tear secretion as tested by the Schirmer test. The resulting keratopathy was most severe in the oldest child, who developed bilateral corneal melting. The two younger children showed interpalpebral corneal staining with rose bengal. All three children improved after punctal occlusion. Addison's disease was present in the oldest child. Computed tomographic scanning showed absent lacrimal and shrunken adrenal glands in association with achalasia of the cardia in the oldest child. The lacrimal glands were found to be reduced in size in the next eldest child. When evaluated by electron microscopy, the lacrimal gland biopsy specimens from the two younger children showed neuronal degeneration associated with depletion of secretory granules in the acinar cells. CONCLUSION: In this disease, radiologic evidence of reducing lacrimal gland size with increasing age could represent a degenerative process. This may be paralleled by other signs and the possibility of adrenocortical insufficiency and achalasia of the cardia should be investigated in all children presenting with dry eyes. These children appear to have a progressive neuronal disease.

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High-density porous polyethylene allografts are used as orbital implants in reconstruction of anophthalmic sockets. The use of this material for integrated buried implants is rather new, and studies on removed implants from humans are limited. We studied the case of a 6-year-old boy with an extruded porous polyethylene implant due to orbital recurrence of retinoblastoma. The clinical and morphological features are reported. Histopathology revealed fibrovascular downgrowth in the outer two thirds of the sphere. Scanning electron microscopy, however, documented further downgrowth of fibrous tissue into the central core. Transmission electron microscopy depicted a spectrum of collagen fiber orientations to polyethylene material, ranging from perpendicular to concentric. Immunohistochemistry studies were inconclusive.

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OBJECTIVE: The study aimed to review pediatric intraocular lens (IOL) implantations performed in the authors' institution for the past 7 years. STUDY DESIGN: The study design was a retrospective chart review. PARTICIPANTS: All children who underwent IOL placement between January 1, 1989, and January 31, 1996, at ages 2 to 16 years were studied. Follow-up was a minimum of 6 months up to 4 years with an average of 13 months. INTERVENTION: Placement of a primary or secondary posterior chamber IOL was performed. MAIN OUTCOME MEASURES: Visual acuity and possible complications were tabulated. RESULTS: Fifty-seven percent of the cataracts were caused by trauma, and 37% were infantile cataracts. Visual acuities of 20/40 or better were attained in 44% of eyes and visual acuities of 20/50 to 20/80 were attained in 27% of eyes. Visual acuities were better in children older than 4 years of age (P = 0.001). Compliance with amblyopia therapy improved vision (P = 0.004). Fibrinous membranes occurred in 41 eyes (13%), and posterior capsule opacification occurred in 120 eyes (39%). There were no significant differences in visual outcome and complications between primary and secondary implants (17%) or between capsular bag and sulcus fixation (27%). The first stable postoperative refraction was predicted accurately in most patients, based on the Sanders-Retzlaff-Kraff II (SRK II) formula and intraoperative corneal curvature and axial length determinations. The mean refraction differed from the goal by only +0.28 diopter and 84% were within +/- 2 diopters. A few patients showed large overcorrections or undercorrections. On average, no myopic shift occurred. CONCLUSIONS: Short-term results of IOLs implanted in children older than 2 years of age were favorable. Visual acuities obtained were better than 20/80 in 71% despite a high prevalence (28%) of penetrating trauma. Major complications were rare.

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PURPOSE: Our purpose was to evaluate the effect of increased orbital soft tissue volume on orbital growth. METHOD: Patients with unilateral or significantly asymmetric bilateral buphthalmos as determined by axial computed tomography scan were recruited. Volumetric determinations of the bony orbit with use of axial 1.5 mm sections on computed tomography were undertaken. Statistical analysis of the paired ocular length measurement and bony orbital volume measurements for each patient were performed. RESULTS: Eight patients (mean age 41 months) with a 15% or greater difference in axial length were enrolled. The mean axial length of the buphthalmic globes was 23% greater than that of the contralateral globes. Orbits harboring a buphthalmic globe had an orbital volume 11% greater than on the contralateral side. CONCLUSION: Increased orbital soft tissue volume as evidenced by buphthalmos was significantly associated with enlarged bony orbital volume. This indicates that soft tissue volume is a determinant of orbital volume and suggests that orbital tissue expanders might enhance bony development in patients with anophthalmos or microphthalmos and after early enucleation.

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BACKGROUND: Primary intraocular lens (IOL) implantation after cataract aspiration is a widely accepted means of correcting pediatric aphakia. However, little is available in the literature on secondary IOL implantation in children. We present our experience over the past 6 years. METHODS: The charts of 57 aphakic children (61 eyes) who underwent secondary posterior chamber IOL implantation between January 1989 and April 1996 were reviewed. In general, these children were either intolerant of or noncompliant with their contact lenses. An attempt was made to correlate visual outcome with patient variables. Evaluation of the ciliary sulcus structure was made in selected patients by ultrasonographic biomicroscopy to reveal any changes resulting from the presence of the IOL haptic in the sulcus. RESULTS: The age range at the time of surgery was 2 to 16 years (mean 8 y). Mean follow-up was 14 months (range 6 to 48 months). Forty-two percent of the patients had a best-corrected visual acuity of 20/40 or better and 78% saw better than 20/80. Posterior capsular opacification occurred in 10 eyes, 8 of which required neodymium:yttrium-aluminum-garnet laser capsulotomy. No major complications occurred. Ciliary sulcus evaluation by biomicroscopy did not reveal any significant ciliary body or scleral erosion. No changes were noted when the implanted sulcus was compared with the normal contralateral side. CONCLUSION: Although follow-up was short, this review suggests that secondary posterior chamber IOL implantation is a safe alternative when other methods of correcting pediatric aphakia fail.

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