Combined cataract extraction and epikeratophakia in children

Nineteen children between the ages of 2 1/2 and 6 1/2 years who had cataracts not associated with trauma and who were judged to be poor candidates for contact lens wear underwent combined cataract extraction and epikeratophakia, followed by intensive amblyopia therapy. Follow-up ranged from 6 months to 7 years. In these monocularly aphakic children, three (16%) of the original 19 procedures failed and the tissue lenses were removed; two of these were repeated successfully. The success rate for the surgery was 86% (18/21) and 95% (18/19) for the patients. Postoperative visual acuity of 20/50 or better was achieved in six (33%) of the 18 patients with successful surgery. Another 11 (61%) achieved between 20/60 and 20/200. Postoperative astigmatism ranged between 0 and 4 diopters, with an average of 1.7 diopters. The results demonstrate that epikeratophakia can be successfully combined with cataract extraction in children with visually significant non-traumatic cataracts and that the majority of such children demonstrate an improvement in visual acuity.     

Secondary cataracts in infants after lensectomies

Three infants, one who was two weeks old, and two who were two months old, underwent lensectomy and vitrectomy in a total of four eyes for congenital cataracts by means of an automated suction cutter. Two patients with unilateral opacities underwent combined cataract extraction and epikeratophakia, and one with bilateral congenital cataracts underwent cataract extractions and was fit with extended wear contact lenses. In all cases, the surgery involved at least a 5-mm posterior capsulotomy with a shallow anterior vitrectomy, and was uneventful. Three of the four eyes developed new opacities that required surgical removal three to five months after the original surgery. Cytological evaluation of the specimen obtained from one patient showed this material to be lens epithelium. In all three cases, the material grew without the support of the posterior capsule; in one patient the material appeared to have seeded onto the iris. This previously unreported complication in infants with congenital cataracts who have undergone posterior capsulotomy and anterior vitrectomy emphasizes the need for frequent retinoscopies on such patients. The absence of the posterior capsule does not guarantee that these children will not develop secondary growth of lens epithelium which may obstruct the visual axis.     

Visual acuity in monocular pediatric aphakia: does epikeratophakia facilitate occlusion therapy in children intolerant of contact lens or spectacle wear?

Twenty-two children had a monocular cataract extracted between 1981 and 1986. Twelve of these patients eventually tolerated a monocular contact lens or anisometropic spectacle wear. Eleven patients initially would not tolerate conventional therapy, and epikeratophakia was performed. Of the 12 patients tolerating a contact lens or spectacles, eight (67%) now see 20/60 or better, and six (50%) see 20/40 or better. Only one (10%) of the patients maintaining the epikeratophakia graft developed vision as good as 20/70. Epikeratophakia did not facilitate occlusion therapy for amblyopia. The critical factor in the development of good vision following monocular cataract extraction was the patient's willingness to patch the better eye, not the method of correcting the refractive error.     

Multistage approach to uveitic cataract management in children

Phototherapeutic keratectomy, cataract extraction and hydrophobic acrylic lens implantation, Nd:YAG laser capsulotomy, glaucoma valve implantation, and intravitreal steroid injections resulted in substantial visual improvement in three eyes of two children with complicated cataract due to severe uveitis. At follow-up of 6 months to 4 years, the children had clear corneas and normal intraocular pressures.     

Sensory deviations subsequent to senile cataract

PURPOSE: To evaluate the effect of extracapsular cataract extraction with posterior chamber intraocular lens (IOL) implantation in patients with sensory deviations subsequent to senile cataract. METHODS: Twenty patients with dense cataract and associated sensory deviations underwent follow-up between April 1996 and April 1998 after extracapsular cataract extraction with posterior chamber IOL implantation in the deviating eye. All patients underwent follow-up for a minimum period of 6 months. RESULTS: Preoperatively, all patients had a visual acuity of PL positive (perception of light present) and PR (projection of rays) accurate in the deviating eye. Two patients had an esodeviation, and 18 patients had an exodeviation. Postoperatively, all patients had a corrected visual acuity of 20/40 or better in the operated eye at 12 weeks. Nineteen patients had ocular alignment within +/-8 prism diopters of orthophoria at 12 weeks. CONCLUSION: Sensory deviations subsequent to senile cataract usually resolve spontaneously after cataract surgery if visual gains are 20/40 or better. The prognosis for binocular vision is good.     

Intraocular lens implantation in children and youth.

PURPOSE: To evaluate the safety and the effectiveness posterior chamber intraocular lenses (PCIOLs) in children and youth with cataract. METHODS: Retrospective study of operative and postoperative results was based on 146 eyes in 125 children ages 6 to 18 years (mean: 11.7 years) who underwent extracapsular cataract extraction with IOL implantation in the period from 1986 to 1996. Follow up was 1 to 10 years. OUTCOME MEASURE: Best corrected distance and near visual acuity, operative complications, and early and late postoperative complications. Exclusion criteria were uveitis, retinal detachment, prior glaucoma, and systemic diseases. RESULTS: Early postoperative complications were observed in 12.3% of cases including iritis in 7.5%, and transient corneal edema in 3.4% of eyes. Posterior capsule opacification requiring capsulotomy was needed in 81.5% of eyes after 8.5 years. YAG laser capsulotomy was performed in 68.1% and surgical intervention in 26.8% of cases. In 5.1% of the cases, both techniques were used. In 74.7% of patients, best corrected visual acuity of 0.5 or better was achieved. The mean postoperative spherical equivalent was +0.35 diopters (D, SD 1.35). Spectacle-corrected near visual acuity of 0.5 on the Snellen chart was obtained in 82.8% of eyes. CONCLUSION: IOL implantation in children older than 6 years appears to be an efficacious, satisfactory, and safe procedure in the management of pediatric cataract.     

Traumatic cataract surgery with intraocular lens implantation in children.

We evaluated our experience of intraocular lens implantation in 22 children with uniocular traumatic cataract. Good visual acuity was achieved in a large proportion of the children despite associated corneal opacities and several intraoperative and postoperative complications. Our study suggests that intraocular lens implantation may have a major role in management of monocular cataracts in children.     

Lensectomy in an infant with cri du chat syndrome and cataracts

We report an 11-month-old infant with cri du chat syndrome and cataracts. The chromosomal abnormality was transmitted via a balanced 5/11 translocation from a phenotypically normal mother. The child underwent bilateral cataract extraction and was fitted with extended wear contact lenses. Congenital cataracts may be associated with cri du chat syndrome.     

Changes in astigmatism after congenital cataract surgery and foldable intraocular lens implantation

PURPOSE: To evaluate the changes in astigmatism after cataract extraction and implantation of a foldable intraocular lens (IOL) in children. Only eyes with astigmatism of 3.0 D or more were included in the study. METHODS: The charts of children who had undergone surgery for nontraumatic cataract using a foldable IOL were retrospectively reviewed. In 13 eyes with astigmatism of 3.0 D or more, the refraction was tested and recorded at 1 week, 3 months, and 5 months postoperatively. A paired t test was used to compare the variables. RESULTS: Mean astigmatism 1 week postoperatively was 4.7 +/- 1.9 D (range, 3.0-10.0 D). Thereafter, the astigmatic component of the refractive error underwent a spontaneous steady decline, reaching a mean value of 0.9 +/- 0.9 D (range, 0-2.25 D) 5 months after surgery. The difference between the mean values at 1 week and 5 months was statistically significant (P < .0001). CONCLUSION: Children who underwent congenital cataract surgery and IOL implantation showed a significant spontaneous reduction in astigmatism postoperatively.     

Educational paper

Congenital cataract is the commonest worldwide cause of lifelong visual loss in children. Although congenital cataracts have a diverse aetiology, in many children, a cause is not identified; however, autosomal dominant inheritance is commonly seen. Early diagnosis either on the post-natal ward or in the community is important because appropriate intervention can result in good levels of visual function. However, visual outcome is largely dependent on the timing of surgery when dense cataracts are present. Good outcomes have been reported in children undergoing surgery before 6 weeks of age in children with unilateral cataract and before 10 weeks of age in bilateral cases. Placement of an artificial intraocular lens implant after removal of the cataract has become established practice in children over 2 years of age. There remains debate over the safety and predictability of intraocular lens implantation in infants. Despite early surgery and aggressive optical rehabilitation, children may still develop deprivation amblyopia, nystagmus, strabismus, and glaucoma. The diagnosis and management of congenital cataracts has improved substantially over the past 30 years with a concurrent improvement in outcomes for affected children. Many aspects of the pre-, intra-, and postoperative management of these patients continue to be refined, highlighting the need for good quality data and prospective collaborative studies in this field.     

Brain metastases in children

We reviewed the records of 31 children under the age of 21 years with parenchymal brain metastases diagnosed by CT scan (13 patients) or necropsy (18). Brain metastases were found in 18 of 139 (13%) children with solid tumors in whom complete postmortem examinations were done. Osteogenic sarcoma and rhabdomyosarcoma were the most frequent primary tumors causing brain metastases in patients younger than 15 years, and testicular germ cell tumor, from age 15 to 21 years. Evidence of intratumoral hemorrhage was found in 50% of autopsy cases. Pulmonary metastases were present in 28 of 31 (90%). The median interval from recognition of pulmonary metastases to the development of brain metastases was 10 months. No patient had evidence of brain metastases at diagnosis of the systemic cancer. In only one patient was the brain the only site of relapse. Following detection of brain metastases, the median survival was seven months in six patients who underwent surgery and whole-brain radiation therapy and four months in 15 given radiotherapy (3000 rads) alone. Patients with relatively radioresistant brain metastases may benefit from surgical excision or higher doses of radiation, or both.     

Epikeratophakia in children with traumatic cataracts

Epikeratophakia provides a permanent optical correction for aphakia in children with congenital or traumatic cataracts; suturing the epikeratophakia graft onto the cornea eliminates the problems of contact lens or spectacle non-compliance in these young and generally uncooperative patients and provides tectonic support to scarred and irregular corneas. Eighteen children under the age of six years underwent epikeratophakia for the correction of aphakia after the removal of trauma-induced cataracts. Graft success rate was 88%; the average change in keratometry in the patients with successful grafts was 14.82 +/- 2.0 diopters. In the 13 patients eligible for visual acuity tabulation, preoperative acuities ranged from light perception to 20/200, and postoperative acuities ranged from hand motions to 20/30. Ten (77%) had acuities of 20/80 or better. Poor results in three patients with less than 20/200 acuities were likely the results of non-compliance with amblyopia therapy. Present work indicates that in cases of traumatic cataract, the epikeratophakia procedure facilitates amblyopia therapy and decreases the astigmatism in scarred and irregular corneas.     

Cataract surgery in children with uveitis

PURPOSE: To report the technique and postoperative results of cataract surgery in children with uveitis. METHOD: Between 1988 and 1998, nine children (age range: 2.5-11 years) who developed secondary uveitic cataract and underwent cataract extraction were studied retrospectively. Seven children had juvenile rheumatoid arthritis and two had chronic anterior uveitis of unknown etiology. The surgical technique was lensectomy and wide anterior vitrectomy with limbal approach, lysis of anterior synechiae and in some cases, peripheral iridectomy. Postoperative aphakia was corrected with soft contact lenses in all patients. Follow-up ranged from 6 months to 6 years. RESULTS: Postoperatively, visual acuity in all patients improved and final visual acuity ranged from 20/70 to 20/25. Significant intraoperative complications did not occcur in any patient. One boy with juvenile rheumatoid arthritis developed cystoid macular edema 1 month postoperatively, which was successfully managed. He also developed hypertonia 1 year later, which was also successfully managed. Seven of the nine children had fewer and milder relapses of uveitis after surgery. CONCLUSION: Cataract surgery, using the lensectomy-vitrectomy technique in children with uveitis, is a safe technique with a relatively small percentage of postoperative complications and good functional results.     

Long-term rates of PCO following small incision foldable acrylic intraocular lens implantation in children

PURPOSE: To evaluate the long-term incidence of postoperative posterior capsular opacification (PCO) in children undergoing small incision foldable acrylic lens implantation with at least 2 years of follow up. METHODS: In 18 children, 26 eyes underwent small incision cataract extraction with posterior chamber foldable acrylic lens implantation. The posterior capsule was left intact in all patients at the time of surgery. RESULTS: With a mean follow up of 2.75 years and a mean age at surgery of 8.25 years, 13 of 26 eyes (50%) developed visually significant PCO requiring intervention. In the group of children under 4 years of age, 5 of 5 eyes (100%) developed visually significant PCO, while 8 of 21 eyes (38%) in the group of children over 4 years of age developed opacification. Four of 26 eyes (15%) required two procedures (either repeat Nd:YAG laser capsulotomy or pars plana secondary membrane removal) to clear the visual axis. CONCLUSION: In this study, the incidence of PCO following small incision acrylic lens implantation in children over 4 years of age is lower than those rates reported by conventional large incision rigid lens techniques with a minimum of 2 years follow up. This technique has advantages over conventional techniques in older children because it offers less surgical intervention, a lower cost to patients, and less risk of vitreous and retinal complications.     

Corneal keloid in Lowe syndrome

We describe a 3-year-old boy with Lowe syndrome who previously underwent bilateral cataract surgery with intraocular lens implantation and strabismus surgery and developed an inferior corneal keloid. The lesion was resected. We report the results of immunohistochemistry analysis of the specimen, as well as alternative treatment modalities for this rare condition.     

Bilateral blinding uveitis in a child after secondary intraocular lens implantation for unilateral congenital cataract

A 3-year-old boy underwent secondary implantation of an iris supported intraocular lens following extraction of a unilateral congenital cataract. Eight months later, he developed a bilateral plastic uveitis which, in spite of intensive medical treatment, resulted in NPL (no perception of light) in the implanted eye and 2/60 vision in the fellow eye. Sympathetic ophthalmia possibly played a role in the pathogenesis of the uveitis.     

High resolution ultrasound with Doppler: A diagnostic adjunct in orbital and ocular lesions in children

Twenty-six infants and children with orbital and ocular pathology were examined with ultrasound (US) utilizing real-time imaging and Duplex Pulsed Doppler evaluation. Twenty-two of these patients underwent concurrent orbital computed tomography (CT) and two had magnetic resonance imaging (MRI). Orbital and periorbital lesions included hemangioma, dermoid, lymphangioma, rhabdomyosarcoma, encephalocoele and abscess. Ocular lesions included infection, trauma, retinal detachment, retinoblastoma, Coat's disease, and persistent hyperplastic primary vitreous. High resolution US with Doppler provided unique diagnostic information in patients with penetrating ocular trauma, orbital and periorbital masses, and intraocular structural abnormalities. High resolution US examination of the eye and periorbital tissues is readily performed using widely available equipment and often delineates subtle structural abnormalities not shown by CT or MRI.     

Surgical technique, visual outcome, and complications of pediatric intraocular lens implantation.

PURPOSE: To evaluate retrospectively the surgical technique, visual outcome, and complications of pediatric cataract extraction (CE) and intraocular lens (IOL) implantation. METHODS: Forty-three patients ages 2 to 12 underwent CE with IOL implantation with a minimum follow up of 1 month. RESULTS: All IOLs were implanted in the posterior chamber with 17 (40%) in the bag, 25 (58%) sulcus fixated, and one (2%) partially in the bag (one haptic in the bag, one in the sulcus). Primary posterior capsulectomy was performed in 12 (28%) cases. A final visual acuity of at least 20/40 was achieved in 26 (60%) and at least 20/80 in 32 (74%). Posterior capsule opacification developed in 18 (42%) and pupillary capture in 7 (16%). Seventeen (40%) patients had postoperative visual acuity worse than 20/40. Of these, nine (53%) had this visual outcome as a result of presumed amblyopia. CONCLUSIONS: Posterior chamber IOL implantation affords a safe and effective method of visual rehabilitation for cataractous children 2 years of age and older. Amblyopia and antecedent posterior segment trauma, rather than IOL-related or surgical complications, are the limiting factors in final visual outcome.     

Results of early treatment of unilateral congenital cataracts

Twelve infants with unilateral congenital cataracts treated at Boston Children's Hospital between 1978 and 1986 have now reached the age of 3.5 years or older. All infants had cataract extractions, aphakic contact lens fitting, and occlusion of the unaffected eye by 6 months of age. Patients with posterior lenticonus or persistent hyperplastic primary vitreous were excluded from this review. Early improvement of visual acuity in the aphakic eye was monitored by preferential looking tests, and occlusion of the sound eye was adjusted accordingly. E card visual acuities are now available on all 12 children. Five patients have 20/70 or better visual acuity, three patients have between 20/100 and 20/400 vision, and four patients have less than 20/400 in the aphakic eye. Two patients whose cataract surgery was done after 4 months of age had the poorest visual results. Difficulty maintaining occlusion therapy and interruptions of contact lens wear limited the development of better vision in some patients. The visual results to date in these 12 patients suggest that early aggressive treatment of unilateral congenital cataracts is worthy of consideration in most instances.     

儿童横纹肌肉瘤23例临床分析

目的：探讨儿童横纹肌肉瘤的临床特点、治疗和转归。方法：对1998年1月至2008年10月收治的23例横纹肌肉瘤患儿临床资料进行回顾性分析。结果：23例患儿中,男15例,女8例,平均发病年龄5岁(7个月至12岁)。依据美国横纹肌肉瘤研究组(IRS)的分期标准,I期2例,Ⅱ期4例,Ⅲ期8例,Ⅳ期9例。原发于头颈部14例,四肢4例,膀胱2例,肾脏、腹膜后及胆道各1例。所有患儿均经病理活检及免疫组织化学染色确诊。临床表现无特异性,主要为肿瘤组织占位、压迫、浸润后引起。治疗严格依照患儿IRS分期进行。2002年前化疗方案以VDCA、VAC 和VadrC 为主,2002年后采用美国肿瘤学中心研究组（COG）横纹肌肉瘤化疗方案。其中19例接受手术、化疗和放疗综合治疗的患儿2年生存率为63%,4例接受单纯手术或手术结合单一化疗或放疗的患儿生存期均未超过2年。结论：儿童横纹肌肉瘤临床表现无特异性;联合手术、放疗、化疗是治疗横纹肌肉瘤的有效方法。