Long-Term Visual Results After Pars Plicata Lensectomy- Vitrectomy for Congenital Cataracts
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Br J Ophthalmol: first published as 10.1136/bjo.72.8.601 on 1 August 1988. Downloaded from British Journal of Ophthalmology, 1988, 72, 601-606 Long-term visual results after pars plicata lensectomy- vitrectomy for congenital cataracts STEPHEN A GROSSMAN' AND GHOLAM A PEYMAN2 From the 'Department of Ophthalmology, Eye and Ear Infirmary, University of Illinois College of Medicine at Chicago, and 2S U Eye Center, Louisiana State University Medical Center School of Medicine, New Orleans, USA SUMMARY We performed a pars plicata lensectomy-vitrectomy on 32 patients (47 eyes) with congenital cataracts. Ocular abnormalities, mainly nystagmus, strabismus, and microphthalmia, were present in 29 patients. No complications occurred intraoperatively or postoperatively in 39 eyes with up to 81/2 years' follow-up (average 2-2 years). The pars plicata approach is a good surgical technique for the management of congenital cataracts. and developmental Congenital and early developmental cataracts have who have had congenital copyright. resulted in a large percentage of cases of childhood cataracts managed with this approach. blindness.2 The surgical management of such eyes has been debated in the literature for over 50 years. Material and methods Early techniques such as discission, linear extraction, and intracapsular extraction were plagued by a Thirty-two patients (47 eyes) were referred to the persistently high rate of surgical complications.?7 vitreous service of the University of Illinois Eye and More recently the discission and aspiration technique Ear Infirmary between 1974 and 1986. Six patients for congenital cataracts has been advocated.'" between the ages of 5 weeks and 18 years had a http://bjo.bmj.com/ However, the incidence of complications with this unilateral cataract. In only one was an autosomal technique, including vitreous loss, secondary dominant mode of inheritance suggested by family glaucoma, retinal detachment, and, in particular, history; no aetiology was identified for the remaining secondary membrane formation, remains quite high. five patients. Despite the use of phacoemulsification instruments, Twenty-seven patients between the ages of3 weeks postoperative complications often necessitated addi- and 10 years had bilateral cataracts. Bilateral tional procedures during the critical period of visual lensectomy-vitrectomy was performed in 15, while 10 on September 24, 2021 by guest. Protected development. I'll of the remaining 11 patients underwent another A major innovation in the management of con- procedure on the contralateral eye. In five patients genital cataracts has been the application of auto- the congenital cataracts were attributed to maternal mated vitrectomy instruments."'6 Both translimbal infection with rubella. All five also had micro- and transciliary body approaches are in use. 17-"9 phthalmia. Three of these patients had valvular heart While the anterior segment approach through the defects, deafness, and mental retardation as well. limbus may effectively produce an optically clear Three patients had dominantly inherited congenital visual axis, the procedure is complicated by a high cataracts. In addition one patient had Down's syn- incidence of cystoid macular oedema and by vitreous drome, another had mental retardation and a seizure incarceration in the limbal incision.2" We have disorder, and a third was diagnosed with Peters' performed pars plicata lensectomy-vitrectomy in anomaly. Among the other patients one was born congenital cataracts for over 12 years with good with raised titres to cytomegalovirus; one had a surgical results and a low incidence of postoperative congenital herpesvirus infection resulting in hydro- cystoid macular oedema.22-' This report describes cephalus, severe mental retardation, and cataracts; the long-term visual outcome of infants and juveniles and one had microphthalmia with cataracts and Correspondence to Dr Gholam A Peyman, 2020 Gravier Street, multiple craniofacial anomalies. Suite B.; New Orleans, LA 70112, USA. Before surgery, whenever possible, all eyes were 601 Br J Ophthalmol: first published as 10.1136/bjo.72.8.601 on 1 August 1988. Downloaded from 602 Stephen A Grossman and Gholam A Peyman examined for corneal size, intraocular pressure, our patients were seen for follow-up at our institution clarity of the media, and visual acuity. Muscle for less than two years. However, the median visual balance and the presence of nystagmus were noted. acuity of those receiving visual rehabilitation for at None had undergone previous surgery. All pro- least two years was 20/100. The patients' best cedures were performed with the patients under corrected visual acuities were recorded preopera- general anaesthesia. tively and at the time of the last visit. No surgical or postoperative complications occur- SURGICAL PROCEDURE red in 39 eyes. Two patients had transient corneal The eye was maximally dilated preoperatively. After oedema postoperatively that resolved spontaneously surgical preparation and sterile draping 4-0 black silk over several days. One patient had transient corneal sutures were used to retract the eyelids. The con- oedema and intravitreal bleeding from the iris. The junctiva was opened through a limbal peritomy, and child with Down's syndrome had bilateral cystoid bridle sutures were placed beneath the rectus macular oedema noted by fluorescein angioscopy muscles. In infants a 3-5 mm long sclerotomy was four months postoperatively, which cleared by 32 made 2-0 to 2-5 mm behind and parallel to the limbus months. One case of increased intraocular pressure with a no. 67 Bard-Parker blade. In older children the and iritis in the immediate postoperative period sclerotomy was made 3 mm from the limbus. responded to acetazolamide and steroid drops. Three The ciliary body was exposed at the sclerotomy site months postoperatively this patient suffered a retinal and gently diathermised. A 5-0 polyglactin 910 detachment that was treated successfully with a (Vicryl) or 7-0 polyfilament (Supramid) mattress scleral buckle and cryotherapy. One eye had a retinal suture was passed through the sclerotomy and tied dialysis intraoperatively. Another patient had a with a loose double-throw knot. A 52-s Beaver blade retinal detachment two weeks postoperatively; both was inserted into the lens with a slicing motion and conditions were treated successfully. No other retinal removed for insertion of a wide-angle Peyman vitro- detachments were observed up to 81/2 years after pars phage.27 If the blade did not easily penetrate the plicata lensectomy-vitrectomy (Table 1). copyright. centre of the lens (rare in children), a Peyman lens Of the 10 patients whose contralateral eye had a fragmentor was used to pulverise the lens nucleus for procedure other than the pars plicata lensectomy- about 60 seconds.2x The wide-angle cutter was placed vitrectomy, five had secondary membrane forma- in the eye, the knot was tightened, and the lens tion. Discission and aspiration in one such case material was cut and aspirated. The lens capsule was resulted in increased intraocular pressure, choroidal not removed until the entire nucleus and cortex were atrophy, and fibrous ingrowth of the cornea; post- aspirated. The lens material behind the iris was operative visual acuity was light perception. The sucked into the vitrophage and brought into the right eye, which underwent a pars plicata http://bjo.bmj.com/ pupillary space to be cut and removed, followed by lensectomy-vitrectomy, suffered no complications; the anterior and posterior capsule. postoperative visual acuity was 20/60. If the pupil did not dilate, a sector iridectomy None of the patients whose contralateral eye had was performed with the vitrophage. An anterior an alternative procedure had a better final visual vitrectomy was preceded by a deep vitrectomy con- acuity in that eye than in the eye that had pars plicata fined to the central core down to the optic disc. A lensectomy-vitrectomy (Table 2). modified Goldmann posterior pole lens and an Results of the long-term outcome in our monocu- on September 24, 2021 by guest. Protected operating microscope were used for direct visualisa- lar cases are hindered by a lack of follow-up informa- tion. The vitrophage was then removed, and the tion. However, the children with acquired monocular scleral mattress suture was tightened until the cataracts who were operated on at ages 4, 18, and 10 sclerotomy was watertight. If the eye was soft, years, respectively, showed an improvement of 5 and intraocular pressure was re-established with an injec- 6 lines on the Snellen chart in two cases and from tion of normal saline solution through the sclerotomy counting fingers to 20/50 in the third. There was one site- into the vitreous chamber with a 30-gauge case of a choroidal detachment postoperatively that needle. The conjunctiva was closed with 6-0 plain resolved after six weeks (Table 3). catgut, and atropine and Maxitrol (neomycin, poly- All but four patients with bilateral congenital mixin B, and dexamethasone) drops were instilled, cataracts had nystagmus, strabismus, or both. Two and patch and shield were placed. (patients 14 and 23) had a visual acuity of 20/200 preoperatively. One (patient 13) with central, Results steady, and maintained vision preoperatively was operated on after 3 years of age. Severe mental Our follow-up has ranged from one week after retardation in the fourth patient (patient 8) made discharge to 11 years (average 2-2 years). Nineteen of accurate visual assessment difficult.