Clinical Features Of Microphthalmos-Anophthalmos-Coloboma And Observation On The Treatment Of Cataract Surgery

BackgroundMicrophthalmos-anophthalmos-coloboma(MAC)are a group of structurally and clinically correlated congenital ocular malformation,which are related with abnormal development of eyeball.In the same pedigree of MAC,different members may have the same or different phenotypes,and the same or different phenotypes can also appear between the eyes of the same person.At present,MAC are a major cause of blindness in children,which brings great pressure to the prevention and treatment of blindness.At the same time,due to the abnormal development of the eye and the special anatomical structure,MAC are often complicated with congenital or complicated cataract,angle-closure glaucoma,uveal effusion,retinal detachment,lens dislocation and other complications,which are difficult to be treated.Purpose1.To explore the clinical manifestation and treatment of MAC.2.To evaluate the efficacy and safety of cataract surgery in microphthalmos and coloboma.Methods1.The data of MAC patients hospitalized in Qilu Hospital of Shandong University from October 2014 to October 2022 were analyzed retrospectively.According to the specific eye manifestations,MAC were divided into the following four categories:(1)simple microphthalmos:microphthalmos without coloboma.(2)isolated coloboma:only coloboma occurred,and the ocular size was basically normal.(3)colobomatous microphthalmos:microphthalmos and coloboma exist in one eye at the same time.(4)extremely dysplastic eyeball:included anophthalmos as observed by slit-lamp microscopy and extreme microphthalmos in appearance.Compared and analyzed whether there were differences in best corrected visual acuity(BCVA),intraocular pressure(IOP)and ocular biological parameters which include axial length(AL),horizontal corneal diameter(HCD),anterior chamber depth(ACD)and keratometry(K).The main complications of MAC,including cataract,angle closure glaucoma,retinal detachment,uveal effusion,lens dislocation and treatment of complications were summarized.2.Selected the cases of microphthalmos and coloboma treated by cataract surgery,summarized the specific surgical methods,compared preoperative and postoperative BCVA and IOP,also summarized the complications of the operation.Results1.A total of 112 eyes were diagnosed with MAC,of which 45 eyes were simple microphthalmos,49 eyes were isolated coloboma,7 eyes were colobomatous microphthalmos,11 eyes were extremely dysplastic eyeball.In terms of BCVA,60.7%of MAC were blind(BCVA<0.05),25.0%were low vision(0.005≤BCVA<0.3),and the number of eyes with BCVA≥0.3 was only 14.3%;the BCVA of colobomatous microphthalmos was worse than that of simple microphthalmos and isolated coloboma.In terms of IOP,the IOP of simple microphthalmos was higher than that of isolated coloboma.In terms of biological parameters,the AL of simple microphthalmos and colobomatous microphthalmos was shorter than that of isolated coloboma;the ACD of simple microphthalmos was shallower than that of isolated coloboma;the Kf and Ks were higher than those of isolated coloboma;there was no significant difference in HCD among the three groups.In terms of complications,the prevalence rates of cataract,glaucoma,retinal detachment,uveal effusion and lens dislocation in MAC were 68.3%,23.8%,15.8%,6.9%and 5%,respectively;the prevalence rate of glaucoma in simple microphthalmos was higher than that in isolated coloboma(P<0.05);the prevalence rate of retinal detachment in isolated coloboma was higher than that in simple microphthalmos(P<0.05);uveal effusion only occurred in simple microphthalmos;lens dislocation only occurs in coloboma,which including isolated coloboma and colobomatous microphthalmos.In terms of treatment,in MAC,the rates of cataract extraction,vitrectomy,glaucoma and sclerectomy were 36.6%,29.7%,16.8%and 4.0%,respectively.2.By cataract surgery,the BCVA of simple microphthalmos was improved(P<0.05)and the IOP was decreased(P<0.05);the BCVA of simple microphthalmos with AL>18mm was improved(P<0.05),but there was no significant difference in IOP(P>0.05);the BCVA of simple microphthalmos with AL<18mm was not significantly different from preoperative BCVA(P>0.05),but the IOP was decreased(P<0.05);the BCVA of isolated coloboma was increased(P<0.05),but there was no significant difference in IOP(P>0.05).The BCVA of colobomatous microphthalmos was slightly improved in 1 eye after surgery,and IOP was in the normal range before and after surgery.A total of 8 eyes(21.6%)had operative complications,including 2 eyes of simple microphthalmos,5 eyes of isolated coloboma and 1 eye of colobomatous microphthalmos.Conclusion1.The visual acuity of MAC are poor,and the proportion of blind eyes are relatively high.Simple microphthalmos and colobomatous microphthalmos have shorter AL;simple microphthalmos has shallower ACD and steeper cornea than isolated coloboma;although there is no measurement of ACD and Kf or Ks in colobomatous microphthalmos,we speculate that it is similar to simple microphthalmos in ACD and Kf or Ks.Cataract is a common ocular complication of MAC;simple microphthalmos is more likely to be combined with angle-closure glaucoma and uveal effusion;coloboma is more likely to be combined with retinal detachment and lens dislocation.Complications required related surgical treatment.2.Phacoemulsification is safe and effective for simple microphthalmos with AL>18mm.For simple microphthalmos with AL<18mm,cataract surgery may cause serious complications,and the visual prognosis is poor.For colobomatous microphthalmos,cataract surgery is only to maintain the existing state and function of eyeball.Cataract surgery is also safe and effective for coloboma,but posterior capsule rupture may occur during surgery.