CAJ | 학술논문

目的描述不同时期的家族性渗出性视网膜病变(familial exudativevitreoretinopathy,FEVR)患者,特别是新生儿期患者眼底病变的临床特征.方法回顾性系列病例研究.对2009～2013年在广州市妇女儿童医疗中心及珠海市妇幼保健院,经RetCam Ⅱ眼底检查确诊为FEVR患者的家族史及眼底病变特征进行分析,其中31例51只眼为新生儿患者,4例8只眼为其他年龄段患者.结果 35例患者中,2例4只眼有家族遗传病史,所有病人均无吸氧病史,且所有患者均为顺产足月儿,包括了1～5期各期的眼底病变.出生后48 h内进行眼底检查即可发现早期眼底病变,表现为出现分界线及不同范围的无血管灌注区,均未见渗出性病变.晚期病变则出现纤维条索视网膜脱离.其中1期27例44只眼,2期3例5只眼,3期3例6只眼,4期1例1只眼,5期1例3只眼(含4期1例另1只眼).结论 (1)按现行标准诊断的FEVR临床特征与早产儿视网膜病变十分类似,但很多FEVR视网膜病变发生在仍然处于视网膜发育阶段的母体内.在出生48h内婴儿中,发现的FEVR病例均为早期病变.(2)多数FEVR病人病变程度轻微,少部分FEVR患者病情可在短期内迅速发展至晚期.(3) FEVR早期病变通过早期激光治疗可以获得良好效果.
목적 묘술불동시기적가족성삼출성시망막병변(familial exudativevitreoretinopathy,FEVR)환자,특별시신생인기환자안저병변적림상특정.방법 회고성계렬병례연구.대2009～2013년재엄주시부녀인동의료중심급주해시부유보건원,경RetCam Ⅱ안저검사학진위FEVR환자적가족사급안저병변특정진행분석,기중31례51지안위신생인환자,4례8지안위기타년령단환자.결과 35례환자중,2례4지안유가족유전병사,소유병인균무흡양병사,차소유환자균위순산족월인,포괄료1～5기각기적안저병변.출생후48 h내진행안저검사즉가발현조기안저병변,표현위출현분계선급불동범위적무혈관관주구,균미견삼출성병변.만기병변칙출현섬유조색시망막탈리.기중1기27례44지안,2기3례5지안,3기3례6지안,4기1례1지안,5기1례3지안(함4기1례령1지안).결론 (1)안현행표준진단적FEVR림상특정여조산인시망막병변십분유사,단흔다FEVR시망막병변발생재잉연처우시망막발육계단적모체내.재출생48h내영인중,발현적FEVR병례균위조기병변.(2)다수FEVR병인병변정도경미,소부분FEVR환자병정가재단기내신속발전지만기.(3) FEVR조기병변통과조기격광치료가이획득량호효과.
Objective To describe the clinical characteristics of FEVR patients with different stage,especially the clinical characteristics of newbom.Methods Case series,retrospective review.The family history and fundus pictures of 35 patients (59 eyes) who diagnosed as FEVR were reviewed and analyzed.These patients were made a definite diagnosis as FEVR by RetCam Ⅱ in Guangzhou women and children medical center or the Zhuhai municipal maternal and children care from 2009 to 2013.Among these patients,31 cases of patients were neonatal patients while the 4 cases of patients with other age groups.Results In 35 cases of patients,4 eyes of 2 cases had familial heredity history and all the patients were without oxygen uptake.The fundus lesions of these cases included 5 stages.Fundus lesions of FEVR could be found within 48 hours after the birth by fundus examination which showed the line of demarcation and different range avascular area and without exudative lesions.With the fiber cords strained the retina; it may appearance exudate and retinal detachment in the later period of FEVR.Stage 1 FEVR was identified in 44 eyes of 27 cases,stage 2 in 33 eyes of 5 cases,stage 3 in 6 eyes of 3 cases,stage 4 in 1 eye of 1 case,and stage 5 in 3 eyes of 1 case (including 1 case 1 eye of stage 4).Conclusions According to the diagnostic criteria of FEVR at present,the presentation FEVR is similar to ROP.The lesion occurs when the FEVR patients still in the womb with the retina still in the stage of development.The lesion of retina is in the early stage of newborn babies whose age is within 48 hours.The lesion degrees of most FEVR patients are slightly,but the lesion of a few FEVR patients can be rapid development to later stage at short notice.By treating with laser in the early time,the FEVR patients with early stage can obtain a good outcome.