先天性瞳孔残膜误诊为无瞳孔1例

患者，男，9岁。因双眼视力不好9年余于2004年8月11日入院。其父母代诉患儿自出生后视力不好，注意力差，曾到昆明等地就诊。近年来视力下降加重，于当日到本院就诊，以“双眼先天性瞳孔闭锁”住院。入院查体温37．5，心率84次／min，呼吸20次／min，体重22妇，一般情况可，神清，查体合作，心肺阴性，肝脾未及。眼科情况：右眼视力4．1，左眼视力指数／眼前，双眼球水平震颤，     

Nd：YAG激光切除术治疗影响视力的先天性瞳孔残膜

用Ｎｄ：ＹＡＧ激光节除影响视力的先天性瞳孔残膜８５例。残膜被成功地切除后，除１３眼仍有废用性弱视外，视力均有明显提高，其中矫正视力为０．８－０．９者占３５．０％，＞１．０者占６５．０％。弱视眼视力为０．１－０．２，经过０．５－１．０年的弱视训练治疗，视力均优于０．６。无明显并发症。     

先天性瞳孔残膜的手术治疗

目的评价先天性瞳孔残膜手术治疗的疗效.方法对18例24眼膜性先天性瞳孔残膜进行手术切除,术后10天对合并有斜视的患者行斜视矫正术.根据弱视性质进行综合治疗,随访疗效.就残膜切除的手术方法、技术要点和术后治疗进行分析.结果膜性先天性瞳孔残膜的手术治疗22眼完整切除残膜,2眼晶体前囊破裂,改行晶状体囊外摘除术联合人工晶体植入术.随访6月～3年(平均12.2月),视力全部提高至0.5或0.5以上,治愈率(视力≥1.0)达20.8%,切除残膜的22眼瞳孔圆形,对光反射灵敏,晶状体透明,无远期并发症.结论通过手术切除的方法治疗先天性瞳孔残膜效果满意,但应注意手术技巧和术后治疗.     

瞳孔成形术治疗双眼先天性瞳孔残膜一例

6岁女性,双眼视力差。双眼瞳孔区丝状及膜状虹膜残留,覆盖大部分瞳孔区及视轴,周边丝状膜与虹膜相连。诊断双眼先天性瞳孔残膜。行双眼瞳孔成形术,术后瞳孔圆,晶状体前囊可见少许虹膜色素沉着。(眼科,2022,31:452-453)     

双眼先天性膜状瞳孔残膜

0引言瞳孔残膜又称永存瞳孔膜,系胎儿期瞳孔膜的残留。在新生婴儿中常可观察到不同程度的瞳孔残膜,但随年龄增长,大部分婴儿眼内残膜逐渐消失。     

透明角膜小切口无缝合切除先天性瞳孔残膜

目的：探讨治疗严重的先天性瞳孔残膜的新途径。方法：4例（6眼）通过颞侧透明角膜隧道切口、前房注入粘弹剂后,将瞳孔残膜紧贴虹膜附着处剪断。结果：术后无一例高眼压及前房出血,随访1年以上,3例（4眼）晶状体保持透明。术后有0．12－0．25D角膜散光,角膜内皮细胞少量丢失。结论：通过颞侧透明角膜隧道切口切除先天性瞳孔残膜,术后并发症少,散光小,病人恢复快,是治疗严重瞳孔残膜的理想手术方法。眼科学报2001;17：76－77。     

瞳孔残膜的治疗

瞳孔残膜又称永存瞳孔膜，严重者影响视力。本文采用广东威达医疗器械公司生产的DI92型染料激光机治疗严重瞳孔残膜2例，4眼。效果良好，无并发症，现报告如下。病例报告：冽1；何XX，男，7岁。家长发现多个瞳孔7年。无家族史。全身及其它脏器检查无异常。视力：右眼0．4，左眼0．3o双眼指测眼压正常，外眼光光无异常。双眼虹膜纹理清晰，瞳孔直径4Inln，薄片状残膜位于瞳孔中央，并由多条色素条带与瞳孔相连，瞳孔光反射灵敏。散瞳后由瞳孔间隙见到晶状体透明。眼底无法窥见。例2：李x，男、ste。视物不清3年。无家族史。全身其它""-""'"…     

双眼典型先天性瞳孔残膜1例

瞳孔残膜,与虹膜基质前层增殖不同,是一种先天性的发育异常,笔者临床发现1例,现报告如下。1　临床资料患者,男,15a,学生,自发现双眼“虹膜增生”后就诊,双眼1a前出现“近视”,配镜至今,不伴其它全身不适,查体裸眼视力OD:1.0,OS:0.8,矫正OS-0.50DS1.0,双外眼未见异常,角膜?..     

先天性瞳孔残膜误诊为无瞳孔1例

1、病历摘要 患者，男，9岁。因双眼视力差9年余，于2004年8月11日入院，其父母代诉患儿自出生后视力差，注意力差，曾经到昆明等地就诊，经过不详，近年来视力下降加重，于今日到本院就诊，以“双眼先天性瞳孔闭锁”收住院，入院查体：T37．5℃，P84次／min，R20／min，体重22kg，一般情况可，神志清，     

Paradoxical pupil in congenital achromatopsia

Children with congenital achromatopsia possess an interesting paradoxical pupillary constriction to darkness that has not been previously described. This paper describes three children in whom this paradoxical pupillary reaction was documented with infrared pupillometry. The literature on the pupil in achromatopsia is reviewed and a tentative testable model of the reaction is hypothesized.This work was supported in part by Public Health Service Institutional National Research Study Award # EY 07021 from the National Institutes of Health, Bethesda, Maryland and National Institutes of Health Research Grant 1236-03-04.     

先天性脉络膜缺损2例

脉络膜缺损属先天性眼底异常,近20年来,国内报道并不多见。我们近半年来收到脉络膜缺损病例2例,现报告如下。病例1,女,17岁。于2008年6月19日至本院就诊。患者自诉半年前因近视开始配戴硬性角膜接触镜,现右眼视力矫正至0.8,左眼戴镜后视力没有提高,当地医院未查明病     

Two cases of hereditary keratoderma with congenital glaucoma

Abstract
Two cases are reported: a father and son with a new syndrome consisting of a severe congenital glaucoma associated with a form of palmoplantar keratoderma showing features of mutilating palmoplantar keratoderma. Both the glaucoma and keratoderma may be attributed to an autosomal dominantly inherited genetic mutation of a single chromosome affecting two tissues of neural crest origin, the ectodermal tissues of the hands and feet and the cranial mesenchyme from which the tissues of the chamber angle develop. Elucidation of the precise chromosomal abnormality causing these defects may help in understanding the inheritance and aetiology of glaucoma.     

Two cases of hereditary keratoderma with congenital glaucoma

Two cases are reported: a father and son with a new syndrome consisting of a severe congenital glaucoma associated with a form of palmoplantar keratoderma showing features of mutilating palmoplantar keratoderma. Both the glaucoma and keratoderma may be attributed to an autosomal dominantly inherited genetic mutation of a single chromosome affecting two tissues of neural crest origin, the ectodermal tissues of the hands and feet and the cranial mesenchyme from which the tissues of the chamber angle develop. Elucidation of the precise chromosomal abnormality causing these defects may help in understanding the inheritance and aetiology of glaucoma.     

Two cases of CHARGE syndrome with multiple congenital anomalies

We report on two cases of bilateral chorioretinal colobomas with ocular anomalies in patients with CHARGE syndrome. In the first case, a female infant was born at 36 + 5 weeks gestation. At birth, the patient demonstrated a small left eye. Slit-lamp examination revealed colobomas of both irises. Fundus examination showed both chorioretinal colobomas. Brain magnetic resonance imaging (MRI) showed left microphthalmia. Systemic evaluation revealed multiple congenital anomalies: benign external hydrocephalus, esophageal atresia with imperforate anus, atrial septal defect (ASD), ventricular septal defect, patent ductus arteriosis (PDA), and right mild hydronephrosis. In the second case, a male infant was born at 39 + 5 weeks gestation and demonstrated a dysmorphic appearance with an irregular left pupil and ptosis. Fundus examination of both eyes showed large chorioretinal colobomas involving the optic disc and posterior pole. The patient had multi-organ anomalies: right facial palsy, a left short, wide ear with a small lobe, congenital heart defects, such as ASD and PDA, left renal atresia, seizure disorder, and micropenis. Both cases revealed multiple anomalies including nearly all major and minor criteria of CHARGE syndrome which could be life-threatening to neonates. Thus, all neonates with ocular colobomas should have fully and detailed systemic examinations checking all minor criteria and even occasional findings of CHARGE syndrome.     

The pupil in congenital third nerve misdirection syndrome

The involved pupil in the syndrome of aberrant regeneration of the oculomotor nerve usually remains larger than the uninvolved side, or approaches equality in size. A 4-year-old child was seen with congenital aberrant regeneration of the right third nerve. The right pupil was consistently smaller than the left, but particularly so in dim illumination. The question can be raised as to whether relative pupillary miosis more commonly follows third nerve misdirection in children than adults.     

Relationship between residual aberration and light-adapted pupil size.

PURPOSE: The relationship between residual aberration (residual astigmatism and higher-order aberration) and light-adapted pupil size was investigated in human subjects to verify that the pupil size is adjusted to provide good image quality on the retina through control of the image sharpness and illuminance. METHODS: Monochromatic wave aberration and light-adapted pupil diameters were measured from 20 subjects. The measured wave aberration was reconstructed using a sixth-order Zernike polynomial expansion; the neural sharpness was computed as a metric of the residual aberration. Subsequently, the correlation of the neural sharpness and the light-adapted pupil diameter was examined statistically across the subjects in each luminance level. RESULTS: The light-adapted pupil diameter showed a significant positive correlation with the neural sharpness, except under dark-adapted conditions. In contrast, the pupil diameter showed no significant correlation with the spherical equivalent, as previously shown. CONCLUSION: Our results suggest that the level of aberration present in an eye has an influence on pupil size.