Case 6

A 12-year-old boy presented with a reddish brown, firm, dome-shaped tumor on his right arm. The tumor was 1.5 cm in diameter and its surface was smooth, without signs of ulceration. The boy's mother had noted a mild inflammatory reaction surrounding the tumor following minor injury, but no such reaction was seen at the time of surgery.     

Enhanced Thermal Conductivity of Thermoplastics by Lamellar Crystal Alignment of Polymer Matrices

Thermal conductivity (TC) of injection‐molded main‐chain smectic liquid‐crystalline polymers and the composites containing hexagonal boron nitride (h‐BN) particles is investigated. Shear flow during injection molding induces alignment of chain‐folding lamellar crystals of polymer matrices, in which polymer chains are aligned in the normal direction (ND) with respect to the molding surface, thus leading to a high TC (1.2 W m^?1 K^?1) in the ND. The composites exhibit a dramatic enhancement of TC in not only the ND but also the in‐plane direction. The enhanced TC is much higher than that of common thermoplastic composites at comparable loading levels. These results indicate that the polymer matrices serve as effective heat conductors between h‐BN particles.     

Influence of different keratoplasty techniques on the biomechanical properties of the cornea

Purpose: To evaluate and compare corneal biomechanical changes after deep anterior lamellar keratoplasty (DALK) versus penetrating keratoplasty (PK) using the ocular response analyzer (ORA). Methods: This prospective comparative study included 65 eyes (65 patients). Patients were divided into three groups: DALK (20 eyes), PK (25 eyes) and control group of normal subjects (20 eyes). Ocular response analyzer was used to measure corneal hysteresis (CH), corneal resistance factor (CRF), Goldmann‐correlated intraocular pressure and cornea‐compensated intraocular pressure at 6 and 12 months postoperatively. Central corneal thickness (CCT) was measured using ultrasonic pachymetry. Results: Both mean CH and mean CRF were significantly lower in PK group (sutures on; 10.1 ± 1.11 and 9.6 ± 1.08 mmHg) than in DALK (sutures out; 12.25 ± 1.13 and 12.09 ± 1.05 mmHg) and control groups (12.98 ± 1.19 and 12.59 ± 0.94 mmHg) at 6 months, respectively (p < 0.0001). After 1 year, there was no statistically significant difference in mean CH and CRF between DALK (12.68 ± 1.11 and 12.18 ± 1.11 mmHg) and PK groups (still sutures on; 12.36 ± 1.32 mmHg, p = 0.39 and 11.83 ± 1.26 mmHg, p = 0.33, respectively); however, the mean CRF was significantly lower in the PK than the control group (p = 0.03). No statistically significant difference in mean CH or mean CRF was found between DALK and control groups at any time‐point. Conclusions: Compared with PK, DALK provides rapid return of normal corneal biomechanics, perhaps because of the combined healing at both the deep interface and graft margin, shorter period of steroid treatment and the intactness of Descemet membrane in these eyes.     

自体角膜缘移植联合深板层角膜移植治疗单眼瘢痕期热、化学烧伤

目的 探讨自体角膜缘移植联合深板层角膜移植治疗单眼瘢痕期热、化学烧伤的临床疗效。方法 对单眼热、化学烧伤后，临床表现为角膜表面严重结膜化、新生血管化和瘢痕化的患者共39例(39眼)，行自体角膜缘移植联合深板层角膜移植术。观察术中并发症、术后眼表面的重建、角膜透明度恢复及术后视力提高等指标。结果 39眼中的34眼符合本研究的纳入标准。其中的32眼(94．1％)术后7d内完成角膜上皮化。重建的角膜上皮稳定，印迹细胞学检查表现为无杯状细胞的非角化鳞状上皮。34眼中的11眼术后出现双前房，10眼的双前房于1个月内消失。34眼中的29眼(85．3％)术后角膜透明，3眼有不同程度的角膜瘢痕，2眼出现持续性的角膜水肿。术后30眼(88．2％)视力显著提高。结论 自体角膜缘移植联合深板层角膜移植术能有效地重建单眼瘢痕期热、化学烧伤患者的眼表面，恢复角膜的透明性，明显提高视力。     

角膜移植治疗中重度眼化学伤并发症的疗效分析

目的对比评价不同术式角膜移植治疗中、重度眼化学烧伤并发症和后遗症的疗效。方法收集我院1993年2月至2005年2月手术治疗的中、重度化学眼烧伤27例34眼,分别行全角膜板层移植术11眼,带巩膜环全角膜板层移植术4眼及部分穿透性角膜移植术19眼。随访6～24个月。术后观察角膜植片愈合情况、排斥反应的发生、角膜植片透明度、新生血管、视力及术后并发症,并对出现情况给予相应处理。结果术后视力提高者25眼（73.5%）,视力不变者9眼（26.5%）。9眼植片透明,24眼半透明,1眼混浊。术后并发症有排斥反应15眼（44.1%）,继发性青光眼3眼（8.8%）,角膜新生血管34眼（100%）,假性胬肉复发12眼（35.3%）,感染1眼,层间积血3眼。结论角膜移植术是治疗中、重度眼化学烧伤并发症的有效方法。针对不同眼部病变选用不同术式可提高手术成功率。术前或术中眼部基本条件的改善对角膜移植术的成功至关重要。带巩膜环全角膜板层移植术对于伴有假性胬肉,且不伴有角膜穿孔的中、重度眼化学烧伤并发症是一种有效的治疗方法,优于全角膜板层移植术。部分穿透性角膜移植术对于单纯性角膜白斑、角膜穿孔者疗效较好。     

深板层角膜移植术患者的围术期护理

目的探讨深板层角膜移植术患者的围术期护理。方法2005年1月至2008年1月角膜组病房收治角膜边缘变性、蚕蚀性角膜溃疡21例共25只眼。患眼视力为指数～0．4不等,均行深板层角膜移植,并对患者进行充分的围术期护理。结果术后视力均有明显提高,无严重并发症发生,临床刺激症状明显改善,视力有不同程度的提高。结论深板层角膜移植术可以控制角膜边缘变性、蚕蚀性角膜溃疡的病情发展,及时的手术治疗,配合良好的心理护理、术前准备及有效的术后护理,可使角膜变性、溃疡患者视力得到最大程度的恢复。     

Outcomes of keratoplasty in a cohort of Indian patients with xeroderma pigmentosum

Purpose:To evaluate the outcomes of keratoplasty for xeroderma pigmentosum (XP) performed at a tertiary eye care center.Methods:A retrospective review of medical records of those patients who were clinically diagnosed to have XP (54 eyes of 36 patients) and underwent keratoplasty; either deep anterior lamellar keratoplasty (DALK, four eyes), endothelial keratoplasty (EK, eight eyes), or penetrating keratoplasty (PK, 42 eyes) from 1994 to 2018.Results:The median age at surgery was 20.6 years (interquartile range [IQR], 14.6–27.6 years) and 20 (55.6%) were males. Graft failure occurred in 15 eyes (35.7%) in the PK group and two eyes (50%) in the DALK group; none failed in the EK group. The probability of graft survival in the PK group was 97.2% ± 2.7% at 1 year, 74.0% ± 8.0% at 2 years, and 54.8% ± 11.7% at 5 years. In the PK group, 13 eyes needed antiglaucoma medications, 11 eyes developed graft infiltrate, and 13 eyes needed secondary interventions (cataract surgery, excision biopsy, and tarsorrhaphy). In the EK group, three eyes needed secondary interventions (excision biopsy). Median postoperative endothelial cell density at the last follow-up in the PK group was 1214 cells/mm² (IQR, 623–2277 cells/mm²).Conclusion:Despite the complexities of the ocular surface and adnexal issues in XP, keratoplasty had reasonably good outcomes. More than half of the PK grafts survived 5 years with no failures in the EK group. Regular follow-up and timely management of suture-related infections raised intraocular pressure, and suspicious ocular surface lesions, in addition to solar protection, are important for the success of keratoplasty in these eyes.     

Lamellar ichthyosis with pseudoexon activation in the transglutaminase 1 gene

We report a case of a 12‐year‐old boy who was born as a collodion baby after which thick scales developed on his entire body surface. His younger brother showed a similar skin condition. Arcuate‐shaped, large, brownish scales covered his face with ectropion. His lower legs were also covered with larger thick, brownish, plate‐like scales, but other areas were covered with smaller scales. Next‐generation sequencing for exons and splice sites detected a stop‐gain TGM1 mutation leading to p.R71* in transglutaminase 1 (TG1). Another mutation identified was a cryptic mutation in intron 3 that activated a pseudoexon, which was detected by RNA‐based analysis of hair bulbs. The deep intronic mutation caused another truncation mutation, p.N171Tfs^*45, in TG1. An in situ TG1 assay demonstrated that TG1 activity was totally lost in this case. Thus, we conclude that the severe phenotype of the patient developed due to those novel compound heterozygous null truncation mutations in TGM1.     

Severe ectropion in lamellar ichthyosis managed medically with oral acitretin

Congenital ectropion is commonly associated with lamellar ichthyosis. Severe eyelid ectropion may cause corneal exposure, keratopathy, and permanent corneal scarring. We report a neonate with severe, bilateral, congenital ectropion and eclabium managed using oral retinoids. Both corneas were protected with topical antibiotics and lubricating eyedrops and eye ointments. At 12‐month follow‐up, the child was doing well, with no ectropion or corneal opacity.     

板层角巩膜移植治疗角结膜皮样瘤12例

目的:探讨板层角巩膜移植术(LKP)治疗角结膜皮样瘤的疗效,并发症及处理方法。方法:对12例(12眼)角结膜皮样瘤患者施行肿瘤切除联合板层角巩膜移植术,观察6～24mo,对并发症及时给予局部及全身药物治疗。结果:术后视力与术前相比无明显变化,差异无显著性,P >0.05。随访6～24(平均14.5)mo,植片透明11眼(92%),半透明1眼(8%)均未出现肿瘤复发。1眼发生上皮型排斥反应。结论:LKP术是治疗角结膜皮样瘤,恢复正常眼表的一种理想方法。