Fabrication of a facial prosthesis for a 13-year-old child by using a point-and-shoot three-dimensional scanner and CAD/CAM technology.

Patients cannot wear ocular prostheses after undergoing orbital exenteration. They require a facial prosthesis to obtain a more favorable appearance, which greatly affects their social life and psychological health. In addition, conventional prosthesis-making processes require substantial time and expense. The economic burden is particularly heavy on children, who may require many prosthesis replacements as they mature. We report a method of fabricating a facial prosthesis by three-dimensional (3D) facial scanning and 3D printed for a 13-year-old girl who underwent partial orbital exenteration for malignant ciliary body medulloepithelioma 2 years ago. The patient's facial contour was captured with a hand-held, point-and-shoot 3D scanner. A facial prosthesis was designed using a mirror image technique with 3D modeling software and 3D printed. The prosthesis was then postprocessed and cast in silicone rubber. An ocular prosthesis was integrated into the facial prosthesis. The prosthesis was retained by prosthetic adhesives. This digitally assisted, impression-free method may lower the cost and effort of making facial prostheses and improve patient comfort, especially for children.

Porcine reproductive and respiratory syndrome virus non-structural protein 4 cleaves guanylate-binding protein 1 via its cysteine proteinase activity to antagonize GBP1 antiviral effect.

Porcine reproductive and respiratory syndrome (PRRS) is a highly infectious disease caused by PRRS virus (PRRSV) that causes great economic losses to the swine industry worldwide. PRRSV has been recognized to modulate the host antiviral interferon (IFN) response and downstream interferon-stimulated gene expression to intercept the antiviral effect of host cells. Guanylate-binding proteins (GBPs) are IFN-inducible GTPases that exert broad antiviral activity against several DNA and RNA viruses, of which GBP1 is considered to play a pivotal role. However, the role of GBP1 in PRRSV replication remains unknown. The present study showed that overexpression of GBP1 notably inhibited PRRSV infection, while the knockdown of endogenous GBP1 promoted PRRSV infection. The K51 and R48 residues of GBP1 were essential for the suppression of PRRSV replication. Furthermore, GBP1 abrogated PRRSV replication by disrupting normal fibrous actin structures, which was indispensable for effective PRRSV replication. By using a co-immunoprecipitation assay, we found that GBP1 interacted with the non-structural protein 4 (nsp4) protein of PRRSV, and this interaction was mapped to the N-terminal globular GTPase domain of GBP1 and amino acids 1-69 of nsp4. PRRSV infection significantly downregulated GBP1 protein expression in Marc-145 cells, and nsp4, a 3C-like serine proteinase, was responsible for GBP1 cleavage, and the cleaved site was located at glutamic acid 338 of GBP1. Additionally, the anti-PRRSV activity of GBP1 was antagonized by nsp4. Taken together, these findings expand our understanding of the sophisticated interaction between PRRSV and host cells, PRRSV pathogenesis and its mechanisms of evading the host immune response.

Debulking Surgery Combined with Low-Dose Oral Prednisolone and Azathioprine for Intractable IgG4-Related Orbital Disease: A Case Report.

A case of intractable IgG4-related orbital disease (IgG4-ROD) was successfully treated by debulking surgery combined with low-dose prednisolone and azathioprine as maintenance therapy. A 64-year-old man visited our clinic with progressive bilateral upper eyelid swelling and right eye fullness of a year's duration. He was previously treated with systemic corticosteroids for the IgG4-ROD and experienced a partial clinical response but relapsed upon prednisolone cessation. The patient underwent debulking surgery of the right lacrimal gland and right upper eyelid. His post-operative medication was oral prednisolone (5 mg) every other day and 50 mg azathioprine per day. The patient's right eye remained asymptomatic during the 18 months of follow-up. Debulking surgery combined with low-dose prednisolone and azathioprine, as a maintenance therapy, is an effective and alternative treatment for the long-term control of intractable IgG4-ROD.

Early Versus Late Canalicular Laceration Repair Outcomes.

PURPOSE: To determine whether delayed repair of traumatic canalicular laceration affects the final outcome. DESIGN: Retrospective case series. METHODS: The medical records of 334 patients who underwent primary traumatic canalicular laceration repair were retrospectively reviewed. Patients were divided into 2 groups according to the surgical timing within 48 hours (early) or after 48 hours (delayed). The anatomic results were compared between these 2 groups. The causes of delayed repair and the mean operation time were also analyzed. RESULTS: There were 23 failed cases among 301 patients (7.6%) who had a repair within 48 hours and 3 failed cases among 33 patients (9.1%) who had a repair after 48 hours (P = .732). The mean operation time was 62 minutes in the early group and 66.3 minutes in the delayed group, which showed no significant difference (P = .371). The major cause of delayed surgery was traumatic brain injury, followed by facial or orbital fracture, long bone fracture, and chest injury. CONCLUSIONS: Delayed canalicular repair in unstable patients did not lead to poor results. An elective scheduling surgery, instead of an urgent repair, is feasible for an experienced surgeon.

Malignant Ciliary Body Medulloepithelioma With Brain and Parotid Metastasis.

A 12-year-old girl with malignant ciliary medulloepithelioma and parotid metastasis was treated with semi-exenteration of the orbit and external beam radiotherapy. She had brain metastasis 7 months later and was treated with salvage chemotherapy. The patient was disease free at 11 months of follow-up. In aggressive cases, exenteration combined with radiotherapy and chemotherapy are necessary for disease control. [J Pediatr Ophthalmol Strabismus. 2017;54:e18-e22].

Factors Associated With Surgical Outcomes in Congenital Ptosis: A 10-Year Study of 319 Cases.

PURPOSE: To evaluate the 10-year results of surgery for congenital ptosis and identify factors associated with excellent outcomes and recurrence. DESIGN: Retrospective, interventional case series. METHODS: A retrospective analysis was conducted of 319 patients who underwent surgical correction at a tertiary medical center for congenital ptosis. The main outcome measures were postoperative surgical outcomes and recurrence rates. Excellent lid height was assessed as a marginal reflex distance (MRD) greater than 3 mm. Recurrence was defined as a lid height less than 50% of the initial postoperative lid height. RESULTS: The overall surgical success rate was 97.2%. On multivariate analysis, 3 factors were significantly associated with a greater probability of achieving excellent lid height: treatment using levator muscle resection (LMR) (adjusted odds ratio [OR], 1.76; P = .04), better preoperative MRD (adjusted OR, 2.21; P < .001), and absence of Marcus Gunn (jaw-winking) syndrome (adjusted OR, 0.12; P = .01). For recurrence, 7 significant risk factors were identified: children less than 1 year old (adjusted OR, 4.92; P = .02), poorer preoperative MRD (adjusted OR, 0.64; P = .04), poorer postoperative MRD (adjusted OR, 0.32; P < .001), treatment with frontalis suspension (FS) (adjusted OR, 5.86; P < .001), wound infection (adjusted OR, 9.45; P = .02), postoperative entropion (adjusted OR, 11.25; P = .003), and conjunctival prolapse (adjusted OR, 7.10; P = .03). Kaplan-Meier analysis showed that the 1-, 5-, and 10-year recurrence-free rates were 97.3% ± 1.2%, 80.5% ± 4.4%, and 76.7% ± 5.6%, respectively, for the LMR group and 90.9% ± 3.1%, 42.9% ± 8.1%, and 20.8% ± 10.1%, respectively, for the FS group (P < .001, log-rank test). CONCLUSIONS: Surgical treatment of congenital ptosis had a high success rate. Identifying the risk factors and taking appropriate measures may result in better surgical outcomes and less recurrence. Our retrospective study showed that the likelihood of achieving excellent outcomes with lower recurrence rates was higher with LMR than with FS. However, a prospective randomized study is necessary to clarify their efficacy.

Expression of retinoic acid-binding proteins and retinoic acid receptors in sebaceous cell carcinoma of the eyelids.

BACKGROUND: Sebaceous cell carcinoma of the eyelid is a malignant tumor. However, the pathoetiology of sebaceous cell carcinoma is not clear. Retinoic acid (RA) signaling is essential for skin epidermal differentiation including the eyelids. In this study, we investigate the expression of ß-catenin, RA-binding proteins and RA receptors in sebaceous cell carcinoma of the eyelid and try to estimate their influence on its pathoetiology. METHODS: Retrospective, noncomparative, consecutive interventional case series. Sixteen cases of eyelid sebaceous gland carcinoma who received tumor excision at our hospital between 2001 and 2011 were included. Immunohistochemical staining for ß-catenin, cellular retinoic acid binding protein 1 (CRABP1), cellular retinoic acid binding protein 2 (CRABP2), fatty acid-binding protein 5 (FABP5), retinoic acid receptors (RAR-α, -ß, -γ), and retinoid X receptors (RXR-α, -ß, -γ) was performed on tissue samples obtained from tumor excision. RESULTS: Of the 16 sebaceous cell carcinoma cases reviewed, six were male and 10 female. The mean follow-up period was 6.7 ± 3.66 years (range, 0.3-13 years). Of these 16 cases, the expression of ß-catenin was significantly increased in sebaceous cell carcinoma cases. CRABP1 was similarly expressed in the sebaceous cell carcinoma and control groups. CRABP2 and FABP5 were expressed in hair follicles of lid skin in both groups, whereas the CRABP2 and FABP5 were aberrantly expressed in the tumor cells of the sebaceous glands. Notably, the expression of retinoic acid receptor (RAR-ß) and retinoid X receptors (RXR-ß, -γ) was significantly upregulated in sebaceous cell carcinoma of the eyelids. CONCLUSIONS: Our findings indicate that retinoic acid signaling is related to the pathogenesis of sebaceous cell carcinoma of the eyelids.

Use of extraocular muscle flaps in the correction of orbital implant exposure.

PURPOSES: The study is to describe a new surgical technique for correcting large orbital implant exposure with extraocular muscle flaps and to propose a treatment algorithm for orbital implant exposure. METHODS: In a retrospective study, seven patients with orbital implant exposure were treated with extraocular muscle flaps. All data were collected from patients in Chang Gung Memorial Hospital, Taiwan during 2007-2012. All surgeries were performed by one surgeon (Y.J.T). Patient demographics, the original etiology, details of surgical procedures, implant types, and follow-up interval were recorded. Small exposure, defined as exposure area smaller than 3 mm in diameter, was treated conservatively first with topical lubricant and prophylactic antibiotics. Larger defects were managed surgically. RESULTS: Seven patients consisting of two males and five females were successfully treated for orbital implant exposure with extraocular muscle flaps. The average age was 36.4 (range, 3-55) years old. Five patients were referred from other hospitals. One eye was enucleated for retinoblastoma. The other six eyes were eviscerated, including one for endophthalmitis and five for trauma. Mean follow-up time of all seven patients was 19.5 (range, 2-60) months. No patient developed recurrence of exposure during follow-up. All patients were fitted with an acceptable prosthesis and had satisfactory cosmetic and functional results. CONCLUSIONS: The most common complication of orbital implant is exposure, caused by breakdown of the covering layers, leading to extrusion. Several methods were reported to manage the exposed implants. We report our experience of treating implant exposure with extraocular muscle flaps to establish a well-vascularized environment that supplies both the wrapping material and the overlying ocular surface tissue. We believe it can work as a good strategy to manage or to prevent orbital implant exposure.

Analysis of visual outcomes and complications following levator resection for unilateral congenital blepharoptosis without strabismus.

BACKGROUND: It is challenging to manage congenital blepharoptosis, especially unilateral, because symmetry is difficult to achieve under general anesthesia and age at which the ptosis should be corrected is still controversial. The aim of our study is to analyze visual and surgical outcomes after levator resection for unilateral congenital blepharoptosis. METHODS: Charts of patients with unilateral congenital blepharoptosis who underwent levator resection at the Chang Gung Memorial Hospital from 1991 through 2000 were reviewed. The resultant database was interrogated for demographic data, severity, surgical timing, visual outcomes, surgical outcomes, and complications. RESULTS: Eighty-four children underwent levator resection for unilateral congenital blepharoptosis: 16.7% of these patients had amblyopia and 84.5% had surgical success following levator resection. Severe ptosis (p = 0.0288, p < 0.05) and surgery at less than 2 years of age (p = 0.0126, p < 0.05) were the important factors contributing to surgical failure. Age at surgery (p = 0.0058, p < 0.01) and amblyogenic ametropia (p = 0.0001, p < 0.001) were found to be significantly associated with the postoperative visual results. CONCLUSION: The levator resection provides satisfactory results both in function and cosmesis in patients with unilateral congenital blepharoptosis. Amblyogenic ametropia is the leading cause of amblyopia in the patients with unilateral isolated congenital blepharoptosis. However, patients with unilateral congenital blepharoptosis should have cycloplegic refraction as early as possible, and their visual status monitored until visual maturity.

Comparing pericanalicular sutures with direct canalicular wall sutures for canalicular laceration.

PURPOSE: To determine whether a statistically significant difference exists between direct canalicular wall sutures (DCs) and pericanalicular sutures (PCs) in the repair of traumatic canalicular lacerations. METHODS: The medical records of 63 patients who underwent primary repairs for traumatic canalicular lacerations were retrospectively reviewed. Patients were divided in 2 groups according to the suturing techniques used: the DC group (n = 41) and the PC group (n = 22). Anatomic results were compared between these 2 groups. A successful result was determined by attempted irrigation and probing of the injured canaliculus at the last follow-up visit. RESULTS: There were 6 failed procedures among the patients who underwent pericanalicular repair and 1 failed procedure among the patients who underwent direct canalicular wall repair (p = 0.024). CONCLUSIONS: These data support the higher success rates in patients treated with direct canalicular repair compared with pericanalicular repair.

Partial removal of orbital tumor in Rosai-Dorfman disease.

BACKGROUND: Rosai-Dorfman disease is a rare idiopathic histiocytic proliferation disorder that typically presents with painless cervical lymphadenopathy. We report our experience with the management of a case of Rosai-Dorfman disease with compressive optic neuropathy. CASE: Rosai-Dorfman disease involving the bilateral orbital and paranasal sinuses was diagnosed in a 14-year-old boy. Diagnosis was based on the characteristic histopathologic features of sinus histiocytosis, composed of large, round S-100 protein-positive histiocytes with striking emperipolesis. The boy received chemotherapy to resolve the bilateral proptosis and compressive optic neuropathy in the right eye, but this treatment failed. Orbital debulking surgery using the Lynch approach was performed. OBSERVATIONS: Corneal exposure was resolved and visual acuity recovered from 14/20 to 20/20 after partial removal of the tumor mass. There were no complications after surgery. During the 22 months of follow-up, orbital tumor masses redeveloped to cause lagophthalmos again, but did not cause visual impairment. CONCLUSIONS: Rosai-Dorfman disease is a rare disorder, especially in Asia. The disease is usually chronic with spontaneous remission and is refractory to treatment. Partial removal of tumor masses is a workable way to improve visual acuity and correct corneal exposure. Before carrying out this procedure, we discussed with the parents of the patient the potential complications that might follow surgery and secured their permission before proceeding further.