Daphne L. Mourits

Orbital implants in retinoblastoma patients: 23 years of experience and a review of the literature

To evaluate complications of different types of orbital implants following enucleation for retinoblastoma.

Worldwide Enucleation Techniques and Materials for Treatment of Retinoblastoma: An International Survey

Purpose To investigate the current practice of enucleation with or without orbital implant for retinoblastoma in countries across the world. Methods A digital survey identifying operation techniques and material used for orbital implants after enucleation in patients with retinoblastoma. Results We received a response of 58 surgeons in 32 different countries. A primary artificial implant is routinely inserted by 42 (72.4%) surgeons. Ten (17.2%) surgeons leave the socket empty, three (5.2%) decide per case. Other surgeons insert a dermis fat graft as a standard primary implant (n=1), or fill the socket in a standard secondary procedure (n=2; one uses dermis fat grafts and one artificial implants). The choice for porous implants was more frequent than for non-porous implants: 27 (58.7%) and 15 (32.6%), respectively. Both porous and non-porous implant types are used by 4 (8.7%) surgeons. Twenty-five surgeons (54.3%) insert bare implants, 11 (23.9%) use separate wrappings, eight (17.4%) use implants with prefab wrapping and two insert implants with and without wrapping depending on type of implant. Attachment of the muscles to the wrapping or implant (at various locations) is done by 31 (53.4%) surgeons. Eleven (19.0%) use a myoconjunctival technique, nine (15.5%) suture the muscles to each other and seven (12.1%) do not reattach the muscles. Measures to improve volume are implant exchange at an older age (n=4), the use of Restylane SQ (n=1) and osmotic expanders (n=1). Pegging is done by two surgeons. Conclusion No (worldwide) consensus exists about the use of material and techniques for enucleation for the treatment of retinoblastoma. Considerations for the use of different techniques are discussed.

Orbital apex cavernous hemangioma - beware of the pear!

2001). It has been discovered that Morcher occlusive IOLs transmit high levels of near infrared light permitting retinal and macular imaging with optical coherence tomography (OCT) (Yusuf et al. 2011). We sought to evaluate the clinical utility of anterior segment OCT to detect subtle degenerative changes in a Clear IOL through a Black IOL in Black-on-clear polypseudophakia. Preoperative imaging in this context would permit accurate surgical planning for a high-stakes operation in a patient’s only seeing eye. We selected six intraocular lenses and implanted them sequentially in the posterior chamber of a model eye (Fig. 1A). Topcon Spectral-Domain Optical Coherence Tomography (3DOCT 2000) was used to acquire Anterior Segment OCT (AS-OCT) profiles of the model eye with each Clear and Black IOL individually, and in combination with replicate Black-onClear Polypseudophakia (Fig. 1B–D). AS-OCT was able to clearly image the Clear IOL through Morcher occlusive IOLs in simulated Black-on-clear polypseudophakia with no attenuation of OCT signal from the Clear IOL (Fig. 1B). AS-OCT was not able to image through Dr Schmidt’s Black IOLs or Artisan phakic iris-claw IOLs with AS-OCT identifying only the anterior optical interface of both IOLs, and not the posteriorly located Clear IOL (Fig. 1C). To evaluate the clinical utility of AS-OCT in Black-on-clear polypseudophakia, pathological states such as extracellular deposition, IOL fusion, IOL degradation and flattening were replicated in the model eye. AS-OCT was able to clearly identify soft tissue deposition and fusion between the IOLs, and subtle degradation and flattening of the anterior optic of the Clear IOL through Morcher Black IOLs (Fig. 1D). This study presents original data supporting the use of Anterior Segment OCT to evaluate the position and integrity of Clear IOLs through Black Morcher IOLs in Black-onclear Polypseudophakia. Dr Schmidt’s and Artisan iris-claw Black IOLs do not transmit infrared light, and patients with these prostheses cannot undergo preoperative or follow-up AS-OCT imaging (Yusuf et al. 2012). This may impact on preoperative occlusive IOL selection in primary Black-on-clear Polypseudophakia surgery. We believe AS-OCT should be used routinely to evaluate the integrity and position of the clear IOL preoperatively in patients with Black-on-clear polypseudophakia undergoing occlusive IOL explantation. This may identify critical preoperative details such as biometric indices for iris or sulcusfixated IOLs or the likelihood of vitreo-retinal surgery should AS-OCT reveal the clear IOL nonviable with consequent risk of capsular damage. AS-OCT in this setting will provide valuable information on the integrity and configuration of the clear IOL for accurate informed consent and surgical planning before, what is by definition, a high-stakes operation on a patient’s only seeing eye.

3D Orbital Reconstruction in a Patient with Microphthalmos and a Large Orbital Cyst—A Case Report

Abstract Background: Orbital cysts are rare developmental anomalies that can occur in microphthalmic and anophthalmic patients. Such cysts can promote orbital growth and subsequently markedly increase the size of the orbit, which is commonly underdeveloped in these patients. Cyst removal is therefore generally dissuaded (at least) in the first 5 years. Clinical case: A 6-year-old boy with a microphthalmos and a cyst developed protrusion of his prosthetic eye and a swelling of the lower eyelid. MRI showed a large cyst causing distortion of the right orbit. Due to the expansive orbital growth and subsequent misfitting of the prosthesis, cystectomy and orbital floor reconstruction was performed using 3D technology. Conclusion: 3D imaging and printing enables exact delineation of orbital cysts and the adjacent bony structures. Furthermore it offers the possibility to plan an individual surgical approach and to design and fabricate a custom fit orbital floor implant.

Case series: effect of dermis-fat implants in different late onset socket problems encountered in retinoblastoma patients

ABSTRACT Background: Cosmetic dissatisfaction, pain, and chronic discharge may present months till years after enucleation in patients operated because of retinoblastoma. If noninvasive treatment modalities are insufficient, socket reconstruction can be considered. In this study, we discuss the results of dermis-fat exchange to treat these problems. Method: Four patients with late onset post enucleation socket problems with a request for treatment were included in this prospective study. Socket inspection was documented and pictures at baseline and at a follow-up of at least 6 months were taken. To quantify the problem ‘pain’, a VAS score at baseline and at follow up was used. For the problem ‘cosmetic dissatisfaction’ standardized questionnaires were used. Results: Two patients were included because of cosmetic dissatisfaction; one was included with chronic pain and one with chronic discharge. Reconstruction of the socket using autologous dermis-fat insertion was done in all four. In one of them, severe shrinking of the fat developed. This patient was treated with additional injectable fillers. Both of them, ultimately, had satisfactory results. Autologous fat transplantation also solved the problem of chronic discharge and pain in the two other patients. Conclusion: Socket reconstruction by autologous dermis-fat exchange may solve different post enucleation socket problems. However, shrinking of the transplanted fat may occur and require additional procedures.

Cosmetic results of enucleation and/or external beam radiation therapy in 195 retinoblastoma survivors

To report the cosmetic outcome and late effects of enucleation and/or irradiation for retinoblastoma and to evaluate the role of orbital implants.

Discharge and infection in retinoblastoma post-enucleation sockets

Purpose To investigate the causes and treatment options for socket discharge and infection in patients enucleated for retinoblastoma (Rb). Methods A questionnaire was filled out by (parents of) ocular prosthesis-wearing patients with a history of enucleation as treatment for Rb. We collected data on patients’ characteristics, cleaning habits of the prosthesis, frequency of socket irritation, discharge, and infection, and use of antibiotics. With ordinal logistic regression analysis, factors related to the outcome parameters (frequency of irritation, mucoid and purulent discharge) were identified. In a subset of young asymptomatic and symptomatic patients, a swab culture of the socket was performed to determine the presence of microorganisms. Results A total of 186 patients or their parents (mean age of the patients: 17.3 years, ranging from 0.8 to 88.3 years) filled out the questionnaire. Irritation, mucoid discharge, and purulent discharge were frequently (once a month or more often) experienced in 75 (39.5%), 127 (66.8%), and 15 (13.2%) sockets, respectively. Younger age was associated with a higher frequency of mucoid and purulent discharge. Radiation therapy, chemotherapy, gender, age at surgery, cleaning frequency, and nocturnal wear were not associated with the outcome parameters. In a subgroup of 26 patients, the sockets were swabbed and cultured. All symptomatic patients had a positive bacterial culture versus 15% (2/13) of the asymptomatic patients (P<0.001). Common cold was correlated with both symptoms and presence of bacteria. Haemophilus influenzae and Staphylococcus aureus were the species most frequently cultured. Conclusion Ocular prosthesis-wearing patients often experienced mucoid discharge, and less often irritation and socket infection. These complaints were found to decrease with increasing age, but did not seem to be influenced by cleaning or wearing habits. Symptomatic sockets, with and without discharge, were correlated with the presence of pathogenic bacteria for which local antibiotic treatment seemed effective in most cases.

The value of magnetic resonance imaging in cases with unilateral retinal dysplasia

To the Editor: In their report, Lambert et al did not perform magnetic resonance imaging (MRI) to differentiate between intraocular pathologies that present with leukocoria. MRI can provide additional information to improve characterization of different intraocular pathologies. Furthermore, MRI can reveal accompanying central nervous system abnormalities in patients presenting with leukocoria. The combination of retinal dysplasia with central nervous system abnormalities is considered to be a condition occurring in patients with bilateral disease. Although unilateral congenital dysplasia is not thought to be related to associated pathologies, which subsequently discards the need for additional examinations, we present a very comparable case, which underlines the value of MRI. A baby girl conceived with in vitro fertilization was born at 37 weeks’ gestation after a complicated last term due to preeclampsia. She was born with a large periorbital hematoma, possibly due to the manual compression performed during delivery. Otherwise, she appeared healthy. In the second week, the parents visited the general practitioner because of a different appearance of the left eye, which was diagnosed as conjunctivitis. Parents were instructed to administer fucithalmic eye drops for 2 weeks, but discharge and redness of the left eye persisted, which resulted in referral to our clinic with the suspicion of retinoblastoma. Findings of an ophthalmic examination showed rubeosis iridis, an absent anterior chamber, and a yellowish mass (Figure 1, A and B; available at www.jpeds.com). The corneal diameter and intraocular pressure were similar to the contralateral eye. The mass and narrow pupil obscured visualization of the fundus. Ultrasonography scan showed a noncalcified white mass occupying more than 50% of the globe (Figure 2; available at www.jpeds.com). The right eye did not show any abnormalities (Figure 1, C). Subsequent MRI scanning showed a persistent hyperplastic primary vitreous, retinal dysplasia, and intraocular bleeding. The anterior chamber was shallow and combined with a lens luxation and cyclitic membrane. In the left orbit the optic nerve was hypoplastic (Figure 3, A and B; available at www. jpeds.com). The right orbit did not show any abnormalities. Findings on MRI were consistent with a congenital disorder, and a retinoblastoma was ruled out. MRI of the brain showed agenesis of the corpus callosum combined with extensive left-sided frontal and insular polymicrogyria and gray matter heterotopia (Figure 3, C and D). Ocular treatment was conservative, and the patient was referred to a pediatric neurologist and a clinical geneticist.