Contemporary management of retinoblastoma in the context of a low-resource country.

Retinoblastoma (RB) is the most common ocular cancer, and it typically presents before the age of 5 years in over 90% of cases. In high resource countries, RB patients tend to survive and retain their sight. This is not the case in low-resource countries because of late presentation and delayed intervention arising mostly from sociocultural and socioeconomic challenges. RB has no gender or racial predilection; the incidence is estimated as 1:16,000-1:18,000 live-births or 11/1 million children under 5 years. Most of the world's RB cases are found in Asia and Africa while most RB treatment centres are in America and Europe. RB is easy to detect by caregivers as a glowing white 'cat eye reflex' at night or when captured on camera. Health workers at primary care level can detect RB in early life if red reflex test and/or squint (Hirschberg) tests are deployed as part of wellness checks done especially during routine immunisation and well-baby clinics in the first 24 months of life. In most cases of RB, biopsies for histological confirmation are not required for diagnosis and treatment decisions to be made. Clinical information, ophthalmic evaluation and imaging modalities are typically used. There have been significant changes in the management of RB using various treatment modalities such as enucleation with orbital implant, use of chemotherapy delivered through intravenous, intravitreal, periocular and intra-arterial routes and targeted treatment with laser, cryotherapy and brachytherapy. Algorithm for management and development of the national RB program within the context of a low-resource country is presented from review of data extracted from Mendeley library, PubMed library, Google Scholar and One Network; full-text articles were mostly retrieved through the American Academy of Ophthalmology.

[Alcohol Injection in a Patient with Chronic Orbital Pain after Enucleation - a Case Report and Review of the Literature]. / Alkoholinjektion bei chronischem orbitalem Schmerz nach Enukleation ­ Falldarstellung und Literaturübersicht.

A case is presented of a 54-year old patient who had been treated 10 months previously with enucleation for a painful blind eye. This led to severe and chronic pain in the orbital region that did not respond to conventional pain management. However, a single 1.5 ml injection of 96 % ethanol led to almost complete resolution of pain for the follow-up period of 6 months. Orbital pain after enucleation or evisceration may originate from the implant itself, the prosthesis, the socket or the sinuses. Taking a careful medical history and an examination, including orbital scans, are necessary to decide on the correct differential diagnosis. If any pathology is excluded, one should keep in mind that phantom pain in the orbit seems common after removing an eye, more often when pain originating from the ball and/or headache was present before removal. The management of chronic pain in the orbital region has received little attention. Retrobulbar alcohol injection still has a place in modern ophthalmology, because it delivers effective pain relief in certain chronic conditions.

[Post enucleation socket syndrome - new and established surgical solutions]. / Das Syndrom der anophthalmischen Höhle oder das "post enucleation socket syndrome" - neue und bewährte chirurgische Konzepte.

The orbital volume after enucleation or evisceration might shrink over time due to atrophy of the orbital fat and connective tissue. This can lead to prosthesis misfit and an unsatisfactory cosmetic result. This so-called post enucleation socket syndrome (PESS) needs a secondary orbital volume augmentation. The techniques for orbital volume augmentation are secondary orbital implants or implant exchange, orbital floor implants or injectable augmentation. The surgical possibilities are described and discussed with special attention to indications and chances.

[Avulsion of the Globe after Bicycle Accident--Clinical and Histopathological Evaluation]. / Avulsio bulbi nach Fahrradsturz - klinische und histopathologische Begutachtung.

BACKGROUND: Potential pathological mechanisms and complications of traumatic avulsio bulbi will be discussed based on the history, clinical, and histopathological findings of two patients. HISTORY AND SIGNS: Both patients were heavily affected by alcohol and had a bicycle accident with subsequent perforation of the upper eyelid by the handlebar-mounted hand brake levers. The accident resulted in disruption of the optic nerve and anterior displacement of the globe. THERAPY AND OUTCOME: In one patient, it was possible to reposition the globe into the orbit; however, the second patient's eye had to be enucleated. Functional testing demonstrated permanent loss of vision of the primary involved eyes, visual field defects, and visual impairment of one fellow eye. CONCLUSION: Avulsio bulbi describes a severe trauma to the eye often induced by sport and traffic accidents. Special attention should be addressed to the primary non-affected fellow eye with regard to potential functional defects.

[Available approaches to retinoblastoma therapy--literature review]. / Dostepne metody leczenia siatkówczaka--przeglad pismiennictwa.

Retinoblastoma is a malignant tumor and treatment should be started as soon as possible. Currently, the most common approach combines local therapy with chemotherapy. Chemoreduction represents a significant advance in the treatment of retinoblastoma. This paper presents treatment approaches including local chemotherapy, intraarterial and intravitreous chemiotheraphy. retinoblastoma, chemotherapy chemoreduction, brachytherapy, thermotherapy, laser photocoagulation, proton radiotherapy, teleradiotherapy, intraarterial chemotherapy.

Update on intra-arterial chemotherapy for retinoblastoma.

The tools for managing retinoblastoma have been increasing in the past decade. While globe-salvage still relies heavily on intravenous chemotherapy, tumors in advanced stage that failed chemotherapy are now referred for intra-arterial chemotherapy (IAC) to avoid enucleation. However, IAC still has many obstacles to overcome. We present an update on the indications, complications, limitations, success, and technical aspects of IAC. Given its safety and high efficacy, it is expected that IAC will replace conventional strategies and will become a first-line option even for tumors that are amenable for other strategies.

[Uveal schwannoma mimicking melanoma (case report)].

The article presents a rare clinical case of intraocular schwannoma localized in the pre-equatorial choroidea and the pars plana and complicated by secondary glaucoma. Prolonged course of the disease and the presence of scleral staphyloma allowed to assume benign nature of the tumor. Enucleation was performed due to painful secondary glaucoma. Histological examination of the enucleated eye revealed a choroidal schwannoma. Despite the absence of pathognomonic signs and rare occurence, the possibility of intraocular schwannoma must be kept in mind when examining patients for uveal melanoma.

A case of accidental self-enucleation caused by obstructive sleep apnea.

A novel form of injury associated with obstructive sleep apnea (OSA) that was comorbid with obesity hypoventilation syndrome and severe daytime somnolence is reported in a 55-year-old woman, manifesting as severe ocular and extraocular muscle injuries sustained from suddenly falling asleep and colliding with a sharp object, resulting in surgical enucleation of the right eye and orbital implant. The literature on injuries (falls, motor vehicle accidents) related to OSA and excessive day time sleepiness is reviewed, along with the literature on injuries from OSA-related parasomnias. The diverse health hazards, including physical injury, associated with OSA-excessive daytime sleepiness, are emphasized, further encouraging the need to educate primary care providers on early detection of OSA with prompt treatment intervention. CITATION: Baker N, Schenck CH, Golden E, Varghese R. A case of accidental self-enucleation caused by obstructive sleep apnea. J Clin Sleep Med. 2024;20(8):1395-1397.

[Early surgical rehabilitation due to modified exenteration technique]. / Frühe chirurgische Rehabilitation durch modifizierte orbitale Exenteration.

BACKGROUND: Orbital exenteration is a life intruding surgical procedure with severe functional, aesthetic and psychological consequences. Apart from the correct tumor treatment, early aesthetic and psychosocial rehabilitation is crucial for the well-being of the patient. We discuss reasons leading to exenteration and present new surgical techniques. Local flaps in the anterior socket improve wound healing allowing early placement of the prosthesis and therefore faster social rehabilitation of the patient. PATIENTS AND METHODS: Between 2007 and 2011 seven patients with malignant orbital tumors (1 × plasmocytoma, 1 × melanoma, 1 × sarcoma, 1 × squamous cell carcinoma and 3 × basal cell carcinoma) received a radical orbital exenteration at the Department of Ophthalmology of the Zurich University Hospital. The medical histories were evaluated according to reasons for exenteration, surgical techniques and postoperative follow-up. Reconstruction of the anterior socket border succeeded using local flaps (Mustardé, Glabella and combined with further modified pedicled local full thickness skin flaps). The central defects were covered with split skin graft from the thigh. RESULTS: Three weeks after surgery the anterior border of the socket was completely healed without problems by local flaps with good blood supply. This allowed the early prosthetic fitting and wearing as well as quick social rehabilitation of the patient. CONCLUSIONS: The use of local flaps improves wound healing even in anticoagulated patients. This reduces the time of hospitalization and rehabilitation, and allows an early, satisfactory, social reintegration of the patient.

Bilateral retinoblastoma: clinical presentation, management and treatment.

Management of retinoblastoma (Rb), the most common intraocular malignant tumor in childhood, is tailored to each individual case and based on the overall situation. We present a case of bilateral Rbs in a 4-month-old girl, referred to our center for bilateral leukocoria. In the right eye, the optic disc was partially visible, and three large foci of retinoblastoma were noted adjective in the vitreous cavity with satellite retinal detachment. The macula was obscured by the tumors. The tumor was therefore classified as group D (International classification of retinoblastoma), and thus underwent intravenous chemotherapy with the standard three-agent protocol of ifosfamide, carboplatin and etoposide (ICE protocol) delivered monthly for six cycles, regressing with a type I calcified pattern. The left eye presented three multifocal yellow-white retinal masses, with a total retinal detachment, and secondary glaucoma. The lesions were classified as group E and, therefore, taking the results obtained by Shields et al. in group E tumors into consideration, underwent enucleation. This case clearly shows that this cancer is curable if detected at a stage in which it is still contained within the retina, subretinal space or vitreous, and that the management of Rb relies on an experienced team of ocular oncologists, pediatricians and pediatric ophthalmologists working together for the single goal of saving the child's life.

[Complications of anophthalmic orbits-Treatment and aftercare]. / Komplikationen der anophthalmischen Orbita ­ Therapie und Nachsorge.

The possible complications of anophthalmic eye sockets can occur due to many different pathomechanisms. A differentiation is made between allergic, infectious, inflammatory or mechanical causes. This article gives an overview on the different etiologies of socket complications with their pathophysiology and treatment options.

Resection of intraocular tumors (partial lamellar sclerouvectomy) in the pediatric age group.

PURPOSE: To describe the outcome of intraocular tumor resection by partial lamellar sclerouvectomy (PLSU) in the pediatric age group. DESIGN: Retrospective, interventional case series. PARTICIPANTS: We included 37 patients who underwent PLSU for intraocular tumors under age 21 years. METHODS: Medical records were reviewed for clinical, operative, and histopathologic features. MAIN OUTCOME MEASURES: Globe salvage, visual acuity, surgical side effects, tumor control, and tumor-related metastasis and death. RESULTS: The mean patient age was 9 years (median, 10; range, 0.25-17) and there were 22 (59%) females and 15 (41%) males. The tumor involved mainly the iris (n = 27; 73%), ciliary body (n = 7; 19%), or choroid (n = 3; 8%). The mean tumor basal dimension was 7 mm (median, 6; range, 3-15) and thickness was 3.6 mm (median, 3; range, 0.2-9.6). The histopathologic diagnosis was melanoma (n = 19; 51%), cyst (n = 5; 14%), medulloepithelioma (n = 4; 10%), melanocytoma (n = 3; 8%), lacrimal gland choristoma (n = 2; 5%), gliosis (n = 1; 3%), nevus (n = 1; 3%), hemorrhage (n = 1; 3%), and leiomyoma (n = 1; 3%). Intraoperative adverse effects included vitreous loss in 19 (51%) and hyphema in 4 (11%). Postoperative side effects included early transient hyphema in 9 (24%) and late cataract in 12 (32%). Local tumor recurrence was detected in 5 cases (13%) at mean interval of 28 months, including melanoma (n = 3) and medulloepithelioma (n = 2). Enucleation was necessary in 9 (24%) cases for recurrence in 3 (8%), prophylactic in eyes with high-grade malignancy in 5 (13%), and for blind painful eye in 1 (3%). Of the 28 salvaged eyes, final visual acuity was ≥ 20/40 in 18 (64%), 20/50 to 20/100 in 5 (18%), and <20/200 in 5 (18%). There were no cases of metastasis or death at a mean of 51 months of follow-up. CONCLUSIONS: A surgically challenging procedure, PLSU can achieve control of selected intraocular tumors. Medulloepithelioma responds poorly to local resection and in most cases enucleation is required. If the globe is salvaged, visual acuity is ≥ 20/40 in 64% of children. FINANCIAL DISCLOSURE(S): The authors have no proprietary or commercial interest in any of the materials discussed in this article.

Application of the lateral orbital flap to reconstruction of the upper and lower eyelids and the eye socket after enucleation.

The wide applications of the subcutaneous pedicle flap "lateral orbital flap" named by the author is presented. The flap is designed between the lateral canthus and the sideburn as a spindle or crescent shaped island 2- to 3-cm wide and 5-cm long. To reconstruct the upper or lower eyelids, the flap is elevated as thin as possible, whereas for the eye socket reconstruction, an adequate volume of subcutaneous fat tissue is attached to the flap. The pedicle is designed inferomedially for the reconstruction of the recipient, any shape of the flap can be designed. As the anatomic structure of the donor site is similar to the eyelid, the excellent result can be obtained both functionally and cosmetically. The donor site is primarily closed without a marked scar. The cases of reconstructions using lateral orbital flap for the upper eyelid and the eye socket in the anophtalmic condition are presented.

[Ligation of the retrobulbar vascular-nervous bunch during performance of evisceration and enucleation using titanic clips in ophthalmic surgery].

The efficacy of hemostasis achievement during conduction of a retrobulbar vascular-nervous bunch ligation (RVNBL), using titanic clips while evisceroenucleation performance, was studied up. A comparative analysis of clinical and functional results of surgical treatment of 36 patients, suffering terminal dolorous glaucoma and disaster of a sympathetic ophthalmia complication after an eye penetrating wounding occurrence. In 16 patients (the first group) a standard method of a hemostasis achievement was used while doing evisceroenucleation - a deep orbital tamponade for 5 minutes. In 20 patients (the second group) a procedure of RVNBL was conducted, using titanic clips before the bunch transsection doing. There was established, that while doing a RVNPL using titanic clips, a hemorrhage never occurs, a retrobulbar hematoma do not formated, the soft tissues reaction in the early postoperative period is less pronounced, and the patients postoperative rehabilitation period shortens.

Comparison of different laser-based enucleation techniques for benign prostate hyperplasia: A systematic review and meta-analysis.

BACKGROUND: To summarize the current evidence on different laser-based enucleation techniques for benign prostate hyperplasia and compare the efficacy and safety of en-bloc, two-lobe and three-lobe techniques. MATERIALS AND METHODS: Through a systematical search of multiple scientific databases in March 2021, we performed a systematic review and cumulative meta-analysis of the primary outcomes of interest according to the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) and AMSTAR (Assessing the methodological quality of systematic reviews) Guidelines, whose protocol was registered with PROSPERO(CRD42021240684). RESULTS: A total of 9 studies were included. All three laser enucleation techniques had no statistically significant difference in terms of enucleated prostate weight, maximum urinary flow rate (Qmax), postvoid residual urine volume (PVR), international prostate symptom score (IPSS), transient UI (TUI), persistent UI (PUI) and early postoperative complications. A shorter enucleation time was associated with the en-bloc technique compared to three technique (MD: -8.26, 95%CI: -12.73--3.79, p = 0.0003), whereas no significant difference was found in en-bloc versus two-lobe technique (MD:0.97,95%CI: -0.30-2.24,p = 0.13) and two-lobe versus three-lobe technique (MD: -3.19, 95%CI: -7.45-1.06, p = 0.14). A higher enucleation rate was associated with the en-bloc and two-lobe technique (MD: 0.05, 95%CI: 0.00-0.10, p = 0.03; MD: 0.09, 95%CI: 0.01-0.17, p = 0.03, respectively). A superior QoL was related to the two-lobe enucleation technique compared to three-lobe technique (MD: 0.22, 95%CI: 0.06-0.39, p = 0.009), whereas no meaningful difference was found in the group of en-bloc versus two-lobe (MD: -0.12, 95%CI: -0.62-0.37, p = 0.62) and group of en-bloc versus three-lobe (MD: -0.14, 95%CI: -0.56-0.29, p = 0.52). CONCLUSIONS: En-bloc and two-lobe laser-based enucleation techniques are feasible and safe alternative to three-lobe technique with comparable surgical outcomes and similar functional outcomes. A superior enucleation efficiency was associated with En-bloc and the two-lobe techniques compared to the three-lobe technique.

Outcomes of orbital implants after evisceration and enucleation in patients with endophthalmitis.

PURPOSE OF REVIEW: This manuscript reviews enucleation and evisceration and primary versus secondary orbital implant placement in patients with endophthalmitis. A brief discussion of sympathetic ophthalmia is included as well. RECENT FINDINGS: Opinions remain variable when considering enucleation versus evisceration and primary versus secondary orbital implant placement in patients with endophthalmitis. Although rare, sympathetic ophthalmia remains a concern among surgeons. SUMMARY: Recent findings indicate enucleation and evisceration are performed for blind eyes with endophthalmitis and both porous and nonporous implants may be placed primarily with acceptable outcomes in these patients.

Undergoing enucleation of the eye. Part 2: postoperative care.

In this, the second of a 2-part article, the author discusses the postoperative care of patients who undergo enucleation of the eye. Part one explored aspects of preoperative care of enucleated patients. In the immediate period following surgery, patients require support and advice regarding pain, nausea and simple care of the eye socket. Apart from the intrinsic postoperative demands of the surgery, there are also the difficulties and challenges associated with providing psychological support and helping patients cope with the loss of an eye. Ultimately, the patient can overcome any initial problems and eventually be able to wear a custom-made prosthesis, which will hopefully allow the patient to move on with their lives. As in the first part, the author will use both referenced material and anecdotal references (upon personal reflection) to analyze the ongoing postoperative care and support of these patients.

Management of devastating ocular trauma--experience of maxillofacial surgeons deployed to a forward field hospital.

Combat-related eye injuries continue to increase in frequency and are generally secondary to Improvised Explosive Devices. Many ocular injuries are potentially preventable by the wearing of ballistic eye protection. The management of penetrating eye trauma is normally outside the routine practice of maxillofacial surgeons in the UK. The aim of this paper is to describe the surgical techniques used in the modern management of devastating ocular trauma including selected case examples managed by British military maxillofacial surgeons deployed to Afghanistan.