Vol 20, No 3 (2025)

BACKGROUND: Hamartomas (from the Greek hamartia—error) are developmental anomalies caused by abnormal proliferation of cells in their physiological location. Among them, combined hamartoma of the retina and retinal pigment epithelium is of particular interest due to its rarity, diverse clinical manifestations and the challenges associated with interpreting instrumental diagnostic findings.

AIM: The work aimed to analyze the differential diagnostic features of combined hamartoma of the retina and retinal pigment epithelium in children based on clinical examination and optical coherence tomography data.

METHODS: A single-center, cross-sectional retrospective study was conducted. The study included medical records of patients examined at the Helmholtz National Medical Research Center of Eye Diseases between 2016 and 2025. Clinical and morphological characteristics of combined hamartoma of the retina and retinal pigment epithelium in children were analyzed with emphasis on identifying a set of differential diagnostic criteria.

RESULTS: The study included 14 children (16 eyes) with a confirmed diagnosis of combined hamartoma of the retina and retinal pigment epithelium. The age of the children at examination ranged from 1.4 to 8 years, with a mean of 6 ± 2.8 years. The retrospective analysis revealed that the most typical manifestation of combined hamartoma of the retina and retinal pigment epithelium was the presence of an epiretinal membrane. In some cases, signs of traction syndrome were observed, characterized by specific retinal architectural changes on optical coherence tomography: mini-peaks, maxi-peaks, the “omega sign” and the “shark teeth” phenomenon. In addition, some patients exhibited retinal thickening at the site of the hamartoma, the development of choroidal neovascularization and other traction-related changes. These findings confirm that the combination of ophthalmoscopic appearance, patient history and structural characteristics identified by optical coherence tomography provides the most comprehensive assessment of disease course and allows differentiation from vitreoretinal traction syndromes of other etiologies.

CONCLUSION: Combined hamartoma of the retina and retinal pigment epithelium is a very rare, often unilateral developmental anomaly of the retina that can lead to significant visual loss in cases with central fundus involvement. The condition has characteristic ophthalmoscopic and optical coherence tomography features, knowledge of which enables timely diagnosis and appropriate management of affected patients.

BACKGROUND: Surgical treatment of eye adnexa disorders, including symblepharon, requires effective repair techniques. An optimal material to replace the conjunctival defect is currently being sought. Given the limited volume of autologous tissues, modifying existing surgical treatment methods may allow improving the functional outcome of reconstructive surgeries. However, suturing of graft flaps is associated with an increased risk of postoperative complications. That is why flap adherence with sulfacrylate seems to be promising.

AIM: This study aimed to evaluate the efficacy of partial closure of a local conjunctival defect with sulfacrylate adhesive as part of the treatment of local symblepharon in an experiment by morphological examination.

METHODS: A single-center, uncontrolled, experimental study was performed. All participants were divided into three equal groups depending on the study completion date: group 1 at day 7; group 2 at day 21, and group 3 at day 45. Sulfacrylate tissue adhesive (Russia) was used in the surgery. The bulbar conjunctiva was incised in all rabbits in the superior nasal quadrant, then a 10 × 10 mm flap was created. Three fragments were cut out of the resulting flap, evenly staggered, and adhered to the exposed sclera with tissue adhesive. Approximately 27%–30% of the total area of the conjunctival defect was covered this way. The clinical condition and histology of the ocular surface tissues, including the cornea, host and graft conjunctiva, and the exposed sclera, were assessed on post-op days 7, 21, and 45.

RESULTS: The experiment was conducted in 15 gray Chinchilla rabbits (30 eyes). The use of autologous conjunctival flaps as the most preferred transplant material for conjunctival defects prevented a pronounced inflammatory reaction in the postoperative period. Sulfacrylate glue for tissue adhesion significantly shortened the surgery duration and reduced injury to the grafted flaps, eliminating the need for sutures. No toxic allergic reactions or decreased depth of the conjunctival cul-de-sac were reported in all experimental cases. The adhesive film was completely resorbed by post-op day 21.

CONCLUSION: Our findings show the regenerative potential of the conjunctival tissue, which requires further research and offers new perspectives for ophthalmic plastic.

Cavernous sinus thrombosis is a rare but potentially life-threatening condition, especially in children. Given high mortality of this condition, rapid diagnosis and early treatment are important and can reduce the risk of a poor outcome or death. Clinical presentation is described mainly in adults. The main signs include fever (sometimes with spikes typical for septic thrombophlebitis), tachycardia or hypotension. Ocular symptoms are almost universal and include periorbital edema (initially unilateral, but usually bilateral), eyelid hyperemia, conjunctival chemosis, upper eyelid ptosis, exophthalmos (caused by impaired venous outflow from the orbit), limited or painful eye movements, and less commonly, optic disc edema, retinal hemorrhages, and decreased visual acuity. Most publications on this topic include an analysis of clinical cases; there is very little pooled data on anticoagulant therapy in the publications. As the condition is rare, treatment is based on expert judgment. In general, therapy includes two main strategies—antimicrobial and antithrombotic. Published data on the treatment of children is limited.

The article presents a clinical case of cavernous sinus and orbital vein thrombosis associated with sphenoiditis in a child. The patient had complaints several months before the onset of clear clinical manifestations. He had left upper eyelid edema, severe headaches accompanied by pain in the left eye, and increased body temperature.

Otolaryngologists carried out a surgery for urgent indications the day after the diagnosis. A sphenoidotomy was performed using video endoscopic equipment to debride the left sphenoid sinus. On the day of surgery, sodium heparin was administered as anticoagulant therapy followed by sodium enoxaparin and then long-term rivaroxaban. The case outcome was a complete recovery.

The presented clinical case demonstrates challenging timely diagnosis of cavernous sinus thrombosis in children. It highlights the importance of a detailed medical history and a comprehensive ophthalmological examination for diagnosis. The clinical case features include long-lasting manifestations with many nonspecific complaints from the patient, prolonged diagnosis from the first symptoms, and the onset of symptoms during the COVID-19 pandemic.