This technique is both feasible and effective to manage severe ectopia lentis in children. Lifetime ophthalmic follow-up including cECC measurement, IOL position monitoring and fundus examination is mandatory in these patients.
Purpose:
To describe the management of dermoid cysts in a pediatric referral hospital.
Methods:
Retrospective review of 115 patients with pathological diagnosis of dermoid cyst in a pediatric referral hospital between 2003 and 2019.
Results:
One hundred fifteen patients, 51 (44.3%) males, and 64 (55.7%) females were retrospectively reviewed. There were fifty-one (44.3%) right eyes and 64 (55.7%) left eyes. Mean age at surgery was 39.15 months (5.6 months–16.4 years). One hundred four (90.4%) lesions were superficial, and 11 (9.6%) were deep. Quadrant location was 63 (54.8%) superior-temporal, 45 (39.1%) superior-nasal, 4 (3.5%) inferior-temporal, and 2 (1.7%) in the nasal inferior quadrant. Most patients only had aesthetic concerns, but 3 (2.61%) showed lacrimal drainage obstruction symptoms, 2 (1.74%) had proptosis, and 1 case spontaneously drained to the cutaneous surface. Imaging was performed in 51 (44.3%) patients. Regarding to their radiodensity, 71.9% had low density content, 28.1% high density, and only 1 patient showed full liquid content; 10.9% showed heterogeneous content; 53.1% showed bone remodeling. Every patient but 1 underwent surgery for a barely accessible asymptomatic retrobulbar cyst. Nine cysts (7.8%) were breached during surgery. Three recurrences were found (2.6%), but only 1 was related to intraoperative breach.
Conclusions:
Dermoid cysts are the most common benign periorbital tumors in the pediatric population. Imaging is required for evaluation of lesions in atypical locations, deep or fixed to underlying tissue. Surgical removal is the gold standard of treatment. Multidisciplinary approach may be required in the most complex cases. After surgery, few complications and recurrences were found in our series.
RESUMENCaso clínico: Paciente de tres años con sospecha de uveítis intermedia. La exploración oftalmológica, ecografía ocular, resonancia nuclear magnética y tomografía computerizada orbitaria no fueron concluyentes. La determinación de la tasa de lactato deshidrogenasa (LDH) en humor acuoso/suero fue la clave para llegar al diagnóstico de un retinoblastoma difuso. Discusión: El síndrome mascarada es la forma de debut en un 1-3% de los retinoblastomas. La punción de cámara anterior está contraindicada en pacientes con retinoblastoma pero podría ser la úni-ca forma de llegar a un diagnóstico correcto en estos casos difíciles y poco frecuentes. Las pruebas enzimáticas como la LDH nos ofrecen una buena sensibilidad y especificidad para el diagnóstico de estos pacientes.Palabras clave: Uveítis intermedia, LDH, mascarada, retinoblastoma.
ABSTRACT Clinic report:A 3-year-old boy presented with an intermediate uveitis. Complete ophthalmic exam, ocular ultrasonography, magnetic resonance imaging and computerized tomography of the orbit were inconclusive. Determination of the aqueous humor/serum rate of Lactate dehydrogenase (LDH) was the key for the diagnosis of a diffuse retinoblastoma. Discussion: A masquerade syndrome is the initial presentation in 1-3% of retinoblastomas. Aqueous humor punction is contraindicated in patients with retinoblastoma but it might be the only way to achieve a correct diagnosis in these difficult and very unusual cases: enzymatic assays such as LDH offer a good sensitivity and specificity for the diagnosis of these patients (Arch Soc Esp Oftalmol 2009; 84: 477-480).
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.