Rhabdomyosarcoma (RMS) is the most common malignant tumor of the orbit in children, but it is rarely present at birth. We report a large congenital orbital RMS with intracranial extension in a newborn baby. A newborn baby girl was referred to our hospital due to severe right-eye exophthalmia. Imaging studies showed an orbital mass with intracranial extension. Treatment was started with a course of neoadjuvant chemotherapy followed by right orbital exenteration and intracranial resection of the tumor. Histologic examination of the mass showed undifferentiated malignant small-cell tumor. Immunohistochemical study proved it to be RMS. The patient was categorized as intermediate-risk RMS and chemotherapy was continued accordingly with VAC regimen. In spite of treatment, the infant developed intracranial recurrence at the age of 6 months and died 1 month later. Congenital orbital RMS has a poor prognosis. Reconstruction surgery should be deferred due to high rate of recurrence and low chance of survival. A multidisciplinary approach might increase the survival of these patients.
PURPOSE:
The purpose of this study is to compare the corneal biomechanical properties of hyperthyroids without ophthalmopathy (HWO), thyroid eye disease (TED), and euthyroid participants.
METHODS:
In this prospective comparative study, one eye per patient included in the analysis and according to exclusion criteria, 38 eyes of 40 HWO patients, 31 eyes of 40 TED patients, and 150 eyes of 160 age- and sex-matched euthyroid participants were enrolled. All participants were evaluated by an endocrinologist and oculoplastic surgeon for evaluation of thyroid function and orbitopathy, respectively. Measurements of corneal biomechanical properties were performed using ocular response analyzer and Corvis ST tonometers.
RESULTS:
In the HWO group, applanation-1 length (A1 L), applanation-2 velocity (A2V) (
P
< 0.001), and corneal resistant factor (
P
= 0.043) were higher than the control group and corneal hysteresis (CH) was lower (
P
= 0.018). In the TED group, A1 L, A2V (
P
< 0.001), and highest concavity radius (HCR) (
P
= 0.027) were higher than the control group, and the CH (
P
= 0.007) and highest concavity deformation amplitude (HCDA) (
P
= 0.001) were lower. Furthermore, the level HCDA in the TED group was lower than HWO group (
P
= 0.011). The level of upper scleral show and palpebral fissure had a negative correlation with CH and HCDA. The amount of exophthalmos level had a positive correlation with HCR.
CONCLUSION:
Corneal biomechanical properties in the HWO and TED groups were different from the normal euthyroid individuals and may be taken into account in managing situation like glaucoma and refractive surgeries.
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