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Melnick-Needles syndrome.

A 10-year-old female with a generalized bone dysplasia was found to have a rare condition, Melnick-Needles syndrome. Characteristic clinical features of this syndrome include exophthalmos, hypertelorism, micrognathia, malaligned teeth, and large ears in a person with multiple bone deformities. In addition to the usual characteristic clinical findings, sclerocornea and cornea plana were also present in this patient. This is the first case of Melnick-Needles syndrome reported in the ophthalmological literature and the first case reported with ophthalmological findings other than exophthalmos and strabismus.

Abnormalities, Multiple↗

[Orbital involvement as the first manifestation of Hand-Schuller-Christian disease. A case report].

UNLABELLED: PURPOSE/MATERIAL AND METHOD: To describe the clinical case of a 20-month-old boy with Hand-Schuller-Christian disease. Exophthalmos was the only systemic manifestation evident over a period of at least 6 weeks, but later the triad of exophthalmos with multiple lytic lesions of the skull and diabetes insipidus were present. RESULTS/CONCLUSION: Histiocytosis X should be considered in the differential diagnosis of orbital tumors in children.

Age Factors↗

[Malignant exophtalmos in hyperthyroidism with a hypothyroid crisis].

CASE REPORT: We present the case of a 56-year-old male with a malignant exophthalmos in the context of Graves' disease. He suffered a significant worsening coincident with a hypothyroid crisis, and various therapeutic approaches were required for reduction of the proptosis. DISCUSSION: When managing this condition, both the severity of the exophthalmos and the activity of the thyroid must be considered when choosing which of the different therapeutic approaches should be employed.

Acute Disease↗

[Subperiosteal hematoma of the orbit associated with subfrontal extradural hematoma].

Subperosteal hematoma is rarely associated with extradural hematoma and orbital roof fracture. We report a case observed in a 16-year-old boy who developed exophthalmos, diplopia and visual loss after blunt head trauma. Computed tomography demonstrated the subperiosteal hematoma associated with a thin extradural hematoma and an orbital roof fracture. The hematomas were removed during the same procedure via fronto-orbito craniotomy. Surgery led to complete recovery without functional sequelae. We stress the importance of early diagnosis and treatment of post-traumatic exophthalmos.

Adolescent↗

[Our experience with the 131-I treatment of thyrotoxicosis].

The authors analyze 100 patients, with thyrotoxicosis, 84 females and 16 males, treated with 131J. The enlargement of the thyroid gland was II degree in 57 per cent and III degree in 34 per cent, and I and II - for the rest of the cases. In 77 per cent the body weight of the patients was below the norm. Exophthalmos was observed in 48 per cent. As a result of treatment an involution of a series of pathological symptoms developed- tachycardia, ECG changes, exophthalmos, degree enlargement of the thyroid gland, blood indicies, weight, capacity for work. Euthyroidism was established in 86 per cent, partial remission in 12 per cent and hypothroidism - in 2 per cent, when followed up during an interval of 2-13 years. No genetic or blastomogenic changes were observed. Observations is recommended in the later periods after the treatment.

Adult↗

Estimating eyeball protrusion from body height, interpupillary distance, and inter-orbital distance in adults.

Eyeball protrusion is one characteristic that must be assessed/predicted in craniofacial identification methods of skull-face superimposition and facial approximation. Previously it has been suggested that average exophthalmometry values, as measured on living individuals, should be used. However, it is unknown if proptosis prediction can be improved beyond the accuracy obtained when using mean values. Some authors have suggested that relationships between exophthalmos, height, interpupillary distance, and interorbital distance exist and it has been reported that these latter variables can be used to estimate eyeball projection. However, crucial tests are yet to be conducted. This study measures these variables and tests the accuracy of exophthalmometry means, a previously proposed prediction equation, and newly derived regression equations to determine which methods provide the best results. Data indicate that variation in exophthalmos is fairly small and as such prediction from other variables, like body height, are weak; thus, exophthalmometry means currently offer the best practical method of prediction. It should be noted that up to 2 mm error from either side of the mean is expected for 68% of cases.

Adult↗

Carotid-cavernous sinus fistula after trauma to the head.

The similarities between the various conditions described are remarkable. The orbital or frontal bruit and pulsating exophthalmos are common only to the carotid-cavernous fistula. A recent history of dental treatment, of facial infection with an elevated white blood cell count, and leukocytosis suggests cavernous sinus thrombosis. The superior orbital fissure syndrome and orbital apex syndrome occur most frequently in conjuction with Le Fort II and III fractures, which can involve the optic foramen and superior orbital fissure, therefore disrupting the sensory and motor innervation to the orbit and adjacent structures (Fig 4). Direct orbital trauma without evidence of fracture and with all symptoms mentioned, except infection, bruit, and pulsating exophthalmos, suggests orbital hematoma. A thorough differential diagnosis with complete data is essential for a correct diagnosis, thereby reducing morbidity by institution of the appropriate treatment. Treatments for carotid cavernous fistula have varied over the years--all aiming toward abolition of the fistula. Currently, the preferred treatment is the supraclinoid clamping of the internal carotid artery, followed by muscle embolization of the internal carotid artery distal to the bifurcation, and clamping of the internal carotid artery in the neck.

Arteriovenous Fistula↗

[New quantitative parameters for CT evaluation of orbitopathy in dysthyroid ophthalmopathy].

The new index (c/o ratio) is defined as the content of the orbit divided by the capacity of the bony orbit on the mid-orbital axial CT slice showing the medial and the lateral rectus muscles and optic nerve. Other quantitative parameters such as for exophthalmos and the capacity of bony orbit were also obtained on CT films. These parameters were measured in 474 eyes of patients with dysthyroid ophthalmopathy. The capacity of bony orbit showed large variations among all patients. The average for males was 17.8cm3 and that for females was 16.1cm3. Exophthalmos measurement on CT is more reliable than that by the Hertel exophthalmometer. The new index (c/o ratio) is a very reliable parameter for evaluating orbitopathy in dysthyroid ophthalmopathy. A large c/o ratio (greater than 1.6) was of clinical significance, because approximately 70% of the enlargement of the extraocular muscle observed in older patients (greater than 40 yrs old) was associated with a large c/o ratio (greater than 1.6).

Adolescent↗

Ocular findings in juvenile nasopharyngeal angiofibroma.

Juvenile nasopharyngeal angiofibroma (JNA) is the most common benign neoplasm of the nasopharynx. While histologically benign, it has the propensity for aggressive local growth. This highly vascular tumor predominantly occurs in adolescent males. The literature fails to provide a thorough description of ocular complications and their incidence in JNA. This report summarizes the data from those clinical series detailing ocular findings in a total of 218 JNA cases. Exophthalmos was found in 14% of all cases. Decreased visual acuity and partial ophthalmoplegia occurred in 5% and 2% respectively. Recognition of ocular involvement in JNA is of the utmost importance, for it is often a manifestation of orbital or intracranial extension or both. We describe the diagnosis and management of a case of JNA in a five-year-old white male. The patient developed ocular findings of marked exophthalmos and optic atrophy. Early multidisciplinary diagnostic evaluation (otolaryngological, neurosurgical, and ophthalmological) followed by a team surgical approach to excision is most likely to yield efficacious results.

Child, Preschool↗

Lid retraction and levator aponeurosis defects in Graves' eye disease.

Vertical palpebral fissure determinants and lid crease height were measured in patients with Graves' eye disease (GED) and in normal subjects. The fissure and crease height are positively correlated with exophthalmometer readings in each group. Compensatory levator aponeurosis defects are shown to occur in patients with GED. These, along with the effect of exophthalmos on the lower lid position, the observation that the lower lid is closer to the inferior limbus than the upper lid is to the superior limbus in normal subjects, and the effect of contracture of the inferior rectus on the lower lid position, explain why inferior scleral show is found to be greater than superior scleral show in patients with GED. Thus, lid retractor surgery in GED patients should be performed only after the palpebral fissure, levator function, and lid crease height stabilize, and after any contemplated surgery that would alter the exophthalmos and extraocular muscles has been performed.

Adolescent↗

[Axial computerised tomography - answered and unanswered questions on the ophthalmologist (author's transl)].

The results of 67 examinations with computerized axial tomography are presented with reference to the indications. 15 examinations were performed in exophthalmos, 11 in unknown visual field defects, 9 in optic atrophy of unknown origin, 8 each in optic neuritis, papilledema and unclear visual disturbance. 8 furthur examinations were performed in different cases. The axial computerized tomography proved to be an efficient complementary examination technique if it is limited to well defined indications. The best results are obtained examining exophthalmos and visual field defects.

Brain Neoplasms↗

Orbital involvement in chronic lymphocytic leukemia.

In a 68-year-old man with chronic lymphocytic leukemia diagnosed on the basis of peripheral lymphocytosis, marked bilateral exophthalmos developed owing to massive orbital involvement by the disease. At the time there was no lymphadenopathy or evidence of organ infiltration. The response to radiotherapy was excellent. Orbital involvement is rare as an early clinical feature of chronic lymphocytic leukemia but should be considered in the differential diagnosis of bilateral exophthalmos in adults.

Aged↗

[Cranio-orbital meningiomas and their surgical treatment].

Since 1968 the authors performed operations on 241 patients for cranio-orbital tumors, 156 of which (64.7%) were meningiomas: 103 (42.8%) were hyperostotic and 53 (22%) were nodular. The authors describe the features of the diagnosis and clinical picture of hyperostotic and modular forms of cranio-orbial meningiomas and the direction of their prevalent growth (intracranial, intraorbital, and extracranial). Unilateral exophthalmos and hyperostosis are characteristic signs of cranio-orbial meningiomas. Single-stage transcranial approach is the only adequate method for the surgical management of cranio-orbial meningiomas at the early stage of the disease. Original modification of this method were elaborated depending on the form and the prevalent direction of the growth of meningiomas. In the early stage of the disease, a wide transcranial approach with the use of microsurgical techniques provides for the radical removal of the tumor, preservation of the eyeball and its neuromuscular apparatus and disappearance or considerable reduction of exophthalmos.

Adult↗

Orbital pseudotumor as a presenting sign of temporal arteritis.

Both unilateral proptosis (2 cases) and radiological evidence of orbital inflammatory pseudotumor in the absence of exophthalmos (1 case) have been separately described as presenting signs of temporal arteritis. We report a patient presenting with bilateral exophthalmos associated with CT and MRI signs of orbital inflammation, who had biopsy-proven temporal arteritis. Our case is unique in view of the association of the above clinical and radiological features, and the bilateral involvement of orbital tissues.

Aged↗

Direct carotid-cavernous sinus fistulas.

1. Direct carotid-cavernous sinus fistulas usually result from multi-trauma or head injuries. These patients are more likely to be seen at trauma based hospitals. 2. Ocular manifestations of direct carotid-cavernous sinus fistulas include exophthalmos, swollen eyelids, ocular pulsation, pulsating exophthalmos, conjunctival chemosis, arterialization of conjunctival vessels, exposure keratopathy, a bruit over the affected eye (heard by the patient as a swishing sound), diplopia, visual loss, and ophthalmoscopic abnormalities that include moderate dilation of retinal veins, optic disc swelling, and intraretinal hemorrhage. 3. The ultimate diagnostic test for direct carotid-cavernous sinus fistulas is cerebral arteriography of both the internal and external carotid arteries. 4. The optimal treatment of a direct carotid-cavernous sinus fistula is closure of the abnormal arteriovenous communication with preservation of internal carotid artery patency. Detachable, flow-guided balloons are presently used to close most direct carotid-cavernous sinus fistulas.

Aged↗

[Embryonal rhabdomyosarcoma of the orbit].

The paper shows the case of a 19-years-old patient, who presented two month before the observation took place marked exophthalmos at the left eye with general state alteration. Computerised tomography pointed out a tumoral formation having 4 centimeters in diameter, which pushed off the anterior ocular globe, eroding to the left frontal lobe. The anatomopathological exam evidenced the embryonal rhabdomyosarcoma. So, every unilateral exophthalmos of the child or teenager must lead the diagnosis of embryonal rhabdomyosarcoma. The therapy consists of exeresis biopsy, followed by complex radiotherapy and chemotherapy. The orbital exenteration, a multilant intervention, does not guarantee for the further evolution of the affection.

Adult↗

[Intra-orbital space occupying lesion. Differential diagnostic considerations based on a rare case].

The differential diagnosis of an intraorbital tumor is discussed on the basis of a rare case of cyst formation that presented clinically in the lacrimal duct. Rapid progression of exophthalmos and visual loss required an immediate surgical intervention. Using a medial orbitotomy, a cystic tumor was extirpated that had no connection to the paranasal sinuses. Postoperatively, the visual defect and exophthalmos resolved completely. This case demonstrates the problems of differential diagnosis within the orbit that require close interdisciplinary cooperation. The final diagnosis can often be made only after surgical exploration and histopathology of tissue specimens.

Aged↗

[Clinical picture of pretibial myxedema and new aspects of diagnosis and pathogenesis].

We have reported on two cases of pretibial myxedema. In one patient, the pretibial myxedema developed symmetrically half a year after the appearance of endocrine exophthalmos. Recently, acropachy developed as well. In the second case, the pretibial myxedema arose unilaterally in an old cicatricial area, six years after the onset of endocrine exophthalmos. In this syndrome, the development of the whole group of symptoms, dermopathy, ophthalmopathy, and osteopathy at the same time is very rare. The clinical picture is seen in thyroid disorders, as a rule occuring in hyperthyroidism, rarely in hypothyroid or euthyroid states. Apparently, the development of the symptoms does not depend on thyroid hormone production. One of our patients showed conspicuous hyperthyroidism, the other, thyroxin deficiency. The thyreohypophyseal feedback mechanism in this syndrome was examined for the first time by means of TRH stimulation tests. In both patients, thyroid auto-antibodies indicative of autoimmune thyroiditis were demonstrated. A high activity of long acting thyroid stimulator was measured in the one patient's serum.

Adult↗