Basal cell carcinoma of the eyelid in children: a report of three cases.

Basal cell carcinoma is the most common eyelid malignancy, accounting for approximately 90% of malignant eyelid tumours. Despite its prevalence in adults, it is extremely rare in children, and usually occurs in the setting of a known genetic defect or following radiotherapy treatment. We report three cases of de novo basal cell carcinoma in children. These children had no known genetic syndromes and had not undergone radiotherapy.

Recurrent orbital inflammation from metastatic orbital carcinoid tumor.

PURPOSE: To report an unusual clinical presentation for carcinoid tumor metastatic to the orbit. METHODS: Two adult patients with metastatic carcinoid tumor and unilateral orbital masses are described. RESULTS: Both patients sought treatment for acute unilateral orbital inflammation. Neuroimaging revealed orbital metastases adjacent to the inflamed adnexal tissue. Based on each patient's description of similar, prior, untreated episodes, minimal medical management was initiated. Resolution of the inflammatory signs was spontaneous in one case and assisted by pulsed, systemic corticosteroids in the second case. Compressive neuropathic vision loss occurred 11 months later in the second case. CONCLUSIONS: Carcinoid tumor metastatic to the orbit may manifest as recurrent orbital and ocular adnexal inflammation. These signs differ from systemic carcinoid syndrome in that they are unilateral, limited to only the orbital and ocular adnexal soft tissues, and resolve over days. Clinicians must carefully differentiate this manifestation from that of tumor necrosis, adnexal infection, or orbital outlet obstruction. This presentation may result from the spontaneous release of local inflammatory mediators intrinsic to the orbital tumor.

Sino-orbital foreign body in a child.

Foreign bodies of the sinuses are uncommon. Few reports exist in the English literature. Among these, the frontal and maxillary sinuses are most often involved. Ethmoid and sphenoid foreign bodies are rare and tend to present with chronic symptoms due to delayed diagnosis. We present a case of an acute presentation of a foreign body involving the orbit and ethmoid sinus in a 12-year-old male.

Basal cell adenocarcinoma of the lacrimal gland.

PURPOSE: Basal cell adenocarcinoma is a recently recognized entity among malignant tumors of the salivary glands. This entity has not received enough attention among ophthalmologists and ophthalmic pathologists. We recommend that basal cell adenocarcinoma be included in the differential diagnosis of lacrimal gland tumors. DESIGN: Case report. METHODS: In 1988 a lacrimal gland tumor was incompletely removed from the left orbit of a 36-year-old woman and diagnosed as "solid basaloid adenoid cystic carcinoma". Soon after, an exenteration of the orbit was performed. In 1998, upon review of the initial histopathology, the diagnosis of solid adenoid cystic carcinoma was changed to basal cell adenocarcinoma. RESULTS AND MAIN OUTCOME MEASURE: The pathologic findings included nests of basaloid cells with minimal atypia and incomplete palisading around the periphery. The cells were of two types. The first type were large, pale cells with round or oval nuclei, scanty cytoplasm, and ill-defined borders. The other type were smaller cells with hyperchromatic nuclei situated mainly near the periphery of the nests. There was no necrosis or perineural invasion. Mitotic figures were present. Cysts within the nests showed Alcian blue negative contents. Immunohistochemistry showed a positive reaction to cytokeratin and a negative reaction with smooth muscle actin (SMA). CONCLUSIONS: The Alcian blue negative stain, the negative reaction to SMA, and the fact that the patient is still alive 10 years after exenteration favored the diagnosis of basal cell adenocarcinoma. It is essential to differentiate a basal cell adenocarcinoma of the lacrimal gland from the solid basaloid type of adenoid cystic carcinoma, because the former has a better prognosis. Patients with basal cell adenocarcinomas of the lacrimal gland should be closely monitored for local recurrences because this tumor has the tendency to show perineural invasion.

Orbital infarction and melting in a patient with systemic lupus erythematosus.

OBJECTIVE: To present a patient with systemic lupus erythematosus who developed infarction and melting of the orbit secondary to her systemic disease. DESIGN: A case report. PARTICIPANT: A 61-year-old white woman with a 5-year history of systemic lupus erythematosus. METHODS: The patient presented with left orbital pain, limitation of extraocular movements, and a fistula from the ethmoid sinus to the upper eyelid. A detailed examination with computerized tomography, ultrasound, and a comprehensive medical evaluation with laboratory testing was performed. Histopathologic analysis with special stains of the orbital tissues was also performed. RESULTS: Histopathologic examination of the biopsy specimens revealed the features of an inflammatory process involving the orbit, similar to a panniculitis. These include a lymphocytic reaction with a predominance of plasma cells, vasculitis with occlusion, and thickening of the vessel walls, necrosis, and hyalinization of fat. CONCLUSION: This is a unique case in which infarction and melting of the entire orbital structures occurred in the presence of systemic lupus erythematosus. The underlying disease process is a lupus-related panniculitis. The authors stress that this is a very rare entity and that other diseases should be ruled out before entertaining this diagnosis.

Periocular necrotizing fasciitis: a review of five cases.

OBJECTIVE: The purpose of the study is to display a spectrum of clinical presentations of periocular necrotizing fasciitis caused by group A streptococci and to discuss recent trends and treatment of this disease. DESIGN AND INTERVENTION: A case series of five patients (four female and one male) was seen between July 1990 and January 1995 in four university centers. All had clinical evidence of periocular necrotizing fasciitis and grew group A streptococci on wound cultures or had serologic evidence of streptococcal infection. Details of patient presentation, treatment, and outcome are examined. RESULTS: The five patients showed a spectrum of clinical severity from a necrotizing infection confined to the eyelid to a potentially fatal, severe shock-like syndrome characterized by sepsis and multiorgan system failure. A history of trauma often was absent. Patients were treated successfully by a combination of appropriate antibiotics and surgical debridement. CONCLUSIONS: Group A streptococci can cause severe necrotizing infections of the eyelids. Early recognition and prompt treatment can be essential to these patients' survival.

Symmetry and synkinesis during rehabilitation of unilateral facial paralysis.

We evaluated biofeedback rehabilitation in patients with severe chronic unilateral facial paralysis, who had intact facial-motor innervation (House grades 3 to 5). Recovery of facial function was characterized (1) by grading facial movement symmetry, and (2) by counting the number of muscles exhibiting synkinesis during maximal execution of selected facial movements (e.g., smiling). Facial function in 21 patients typically improved by one House grade. Facial symmetry recovered rapidly during the first 5 months of treatment, and then improved more slowly. However, during this latter period, examination of the relationship between symmetry and synkinesis (visualized by a graph plotting symmetry grades on the x-axis, against the number of synkinetic muscles on the y-axis) indicated that overall facial control was improving even when House grading suggested that it was not. Such information should aid facial retraining and may clarify understanding of underlying rehabilitation mechanisms.

Upper eyelid retraction in the absence of other evidence for progressive ophthalmopathy is associated with eye muscle autoantibodies.

We have studied 25 clinically euthyroid patients with eyelid lag and retraction referred to thyroid/eye clinic for clinical and orbital imaging evidence of extraocular eye muscle (EM) involvement, evidence of progressive ophthalmopathy and serum antibodies reactive with EM membrane antigens in immunoblotting. Fourteen patients had Graves' hyperthyroidism, 5 had Hashimoto's thyroiditis, and 6 had euthyroid Graves' disease. By carrying out orbital imaging we showed EM abnormalities in 10 of 23 patients (43%). Serum antibodies reactive with EM membrane antigens were detected in 96% of patients. Antibodies reactive with a 64-kDa antigen were detected in 66% of patients, while those reactive with 35-, 55-, and 95-kDa antigens were found in 21, 33, and 25% of patients, respectively. Antibody prevalences compared to normals were significantly different (P < 0.005) only for the 64-kDa protein. The prevalence and the degree of reactivity of 64-kDa antibodies were significantly different in patients with abnormal EM compared to those with normal EM at orbital imaging (P < 0.04 and P < 0.01, respectively). The results of this work suggest that in some patients inflammation of the eyelid muscles may be an isolated feature of ophthalmopathy and remains as the only sign of a "subclinical" eye disease in patients with thyroid autoimmunity.

An association between hypothyroidism and primary open-angle glaucoma.

PURPOSE: To test the hypothesis that there is an association between hypothyroidism and primary open-angle glaucoma. METHODS: The study was conducted in a case-control fashion. Sixty-four patients with primary open-angle glaucoma were evaluated for hypothyroidism by history and by undergoing a thyroid-stimulating hormone immunoradiometric assay. Sixty-four control subjects from the general eye clinic were evaluated in the same manner. Patients found to have elevated thyroid-stimulating hormone immunoradiometric assay were evaluated by an endocrinologist for hypothyroidism. RESULTS: Of the primary open-angle glaucoma group, 23.4% had hypothyroidism. A diagnosis was made previously in 12.5% patients, and 10.9% were newly diagnosed. Of the control subjects, 4.7% had hypothyroidism. A diagnosis had been made previously in 1.6% of the control subjects, and 3.1% were newly diagnosed. The difference between the two groups was found to be statistically significant. CONCLUSION: A statistically significant association between hypothyroidism and primary open-angle glaucoma is demonstrated. There is a large group (10.9%) of patients with primary open-angle glaucoma with undiagnosed hypothyroidism.

Restricted tissue reactivity of autoantibodies to a 64-kDa eye muscle membrane antigen in thyroid-associated ophthalmopathy.

We studied the tissue specificity of eye muscle (EM) membrane-reactive autoantibodies detected in the serum of patients with thyroid-associated ophthalmopathy (TAO). In preliminary studies, such antibodies were shown to react, in sodium dodecyl sulfate-polyacrylamide gel electrophoresis and Western blotting, with human thyroid (THY) and other human skeletal muscle (HSM) membrane antigens. We carried out absorption with human EM (HEM), THY, and HSM membranes of sera from patients with TAO and autoimmune thyroid disease without ophthalmopathy which reacted with one or more of 55-, 64-, and 95-kDa antigens in pig eye muscle (PEM) membrane in immunoblotting, the majority of which were also cytotoxic to HEM cells in an antibody-dependent cell-mediated cytotoxicity assay. In Western blotting, serum antibodies reactive with PEM membrane antigens of 55, 64, and 95 kDa were cross-absorbed by HEM, THY, and HSM but not by spleen or brain membranes and showed some species specificity, being absorbed by pig and human, but not bovine, EM membranes. When incubated with cultured HEM, THY, and HSM cells in vitro, autoantibodies in TAO sera immunoprecipitated a 64-kDa antigen from the first two tissues, but not from HSM, suggesting a specific binding to autoantigenic epitopes in HEM and THY. Sera from patients with TAO as well as those from patients with thyroid autoimmunity without ophthalmopathy immunoprecipitated a approximately 66-kDa protein, shown to be distinct from the 64-kDa antigen. The restricted immunological cross-reactivity of antibodies to a THY and HEM 64-kDa membrane antigen is discussed in the context of the association of ophthalmopathy with thyroid autoimmunity. Further experiments are needed to show whether autoantibodies to the 64-kDa eye muscle and thyroid shared antigen are cytotoxic, and thus likely to play a major role in the pathogenesis of the eye disease, or just markers of the orbital autoimmune process.

Antibodies in the serum of patients with autoimmune thyroid disorders react with a recombinant 98 amino acid fragment of a full length 64 kDa eye muscle membrane protein which is also expressed in the thyroid.

We have tested sera from patients with autoimmune thyroid disorders with or without ophthalmopathy for immunoreactivity, in a dot blot assay, against a recombinant 98 amino acid fragment of a cloned 64 kDa protein, D1, which is expressed in human eye muscle and thyroid, in the form of a Lac Z fusion protein. Tests were positive in 19 out of 40 patients with established thyroid-associated ophthalmopathy (TAO), in 12 out of 21 patients with Graves' hyperthyroidism (GH) without clinically evident ophthalmopathy, in 5 out of 10 patients with thyroid autoimmunity and lid retraction but no other signs of ophthalmopathy, in 4 out of 23 patients with Hashimoto's thyroiditis (HT) without evident ophthalmopathy and in 2 out of 18 patients with benign adenoma or multinodular goitre, but in only 2 out of 37 normal subjects tested. SDS-polyacrylamide gel electrophoresis and Western blotting for an antibody reactive with a 64 kDa antigen in pig eye muscle membranes was also carried out on sera from patients with TAO and GH. While immunoblotting for antibodies reactive with a 64 kDa protein was more often positive in patients with TAO, in whom 58% had serum antibodies which reacted with a 64 kDa protein, this was not the case in patients with GH without eye signs in whom the prevalence of positive immunoblot tests was 35%. Overall there was a fairly close correlation between the two tests although there were many exceptions.(ABSTRACT TRUNCATED AT 250 WORDS)

Significance of antibodies reactive with a 64 kDa eye muscle membrane antigen in patients with thyroid autoimmunity.

SDS-polyacrylamide gel electrophoresis and Western blotting for antibodies reactive with a 64 kDa protein in pig eye muscle membrane was carried out in patients with lid lag and retraction, but no other signs of ophthalmopathy, associated with thyroid disease or nonimmunologic goiter and in patients with Graves' hyperthyroidism without ophthalmopathy who were studied prospectively to determine the relationship of eye muscle antibodies to clinical features of the ophthalmopathy as they appeared in this group of predisposed patients. Seventy-one percent of euthyroid patients with lid lag and retraction but no established ophthalmopathy had detectable serum antibodies to a 64 kDa eye muscle membrane protein. Much smaller proportions had antibodies to proteins of other MW. In normal subjects with previously detectable antibodies to a 64 kDa protein, serum titers, determined by carrying out immunoblotting at serum dilutions of 1:25-1:6400, were low (< or = 1:100) in all cases tested. On the other hand, titers were higher (1:200-1:6400) in 16 of 22 patients with established ophthalmopathy and in 5 of 7 patients with lid lag and retraction tested. Titers tended to be lower in patients with ophthalmopathy of > or = 3 years duration than in those of < or = 1 year duration. Antibody titers were low (1:25) in 6 of 7 patients with Graves' hyperthyroidism without evident eye disease tested. Antibodies to a 64 kDa eye muscle membrane protein were predictive of the development of ophthalmopathy in patients with Graves' hyperthyroidism studied prospectively for periods of 8-42 months.(ABSTRACT TRUNCATED AT 250 WORDS)

Collagen shield contact lens use after eyelid surgery.

The use of collagen shields to enhance comfort and facilitate recovery after eyelid surgery was studied. Thirty-two patients undergoing various eyelid procedures were studied. In 10 patients, bilateral surgery was performed and the contralateral eye was used as a control. In patients with unilateral surgery, 20 of 22 patients exhibited comfortable postoperative courses, and in patients with bilateral surgery, eight of 10 patients had less conjunctival injection, chemosis, corneal staining, or lid edema on the side with the collagen shield. The only complications occurred with the 72-h lens, which tended to irritate the cornea and have a variable dissolution time.

Nasolacrimal disorders and their treatment.

Dysfunction of the excretory part of the nasolacrimal system may be congenital in otherwise normal infants or associated with congenital facial anomalies. It may also occur as a result of soft-tissue trauma to the face and naso-orbital fractures. Dacryocystorhinostomy and conjunctivodacryocystorhinostomy are discussed as treatment techniques.

Efficacy of surgical treatment for paralytic ectropion.

Paralytic ectropion can be corrected with numerous procedures. Advocates of particular procedures have previously been unable to quote statistical rates of success for each procedure over significant lengths of time. This study reviews over 200 cases of paralytic ectropion, representing the spectrum of seventh nerve disease seen at an eye and ear specialty hospital and a general medical facility. Of all eyelid implantation devices 93 to 95% failed to work or needed reoperation by 3 years postoperatively. Soft tissue surgery without prosthetic implants or exoplants provided 60% success after a 3-year follow-up. Eyelid elevation or tightening coupled with surgery for facial reanimation produced a higher rate of success at 3 years (83%). An overwhelming number of patients (62%) complained of some degree of epiphora after any or all procedures. Based on these findings, soft tissue surgery without prosthetic implants or exoplants has a higher rate of success.

Treatment of blepharospasm with medication, surgery and type A botulinum toxin.

A total of 39 patients with essential blepharospasm and 2 patients with hemifacial spasm were treated with one or more forms of therapy. All patients underwent neurologic and ophthalmic assessment to rule out ocular causes of blepharospasm. Thirty-six patients were given a trial of various medications. Only one patient was successfully treated: her condition was markedly improved with pimozide after benztropine mesylate, clonazepam and amantadine hydrochloride had failed to help. Patients who did not respond to drug therapy were offered the option of undergoing eyebrow-eyelid muscle stripping surgery. The six patients who underwent surgery showed considerable improvement; however, side effects such as frontal anesthesia, exposure keratitis, lagophthalmus, scarring and eyelid malposition occurred, and three of the six had residual spasm. At this point type A botulinum toxin became available. A total of 27 patients (26 who did not respond to drug therapy, including the 3 with residual spasm after surgery, and 1 previously untreated patient) received type A botulinum toxin injections. Most experienced rapid relief from their spasms. The beneficial effects lasted weeks to months, and there were no major side effects. Treatment with type A botulinum toxin appears to be a safe and effective means of temporarily relieving blepharospasm. The long-term results with repeated injections are yet to be determined.

Penetrating injuries to the orbit.

Although penetrating orbital wounds are an uncommon entity they are often associated with vision and life-threatening complications. By careful attention to the history and physical signs of the injured patient and the use of modern computed tomography (CT) scan imaging, the physician will be better able to make an accurate analysis and prognosis of the problem at hand as well as a well-planned therapeutic approach.

Medial wall fractures.

Medial wall blowout fractures may accompany orbital floor fractures or may occur as isolated fractures after blunt facial trauma. A "Z-plasty"-type incision over the medial orbital region allows the best exposure and cosmetic result following the repair of an isolated medial wall fracture.