Radiotherapy in combination with exenteration and partial orbitectomy for orbital multilobular tumor of bone in a Cocker Spaniel.

Background: Multilobular tumor of bone or multilobular osteochondrosarcoma is a tumor of flat bone in the skull. The treatment of choice for a multilobular tumor of bone is local aggressive surgical excision. Case Description: A female Cocker Spaniel dog aged 11 years presented with a history of globe displacement of the right eye for 3 months. Ophthalmic examination revealed exophthalmos, third eyelid protrusion, and slightly increased intraocular pressure OD (oculus dexter; right eye). Computed tomography (CT) revealed a mass effect in the right retrobulbar, maxilla, zygomatic, and temporal areas. Right zygomatic and temporal bone lysis were observed. Physical examination, hematology, and blood chemistry results were within normal limits. Exenteration with zygomatic arch removal was performed. During surgery, a firm 2-lobed mass (4.8 × 3.7 and 1.6 × 1.4 cm) adhered to the mandible was found in the retrobulbar area OD. Histopathological findings revealed a multilobular tumor of bone. CT imaging was performed for the remaining tumor and an extended part of the right retrobulbar mass was found. Hypofractioned radiotherapy with 6 fractions of 6 Gy was performed on days 0, 7, 14, 21, 28, and 35. At 1-month and 4-month follow-up inspections, the mass gradually reduced in size. At 8 months and 11 months after radiotherapy, the mass was unremarkable. The dog was alert during all follow-up periods to 1 year and 8 months after hypofractioned radiotherapy combined with exenteration and partial orbitectomy. Conclusion: Hypofractioned radiotherapy combined with exenteration and partial orbitectomy extended the patient's survival and decreased the size of the remaining tumor for the management of orbital multilobular tumor of bone in this dog for at least 1 year and 8 months.

Effect of different prosthetic materials on radiation dose distribution in an orbital defect: a clinical report.

It is challenging to treat and irradiate empty cavities with external-beam radiation therapy (EBRT) because body contour irregularities can result in dose heterogeneities. The use of compensator materials to fill the empty cavities can provide a more homogeneous radiation dose distribution. The purposes of this clinical report are to describe the use of 3 different materials (elastomeric material, water-filled balloon, and acrylic resin) in an orbital defect and compare the dosimetric parameters and photon-electron dose distribution during EBRT.

An anterior appositional electron field technique with a hanging lens block in orbital radiotherapy: a dosimetric study.

A technique for orbital radiotherapy is presented consisting of an anterior, appositional electron beam with a hanging lens block. The beam was modified by introducing two 1.6 mm thick plastic spoilers, at about 3 cm and 15 cm from the lens, to boost in-scattering of electrons under the block. The 9 mm diameter, 2 cm long stainless steel cylindrical block was suspended 0.5-1.0 cm above the eye. We performed film, TLD (Thermo Luminescent Dosimetry), and diode dosimetry to determine the dose fill-in behind the lens. The introduction of the spoilers dramatically changed the dose distribution. The maximum dose under the block increased from 66% to 85% of the open field dose. Moreover, the dose to the posterior surface of the globe directly underneath the block, at a depth of 3 cm, increased from 48% to 76% of maximum dose, while the dose to the lens was still below 20%. This is a simple and easily reproducible treatment and is an improvement on a previously described technique. The dose distribution is adequate for cases where the target volume surrounds and is posterior to the globe.

Long-term effects in children treated with radiotherapy for head and neck rhabdomyosarcoma.

PURPOSE: To examine the long-term effects of treatment in children receiving radiotherapy for head and neck rhabdomyosarcoma. METHODS: From 1967 to 1994, a total of 30 children with head and neck rhabdomyosarcoma received megavoltage radiotherapy at one institution. Seventeen patients (57%) have survived and have at least a 5-year follow-up. There were 11 males and 6 females, with a median age of 5.7 years (range 2.2-11.6) at the time of radiotherapy. Tumor location was orbit in 6 patients, infratemporal fossa in 4, paranasal sinuses in 2, and supraglottic larynx in 2; the nasopharynx, pterygopalatine fossa, and parotid gland were sites for the remaining children. All but 2 patients had tumors of embryonal histology. The Intergroup Rhabdomyosarcoma Study (IRS) Group was I in 2, II in 3, and III in 11 children; 1 patient had a recurrent tumor after surgery alone. Radiotherapy volume was the primary tumor or tumor bed in 13, tumor and whole brain in 3, and tumor and craniospinal axis in 1. Median radiotherapy dose to the primary site was 5,040 cGy (range 4,140-6,500) and to the whole brain was 3,000 cGy. All but 1 were treated with 150-200-cGy fractions; 1 patient received 250-cGy fractions for a tumor in the larynx. Chemotherapy was vincristine (V), actinomycin-D (A), and cyclophosphamide (C) in 10 patients, VAC + adriamycin in 2, VA in 1, VA + ifosfamide in 1, VC + adriamycin in 1, and none in 2. One patient had salvage chemotherapy consisting of cisplatin and etoposide. Median follow-up time was 20 years (range 7.5-33). RESULTS: Late effects of treatment were seen in all patients and included facial growth retardation in 11, neuroendocrine dysfunction in 9, visual/orbital problems in 9, dental abnormalities in 7, hearing loss in 6, and hypothyroidism in 3. Intellectual and academic delays were documented in 3 patients who had received whole brain radiotherapy. While neuroendocrine, thyroid, dental, and cognitive sequelae were primarily attributed to radiotherapy, hearing loss was thought to be a direct result of tumor destruction and, in 1 case, cisplatin chemotherapy. Late effects at or beyond 10 years from radiotherapy were few, but severe, and included chondronecrosis, esophageal stenosis, second malignancy, and brain hemorrhage. CONCLUSION: Late effects of treatment in children receiving radiotherapy for head and neck rhabdomyosarcoma are frequent. Although radiotherapy is a significant contributor of neuroendocrine, dental, thyroid, and cognitive toxicity, it is not usually implicated with hearing loss. Late toxicity of treatment beyond 10 years is not as frequent as those occurring within 10 years of therapy.

Glued-on, rigid gas-permeable contact lens for severe radiation-induced keratitis.

A 68-year-old woman with severe radiation-induced keratoconjunctivitis sicca became progressively unresponsive to conventional medical treatment. Her left eye deteriorated rapidly and required total tarsorrhaphy. In an attempt to stabilize the right eye and preserve some functional vision, we glued a high-Dk rigid, gas-permeable contact lens with tissue-grade cyanoacrylate adhesive to Bowman's membrane. This glued-on contact lens induced rapid and dramatic improvement of the patient's comfort and sight. Recent developments in high-permeability, rigid, contact-lens materials now make artificial replacement of the epithelium a potentially useful treatment for severe ocular surface disease when conventional treatment has failed.

Assessment and treatment of facial deformity resulting from radiation to the orbital area in childhood.

Children who receive radiation for malignant tumors in the orbital area frequently develop widespread craniofacial deformities. These affect the skull, orbit, maxilla, and mandible. When these patients seek treatment at a later age, they require careful assessment using cephalometrics and three-dimensional imaging. It is recommended that the four levels of skeletal deformity be corrected in a single procedure, that is frontotemporal expansion with repositioning of the skull base area, orbital expansion and repositioning together with maxillary and mandibular surgery. Bone grafts should be inlay rather than onlay and soft tissue should be supplied by free-tissue transfer. This counteracts any residual ischemia related to the previous radiation therapy. The second surgical stage is designed to reconstruct the socket and the eyelids to allow more satisfactory rehabilitation with an ocular prosthesis. In patients who have a globe present, the usual enophthalmos can be corrected by repositioning of the eye as part of the first procedure by reducing the anteroposterior dimensions of the socket. In bilateral cases, the deformity is hourglass in nature and requires correction in the frontal and temporal area with lateral displacement of the orbits. A bimaxillary procedure is also indicated. It is emphasized that to formulate a satisfactory operative plan an in-depth three-dimensional analysis of the deformity is mandatory.

Extraoral prostheses using extraoral implants.

The aim of this study was to evaluate extraoral prostheses and the use of extraoral implants in patients with facial defects. 10 cases were treated utilizing maxillofacial prostheses employing extraoral implants in five cases. 16 extraoral implants were installed. Seven implants were placed in irradiated sites in the orbital regions. Six implants were placed in mastoid regions and three in a zygoma region that was irradiated. Two implants failed before initial integration was achieved in irradiated areas. Using 14 extraoral implants as anchors, five extraoral prostheses were set. The other five cases were treated with extraoral prostheses without using extraoral implants due to cost and patient-related factors. The data included age, sex, primary disease, implant length, implant failure, prosthetic attachment, radiation therapy, and peri-implant skin reactions. The use of extraoral implants for the retention of extraoral prostheses has simplified the placement, removal, and cleaning of the prosthesis by the patient. The stability of the prostheses was improved by anchors. Clinical and technical problems are presented with the techniques used for their resolution. Using extraoral implants resulted in a high rate of success in retaining facial prostheses and gave good stability and aesthetic satisfaction.

Periorbital and orbital malignancies: methods of management and reconstruction in Iraq.

Orbital cancer is rare, and in this study, few cases are reported consisted of 16 patients (nine female, seven male). Age ranged from 9 months to 75 years. Tumor types were squamous cell carcinoma, basal cell carcinoma, conjunctival squamous cell carcinoma, retinoblastoma, fibrosarcoma, and ectopic mixed tumor in two, one, two, one, one, and one patients, respectively, in addition to eight patients with jaw lymphoma involving the orbit, out of 24 patients reported by us. Eight patients were treated surgically with adjuvant postoperative radiotherapy, whereas eight patients with lymphoma treated with combination chemotherapy (vincristine, Adriamycin, cyclophosphamide, methotrexate, and prednisolone), the survival rate was very poor. Follow up ranged from 1 to 5 years. Surgery consisted of complete excision of orbital content (exenteration) with or without partial orbitectomy in four patients and wide excision of the tumor in four patients. Reconstruction of the defect was accomplished using various local skin flaps and temporalis muscle flap was used for augmenting the orbit in the four exenterated patients. No complications were encountered. Survival rate was quite good except for patients with lymphoma with 91.9% mortality. There is no single best method for reconstruction of the periorbital and orbital defects left after tumor resection, and different flaps applied for reconstruction had given satisfactory results related to the type and complexity of the deformity. Temporalis muscle flap in this study had proved to be versatile and highly reliable flap in terms of bulk and vascularity for blocking the orbit. The aim of this study was to show our experience in the management of these cases with minimum surgical morbidity and deformity.

Orbital lymphoma with concomitant sarcoid-like granulomas.

A 41-year-old man presented with chronic eyelid swelling, conjunctival injection, and decreased ocular motility in all gaze directions. MRI showed bilateral enlarged extraocular muscles, including the tendons. Laboratory tests revealed elevated levels of angiotensin-converting enzyme. An orbital biopsy showed collections of monotonous small lymphocytes, and granulomatous inflammation that included multinucleated giant cells, predominantly Langhans type. Flow cytometric analysis of tissue demonstrated a light chain-restricted clonal population of B cells, a finding that confirmed the morphologic impression of lymphoma. This case demonstrates that elevated angiotensin-converting enzyme and granulomatous inflammation can occur in lymphoma. Careful histopathologic examination and flow cytometric analysis are essential to avoid an erroneous diagnosis that could lead to inappropriate management.

Orbital venography of Kasabach-Merritt syndrome.

A case of hemangioma involving the orbit and jaw associated with thrombocytopenia in a 1-month-old boy was reported. To our knowledge, this combination of two hemangiomas has never been reported. By utilizing venography we could diagnose the mass as being a hemangioma. Radiotherapy was successful giving 500 rad to the mass on the right jaw. At follow-up after 2 months, the platelet count had returned to the normal range and the hemangiomas were noted to have regressed dramatically.

Carcinoma mucoepideermoide nasal y de antro maxilar y su recuperación orbitaria / Nasal mucoepidermoid carcinoma and maxilar sinus and orbital repercussion

Dentro del grupo de los tumores malignos epiteliales de las fosas nasales y antro maxilar, el carcinoma mucoepidermoide es el más raro. La participación orbitaria es frecuente y se ilustra con dos casos, uno con participación de la cavidad nasal y antro maxilar y el otro exclusivamente del antro maxilar. Las manifestaciones clínicas dependen de las alteraciones mecánicas musculares y la proptosis; las más frecuentes son: exoftalmo no reductible, disminución de la capacidad visual hasta llegar a la ceguera y deformación facial. La tomografía y resonancia nuclear magnética son muy útiles en el diagnóstico y pronóstico. El pronóstico es malo tanto para la función como para la vida. El tratamiento es eminenetemente quirúrgico, la radioterapia y quimioterapia son paliativos y con severas complicaciones deletéreas

Compromiso orbitario en el linfoma de Burkitt / Orbitary involvement in Burkitt's lymphoma

Los tumores linfoides siempre representan un problema diagnóstico, tanto clínico como histopatológico. Se presenta un niño de 4 años con linfomas de Burkitt, cuya manifestación clínica fue proptosis y tumoración en hemicara izquierda. Se hace una revisión de la enfermedad mencionando su distribución universal, su mayor frecuencia en niños entre 2 y 15 años, así como su localización mandibular y abdominal. Se mencionan las características histológicas distintivas y el resultado obtenido con tratamiento combinado de quimioterapia y radioterapia