Amin M. Nasr

Penetrating orbital injury with organic foreign bodies

OBJECTIVE The authors reviewed the clinical features, diagnostic workup, and management of patients of penetrating orbital injuries with retained organic foreign bodies. DESIGN Retrospective, noncomparative case series. PARTICIPANTS Nineteen patients (15 males, 4 females) with penetrating orbital injuries due to organic foreign bodies. RESULTS The series included 15 (78.9%) males and 4 (21.1%) females who ranged in age from 6 months to 40 years (mean = 14.6 years); 12 (63.2%) patients were younger than 12 years of age. Twelve (63.2%) right and 7 (36.8%) left orbits were involved. Time between injury and presentation varied from a few hours to 9 months. Most common injury site was the superior orbit in 11 (57.9%) patients leading to abnormal extraocular motility (84.2%), proptosis (68.4%), and upper lid ptosis (47.4%). Associated pathologies also included acute cellulitis in 11, orbitocutaneous fistula in 5, and osteomyelitis in 2 patients. Preoperative computed tomography (CT) and magnetic resonance imaging (MRI) identified the foreign bodies in 42% and 57% of the patients, respectively. CONCLUSION Preoperative identification of the foreign material in the orbit was found to be very helpful for patient management but was only possible in approximately 50% of our cases with the use of CT and MRI. The vision in our patients usually improved shortly after treatment; the long-term complications more often included extraocular muscle and eyelid motility problems and periorbital scarring.

The Histopathology and the Mechanism of Entropion in Patients with Trachoma

BACKGROUND Eyelids of patients with trachoma may be thickened. This thickening could be attributed to trachomatous changes in the conjunctiva and tarsus. METHODS Biopsies of tarsal plates and palpebral conjunctivae were obtained from 17 upper eyelids of 11 patients with inactive trachoma who underwent posterior tarsotomy procedures for entropion repair. RESULTS Light microscopy studies showed a thick and compact subepithelial fibrous membrane adherent to the tarsal plate. This membrane caused apparent thickening of the tarsus when measured intraoperatively (range, 1.25-2.00 mm). Other histopathologic findings include atrophy of the meibomian glands with thickening of the acinar basement membrane, loss of goblet cells, retention cysts, and hyaline degeneration of the tarsal plate with focal replacement by adipose tissue. CONCLUSION The contraction of the subepithelial fibrous membrane formed by vertically oriented parallel collagen fibers is one of the main factors contributing to the entropion formation.

Orbital xanthogranuloma: Clinical and morphologic features in eight patients

Purpose To describe the clinical and morphologic features of patients with orbital xanthogranuloma (XG) with or without Erdheim-Chester disease (E-Cd). Methods Retrospective, noncomparative case series. A review of 8 consecutive histopathologically proven cases of orbital XG from 3 medical centers. Four male and four female patients with ages ranging from 23 to 79 years presented with 4 bilateral and 4 unilateral orbital XGs. Results Age at diagnosis, ocular and systemic manifestations, histopathologic and radiologic features, type of treatment, and prognosis were evaluated for each patient. Six of 8 patients had proptosis and 2 presented with afferent pupillary defect and severe extraocular motility limitations. Other signs and symptoms included eyelid retraction, mechanical ptosis, and chemosis. Planar xanthomas of eyelids were present in 3 individuals. CT and MRI showed infiltrating soft tissue masses within the orbit in 7 and 2 patients, respectively. Histopathology revealed proliferation of foamy histiocytes intermingled with Touton and multinucleated giant cells and lymphocytes. The absence of Birbeck granules within the histiocytic elements of the tumor, indicating that the cell of origin is a non-Langerhans histiocyte, was documented with electron microscopy in 3 cases. The most common treatment was surgical excision combined with oral corticosteroids. Two patients with E-Cd with involvement of the long bones of the upper and lower extremities and retroperitoneal region died of kidney failure within approximately 1 year of diagnosis. Conclusions Orbital XG is a proliferative lesion of the non-Langerhans histiocytes, which may present as a solitary orbital lesion or may be associated with a systemic condition known as E-Cd with very poor prognosis.

Enchondromatosis and Hemangioma (Maffucci's Syndrome) With Orbital Involvement

Maffuccis syndrome is a rare, congenital disease of unknown cause characterized by the development of multiple enchondromas and soft-tissue hemangiomas. We treated a 34-year-old man with Maffuccis syndrome, bilateral proptosis secondary to multiple intraorbital hemangiomas, corneal exposure secondary to a left facial nerve palsy, and multiple intra-abdominal tumors. The skeletal manifestations were not clinically apparent and were only discovered after a careful radiologic survey. Simultaneous bilateral orbital cavernous hemangiomas should alert the physician to the possibility of Maffuccis syndrome.

Orbital Hemangiopericytoma: Clinical and Morphologic Features

PURPOSE To report the clinical and histopathologic features of orbital hemangiopericytoma. METHOD We review the clinical and histopathologic features in seven patients. RESULTS Ultrasonography, computed tomography, and magnetic resonance imaging defined the location and extent of the tumor in each patient but did not disclose pathognomonic features for the specific diagnosis of hemangiopericytoma. The predominating histopathologic feature of each tumor was a mixed pattern of ovoid cells and sinusoidal space formations. Five patients showed mild to severe cellular atypia; three had obvious pleomorphism and increased number of abnormal mitotic figures. Tumor cells disclosed cytoplasmic reactivity for vimentin but in five cases were negative for other immunologic markers. Six patients received surgical treatment with an attempt for total removal of the tumor; one had biopsy and radiation therapy. In two patients, radiation therapy was given in addition to tumor removal with orbital exenterations. Three patients died with recurrent and metastatic disease, and four patients are alive without tumor for a follow-up period ranging from 3 to 9 years. CONCLUSIONS Orbital hemangiopericytoma may behave as a malignant tumor, leading to local recurrence or metastasis, or both. Clinical and histopathologic findings should be considered jointly to evaluate the clinical course; histopathologic findings alone are not sufficient to predict the biologic behavior of this tumor.

Ectopic (Extradural) Meningioma of the Orbit: A Report of Two Cases in Children

Meningiomas arising from the optic nerve or secondarily invading the orbit from the intracranial cavity are uncommonly encountered in children. Ectopic (extradural) meningiomas are exceedingly rare orbital tumors that do not originate from either the optic nerve or the intracranial meninges. We evaluated and treated two boys aged 7 and 10 years with probable primary ectopic (extradural) orbital meningiomas. Both patients presented with a gradual onset of progressive, painless proptosis. In addition, computed tomographic scans demonstrated involvement of the superomedial orbit without bony erosion or hyperostosis, but with expansion of the adjacent sinus. Neither tumor demonstrated attachment to the optic nerve or intracranial extension at the time of surgery. Both patients were successfully treated with complete surgical excision. Evaluation did not reveal the presence of neurofibromatosis in either child.

Pediatric orbital tumors in Saudi Arabia

Most pediatric orbital tumor series report a low percentage of malignant lesions. A retrospective review of children aged 18 years or less admitted with the diagnosis of orbital tumor included 148 patients over a six-year period. Orbital retinoblastoma accounted for 48 cases (32%), followed by vasculogenic lesions (33 cases, 22%). Benign cystic lesions were the third most common (27 cases, 18%). Malignant neoplasms were identified in 48% of the cases studied. Retinoblastoma may be more common in Saudi Arabia than in Western countries, and delay in presentation appears to be the major cause of secondary orbital invasion.

Conjunctival lymphocyte subsets in trachoma.

Monoclonal antibodies were used to assess the lymphocyte populations in conjunctival biopsy specimens obtained from patients with trachoma (active or inactive) undergoing tarsotomy for the correction of trachoma-induced entropion and in three control patients. Peroxidase-labelled monoclonal antibodies OKT4 (identifies T-helper/inducer lymphocytes), OKT8 (identifies T-suppressor/cytotoxic lymphocytes), OKIal (identifies B-lymphocytes) and antisera specific for IgG, IgA and IgM were used to identify lymphocyte subpopulations and immunoglobulins in the conjunctival biopsy specimens. When grouped by disease activity, conjunctival tissue specimens revealed predominant T-helper/inducer lymphocytes in the substantia propria of patients with active trachoma while inactive trachoma patients had predominant T-suppressor/ cytotoxic lymphocytes in the conjunctival biopsy specimens. B-lymphocytes were seen in moderate numbers in all conjunctival biopsy specimens from patients with active trachoma and all specimens from active cases stained for IgG, IgM, and IgA. Immunoglobulin staining was strongest with IgG and IgM.

Retinoblastoma: the Saudi Arabian experience.

During the period March 1983-May 1987, 74 cases of retinoblastoma were evaluated at the King Khaled Eye Specialist Hospital in Riyadh, Saudi Arabia. 60% of the cases were unilateral with a female preponderance of 59.4% noted for this series. Additionally, the mean age at diagnosis was 22 months, and delay of retinoblastoma diagnosis since the first symptoms were observed ranged from two days to 36 months. Fifty-seven eyes were enucleated, and in 21 of these patients, this was the sole form of therapy. The remainder of patients were treated with radiotherapy alone or combined with chemotherapy, cryotherapy/photocoagulation. Overall, two-year survival statistics of this group of Saudi patients is 80% which compares favorably to other reports in the West. Emphasis is placed on management of the retinoblastoma patients by a multi-disciplinary group, and public educational efforts are imperative.

Anterior orbital varix presenting as a lacrimal sac mucocele.

Summary: A 35-year-old woman was referred to the Oculoplastics Clinic because of a left nasal swelling and intermittent tearing of 1 years duration. The clinical examination and echographic and radiologic findings were consistent with an anterior venous anomaly. Complete surgical excision was performed and histopathology confirmed the diagnosis of a varix.