【摘要】 To study the pattern of presentation and to highlight the common causes of primary benign orbital lesions.A prospective study conducted at the Department of Ophthalmology of Jinnah Postgraduate Medical Centre Karachi from July 1997 to August 2001 and then from September 2001 to date (Continued) at the Department of Ophthalmology of Chandka Medical College & Hospital Larkana. Only patients with primary benign orbital lesions were included in this study. All the patients were admitted in eye ward from the out patients department. The diagnosis of the disease was based on the presentation, clinical examination, investigations and histopathology of excised mass. A total of 68 patients were included in this study. The age range was from 2 months to 60 years. Out of 68, 27 (39.7%) patients were male and 41 (60.3%) were female. The left orbit was involved in 35 (51.5%) and right orbit was involved in 33 (48.5%).The analysis of 68 cases of primary benign orbital lesion revealed that superficial capillary hemangiomas of the eye lid were the most common lesion 26 (38.2%) followed by deep orbital cavernous hemangiomas 5 (7.4%), lymphangiomas 5 (7.4%), orbital varices 4 (5.9%), gliomas 7 (10.3%), meningiomas 5 (7.4%), neurofibromas 5 (7.4%), neurofibromatosis 4 (5.9%), schwannomas 2 (2.9%), and pleomorphic adenoma (benign mixed cell tumor) of lacrimal gland 5 (7.4%) cases.Early arrival, careful evaluation, proper diagnosis and treatment can prevent the patient from visual and life threatening complications.
【关键词】 benign orbital lesion primary diagnosis
INTRODUCTION
The orbital space occupying lesions may cause proptosis, that is displacement of the globe beyond the orbital margins, away from location of the lesion, with the patient looking straight a head. The lesion is usually unilateral, but may be bilateral[1]. The underlying etiology may be: congenital or acquired, inflammation (infective/non infective); tumor (benign/malignant); trauma (blunt/penetrating); degenerative.
The tumors arising from the orbital contents and eyeball are called as primary orbital tumors, from the tissues around the orbit ( Paranasalsinuses, meninges, brain, bone) are called as secondary orbital tumors and initially from distant body organs and then involve the orbit via hemopoietic reticuloendothelial system (RES) or lymphatic system are called as metastatic orbital tumors[2]. The tumor may be benign or malignant and may display infiltrative or non infiltrative behavior. Clinically, the benign noninfiltrative mass is usually associated with mass effect without destruction or entrapment. In contrast, a malignant infiltrative mass is usually associated with evidence of functional damage or entrapment. On investigation, both types are characterized by mass effect with displacement; however, malignant infiltrative lesion may have irregular margins, entrapped structures, and destruction of bone, as opposed to smooth, regular, noninfiltrative benign mass[3].
The symptoms and signs of the orbital lesions are manifested by displacement of the globe (axial; nonaxial), local pain, redness, swelling, decreasing vision, diplopia, congestion and edema of the eye lids and conjunctiva and exposure keratopathy. The fundus examination may shows optic disc edema/pallor/congestion; venous dilatation/tortuosity; choroidal folds and striae in the posterior fundus[4].
MATERIALS AND METHODS
A prospective study from July 1997 till to date (continued) was carried out at Ophthalmology Department of Jinnah Postgraduate Medical Centre, Karachi and Ophthalmology Department of Chandka Medical College Hospital Larkana combinedly to analyze the primary benign orbital lesions. All the patients were admitted in the eye ward from eye out patients department. After getting personal data and detailed history on the specific proforma all these patients were thoroughly examined (Ophthalmic, General and Systemic), investigated and treated properly. The diagnosis of disease was based on symptoms, signs, investigations and histopathology of excised mass. Only patients with primary benign orbital lesions were included in this study. To rule out site of origin and underlying cause of orbital lesion, various noninvasive and invasive investigations (where ever indicated) were performed.
For example, blood cell count and morphology (to rule out leukemia/lymphoma); Xrayorbits, skull, chest, pelvis(to rule out primary/secondary involvement of soft tissues and bones); ultrasound abdomen.(to rule out metastasis); ocular BScan.(to rule out status of eye globe); CT scan/MRI to obtain three dimensional view of involved mass/organ with its site/size/shape/surface/density (to rule out local tissue damage and extension); incisional/excisional biopsy of affected mass/organ/lymph node(to confirm the histopathologic diagnosis).
Table 1 Analysis of primary benign orbital lesions（略）
Figure 1 Superficial capillary haemangioma of eye lid （略）
A: Six months old baby with soft reddish, fleshy mass involving whole of the left upper eye lid. The left eyeball not visible; B: The same patient after local injection of steroids. Total three injections were given with interval of 2 months
RESULTS
The registered total number of patients with any of orbital lesions was 615, out of which 68 (11.1%) benign orbital lesions were picked up. The analysis of primary benign orbital lesions was shown in Table 1, and their treatment, postoperative complications were shown in Table 2 and 3, respectively.
DISCUSSION
Hamartomas means tumor like malformations result from developmental errors of vasoformative tissues. These, unlike true neoplasms, lack the power of unlimited, disproportionate growth, but become stationary after reaching their maximum size, often at a time when bodily growth stops. Therefore, hemangiomas and lymphangiomas are considered as hamartomas. Like the study of Douglas et al [5], we had also noticed hemangiomas more in females 21 (67.7%) cases than in males 10 (32.2%) out of 31 cases, and were characteristically unilateral. In our study the most common type of primary benign orbital lesion was hemangioma. It was of two types: capillary and cavernous. The superficial capillary hemangioma was common in children 26 cases and cavernous in adults 5 cases[6]. Vascular tumors in children are more compressible than those in adults, as connective tissues capsule does not develops till later in life[7]. All patients of superfical capillary hemangioma were presented with reddish fleshly growth with strawberry appearance, increasing in size with crying and cough, occupying the part or whole of the upper or lower eye lid since birth. Among these, 8 lesions were large enough to obstruct visual axis (Figure 1) and 7 lesions bleed frequently, which were treated with intralesion local steroid injections, and rest of small lesions were left as such (Figure 2). In our study, there were 5 cases (3 female and 2 male) of deep orbital cavernous hemangiomas. They were presented at the age of 3550 years with axial proptosis since 12 years. The proptosis was nonpulsatile, non tender, slightly reducible. All underwent for surgical resection of tumor through lateral orbitotomy and histopathology of mass confirmed the cavernous hemangioma (Figure 2).
Table 2 Treatment of primary benign orbital lesions（略）
Table 3 Postoperative complications（略）
Lymphangiomas are thin walled vascular channels, and hemodynamically isolated from the arterial and venous orbital circulation. They arouse curiosity because the orbit is normally devoid of lymphatic channels as well as lymphoid follicles. Wright et al[8] suggested that lymphagiomas are variant of venous malformations, but clinical, hemodynamic and histophathlogic studies strongly suggest that lymphangiomas are distinct orbital hamartomas. Two patients of superficial subcutaneous lymphangimas were presented with bluish coloured, small swelling, beneath the superionasal part of upper lid. The swelling was soft, reducible, and nontender (Figure 3). The other 3 patients of deep seated lymphangiomas were presented with eccentric proptosis with bluish mass in the superior fornix and below the lid. The size of proptosis increased in one patient with the presence of upper respiratory tract infection, reflecting the proliferating lymphoid elements in the connective tissue trabeculas of the tumor. Two superficial small size lymphangiomas were excised surgically and in 2 deep lymphangiomas local intralesion steroid injection was given through conjunctiva and in one case with large proptosis lateral orbitotomy was done in which tumor was excised incompletely because of the deep infiltration of the tumor, and in remnant tissues local intralesion steroids injection was given (Figure 2).
Figure 2 Deep orbital haemangioma（略）
A: Fortyfive years old male showing left eye proptosis; B: Photograph of the orbital mass (reddish, fleshy, 25x17mm size) of the same patient removed through lateral orbitotomy
Figure 3 Superficial lyphangioma of eye lid （略）
A: Seven months old boy with superficial, soft, reducible, nontender subcutaneous swelling involving left eye lids; B: The CT scan (axial view) of the same child showing the soft tissue swelling on the medial side of the left eye
Figure 4 Superficial orbital varix （略）
A: Thirty years old female with right eye superficial orbital varix at resting position; B: The same patient with Valsalvas maneuver showing classical finding of dilatation of blood vessels
Orbital varices are anomalies consist of either a single segmental irregular dilatation of a vein or a tangle of ectatic vascular channels. Varices may be primary (congenital) lesions, secondary (acquired) lesions or the sequelae of intracranial or orbital arteriovenous malformations[9]. Lesions with prominent connections to the systemic venous system are distensible and respond to changes in the venous circulation. They present with classical findings of proptosis and pain, which increase with Valsalvas maneuver[10]. In our study one patient presented with superficial lid varix (Figure 4) and three with deep orbital varices (Figure 5) were typically presented in their second to third decade of life with variable proptosis dependent on a change in downward head position (e.g during paryer) or on coughing. At rest, the affected eye looks relatively enophthalmic and pain free, but during period of extreme engorgement (ie with straining, bending forward or Valsalvas maneuver), the pain, proptosis and segmental dilatation of veins were apparent. For the most part, distensible venous lesions are not of serious consequence and do not require any intervention. The indications for surgical treatment are usually related to pain, cosmesis, or progressive expansion. The direct surgical treatment of distensible venous malformations of the orbit is difficult due to the tortuous tangles of fragile, thinwalled, malformed vessels and their tendency to rupture and bleed excessively. Therefore, all these four patients were referred to department of vascular surgery where excision of abnormal vessels was done after isolation, ligation, clipping and thromboembolization.
Figure 5 Deep orbital varix （略）
A: 25 years old male with right eye deep orbital varix at resting position; B: The same patient with Valsalva's maneuver showing classical finding of increasing proptosis
Figure 6 Optic nerve glioma（略）
A: Six years old boy with left eye axial proptosis; B: Photograph of same patient showing multiple caféaulait spots of varying sizes on the back and buttock; C: Photograph of same patient showing orbital cellulitis just after the lateral orbitotomy. The excised mass biopsy shows optic nerve glioma
Optic nerve glioma was first described in 1821 by PANIZA, and over 700 optic nerve gliomas have been described in the literature since. Approximately 75% of cases have occurred in patients less than 10 years of old; most present between the ages of 5 and 8. Optic nerve gliomas account for approximately 5% to 10% of pediatric orbital tumors. This tumor may occur as an isolated finding or as a part of neurofibromatosis. In patients with neurofibromatosis, the incidence of optic nerve glioma is between 12% and 38%. Conversely, approximately 25% to 50% of patients with optic nerve glioma have neurofibromatosis[11]. In our study, there were 7 cases of optic nerve gliomas of age range in 413 years and were comprised 10.29% of total 68 primary benign orbital tumors. Five presented with unilateral axial proptosis, two with unilateral nonaxal proptosis. The proptosis was insidious, nonreducible, nonpulsatile, and with mild to moderate tenderness in all cases. Visual loss was moderate to severe (unilateral 5 cases, bilateral 2 cases) and with strabismus in 2 cases. The pupil showed relative afferent pupillary defect in 5 cases, afferent pupilliary defect in 2 cases and fundus showed disc hyperemia in 2 cases, disc pallor in 2 cases, disc swelling with blurred margin in 3 cases and chorioretinal folds at posterior pole in 4 cases. The CTscan showed conical shaped mass surrounding the intra orbital part of optic nerve in 3 cases, fusiform shaped mass around the orbital and cranial part of optic nerve in 2 cases, oval shaped mass along the chiasm in 1 case and multiple, small, round masses along the chiasm and optic tract in 1 case. The 4 (57.1%) out of 7 patients showing caféaulait spots on the skin of back, leg, arms, buttocks, neck (Figure 6)and 2 of them were associated with Neurofibroma. One case of orbital glioma with minimal proptosis (2mm), stationary vision was observed for 5 years and more, and 2 patients of orbital glioma with moderate (24mm) proptosis, decreasing vision were underwent for excision of tumor through lateral orbitotomy (Figure 6). Two patients of orbitocranial gliomas were referred to neurosurgeon where pre and para chiasmal tumors were treated by enblock surgical excision of the involved nerve just anterior of chiason and finally 2 cases of chiasmal, diffuse multifocal gliomas with significant proptosis and visual loss were treated by chemotherapy.
Optic nerve sheath meningioma is the most common adult optic nerve tumor. Meningiomas account for approximately 20% of the central nervous system tumors; less than 15% secondarily involve the orbit. Orbital involvement by meningiomas can occur as a result of primary intraorbital neoplasm, almost always arising from the optic nerve sheath, or secondarily from a meningioma that involves the orbit by contiguous spread from the central nervous system. Orbital involvement by meningiomas, both primary and secondary, occurs more frequently in females[12]. Similarly we have seen optic nerve sheath meningiomas in 3 middle aged females and in 2 males with symptoms and signs of decreased vision, headache, diplopia, relevant afferent pupillary defect, optic disc edema in 3 cases, optocilliary shunt vessels in 2 cases, minimal axial proptosis in 4 cases and significant nonaxial
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