Journal of Neuro-Ophthalmology, March 1981, Volume 1, Issue 1

Orbital subperiosteal hematoma (epidural hematoma of the orbit).

Three cases are presented of subperiosteal hematomas, which can be thought of as epidural hematomas of the orbit. One occurred in a child associated with an episode of violent vomiting, the second in an adult with a history of remote head trauma who presented with a slowly progressive course suggestive of thyroid eye disease, and the third in a young patient after acute trauma probably associated with the injections of a local anesthetic agent or possibly the passage of a deep suture. Skull x-rays, computed tomography, and ultrasonographic examinations detected and localized the lesion in each case. Both patients who were operated on enjoyed prompt and total resolution of their symptoms after surgical extirpation of the lesion. In the third patient, the lesion regressed on its own.

f. Clill. Neuro-ophthalmoJ. 1: '45-52, 1981. Orbital Subperiosteal Hematoma (Epidural Hematoma of the Orbit) RAANANAH SWIRSKY KATZ, M.D. GARY ABRAMS, M.D. Abstract Three cases a~ presented of subperiosteal hemato­mas, which can be thought of as epidural hematomas of the orbit. One occurred in a child associated with an episode of violent vomiting, the second in an adult with a history of remote heild triluma who presented with a slowly progressive course suggestive of thyroid eye disease, ilnd the third in a young patient after acute trauma probably ilssociilted with the injections of a local anesthetic ilgent or possibly the passage of a deep sutu~. Skull x-rays, computed tomography, and ultrasonogrilphic examiniltions detected and localized the lesion in each Cilse. Both piltients who were oper­ated on enjoyed prompt ilnd total resolution of their symptoms after surgicill extirpillion of the lesion. In the third patient, the lesion regressed on its own. Introduction Proptosis is an important clinical sign which can be produced by a host of conditions both benign and malignant. An orbital subperiosteal hematoma, although an uncommon condition, is a distinct clinical entity which should be included in the differential diagnosis of proptosis 1-10. We report on three cases of orbital subperiosteal hematoma, the first presenting with acute proptosis in a child, the second with chronic slowly progressive exoph­thalmos in an adult, and the third as acute proptosis following direct trauma to the orbit in an adoles­cent male. Case 1 (BPEI # 07 8215-1) This 9-year-old Caucasian girl was in good health until 1 week prior to admission when she developed malaise, low-grade fever, cou.ghing, nausea, and vomiting. Immediately followmg an episode of vomiting, the chJld complained of retro­ocular pain, and her mother observed that the child's left eye was suddenly displaced forward From the Department of Ophthalmology, Basco~.Palmer Eye Institute, University of Miami School of MediCine, Miami, Florida. March 1981 and laterally. No ecchymosis was noted. The prop­tosis gradually increased over the week, although the systemic symptoms resolved. Neurologic ex­amination was normal but for the ocular disturb­ance. Computed tomography of the left orbit dem­onstrated a soft-tissue density in the superior apex of the left orbit. For this reason the patient was referred to the Bascom Palmer Eye Institute for further evaluation. Ocular examination disclosed a visual acuity of 20/20 in both eyes. Hertel exophthalmometry reading with a base of 90 was 13 mm in the right eye and 20 mm in the left eye. There was marked restriction of the left globe in upgaze, and the patient had horizontal diplopia in all fields of gaze. There was distention of the lid veins but no ecchy­mosis. The orbital rims were intact. No pulsations or bruits were noted. Conjunctiva and sclera were not injected in either eye; anterior segment exam­ination was within normal limits. Funduscopy of the right eye was normal, but choroidal folds were present in the left eye. Laboratory studies including a hemogram, prothrombin time, partial thrombo­plastin, and platelet count were within normal limits. She gave no history of bleeding tendencies or easy bruising. Ultrasound examination of the lefl orbit with contact B-scan and standardized A­scan showed a large extraconal mass lesion in the superior orbit which extended from 10:30 to 2:00 (Fig. 1). The lesion had regular low reflectivity (0­5%) and was thought 10 be diffusely outlined. The lesion was minimally compressible. The extraocu­lar muscles and optic nerve were within normal limits. The lesion was echographically compatible with a sarcoma, lymphoma, or pseudotumor, and a rhabdomyosarcoma was suspected clinically. The patient was taken to the operating room for exploration of the lesion. A curvilinear incision was made beneath the lefl brow in the superonasal aspect of the left orbital rim. The periosteum was reflected downward, and a large, extraperiosteal, pecan-Sized, reddish-black mass was encountered. The lesion was encased in a pseudocapsule, the perforation of which produced a gush of serosan­guinous fluid. Frozen section of the mass revealed an organized blood clot. The bulk of the lesion was excised, the wound closed, and a Penrose drain 45 Subperioste~1 Hem~tom~ Figure t. Contact B and standardized A echogram of the left orbit demonstrating .a large extra(onal mass in the superior orbit. inserted. The patient made an uneventful recovery and 1 year later was asymptomatic. Case 2 (BPEI # 06 45 99-4) A 32-year-old Caucasian female previously in good health presented to her ophthalmologist in August 1977 because of the recent onset of a "protruding right eye." Soon thereafter, she ob­served blurry vision in the right eye and horizontal diplopia on right gaze. There was no history of recent trauma, but she had suffered a minor head injury in an automobile accident several years be­fore. Thyroid function tests were within normal limits. She was referred for evaluation of unilateral proptosis. Ocular examination revealed a best corrected visual acuity of 20/20 in the right eye and 20/15 in the left eye. The right eye was proptosed and displaced downward (Fig. 2). Hertel exophthalmo­metry reading with a base of 98 was 23 mm in the right eye and 20.5 mm in the left eye. Quantitative phorias with the Maddox rod demonstrated 2 diop­ters of left hypertropia in all fields of gaze. The anterior segments were normal on slit lamp ex- 46 Figure 2. Cionical photograph of case 2. Note proptosis and slight downwdfd dISplacement of the right globe. Figure 3. Fundus photogroph of case 2 showing horizontal chorOIdal striae on the posterior pole of the right eye. amination. Funduscopy of the right eye revealed numerous horizontal striae coursing through the posterior pole; the left fundus was unremarkable (Fig. 3). A diagnosis of euthyroid Grave's disease was made. Repeat examination 1 year later revealed the clinical picture to be unchanged except for a slight increase of right proptosis, which now measured 4 mm. Computed tomography of the right orbit dem­onstrated a lesion in the right superotemporal orbit (Fig. 4). On ultrasonographic examination, the ex­traconal mass was poorly outlined and of low reflectivity (Fig. 5). Laminograms displayed a bony defect in the wall of the orbit. A lacrimal gland tumor was suspected, and the patient was admitted for a biopsy of this lesion. Journal of Clinical Neuro-ophthalmology K~IZ. Abrams Ib) Figures 4 .. ~nd 4 b.: cr "dn of thr rillhl orbIt in Cdsr 2 drmonslrdtinl( d Irsion suprrotrmpordlly In thr right orb•• Physical examination and laboratory workup on admission to the hospital were entirely within nor­mal limits. The patient gave no history of blood dyscrasias or bleeding tendencies; clotting studies were unremarkable. At surgery, an incision was made over the ante­rior portion of the orbital rim temporally and carried down to the orbital septum, which was March 1981 opened across the lid. Upon "pening and reflecting the periosteum, d Idrge rubbery firm mass was encountered. Superiorly it was infiltrating the bone and no capsule was present. Frozen section was compatible with an organized hematoma. After subtotal extirpation of the lesion, the wound was closed with a drain in place. On follow-up examination, the patient's acuity 47 Subperiosteal Hematoma Figure S. Contact Band .tandardized A "chogrdm from ca." 2 demonstrating an "~lr.conal ma•• of low reOectivity. was 20/20 in both eyes, with complete resolution of the proptosis and disappearance of the choroidal striae. Case 3 (BPEI # 07 23 11-4) A 12-year-old black youth previously in good health was struck over the left eye with a golf club. He was taken to a local emergency room where the wound over the left brow was treated. He received periorbital injections of a local anesthetic agent, and six deep sutures and multiple superficial stitches were required to reapproximate the tissues. Either coincident with the injections or the passage of one of the sutures, he related that suddenly he developed marked proptosis of the left eye. Over the ensuing ten days his clinical picture remained unchanged. When seen in the triage room at the Bascom Palmer Eye Institute, the patient had marked prop­tosis and downward displacement of the left globe. Visual acuity with correction was 20/25 in the right eye and 20/40 in the left eye. Hertel exophthal­mometry with a base of 112 was 18 in the right eye and 26 in the left eye. The pupils were 5 mm each and reacted briskly and symmetrically to a light stimulus; no afferent pupillary defect was present. There was marked limitation of elevation of the left eye in upgilze and moderate restriction in 48 adduction of the left globe. The patient noted diplopia in all fields of gaze except in the primary position. Versions of the right eye were full. On external examination there were moderate ecchy­moses and edema of the left upper lid and perior­bital area. The wound was healing well. The eyes were white and quiet. Slit lamp and fundus ex­aminations were within normal limits. Ultrasound examination of the left orbit dis­closed the presence of a well-outlined extraconal mass in the superior and superonasal aspects of the orbit extending from 9:00 to 1:30 (Fig. 6). The lesion had regular low reflectivity and was well outlined with a regular surface. These findings were thought to be compatible with a subperiosteal hematoma of the left orbit. Computed tomography (CTI of the left orbit was performed without and with contrast on the high rl'solution GE CT/T 8800 scanner (General Electric Company. 4855 Electric Ave.. Milwaukee, Wis.). Five-millimeter-thick sections were obtained in both the transaxial and coronal projections (Fig. 7). The noncontrast scan revealed a soft tissue mass of unhomogenous denSity in an extraconal position in the left superior orbit. There was extension of this mass into the soft tissues anterior to the orbit. This lesion, which was causing mild proptosis, was characterized by a central area of low density and an isodense soft-tissue periphery. With contrast Journal of Clinical Neuro-ophthalmology KJtz, Abrams I,,) (e) Figu~ 6. Contact B and sldndardiz~d A ~chogr..m from case 3. (d. b) Transocular and (c. d). paraocular. demonstrating w~lI-"ulhned mass of low reflectivity injection there was enhancement of the soft-tissue rim of the lesion but not of its hypodense center. Opacification of the left frontal and ethmoid si­nuses were seen in assocation. There were, how­ever, no osseous abnormalities. The differential diagnosis based on this cr picture was either an orbital abscess or chronic orbital hematoma. Although drainage of the hematoma was consid­ered, it WdS elected simply to observe this patient since he was clinically stable dnd his vision was not threatened. On follow-up eXdmination 1 month later, the patient's visual acuity was 20/20 in both eyes. But for very minimdl restriction in upgaze of the left eye, the patient's versions were quite full. There was a slight residual depression of the left globe with mild lid ecchymoses. Six months Idter there was total resolution of his oculdr problem, and he was asymptomdtic. Discussion Orbital hemorrhages can be divided into two groups based on their location: the more common (orbital) blood cyst occurring within the confines of the orbital cavity, and the subperiosteal hema­toma located between the bony orbit and the ad­herent periosteal tissues. Subperiosteal hemato­mas, although rare, constitute a well-recognized clinical entity.u-IQ Systemic illness, including MMch 1981 scurvy,2.7, hemophilia, and uremia, have been as­sociated with subperiosteal hematomas. While the most common cause of all types of orbital hemor­rhages, both intraorbital and subperiosteal, is blunt traumd to the orbit typically in young males, the spontaneous occurrence of a subperiosteal hema­toma was reported by Whitwe1l5 in a 63-year-old lady. Subperiosteal hematomas typically present with the abrupt onset of unilateral proptosis with downward and lateral displacement of the globe. Lid edema is an infrequent symptom, as is subcon­junctival hemorrhage or signiFicdnt pain. Slowly progressive proptosis over the course of several months also can occur." Our cases illustrate the two disparate presentations of this entity, acute proptosis in two cases dnd slowly progressive prop­tosis in the other case. Acute proptosis in a child as seen in our first pdlient can be cdused by d wide range of disorders. In the past, scurvy WdS d common cause of sub­periostedl hemdtomds, especidlly of the long bones. Orbitdl subperiostedl hemdtomas were apparently rather common in this group of patients. In some series,2- 7 the incidence of orbital subperiosteal he­matomas in scorbutic children is as high as 10%. Others~' 7 concluded that this statistic is falsely elevated by poor documentation. Dunningham's2 review of the literature dnd the report of his Cdses brings the total of well-detailed cases of exoph- 49 Subperiosteal Hematoma (<>/ lbl Figure 7. Trdns,...1(J) dnd coron.1 (to) c"nlra,1 CT SC,lO rrvNI ., rlOg-cnh,lOClng mJSS (arnlwsl with centr,l low dcn ity In Ih(' Icft ,upero", "rbit. thalmos with scurvy to 23. In his series the children were usually less than 1 year of age (average agE' was lOlh months). The youngest patient was 7 months old, and the oldest was two years of age. Treatment with vitamin C was beneficial in his case. 50 In considering the differential diagnosis of our first p.ltient. inflammatory orbital disease including orbitdl cellulitis. abscess, or pseudotumor was un­likely due to the lack of orbital inflammatory signs. There was no vascular dilatation to indicate an drteriovenous fistula. A venous varix with hemor- Journal of Clinical Neuro-ophthalmology rhage could not be clinically ruled out. This child was older than most children with metastatic neu­roblastoma. While there was no history of trauma, the acute onset of downward and lateral displace­ment of the globe associated with the Valsalva effect of vomiting suggested the diagnosis of a subperiosteal hematoma. This is akin to the picture seen in scorbutic infants2 in whom the association of crying (Valsalva effect) and proptosis was noted. This rapid onset also suggested a rhabdomyosar­coma and the ultrasound picture was compatible with this diagnosis. Surgery was indicated to rule out the diagnosis of rhabdomyosarcoma. Less dramatic in presentation but just as confus­ing in diagnosis is the chronic, slowly progressive course subperiosteal hematomas may take, as shown by our second case carried with the diag­nosis of thryoid eye disease for 1 year. Progression of the proptosis, choroidal folds, and the down­ward displacement of the eye suggested the pres­ence of a mass lesion. The clinical suspicion of an orbital mass lesion was confirmed on CT scanning, ultrasonographic examination, and orbital lami­nograms. The laminograms demonstrated bony er­osion which was most likely a consequence of the organization of the hematoma, and the bony in­volvment seen at surgery. The location of the lesion suggested a tumor of the lacrimal gland. The ultra­sonographic examination suggested a lesion of the lymphoma, sarcoma, pseudotumor group of dis­eases, and laminograms with bony erosion pointed to a possible malignant lesion. Surgical biopsy was necessary for diagnosis in this case. In his series of five cases of orbital subperiosteal hematoma ranging in age from a baby boy to an 18-year-old male, WolterlO emphasized that all of his cases were in males and associated with head trauma. All but one presented with acute proptosis, and in that instance the hematoma developed spon­taneously 4 days after repair of a perforated globe. Aspiration of the clot was uniformly successful and without complication in his series with prompt resolution of the proptosis. While it is true that subperiosteal hematomas occur in connection with blunt head trauma, in our first two cases the history of trauma was conspicuous by its absence. The third case illustrates the more typical pre­sentation of proptosis accompanying direct trauma either from the golf club or the injection of anes­thetic solution. There was gradual and total reso­lution of the patient's problem over 3 months without resorting to surgical intervention. Had his vision been in danger, or had the proptosis not resolved within 3 months, drainage or excision of the hematoma would have been undertaken. It is interesting to speculate on the pathogenesis of these lesions. At the orbital end of the optic canal the dura splits into two layers, one becoming continuous with the periorbita, the other with the March 1981 Katz, Abrams dura of the optic nerve. The periosteum, which is loosely adherent to the orbital bones, is divided into several distinct pockets by areas of firm fixa­tion to the orbital structures. The orbital perios­teum extends anteriorly from the orbital margin at the arcus marginalis posteriorly through the supe­rior orbital fissure, optic fissure, and anterior eth­moidal canal where it becomes continuous with the periosteal layers of the dura. Inferiorly, via the inferior orbital fissure, the orbital periosteum is continuous with the periosteal lining of the infra­temporal and pterygopalatine fossa; temporally, it blends with that of the temporal canal and through the zygomatic canal it becomes continuous with that on the front of the lacrimal bone. The trian­gular wedge of periosteum lining the superior orbit is tethered in its anterior aspect at the orbital margin, laterally at the zygomatic suture, and na­sally at the lacrimal fossa and anterior ethmoidal foramen, and at the posterior aspect by the superior orbital fissure, orbital foramen, and various struc­tures. II A sudden hemorrhage into this space would result in proptosis with downward and lat­eral displacement of the globe. InjUry to the small periosteal vessels or possibly the larger anterior or posterior ethmoidal arteries would be a likely source for the hemorrhage. The treatment of orbital subperiosteal hemato­mas is usually surgical. lO • 11 In one of Wolter's cases, the hematoma acted as a large space-occu­pying lesion within the orbit and led to compro­mise of the central retinal artery with resultant loss of vision. While success has been reported in some cases with needle aspiration of the acute hematoma through the superior cul-de-sac approach, surgery allows definitive histologic diagnosis and removal of the organized hematoma. In our first two cases, the hematoma was evacuated following exposure through an anterior orbital incision. A Penrose drain was left in place, and resolution was uncom­plicated and complete in these patients. Sponta­neous resolution of the subperiosteal hematoma occurred in the third patient. Evaluation of the patient suspected of having an orbital subperiosteal hematoma should begin with a good history and ocular examination. Specifi­cally, a history of trauma, Valsalva effect as in coughing or crying, or injection of local anesthetic agents should be sought. On examination the eye will be proptosed and displaced downward or downward and laterally. On funduscopic exami­nation choroidal striae may be present. Forced ductions are negative. Ultrasonographic studies will demonstrate an extraconal well-outlined lesion in the superior orbit of regular low reflectivity (0­5%). CT scan without and with contrast injection will reveal a mass that is characterized by a central nonenhancing area of low density surounded by an isodense soft-tissue periphery which enhances. 51 Subperiosteal Hematoma References 1. Wolter, J.R., Leehouts, J.A., and Coulthard, S.W.: Clinical picture and management of subperiosteal hem<1toma of the orbit. /. Ped. Ophthalmol. Strab. 13: 136, 1976. 2. Dunnington, J.H.: Exophthalmos in infantile scurvy. Arch. Ophthalmol. 6: 731, 1931. 3. Mueller, W., and Geppert, J.: Traumatisches Sub­periostales Hematom der Orbita. Klin. Monatsbl. Augenheilkd. 153: 795, 1968. 4. Reese. A.B.: Tumors of the Eye (3rd ed.). Harper & Row, Hagerstown, Md., 1976, p. 443. 5. Whitwell, J.: Spontaneous hematoma of the orbit. 8r. /. Ophthalmol. 40; 250, 1956. 6. Awerbach, M.1.: Les tumeurs sanguines de la cavite orbitaire. Ann. Ocul. 170: 863, 1933. 7. DeBuys, L.R.: Exophthalmos in scurvy. ].A.M.A. 59: 2040, 1912. 8. Denig, R.: Subperiosteal bloodcyst of the orbit; re­port of a case. Ophthalmol. Rec. 11: 187, 1902. 52 9. Roberts, W.: Hematoma of the orbit. Am. ]. Oph­thalmol. 40: 215, 1955. 10. Wolter, J.R.: Subperiosteal hematomas of the orbit in young males: a serious complication of trauma or surgery in the eye region. Trans. Am. Ophthalmol. Soc. 71: 104, 1979. 11. Warwick, R.: An.Jtomy of the Eye and Orbit. H.K. Lewis and Co., Philadelphia, 1976, p. ZO. Acknowledgment The authors are grateful to Judith Donovan Post, M.D., for reviewing the radiographic interpretations of this paper. Dr. Richard Tenzel evaluated and performed the op­eration on the first patient; Dr. Joel Glaser on the second patient. Write for reprints to: Raananah Swirsky Katz, 900 N.W. 17 Street, Miami, Florida 33136. Joumal of Clinical Neuro-ophthalmology