Surgical Management of Necrotizing Scleritis Scleral Reinforcement With Calvin W, Breslin, MD;
Jeffrey
I. Katz,
Autogenous Periosteum
MD; Herbert
\s=b\ We successfully used autogenous periosteum to reinforce the weakened sclera of two patients with necrotizing scleritis. One of these patients previously had an onlay scleral graft made from homologous banked sclera, which had subsequently melted. Autogenous periosteum is suggested as an alternative to
banked sclera for scleral reinforcement.
(Arch Ophthalmol 95:2038-2040, 1977) scleritis is an uncombut form of scierai disease. It may be associated with long-standing rheumatoid arthritis (scleromalacia perforans), with the acquired connective tissue group of diseases, or the process may be limited to the eye without manifest systemic involvement. Characteristically, there is destruction of the scierai collagen with exposure of the underlying uveal tissue. Therapy aimed at halting the progression of the disease process, at strengthening the weakened sciera, or both, had met with varying degrees of success. In our experience and in the experience of others,'"1 the use of banked
Necrotizing potentially devastating mon
for publication Dec 6, 1976. From the Department of Ophthalmology, College of Medicine, University of Florida, Gainesville. Reprint requests to University of Toronto, Department of Ophthalmology, Rm 115, 1 Spadina Crescent, Toronto, Ontario, M5S 2J6 Canada (Dr Breslin).
Accepted
E.
Kaufman, MD
sciera to support the weakened globes of patients with necrotizing scleritis has, in some instances, met with failure. This was because the donor sciera either became involved in the ongoing necrotic process or the scierai graft was rejected by the host and melted away. A search was initiated, therefore, for a suitable alternative to homologous sciera. The purpose of this report is to describe the successful use of autogenous periosteum in reinforcing the sciera of two patients with necrotizing scleritis. One of the
patients previously had
an
onlay
scierai graft made from homologous banked sciera, which subsequently melted. REPORT OF CASES Case l.-A healthy 78-year-old woman underwent an uneventful left intracapsular cataract extraction on Feb 2, 1975. Postoperatively, there was persistent inflammation and pain in the operated eye, which was unresponsive to topically applied corticosteroids. The development of scierai staphylomas occasioned the patient's referral to the University of
Florida. When first examined on Dec 4,1975, the visual acuity was correctable to 20/30 + 2 in the right eye and 20/60 -2 in the left eye. The right eye was normal in appearance. The bulbar conjunctiva of the left eye was hyperemic and chemotic. The sciera, above the horizontal meridian, was
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thinned and transparent, exposing the underlying bluish uveal tissue. In the 10 o'clock and 2 o'clock meridians, approximately 3 mm behind the limbus, were large scierai staphylomas covered by atrophie conjunctiva. The peripheral cornea in this region was guttered. There was a moderate anterior uveitis. The intraocular pressures were 15 mm Hg in the right eye and 30 mm Hg in the left. History and general physical examination failed to disclose any evidence of a generalized connective tissue disorder.
Laboratory investigations demonstrated a positive rheumatoid factor (1:160) and a negative antinuclear antibody. On Dec 5,1975, the sciera of the left eye
above the horizontal meridian was reinforced with an onlay graft of homologous banked sciera. The graft was sutured into normal cornea and sciera with interrupted sutures of 7-0 chromic catgut and extended from the insertions of the recti muscles posteriorly to the vertical edge of a 2-mm
wide
superficial keratectomy anteriorly.
The graft was covered with conjunctiva from the upper fornix. Postoperatively, the eye was pain-free. Dexamethasone drops, 0.1%, were used four times daily. On Jan 15, 1976, the patient returned to the University of Florida, relating a twoweek history of a painful, red, left eye. Results of the examination showed that the conjunctiva covering the scierai graft had become avascular and necrotic, and there was melting of the scierai graft itself (Fig 1). The peripheral cornea was guttered and anastomotie vessels had formed between the anterior ciliary sys-
1.—Left eye (patient 1) six weeks following scierai reinforcement with homologous banked sciera. Conjunctiva covering scierai graft is avascular and necrotic. There is melting of scierai
as in Fig 1 nine months following scierai reinforcement with autogenous periosteum. Staphylomatous areas are flat and well covered by vascular periosteum and
Fig
Fig 2.—Same eye
graft.
conjunctiva.
Fig 3.—Right eye (patient 2) prior to scierai reinforcement. There is large scierai staphyloma in upper nasal quadrant, covered by necrotic and atrophie conjunctiva and episclera.
Fig
months after scierai reinforcement with banked solera. Lifting of scierai graft is accompanied by dellen formation, corneal vascularization, and lipid deposi-
Fig
Fig 5—Right eye (patient 2) three tion.
4.—Left eye (patient 2) showing scierai staphyloma in upper nasal quadrant. Necrotic scierai nodule is present in the 12 o'clock meridian. Note peripheral guttering of cornea.
6.—Left eye (patient 2) nine months following scierai reinforcement with autogenous periosteum. Staphylomatous areas are flat and well covered by vascular periosteum and conjunctiva. Peripheral guttering of cornea has been halted.
Downloaded From: http://archopht.jamanetwork.com/ by a Michigan State University User on 06/18/2015
tern at the limbus and the vessels at the
origins of the recti muscles. There moderate anterior uveitis. The 10P
was a was
22
Hg. The following day,
mm
Jan 16, 1976, the weakened sciera of the patient's left eye was reinforced with an onlay graft of
autogenous periosteum.
The periosteum was obtained from the anterior tibial crest of the patient's left leg in the following manner. Under local infiltration, commencing 8 cm below the anterior tibial tubercle, a vertical skin incision was made and carried down to the bone. The muscles wore bluntly dissected off the periosteum. A 7 X 2-cm strip of" periosteum was incised with scalpel blade, lifted with periosteal elevator, and excised with scissors. A Penrose drain was placed in the wound, which was subsequently closed in two layers. One gram of cefazolin sodium was administered intravenously. Following lid akinesia and retrobulbar anesthesia, the necrotic scierai graft was removed from the superior aspect of the patient's left globe. Lamellar dissection was carried into normal sciera posteriorly and into clear cornea anteriorly as a 3-mm wide superficial keratectomy. The periosteal graft was sutured in place with interrupted sutures of 8-0 black silk and covered with conjunctiva from the upper fornix. Postoperatively, the patient was given 80 mg of prednisone and 1 gm cefazolin sodium daily. The eye became free of pain. The Penrose drain was mobilized on the second postoperative day and removed on the fourth. The antibiotics were discontinued on the third postoperative day. Dexamethasone drops, 0.1%, were used four times daily. When last examined, nine months' postoperatively, the left eye was quiet and pain-free. The staphylomatous areas were flat and well covered by periosteum and conjunctiva (Fig 2). The visual acuity was correctable to 20/70. Cask 2.-A 58-year-old woman with a ton-year history of severe, crippling rheumatoid arthritis (class IV, stage IV) was seen in referral at the University of Florida on Sept 9, 1975, for evaluation and treatment of severe bilateral scleromalacia
perforans. The patient
of her ocular condition. Her only complaints were of decreasing vision and mild photophobia. Her visual acuity was correctable to 20/ 40 in the right eye and 20/200 in the left eye. The bulbar conjunctiva of both eyes was diffusely injected and abnormal anastomotic vessels were present at the limbus bilaterally. In the upper nasal quadrant of the right eye was a largo scierai staphyloma covered by atrophie conjunctiva and was unaware
episclera (Fig 3).
A smaller scierai staphyvisible in the upper nasal quadrant of the left eye and was accompanied by a yellowish necrotic scierai loma
was
nodule in the 12 o'clock meridian, approximately 3 mm behind the limbus (Fig 4). The peripheral cornea of both eyes was guttered. A moderate anterior uveitis was present bilaterally, accompained by diffuse endothelial keratic precipitates and multiple posterior synechiae. There were bilateral posterior subcapsular cataracts. The IOP was 34 mm Hg in the right eye and 28 mm Hg in the left eye. Laboratory investigations showed a positive rheumatoid factor (1;10, 240) and a negative antinuelear antibody. On Sept 11, 1975, the large scierai defect in the patient's right eye was strengthened with an onlay graft of homologous-banked sciera. The postoperative course was uneventful. Dexamethasone drops, 0.1%, were used four times daily and methazolamide was taken for control of the elevated IOP. Management of the rheumatoid vasculitis was attempted with prednisone (10 mg) and cyclophosphamide (400
mg) daily.
During the subsequent three-month postoperative period, the anterior edge of the scierai graft began to lift. This was accompanied by the development of a peripheral corneal dellen. Vascularization of the base of the dellen was accompanied by lipid deposition midstromally (Fig 5). Progression of the cataracts during this time period reduced visual acuity to 20/200 in the right eye and 8/400 in the left. The patient underwent an uneventful left intracapsular cataract extraction on Dec 2, 1975. Three weeks' postoperatively the vision in the left eye was correctable to 20/60. On Jan 26, 1976, the patient came to the University of Florida, complaining of a painful left eye. The corrected visual acuity was now 20/100 in the right eye and counting fingers at two feet in the left eye. The findings in the right eye were essentially unchanged from the previous examinations. The left eye exhibited substantial progression of the scierai disease with enlargement of the scierai defects in the 10 o'clock and 12 o'clock meridians. The peripheral keratolysis had increased in extent. These
changes
accompanied by a moderate anterior uveitis, an updrawing of the pupil, and the formation of posterior synechiae between the iris and were
the vitreous face. The IOP
was
18
mm
Hg.
The following day, Jan 27, 1976, the weakened cornea and sciera of the patient's left eye was reinforced with an onlay graft of autogenous periosteum. The
Downloaded From: http://archopht.jamanetwork.com/ by a Michigan State University User on 06/18/2015
surgical technique
was similar to that described for case 1 with the exception that a Penrose drain was not placed in the leg wound at the time of closure. Postoperatively, the eye remained quiet and free of pain. Doxamethasone drops, 0.1%, were used four times daily. Rheumatoid vasculitis therapy included prednisono (10 mg) and penicillamine hydrochloride
(750 mg) daily.
Nine months following surgery the periosteal graft was well in place and covered by conjunctiva (Fig 6). The eye was quiet and pain-free. The peripheral keratolysis showed no signs of progression. Visual acuity remained at counting fingers because of the updrawn pupil.
COMMENT Periosteum is the specialized
con-
nective tissue surrounding bone. It consists of an outer dense layer of collagenous fibers and fibroblasts and an inner, looser layer of osteogenic cells.' In the human, transplanted periosteum does not exhibit its osteogenic potential. Periosteum is readily available, strong, and easy to work with. The fact that it is autogenous and vascular gives it distinct advantages over banked sciera. The scleritis seen in our patients would seem to be on an autoimmune basis and may, in fact, be tissuespecific. If this is the case, periosteum may have substantially less risk of being involved with recurrences of the necrotizing process. Cardona has used periosteum for the support of keratoprosthesis with good results.'1 Our experience with autogenous periosteum for scierai reinforcement would suggest that it is as good as homologous-banked sciera and may, in fact, be superior. This study was supported in part of Health fellowship.
Ministry
by an Ontario
References 1. Torchia RT, Dunn RE, Pease PJ: Fascia lata grafting in scleromalacia perforans with lamellar
corneal-scleral dissection. Am J
66:705, 1968.
2. Rosenthal
Ophthalmol
JW, Williams GT: Scleromalacia
perforans as a complication of rheumatoid arthritis. Am J Ophthalmol 54:862, 1962. 3. Watson PG, Hayreh SS: Scleritis and episcleritis. Br J Ophthalmol 60:163, 1976. 4. Peer LA: Transplantation of tissues: Carti-
lage, Bone, Fascia, Tendon and Muscle. New York, Williams & Wilkins Co, 1955, vol 1.
5. Cardona H: Prosthokeratoplasty in chemical burns and ocular pemphigoid. Can J Ophthalmol 11:35, 1976.
Jeffrey
I. Katz,
Autogenous Periosteum
MD; Herbert
\s=b\ We successfully used autogenous periosteum to reinforce the weakened sclera of two patients with necrotizing scleritis. One of these patients previously had an onlay scleral graft made from homologous banked sclera, which had subsequently melted. Autogenous periosteum is suggested as an alternative to
banked sclera for scleral reinforcement.
(Arch Ophthalmol 95:2038-2040, 1977) scleritis is an uncombut form of scierai disease. It may be associated with long-standing rheumatoid arthritis (scleromalacia perforans), with the acquired connective tissue group of diseases, or the process may be limited to the eye without manifest systemic involvement. Characteristically, there is destruction of the scierai collagen with exposure of the underlying uveal tissue. Therapy aimed at halting the progression of the disease process, at strengthening the weakened sciera, or both, had met with varying degrees of success. In our experience and in the experience of others,'"1 the use of banked
Necrotizing potentially devastating mon
for publication Dec 6, 1976. From the Department of Ophthalmology, College of Medicine, University of Florida, Gainesville. Reprint requests to University of Toronto, Department of Ophthalmology, Rm 115, 1 Spadina Crescent, Toronto, Ontario, M5S 2J6 Canada (Dr Breslin).
Accepted
E.
Kaufman, MD
sciera to support the weakened globes of patients with necrotizing scleritis has, in some instances, met with failure. This was because the donor sciera either became involved in the ongoing necrotic process or the scierai graft was rejected by the host and melted away. A search was initiated, therefore, for a suitable alternative to homologous sciera. The purpose of this report is to describe the successful use of autogenous periosteum in reinforcing the sciera of two patients with necrotizing scleritis. One of the
patients previously had
an
onlay
scierai graft made from homologous banked sciera, which subsequently melted. REPORT OF CASES Case l.-A healthy 78-year-old woman underwent an uneventful left intracapsular cataract extraction on Feb 2, 1975. Postoperatively, there was persistent inflammation and pain in the operated eye, which was unresponsive to topically applied corticosteroids. The development of scierai staphylomas occasioned the patient's referral to the University of
Florida. When first examined on Dec 4,1975, the visual acuity was correctable to 20/30 + 2 in the right eye and 20/60 -2 in the left eye. The right eye was normal in appearance. The bulbar conjunctiva of the left eye was hyperemic and chemotic. The sciera, above the horizontal meridian, was
Downloaded From: http://archopht.jamanetwork.com/ by a Michigan State University User on 06/18/2015
thinned and transparent, exposing the underlying bluish uveal tissue. In the 10 o'clock and 2 o'clock meridians, approximately 3 mm behind the limbus, were large scierai staphylomas covered by atrophie conjunctiva. The peripheral cornea in this region was guttered. There was a moderate anterior uveitis. The intraocular pressures were 15 mm Hg in the right eye and 30 mm Hg in the left. History and general physical examination failed to disclose any evidence of a generalized connective tissue disorder.
Laboratory investigations demonstrated a positive rheumatoid factor (1:160) and a negative antinuclear antibody. On Dec 5,1975, the sciera of the left eye
above the horizontal meridian was reinforced with an onlay graft of homologous banked sciera. The graft was sutured into normal cornea and sciera with interrupted sutures of 7-0 chromic catgut and extended from the insertions of the recti muscles posteriorly to the vertical edge of a 2-mm
wide
superficial keratectomy anteriorly.
The graft was covered with conjunctiva from the upper fornix. Postoperatively, the eye was pain-free. Dexamethasone drops, 0.1%, were used four times daily. On Jan 15, 1976, the patient returned to the University of Florida, relating a twoweek history of a painful, red, left eye. Results of the examination showed that the conjunctiva covering the scierai graft had become avascular and necrotic, and there was melting of the scierai graft itself (Fig 1). The peripheral cornea was guttered and anastomotie vessels had formed between the anterior ciliary sys-
1.—Left eye (patient 1) six weeks following scierai reinforcement with homologous banked sciera. Conjunctiva covering scierai graft is avascular and necrotic. There is melting of scierai
as in Fig 1 nine months following scierai reinforcement with autogenous periosteum. Staphylomatous areas are flat and well covered by vascular periosteum and
Fig
Fig 2.—Same eye
graft.
conjunctiva.
Fig 3.—Right eye (patient 2) prior to scierai reinforcement. There is large scierai staphyloma in upper nasal quadrant, covered by necrotic and atrophie conjunctiva and episclera.
Fig
months after scierai reinforcement with banked solera. Lifting of scierai graft is accompanied by dellen formation, corneal vascularization, and lipid deposi-
Fig
Fig 5—Right eye (patient 2) three tion.
4.—Left eye (patient 2) showing scierai staphyloma in upper nasal quadrant. Necrotic scierai nodule is present in the 12 o'clock meridian. Note peripheral guttering of cornea.
6.—Left eye (patient 2) nine months following scierai reinforcement with autogenous periosteum. Staphylomatous areas are flat and well covered by vascular periosteum and conjunctiva. Peripheral guttering of cornea has been halted.
Downloaded From: http://archopht.jamanetwork.com/ by a Michigan State University User on 06/18/2015
tern at the limbus and the vessels at the
origins of the recti muscles. There moderate anterior uveitis. The 10P
was a was
22
Hg. The following day,
mm
Jan 16, 1976, the weakened sciera of the patient's left eye was reinforced with an onlay graft of
autogenous periosteum.
The periosteum was obtained from the anterior tibial crest of the patient's left leg in the following manner. Under local infiltration, commencing 8 cm below the anterior tibial tubercle, a vertical skin incision was made and carried down to the bone. The muscles wore bluntly dissected off the periosteum. A 7 X 2-cm strip of" periosteum was incised with scalpel blade, lifted with periosteal elevator, and excised with scissors. A Penrose drain was placed in the wound, which was subsequently closed in two layers. One gram of cefazolin sodium was administered intravenously. Following lid akinesia and retrobulbar anesthesia, the necrotic scierai graft was removed from the superior aspect of the patient's left globe. Lamellar dissection was carried into normal sciera posteriorly and into clear cornea anteriorly as a 3-mm wide superficial keratectomy. The periosteal graft was sutured in place with interrupted sutures of 8-0 black silk and covered with conjunctiva from the upper fornix. Postoperatively, the patient was given 80 mg of prednisone and 1 gm cefazolin sodium daily. The eye became free of pain. The Penrose drain was mobilized on the second postoperative day and removed on the fourth. The antibiotics were discontinued on the third postoperative day. Dexamethasone drops, 0.1%, were used four times daily. When last examined, nine months' postoperatively, the left eye was quiet and pain-free. The staphylomatous areas were flat and well covered by periosteum and conjunctiva (Fig 2). The visual acuity was correctable to 20/70. Cask 2.-A 58-year-old woman with a ton-year history of severe, crippling rheumatoid arthritis (class IV, stage IV) was seen in referral at the University of Florida on Sept 9, 1975, for evaluation and treatment of severe bilateral scleromalacia
perforans. The patient
of her ocular condition. Her only complaints were of decreasing vision and mild photophobia. Her visual acuity was correctable to 20/ 40 in the right eye and 20/200 in the left eye. The bulbar conjunctiva of both eyes was diffusely injected and abnormal anastomotic vessels were present at the limbus bilaterally. In the upper nasal quadrant of the right eye was a largo scierai staphyloma covered by atrophie conjunctiva and was unaware
episclera (Fig 3).
A smaller scierai staphyvisible in the upper nasal quadrant of the left eye and was accompanied by a yellowish necrotic scierai loma
was
nodule in the 12 o'clock meridian, approximately 3 mm behind the limbus (Fig 4). The peripheral cornea of both eyes was guttered. A moderate anterior uveitis was present bilaterally, accompained by diffuse endothelial keratic precipitates and multiple posterior synechiae. There were bilateral posterior subcapsular cataracts. The IOP was 34 mm Hg in the right eye and 28 mm Hg in the left eye. Laboratory investigations showed a positive rheumatoid factor (1;10, 240) and a negative antinuelear antibody. On Sept 11, 1975, the large scierai defect in the patient's right eye was strengthened with an onlay graft of homologous-banked sciera. The postoperative course was uneventful. Dexamethasone drops, 0.1%, were used four times daily and methazolamide was taken for control of the elevated IOP. Management of the rheumatoid vasculitis was attempted with prednisone (10 mg) and cyclophosphamide (400
mg) daily.
During the subsequent three-month postoperative period, the anterior edge of the scierai graft began to lift. This was accompanied by the development of a peripheral corneal dellen. Vascularization of the base of the dellen was accompanied by lipid deposition midstromally (Fig 5). Progression of the cataracts during this time period reduced visual acuity to 20/200 in the right eye and 8/400 in the left. The patient underwent an uneventful left intracapsular cataract extraction on Dec 2, 1975. Three weeks' postoperatively the vision in the left eye was correctable to 20/60. On Jan 26, 1976, the patient came to the University of Florida, complaining of a painful left eye. The corrected visual acuity was now 20/100 in the right eye and counting fingers at two feet in the left eye. The findings in the right eye were essentially unchanged from the previous examinations. The left eye exhibited substantial progression of the scierai disease with enlargement of the scierai defects in the 10 o'clock and 12 o'clock meridians. The peripheral keratolysis had increased in extent. These
changes
accompanied by a moderate anterior uveitis, an updrawing of the pupil, and the formation of posterior synechiae between the iris and were
the vitreous face. The IOP
was
18
mm
Hg.
The following day, Jan 27, 1976, the weakened cornea and sciera of the patient's left eye was reinforced with an onlay graft of autogenous periosteum. The
Downloaded From: http://archopht.jamanetwork.com/ by a Michigan State University User on 06/18/2015
surgical technique
was similar to that described for case 1 with the exception that a Penrose drain was not placed in the leg wound at the time of closure. Postoperatively, the eye remained quiet and free of pain. Doxamethasone drops, 0.1%, were used four times daily. Rheumatoid vasculitis therapy included prednisono (10 mg) and penicillamine hydrochloride
(750 mg) daily.
Nine months following surgery the periosteal graft was well in place and covered by conjunctiva (Fig 6). The eye was quiet and pain-free. The peripheral keratolysis showed no signs of progression. Visual acuity remained at counting fingers because of the updrawn pupil.
COMMENT Periosteum is the specialized
con-
nective tissue surrounding bone. It consists of an outer dense layer of collagenous fibers and fibroblasts and an inner, looser layer of osteogenic cells.' In the human, transplanted periosteum does not exhibit its osteogenic potential. Periosteum is readily available, strong, and easy to work with. The fact that it is autogenous and vascular gives it distinct advantages over banked sciera. The scleritis seen in our patients would seem to be on an autoimmune basis and may, in fact, be tissuespecific. If this is the case, periosteum may have substantially less risk of being involved with recurrences of the necrotizing process. Cardona has used periosteum for the support of keratoprosthesis with good results.'1 Our experience with autogenous periosteum for scierai reinforcement would suggest that it is as good as homologous-banked sciera and may, in fact, be superior. This study was supported in part of Health fellowship.
Ministry
by an Ontario
References 1. Torchia RT, Dunn RE, Pease PJ: Fascia lata grafting in scleromalacia perforans with lamellar
corneal-scleral dissection. Am J
66:705, 1968.
2. Rosenthal
Ophthalmol
JW, Williams GT: Scleromalacia
perforans as a complication of rheumatoid arthritis. Am J Ophthalmol 54:862, 1962. 3. Watson PG, Hayreh SS: Scleritis and episcleritis. Br J Ophthalmol 60:163, 1976. 4. Peer LA: Transplantation of tissues: Carti-
lage, Bone, Fascia, Tendon and Muscle. New York, Williams & Wilkins Co, 1955, vol 1.
5. Cardona H: Prosthokeratoplasty in chemical burns and ocular pemphigoid. Can J Ophthalmol 11:35, 1976.
