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Total Unilateral Visual Loss Following Orbital Surgery
TOTAL
UNILATERAL VISUAL LOSS FOLLOWING ORBITAL SURGERY
JOHN C . LONG, M . D , AND P H I L I P P . ELLIS, M . D .
Denver, Colorado Complete irreversible visual loss as a possible complication of orbital surgery is not appreciated generally except when there has been direct trauma to the optic nerve. The constriction
of
numerous
and
important
structures into a tight bony cage makes surgery of the orbit difficult. It is not surprising, therefore, to have occasional undesirable complications of such surgery. Each of the authors has
twice experienced the
grave
complication of total and permanent loss of vision in the eye of the involved orbit. Concern over this complication prompted a survey of 33 ophthalmic centers during the past four years to learn if others had experienced this problem. This report includes several case histories from other centers in addition to those of the authors. There is scant mention of this complication in the ophthalmic literature. Berke
1
re-
cords one such incident ; it is designated here as case 8. He also cites injury to the optic nerve in two cases during the removal of a tumor from the apex of the orbit, and partial optic atrophy following another such operation.
2
Long and
Ellis
3
have recorded two
such complications; they are described here as cases 1 and 2. CASE REPORTS
Case 1—A 52-year-old Caucasian man was seen (by J. C. L.) because of severe bilateral exophthalmos with Chemosis 18 months after the onset of Graves' disease. Treatment had consisted of propylthiouracil followed by radioactive iodine. Vision was good but there was mild bilateral papilledema. A left lateral orbital decompression by the BerkeKrönlein technique was done by a colleague. An excessive amount of bleeding occurred and the orbital From the Division of Ophthalmology, University of Colorado Medical Center, Denver, Colorado. Reprint requests to Philip P. Ellis, M.D.. Division of Ophthalmology, University of Colorado Medical Center, 4200 East Ninth Avenue, Denver, Colorado 80220.
contents were manipulated more than usually. A rubber drain was left in the temporal wound and a moderate pressure dressing applied with adhesive tape. Severe swelling and ecchymosis of the orbit followed. When the intermarginal eyelid sutures were cut and the eyelids opened on the sixth postoperative day, the patient was unable to see light with the eye. The retinal vessels were patent but the macular area seemed unusually red. Severe edema of both eyes was present and persisted for many weeks. As the papilledema gradually subsided, pallor of the left disk became apparent. The patient died eight months after the orbital surgery of acute hepatic atrophy of undetermined cause. There had been no visual recovery. Case 2—A-60-year-old Caucasian housewife was referred (to J. C. L.) when left unilateral exophthalmos and x-ray findings suggestive of retrobulbar tumor. The patient had been treated for diabetes mellitus for 10 years and was hypertensive. A cerebral vascular accident two years before had left her with a right homonymous hemianopsia. A medical consultant found thyroid function to be normal. A lateral exposure of the orbit was made by the Berke-Krönlein approach. As no tumor became obvious, the muscle cone was opened below the lateral rectus by blunt dissection and was palpated with negative results. A second incision was made medial to the globe through the conjunctiva so that a finger could be inserted behind the globe to palpate against a finger placed in the temporal wound. No tumor was found. The wounds were closed after the temporal bony orbital wall was removed to effect a decompression. A rubber drain was left in the temporal wound and a moderate pressure dressing applied by adhesive tape. There was only moderate bleeding during the surgery, but severe edema of the orbit followed. When the intermarginal eyelid sutures were removed on the seventh postoperative day, there was no light perception in the left eye and there was no direct pupillary response to light. Because of the edema of the eyelid and conjunctiva, the fundus was difficult to observe, but it did not appear remarkable. Visual loss persisted and pallor of the disk became apparent in approximately one month. Proptosis of the right eye developed a year later and studies by an endocrinologist established a thyroid basis for the exophthalmos. Case 3—-An 18-year-old girl was seen (by P. P. E.) because of a slowly progressive exophthalmos of the right eye of eight years duration. Discomfort had been noted only during the preceding month. Visual acuity was RE 20/20, LE 20/15. The visual fields were full. Exophthalmometer readings showed a relative right proptosis of 6 mm. No mass was palpable in the orbit. The right orbit was ex-
218
VOL. 71, NO. 1
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VISUAL LOSS A F T E R SURGERY
plored by the Berke-Krönlein technique and a large hemiangioma was found. This tumor extended from the lateral posterior surface of the globe back into the muscle cone for a distance of 2.5 cm. The tumor was carefully freed by blunt dissection, and the entire mass was removed. There was only a small amount of bleeding during the procedure. A pressure dressing was applied. The surgery was followed by slight eyelid edema which cleared within a week. At this time the patient complained that she could not see with the eye and it was determined that she could not perceive light. The fundus showed a mild papilledema which cleared within a month. Two months after the surgery the disk was white and there was no light perception. Case 4—A 42-year-old Caucasian woman was seen (by P. P. E.) with a history of hyperthyroidism followed by progressive exophthalmos during the preceding seven months. Treatment had consisted of propylthiouracil and 30 to 40 mg of prednisone daily. The hyperthyroidism was adequately controlled, but the exophthalmos increased in spite of therapy. There was definite limitation of motility and some exposure keratitis. Bilateral temporal decompressions of the orbits were done by the BerkeKrönlein technique. The periorbital tissue was thickened. Several radial incisions were made in the periorbita, but the orbital fat did not prolapse well. Moderate bleeding was encountered on the right side. A light pressure dressing was applied with adhesive tape bilaterally. This dressing was removed on the fourth postoperative day, revealing that the exophthalmos had improved considerably and that the intermarginal eyelid sutures were lax. There was a moderate amount of Chemosis of the right bulbar conjunctiva interiorly, but definitely less eyelid edema than preoperatively. The vision of the right eye was reduced to questionable light perception; the vision in the left eye remained normal. Both fundi seemed quite normal. Ten weeks postoperatively there was marked improvement in exophthalmos and motility of the eyes, but the right eye had a pale disk and no light perception. Case 5—A 25-year-old woman was seen by a colleague because of exophthalmos and inability to wear contact lenses. She gave a history of hyperthyroidism treated by propylthiouracil and later by thyroidectomy. A bilateral temporal decompression by the Berke-Krönlein technique combined with a lateral antral decompression was done. The procedure was attended by a marked amount of bleeding. Pressure dressings were applied for 24 hours but no head roll was utilized. Both eyes were closed by edema. On the third postoperative day the patient complained of visual loss in one eye. There was no light perception, the pupil was dilated, and the disk margin was blurred. A diagnosis of vascular occlusion was made. Treatment consisting of ACTH, methylprednisolone, nicotinic acid, multiple vitamins, and acetazolamide, was not effective and optic atrophy ensued. Vision in the opposite eye remained normal. Case 6—A 35-year-old woman was seen by Dr.
Daniel Silva of Mexico City, who diagnosed a meningioma of the orbital apex. Permission was granted for an exploratory orbitotomy only. A lateral orbitotomy of the Krönlein type was done allowing digital palpation of the tumor mass and the removal of a small bit of tumor for biopsy purposes. Visual loss was noted two days later at the first postoperative dressing. Dr. Silva writes, "Ophthalmoscopically, I found a retrobulbar obstruction of the central retinal artery which did not yield to medical treatment. This obstruction was attributed to the digital exploratory maneuvers. Permission to remove the tumor was not granted after this unfortunate incident" Case 7—An 11-year-old girl with exophthalmos was also seen by Dr. Silva, who made a provisional diagnosis of pseudotumor. Of this case, Dr. Silva writes, "A Krönlein operation exposed a diffuse temporal mass occupying all of the upper and outer orbital space and being so within reach that deep digital exploration was not necessary. A bit of tissue was removed for biopsy from the anterior portion near the lacrimal gland and the wound provisionally closed pending the laboratory diagnosis. When first dressed on the third postoperative day, there was intense Chemosis and a large retrobulbar hematoma filled the orbital tissues. Debridement of the periorbita was done and Hyaluronidase was injected to facilitate diffusion and reabsorption of the hemorrhage. The hemorrhage had disappeared by the tenth postoperative day. At that time it was found there was complete loss of vision which was attributed to obstructive alterations of both artery and vein due to the pressure caused by the hematoma." Case 8—In a case previously described by Dr. Raynold Berke,* a 39-year-old man with congenital syphilis and 13 mm of exophthalmos was explored by lateral orbitotomy. Dr. Berke states, "Operation disclosed an extensive retrobulbar hemangioma surrounding the lateral side of the optic nerve and extending from the apex of the orbit to the region of the lacrimal gland." Occulusion of the central retinal artery followed the surgery. "It was thought that the occlusion in this case occurred because of the extensive manipulation necessary to remove the tumor," Dr. Berke reported. DISCUSSION
It is quite evident that total loss of vision following orbitotomy is a rare occurance. Kroll and Casten have recorded 32 lateral decompressions without this complication, although one case had lost light perception before surgery and went on to complete optic atrophy. Moran performed 55 decompression operations without serious incident. Long and Ellis described visual loss under 4
5
3
AMERICAN JOURNAL OF OPHTHALMOLOGY
220
somewhat unusual circumstances in two of 67 patients undergoing lateral decompressions (cases 1 and 2 ) . Silva has indicated that visual loss occurred in two eyes in a series of over 300 orbitotomies, approximately onehalf of which were done by the Krönlein technique (cases 5 and 6 ) . Hogan and Beard both said they had not had this complication in over 60 orbitotomies. The exact mechanism of the visual loss is not fully understood, but it is assumed that it is the result of interference with the blood supply. Occlusion of the central retinal artery is suspected in some cases, but in others, obstruction may be in the nutrient blood supply of the optic nerve. None of the patients has been examined immediately following the surgery and some of them only after five or six days. In some instances, evaluation of the fundus has been confused by pre-existing papilledema. By excluding cases in which the blood vessels might have been actually injured by the excision of a growth, we theorize that the underlying cause of the visual loss may be high orbital pressure either from edema, hemorrhage, or bandaging. Excessive bleeding or severe postoperative edema were encountered in several of the cases. These experiences demonstrate that while risk is inherent to any major surgery of this type, certain precautions should be taken. Obviously, the surgery should be performed with as little trauma as possible, especially during palpation of the orbit, blunt dissection, and retraction of the orbital contents. Retraction should be interrupted frequently to permit good orbital circulation. Sharp dissection within the orbit should be kept at a minmum and done only under direct clear observation. If the surgery is limited to a temporal decompression, the orbit should be disturbed as little as possible. Good hemostasis is important, although sometimes it is very difficult to attain. Diathermy coagulation of some of the bleeding points may be of value. Some form of drainage, placed deep in the orbital wound, may prevent the formation of a large 8
7
8
JANUARY, 1971
hematoma. Initially, drains were removed after 24 hours, but they are now left in position for 48 hours. Continuous suction drainage, such as provided by Hemovac and similar devices, would seem almost ideal, although we have not yet had any personal experience with them. Pressure dressings, if applied, should be mild. This would exclude the dressings rolled around the head. The administration of substantial doses of systemic corticosteroids during the first few postoperative days would seem advisable in the hope of diminishing orbital swelling. Despite these precautions, however, a rare individual may develop sudden blindness. SUMMARY
Eight cases of total unilateral visual loss after orbital surgery are presented. Four patients suffered from dysthyroid exophthalmos, two had orbital hemangiomas, one had an orbital meningioma, and the other patient had an orbital pseudotumor. It is postulated that the visual loss may have resulted from interference with the blood supply of the optic nerve or retina. Possible causes include undue surgical manipulation, excessive postoperative edema or hemorrhage, and tight bandaging. Nutrient vessels to the optic nerve may have been damaged during surgical removal of vascular tumors adjacent to the nerve. REFERENCES
1. Berke, R. N. : A modified Krönlein operation. Tr. Am. Ophth. Soc. 51:193, 1953. 2. Berke, R. N. : Management of complications of orbital surgery. In Fasanella, R. M. (ed. ) : Complications in Eye Surgery, 2nd ed. Philadelphia, Saunders, 1965, p. 382. 3. Long, J. C , and Ellis, G. D. : Temporal decompression of the orbit for thyroid exophthalmos. Am. J. Ophth. 62:1089, 1966. 4. Kroll, A. J, and Casten, V. G. : Dysthyroid exophthalmos. Palliation by lateral orbital decompression. Arch. Ophth. 76:205, 1966. 5. Moran, R. E. : The correction of exophthalmos and levator spasm. Plast. Reconstr. Surg. 18 : 411, 1956. 6. Silva, D. : Personal communication. 7. Hogan, M. : Personal communication. 8. Beard, C. : Personal communication.
UNILATERAL VISUAL LOSS FOLLOWING ORBITAL SURGERY
JOHN C . LONG, M . D , AND P H I L I P P . ELLIS, M . D .
Denver, Colorado Complete irreversible visual loss as a possible complication of orbital surgery is not appreciated generally except when there has been direct trauma to the optic nerve. The constriction
of
numerous
and
important
structures into a tight bony cage makes surgery of the orbit difficult. It is not surprising, therefore, to have occasional undesirable complications of such surgery. Each of the authors has
twice experienced the
grave
complication of total and permanent loss of vision in the eye of the involved orbit. Concern over this complication prompted a survey of 33 ophthalmic centers during the past four years to learn if others had experienced this problem. This report includes several case histories from other centers in addition to those of the authors. There is scant mention of this complication in the ophthalmic literature. Berke
1
re-
cords one such incident ; it is designated here as case 8. He also cites injury to the optic nerve in two cases during the removal of a tumor from the apex of the orbit, and partial optic atrophy following another such operation.
2
Long and
Ellis
3
have recorded two
such complications; they are described here as cases 1 and 2. CASE REPORTS
Case 1—A 52-year-old Caucasian man was seen (by J. C. L.) because of severe bilateral exophthalmos with Chemosis 18 months after the onset of Graves' disease. Treatment had consisted of propylthiouracil followed by radioactive iodine. Vision was good but there was mild bilateral papilledema. A left lateral orbital decompression by the BerkeKrönlein technique was done by a colleague. An excessive amount of bleeding occurred and the orbital From the Division of Ophthalmology, University of Colorado Medical Center, Denver, Colorado. Reprint requests to Philip P. Ellis, M.D.. Division of Ophthalmology, University of Colorado Medical Center, 4200 East Ninth Avenue, Denver, Colorado 80220.
contents were manipulated more than usually. A rubber drain was left in the temporal wound and a moderate pressure dressing applied with adhesive tape. Severe swelling and ecchymosis of the orbit followed. When the intermarginal eyelid sutures were cut and the eyelids opened on the sixth postoperative day, the patient was unable to see light with the eye. The retinal vessels were patent but the macular area seemed unusually red. Severe edema of both eyes was present and persisted for many weeks. As the papilledema gradually subsided, pallor of the left disk became apparent. The patient died eight months after the orbital surgery of acute hepatic atrophy of undetermined cause. There had been no visual recovery. Case 2—A-60-year-old Caucasian housewife was referred (to J. C. L.) when left unilateral exophthalmos and x-ray findings suggestive of retrobulbar tumor. The patient had been treated for diabetes mellitus for 10 years and was hypertensive. A cerebral vascular accident two years before had left her with a right homonymous hemianopsia. A medical consultant found thyroid function to be normal. A lateral exposure of the orbit was made by the Berke-Krönlein approach. As no tumor became obvious, the muscle cone was opened below the lateral rectus by blunt dissection and was palpated with negative results. A second incision was made medial to the globe through the conjunctiva so that a finger could be inserted behind the globe to palpate against a finger placed in the temporal wound. No tumor was found. The wounds were closed after the temporal bony orbital wall was removed to effect a decompression. A rubber drain was left in the temporal wound and a moderate pressure dressing applied by adhesive tape. There was only moderate bleeding during the surgery, but severe edema of the orbit followed. When the intermarginal eyelid sutures were removed on the seventh postoperative day, there was no light perception in the left eye and there was no direct pupillary response to light. Because of the edema of the eyelid and conjunctiva, the fundus was difficult to observe, but it did not appear remarkable. Visual loss persisted and pallor of the disk became apparent in approximately one month. Proptosis of the right eye developed a year later and studies by an endocrinologist established a thyroid basis for the exophthalmos. Case 3—-An 18-year-old girl was seen (by P. P. E.) because of a slowly progressive exophthalmos of the right eye of eight years duration. Discomfort had been noted only during the preceding month. Visual acuity was RE 20/20, LE 20/15. The visual fields were full. Exophthalmometer readings showed a relative right proptosis of 6 mm. No mass was palpable in the orbit. The right orbit was ex-
218
VOL. 71, NO. 1
219
VISUAL LOSS A F T E R SURGERY
plored by the Berke-Krönlein technique and a large hemiangioma was found. This tumor extended from the lateral posterior surface of the globe back into the muscle cone for a distance of 2.5 cm. The tumor was carefully freed by blunt dissection, and the entire mass was removed. There was only a small amount of bleeding during the procedure. A pressure dressing was applied. The surgery was followed by slight eyelid edema which cleared within a week. At this time the patient complained that she could not see with the eye and it was determined that she could not perceive light. The fundus showed a mild papilledema which cleared within a month. Two months after the surgery the disk was white and there was no light perception. Case 4—A 42-year-old Caucasian woman was seen (by P. P. E.) with a history of hyperthyroidism followed by progressive exophthalmos during the preceding seven months. Treatment had consisted of propylthiouracil and 30 to 40 mg of prednisone daily. The hyperthyroidism was adequately controlled, but the exophthalmos increased in spite of therapy. There was definite limitation of motility and some exposure keratitis. Bilateral temporal decompressions of the orbits were done by the BerkeKrönlein technique. The periorbital tissue was thickened. Several radial incisions were made in the periorbita, but the orbital fat did not prolapse well. Moderate bleeding was encountered on the right side. A light pressure dressing was applied with adhesive tape bilaterally. This dressing was removed on the fourth postoperative day, revealing that the exophthalmos had improved considerably and that the intermarginal eyelid sutures were lax. There was a moderate amount of Chemosis of the right bulbar conjunctiva interiorly, but definitely less eyelid edema than preoperatively. The vision of the right eye was reduced to questionable light perception; the vision in the left eye remained normal. Both fundi seemed quite normal. Ten weeks postoperatively there was marked improvement in exophthalmos and motility of the eyes, but the right eye had a pale disk and no light perception. Case 5—A 25-year-old woman was seen by a colleague because of exophthalmos and inability to wear contact lenses. She gave a history of hyperthyroidism treated by propylthiouracil and later by thyroidectomy. A bilateral temporal decompression by the Berke-Krönlein technique combined with a lateral antral decompression was done. The procedure was attended by a marked amount of bleeding. Pressure dressings were applied for 24 hours but no head roll was utilized. Both eyes were closed by edema. On the third postoperative day the patient complained of visual loss in one eye. There was no light perception, the pupil was dilated, and the disk margin was blurred. A diagnosis of vascular occlusion was made. Treatment consisting of ACTH, methylprednisolone, nicotinic acid, multiple vitamins, and acetazolamide, was not effective and optic atrophy ensued. Vision in the opposite eye remained normal. Case 6—A 35-year-old woman was seen by Dr.
Daniel Silva of Mexico City, who diagnosed a meningioma of the orbital apex. Permission was granted for an exploratory orbitotomy only. A lateral orbitotomy of the Krönlein type was done allowing digital palpation of the tumor mass and the removal of a small bit of tumor for biopsy purposes. Visual loss was noted two days later at the first postoperative dressing. Dr. Silva writes, "Ophthalmoscopically, I found a retrobulbar obstruction of the central retinal artery which did not yield to medical treatment. This obstruction was attributed to the digital exploratory maneuvers. Permission to remove the tumor was not granted after this unfortunate incident" Case 7—An 11-year-old girl with exophthalmos was also seen by Dr. Silva, who made a provisional diagnosis of pseudotumor. Of this case, Dr. Silva writes, "A Krönlein operation exposed a diffuse temporal mass occupying all of the upper and outer orbital space and being so within reach that deep digital exploration was not necessary. A bit of tissue was removed for biopsy from the anterior portion near the lacrimal gland and the wound provisionally closed pending the laboratory diagnosis. When first dressed on the third postoperative day, there was intense Chemosis and a large retrobulbar hematoma filled the orbital tissues. Debridement of the periorbita was done and Hyaluronidase was injected to facilitate diffusion and reabsorption of the hemorrhage. The hemorrhage had disappeared by the tenth postoperative day. At that time it was found there was complete loss of vision which was attributed to obstructive alterations of both artery and vein due to the pressure caused by the hematoma." Case 8—In a case previously described by Dr. Raynold Berke,* a 39-year-old man with congenital syphilis and 13 mm of exophthalmos was explored by lateral orbitotomy. Dr. Berke states, "Operation disclosed an extensive retrobulbar hemangioma surrounding the lateral side of the optic nerve and extending from the apex of the orbit to the region of the lacrimal gland." Occulusion of the central retinal artery followed the surgery. "It was thought that the occlusion in this case occurred because of the extensive manipulation necessary to remove the tumor," Dr. Berke reported. DISCUSSION
It is quite evident that total loss of vision following orbitotomy is a rare occurance. Kroll and Casten have recorded 32 lateral decompressions without this complication, although one case had lost light perception before surgery and went on to complete optic atrophy. Moran performed 55 decompression operations without serious incident. Long and Ellis described visual loss under 4
5
3
AMERICAN JOURNAL OF OPHTHALMOLOGY
220
somewhat unusual circumstances in two of 67 patients undergoing lateral decompressions (cases 1 and 2 ) . Silva has indicated that visual loss occurred in two eyes in a series of over 300 orbitotomies, approximately onehalf of which were done by the Krönlein technique (cases 5 and 6 ) . Hogan and Beard both said they had not had this complication in over 60 orbitotomies. The exact mechanism of the visual loss is not fully understood, but it is assumed that it is the result of interference with the blood supply. Occlusion of the central retinal artery is suspected in some cases, but in others, obstruction may be in the nutrient blood supply of the optic nerve. None of the patients has been examined immediately following the surgery and some of them only after five or six days. In some instances, evaluation of the fundus has been confused by pre-existing papilledema. By excluding cases in which the blood vessels might have been actually injured by the excision of a growth, we theorize that the underlying cause of the visual loss may be high orbital pressure either from edema, hemorrhage, or bandaging. Excessive bleeding or severe postoperative edema were encountered in several of the cases. These experiences demonstrate that while risk is inherent to any major surgery of this type, certain precautions should be taken. Obviously, the surgery should be performed with as little trauma as possible, especially during palpation of the orbit, blunt dissection, and retraction of the orbital contents. Retraction should be interrupted frequently to permit good orbital circulation. Sharp dissection within the orbit should be kept at a minmum and done only under direct clear observation. If the surgery is limited to a temporal decompression, the orbit should be disturbed as little as possible. Good hemostasis is important, although sometimes it is very difficult to attain. Diathermy coagulation of some of the bleeding points may be of value. Some form of drainage, placed deep in the orbital wound, may prevent the formation of a large 8
7
8
JANUARY, 1971
hematoma. Initially, drains were removed after 24 hours, but they are now left in position for 48 hours. Continuous suction drainage, such as provided by Hemovac and similar devices, would seem almost ideal, although we have not yet had any personal experience with them. Pressure dressings, if applied, should be mild. This would exclude the dressings rolled around the head. The administration of substantial doses of systemic corticosteroids during the first few postoperative days would seem advisable in the hope of diminishing orbital swelling. Despite these precautions, however, a rare individual may develop sudden blindness. SUMMARY
Eight cases of total unilateral visual loss after orbital surgery are presented. Four patients suffered from dysthyroid exophthalmos, two had orbital hemangiomas, one had an orbital meningioma, and the other patient had an orbital pseudotumor. It is postulated that the visual loss may have resulted from interference with the blood supply of the optic nerve or retina. Possible causes include undue surgical manipulation, excessive postoperative edema or hemorrhage, and tight bandaging. Nutrient vessels to the optic nerve may have been damaged during surgical removal of vascular tumors adjacent to the nerve. REFERENCES
1. Berke, R. N. : A modified Krönlein operation. Tr. Am. Ophth. Soc. 51:193, 1953. 2. Berke, R. N. : Management of complications of orbital surgery. In Fasanella, R. M. (ed. ) : Complications in Eye Surgery, 2nd ed. Philadelphia, Saunders, 1965, p. 382. 3. Long, J. C , and Ellis, G. D. : Temporal decompression of the orbit for thyroid exophthalmos. Am. J. Ophth. 62:1089, 1966. 4. Kroll, A. J, and Casten, V. G. : Dysthyroid exophthalmos. Palliation by lateral orbital decompression. Arch. Ophth. 76:205, 1966. 5. Moran, R. E. : The correction of exophthalmos and levator spasm. Plast. Reconstr. Surg. 18 : 411, 1956. 6. Silva, D. : Personal communication. 7. Hogan, M. : Personal communication. 8. Beard, C. : Personal communication.