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Problems Encountered in an Eye Clinic for Children*
PROBLEMS E N C O U N T E R E D IN AN EYE CLINIC FOR CHILDREN ALGERNON
B. REESE,
M.D.
New York There has evolved over the years a need thesia, which is ether, supplemented with for me to hold, at some specified time each various measures to make induction week, a clinic for the examination under gen smoother. This would require admission to eral anesthesia of infants and children hav the hospital and all that is attendant thereto, ing, or suspected of having, ocular pathol even if the supply of beds permitted it. ogy. At first this was necessary because of We find that the use of chloroform for an interest in the treatment of retinoblas- anesthesia makes our program possible. At toma which started over 20 years ago. Then a designated time each week an anesthetist the appearance of retrolental fibroplasia in is present to give the chloroform on an am creased the need. Now, I see at this clinic a bulatory basis. The method employed is great variety of lesions of infants or young reported in this issue by Dr. Herman 1 children which actually runs the gamut of Schwartz. We have used chloroform for ocular pathology encountered in this age this light primary anesthesia for 20 years with no untoward results. The patients have group. The patients are sent for examination a relatively smooth and quick induction. If usually for one of the following reasons: the patient becomes too light, additional (1) Opaque tissue or a light reflex had been chloroform can be given without the prepa noted in the pupillary area; (2) a tropia rations necessary when ether is used. The prompted an examination and some patho patients do not get obstruction from overlogic condition had been noted in the de secretion of mucus; vomiting is rare; they viating eye; (3) poor fixation and the ques awaken promptly; and they leave the hospital tion of defective vision and its cause arose;* in 20 to 30 minutes. The anesthetist keeps a (4) a disparity in the size of the two eyes stethoscope over the precordium and any slowing of the pulse is indication not to give had been detected;f and (5) pain (glau any more chloroform. The instillation of coma) or a congested eye had led to the pontocaine into the conjunctival sac before appreciation of some trouble. the use of forceps to facilitate the examina The clinic is conducted once a week when tion enables the anesthetist to keep the pa from six to 12 infants or children are ex tients lighter. The fact that the patients are amined under general anesthesia in a period kept light militates against aspiration. of from one to one and a half hours. This I can recommend this type of clinic, partic would not be possible were it necessary to ularly for centers where a number of ophthal give these patients the usual type of anesmologists work. It is difficult to determine ac curately the pathology present and its status * Some infants, and particularly those with sub normal vision, are slow in learning to fix their gaze from time to time in children's eyes without on objects. This may cause concern and prompt an general anesthesia. In the past I think we investigation. have been handicapped in the diagnosis and t The diseased eye may be the larger one due to glaucoma but more often the smaller one shows the follow-up of young children with serious eye pathologic process. Some degree of microphthalmos pathology because of an inability to have an may be viewed not only as a feature of congenital adequate examination except by admission to aberration but also of an acquired pre- or postnatal pathologic condition. It is a feature of retrolental the hospital. fibroplasia and is of a greater degree in the eye An idea of the scope of this clinic can be more severely affected. It may be a sequela of obtained from the following breakdown of intraocular inflammation.
EYE CLTNIC FOR CHILDREN the over 1,400 patients examined since the clinic started in 1947. Most of these patients have required multiple examinations. 1. R E T R O L E N T A L FIBROPLASIA
Premature infants u p to about the age of three months can be examined very well without anesthesia but not afterward. T h e infants are brought, usually, for the follow ing reasons: ( 1 ) T o determine if vision is present, and, if so, to estimate approxi mately how much; ( 2 ) to determine if glau coma is present; ( 3 ) to determine if a pre mature infant has suffered any degree of previously unrecognized cicatricial retrolental fibroplasia; ( 4 ) to assure parents that noth ing can be done to help the condition present. 2. RETINOBLASTOMA
Examination under general anesthesia is necessary to answer the following: ( 1 ) Is the lesion present a retinoblastoma, or some other form of leukokoria?* ( 2 ) W h a t treat ment is indicated, especially if the disease is bilateral? ( 3 ) H a s the treatment been effec tive, and is there evidence of recurrent growth? ( 4 ) Does the fellow eye show dis ease ? If the tumor is thought to be only uni lateral, the fellow eye is examined at threemonth intervals for a year in case subtle intraretinal lesions were not detected. Al though this tumor certainly is congenital, we find that one third of our bilateral cases had a tumor in the fellow eye overlooked at the time of enucleation. 3. CONGENITAL A N O M A L I E S OF VARIOUS SORTS
1. Malformation of the optic nerve, in cluding oblique insertion and inferior, su perior, or nasal conus. 2. Colobomatous lesions. This would in clude cases of aniridia where the intraocular pressure must be watched. * This term is used to refer to the group of condi tions in infants and children characterized by a white pupil and frequently called pseudoretinoblastoma.
25
3. Hydrophthalmos and follow-up after surgery, especially for tension readings. 4. Nystagmus, in an effort to rule out any intraocular pathologic process. 5. Persistent hyperplastic vitreous. 2 This lesion is seen generally for one of several reasons: first, it is more often confused with retinoblastoma than any one of the other leukokorias; second, cataract ensues and as sessment of the situation from this angle is sought; third, glaucoma and secondary corneal changes occur; and fourth, an esotropia is present. 6. Hyaloid artery. 7. Retinal cyst. 8. Pupillary membrane. 9. Cataract. As reported by Guy, 3 prema ture infants without retrolental fibroplasia may develop a cataract in each eye which has all the clinical appearance of the so-called congenital cataract but it appears several months after birth. 10. Extensive medullated nerve fibers which may give a reflex in the pupil and be confused with retinoblastoma. 11. Colloid bodies of the disc or retina which, if extensive, may be confused with retinoblastoma, or may be a feature of tu berous sclerosis. 12. Retinal dysplasia. 4 4. O P T I C ATROPHY
It is well know that sometimes the optic discs of infants look pale and if the babies show poor fixation the question as to whether or not an optic atrophy exists may arise. It is possible that the pale appearance of the disc is explained on the basis of late myelinization which we know normally is completed u p to the lamina cribrosa several months after birth. 5. A M A U R O T I C F A M I L Y IDIOCY ( T A Y - S A C H ' S DISEASE)
W h e n infants do not develop normally this question sometimes arises and the ap pearance of the cheery-red spot of the macula may be decisive.
26
ALGERNON B. REESE
6. A N G I O M A T O U S LESIONS
These are more often of the skin but when the lids are involved the lesion may also extend into the orbit. Twenty percent of these skin hemangiomas of infants, often re ferred to as strawberry angiomas, are pres ent at birth and 80 percent appear within the first five weeks after birth. They usually in crease in size for from six to eight months and then show, without treatment, a regres sion which is usually complete within five years. T h e incidence of these lesions in our premature nursery on the low oxygen regi men has shown a striking decrease. Also belonging to this group are the cases of Coats' disease, telangiectasis of the ret ina, Sturge-Weber disease, and angiomatosis retinae (von Hippel-Lindau). 7. M A S S I V E RETINAL FIBROSIS 5
W e know that retinal hemorrhages are common in the newborn at the time of birth. Sometimes such a hemorrhage in the retina is massive in nature just as it may be in the brain. As a result, in the eye, it leads to organization and the occurrence of a white mass of tissue which may have to be differ entiated from retinoblastoma. 8. V A R I O U S RARE TUMORS
Besides the retinoblastoma and hemangioma previously mentioned, under this heading would be, ( 1 ) Astrocytoma, or glioma, of the optic nerve which may be a feature of early neurofibromatosis; ( 2 ) neurofibromatosis involving the orbital tis sues (cafe-au-lait pigmentation of the skin may be the earliest manifestation of neuro fibromatosis) ; ( 3 ) neuroblastoma which more often is metastatic to the orbit from a manifest or occult tumor involving the su prarenal gland; ( 4 ) sarcomas from the eye adnexa and particularly rhabdomyosarcoma and embryonal rhabdomyoma; ( 5 ) leukemia, manifesting itself as an expanding lesion around the eye.
9.
INFLAMMATORY
OR
POSTINFLAMMATORY
CONDITIONS
These fall in the following groups: First, granulomatous uveitis is conceivably due to toxoplasmosis, tuberculosis, or any of the granulomatous diseases. Second, metastatic retinitis occurring at the time of any systemic infection of infancy and childhood. Characteristically, the lesion goes unapprehended at its inception during the active infection but is detected much later as a leukokoria when organization, contracture, and retinal detachment ensue. Third, nematode endophthalmitis which I have not been able to more than suspect. N o doubt all three of the above conditions may lead to the same end-stage. This is an atrophic globe with opaque tissue back of the lens which may be partially or totally cataractous, or the eye may be atrophic fol lowing a period of glaucoma which produced some buphthalmos, or the lesion may be seen in the stage of buphthalmos with non descript tissue back of a clear or partially opaque lens. Fourth, opaque corneas. These are bi lateral and present at birth. F u r t h e r char acteristics are ( a ) a round, central, dense corneal opacity; ( b ) the tonometer registers an elevated tension but to palpation the ten sion seems normal. These changes are thought to be the result of intrauterine cor neal ulceration or the manifestation of an intrauterine keratomalacea. 10.
UNCLASSIFIED
Sometimes secondary changes mask the primary pathology in the eye so that the nature of the basic lesion can not be deter mined. W e may see, therefore, one of the following two terminal pictures which defy further classification: First, clouding of the vitreous with or without a clouded lens and cornea. The ques tion may arise as to whether or not we are dealing primarily with a hemorrhagic, exu dative, or neoplastic process.
27
EYE CLINIC FOR CHILDREN Second, retinal detachment with acquired tissue over the surface of the retina or in the vitreous. The two groups above may be further complicated by glaucoma with or without buphthalmos or by atrophy of the globe. SUMMARY
The advantages of having available the
facilities for examining babies and children under general anesthesia on an ambulatory basis are discussed. F o r this, chloroform has proven to be a satisfactory anesthesia and the method employed is given in a report in this issue. A break-down of the types of cases examined in our clinic during the past eight years is given. 73 East 71st Street
(21).
REFERENCES
1. Schwartz, H.: Chloroform anesthesia for ophthalmic examination: a useful technique for ambula tory children. Am. J. Ophth., 43:27 (Tan.) 1957. 2. Reese, A. B.: Persistent hyperplastic vitreous. Tr. Am. Acad. Ophth., May-June, 1955, pp. 271-286. 3. Guy, L. P.: Postnatal cataracts in premature infants. Am. J. Ophth., 38:65-68 (July) 1954. 4. Reese, A. B.: Retinal dysplasia. Am. J. Ophth., 33:23-32 (Jan.) 1950. 5. : Massive retinal fibrosis in children. Am. J. Ophth., 19:576-582 (July) 1936.
CHLOROFORM ANESTHESIA FOR OPHTHALMIC
EXAMINATION*
A U S E F U L T E C H N I Q U E FOR AMBULATORY C H I L D R E N HERMAN
SCHWARTZ,
M.D.
New York Careful examination of the eyes of in fants and children under the age of three or four necessitates the use of general anesthe sia. It is the purpose of this paper to discuss the problems of general anesthesia for am bulatory children and to present the details (with some modifications) of a useful and safe technique using chloroform described originally by Martin and Reese in 1945. J Since 1947 over 3,200 ophthalmic examina tions have been performed at the Institute of Ophthalmology of the Columbia-Presby terian Medical Center with this method with out serious sequelae. There were no deaths or any postanesthetic liver, kidney, or res piratory complications. These excellent re sults do not imply that the potential hazards * From the Department of Anesthesiology, Col lege of Physicians and Surgeons, Columbia Uni versity, and the Anesthesiology Service, The Pres byterian Hospital in the City of New York.
of chloroform are unimportant. They exist, but can be eliminated or minimized by care ful attention to details which will be de scribed. Even the specialist in anesthesiology, if he is unfamiliar with chloroform, must approach this method cautiously to master its intricacies without danger to patients. T h e objectives of general anesthesia for these ophthalmic patients may be listed as follows : 1. Safe and efficient production of anes thesia. 2. Nonexplosive agents and methods. 3. Quick induction and fast recovery from anesthesia because of the ambulatory status of the patients. However, the recovery should be gradual enough after administra tion is discontinued to allow five to 15 minutes for examination of the eyes. 4. Avoidance of salivation and vomiting in these unpremedicated patients.
B. REESE,
M.D.
New York There has evolved over the years a need thesia, which is ether, supplemented with for me to hold, at some specified time each various measures to make induction week, a clinic for the examination under gen smoother. This would require admission to eral anesthesia of infants and children hav the hospital and all that is attendant thereto, ing, or suspected of having, ocular pathol even if the supply of beds permitted it. ogy. At first this was necessary because of We find that the use of chloroform for an interest in the treatment of retinoblas- anesthesia makes our program possible. At toma which started over 20 years ago. Then a designated time each week an anesthetist the appearance of retrolental fibroplasia in is present to give the chloroform on an am creased the need. Now, I see at this clinic a bulatory basis. The method employed is great variety of lesions of infants or young reported in this issue by Dr. Herman 1 children which actually runs the gamut of Schwartz. We have used chloroform for ocular pathology encountered in this age this light primary anesthesia for 20 years with no untoward results. The patients have group. The patients are sent for examination a relatively smooth and quick induction. If usually for one of the following reasons: the patient becomes too light, additional (1) Opaque tissue or a light reflex had been chloroform can be given without the prepa noted in the pupillary area; (2) a tropia rations necessary when ether is used. The prompted an examination and some patho patients do not get obstruction from overlogic condition had been noted in the de secretion of mucus; vomiting is rare; they viating eye; (3) poor fixation and the ques awaken promptly; and they leave the hospital tion of defective vision and its cause arose;* in 20 to 30 minutes. The anesthetist keeps a (4) a disparity in the size of the two eyes stethoscope over the precordium and any slowing of the pulse is indication not to give had been detected;f and (5) pain (glau any more chloroform. The instillation of coma) or a congested eye had led to the pontocaine into the conjunctival sac before appreciation of some trouble. the use of forceps to facilitate the examina The clinic is conducted once a week when tion enables the anesthetist to keep the pa from six to 12 infants or children are ex tients lighter. The fact that the patients are amined under general anesthesia in a period kept light militates against aspiration. of from one to one and a half hours. This I can recommend this type of clinic, partic would not be possible were it necessary to ularly for centers where a number of ophthal give these patients the usual type of anesmologists work. It is difficult to determine ac curately the pathology present and its status * Some infants, and particularly those with sub normal vision, are slow in learning to fix their gaze from time to time in children's eyes without on objects. This may cause concern and prompt an general anesthesia. In the past I think we investigation. have been handicapped in the diagnosis and t The diseased eye may be the larger one due to glaucoma but more often the smaller one shows the follow-up of young children with serious eye pathologic process. Some degree of microphthalmos pathology because of an inability to have an may be viewed not only as a feature of congenital adequate examination except by admission to aberration but also of an acquired pre- or postnatal pathologic condition. It is a feature of retrolental the hospital. fibroplasia and is of a greater degree in the eye An idea of the scope of this clinic can be more severely affected. It may be a sequela of obtained from the following breakdown of intraocular inflammation.
EYE CLTNIC FOR CHILDREN the over 1,400 patients examined since the clinic started in 1947. Most of these patients have required multiple examinations. 1. R E T R O L E N T A L FIBROPLASIA
Premature infants u p to about the age of three months can be examined very well without anesthesia but not afterward. T h e infants are brought, usually, for the follow ing reasons: ( 1 ) T o determine if vision is present, and, if so, to estimate approxi mately how much; ( 2 ) to determine if glau coma is present; ( 3 ) to determine if a pre mature infant has suffered any degree of previously unrecognized cicatricial retrolental fibroplasia; ( 4 ) to assure parents that noth ing can be done to help the condition present. 2. RETINOBLASTOMA
Examination under general anesthesia is necessary to answer the following: ( 1 ) Is the lesion present a retinoblastoma, or some other form of leukokoria?* ( 2 ) W h a t treat ment is indicated, especially if the disease is bilateral? ( 3 ) H a s the treatment been effec tive, and is there evidence of recurrent growth? ( 4 ) Does the fellow eye show dis ease ? If the tumor is thought to be only uni lateral, the fellow eye is examined at threemonth intervals for a year in case subtle intraretinal lesions were not detected. Al though this tumor certainly is congenital, we find that one third of our bilateral cases had a tumor in the fellow eye overlooked at the time of enucleation. 3. CONGENITAL A N O M A L I E S OF VARIOUS SORTS
1. Malformation of the optic nerve, in cluding oblique insertion and inferior, su perior, or nasal conus. 2. Colobomatous lesions. This would in clude cases of aniridia where the intraocular pressure must be watched. * This term is used to refer to the group of condi tions in infants and children characterized by a white pupil and frequently called pseudoretinoblastoma.
25
3. Hydrophthalmos and follow-up after surgery, especially for tension readings. 4. Nystagmus, in an effort to rule out any intraocular pathologic process. 5. Persistent hyperplastic vitreous. 2 This lesion is seen generally for one of several reasons: first, it is more often confused with retinoblastoma than any one of the other leukokorias; second, cataract ensues and as sessment of the situation from this angle is sought; third, glaucoma and secondary corneal changes occur; and fourth, an esotropia is present. 6. Hyaloid artery. 7. Retinal cyst. 8. Pupillary membrane. 9. Cataract. As reported by Guy, 3 prema ture infants without retrolental fibroplasia may develop a cataract in each eye which has all the clinical appearance of the so-called congenital cataract but it appears several months after birth. 10. Extensive medullated nerve fibers which may give a reflex in the pupil and be confused with retinoblastoma. 11. Colloid bodies of the disc or retina which, if extensive, may be confused with retinoblastoma, or may be a feature of tu berous sclerosis. 12. Retinal dysplasia. 4 4. O P T I C ATROPHY
It is well know that sometimes the optic discs of infants look pale and if the babies show poor fixation the question as to whether or not an optic atrophy exists may arise. It is possible that the pale appearance of the disc is explained on the basis of late myelinization which we know normally is completed u p to the lamina cribrosa several months after birth. 5. A M A U R O T I C F A M I L Y IDIOCY ( T A Y - S A C H ' S DISEASE)
W h e n infants do not develop normally this question sometimes arises and the ap pearance of the cheery-red spot of the macula may be decisive.
26
ALGERNON B. REESE
6. A N G I O M A T O U S LESIONS
These are more often of the skin but when the lids are involved the lesion may also extend into the orbit. Twenty percent of these skin hemangiomas of infants, often re ferred to as strawberry angiomas, are pres ent at birth and 80 percent appear within the first five weeks after birth. They usually in crease in size for from six to eight months and then show, without treatment, a regres sion which is usually complete within five years. T h e incidence of these lesions in our premature nursery on the low oxygen regi men has shown a striking decrease. Also belonging to this group are the cases of Coats' disease, telangiectasis of the ret ina, Sturge-Weber disease, and angiomatosis retinae (von Hippel-Lindau). 7. M A S S I V E RETINAL FIBROSIS 5
W e know that retinal hemorrhages are common in the newborn at the time of birth. Sometimes such a hemorrhage in the retina is massive in nature just as it may be in the brain. As a result, in the eye, it leads to organization and the occurrence of a white mass of tissue which may have to be differ entiated from retinoblastoma. 8. V A R I O U S RARE TUMORS
Besides the retinoblastoma and hemangioma previously mentioned, under this heading would be, ( 1 ) Astrocytoma, or glioma, of the optic nerve which may be a feature of early neurofibromatosis; ( 2 ) neurofibromatosis involving the orbital tis sues (cafe-au-lait pigmentation of the skin may be the earliest manifestation of neuro fibromatosis) ; ( 3 ) neuroblastoma which more often is metastatic to the orbit from a manifest or occult tumor involving the su prarenal gland; ( 4 ) sarcomas from the eye adnexa and particularly rhabdomyosarcoma and embryonal rhabdomyoma; ( 5 ) leukemia, manifesting itself as an expanding lesion around the eye.
9.
INFLAMMATORY
OR
POSTINFLAMMATORY
CONDITIONS
These fall in the following groups: First, granulomatous uveitis is conceivably due to toxoplasmosis, tuberculosis, or any of the granulomatous diseases. Second, metastatic retinitis occurring at the time of any systemic infection of infancy and childhood. Characteristically, the lesion goes unapprehended at its inception during the active infection but is detected much later as a leukokoria when organization, contracture, and retinal detachment ensue. Third, nematode endophthalmitis which I have not been able to more than suspect. N o doubt all three of the above conditions may lead to the same end-stage. This is an atrophic globe with opaque tissue back of the lens which may be partially or totally cataractous, or the eye may be atrophic fol lowing a period of glaucoma which produced some buphthalmos, or the lesion may be seen in the stage of buphthalmos with non descript tissue back of a clear or partially opaque lens. Fourth, opaque corneas. These are bi lateral and present at birth. F u r t h e r char acteristics are ( a ) a round, central, dense corneal opacity; ( b ) the tonometer registers an elevated tension but to palpation the ten sion seems normal. These changes are thought to be the result of intrauterine cor neal ulceration or the manifestation of an intrauterine keratomalacea. 10.
UNCLASSIFIED
Sometimes secondary changes mask the primary pathology in the eye so that the nature of the basic lesion can not be deter mined. W e may see, therefore, one of the following two terminal pictures which defy further classification: First, clouding of the vitreous with or without a clouded lens and cornea. The ques tion may arise as to whether or not we are dealing primarily with a hemorrhagic, exu dative, or neoplastic process.
27
EYE CLINIC FOR CHILDREN Second, retinal detachment with acquired tissue over the surface of the retina or in the vitreous. The two groups above may be further complicated by glaucoma with or without buphthalmos or by atrophy of the globe. SUMMARY
The advantages of having available the
facilities for examining babies and children under general anesthesia on an ambulatory basis are discussed. F o r this, chloroform has proven to be a satisfactory anesthesia and the method employed is given in a report in this issue. A break-down of the types of cases examined in our clinic during the past eight years is given. 73 East 71st Street
(21).
REFERENCES
1. Schwartz, H.: Chloroform anesthesia for ophthalmic examination: a useful technique for ambula tory children. Am. J. Ophth., 43:27 (Tan.) 1957. 2. Reese, A. B.: Persistent hyperplastic vitreous. Tr. Am. Acad. Ophth., May-June, 1955, pp. 271-286. 3. Guy, L. P.: Postnatal cataracts in premature infants. Am. J. Ophth., 38:65-68 (July) 1954. 4. Reese, A. B.: Retinal dysplasia. Am. J. Ophth., 33:23-32 (Jan.) 1950. 5. : Massive retinal fibrosis in children. Am. J. Ophth., 19:576-582 (July) 1936.
CHLOROFORM ANESTHESIA FOR OPHTHALMIC
EXAMINATION*
A U S E F U L T E C H N I Q U E FOR AMBULATORY C H I L D R E N HERMAN
SCHWARTZ,
M.D.
New York Careful examination of the eyes of in fants and children under the age of three or four necessitates the use of general anesthe sia. It is the purpose of this paper to discuss the problems of general anesthesia for am bulatory children and to present the details (with some modifications) of a useful and safe technique using chloroform described originally by Martin and Reese in 1945. J Since 1947 over 3,200 ophthalmic examina tions have been performed at the Institute of Ophthalmology of the Columbia-Presby terian Medical Center with this method with out serious sequelae. There were no deaths or any postanesthetic liver, kidney, or res piratory complications. These excellent re sults do not imply that the potential hazards * From the Department of Anesthesiology, Col lege of Physicians and Surgeons, Columbia Uni versity, and the Anesthesiology Service, The Pres byterian Hospital in the City of New York.
of chloroform are unimportant. They exist, but can be eliminated or minimized by care ful attention to details which will be de scribed. Even the specialist in anesthesiology, if he is unfamiliar with chloroform, must approach this method cautiously to master its intricacies without danger to patients. T h e objectives of general anesthesia for these ophthalmic patients may be listed as follows : 1. Safe and efficient production of anes thesia. 2. Nonexplosive agents and methods. 3. Quick induction and fast recovery from anesthesia because of the ambulatory status of the patients. However, the recovery should be gradual enough after administra tion is discontinued to allow five to 15 minutes for examination of the eyes. 4. Avoidance of salivation and vomiting in these unpremedicated patients.