Ophthalmologica, Basel 172: 457 465 (1976)
Retinoblastoma in Bloch-Sulzberger Syndrome J ohn B lake and J oan M ui.laney Eye and Ear Hospital, and National Ophthalmic Pathology Laboratory, Dublin
Primary dermal incontinentia pigmenti may be associated with three eponymous syndromes - Bloch-Sulzberger, Naegeli and Ito types. Ocular changes occur in the Bloch-Sulzberger group but are sometimes found in incontinentia pigmenti without the other anomalies. The ophthalmological manifestations have recently been summarized by M ensheha-M anhart et al. [1975] who reported an enucleated eye with retinal detachment and unusual nodular or placoid proliferations of the retinal pigment epithelium in a case of simple dermal incontinentia pigmenti. Ocular histopathological studies of enucleated globes in classical Bloch-Sulzberger syndrome appear to be very rare and to have been performed only by Scott et al. [1955], K rummel and R ausch, [1955] and Z weifach [1966] (table I). Two other globes studied microscopically in the literature were from cases with only dermal pigmentary changes and without malformations. The present paper describes a typical case of Bloch-Sulzberger syn drome with retinoblastoma. This would appear to be the first report of such an association. Case History
Downloaded by: Univ. of California Santa Barbara 128.111.121.42 - 3/5/2018 9:49:25 PM
A 43-year-old mother brought her 8th child, a 3-week-old baby, to the skin clinic because she had noted a rash on the baby's chin at birth, and on uncovering the baby 2 days later, she had seen a rash on the skin of the legs, especially on the front of the knees, under the armpits, and on the vertex of the head. The skin lesions were considered to be the early stages of the Bloch-Sulzberger syndrome, a view which was confirmed as the lesions subsided leaving a linear pigmentation under the left axilla. A haemangioma was noted on the right buttock (fig. I).
458
Blake/M ullaney
Table 1 Bloch-Sulzberger cases with histological ocular reports
Uveal pigmentary disturbances
Retinal changes
K rummel and R ausch
dark brown plaques of pigment epithelium of pars ciliaris retinae. Desquamation of pig ment to subretinal fluid
retinal dysplasia and PHPV
Scott et al. | 1955]
many detached RPE cells, ghost cells
funnel-shaped detachment
Z weifach (1966]
numerous retinal pig ment cells in subretinal fluid and in choroid
dysplastic including intraretinal pigment deposition
Fig. 1. Haemangioma right buttock. Downloaded by: Univ. of California Santa Barbara 128.111.121.42 - 3/5/2018 9:49:25 PM
[1955]
Retinoblastoma in Bloch-Sulzberger Syndrome
. r •
Downloaded by: Univ. of California Santa Barbara 128.111.121.42 - 3/5/2018 9:49:25 PM
Fig. 3. Delayed eruption of teeth and ‘screw driver’ shape of central incisors.
459
460
Blake/M ui.lanuy
At 6 weeks of age, the baby suffered convulsions with projectile vomiting. She was admitted to hospital under the care of the paediatric consultant who examined the cerebrospinal fluid and found it to be normal. Blood examination revealed a white cell count of 16,100 mm* with a differential count as follows: neutrophils 12%, lymphocytes 70%, monocytes 4% and eosinophils 8%. At the age of 5 months, the dermatologist referred her to the eye clinic be cause of a discharge from both eyes (due to a mild infective conjunctivitis) and for opinion on whether ocular features known to be associated with the BlochSulzberger syndrome were present. The fundi could not be examined adequately in the outpatient department and so arrangements were made to admit the child for examination under anaesthesia at the age of 1 year. Inferonasal to the left disc was a well-circumscribed pale grey swelling, about 21/* disc diameters across, which appeared to be cystic and slightly translucent.
Downloaded by: Univ. of California Santa Barbara 128.111.121.42 - 3/5/2018 9:49:25 PM
Fig. 4. Appearance of right eye shortly before enucleation.
Retinoblastoma in Bloch-Sulzberger Syndrome
461
Apart from a mild leucopoenia, blood count was normal as were X-rays of optic foramina and skull. The eyes were examined again after an interval of 2 months and the fundus lesion was seen to have increased in size and was again noted to be cystic. 3 months later, the child came under the care of one of us (J.B.). On examina tion under anaesthesia, a large slightly craggy white mass was seen to occupy the inferonasal quadrant of the right eye partly covering the optic disc and ex tending from 1.30 to 6.30 o’clock. Near the upper margin of the white mass, superonasal to the disc, were two holes through which a red reflex could be seen; between the edges of these holes and the underlying reflex a marked parallax was evident. Numerous irregular and rounded snowball-like white opacities were visible in the vitreous especially near the surface of the mass. An intensely white lesion resembling hard exudate extended from the disc to the macula. Examination
Downloaded by: Univ. of California Santa Barbara 128.111.121.42 - 3/5/2018 9:49:25 PM
Fig. 5. Right eye filled by tumour containing calcified foci.
462
Blake/M ullaney
of the left eye revealed a rounded white mass roughly IV2 disc in diameter near the periphery of the retina at 6 o’clock. There were numerous small blood vessels on the surface of the mass and the retinal veins in the vicinity were dilated. On general inspection, the hair on the head was noted to be thin and there was a localized atrophic alopecia of the scalp (fig. 2). A dental opinion was sought which confirmed extremely delayed eruption of the deciduous teeth, the two central incisors having a ‘screw driver’ shape (fig. 3). Paediatric assessment including electro cardiography disclosed no abnormality, while X-ray of skull, optic foramina and chest were again reported as normal. A biopsy was taken of the pigmented area of the skin in the left axilla, and microscopy showed the following features: (1) a tendency to hyperkeratosis, the keratin being loose and fragmented; (2) collections of large pigment-containing phagocytes in the superficial corium usually in relation to the small blood vessels around which were also congregated groups of chronic inflammatory cells; (3) the pigment could be seen arising from the pigmented cells of the basal-cell layer and in scattered areas these cells seemed to be disintegrating and projecting their pigment downwards into the corium. The findings were reported to be consistent with a diagnosis of incontinentia pigmenti in a chronic phase. Subsequently, the intraocular masses increased rapidly in size (fig. 3, 4) and enucleation of the right eye was performed.
Downloaded by: Univ. of California Santa Barbara 128.111.121.42 - 3/5/2018 9:49:25 PM
Fig. 6. Necrosis and DNA deposition. HE x 100.
Retinoblastoma in Bloch-Sulzberger Syndrome
463
Pal ho logy R eporl Right eye measured 26 x 25 x 24 mm with a corneal diameter of 12 mm and a temporal equatorial staphyloma. A massive intraocular tumour with central spotty calcification replaced the interior of the globe (fig. 5). Microscopically there were secondary inflammatory corneal changes. The an terior chamber was obliterated, the cataractous lens lying in contact with the cornea. The ocular cavity was filled by a malignant tumour composed of darkly staining round cells, with massive central necrosis, calcification and typical DNA vascular encrustation (fig. 6). The pattern was uniform and no rosettes were found. There was infiltration through the choroid, the sclera and the optic nerve, with extension to the level of resection. The tumour was an undifferentiated retinoblastoma. The child died 3 months later. In the opinion of the pathologist who carried out the autopsy, death was due to cardiorespiratory failure secondary to retino blastoma with multiple organ métastasés. The left eye then showed evidence of post-irradiation endophthalmitis, cataract and amorphous calcified areas suggesting necrotic retinoblastoma.
This syndrome is associated ophthalmologically with various changes particularly retinal disturbances of dysplastic and detachment types. How ever, pigmentary changes of the retinal pigment epithelium have also been reported, some of these being of considerable degree with occasional vascular anomalies |G arrod, 1906; Sulzberger, 1938; C arney, 1951; F indlay, 1952; Scott el al., 1955; J ensen, 1965; C ole and C ole , 1959; J ones, 1966; M iller and A nderson , 1966; Z weii ach, 1966; M cC rary and Smith , 1968; F ischbein el al., 1972; W atzke et al., 1974], It is generally agreed that the Bloch-Sulzberger syndrome occurring predominantly in females is a familial disease, genetically dominant with variable penetrance. A maternal viral infection has been inculpated. The congenital aspect, the manifestation of a bullous rash and the occasional report of metastatic ophthalmia, could correlate with a viral component. Z weifach [1966) has described retinal dysplasia in incontinentia pigmenti. He observed his patient for 6 months and enucleated the globe on suspicion of retinoblastoma because of the degree of progression of the retinal thickening. In fact, histological examination revealed dys plastic alterations. Dysplasia can be produced experimentally in the fetal Iamb by live bluetongue virus vaccine of sheep [Silverstein et al., 1971], Retinoblastoma which may be familial can be transmitted as a domi nant autosome with incomplete penetrance and the case for a viral aetiol
Downloaded by: Univ. of California Santa Barbara 128.111.121.42 - 3/5/2018 9:49:25 PM
Comment
Blake/M ullaney
464
ogy has been made by Z immerman [1970]. Retinoblastoma-like lesions can be produced experimentally in rats by human adenovirus type 12 [M ukai et al., 1974] and it is also of some interest that 3% of pregnant women are said to be deficient in antibody against that adenovirus [Y abe , 1964]. The third facet of the pathological ocular changes in incontinentia pigmenti is the pigmentary disturbance. This alteration has been particu larly well illustrated by the case of M ensheha -M anhari et al. [1975] in which there were diffuse and localized nodules of pigment on the retinal epithelium. The authors state that 'The retinal dysplasia and de tachment could be secondary to the underlying abnormalities in the RPE which in turn may reasonably be considered an expression of the underlying disorder of pigmentation.’ They did not mention the possi bility of a viral clement for the retinal changes. G ass 's [1968] paper describing acute posterior multifocal placoid pigment epitheliopathy sug gests an ‘infectious’ agent causing this pigment epitheliopathy, a condition as yet unexplained. We would like to postulate a viral aetiology in the causation of the primary incontinentia pigmenti group of conditions. This could account for the wide spectrum of the associated ocular changes which has been described in the literature. The severity of the eye alterations in the Bloch-Sulzberger syndrome may represent a potentiated viral effect su perimposed on the genetic weakness existing in these patients. If this thesis is tenable and if a virus can be related to the development of retino blastoma, then a basis exists for the association of the two conditions in one patient as reported in this paper.
A cknowledgements We wish to thank Miss P atricia M urphy for secretarial assistance, Mr. J. M ulfor photographic help, and Mr. R. Lester, FIMI.T. for technical aid.
vanny
C arney, R. G.: Incontinentia pigmenti. Archs Derm. 64: 126 (1951). C ole, 1. G. and C ole, H. G.: Incontinentia pigmenti. Am. J. Ophthal. 47: 321 (1959). F indlay, G. H.: On the pathogenesis of incontinentia pigmenti. Br. J. Derm. 64: 141 (1952).
Downloaded by: Univ. of California Santa Barbara 128.111.121.42 - 3/5/2018 9:49:25 PM
References
Retinoblastoma in Bloch-Sulzberger Syndrome
465
F ischbf.in , F. I.; Schub, M„ and L esko, W. S.: Incontinentia pigmenti, pheochromo-
cytoma, and ocular abnormalities. Am. J. Ophthal. 73: 961 (1972). G arrod, A. E.: Peculiar pigmentation of the skin. Trans, clin. Soc. Lond. 39: 216
(1906). G ass, J. D.:
Acute posterior multifocal placoid pigment epitheliopathy. Archs Ophthal., N Y. 80: 177 (1968). J ensen , V. A.: Incontinentia pigmenti (Bloch-Sulzberger) associated with proliferative eyeground changes and positive toxoplasmosis reaction. Acta psychiatr. scand. 31: suppl. 108 p. 197 (1956). J ones, S. T.: Retrolental membrane associated with Bloch-Sulzberger syndrome (in continentia pigmenti). Am. J. Ophthal. 62: 330 (1966). M ensheha-M anhart, O.: Rodrigues , M.; Shields , J.; S hannon, G.. and M irabelli, R.: Retinal pigment epithelium in incontinentia pigmenti. Am. J. Ophthal. 79: 571 (1975). M iller, R. J. and Anderson, R. E.: A retrolental mass in incontinentia pigmenti. Surv. Ophthal. II: 41 (1966). M ukai, N.; Kobayashi, A. and O guri, M.: Ultrastructural studies of human adeno virus-produced retinoblastoma-like neoplasms in Sprague-Dawley rats. Investve Ophthal. 138: 593 (1974). McC rary, J. A. Ill and Smith, J. L.: Conjunctival and retinal incontinentia pigmenti. Archs Ophthal. 79: 417 (1968). Scott , J. G.; F riedman , A. I.; C hitters , M„ and P epler , W. J.: Ocular changes in the Bloch-Sulzberger syndrome (incontinentia pigmenti). Its association with retinal dysplasia. Am. J. Ophthal. 62: 716 (1966). Stt.vERSTEiN, A. M.; P arshall, C. J., jr.; O svurn, B. I., and P rendergast, R. A.: An experimental virus-induced retinal dysplasia in the fetal lamb. Am. J. Ophthal. 72: 22 (1971). S ulzberger, M. B.: Incontinentia pigmenti (Bloch-Sulzberger). Arch. Derm. Syph. 38: 57 (1938). K rummel, H. von and R ausch, L.: Ophthalmologica, Basel 130: 31 (1955). W atzke, R.C.; Stevens , T. S., and C arney, R.G., jr.: Retinal vascular hypoplasia and telangiectasia associated with incontinentia pigmenti. Read before the Ass. for Res. in Vision and Ophthalmology Meet., Sarasota 1974. Yabf., Y.: Antibody to adenovirus types 12 among infants. Lancet i: 1447 (1964). Z immerman, L. E.: Pathogenesis of infectious diseases. Am. J. Ophthal. 69: 947 (1970). Z weifach , P. H.: Incontinentia pigmenti. Its association with retinal dysplasia. Am. J. Ophthal. 62: 716 (1966).
Downloaded by: Univ. of California Santa Barbara 128.111.121.42 - 3/5/2018 9:49:25 PM
Dr. J. Blake, Eye and Ear Hospital, Dublin 2 (Ireland)
Retinoblastoma in Bloch-Sulzberger Syndrome J ohn B lake and J oan M ui.laney Eye and Ear Hospital, and National Ophthalmic Pathology Laboratory, Dublin
Primary dermal incontinentia pigmenti may be associated with three eponymous syndromes - Bloch-Sulzberger, Naegeli and Ito types. Ocular changes occur in the Bloch-Sulzberger group but are sometimes found in incontinentia pigmenti without the other anomalies. The ophthalmological manifestations have recently been summarized by M ensheha-M anhart et al. [1975] who reported an enucleated eye with retinal detachment and unusual nodular or placoid proliferations of the retinal pigment epithelium in a case of simple dermal incontinentia pigmenti. Ocular histopathological studies of enucleated globes in classical Bloch-Sulzberger syndrome appear to be very rare and to have been performed only by Scott et al. [1955], K rummel and R ausch, [1955] and Z weifach [1966] (table I). Two other globes studied microscopically in the literature were from cases with only dermal pigmentary changes and without malformations. The present paper describes a typical case of Bloch-Sulzberger syn drome with retinoblastoma. This would appear to be the first report of such an association. Case History
Downloaded by: Univ. of California Santa Barbara 128.111.121.42 - 3/5/2018 9:49:25 PM
A 43-year-old mother brought her 8th child, a 3-week-old baby, to the skin clinic because she had noted a rash on the baby's chin at birth, and on uncovering the baby 2 days later, she had seen a rash on the skin of the legs, especially on the front of the knees, under the armpits, and on the vertex of the head. The skin lesions were considered to be the early stages of the Bloch-Sulzberger syndrome, a view which was confirmed as the lesions subsided leaving a linear pigmentation under the left axilla. A haemangioma was noted on the right buttock (fig. I).
458
Blake/M ullaney
Table 1 Bloch-Sulzberger cases with histological ocular reports
Uveal pigmentary disturbances
Retinal changes
K rummel and R ausch
dark brown plaques of pigment epithelium of pars ciliaris retinae. Desquamation of pig ment to subretinal fluid
retinal dysplasia and PHPV
Scott et al. | 1955]
many detached RPE cells, ghost cells
funnel-shaped detachment
Z weifach (1966]
numerous retinal pig ment cells in subretinal fluid and in choroid
dysplastic including intraretinal pigment deposition
Fig. 1. Haemangioma right buttock. Downloaded by: Univ. of California Santa Barbara 128.111.121.42 - 3/5/2018 9:49:25 PM
[1955]
Retinoblastoma in Bloch-Sulzberger Syndrome
. r •
Downloaded by: Univ. of California Santa Barbara 128.111.121.42 - 3/5/2018 9:49:25 PM
Fig. 3. Delayed eruption of teeth and ‘screw driver’ shape of central incisors.
459
460
Blake/M ui.lanuy
At 6 weeks of age, the baby suffered convulsions with projectile vomiting. She was admitted to hospital under the care of the paediatric consultant who examined the cerebrospinal fluid and found it to be normal. Blood examination revealed a white cell count of 16,100 mm* with a differential count as follows: neutrophils 12%, lymphocytes 70%, monocytes 4% and eosinophils 8%. At the age of 5 months, the dermatologist referred her to the eye clinic be cause of a discharge from both eyes (due to a mild infective conjunctivitis) and for opinion on whether ocular features known to be associated with the BlochSulzberger syndrome were present. The fundi could not be examined adequately in the outpatient department and so arrangements were made to admit the child for examination under anaesthesia at the age of 1 year. Inferonasal to the left disc was a well-circumscribed pale grey swelling, about 21/* disc diameters across, which appeared to be cystic and slightly translucent.
Downloaded by: Univ. of California Santa Barbara 128.111.121.42 - 3/5/2018 9:49:25 PM
Fig. 4. Appearance of right eye shortly before enucleation.
Retinoblastoma in Bloch-Sulzberger Syndrome
461
Apart from a mild leucopoenia, blood count was normal as were X-rays of optic foramina and skull. The eyes were examined again after an interval of 2 months and the fundus lesion was seen to have increased in size and was again noted to be cystic. 3 months later, the child came under the care of one of us (J.B.). On examina tion under anaesthesia, a large slightly craggy white mass was seen to occupy the inferonasal quadrant of the right eye partly covering the optic disc and ex tending from 1.30 to 6.30 o’clock. Near the upper margin of the white mass, superonasal to the disc, were two holes through which a red reflex could be seen; between the edges of these holes and the underlying reflex a marked parallax was evident. Numerous irregular and rounded snowball-like white opacities were visible in the vitreous especially near the surface of the mass. An intensely white lesion resembling hard exudate extended from the disc to the macula. Examination
Downloaded by: Univ. of California Santa Barbara 128.111.121.42 - 3/5/2018 9:49:25 PM
Fig. 5. Right eye filled by tumour containing calcified foci.
462
Blake/M ullaney
of the left eye revealed a rounded white mass roughly IV2 disc in diameter near the periphery of the retina at 6 o’clock. There were numerous small blood vessels on the surface of the mass and the retinal veins in the vicinity were dilated. On general inspection, the hair on the head was noted to be thin and there was a localized atrophic alopecia of the scalp (fig. 2). A dental opinion was sought which confirmed extremely delayed eruption of the deciduous teeth, the two central incisors having a ‘screw driver’ shape (fig. 3). Paediatric assessment including electro cardiography disclosed no abnormality, while X-ray of skull, optic foramina and chest were again reported as normal. A biopsy was taken of the pigmented area of the skin in the left axilla, and microscopy showed the following features: (1) a tendency to hyperkeratosis, the keratin being loose and fragmented; (2) collections of large pigment-containing phagocytes in the superficial corium usually in relation to the small blood vessels around which were also congregated groups of chronic inflammatory cells; (3) the pigment could be seen arising from the pigmented cells of the basal-cell layer and in scattered areas these cells seemed to be disintegrating and projecting their pigment downwards into the corium. The findings were reported to be consistent with a diagnosis of incontinentia pigmenti in a chronic phase. Subsequently, the intraocular masses increased rapidly in size (fig. 3, 4) and enucleation of the right eye was performed.
Downloaded by: Univ. of California Santa Barbara 128.111.121.42 - 3/5/2018 9:49:25 PM
Fig. 6. Necrosis and DNA deposition. HE x 100.
Retinoblastoma in Bloch-Sulzberger Syndrome
463
Pal ho logy R eporl Right eye measured 26 x 25 x 24 mm with a corneal diameter of 12 mm and a temporal equatorial staphyloma. A massive intraocular tumour with central spotty calcification replaced the interior of the globe (fig. 5). Microscopically there were secondary inflammatory corneal changes. The an terior chamber was obliterated, the cataractous lens lying in contact with the cornea. The ocular cavity was filled by a malignant tumour composed of darkly staining round cells, with massive central necrosis, calcification and typical DNA vascular encrustation (fig. 6). The pattern was uniform and no rosettes were found. There was infiltration through the choroid, the sclera and the optic nerve, with extension to the level of resection. The tumour was an undifferentiated retinoblastoma. The child died 3 months later. In the opinion of the pathologist who carried out the autopsy, death was due to cardiorespiratory failure secondary to retino blastoma with multiple organ métastasés. The left eye then showed evidence of post-irradiation endophthalmitis, cataract and amorphous calcified areas suggesting necrotic retinoblastoma.
This syndrome is associated ophthalmologically with various changes particularly retinal disturbances of dysplastic and detachment types. How ever, pigmentary changes of the retinal pigment epithelium have also been reported, some of these being of considerable degree with occasional vascular anomalies |G arrod, 1906; Sulzberger, 1938; C arney, 1951; F indlay, 1952; Scott el al., 1955; J ensen, 1965; C ole and C ole , 1959; J ones, 1966; M iller and A nderson , 1966; Z weii ach, 1966; M cC rary and Smith , 1968; F ischbein el al., 1972; W atzke et al., 1974], It is generally agreed that the Bloch-Sulzberger syndrome occurring predominantly in females is a familial disease, genetically dominant with variable penetrance. A maternal viral infection has been inculpated. The congenital aspect, the manifestation of a bullous rash and the occasional report of metastatic ophthalmia, could correlate with a viral component. Z weifach [1966) has described retinal dysplasia in incontinentia pigmenti. He observed his patient for 6 months and enucleated the globe on suspicion of retinoblastoma because of the degree of progression of the retinal thickening. In fact, histological examination revealed dys plastic alterations. Dysplasia can be produced experimentally in the fetal Iamb by live bluetongue virus vaccine of sheep [Silverstein et al., 1971], Retinoblastoma which may be familial can be transmitted as a domi nant autosome with incomplete penetrance and the case for a viral aetiol
Downloaded by: Univ. of California Santa Barbara 128.111.121.42 - 3/5/2018 9:49:25 PM
Comment
Blake/M ullaney
464
ogy has been made by Z immerman [1970]. Retinoblastoma-like lesions can be produced experimentally in rats by human adenovirus type 12 [M ukai et al., 1974] and it is also of some interest that 3% of pregnant women are said to be deficient in antibody against that adenovirus [Y abe , 1964]. The third facet of the pathological ocular changes in incontinentia pigmenti is the pigmentary disturbance. This alteration has been particu larly well illustrated by the case of M ensheha -M anhari et al. [1975] in which there were diffuse and localized nodules of pigment on the retinal epithelium. The authors state that 'The retinal dysplasia and de tachment could be secondary to the underlying abnormalities in the RPE which in turn may reasonably be considered an expression of the underlying disorder of pigmentation.’ They did not mention the possi bility of a viral clement for the retinal changes. G ass 's [1968] paper describing acute posterior multifocal placoid pigment epitheliopathy sug gests an ‘infectious’ agent causing this pigment epitheliopathy, a condition as yet unexplained. We would like to postulate a viral aetiology in the causation of the primary incontinentia pigmenti group of conditions. This could account for the wide spectrum of the associated ocular changes which has been described in the literature. The severity of the eye alterations in the Bloch-Sulzberger syndrome may represent a potentiated viral effect su perimposed on the genetic weakness existing in these patients. If this thesis is tenable and if a virus can be related to the development of retino blastoma, then a basis exists for the association of the two conditions in one patient as reported in this paper.
A cknowledgements We wish to thank Miss P atricia M urphy for secretarial assistance, Mr. J. M ulfor photographic help, and Mr. R. Lester, FIMI.T. for technical aid.
vanny
C arney, R. G.: Incontinentia pigmenti. Archs Derm. 64: 126 (1951). C ole, 1. G. and C ole, H. G.: Incontinentia pigmenti. Am. J. Ophthal. 47: 321 (1959). F indlay, G. H.: On the pathogenesis of incontinentia pigmenti. Br. J. Derm. 64: 141 (1952).
Downloaded by: Univ. of California Santa Barbara 128.111.121.42 - 3/5/2018 9:49:25 PM
References
Retinoblastoma in Bloch-Sulzberger Syndrome
465
F ischbf.in , F. I.; Schub, M„ and L esko, W. S.: Incontinentia pigmenti, pheochromo-
cytoma, and ocular abnormalities. Am. J. Ophthal. 73: 961 (1972). G arrod, A. E.: Peculiar pigmentation of the skin. Trans, clin. Soc. Lond. 39: 216
(1906). G ass, J. D.:
Acute posterior multifocal placoid pigment epitheliopathy. Archs Ophthal., N Y. 80: 177 (1968). J ensen , V. A.: Incontinentia pigmenti (Bloch-Sulzberger) associated with proliferative eyeground changes and positive toxoplasmosis reaction. Acta psychiatr. scand. 31: suppl. 108 p. 197 (1956). J ones, S. T.: Retrolental membrane associated with Bloch-Sulzberger syndrome (in continentia pigmenti). Am. J. Ophthal. 62: 330 (1966). M ensheha-M anhart, O.: Rodrigues , M.; Shields , J.; S hannon, G.. and M irabelli, R.: Retinal pigment epithelium in incontinentia pigmenti. Am. J. Ophthal. 79: 571 (1975). M iller, R. J. and Anderson, R. E.: A retrolental mass in incontinentia pigmenti. Surv. Ophthal. II: 41 (1966). M ukai, N.; Kobayashi, A. and O guri, M.: Ultrastructural studies of human adeno virus-produced retinoblastoma-like neoplasms in Sprague-Dawley rats. Investve Ophthal. 138: 593 (1974). McC rary, J. A. Ill and Smith, J. L.: Conjunctival and retinal incontinentia pigmenti. Archs Ophthal. 79: 417 (1968). Scott , J. G.; F riedman , A. I.; C hitters , M„ and P epler , W. J.: Ocular changes in the Bloch-Sulzberger syndrome (incontinentia pigmenti). Its association with retinal dysplasia. Am. J. Ophthal. 62: 716 (1966). Stt.vERSTEiN, A. M.; P arshall, C. J., jr.; O svurn, B. I., and P rendergast, R. A.: An experimental virus-induced retinal dysplasia in the fetal lamb. Am. J. Ophthal. 72: 22 (1971). S ulzberger, M. B.: Incontinentia pigmenti (Bloch-Sulzberger). Arch. Derm. Syph. 38: 57 (1938). K rummel, H. von and R ausch, L.: Ophthalmologica, Basel 130: 31 (1955). W atzke, R.C.; Stevens , T. S., and C arney, R.G., jr.: Retinal vascular hypoplasia and telangiectasia associated with incontinentia pigmenti. Read before the Ass. for Res. in Vision and Ophthalmology Meet., Sarasota 1974. Yabf., Y.: Antibody to adenovirus types 12 among infants. Lancet i: 1447 (1964). Z immerman, L. E.: Pathogenesis of infectious diseases. Am. J. Ophthal. 69: 947 (1970). Z weifach , P. H.: Incontinentia pigmenti. Its association with retinal dysplasia. Am. J. Ophthal. 62: 716 (1966).
Downloaded by: Univ. of California Santa Barbara 128.111.121.42 - 3/5/2018 9:49:25 PM
Dr. J. Blake, Eye and Ear Hospital, Dublin 2 (Ireland)
