of the With Lens Involvement
Congenital Syphilis
Eye
Francisco Contreras, MD, Jose Pereda, MD
examination of a girl congenital syphilis revealed a focal granulomatous reaction involving the anterior uvea and lens. Krajian's (silver) stain exhibited spiral forms that were morphologically compatible with Trepo\s=b\ Pathological
with
pallidum within the lens cortex. These findings have been rarely reported in early congenital lues.
nema
(Arch Ophthalmol 96:1052-1053, 1978)
anterior pole transilluminated poorly. The eye was sectioned horizontally. The anterior chamber was partially filled with a white material that occu¬ pied the pupillary space and was adherent to the anterior pole of the lens and the central part of the iris. The iris-lens diaphragm was displaced anteriorly. Retinal folds were present. The sclera and optic nerve-head were normal.
ocular lesions in
histological Thecongenital syphilis
are
not
com¬
monly mentioned in the current litera¬
present a case of an unusual iridolenticular granuloma in a syphilitic newborn. ture.13 We
REPORT OF A CASE A 2.32-kg girl was born on Oct 24,1971 at the Maternity Hospital in Lima, Peru, after 35 weeks' gestation. She had an opaque, gray mass in the left pupil, and erythematous maculae on the entire body, particularly on the face. Twenty-four hours after her birth, the girl had notable jaun¬ dice of the skin and mucosa. Results of serological tests given to the child's mother were positive (VDRL test for syphilis, 3 + and quantitative Kahn's test, dilution, 1:32). The girl died three days after birth. The main autopsy findings were: hepatosplenomegaly, fibrosis of the pancreas, and
pulmonary hemorrhage. OCULAR PATHOLOGICAL FINDINGS Macroscopic Examination
The left measured globe 17 x 17 X 16 mm, with 3 mm of optic nerve attached to the sclera. The cornea measured 9 x 8.5 mm. The Accepted for publication Oct 15, 1977. From the Ophthalmic Pathology Laboratory, Department of Ophthalmology, Hospital Santo Toribio de Mogrovejo, Lima, Peru (Dr Contreras), and the Department of Pathology, Maternity Hospital, Lima. Reprint requests to Hospital Santo Toribio de Mogrovejo, Ophthalmic Pathology Laboratory, Ancas 1271,
Lima, Peru (Dr Contreras).
Light Microscopic
Examination
Bowman's membrane was discon¬ tinuous in some sectors, and chronic inflammatory infiltrates in the ante¬ rior layers of the central corneal stroma were seen. The anterior cham¬ ber was narrow and filled with fibrin¬ ous exúdate and inflammatory cells. The iris exhibited peripheral ante¬ rior synechiae to the trabecular mesh¬ work (Fig 1) and posterior central synechiae to the lens (Fig 2). In the latter area, a dense, inflammatory exúdate that covered the pupil and involved the anterior pole of the lens was observed. The lens capsule was ruptured. There were numerous poly-
COMMENT The most frequent clinical manifes¬ tations of early congenital syphilis include "salt and pepper" chorioretini¬ tis.4-5 Less frequent involvement of the cornea, iris, and ciliary body are reported." Duke-Elder quotes several publications from the middle of the 19th century that call attention to the acute iridocyclitis as a clinical mani¬ festation of congenital syphilis, in¬ cluding the presence of exudative membranes that occlude the pupil. In this century, Igersheimer* pointed out the iritis of the newborn as a sign of congenital syphilis. This iritis usually is mild, with little inflammatory reaction, and may be overlooked by the clinician. Iger¬ sheimer mentions two clinicopathological cases of patients similar to ours. These patients were 4- and 6-
nuclears, macrophages, lymphocytes, and plasma cells surrounding a necrot¬ ic focus. Krajian's stain showed a large number of spiral forms within the lens cortex that were morphologi¬ cally compatible with Treponema pallidum (Fig 3).
The iris stroma showed vascular
congestion and chronic inflammatory cell infiltration (Fig 4). The ciliary body showed inflammatory infiltra¬ tion, hemorrhage, and splitting of the nonpigmented epithelial layer at the pars plana. The vitreous base exhib¬ ited moderate inflammatory infiltra¬ tion and hemorrhage. In the choroid, the inflammatory response was less intensive. The retina presented some folds, vascular congestions, and local¬ ized hemorrhages.
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Fig 1 .—Peripheral anterior synechia of iris. Chronic inflammatory exúdate in anterior and posterior chamber (hematoxylin-
eosin, x40)
Fig 2.—Focal necrotic inflammatory reac¬ tion occluding pupil with lens cortex involvement. Central posterior synechia of Iris (hematoxylin-eosin 4). month-old syphilitic gray-yellow pupillary
Fig
be
3—Numerous seen
spirochete-like forms (Krajian's stain, 400).
in lens cortex. Small
portion of lens capsule can
infants masses,
with
narrow
anterior chambers, and ocular hyper¬ tension. In one of these eyes, Iger¬ sheimer performed an Elliott filtering procedure, followed some months later by phthisis bulbi. In both cases, the eyes were enucleated. On pathological examination, the eyes had marked chronic inflammatory pupillary mem¬ branes with iris stroma involvement. In the eye on which operation had previously been performed the lens capsule was ruptured, and the cortex showed inflammatory infiltration. Friedenwald,- in routine postmor¬ tem examination of 175 newborn or stillborn infants, found 30 cases of congenital syphilis. In nine of these 30 infants, ocular inflammatory lesions were found. In five of these nine cases, the anterior uvea was involved. Interestingly, Friedenwald found spirochetes in only two cases, along peri¬ vascular sheathing of the penetrating vessels of the sclera at the ciliary body level. The present case shows the inflam¬ matory lesions in early ocular con¬ genital syphilis with involvement mainly in the anterior uvea. There is a focal granulomatous reaction in the pupil, with rupture of the lens capsule and the presence of numerous spirochetes in the lens cortex. References 1. Igersheimer J: Beitrag zur Klinik und Pathologischen Anatomie der Augensyphilis. Graefes Arch Ophthalmol 84:48-66, 1913. 2. Friedenwald JS: Ocular lesions in fetal syphilis. Trans Am Ophthalmol Soc 27:203-218, 1929. 3. Hogan MJ, Zimmerman LE: Ophthalmic Pathology. Philadelphia, WB Saunders Co, 1962, p 398. 4. Klauder
JV, Meyer GP: Chorioretinitis of congenital syphilis. Arch Ophthalmol 49:139-157, 1953. 5. Walsh
FB, Hoyt WF: Clinical Ophthalmology. Baltimore, Williams &
NeuroWilkins
Co, 1969, vol 2, p 1576. 6. Thiel HJ, Harms D: Pathologisch-anatomische Augenbefunde bei f\l=o"\talerLues. Klin Monatsbl Augenheilkd 154:712-716, 1969. 7. Duke-Elder S: System of Ophthalmology. London, Henry Kimpton, 1966, vol 9, pp 306\x=req-\
Fig 4.—Chronic inflammatory (hematoxylin-eosin, 100).
cells
infiltrating
iris stroma and
covering
anterior surface
Downloaded From: http://archopht.jamanetwork.com/ by a University of Manitoba User on 06/06/2015
318. 8. Igersheimer J: Syphilis and Auge, in Jadassohn J (ed): Handbuch der Haut und Geschlechtskrankheiten. Berlin, Julius Springer, 1928, vol 17, pp 228-232.
Congenital Syphilis
Eye
Francisco Contreras, MD, Jose Pereda, MD
examination of a girl congenital syphilis revealed a focal granulomatous reaction involving the anterior uvea and lens. Krajian's (silver) stain exhibited spiral forms that were morphologically compatible with Trepo\s=b\ Pathological
with
pallidum within the lens cortex. These findings have been rarely reported in early congenital lues.
nema
(Arch Ophthalmol 96:1052-1053, 1978)
anterior pole transilluminated poorly. The eye was sectioned horizontally. The anterior chamber was partially filled with a white material that occu¬ pied the pupillary space and was adherent to the anterior pole of the lens and the central part of the iris. The iris-lens diaphragm was displaced anteriorly. Retinal folds were present. The sclera and optic nerve-head were normal.
ocular lesions in
histological Thecongenital syphilis
are
not
com¬
monly mentioned in the current litera¬
present a case of an unusual iridolenticular granuloma in a syphilitic newborn. ture.13 We
REPORT OF A CASE A 2.32-kg girl was born on Oct 24,1971 at the Maternity Hospital in Lima, Peru, after 35 weeks' gestation. She had an opaque, gray mass in the left pupil, and erythematous maculae on the entire body, particularly on the face. Twenty-four hours after her birth, the girl had notable jaun¬ dice of the skin and mucosa. Results of serological tests given to the child's mother were positive (VDRL test for syphilis, 3 + and quantitative Kahn's test, dilution, 1:32). The girl died three days after birth. The main autopsy findings were: hepatosplenomegaly, fibrosis of the pancreas, and
pulmonary hemorrhage. OCULAR PATHOLOGICAL FINDINGS Macroscopic Examination
The left measured globe 17 x 17 X 16 mm, with 3 mm of optic nerve attached to the sclera. The cornea measured 9 x 8.5 mm. The Accepted for publication Oct 15, 1977. From the Ophthalmic Pathology Laboratory, Department of Ophthalmology, Hospital Santo Toribio de Mogrovejo, Lima, Peru (Dr Contreras), and the Department of Pathology, Maternity Hospital, Lima. Reprint requests to Hospital Santo Toribio de Mogrovejo, Ophthalmic Pathology Laboratory, Ancas 1271,
Lima, Peru (Dr Contreras).
Light Microscopic
Examination
Bowman's membrane was discon¬ tinuous in some sectors, and chronic inflammatory infiltrates in the ante¬ rior layers of the central corneal stroma were seen. The anterior cham¬ ber was narrow and filled with fibrin¬ ous exúdate and inflammatory cells. The iris exhibited peripheral ante¬ rior synechiae to the trabecular mesh¬ work (Fig 1) and posterior central synechiae to the lens (Fig 2). In the latter area, a dense, inflammatory exúdate that covered the pupil and involved the anterior pole of the lens was observed. The lens capsule was ruptured. There were numerous poly-
COMMENT The most frequent clinical manifes¬ tations of early congenital syphilis include "salt and pepper" chorioretini¬ tis.4-5 Less frequent involvement of the cornea, iris, and ciliary body are reported." Duke-Elder quotes several publications from the middle of the 19th century that call attention to the acute iridocyclitis as a clinical mani¬ festation of congenital syphilis, in¬ cluding the presence of exudative membranes that occlude the pupil. In this century, Igersheimer* pointed out the iritis of the newborn as a sign of congenital syphilis. This iritis usually is mild, with little inflammatory reaction, and may be overlooked by the clinician. Iger¬ sheimer mentions two clinicopathological cases of patients similar to ours. These patients were 4- and 6-
nuclears, macrophages, lymphocytes, and plasma cells surrounding a necrot¬ ic focus. Krajian's stain showed a large number of spiral forms within the lens cortex that were morphologi¬ cally compatible with Treponema pallidum (Fig 3).
The iris stroma showed vascular
congestion and chronic inflammatory cell infiltration (Fig 4). The ciliary body showed inflammatory infiltra¬ tion, hemorrhage, and splitting of the nonpigmented epithelial layer at the pars plana. The vitreous base exhib¬ ited moderate inflammatory infiltra¬ tion and hemorrhage. In the choroid, the inflammatory response was less intensive. The retina presented some folds, vascular congestions, and local¬ ized hemorrhages.
Downloaded From: http://archopht.jamanetwork.com/ by a University of Manitoba User on 06/06/2015
Fig 1 .—Peripheral anterior synechia of iris. Chronic inflammatory exúdate in anterior and posterior chamber (hematoxylin-
eosin, x40)
Fig 2.—Focal necrotic inflammatory reac¬ tion occluding pupil with lens cortex involvement. Central posterior synechia of Iris (hematoxylin-eosin 4). month-old syphilitic gray-yellow pupillary
Fig
be
3—Numerous seen
spirochete-like forms (Krajian's stain, 400).
in lens cortex. Small
portion of lens capsule can
infants masses,
with
narrow
anterior chambers, and ocular hyper¬ tension. In one of these eyes, Iger¬ sheimer performed an Elliott filtering procedure, followed some months later by phthisis bulbi. In both cases, the eyes were enucleated. On pathological examination, the eyes had marked chronic inflammatory pupillary mem¬ branes with iris stroma involvement. In the eye on which operation had previously been performed the lens capsule was ruptured, and the cortex showed inflammatory infiltration. Friedenwald,- in routine postmor¬ tem examination of 175 newborn or stillborn infants, found 30 cases of congenital syphilis. In nine of these 30 infants, ocular inflammatory lesions were found. In five of these nine cases, the anterior uvea was involved. Interestingly, Friedenwald found spirochetes in only two cases, along peri¬ vascular sheathing of the penetrating vessels of the sclera at the ciliary body level. The present case shows the inflam¬ matory lesions in early ocular con¬ genital syphilis with involvement mainly in the anterior uvea. There is a focal granulomatous reaction in the pupil, with rupture of the lens capsule and the presence of numerous spirochetes in the lens cortex. References 1. Igersheimer J: Beitrag zur Klinik und Pathologischen Anatomie der Augensyphilis. Graefes Arch Ophthalmol 84:48-66, 1913. 2. Friedenwald JS: Ocular lesions in fetal syphilis. Trans Am Ophthalmol Soc 27:203-218, 1929. 3. Hogan MJ, Zimmerman LE: Ophthalmic Pathology. Philadelphia, WB Saunders Co, 1962, p 398. 4. Klauder
JV, Meyer GP: Chorioretinitis of congenital syphilis. Arch Ophthalmol 49:139-157, 1953. 5. Walsh
FB, Hoyt WF: Clinical Ophthalmology. Baltimore, Williams &
NeuroWilkins
Co, 1969, vol 2, p 1576. 6. Thiel HJ, Harms D: Pathologisch-anatomische Augenbefunde bei f\l=o"\talerLues. Klin Monatsbl Augenheilkd 154:712-716, 1969. 7. Duke-Elder S: System of Ophthalmology. London, Henry Kimpton, 1966, vol 9, pp 306\x=req-\
Fig 4.—Chronic inflammatory (hematoxylin-eosin, 100).
cells
infiltrating
iris stroma and
covering
anterior surface
Downloaded From: http://archopht.jamanetwork.com/ by a University of Manitoba User on 06/06/2015
318. 8. Igersheimer J: Syphilis and Auge, in Jadassohn J (ed): Handbuch der Haut und Geschlechtskrankheiten. Berlin, Julius Springer, 1928, vol 17, pp 228-232.
