Primary Aberrant Oculomotor Nerve Regeneration From A Posterior Communicating Artery Aneurysm (PDFDrive)

CLINICOPATHOLOGIC REPORTS, CASE REPORTS, AND SMALL CASE SERIES
SECTION EDITOR: W. RICHARD GREEN, MD
Human Subconjunctival
Infection of Macacanema
formosana: The First Case
of Human Infection
Reported Worldwide
In the last few decades, the appar-

ently more frequent occurrence of
zoonotic helminth infections in man
have attracted the attention of para-
sitologists.1 In particular have been
those brought about by the genus
Dirofilaria, which has been re-
ported to cause more than 900 cases
of human infection. Most of them
were due to Dirofilaria repens and Di-
rofilaria immitis. Therefore, it is
worth reporting on a recent human
case caused by Macacanema formo-
sana, a filarial parasite of the catar- Figure 1. The subconjunctival worm under slitlamp biomicroscope. It was motile and active.
rhine monkeys (Macaca cyclopsis),
that came to our attention and ap-
parently has never been reported in
humans.
Report of a Case. The patient was a

19-year-old woman who had been
living in an urban area of northern
Taiwan since childhood. She visited
the Department of Ophthalmology,
Taipei Veterans General Hospital in
November 1999 owing to sudden
pain and redness in her left eye. Mild
For editorial comment

see page 634
swelling of the left lower eyelid was
noted for 1 month and several epi-
sodes of severe left periorbital pain
happened within these few months.
She had never traveled outside Tai- Figure 2. The whole nematode measured 7.5 ⫻ 0.5 cm.
wan and never had animals as pets.
Her family had a routine annual pic- lamp examination disclosed a sub- normal. No microfilaria was found
nic at the riverside of the Hsin-Tien conjunctival active worm at the nasal in blood and urine samples. No le-
Shi in northern Taiwan until 3 years side of her left eye with marked con- sion was seen on chest radiographs
previously. She could not recall any junctival congestion (Figure 1). In- or brain computed tomographic
unusual insect bite or any contact direct ophthalmoscopic examina- scans.
with monkeys during these picnics. tion revealed a normal fundus. Under local anesthesia, the con-
Complete ophthalmologic ex- Findings on examination of the right junctiva was incised and dissected.
amination was performed. Best- eye were unremarkable. The re- The worm was removed alive and in-
corrected visual acuity was 6/4 OU sults of blood and biochemical tests, tact and preserved in 5% formalde-
with Snellen distance chart. Slit- including the eosinophil count, were hyde for identification. Histologi-
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643
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a row of approximately 7 caudal pa-
pillae, asymmetrically arranged, on
either side of the cloaca.
The esophagus could be visu-
alized through the semitransparent
body wall. It was divided into a short
muscular portion and a much longer
glandular portion. The lateral chords
contained dark, granular material
that could be seen throughout the
length of the chords (Figure 5).
In the transverse section of the
worm (Figure 6), the multilay-
ered structure of the cuticle was evi-
dent as was the underlying thin hy-
podermis. The large lateral chords
displayed clusters of pigmented
granules that were scattered in the
hypodermis as well. The muscle cells
were coelomyarian and numerous in
Figure 3. The cephalic end of the nematode (original magnification ⫻60). each quadrant of the body. The pseu-
docoele was virtually filled with the
large genital tube packed with de-
veloping spermatozoa (spermato-
cytes). The digestive tube was small
and round and lined with a rela-
tively thick endothelium. On the
basis of these morphological char-
acteristics, the nematode can be
identified as an adult male of Maca-
canema formosana (Nematoda, On-
chocercidae, Dirofilariinae). The
morphological characteristics to dif-
ferentiate M formosana from other
nematodes (Filarioidea, Ascari-
doid, Oxyuroidea, Strongylid, Spi-
rurid, Strongyloid, Rhabditoid, and
Trichinellae) were well described by
Orihel and Ash.1 Schad and Ander-
son2 had reported the differential di-
agnosis of M formosana with other
Onchocercidae, in particular with
Figure 4. The caudal end of the nematode where the 2 asymmetrical spicules (S) and some papillae (P) Edesonfilaria malayensis, the nema-
can be seen (original magnification ⫻60). tode most similar to M formosana.
cal sections were prepared for further ther confirmation of the identity. It Comment. Macacanema formosana
study of the parasite. Periorbital pain was threadlike and approximately was first identified and described by
and conjunctival congestion re- 7.5 cm long with a maximum diam- Schad and Anderson in 1963.2 It was
solved after the surgery. There was eter of 510 µm (Figure 2). The an- a nematode of new genus and spe-
no sign of recurrent infection after terior extremity (Figure 3) was sub- cies of the family Onchocercidae,
2 years’ follow-up. conical with a small, round oral subfamily Dirofilariinae. They found
opening without lips and sur- the filaria in the peritracheal tissue
Parasitologic Findings. The para- rounded by 2 pairs of circumoral and diaphragm of Macaca cyclop-
site was sent to the Department of papillae. The cuticle was thin with sis, a monkey native to Formosa
Public Veterinary Health and Ani- fine transverse striations. The tail (Taiwan). Like all filariae, the mi-
mal Pathology, University of Bolo- (Figure 4) was short with 2 spic- crofilariae were found in the blood
gna, Bologna, Italy, and the Depart- ules of unequal length. The longer of the host. Little was known about
ment of Tropical Medicine, Tulane one (on the worm’s left side) mea- this parasite except for a report pub-
University Medical Center, New Or- sured 512 µm and the shorter (on lished in 1968 by Bergner and
leans, La, for identification. It was the worm’s right side) one, par- Jachowski.3 They found the para-
later sent to the Museum of Natu- tially protruding from the cloaca, site in the peritracheal and man-
ral History, Paris, France, for fur- was about 120 µm long. There was dibular intermuscular connective tis-

644

sues. There was no subconjunctival
infection reported in the monkey.
The prevalence rate of M formosana
in the Taiwanese monkey was re-
ported to be as high as 42% in north-
ern Taiwan where the patient had
picnicked. Culicoides (Insecta, Dip-
tera, Ceratopogonidae), a cosmo-
politan genus of biting midges of-
ten causing great annoyance to man
and animals, was proposed as the
most probable transmitting vector of
the parasite. Many species of Culi-
coides were identified in northern
Taiwan. This large reservoir of the
parasite and the existence of the vec-
tors made human infection pos-
sible. To our knowledge, this is the
first case of human infection caused
by M formosana to be reported
worldwide. However, other cases in Figure 5. The detail of a segment of Macacanema formosana where the scattered brownish granules
the same area may well have taken within the lateral chords (L) can be seen (original magnification ⫻60).
place, but unobserved, as seemed to
happen with D repens in endemic
zones.4 The prepatent period of the
worm has not yet been determined.
Based on the size of the worm, the
patient would have been infected for
at least 1 year and symptoms ap-
peared only after the worm mi-
grated into the subconjunctival
space. The reported human Dirofi-
laria infections usually consisted of
a single worm,5 and treatment for the
soreness consisted of the worm’s sur-
gical removal. There was no evi-
dence of recurrent infection in our
patient after 2 years’ follow-up.
Cases of subconjunctival D re-
pens infection occur widely through-
out European, African, Middle East-
ern, and Asian countries. However, it
has never been reported in Taiwan.
From the cases reported and our ob- Figure 6. The transverse section of the nematode (partial). C indicates cuticle; I, intestine; M, muscular
servation, subconjunctival D repens fibers; T, testicle; and arrowheads, brownish granules in the lateral chord (trichrome-Masson-Goldner
and M formosana infections shared stain, original magnification ⫻250).
the characteristics of sudden onset
and were treated solely by surgical re- nematodes, such as Mansonella per- sible zoonotic infection should be
moval of the worm. From the size of stans and Edesonfilaria species, and kept in mind.
the worms recovered, they might in the selfsame M formosana. Their
have been migrating in subcutane- origin is obscure and further stud- Ling-Ing Lau, MD
ous space for a period of time and ies are necessary to understand Fenq-Lih Lee, MD
caused symptoms only when they ap- their nature. Wen-Ming Hsu, MD
peared in the subconjunctiva. The This is, to our knowledge, the Taipei, Taiwan
episodic periorbital pain preceding first case report of human infection Silvio Pampiglione, MD, PhD
subconjunctival infection in our pa- caused by M formosana although Maria Letizia Fioravanti, PhD
tient could be caused by the worm’s other cases may have gone by un- Bologna, Italy
migration within this area. observed or unidentified. Since the Thomas C. Orihel, PhD
The presence of brownish gran- Taiwan monkeys (Macaca cyclop- New Orleans, La
ules in the lateral chords is a phe- sis) have been largely involved in
nomenon that has been observed laboratory studies and have close We would like to thank Shyh-Haw
on other occasions both in other contact with human beings, pos- Tsay, MD, Department of Pathology,

645

Taipei Veterans General Hospital, and tinel lymph node (SLN) biopsy soon sion in his left upper eyelid, and the
F. Rivasi, PhD, histopathologist at after the diagnosis of his primary tu- area of the neck dissection and pa-
Modena University, Emilia, Italy, for mor. An SLN was identified and rotidectomy had healed. Findings on
preparing the histological sections of showed histologic evidence of MCC. the patient’s ophthalmologic exami-
the parasite; and Kin-Mu Lee, PhD, To our knowledge, this is the first nation were essentially normal, with
Department of Parasitology, Na- reported case of a positive SLN sec- no clinical evidence of local or re-
tional Yang Ming University, Taipei, ondary to MCC of the eyelid. gional recurrence of cancer. His sys-
and Odile Bain, PhD, Museum of temic workup, including com-
Natural History, Paris, France, for Report of a Case. A 61-year-old man puted tomography of the head and
their kind suggestions on the identity noted an erythematous lesion on his neck, abdomen, and pelvis, chest ra-
of the parasite. left upper eyelid in May 2001. He diography, and magnetic reso-
Presented as a poster at the 18th went to his local ophthalmologist, nance imaging of the brain, was
Congress of the Asia-Pacific Academy who excised the lesion but did not negative for tumor. A multidisci-
of Ophthalmology, Taipei, Taiwan, examine it histologically. In June plinary team, including an ophthal-
March 10-14, 2001. 2001, the lesion recurred. The re- mic surgeon, a head and neck sur-
Corresponding author: Fenq- current lesion measured approxi- geon, a head and neck medical
Lih Lee, MD, Department of Oph- mately 12 mm in diameter. The pa- oncologist, and a head and neck ra-
thalmology, Taipei Veterans General tient sought an opinion from an diation oncologist examined the
Hospital, No. 201, Section 2, Shih-Pai oculoplastic surgeon, who per- patient and recommended that he
Road, Shih-Pai, Taipei 11217, Tai- formed a wide local excision of the receive adjuvant external beam
wan (e-mail address: fllee@ms3 lesion with frozen section control of radiation therapy to the eyelid,
.hinet.net). the margins. The histologic find- parotid nodes, and deeper cervical
Reprints: Ling-Ing Lau, MD, De- ings were consistent with MCC of nodes. The team also recom-
partment of Ophthalmology, Taipei the eyelid (Figure, A and B). Sen- mended that the patient be given 4
Veterans General Hospital, No. 201, tinel lymph node biopsy was sched- courses of chemotherapy with eto-
Section 2, Shih-Pai Road, Taipei uled but had to be delayed because poside and cisplatin after the
11217, Taiwan (e-mail: lilau@ms41 the patient developed acute appen- completion of radiation therapy.
.hinet.net). dicitis, necessitating an emergent ap-
pendectomy. In July 2001, the pa- Comment. In patients with MCC,
1. Orihel Th C, Ash LR. Parasites in Human Tis-
sues. Chicago, Ill: American Society of Clinical tient underwent SLN biopsy using the regional lymph nodes are
Pathologists;1995. a combination of isosulfan blue dye thought to be the most common and
2. Schad GA, Anderson RC. Macacanema formo-
sana n.g., n.sp. (Onchocercidae: Dirofilariinae)
and Tc 99m–labeled sulfur colloid. earliest site of metastasis; thus, ad-
from Macaca cyclopsis of Formosa. Can J Zool The afferent lymphatics were iden- juvant treatment of the regional
1963;41:797-801. tified before surgery using radio- lymph nodes has been advocated by
3. Bergner JF, Jachowski LA. The filarial parasite,
Macacanema formosana, from the Taiwan mon- nucleotide imaging. Intraopera- many investigators.3,5,6 Jean et al7
key and its development in various arthropods. tively, a combination of radiolabeled successfully identified 1 or more
Formosan Sci. 1968;22:1-68. sulfur colloid and isosulfan blue dye SLNs in 19 of 20 patients with stage
4. Pampiglione S, Canestri Trotti G, Rivasi F. Hu-
man dirofilariasis due to Dirofilaria (Noch- was used to identify SLNs. An area I MCC who underwent SLN biopsy
tiella) repens: a review of world literature. Paras- of focal radioactive uptake was iden- at the time of initial wide local ex-
sitologia. 1995;37:149-193.
5. Ruiz-Moreno JM, Bornay-Llinares FJ, Prieto Maza
tified in the left preauricular (pa- cision. The authors found that 5
G, Medrano M, Simon F, Eberhard ML. Subcon- rotid) area using a handheld gamma (26%) of the 19 patients in whom
junctival infection with Dirofilaria repens: sero- probe and was marked on the skin. SLNs were successfully identified
logical confirmation of cure following surgery.
Arch Ophthalmol. 1998;116:1370-1372. The corresponding SLN was re- had at least 1 histologically posi-
moved and analyzed histologically tive SLN. Other isolated cases of SLN
using serial sectioning and immu- biopsy for MCC have also been re-
nohistochemical staining. The node ported.8
Merkel Cell Carcinoma was found to be positive for MCC Merkel cell carcinoma of the
of the Eyelid With a (Figure, C and D). The patient sub- eyelid is thought to account for 10%
Positive Sentinel Node sequently underwent a total paroti- of all cases of MCC.2 In a review of
dectomy and completion neck all previously reported cases of MCC
Merkel cell carcinoma (MCC) of the dissection. The parotidectomy spe- of the eyelid, Kivela and Tarkkat-
eyelid is a rare but aggressive ma- cimen included 1 additional lymph nen2 concluded that up to two thirds
lignancy that metastasizes early to re- node that was positive for MCC. The of patients eventually develop re-
gional lymph nodes.1 Most clinical node was located in the deep lobe gional nodal involvement. This rate
series suggest a rate of regional nodal of the left parotid gland and showed is higher than the rate reported in
involvement between 21% and extracapsular extension. most single series of MCC of the eye-
66%.2-4 Early detection of occult re- In September 2001, the pa- lid. In the largest single series to date,
gional nodal disease may allow for tient self-referred to the University Peters et al3 reported clinical re-
early institution of adjuvant therapy. of Texas M. D. Anderson Cancer gional nodal involvement in 3 (21%)
We describe a patient with MCC of Center (Houston) for further man- of their 14 patients.
the eyelid with clinically unin- agement of his tumor. At this time, Sentinel lymph node biopsy al-
volved nodes who underwent sen- he had a well-healed area of exci- lows for early detection of occult re-

646

A B
C D
A, Histologic section of Merkel cell carcinoma (MCC) of the upper eyelid (primary lesion) demonstrates a diffuse, poorly cohesive proliferation of small cells with
finely dispersed chromatin, lacking prominent nucleoli, and without significant intervening stroma (original magnification ⫻4). B, High-powered view shows round
cells with finely distributed chromatin. Many of the cells display hyperchromatic nuclei consistent with apoptotic bodies (original magnification ⫻40). C, Histologic
section of the sentinel lymph node shows small foci of round cells similar in appearance to the primary lesion (original magnification ⫻20). D, High-powered view
of the same node immunostained with antibodies against cytokeratin shows positive cytokeratin expression supporting the diagnosis of metastatic MCC (original
magnification ⫻40).
gional lymph node metastasis and moid carcinoma), using radiola- tion of micrometastasis in the re-
thus, more accurate staging of MCC beled sulfur colloid alone. These gional nodes allows for immediate
and the possible institution of early authors reported successful identi- institution of adjuvant therapy,
adjuvant therapy. Although SLN bi- fication of at least 1 SLN in 5 pa- which may include completion neck
opsy has recently become the stan- tients, although the technique used dissection, external beam radiation
dard of care for most solid tumors in the latter report is believed by therapy, and adjuvant chemo-
throughout the body, SLN biopsy in some investigators not likely to lead therapy.
the periocular area remains investi- to the correct identification of the
gational.9 To our knowledge, there sentinel nodes.12 Neither of the 2 Bita Esmaeli, MD
have been only 2 previous reports of previous reports of SLN biopsy for Aresu Naderi, MD
application of SLN biopsy tech- conjunctival and periocular tu- Lillie Hidaji, BS
niques for conjunctival and periocu- mors found a positive SLN. To our George Blumenschein, MD
lar tumors.10,11 We described suc- knowledge, ours is the first re- Victor G. Prieto, MD, PhD
cessful identification of SLNs in a ported case in which an SLN was Houston, Tex
single patient with a conjunctival successfully identified in a patient
melanoma, using a combination of with an eyelid tumor and was also Corresponding author and reprints:
radiolabeled sulfur colloid and iso- found to be histologically positive. Bita Esmaeli, MD, Ophthalmology Sec-
sulfan blue dye.10 Wilson et al11 at- This case underscores the feas- tion, Department of Plastic Surgery,
tempted identification of SLNs in 5 ability and potential usefulness of Box 443, M. D. Anderson Cancer Cen-
patients with periocular tumors (2 SLN biopsy as a method for identi- ter, 1515 Holcombe Blvd, Houston, TX
melanomas, 2 meibomian gland car- fying occult metastatic disease from 77030 (e-mail: besmaeli@mdanderson
cinomas, and 1 case of mucoepider- an MCC of the eyelid. Early detec- .org).

647

1. Singh AD, Eagle RC Jr, Shields CL, Shields JA. related macular degeneration with- Clinic, Rochester, Minn, on March
Merkel cell carcinoma of the eyelids. Int Oph-
thalmol Clin. 1993;33:11-17. out observing deleterious retinal 8, 2000, because of a growing pig-
2. Kivela T, Tarkkanen A. The Merkel cell and as- complications.5 The encouraging re- mented choroidal lesion in the left
sociated neoplasms in the eyelids and periocu- sults in pilot studies with TTT in the eye that had been observed to in-
lar region. Surv Ophthalmol. 1990;35:171-
187. management of occult choroidal crease in thickness from 2.3 to
3. Peters GB, Meyer DR, Shields JA, et al. Man- neovascular membranes has led to greater than 4 mm during an inter-
agement and prognosis of Merkel cell carci-
noma of the eyelid. Ophthalmology. 2001;108:
the development of a multicenter val of 9 years.
1575-1579. prospective randomized clinical trial The visual acuity was 20/20 OD
4. Victor NS, Morton B, Smith JW. Merkel cell can- (Transpupillary Thermotherapy and 20/25+3 OS. The right eye was
cer: is prophylactic lymph node dissection in-
dicated? Am Surg. 1996;62:879-882. [TTT] of Occult Subfoveal Choroi- normal. Results of examination of the
5. Fenig E, Brenner B, Katz A, et al. The role of dal Neovascularization in Patients left eye showed a normal anterior seg-
radiation therapy and chemotherapy in the With Age-Related Macular Degen- ment and a pigmented, elevated cho-
treatment of Merkel cell carcinoma. Cancer.
1997;80:881-885. eration Trial) in which patients with roidal lesion, measuring 9⫻9 mm in
6. Boyle F, Pendlebury S, Bell D. Further in- subfoveal choroidal neovascular base dimension, located approxi-
sights into the natural history and manage-
ment of primary cutaneous neuroendocrine
membranes are randomized to a mately 4 mm superonasal to the disc
(Merkel cell) carcinoma. Int J Radiat Oncol Biol sham treatment or a treatment with (Figure 1). The ultrasonographic
Phys. 1995;31:315-323. a single 60-second exposure of in- studies demonstrated a solid, dome-
7. Jean M, Solorzano C, Ross M, et al. Role for sen-
tinel node biopsy in Merkel cell carcinoma pa- frared light from the diode laser (810 shaped tumor (B-scan) with low in-
tients . Proc Am Soc Clin Oncol. 2001;20:358a. nm) using a beam diameter of 3 mm ternal reflectivity (A-scan) consis-
8. Wasserberg N, Feinmesser M, Schachter J, Fenig and 800 mW of power (Elias Rei- tent with the diagnosis of melanoma.
E, Gutman H. Sentinel-node guided lymph-
node dissection for Merkel cell carcinoma. Eur chel, MD, oral and written commu- The thickness of the lesion was 4.4
J Surg Oncol. 1999;252:444-446. nication, January 12, 2000). The mm. A subretinal fibrotic plaque over-
9. Esmaeli B. Sentinel lymph node mapping for
patients with cutaneous and conjunctival mela-
unique opportunity afforded by a pa- lying the central portion of the tu-
noma. Ophthalmol Plast Reconstr Surg. 2000; tient scheduled for enucleation for mor and a secondary retinal detach-
16:170-172. a malignant melanoma located in the ment overlying the nasal portion of
10. Esmaeli B, Eicher S, Popp J, et al. Sentinel lymph
node biopsy for conjunctival melanoma. Oph- nasal choroid led to this experi- the tumor were seen. Subretinal fluid
thalmol Plast Reconstr Surg. 2001;17:436-442. ment in which infrared light from a extended approximately 1 disc diam-
11. Wilson MW, Fleming JC, Fleming RM, Haik diode laser was directed to the eter beyond the nasal periphery of the
B. Sentinel node biopsy for orbital and ocular
adnexal tumors. Ophthalmol Plast Reconstr Surg. macula through a contact lens us- mass. Small accumulations of exu-
2001;17:338-344. ing the variables identical to those dates at the nasal boundary of the tu-
12. Esmaeli B. In discussion of: Wilson MW, Flem-
ing JC, Fleming RM, Haik B. Sentinel node bi-
recommended in the TTT of Oc- mor were also seen. The retina in the
opsy for orbital and ocular adnexal tumors. Oph- cult Subfoveal Choroidal Neovas- central macular region appeared
thalmol Plast Reconstr Surg. 2001;17:338-345. cularization in Patients With Age- subtly thickened on biomicroscopy
Related Macular Degeneration Trial. findings.
The diagnosis of actively grow-
Report of a Case. A 65-year-old ing malignant melanoma was made,
The Effect of Transpupillary woman was referred to the Depart- and definitive therapy was recom-
Thermotherapy on the ment of Ophthalmology of the Mayo mended. Therapeutic options were
Human Macula
Transpupillary thermotherapy (TTT)

was introduced by investigators from
the Netherlands in 1995 as an alter-
native treatment for choroidal mela-
noma.1,2 Since that time, TTT has
been used to treat small choroidal
melanomas, and preliminary results
indicating that TTT can control small
melanomas with follow-up of 5 or
more years have been published.3,4
However, localized retinal destruc-
tion, vascular occlusions, and nerve
fiber bundle defects are commonly as-
sociated with effective treatment of
small melanomas with TTT. De-
spite these observed retinal compli-
cations, some investigators have re-
cently reported that TTT, using the
same laser intensity to treat choroi-
dal melanoma, may successfully treat
Figure 1. Color fundus photograph showing the location of a choroidal melanoma in the left eye.
occult subfoveal choroidal neovas- A serous detachment of the sensory retina was clinically confined to the region overlying
cularizations in patients with age- the nasal portion of the tumor.

648

A B
C D
Figure 2. Prelaser exposure. A, Color fundus photograph of posterior pole showing a normal-appearing macular region. B, Early arteriovenous phase during
fluorescein angiography. C, Arteriovenous phase during fluorescein angiography showing subtle hyperfluorescence in the posterior pole and early cystoid edema.
D, Recirculation phase showing patchy diffuse intraretinal dye staining and pattern of sector cystoid macular edema.
discussed. Brachytherapy was en- the macula of the left eye was ex- dehyde. The retina and choroid in
couraged, but the patient was con- posed to a 3-mm beam of infrared the macula were dissected en bloc,
cerned about the potential for con- laser light (810 nm) using 800 mW fixed in solution, and embedded for
tinuing problems with the eye and of power. The laser beam was deliv- transmission electron microscopy.
strongly desired an enucleation. ered to the posterior pole through Light microscopy of this region was
We obtained institutional re- a standard fundus contact lens (Carl not performed. The remaining tis-
view board approval for the experi- Zeiss, Inc, Thornwood, NY), expos- sue was examined by means of light
ment. The patient was fully in- ing the macula to light from the in- microscopy.
formed regarding the use of infrared frared laser for 60 seconds. Visual
laser light in the management of acuity was measured with a stan- Histopathologic Findings. Color
macular disease and the possibility dard Snellen chart, and the central photographs of the macular region
that the treatment could cause an al- field was evaluated with an Amsler showed no obvious abnormality
teration of the pigment epithelium grid 3 minutes after light exposure. (Figure 2A). The pretreatment fluo-
and retina overlying the choroidal Five days after laser exposure, we re- rescein angiogram showed an early
target tissue. She agreed to have her examined the eye. Best corrected vi- pattern of complex vascular loops
retina exposed to light from the in- sual acuity was determined, and the within the tumor and patchy leak-
frared laser for 60 seconds using a central visual field was evaluated age of dye from these sites leading
power of 800 mW. She also agreed with an Amsler grid. Biomicros- to early patchy tissue staining of the
to undergo color fundus photogra- copy and ophthalmoscopy were per- tumor. Later frames showed diffuse
phy and fluorescein angiography of formed, and the appearance of the staining of the retina overlying the
the retina before and after light ex- posterior pole was documented with tumor. Dye leakage from fluorescein-
posure. color fundus photography and fluo- incompetent capillaries in the peri-
After documenting the appear- rescein angiography. The eye was foveal region was seen, which pro-
ance of the tumor (Figure 1) and the enucleated approximately 3 hours duced an incomplete pattern of
macula by means of slitlamp biomi- later and fixed in a 0.1M phosphate- cystoid edema visible in the late
croscopy color photographs and buffered solution containing 4% frames of the study and arclike ar-
fluorescein angiography before TTT, paraformaldehyde and 1% glutaral- eas of diffuse intraretinal staining su-

649

A B
Figure 3. Five days after infrared laser exposure. A, Color

fundus photographs 5 days after 60-second infrared laser
exposure of the macula to a 3-mm beam of infrared
light from the diode laser using 800 mW of power.
B, Arteriovenous phase during fluorescein angiography
showing subtle hyperfluorescence in posterior pole and early
sector cystoid edema. C, Recirculation phase showing
diffuse intraretinal dye leakage and sector cystoid macular
edema. The findings are unchanged from those documented
in Figure 2.
perior to the fovea, temporal to the change in the appearance of the fun- shapes rather than the usual oval-
fovea, and to a lesser extent infero- dus. The fluorescein angiogram at shaped melanosome granules (Fig-
temporal to the fovea (Figure 2B-D). the 5-day follow-up visit demon- ure 4C). Focal disruption of cellu-
The largest dimension of the stain- strated findings identical to those lar membranes and dispersion of
ing site had a diameter of approxi- seen in the pretreatment angio- pigment granules among outer seg-
mately 3 mm (before and after laser gram (Figure 3). ments of photoreceptor cells were
exposure). Light microscopy of the enucle- seen. Vacuolation and distension of
During and immediately after ated eye showed a malignant mela- the outer segments of the photore-
transpupillary exposure of the macula noma located in the posterior cho- ceptors were also observed, with par-
to light from the infrared laser, no dis- roid, nasal to the optic disc, that tial disintegration of the lamellar
cernible alteration in the ophthalmo- formed a mass measuring 10 ⫻ 10 structure with a rare thumbprint-
scopic appearance of the fundus was ⫻ 3 mm and consisted predomi- like configuration (Figure 4D). The
seen. Three minutes after light ex- nantly of epithelioid cells. A serous underlying choriocapillaris showed
posure, the patient noticed a bluish detachment of the sensory retina congestion, but the vessels were nor-
discoloration in the central field of was seen overlying the nasal por- mal, with intact walls and normal en-
vision that she outlined on the Am- tion of the tumor. A plaque of fi- dothelial cells. The larger choroi-
sler grid. The best-corrected dis- brous tissue was visible over the dal vessels also appeared normal
tance visual acuity 3 minutes after central portion of the tumor, and (Figure 4E).
light exposure was 20/100. When the cystoid degeneration was present in
patient returned 5 days later, the cen- the overlying retina (Figure 4A Comment. Transpupillary thermo-
tral visual acuity had recovered to the and B). therapy has been used in recent years
pretreatment level (20/25), but she Results of the ultrastructural as a therapeutic alternative in the
still recognized a faint bluish dis- examination of the macular and management of some choroidal
coloration in the central part of her paramacular region, which had been melanomas.1-4 We have shown that
vision on Amsler grid testing. She re- dissected and embedded for trans- effective treatment of selected small
ported that this dyschromatopsia mission electron microscopy, choroidal melanomas almost al-
had been decreasing in intensity each showed retinal pigment epithelial ways leads to a profound field de-
day since the exposure. Examina- cells with numerous cytoplasmic fect because of destruction of the
tion of the fundus 5 days after laser granules of lipofuscin and melano- photoreceptors and nerve fibers in
exposure showed no discernible fuscin with round and irregular the retina overlying the treated tu-

650

A B
C D
Figure 4. A, Low-power light microscopy showing choroidal

malignant melanoma with a layer of heavily pigmented spindle
cells near its surface. A fibrous plaque is visible over the
central portion of the tumor, and the overlying retina shows
marked cystoid degeneration (hematoxylin-eosin, original
magnification ⫻20). B, High-power light microscopy showing
that this neoplasm is composed of a predominance of
epithelioid cells. No mitoses or necrosis is seen
(hematoxylin-eosin, original magnification ⫻200). C, Electron
microscopy of the macular region at the site of laser exposure
showing retinal pigment epithelium containing cytoplasmic
granules of lipofuscin (arrowhead) and melanofuscin (arrow)
with round and irregular shapes. Focal disruption of cellular
membranes with dispersion of pigment granules into outer
segments of photoreceptor cells is seen (lead citrate, original
magnification ⫻25000; bar indicates 2 µm). D, Electron
microscopy showing vacuolization and distension of the outer
segments of the photoreceptors with partial disintegration of
the lamellar structures and focal thumbprintlike configuration
of some lamellae (lead citrate, original magnification ⫻25000;
bar indicates 2 µm). E, Electron microscopy showing
congested but otherwise normal-appearing vessels with intact
walls in the choroid underlying the macular retina at the site of
laser exposure (lead citrate, original magnification ⫻48000;
bar indicates 3 µm).
mor.4 When used for treatment of a multicenter randomized study of oc- generation (800 mW, 60 seconds of
choroidal melanoma, the same vari- cult choroidal neovascular mem- exposure, and 3-mm beam diam-
ables recommended by the ongoing branes in age-related macular de- eter) are usually associated with ex-

651

tensive retinal damage, causing a lo- pears similar to the relative absence lar region by means of ultrastruc-
calized scotoma and, frequently, a of a take that we have observed when tural studies.
wedge-shaped field defect as a re- attempting to prophylactically treat The absence of recognizable de-
sult of nerve fiber bundle destruc- normal-appearing tissue adjacent to struction of the retina and retinal
tion. Therefore, some concern ex- a pigmented choroidal melanoma. vasculature observed in this single
ists that these variables have been Takes are often not well seen in the experiment does not ensure that vas-
chosen for treatment of choroidal normal-appearing tissue. For ex- cular closure and retinal destruc-
neovascular membranes. Our pa- ample, a 3-mm beam of laser light tion will not occur when TTT is used
tient with a malignant melanoma that is placed so as to equally straddle to treat occult choroidal neovascu-
located in the nasal choroid, sched- the edge of a pigmented choroidal tu- lar membrane. However, in this case,
uled for enucleation, consented to mor and clinically normal tissue with clinical and angiographic evi-
have her macula exposed to light adjacent to it often dramatically out- dence of mild retinal edema and no
from the diode laser using the vari- lines the ophthalmoscopically rec- retinal or subretinal blood, a 60-
ables cited above. In this patient’s af- ognizable perimeter of the tumor. The second exposure of 800 mW using
fected eye, subtle intraretinal edema tumor, the overlying retina, and the a 3-mm beam diameter did not cause
involved the posterior pole, presum- retinal pigment epithelium become clinically recognizable damage to the
ably related to the actively growing gray white, whereas the adjacent macular retina, the retinal vessels, or
melanoma located in the superior retina overlying normal-appearing the underlying choriocapillaris and
nasal fundus. This intraretinal ede- choroid frequently remains clini- other choroidal vessels.
ma was recognized in the macula cally unchanged or only minimally
before exposure to the laser light. The gray. We believe that the pigment Dennis M. Robertson, MD
melanomaitselfshowedcomplexvas- within the tumor is largely respon- Diva R. Salomão, MD
cular patterns on fluorescein angi- sible for generating a considerable Rochester, Minn
ography with extensive leakage of amount of heat, which causes the tu-
dye that diffused into the overlying mor, the overlying pigment epithe-
retina, where heavy fluorescein stain- lium, and retina to turn gray more This study was supported in part by a
ing was seen in the late part of the readily. In addition, the choriocap- grant from Research to Prevent Blind-
fluorescein study. illaris and larger vessels in the cho- ness, Inc, New York, NY, and in part
Laser exposure of the macula roid are altered in the presence of a by the Mayo Foundation, Rochester,
failed to produce a clinically recog- melanoma, thus decreasing the abil- Minn.
nizable reaction in the retina dur- ity of the choroid to play its role as a We thank Bonnie Ronken for her
ing the treatment, and no changes heat sink to disperse energy. The ab- secretarial support and Cheryl Hann,
were recognized on results of a care- sence of a heavy concentration of pig- MS, for her support with the electron
ful clinical examination 5 days af- ment and the presence of a normal microscopy studies.
ter exposure. A fluorescein angio- choroid, providing a normal heat Corrresponding author and
gram 5 days after the laser exposure sink, can facilitate dispersement of reprints: Dennis M. Robertson, MD,
showed no difference from the fluo- energy, thereby minimizing the po- Department of Ophthalmology,
rescein angiogram obtained imme- tential for thermal damage to the Mayo Clinic, Rochester, MN 55905
diately before the laser exposure. Al- overlying retina. (e-mail: robertson.dennis@mayo.edu).
though the central visual acuity was In this study, we were unable 1. Oosterhuis JA, Journee-de Korver JG, Kake-
reduced to 20/100 immediately af- to identify TTT-induced adverse ef- beeke-Kemme HM, Bleeker JC. Transpupillary
ter exposure, 5 days after TTT, the fects in the retina or the pigment epi- thermotherapy in choroidal melanomas. Arch
Ophthalmol. 1995;113:315-321.
central visual acuity had recovered thelium by means of clinical exami- 2. Journee-de Korver JG. Transpupillary Thermo-
to the pretreatment visual acuity. nation or fluorescein angiography. therapy: A New Treatment of Choroidal Mela-
We were unable to see a gray- However, we observed histological noma. The Hague, the Netherlands: Kugler
Publications; 1998.
ing of the retina after exposure to la- and ultrastructural abnormalities in 3. Shields CL, Shields JA, Cater J, et al. Trans-
ser energies with the same dosage that the tissue after the eye was enucle- pupillary thermotherapy for choroidal mela-
noma: tumor control and visual results in 100
ordinarily causes a graying (“take”) ated. We believe that these abnor- consecutive cases. Ophthalmology. 1998;105:
when directed to a choroidal mela- malities can be explained by the 581-590.
noma. The subtle edema of the macu- presence of the preexisting retinal 4. Robertson DM, Buettner H, Bennett SR. Transpu-
pillary thermotherapy as primary treatment for
lar retina in this case was similar to edema. However, some of the ob- small choroidal melanomas. Arch Ophthalmol.
the mild retinal edema frequently served abnormalities could have 1999;117:1512-1519.
seen with occult choroidal neovas- been caused by light alone, as shown 5. Reichel E, Berrocal AM, Ip M, et al. Transpupil-
lary thermotherapy of occult subfoveal choroi-
cular membranes. However, we do by Robertson and Erickson6 and dal neovascularization in patients with age-
not believe that the presence of reti- Green and Robertson.7 Although we related macular degeneration. Ophthalmology.
1999;106:1908-1914.
nal edema could explain the ob- looked for evidence of vascular clo- 6. Robertson DM, Erickson GJ. The effect of pro-
served absence of a take in the retina sure or coagulative necrosis in the longed indirect ophthalmoscopy of the human
and/or retinal pigment epithelium small capillaries in the choroid, we eye. Am J Ophthalmol. 1979;87:652-661.
7. Green WR, Robertson DM. Pathologic findings
during or 5 days after the laser ex- were unable to demonstrate such of photic retinopathy in the human eye. Am J
posure. This absence of a take ap- changes in the choroid in the foveo- Ophthalmol. 1991;112:520-527.

652

clearly separate from the optic nerve nent. Both the round cell and spindle
Malignant Peripheral (Figure 1) and had produced pres- cell components were positive for
Nerve Sheath Tumor sure erosion of the lateral orbital S100 protein and glial fibrillary
in the Orbit of a Child wall. It showed a homogeneous sig- acidic protein (GFAP) (Figure 6).
With Acute Proptosis
nal on T1 images with small cystic Test results for neuron-specific
areas on T2 scans. There was dif- enolase (NSE) were positive, pre-
fuse enhancement following con- dominantly in the small round cell
Malignant peripheral nerve sheath tu- trast, with focal areas of more promi- component. Test findings for neu-
mor (MPNST) is an extremely rare nent enhancement (Figure 2). rofilament, synaptophysin, chromo-
orbital tumor and only 34 cases have A medial transconjunctival in- granin, cytokeratin, CAM 5.2, epi-
previously been reported1-10 Of these, cisional biopsy of the tumor was per- thelial membrane antigen, desmin,
only 5 were childhood cases of formed and 4 pieces of tissue were and actin were all negative. (All an-
MPNST occurring in the orbit.3,4,7-9 sent for histologic evaluation. His- tibodies were provided by Dako, Ely,
We report a sixth case of this dis- tologic findings showed a biphasic Cambridgeshire, United King-
ease in the orbit of an 11-year-old girl tumor with a cellular small round dom.) Test results for the MIC-2 gene
who had rapidly progressive propto- blue cell component (Figure 3) product (Dako) and ␤2-microglobu-
sis and optic nerve compression. merging with a cellular spindle cell lin (Dako) were also negative.
component (Figure 4). There was Electron microscopy showed
Report of a Case. An 11-year-old girl no necrosis, but apoptotic cells of- that the cells in both the round cell
was referred with a 5-day history of ten were noted in the specimen. Al- and the spindle cell areas were simi-
rapidly progressive painless propto- though mitoses were infrequent lar. They contained a few cisternae
sis of the left eye and a corrected vi- (1/20 high-power field), some mor- of rough endoplasmic reticulum and
sual acuity that had decreased from phologically abnormal mitotic fig- mitochondria. The cytoplasmic ma-
6/5 to 6/12 over the previous 24 ures were present. The spindle- trix contained monoribosomes,
hours. On examination, the left eye shaped cells showed ill-defined polyribosomes, and focally abun-
was noted to have a 5-mm axial prop- cytoplasm and contained hyperchro- dant intermediate filaments; in the
tosis with a relative afferent pupil- matic, pleomorphic, serpiginous nu- round cell areas, cells in clusters
lary defect and a swollen optic disc. clei. The round cells showed little cy- were closely juxtaposed with no in-
Ocular motility was unrestricted and toplasm and contained moderately tervening matrix. Where the clus-
she did not complain of diplopia. pleomorphic nuclei with coarse hy- ters contacted stroma, cell surfaces
There was no palpable periorbital perchromatic chromatin and irregu- exhibited stretches of lamina, as well
mass and no periorbital neurosen- lar nuclear contours. Nucleoli were as bundles of slender collagen fibrils
sory deficit. She was otherwise inconspicuous. The round cell com- (Figure 7). Better developed lamina
healthy with no significant medical ponent contained rosettelike struc- was also present over spindle cells
history. A magnetic resonance im- tures with hypereosinophilic fibril- (Figure 8). These cells also had
aging (MRI) scan showed a large, lary cores (Figure 5). The overall long coarse cytoplasmic processes
ovoid, intraconal mass in the left or- appearances raised the differential containing relatively large num-
bit that extended from the orbital diagnosis of spindle cell neuroblas- bers of intermediate filaments and
apex to the posterior aspect of the toma, primitive neuroectodermal tu- fewer membranous organelles. No
globe. There was no evidence of ex- mor (PNET), neuroepithelioma, and glycogen, neuroendocrine gran-
tra-orbital extension. The mass was MPNST with a PNET-like compo- ules, processes containing microtu-
Figure 1. T1-weighted precontrast axial magnetic resonance image Figure 2. T1-weighted postcontrast coronal magnetic resonance image
demonstrating a well-defined, homogeneous, intraconal mass in the left demonstrating diffuse enhancement of the mass with focal peripheral
orbit. The mass is clearly separate from the optic nerve and displaces it enhancement.
superomedially.

653

Figure 3. Section of tumor showing a primitive neuroectodermal tumor–like Figure 6. Glial fibrillary acidic protein immunohistochemistry (original
area with a mitotic figure (hematoxylin-eosin, original magnification ⫻400). magnification ⫻160).
Figure 4. Section of tumor showing a spindle cell region with nuclear Figure 7. Electron micrograph showing primitive neuroectodermal
palisading (hematoxylin-eosin, original magnification ⫻160). tumor–like and spindle cell components (original magnification ⫻16000).
Figure 5. Section of tumor showing spindle cells merging with the primitive Figure 8. Electron micrograph showing reduplicated basal lamina (original
neuroectodermal tumor–like component. Some of the primitive magnification ⫻33 000).
neuroectodermal tumor–like structures showed eosinophilic fibrillary cores
(hematoxylin-eosin, original magnification ⫻160).
bules, or bundles of striated muscle Comment. Thirty-four cases of After confirmation from anterior or-
myofilaments were seen. MPNST of the orbit have been re- bital biopsy results at age 5 days, he
The patient underwent left or- ported.1-10 Seventeen arose from the underwent an exenteration at age 6
bital exenteration with a complete trigeminal nerve, mainly from its su- weeks and has been reported to be
apical clearance, and 6 weeks later praorbital branch. 3 Five previ- tumor free at age 27 months.8 One
she received postoperative radio- ously reported tumors occurred in tumor in the orbit of a 50-day-old
therapy. She has since made a com- children. The youngest reported pa- male infant showed features of a
plete recovery with no recurrence or tient, born with Kartagener syn- plexiform MPNST.4 Despite orbital
metastatic disease after 4 years’ fol- drome, was 4 days old when the tu- exenteration and radiotherapy, he
low-up. mor was detected on an MRI scan. died within 6 months of diagnosis.

654

The third child had previously re- hood malignancies, and complete 10. Gray NW, Rosenberg AE, Dickersin GR, Bhan
AK. Glial fibrillary acidic protein and keratin ex-
ceived radiotherapy for bilateral reti- surgical excision is essential to pro- pression by benign and malignant nerve sheath
noblastomas and developed an ana- vide a chance of cure. Our case tumours. Hum Pathol. 1989;20:1089-1096.
plastic sarcoma arising in an orbital showed an unusually rapid progres-
neurofibroma.3 The fourth child, a sion with acute presentation of prop-
15-month-old boy, underwent lat- tosis and optic nerve compression.
eral orbitotomy with cryoexcision of Immunohistochemistry showed the Ocular Findings in
a retrobulbar MPNST in association presence of GFAP although reports Spinocerebellar Ataxia 7
with a myxoid neurofibroma. Histo- of GFAP positivity are exceptional
logically the MPNST pseudocapsule in MPNSTs. A poorly differentiated Spinocerebellar ataxia (SCA) 7, also
was intact and 9 years later the child PNET-like small cell component was known as autosomal dominant cere-
was still disease free.9 The fifth case also present. bellar ataxia (ADCA) type II or olivo-
was diagnosed in a 23-year-old man pontocerebellar atrophy with reti-
who had rapid-onset orbital propto- Daniel Briscoe, MB LRCP&SI, BAO nal degeneration, is one of at least
sis and pain in the left eye.7 Biopsy Kfar Saba, Israel 14 genetically distinct forms of he-
specimens taken at the time were S. Mahmood, MRCOphth reditary SCA. All of these forms are
compared with specimens from an in- Manchester, England characterized by variable degenera-
complete excision of the tumor taken D. G. O’Donovan, MD, FRCPath tion of the cerebellar cortex, the basal
when he was 5 years old and that was Cambridge, England ganglia, the brainstem, the spinal
previously diagnosed as fibromato- cord, and the peripheral nerves. Prior
R. E. Bonshek, MD, FRCPath
sis. Both specimens were found to be to the identification of the caus-
B. Leatherbarrow, FRCS, FRCOphth
MPNST. He underwent orbital ex- ative genes, ADCAs were divided
B. P. Eyden, PhD
enteration and was reported to be into 3 subtypes.1 In ADCA type I,
Manchester
alive without recurrence 8 months cerebellar ataxia is associated with
later. This was 18 years after initial ophthalmoplegia, optic atrophy, ex-
tumor presentation and is the long- We wish to thank Roger Lait, MRCP, trapyramidal signs, and dementia.
est reported survival. FRCR, for providing the MR scans and Patients with ADCA type II de-
The current case clearly showed Pat Tarpey for electron microscopy. velop retinal degeneration and cer-
features of a rapidly evolving malig- Corresponding author: B. Leath- ebellar ataxia. Ophthalmoplegia, ex-
nant neoplasm: rapid growth, marked erbarrow,FRCS,FRCOphth,RoyalEye trapyramidal signs, and dementia are
cellularity, mitotic activity, and apop- Hospital, Oxford Road, Manchester variably present. Autosomal domi-
totic figures, and abnormal mitoses M13 9WH, England (e-mail: bollin nant cerebellar ataxia type III is de-
indicated its malignant potential. The @mighty-micro.co.uk). scribed as a “pure” cerebellar syn-
ultrastructure indicated schwann- 1. Jakobiec FA, Font RL, Zimmerman LE. Malig-
drome. All 3 ADCA types are
ian differentiation that supported a nant peripheral nerve sheath tumors of the or- genetically heterogeneous. Almost
diagnosis of MPNST. In addition, the bit: a clinicopathologic study of eight cases. all of ADCA type II cases are due to
Trans Am Ophthalmol Soc. 1985;83:332-366.
round blue cell component of the tu- 2. Erzurum SA, Melen O, Lissner G, et al. Orbital mutations in the SCA7 locus; thus,
mor showed no immunoreactivity for malignant peripheral nerve sheath tumors: treat- SCA7 is unique in that it is the only
synaptophysin, MIC-2 gene prod- ment with surgical resection and radiation SCA invariably associated with reti-
therapy. J Clin Neuroophthalmol. 1993;13:1-7.
uct, or ␤2-microglobulin, and elec- 3. McLean IW, Burnier MN, Zimmerman LE, Ja- nal degeneration. Here, we de-
tron microscopy showed it was de- kobiec FA. Tumors of the Eye and Ocular Ad- scribe the ocular findings in a pa-
nexa. Washington, DC: Armed Forces Insti-
void of neuroendocrine granules and tute of Pathology; 1994. Atlas of Tumor
tient diagnosed after postmortem
processes containing microtubules. Pathology; 3rd ser, pt 12:233-298. examination as having had SCA7.
All of these features exclude the di- 4. Meis-Kindblom JM, Enzinger FM. Plexiform
malignant peripheral nerve sheath tumour of
agnoses of neuroblastoma, neuro- infancy and childhood. Am J Surg Pathol. 1994; Report of a Case. A 17-year-old
epithelioma, and true PNET.6 Meis 18:479-485. black male died of aspiration pneu-
et al5 reported on PNET-like foci in 5. Meis JM, Enzinger FM, Martz KL, Neal JA. Ma- monia and a urinary tract infec-
lignant peripheral nerve sheath tumours (ma-
15 cases of nonorbital childhood lignant Schwannomas) in children. Am J Surg tion. No notable developmental or
MPNSTs that closely resemble those Pathol. 1992;16:694-707. medical problems were evident un-
6. Banerjee SS, Agbamu DA, Eyden BP, Harris M.
in the current case. They found that Clinicopathological characteristics of periph-
til the age of 8 years when he was
PNET-like foci do not adversely eral primitive neuroectodermal tumour of skin noted to have poor vision. He was
affect the prognosis. They also de- and subcutaneous tissue. Histopathology. 1997; diagnosed with retinitis pigmen-
31:355-366.
termined that 56% of MPNSTs were 7. Morton AD, Elner VM, Frueh B. Recurrent or- tosa at age 9.5 years and was legally
positive for S100 protein.5 The pres- bital malignant peripheral nerve sheath tumor blind (20/300 OD and 20/400 OS)
ence of GFAP in MPNSTs is uncom- 18 years after initial resection. Ophthalmic Plast by age 11 years (Figure 1). At age
Reconstr Surg. 1997;13:239-243.
mon, but Gray et al10 reported GFAP 8. Fezza JP, Wolfley DE, Flynn SD. Malignant pe- 10 years, he started to have diffi-
reactivity in 2 cases in their series. ripheral nerve sheath tumor of the orbit in a culty walking. He had full muscle
newborn: a case report and review. J Pediatr
In conclusion, MPNSTs should Ophthalmol Strabismus. 1997;34:128-131.
strength but poor coordination and
be considered in the differential di- 9. Eviatar JA, Hornblass A, Herschorn B, Jako- dysmetric finger-to-nose and knee-
agnosis of malignant orbital tu- biec FA. Malignant peripheral nerve sheath tu- to-chin movements. Speech was
mor of the orbit in a 15-month-old child: nine-
mors in children. This disease has year survival after local excision. Ophthalmology. slow and dysarthric. There was lim-
a poorer prognosis than most child- 1992;99:1595-1599. ited upward gaze, limited adduc-

655

quiring assisted ventilation, espe-
cially at night. He had 15 hospital ad-
missions in the last year of life for
respiratory distress, aspiration pneu-
monia, and episodes of lethargy and
unresponsiveness. One month prior
to death, the patient was admitted
to the hospital with seizure activity
characterized by jerking of the head,
both arms, and right leg. A head
computed tomography scan re-
vealed no focal lesions. He was
treated for presumed status epilep-
ticus, after which an electroencepha-
logram showed findings suggestive
of diffuse cortical dysfunction but no
evidence of seizure activity. He de-
veloped a left lower lobe pulmo-
Figure 1. Fundus photograph of the right eye at age 11 years shows attenuated retinal vessels, diffuse nary infiltrate and a urinary tract in-
areas of hypopigmentation of the retinal pigment epithelium, and severe atrophy of the retinal pigment
epithelium in the macula. fection. Given the patient’s poor
neurologic and pulmonary status,
mechanical respiratory support was
discontinued and the patient died the
following day.
The right globe and central ner-
vous system were obtained at au-
topsy. On gross examination, the reti-
nal pigment epithelium (RPE) had a
mottled appearance throughout the
fundus (Figure 2). Ciliary epithe-
lial cysts containing acellular eosino-
philic material were present on mi-
croscopic examination (Figure 3)
but were not appreciated grossly. The
ganglion cell layer of the retina ap-
peared normal. There was diffuse
photoreceptor degeneration that ap-
peared more severe in the posterior
pole than in the periphery. There
were very rare intraretinal pigment
deposits and areas with subretinal
Figure 2. Postmortem gross examination of the left eye showing a mottled retinal pigment epithelium.
proteinaceous material. Patches of
atrophic RPE were intermixed with
tion, dysconjugate gaze, and bilat- plex I and normal ETC complexes II, hyperpigmented and hypertrophic
eral ptosis. There was no family III, and IV. No deletions of mitochon- RPE cells (Figure 4).
history of relevant ocular or neuro- drial DNA were detected by South- Immunohistochemical stain-
logic diseases. ern blot analysis; no sequence analy- ing with an antibody against ubi-
Electrocardiogram results sis was performed to search for point quitin, a component of many types
showed no evidence of cardiac con- mutations in the mitochondrial ge- of inclusions, revealed rare intra-
duction block. Electroencephalo- nome. Nevertheless, the patient car- nuclear inclusions in the inner and
gram findings were normal. Mag- ried the diagnosis of an atypical form outer nuclear layers and the gan-
netic resonance imaging of the head of mitochondrial disease, such as a glion cell layer (Figure 5). The
revealed cerebellar atrophy, a di- variant of Kearns-Sayre syndrome or ubiquitin-positive inclusions were
lated fourth ventricle, and thin but myoclonic epilepsy and ragged red fi- either round and compact or dif-
otherwise normal-appearing optic ber disease/progressive external oph- fuse. We were unable to convinc-
nerves. At age 12 years, a muscle bi- thalmoplegia. ingly detect these inclusions with an
opsy specimen showed no ragged red By age 13 years, the patient was antibody against expanded polyglu-
fibers with the Masson trichrome confined to a wheelchair. He began tamine repeats. However, electron
stain and no abnormal mitochon- to have myoclonic seizures. A per- microscopy of the retina confirmed
dria by electron microscopy. Elec- cutaneous gastrostomy tube was the presence of 2 distinct types of in-
tron transport chain (ETC) analysis placed for feeding. At age 16 years, tranuclear inclusions (Figure 6).
revealed a partial defect in ETC com- the patient had respiratory failure re- The first type of inclusion was round,

656

compact, and predominantly fila- The brain weighed 950 g (nor- active gliosis. There was severe atro-
mentous, while the second type was mal for an adult male is 1350-1450 phy of the cerebellum with gliosis and
larger and more diffuse with granu- g). The cerebrum was mildly atro- only rare Purkinje cells (Figure 7).
lar and filamentous structures. phic and microscopically showed re- Severe gliosis and neuronal loss were
also found in the brainstem and the
spinal cord in a pattern consistent
with SCA.
Molecular analysis of the SCA7
gene showed that the patient was a
heterozygote, with 1 allele having ap-
proximately 12 CAG repeats and the
other having 70 to 87 repeats (nor-
mal, ⱕ36 repeats) (Figure 8). DNA
from the patient’s parents was not
available.
Comment. This case is noteworthy

because the diagnosis of SCA7 was
made only after postmortem exami-
nation. The patient had a prior di-
agnosis of an atypical mitochon-
drial disorder because the clinical
symptoms overlapped with those
found in mitochondrial myopa-
Figure 3. Ciliary epithelial cysts contain acellular eosinophilic material (hematoxylin-eosin; thies and because of an abnormal
original magnification ⫻40). ETC complex I analysis. The signifi-
A B
Figure 4. Severe photoreceptor degeneration in the posterior pole of the retina (A) with very rare intraretinal pigment deposits (A and B). Patches of atrophic
retinal pigment epithelium (RPE) are intermixed with hyperpigmented and hypertrophic RPE cells (A and B) (hematoxylin-eosin; original magnifications ⫻100 and
⫻400, respectively).
A B
Figure 5. Retinal intranuclear inclusions containing ubiquitin. Immunohistochemical staining with an antibody against ubiquitin reveals a round, compact,
intranuclear inclusion in the inner nuclear layer (A) and a more diffuse, intranuclear inclusion in the ganglion cell layer (B). Intranuclear inclusions are designated
by arrows. Immunohistochemistry was performed on formalin-fixed tissue (original magnification ⫻1000).

657

cance of the abnormal ETC com- mine residues is encoded by the rep- + pt –
plex I analysis is uncertain. etition of the sequence CAG in the
Spinocerebellar ataxia 7 was not coding region of the gene. The num-
seriously considered, possibly be- ber of CAG repeats (and the en-
cause of the absence of a positive fam- coded polyglutamine tract) varies nor-
ily history. Like other dominant neu- mally from 7 to 17 repeat units. Rarely
rodegenerative disorders, such as and for obscure reasons, an allele with
Huntington disease, SCA7 shows 18 to 35 repeats will arise. Individu-
strong anticipation: the age of onset als with these intermediate alleles are
and disease severity increase with each usually asymptomatic. However, once
–87
successive generation. The molecu- the repeat has expanded, it is prone
lar basis for anticipation in SCA7 is to expand even further during parent-
the expansion of a polyglutamine tract to-child transmission and especially
in ataxin 7, the protein product of the during father-to-child transmis-
SCA7 gene.2 This stretch of gluta- sion.3 Individuals affected with SCA7
–70
–15
–12
Figure 8. Molecular genetic analysis of the

patient’s DNA purified from a fragment of unfixed,
frozen liver. The portion of the SCA7 gene
containing the polyglutamine region was amplified
by polymerase chain reaction (PCR). The
PCR-amplified DNA products were separated by
denaturing gel electrophoresis. Size was
determined by comparing the migration of the
PCR-amplified DNA products with a DNA
sequence ladder of known size (not shown). DNA
from a patient known to have spinocerebellar
c ataxia (SCA) 7 is the positive control (+). DNA
from an unaffected individual is the negative
control (−). DNA from our patient is in the middle
lane (pt). One allele of the SCA7 gene in our
patient contains expanded CAG repeats (70-87
repeats). The second allele is wild type with
Figure 6. An electron micrograph shows 2 intranuclear inclusions in a retinal cell. One is compact and approximately 12 repeats. Multiple bands are due
predominantly filamentous (arrow). The second is larger and more diffuse with granular and filamentous to “stuttering” of the polymerase as it replicates
structures (surrounded by arrowheads). n indicates the nucleus; c, cytoplasm. Electron microscopy was the polyglutamine region. Numbers along the right
performed on glutaraldehyde-fixed tissue stained with lead citrate. Scale bar indicates 1 µm. side of the gel indicate the number of CAG repeats.
A B
p
p
ig
ig
Figure 7. A normal cerebellar cortex (A) compared with the cerebellar cortex in the patient in this report with spinocerebellar ataxia (SCA) 7 (B). There is severe
atrophy of the cerebellar cortex in the patient with SCA7, with loss of Purkinje cells (p) and internal granule cells (ig). There are no identifiable Purkinje cells
in most sections of the cerebellum; this section is one of the few with a remaining Purkinje cell (seen in the center of the field) (hematoxylin-eosin;
original magnification ⫻40).

658

carry an allele with 37 to 200 repeat ever, in SCA7, intranuclear inclu- 1. Harding AE. The clinical features and classifi-
cation of the late onset autosomal dominant cer-
units. Thus, one possible explana- sions are not restricted to regions af- ebellar ataxias. Brain. 1982;105:1-28.
tion for why this patient did not have fected by the disease.14 These findings 2. David G, Abbas N, Stevanin G, et al. Cloning of
a positive family history is that one suggest that the inclusions may be the SCA7 gene reveals a highly unstable CAG re-
peat expansion. Nature Genet. 1997;17:65-70.
parent was an asymptomatic carrier necessary but not sufficient to cause 3. David G, Durr A, Stevanin G, et al. Molecular and
of an intermediate allele, and the cell dysfunction and death. clinical correlations in autosomal dominant cer-
ebellar ataxia with progressive macular dystro-
pathologic expansion of the repeat oc- In this study, we described the phy (SCA7). Hum Mol Genet.1998;7:165-170.
curred between generations. ultrastructural appearance of 2 types 4. Neetens A, Martin JJ, Libert J, van den Ende P.
Retinal degeneration is always of intranuclear inclusions in the retina Autosomal dominant cone dystrophy-
cerebellar atrophy (ADCoCA) (modified ADCA
present in patients with SCA7. The of a patient with SCA7. The diffuse, Harding II). Neuro-ophthalmology. 1990;10:
onset of visual symptoms may pre- granulofilamentous inclusions re- 261-275.
semble the inclusions found in the 5. Abe T, Tsuda T, Yoshida M, et al. Macular de-
cede or follow the onset of neuro- generation associated with aberrant expan-
logic symptoms. The disease affects retina of one patient with early- sion of trinucleotide repeat of the SCA7 gene
cone function more severely than rod onset SCA7 described by Mauger et in 2 Japanese families. Arch Opthalmol. 2000;
118:1415-1421.
function. Patients never complain of al.10 Similar granulofilamentous in- 6. Giunti P, Stevanin G, Worth PF, David G, Brice
night blindness. The first visual com- clusions have been found in patients A, Wood NW. Molecular and clinical study of
plaint is usually reduced central vi- with Huntington disease and in mice 18 families with ADCA type II: evidence for ge-
netic heterogeneity and de novo mutation. Am
sual acuity or abnormal color vi- hemizygous for a mutant form of hu- J Hum Genet. 1999;64:1594-1603.
sion.4,5 Electroretinograms document man huntingtin (hd).11,15 The com- 7. Babovic-Vuksanovic D, Snow K, Patterson MC,
pact, filamentous inclusions in our Michels VV. Spinocerebellar ataxia type 2 (SCA
that cone function is more severely 2) in an infant with extreme CAG repeat ex-
impaired than rod function. Fun- SCA7 case differ from those de- pansion. Am J Med Genet. 1998;79:383-387.
dus examination early in the disease scribed by Mauger et al10 and most 8. To KW, Adamian M, Jakobiec FA, Berson EL.
Olivopontocerebellar atrophy with retinal de-
shows atrophy of the RPE in the closely resemble the amyloidlike generation. An electroretinographic and histo-
macula; later there is attenuation of structures observed in mice homo- pathologic investigation. Ophthalmology. 1993;
retinal vessels and a mottled RPE zygous for the hd mutation.16 As in the 100:15-23.
9. Martin JJ, van Regemorter N, Krols L, et al. On
throughout the fundus. There is little SCA7 case reported by Mauger et al, an autosomal dominant form of retinal-
or no intraretinal pigmentation of the the retinal intranuclear inclusions in cerebellar degeneration: an autopsy study of five
our patient contained ubiquitin and patients in one family. Acta Neuropathol (Berl).
sort that is ordinarily seen in retini- 1994;88:277-286.
tis pigmentosa. were present in the inner and outer 10. Mauger C, Del-Favero J, Ceuterick C, Lobke U,
Most but not all patients with nuclear layers as well as the gan- van Broeckhoven C, Martin J-J. Identification
and localization of ataxin-7 in brain and retina
ADCA type II harbor mutations in the glion cell layer. Our data support the of a patient with cerebellar ataxia type II using
SCA7 gene. Giunti et al6 identified finding of Mauger and coworkers10 anti-peptide antibody. Mol Brain Res. 1999;74:
one family with ADCA type II that did that intranuclear inclusions in the 35-43.
11. DiFiglia M, Sapp E, Chase KO, et al. Aggrega-
not have CAG expansion in the SCA7 retina are not restricted to the neu- tion of Huntingtin in neuronal intranuclear in-
gene and no linkage to the SCA7 lo- ronal population that degenerates. clusions and dystrophic neurites in brain. Sci-
To our knowledge, no previ- ence. 1997;277:1990-1993.
cus, indicating genetic heterogene- 12. Skinner PJ, Koshy BT, Cummings CJ, et al.
ity. Furthermore, Babovic-Vuksa- ous report of the ocular pathology of Ataxin-1 with an expanded glutamine tract
novic et al7 reported a case of SCA presumed SCA7 disease mentions alters nuclear matrix-associated structures.
Nature.1997;389:971-974.
with retinal degeneration in an in- ciliary epithelial cysts. Such cysts con- 13. Paulson HL, Perez MK, Trottier Y, et al. Intra-
fant who died with a severe form of taining proteinaceous material, as nuclear inclusions of expanded polygluta-
SCA2. These rare cases emphasize the found in our case, are considered a mine protein in spinocerebellar ataxia type 3.
Neuron. 1997;19:333-344.
importance of molecular genetic specific feature of multiple my- 14. Holmberg M, Duyckaerts C, Durr A, et al. Spi-
analysis to establish the diagnosis. eloma and other hypergammaglobu- nocerebellar ataxia type 7 (SCA7): a neurode-
linemic conditions,17 which were not generative disorder with neuronal intranuclear
The ocular pathologic characteris- inclusions. Hum Mol Genet. 1998;7:913-918.
tics of 2 cases of ADCA type II de- present in this case. 15. Davies SW, Turmaine M, Cozens BA, et al. For-
scribed by To et al8 and Martin et al9 mation of neuronal intranuclear inclusions un-
derlies the neurological dysfunction in mice
are now known to be from families Margaret E. McLaughlin, MD transgenic for the HD mutation. Cell. 1997;90:
with SCA7 gene defects determined Thaddeus P. Dryja, MD 537-548.
by DNA analysis (Eliot Berson, MD, Boston, Mass 16. Scherzinger E, Lurz R, Turmaine M, et al. Hun-
tingtin-encoded polyglutamine expansions form
oral communication, 1999, and amyloid-like protein aggregates in vitro and in
Mauger et al,10 respectively). This work was supported by grant vivo. Cell. 1997;90:549-558.
17. Johnson BL, Storey JD. Proteinaceous cysts of
Neuronal intranuclear inclu- EY08683 from the National Insti- the ciliary epithelium. Arch Ophthalmol. 1970;
sions are a common feature of tutes of Health, Bethesda, Md. 84:166-175.
diseases related to polyglutamine Dr McLaughlin is a Howard
expansion. In Huntington disease, Hughes Medical Institute Postdoc-
SCA1, and SCA3, intranuclear in- toral Fellow. Acquired Retinal
clusions develop mainly in neu- Corresponding author and Myelination in
rons from regions affected by the dis- reprints: Margaret E. McLaughlin, Neurofibromatosis 1
ease,11-13 leading to the hypothesis MD, Children’s Hospital, Depart-
that the formation of intranuclear in- ment of Pathology, 300 Longwood Myelination of the anterior visual
clusions is an important step in the Ave, Boston, MA 02115 (e-mail: pathways begins centrally at the
neurodegenerative process. How- memclaughlin@partners.org). lateral geniculate body and is com-

659

Figure 1. Swollen optic disc with opticociliary shunts (case 1). Figure 2. Pale optic disc with myelinated nerve fibers (case 1).
Figure 3. Magnetic resonance imaging scan showing optic nerve glioma (case 2). Figure 4. Acquired myelination adjacent to optic disc (case 2).
pleted at term. This process normal- duced vision and optic disc swelling stable at 20/30 OD, and the MRI find-
ly terminates at the lamina cribrosa, in the right eye. There was a mater- ings were unchanged. The retinal fea-
but it occasionally may extend into nal family history of NF1, and he had tures have remained unchanged for
and beyond the peripapillary reti- cutaneous features of NF1 and Lisch 4 years.
nal nerve fiber layer. Congenital reti- nodules. The right eye was proptosed, Case 2. An 8-year-old boy was
nal myelination is not uncommon, with optic disc edema, opticociliary referred with reduced vision and a
occurring in 0.3% to 0.6% of the shunt vessels, and a relative afferent pale left optic disc. He had features of
population as an isolated develop- pupil defect (RAPD) (Figure 1). Cor- segmental NF1, with café-au-lait spots
mental anomaly, or rarely as part of rected visual acuity was reduced to on the left chest wall and the imag-
a generalized disorder, such as the 20/80 OD, and a magnetic resonance ing features of a left optic nerve glioma
Goltz-Gorlin syndrome, or the syn- imaging (MRI) scan confirmed the extending to the optic nerve head on
drome of anisometropia high myo- presence of an optic nerve glioma. Vi- MRI scan (Figure 3). Corrected vi-
pia, and amblyopia.1 Although re- sual field testing, which was reliably sual acuities were 20/15 OD and 20/30
cent evidence suggests that the repeatable in this child, showed an OS with left optic atrophy. A full sys-
retinal myelination may progress in enlarged blind spot and peripheral temic evaluation was performed, but
such cases,2 acquired retinal my- constriction on the right side. No no treatment was initiated. Visual acu-
elination is exceptionally rare.3 treatment was initiated, and annual ity remained stable during the next 2
We describe 2 cases in which follow-up was arranged. years, and then gradually improved
myelinated retinal nerve fibers ap- Within a year, visual acuity to 20/20 OS. Five years after the ini-
peared in children with neurofibro- 20/30 OD, the RAPD resolved, and tial visit, he had developed segmen-
matosis 1 (NF1) and optic nerve the visual field enlarged. The disc tal myelination of the peripapillary
glioma. In both cases, spontaneous edema resolved, and the shunt ves- retinal nerve fibers in the left eye
improvement in visual function pre- sels disappeared. (Figure 4). These findings have re-
ceded the retinal myelination. Four years after the initial visit, mained unchanged for 2 years.
examination showed myelinated
Report of Cases. Case 1. An 8-year- nerve fibers adjacent to the right op- Comment. Myelin is deposited in the
old boy was referred to us with re- tic disc (Figure 2). Visual acuity was central nervous system by oligoden-

660

drocytes. The 2 major glial cells of but to axonal remyelination by oli- better than 20/50 was observed in
the central nervous system (the type godendrocytes locally reactivated by only 1% of eyes that had a macular
2 astrocyte and the oligodendro- the same process that triggered the hole larger than 400 µm with no pos-
cyte) are derived from stem cells optic glioma. If the lamina cribrosa terior vitreous detachment. We re-
known as oligodendrocyte precur- is also disrupted, remyelination port a case with large bilateral macu-
sor cells, which actively produce my- could extend into the retina, with the lar holes, 1800 µm in vertical diameter
elin prenatally. Myelination ceases process stopping, as the stem cell in the right eye and 1500 µm in the
at birth as the oligodendrocytes ma- proliferation is self-limiting.4 left eye. The visual acuity was sur-
ture into adult type cells and the bio- These 2 cases, therefore, pro- prisingly good, ie, 20/40 OU, and the
chemical stimulus for myelination vide an insight into the complex dy- stereoacuity was 50 seconds with the
(notably platelet-derived growth fac- namic of focal tissue growth disor- Titmus stereo test. The possible rea-
tor) is withdrawn.4 ders in NF1. Tumor growth is not son for this good visual outcome is
The mechanisms of termina- exponential, but modified by focal discussed.
tion of the myelination at the lamina repair mechanisms.
cribrosa are not clearly under- Report of a Case. A 33-year-old
stood. This process is important, as Manoj V. Parulekar, FRCS woman was referred to our depart-
opaque nerve fibers would affect vi- John S. Elston, BSc, MD ment with bilateral macular holes.
sion. Regulatory proteoglycans have Oxford, England She had first noticed a small sco-
been shown to determine where the toma about 2 years previously and
retinal ganglion cell body ends and Corresponding author and reprints: experienced a gradual decrease in vi-
the axon begins. This ganglion- John S. Elston, BSc, MD, Radcliffe In- sual acuity. She had no personal or
axon junction is at the level of the firmary, Oxford Eye Hospital, Wood- family history of ocular disease and
lamina cribrosa in the human optic stock Road, Oxford OX2 6HE, En- did not use any medications.
nerve. Myelination is limited to the gland (e-mail: Mary.Spearman@orh Her best-corrected visual acu-
axon, and therefore does not ex- .anglox.nhs.uk). ity at the time of her first visit was
tend beyond the lamina cribrosa.5 20/40 OD (−5.5 diopters [D] sphere)
1. Lee MS, Gonzalez C. Unilateral peripapillary my-
Certain animals such as rab- elinated retinal nerve fibers associated with stra-
and 20/40 OS (−5.0 D sphere). Her
bits lack a lamina cribrosa, and my- bismus, amblyopia, and myopia. Am J Ophthal- best stereoacuity was 50 seconds ac-
elinated retinal nerve fibers are a nor- mol. 1998;126:853. cording to the Titmus stereo test. Fun-
2. Ali BH, Logani S, Kozlov KL, Arnold AC, Bate-
mal feature of their eyes. The lamina man B. Progression of retinal nerve fiber my- dus examination showed a large
cribrosa may therefore act as a physi- elination in childhood. Am J Ophthalmol. 1994; macular hole in each eye with verti-
118:515-517.
cal barrier to keep oligodendro- 3. Jean-Louis G, Katz BJ, Digre KB, Warner JE,
cal diameters of 1800 µm OD and
cytes out of the retina. Creger DD. Acquired and progressive retinal 1500 µm OS (Figure 1). The retina
There is experimental evi- nerve fiber layer myelination in an adolescent. partially bridged the hole in her right
Am J Ophthalmol. 2000;130:361-362.
dence of a physical barrier at the ocu- 4. Tang DG, Tokumoto YM, Raff MC. Long-term
eye. No posterior vitreous detach-
lar end of the rat optic nerve, and a culture of purified postnatal oligodendrocyte pre- ment was observed in either eye.
similar mechanism has been postu- cursor cells: evidence for an intrinsic matura- Fluorescein angiography showed a
tion program that plays out over months. J Cell
lated in humans.6 Biol. 2000;148:971-984. window defect corresponding to the
Myelination could occur post- 5. Perry VH, Lund RD. Evidence that the lamina hole. Microperimetry images ob-
natally if the barrier were disrupted cribrosa prevents intraretinal myelination of reti- tained by scanning laser ophthalmos-
nal ganglion cell axons. J Neurocytol. 1990;19:
and/or the oligodendrocytes were 265-272. copy showed that the fixation points
stimulated. The barrier may be di- 6. French-Constant C, Miller RH, Burne JF, Raff were at the upper edges of the macu-
MC. Evidence that migratory oligodendrocyte-
rectly distorted by the tumor as in type-2 astrocyte (O-2A) progenitor cells are kept
lar holes in both eyes (Figure 2). Op-
case 2, or disrupted by disc edema out of the rat retina by a barrier at the eye-end tical coherence tomography demon-
in case 1. Previously described cases of the optic nerve. J Neurocytol. 1988;17:13-25. strated a detailed structure around
7. Lau N, Feldkamp MM, Roncari L, et al. Loss of
of acquired retinal myelination have neurofibromin is associated with activation of the holes, the edges of which were
occurred following resolution of RAS/MAPK and PI3-K/AKT signaling in a neu- swollen inferiorly in the right eye
papilloedema2 or associated with op- rofibromatosis 1 astrocytoma. J Neuropathol Exp (Figure 3A) and inferiorly and su-
Neurology. 2000;59:759-767.
tic disc drusen,3 where there would 8. Parsa CF, Hoyt CS, Lesser RL, et al. Spontane- periorly in the left eye (Figure 3B).
be disruption of the lamina cribrosa. ous regression of optic gliomas: thirteen cases Both eyes retained similar visual func-
documented by serial neuroimaging. Arch Oph-
Stem cell proliferation in NF1 thalmol. 2001;119:516-529.
tion during a 7-month observation
occurs due to local inactivation of a period.
tumor supressor gene.7 In the ante-
rior visual pathway, this process pro- Comment. Eyes with stage 3 macu-
duces optic glioma—characteristi- Bilateral Giant Macular lar holes tend to have progressive de-
cally an indolent tumour. It is Holes With Excellent terioration of visual acuity before the
recognized that visual function in Visual Function vision stabilizes at 20/200 or worse.2
optic gliomas can improve sponta- A previous report indicated that vi-
neously, with improvement in the Eyes with large macular holes have sual acuity better than 20/50 is ob-
scan appearance. 8 The improve- poor visual acuity and poor visual served in only 1% of eyes that had a
ment in vision in this case was most function in general. A previous re- macular hole larger than 400 µm with
likely not due to tumor regression, port1 indicated that visual acuity of no posterior vitreous detachment.1

661

A
Figure 1. Color fundus photographs. A, The

macular hole in the right eye is 1800 µm in
vertical diameter and the retina partially bridges
the hole. B, The hole in the left eye is 1500 µm
B in vertical diameter.
A B
Figure 2. Microperimetry images obtained by scanning laser ophthalmoscopy. The crosses represent fixation points in the right (A) and the left (B) eyes. The
fixation points are at the upper edges of the macular holes for eccentric fixation. The open squares indicate a dense scotoma at that part of the hole; the white
squares indicate the points at which the stimulus was recognized.

662

A B
Inferior Superior Inferior Superior
Figure 3. Optical coherence tomography in vertical scan. The edges of the macular hole are swollen inferiorly in the right eye (A) (arrow) and inferiorly and
superiorly in the left eye (B) (arrows).
Our patient had surprisingly good vi- increased in upward gaze to 40 PD

sual acuity compared with that of Primary Aberrant and the exotropia increased to 12 PD.
patients with smaller holes. More- Oculomotor Nerve The left upper eyelid retracted in
over, bilateral fixation points were at Regeneration From a downward gaze and adduction (pseu-
almost the same locus in each eye, so Posterior Communicating do–von Graefe sign). Slitlamp exami-
that each eye could have steady fixa- Artery Aneurysm nation, tonometry, and ophthalmo-
tion with that locus. scopic examination results were
Although the cause of our pa- normal.
tient’s excellent visual function in- Primary aberrant regeneration or Since these findings suggested
cluding visual acuity and stereoa- oculomotor nerve synkinesis is a rare aberrant regeneration of the oculo-
cuity was uncertain, we assumed that condition in which cranial nerve III motor nerve in her left eye, mag-
the macular hole function was re- regeneration occurs without a pre- netic resonance imaging and mag-
lated to the fixation loci and stabil- ceding, acute palsy. It has been typi- netic resonance angiography of the
ity. We theorize that the bilateral en- cally associated with aneurysms or brain were performed. The results
largement of the holes and the meningiomas in the cavernous si- revealed an enhancing lesion in the
shifting of the fixation points oc- nus that do not warrant urgent im- interpeduncular cistern, most likely
curred simultaneously and gradu- aging.1 We describe a patient with impinging the left oculomotor nerve
ally in our patient. Because of this, primary aberrant regeneration (Figure 2). Results of cerebral cath-
we believe, our patient retained good caused by an aneurysm located at the eter angiography confirmed this to
visual function. junction of the left posterior com- be a partially thrombosed aneu-
municating (PCOM) and posterior rysm at the junction of the left
Atsushi Takahashi, MD cerebral arteries, indicating that im- PCOM and posterior cerebral arter-
Masumi Takeda, MD, PhD aging should not be delayed. ies (Figure 3). After neurosurgi-
Norihiko Kitaya, MD, PhD cal consultation, it was decided that
Junichi Takahashi, MD Report of a Case. A 65-year-old the patient receive follow-up with-
Suguru Konno, MD, PhD woman complained of right eyelid out surgery.
Satoshi Ishiko, MD, PhD ptosis for 3 years. She denied dip-
Taiichi Hikichi, MD, PhD lopia. Her medical history was un- Comment. Aberrant regeneration oc-
Akitoshi Yoshida, MD, PhD remarkable. Her visual acuity was curs after damage to the oculomo-
Asahikawa, Japan 20/25 OD and 20/20 OS. The eye- tor nerve and is thought to be caused
lid margin to corneal reflex dis- by misdirection of regenerating axons
We are grateful to thank Katsuhiko tance measured 2 mm OD and 4 mm to anomalous connections.2 Clini-
Fukui, MT, for technical assistance. OS. Her right pupil was 4 mm and cally, the upper eyelid retracts on at-
Corresponding author and reacted briskly to light. Her left pu- tempted downward gaze (pseudo–
reprints: Masumi Takeda, MD, PhD, pil was 5 mm and did not react to von Graefe sign) or adduction; there
Department of Ophthalmology, Asa- light. No relative afferent pupillary is minimal to no ptosis in primary po-
hikawa Medical College, Midoriga- defect was observed. sition and there may be limited el-
oka-Higashi 2-1-1-1, Asahikawa The patient assumed a chin-up evation, adduction, or infraduction.
078-8510, Japan (e-mail: masumi position in primary gaze. The left eye Also, the pupil, which does not re-
@asahikawa-med.ac.jp). had decreased elevation, adduction, act to light, may constrict on adduc-
and infraduction; motility was full in tion and infraduction.
1. Hikichi T, Trempe CL. Risk of decreased visual
acuity in full-thickness idiopathic macular holes.
the right eye (Figure 1). With the Primary aberrant regeneration
Am J Ophthalmol. 1993;116:708-712. right eye fixating at distance in pri- occurs without a history of acute
2. Lewis ML, Cohen SM, Smiddy WE, Gass JDM. mary position, she had 35–prism di- third cranial nerve palsy. This syn-
Bilaterality of idiopathic macular holes.
Graefes Arch Clin Exp Ophthalmol. 1996;234: opter (PD) hypotropia and 8 PD exo- drome was originally thought to be
241-245. tropia of the left eye. The hypotropia pathognomonic of cavernous sinus

663

meningiomas.1 Immediate imaging quently died of a ruptured aneu- tient underwent successful aneu-
was not recommended since these rysm at the junction of the left inter- rysm clipping.
masses are slow growing. Subse- nal carotid and PCOM arteries. Cox Our patient had painless pri-
quently, other intracavernous mass et al3 described a 76-year-old woman mary aberrant regeneration caused
lesions (aneurysms) were found to with primary aberrant oculomotor by a partially thrombosed aneu-
produce the syndrome.3 regeneration and chronic eye pain rysm at the junction of the left PCOM
However, unruptured, extra- due to a large, partially thrombosed and posterior cerebral arteries. This
cavernous, intradural aneurysms aneurysm at the junction of the right is the first reported instance of an an-
may rarely cause primary aberrant PCOM and internal carotid arter- eurysm at this location causing pri-
regeneration.3,4 Our patient is the ies. No surgery was performed. mary oculomotor nerve synkinesis.
fourth in the literature, to our knowl- Varma and Miller4 described a 64- Although primary aberrant regen-
edge, in whom a PCOM aneurysm year-old woman with painless pri- eration is most likely associated with
has caused primary aberrant regen- mary oculomotor nerve regenera- slow-growing cavernous sinus le-
eration.3-5 In 1952, Levin5 described tion caused by an aneurysm at the
a 68-year-old woman with primary junction of the right PCOM and in-
aberrant regeneration who subse- ternal carotid arteries. Their pa-
Figure 3. Frontal view of the cerebral angiogram

of the posterior circulation following injection of
the right vertebral artery. A focal outpouching is
seen (arrow) at the P1-P2 junction of the left
Figure 1. In primary position (center), there is a left hypotropia and left exotropia. A mild right ptosis is posterior cerebral and posterior communicating
present. There is limited elevation, adduction, and infraduction in the left eye. Upper eyelid retraction is arteries, consistent with a partially thrombosed
seen on adduction and infraduction in the left eye (lower left). aneurysm.
A B
Figure 2. Axial T1-weighted pregadolinium (A) and gadolinium-enhanced (B) magnetic resonance image showing a 1-cm enhancing mass in the interpeduncular
cistern region (arrows), consistent with a partially thrombosed aneurysm.

664

sions, PCOM aneurysms rarely cause Further inquiry revealed that the significantly improved the follow-
this syndrome. We believe that pa- child had been playing with the fam- ing day. To our knowledge, this is the
tients with primary aberrant oculo- ily dog, which was recently treated first reported case of mydriasis oc-
motor nerve regeneration should un- with Sergeant’s Flea and Tick Spray curring in association with expo-
dergo immediate imaging to detect (Sergeant’s Pet Care Products, sure to flea pesticide products. It is
potentially treatable extracavern- Omaha, Neb). His behavior and ac- important to consider exposure to
ous, intradural aneurysms. tivity had been normal. Vital signs pesticides during the evaluation of
were normal for his age, and results anisocoria since it may prevent costly
Jacqueline R. Carrasco, MD of a pediatrician’s examination re- and unnecessary imaging in the oth-
Peter J. Savino, MD vealed no abnormalities other than erwise healthy patient.
Jurij R. Bilyk, MD anisocoria. Ophthalmologic exami-
Philadelphia, Pa nation results revealed a visual acu- Jason D. Burns, MD
ity of 20/30 OU using Allen figures Laura T. Muller, MD
Corresponding author and reprints: at near. Anterior segments were nor- Pattye F. Jenkins, CO
Peter J. Savino, MD, Wills Eye Hos- mal when examined with a hand- Charlise A. Gunderson, MD
pital, 900 Walnut St, Philadelphia, PA held light. Versions, alignment, and Galveston, Tex
19107. dilated fundus examination results
were normal. There was no evi-
1. Shatz NJ, Savino PJ, Corbett JJ. Primary aber- dence of disc edema. Intraocular pres- None of the authors have any propri-
rant oculomotor regeneration: a sign of intra-
cavernous meningioma. Arch Neurol. 1977;34:
sure readings were 13 mm Hg OD etary interests in the product men-
29-32. and 14 mm Hg OS. Under lighted tioned in this article.
2. Sibony PA, Evinger C, Lessell S. Retrograde conditions, the right pupil was 6.5 Corresponding author and re-
horseradish peroxidase transport after oculo-
motor nerve injury. Invest Ophthalmol Vis Sci. mm and the left pupil was 4 mm. In prints: Charlise A. Gunderson, MD,
1986;27:975-980. dim light, the right pupil measured University of Texas Medical Branch,
3. Cox TA, Wurster JB, Godfery WA. Primary ab- 7 mm and the left pupil measured 5
errant regeneration due to intracranial aneu- Department of Ophthalmology and Vi-
rysm. Arch Neurol. 1979;36:570-571. mm. Both the direct and consensual sual Sciences, 301 University Blvd,
4. Varma R, Miller NR. Primary oculomotor nerve pupillary light responses were 1+ OD Galveston, TX 77555-0787 (e-mail:
synkinesis caused by an extracavernous intra-
dural aneurysm. Am J Ophthalmol. 1994;118:83-
and 3+ OS. The near response was cgunders@utmb.edu).
87. also 3+ OS and 1+ OD. Results of
5. Levin PM. Intracranial aneurysms: clinicopatho- computed tomography of the head 1. Lee AG, Taber KH, Hayman LA, et al. A guide
logic considerations of oculomotor-nerve regen- to the isolated dilated pupil. Arch Fam Med. 1997;
eration and intracerebral hemorrhage. Arch Neu- performed without contrast showed 6:385-388.
rol Psych. 1952;67:771-786. no evidence of intracranial masses, 2. Heard JM. Anisocoria—another cause. Vet Hum
edema, or dilated ventricles. The my- Toxicol. 1984;26:20.
3. Flach AJ, Donahue ME. Pet flea and tick collar-
driasis and pupillary reactivity of the induced anisocoria. Arch Ophthalmol. 1994;112:
right eye had improved by the ex- 586.
Unilateral Mydriasis amination on the following day. Al- 4. Ray DE, Forshaw PJ. Pyrethroid insecticides: poi-
soning syndromes, synergies, and therapy. Clin
Associated With Exposure though the child failed to return for Toxicol. 2000;38:95-101.
to Flea Spray a subsequent examination, a tele- 5. Nishimura M, Obana N, Yagasaki O, et al. In-
volvement of adrenergic and serotonergic ner-
phone conversation with his mother vous mechanisms in allethrin-induced tremors
Anisocoria is a frequently encoun- revealed that the pupil inequality had in mice. J Toxicol Sci. 1984;9:131-142.
tered condition that often requires resolved and that he was doing fine.
neurologic evaluation. Although it
may occur with third nerve palsy, Comment. The active pesticide in
other causes include benign condi- Seargant’s Flea and Tick Spray and re-
tions, such as pharmacologic dila- lated products permethrin is a type Acute Psychosis
tion, local iris sphincter abnormali- I pyrethroid. Pyrethroids have been Following the Use of
ties, tonic pupil, or sympathetic found to produce potent sympa- Topical Ciprofloxacin
irritation.1 Unilateral miosis has been thetic activation; local effects, such
reported to result from exposure to as paresthesia, have been reported Ciprofloxacin hydrochloride is a fluo-
cholinesterase inhibitors found in with skin contamination.4 Addition- roquinolone antimicrobial fre-
both flea foggers2 and pet flea and ally, ␣-adrenergic–mediated effects quently used in both ophthalmic and
tick collars.3 We report a case of uni- have been noted in animal studies us- general medical practice. Whereas ad-
lateral mydriasis associated with ex- ing allethrin, another type I pyre- verse effects of this drug are not un-
posure to flea spray. throid.5 We suggest that the pa- common following its systemic use,
tient’s unilateral mydriasis may have they are uncommon following its
Report of a Case. A 31⁄2-year-old boy been due to a local effect of perme- topical use. We present the case of a
was brought to the emergency de- thrin that occurred after inadvert- young woman who developed an
partment by his parents, who no- ent rubbing of the eye following skin acute psychotic reaction following the
ticed that his pupils were of un- contamination. The local sympa- use of ciprofloxacin eyedrops. To our
equal size. There was no history of thetic effect did not alter near vision knowledge, this is the first reported
trauma, recent illness, medication but produced mydriasis that was par- case of such a complication follow-
use, or access to ophthalmic drops. tially overcome by bright light and ing topical ciprofloxacin use.

665

Report of a Case. An otherwise fit a single 500-mg dose, with 19% of an 1. LeBel M. Ciprofloxacin: chemistry, mechanism
of action, resistance, antimicrobial spectrum,
and healthy 27-year-old woman came oral dose being excreted as metabo- pharmacokinetics, clinical trials, and adverse re-
to the emergency eye clinic with a lites in both urine and feces.1 In ad- actions. Pharmacotherapy. 1988;8:3-33.
3-day history of bilateral reddened dition to cutaneous and gastrointes- 2. Stahlmann R, Lode H. Toxicity of quinolones.
Drugs. 1999;58(suppl 2):37-42.
eyes associated with ecchymosis and tinal adverse effects, central nervous 3. Royer RJ. Adverse drug reactions with fluoro-
swelling of the eyelids. There was no system adverse effects, such as diz- quinolones. Therapie. 1996;51:414-416.
4. Ball P. Adverse drug reactions and interactions
history of pain or impairment of vi- ziness and lightheadedness,2 acute of fluoroquinolones. Clin Invest Med. 1989;12:
sion. Examination results revealed se- psychosis, and other neuropsychiat- 28-34.
vere bilateral conjunctival conges- ric disorders3,4 have been described 5. Mulhall JP, Bergmann LS. Ciprofloxacin-
induced acute psychosis. Urology. 1995;46:102-
tion, marked papillary reaction, and following its systemic use. In ex- 103.
membranous conjunctivitis. The re- tremely rare situations, more seri- 6. McCue JD, Zandt JR. Acute psychosis associ-
ous central nervous system effects, ated with the use of ciprofloxacin and trimeth-
sults of the remainder of the ocular oprim-sulfamethoxazole. Am J Med. 1991;90:
examination were entirely normal such as seizures, have also been de- 528-529.
and there was no lymphadenopa- scribed.4 Cases of ciprofloxacin- 7. Varghese S, Vettath N, Iyer K, Puliyel JM, Puli-
yel MM. Ocular atropine induced psycho-
thy. The patient had no history of any induced psychosis have been re- sis—is there a direct access route to the brain.
systemic complaints, was taking no ported, with resolution of psychotic J Assoc Physicians India. 1990;38:444-445.
medication for therapeutic or recre- symptoms on discontinuation of the 8. Harel L, Frydman M, Kauschansky A. Pro-
longed parasympathetic paralysis and psycho-
ational use, did not have alcohol- drug5 and reappearance of these sis caused by atropine eye drops. J Pediatr Oph-
ism, and did not smoke. Conjuncti- symptoms on resumption of cipro- thalmol Strabismus. 1985;22:38-39.
floxacin treatment.6 However, to our 9. Khurana AK, Ahluwalia BK, Rajan C, Vohra AK.
val swabs were taken and sent for Acute psychosis associated with topical cyclo-
microbial isolation and sensitivity as- knowledge, there is no report of these pentolate hydrochloride. Am J Ophthalmol. 1988;
says and the patient was prescribed adverse effects following the use of 105:91.
ciprofloxacin eye drops, 1 drop topical ciprofloxacin eye drops. In the
hourly, in each eye. About a half hour case reported here, we believe that the
after the third dose, the patient com- systemic adverse event experienced Here’s Egg in Your Eye:
plained of dizziness and light- by this patient was an idiosyncratic An Unusual Penetrating
headedness. Following this, family one, aided by the increased sys- Eye Injury
members noted a distinct change in temic absorption of the drug second-
the behavior of the patient, who began ary to severe conjunctival inflamma- Many unique, interesting, and even
to have well-defined visual halluci- tion. Although the patient did not bizarre cases of penetrating eye in-
nations, ill-defined auditory halluci- resume use of the topical ciprofloxa- juries have previously been re-
nations, and irrational conversation. cin, the absence of other confound- ported. Examples include injuries
The next day, the patient was seen in ing illnesses (drug or alcohol abuse caused by a fishing hook1 and a golf
the emergency eye department, ac- and previous psychiatric distur- tee,2 pecking injuries due to mag-
companied by her mother, with in- bances) and the rapid resolution of pies3 and cormorants,4 and even a boa
creasing behavioral problems. A psy- psychosis support the case for topi- constrictor bite that perforated its
chiatric opinion was sought. The cal ciprofloxacin as the causative fac- owner’s right eye.5 This report pre-
patient was deemed to have had an tor. Whereas psychotic reactions are sents a case of a penetrating eye in-
acute psychotic reaction secondary to well known after the use of atro- jury caused by an exploding emu egg,
topical ciprofloxacin. The patient was pine7,8 and cyclopentolate9 eye drops, which to the best of our knowledge
advised to stop using ciprofloxacin this is the first reported case, to our is the first such case reported in the
eye drops (nearly 24 hours after ini- knowledge, of such a reaction after ophthalmic literature. (Note: The
tiation of treatment) and began tak- the use of ciprofloxacin eye drops. emu is a large native bird of Austra-
ing teicoplanin (1%) eye drops. Therefore, the possibility of such an lia, and adult birds may grow to more
Within about 12 hours of changing adverse effect should always be kept than 6 ft in standing height. Emu eggs
the treatment, the patient and family in mind in patients using these drops, are routinely about 5 times the size
members noted an improvement in particularly in young women, who of a normal chicken egg.)
behavior and within a further 12 to seem to be especially sensitive to
14 hours, the hallucinations and be- these effects.2 Report of a Case. A 10-year-old boy
havioral disturbances disappeared sustained a severe penetrating injury
completely. The patient was later able Ajay Tripathi, MS, FRCS(Ed), to his left eye from an exploding emu
to describe her visual and auditory FRCS(Glas) egg. He was expelling the egg’s con-
hallucinations in detail. The conjunc- Birmingham, England tents with an air compressor when
tivitis responded completely to the te- Sean I. Chen, MRCOphth yolk blocked the exit hole, causing
icoplanin regimen. Sheila O’Sullivan, MRCPsych the egg to explode. An examination
Liverpool, England revealed a full-thickness paracentral
Comment. Ciprofloxacin is a fluo- laceration of the cornea, extending
roquinolone antimicrobial that in- Corresponding author: Ajay Tripathi, from the superior to inferior limbus.
hibits DNA gyrase, and when given MS, FRCS(Ed), FRCS(Glas), Bir- The boy’s visual acuity was light per-
orally, exhibits 70% bioavailability mingham and Midland Eye Hospital, ception only OS. A computed tomo-
and attains peak serum levels rang- City Hospital, Dudley Road, Birming- graphic scan revealed a large foreign
ing between 1.5 and 2.9 µg/mL after ham B18 7QU, England. body lodged in the nasal retina.

666

cus aureus (coagulase-positive). Oral
and topical ciprofloxacin hydrochlo-
ride therapy was commenced post-
operatively.
Six months later, further sur-
gery was performed involving a
sutured-in posterior chamber intra-
ocular lens. At the most recent fol-
low-up visit, the boy’s pinhole visual
acuity had improved to 20/30 OS.
Comment. For penetrating eye in-

juries involving organic matter, it is
essential to collect vitreous and/or
other appropriate intraocular speci-
mens during the initial surgical pro-
cedure. Once intravitreal antibiot-
ics are given, it may be very difficult
to isolate the infecting organisms
from subsequent aspirates.4 This is
Figure 1. Removal of the emu eggshell fragment from the eye via the corneal laceration. particularly important in penetrat-
ing injuries with a high risk of in-
fection such as those including ani-
mal, soil, or water contamination. In
addition, when gas or oil exchange
is performed, injecting intravitreal
antibiotics beforehand enables an ap-
propriate concentration to be main-
tained after the exchange.
This case report presents a very
unusual penetrating eye injury that,
with appropriate and timely treat-
ment, resulted in a good clinical out-
come.
David J. Hilford, MBBS(Hons)

Lawrence R. Lee, FRANZCO
Brisbane, Queensland
The authors thank Claire Cantwell and

the Central Medical Illustration Unit,
Royal Brisbane Hospital, Herston,
Queensland, for their assistance with
the preparation of the photographs.
Corresponding author and re-
prints: Lawrence R. Lee, FRANZCO,
Department of Ophthalmology, Royal
Figure 2. The colorful, triangular, 11-mm emu eggshell fragment adjacent to the corneal laceration.
Brisbane Hospital, PO Box 41, Hers-
ton, Queensland 4029, Australia
Later that day, the boy under- tured, an inferonasal segmental (e-mail: eye@cityeye.com.au).
went surgery. The prolapsed vitre- scleral buckle was applied, and per-
ous was excised, and the corneal fluoropropane gas was injected into 1. Krott R, Bartz-Schmidt KV, Heimann K. Lacera-
tion of the eye with a fishing hook. Br J Ophthal-
wound was sutured so that the in- the eye. mol. 1999;83:1194.
traocular pressure could be main- An intraoperative vitreous bi- 2. Mulvihill A, O’Sullivan J, Logan P. Penetrating
tained. A 3-port pars plana vitrec- opsy was obtained, and intravitreal eye injury caused by a golf tee. Br J Ophthalmol.
1997;81:91.
tomy and lensectomy were performed. injections of vancomycin hydrochlo- 3. Horsburgh BJ, Stark DJ, Harrison JD. Ocular in-
The eggshell fragment embedded in ride (1 mg/0.1 mL) and ceftazidime juries caused by magpies. Med J Aust. 1992;157:
756-759.
the nasal retina was identified and re- (2.25 mg/0.1 mL) were adminis- 4. Lee LR, O’Hagan S, Dal Pra M. Aeromonas so-
moved from the eye with intraocular tered. Biopsy cultures revealed a bria endophthalmitis. Aust N Z J Ophthalmol.
forceps via the corneal laceration, mixed growth of viridans strepto- 1997;25:299-300.
5. Kleinman DM, Dunne EF, Taravella MJ. Boa con-
which was reopened (Figures 1 and cocci, Haemophilus parainfluenzae, strictor bite to the eye. Arch Ophthalmol. 1998;
2). The corneal wound was resu- Neisseria species, and Staphylococ- 116:949-950.

667


Primary Aberrant Oculomotor Nerve Regeneration From A Posterior Communicating Artery Aneurysm (PDFDrive)

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CLINICOPATHOLOGIC REPORTS, CASE REPORTS, AND SMALL CASE SERIES

SECTION EDITOR: W. RICHARD GREEN, MD

In the last few decades, the appar-

Report of a Case. The patient was a

For editorial comment

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Transpupillary thermotherapy (TTT)

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Figure 3. Five days after infrared laser exposure. A, Color

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Figure 4. A, Low-power light microscopy showing choroidal

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Comment. This case is noteworthy

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Figure 8. Molecular genetic analysis of the

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Figure 1. Color fundus photographs. A, The

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Inferior Superior Inferior Superior

Our patient had surprisingly good vi- increased in upward gaze to 40 PD

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Figure 3. Frontal view of the cerebral angiogram

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Comment. For penetrating eye in-