NOTES, CASES, INSTRUMENTS REFRACTIVE CHANGE AS T H E INITIAL SIGN O F PROGRESSIVE FACIAL H E M I A T R O P H Y H A I M KARNY, M.D., JULES L. BAUM,
AND M.D.
Boston, Massachusetts Patients with progressive facial hemiatrophy a r e usually referred to ophthalmologists for ocular complications long after the origi nal diagnosis is made. W e had a case in which arrested myopia preceded other signs. CASE REPORT
A 13-year-old girl, first seen by us in August 1971, was myopic and had been wearing eye glasses since the age of 7 years. She required frequent changes in eye glasses for progressive myopia (Table). Visual acuity with each of these correc tions ranged between 20/20 and 20/25 in each eye.
left hypertropia that was corrected with 5 prism diopters appeared one year later but progressed no further. In August 1971, visual acuity with correction was 20/25+ in each eye. Color vision using H-R-R pseudoisochromatic plates was normal. Minimal im pairment of stereoscopic vision was demonstrated. We noted 3 mm of enophthalmos on the right side. A right superior rectus muscle and right in ferior oblique muscle weakness was demonstrated on the Hess screen. Horizontal corneal diameters were 11 mm in the right eye and 11.5 mm in the left eye. The right pupil measured 5 mm in diameter and reacted less to light and near than the left pupil which was 3 mm in diameter. By applanation tonometry, intraocular pressures were R.E. : 10 mm Hg, and L.E. : 18 mm Hg. Soft tissue atrophy of the right side of the face was noted without motor or sensory impairment. The remainder of the eye examination was within normal limits. X-ray films of the facial bones, electromyogram of the facial muscles, and tonography showed nega tive findings. An electroencephalogram showed bi lateral diffuse disturbances. DISCUSSION
TABLE
P a r r y first described progressive facial hemiatrophy in 1825, 1 and Romberg clinically defined it in 1846. 2 Characteristically, it com mences in early life, usually before puberty, and is manifested by a progressive facial atrophy. T h e subcutaneous fat, connective tissue, and subsequently bone and cartilage are affected. Systemically, this disease may be associated with neural defects, hormonal, cutaneous, skeletal, and circulatory distur bances. 3 · 4
CHANGES IN REFRACTION
Date, mo/yr
Age of Patient (yrs)
R.E.*
L.E.
9/64 4/66 10/66 10/67 9/68 2/69 2/70 8/71 6/74
6 7 8 9 10 10 11 13 IS
+0.25 -0.75 -0.75 -0.7S -0.75 -1.00 -1.00 piano +2.25
+0.25 -1.S0 -1.75 -2.75 -3.50 -3.75 -4.25 -4.25 -4.50
' Note arrested myopia and increasing hyperopia in the right eye. In 1968, an area of brown pigmentation over the right side of the mandible was noted, followed by the appearance of a scar over the right side of the upper lip. A painless atrophy of the right facial soft tissues appeared later. Diplopia resulting from a From the Department of Ophthalmology, New England Medical Center and Tufts University School of Medicine, Boston, Massachusetts. Reprint requests to Jules L. Baum, M.D., 171 Har rison Ave., Boston, MA 02111.
Ocular involvement is frequent (approx imately 1 6 % of cases 5 ) and includes atrophy of the eyelids and eyebrows, enophthalmos, rarely exophthalmos, H o m e r ' s syndrome or pupillary dilatation, and extraocular muscle weakness.® Atrophie changes of the cornea were described. 5 · 7 ' 8 Cyclitis 9 and choroidal and chorioretinal lesions were documented. 10 Usually the atrophie process begins over the mandible and spreads to involve other areas of the face a n d ipsilateral e y e ; the contralateral eye and face are involved less often.
878
T h e atrophie process may affect the globe
NOTES, CASES, INSTRUMENTS
VOL. 79, NO. 5
directly5'7 and the enophthalmos may be a manifestation of both retrobulbar fat atrophy and shrinkage of the globe itself, sometimes resulting in phthisis bulbi. Our patient demonstrated many of the fa cial and ocular signs of progressive facial hemiatrophy, including soft tissue atrophy on the right side of the face, enophthalmos, arrested myopia with increasing hyperopia of the right eye, pupillary dilatation in the right eye with decreased pupillary reaction, and right superior recrus muscle and right in ferior oblique muscle underaction. The ap pearance of arrested myopia before any of the other signs represents an unusual clini cal pattern in the development of progressive facial hemiatrophy. SUMMARY
Unilateral arrested myopia preceded other signs of progressive facial hemiatrophy in a 13-year-old girl. Facial signs including soft tissue atrophy, enophthalmos, pupillary dila tation with decreased pupillary reaction, and extraocular muscle involvement later ap peared on the same side as the eye with the arrested myopia. REFERENCES
1. Parry, C. H. : Collections from the Unpub lished Medical Writings of the Late Caleb Hillier Parry. London, Underwoods, 1825, p. 478. 2. Romberg, M. H. : Klinische Ergebnisse. Berlin, Forstner, 1846, p. 75. 3. Johnson, R. V., and Kennedy, W. R. : Progres sive facial hemiatrophy (Parry-Romberg syn drome), contralateral extraocular muscle impair ment. Am. J. Ophthalmol. 67 :S61, 1969. 4. François, J. : Heredity in Ophthalmology. St. Louis, C. V. Mosby Co., 1961, p. 617. 5. Lindemann, H. O. : Interessante befunde bei hemiatrophia facialis progressiva. Albrecht von Graefe's Arch. Ophthalmol. 142:409, 1940. 6. Walsh, F. B., and Hoyt, W. F. : Clinical Neuro-Ophthalmology. Baltimore, Williams & Wil lems Co., 1969, vol. 1, p. 972. 7. Franceschetti, A., and König, H. : L'importance du facteur heredo-degeneratif dans l'hemiatrophie faciale progressive (Romberg). Etude des complica tions oculaires dans ce syndrome. J. Genet. Hum. 1 : 27, 1952. 8. Collier, M. : Hemiatrophic faciale progressive
879
avec megalocornea, micropapille et dystrophie naugeuse centrale de la cornea. Acta Ophtalmol. 49: 946, 1971. 9. Sugar, H. S., and Banks, T. L. : Fuchs' heterochromic cyclitis associated with facial hemiatrophy. Am. J. Ophthalmol. 57:627, 1964. 10. Moura, R. A. : Progressive facial hemia trophia. Am. J. Ophthalmol. 55 :635, 1963.
MECHANICAL SYRINGE DRIVE FOR V I T R E O U S SURGERY STEVE CHARLES, M.D.,
CHARLES
M C C A R T H Y , AND D A N I E L EICHENBAUM,
M.D.
Bethesda, Maryland Current methods of trans pars plana vitrectomy utilize suction force to imbricate the vitreous humor into the port of the vitrectomy instrument. 1 The surgical assistant provides the suction by manipulating a 5- to 50-ml syringe while observing the surgeon's operative field through a stereoscopic ob servation tube on the operating microscope. This method is inherently simple and there may be a rapid reversal of the suction to dis engage tissue from the cutting tip. Operating the syringe, however, can be fatiguing and can be relatively imprecise at times, depend ing on the internal friction of the syringe. In addition, it is difficult to operate the syringe with one hand while assisting the surgeon with the other hand. Any mechanical replace ment for the present system, therefore, must ideally be operated by one hand, offer rapid reversibility, eliminate fatigue, be autoclavable, and must provide precise, accurate control of suction force. We developed a manually operated, meFrom the Clinical Branch, National Eye Institute (Drs. Charles and Eichenbaum), and the Biomedi cai Engineering and Instrument Branch (Mr. Mc Carthy), National Institutes of Health, Bethesda, Maryland. Reprint request to Steve Charles, M.D., Clinical Branch, National Eye Institute, National Insti tutes of Health, Bldg. 10, Rm. 10N313, 9000 Rockville Pike, Bethesda, MD 20014.
AND M.D.
Boston, Massachusetts Patients with progressive facial hemiatrophy a r e usually referred to ophthalmologists for ocular complications long after the origi nal diagnosis is made. W e had a case in which arrested myopia preceded other signs. CASE REPORT
A 13-year-old girl, first seen by us in August 1971, was myopic and had been wearing eye glasses since the age of 7 years. She required frequent changes in eye glasses for progressive myopia (Table). Visual acuity with each of these correc tions ranged between 20/20 and 20/25 in each eye.
left hypertropia that was corrected with 5 prism diopters appeared one year later but progressed no further. In August 1971, visual acuity with correction was 20/25+ in each eye. Color vision using H-R-R pseudoisochromatic plates was normal. Minimal im pairment of stereoscopic vision was demonstrated. We noted 3 mm of enophthalmos on the right side. A right superior rectus muscle and right in ferior oblique muscle weakness was demonstrated on the Hess screen. Horizontal corneal diameters were 11 mm in the right eye and 11.5 mm in the left eye. The right pupil measured 5 mm in diameter and reacted less to light and near than the left pupil which was 3 mm in diameter. By applanation tonometry, intraocular pressures were R.E. : 10 mm Hg, and L.E. : 18 mm Hg. Soft tissue atrophy of the right side of the face was noted without motor or sensory impairment. The remainder of the eye examination was within normal limits. X-ray films of the facial bones, electromyogram of the facial muscles, and tonography showed nega tive findings. An electroencephalogram showed bi lateral diffuse disturbances. DISCUSSION
TABLE
P a r r y first described progressive facial hemiatrophy in 1825, 1 and Romberg clinically defined it in 1846. 2 Characteristically, it com mences in early life, usually before puberty, and is manifested by a progressive facial atrophy. T h e subcutaneous fat, connective tissue, and subsequently bone and cartilage are affected. Systemically, this disease may be associated with neural defects, hormonal, cutaneous, skeletal, and circulatory distur bances. 3 · 4
CHANGES IN REFRACTION
Date, mo/yr
Age of Patient (yrs)
R.E.*
L.E.
9/64 4/66 10/66 10/67 9/68 2/69 2/70 8/71 6/74
6 7 8 9 10 10 11 13 IS
+0.25 -0.75 -0.75 -0.7S -0.75 -1.00 -1.00 piano +2.25
+0.25 -1.S0 -1.75 -2.75 -3.50 -3.75 -4.25 -4.25 -4.50
' Note arrested myopia and increasing hyperopia in the right eye. In 1968, an area of brown pigmentation over the right side of the mandible was noted, followed by the appearance of a scar over the right side of the upper lip. A painless atrophy of the right facial soft tissues appeared later. Diplopia resulting from a From the Department of Ophthalmology, New England Medical Center and Tufts University School of Medicine, Boston, Massachusetts. Reprint requests to Jules L. Baum, M.D., 171 Har rison Ave., Boston, MA 02111.
Ocular involvement is frequent (approx imately 1 6 % of cases 5 ) and includes atrophy of the eyelids and eyebrows, enophthalmos, rarely exophthalmos, H o m e r ' s syndrome or pupillary dilatation, and extraocular muscle weakness.® Atrophie changes of the cornea were described. 5 · 7 ' 8 Cyclitis 9 and choroidal and chorioretinal lesions were documented. 10 Usually the atrophie process begins over the mandible and spreads to involve other areas of the face a n d ipsilateral e y e ; the contralateral eye and face are involved less often.
878
T h e atrophie process may affect the globe
NOTES, CASES, INSTRUMENTS
VOL. 79, NO. 5
directly5'7 and the enophthalmos may be a manifestation of both retrobulbar fat atrophy and shrinkage of the globe itself, sometimes resulting in phthisis bulbi. Our patient demonstrated many of the fa cial and ocular signs of progressive facial hemiatrophy, including soft tissue atrophy on the right side of the face, enophthalmos, arrested myopia with increasing hyperopia of the right eye, pupillary dilatation in the right eye with decreased pupillary reaction, and right superior recrus muscle and right in ferior oblique muscle underaction. The ap pearance of arrested myopia before any of the other signs represents an unusual clini cal pattern in the development of progressive facial hemiatrophy. SUMMARY
Unilateral arrested myopia preceded other signs of progressive facial hemiatrophy in a 13-year-old girl. Facial signs including soft tissue atrophy, enophthalmos, pupillary dila tation with decreased pupillary reaction, and extraocular muscle involvement later ap peared on the same side as the eye with the arrested myopia. REFERENCES
1. Parry, C. H. : Collections from the Unpub lished Medical Writings of the Late Caleb Hillier Parry. London, Underwoods, 1825, p. 478. 2. Romberg, M. H. : Klinische Ergebnisse. Berlin, Forstner, 1846, p. 75. 3. Johnson, R. V., and Kennedy, W. R. : Progres sive facial hemiatrophy (Parry-Romberg syn drome), contralateral extraocular muscle impair ment. Am. J. Ophthalmol. 67 :S61, 1969. 4. François, J. : Heredity in Ophthalmology. St. Louis, C. V. Mosby Co., 1961, p. 617. 5. Lindemann, H. O. : Interessante befunde bei hemiatrophia facialis progressiva. Albrecht von Graefe's Arch. Ophthalmol. 142:409, 1940. 6. Walsh, F. B., and Hoyt, W. F. : Clinical Neuro-Ophthalmology. Baltimore, Williams & Wil lems Co., 1969, vol. 1, p. 972. 7. Franceschetti, A., and König, H. : L'importance du facteur heredo-degeneratif dans l'hemiatrophie faciale progressive (Romberg). Etude des complica tions oculaires dans ce syndrome. J. Genet. Hum. 1 : 27, 1952. 8. Collier, M. : Hemiatrophic faciale progressive
879
avec megalocornea, micropapille et dystrophie naugeuse centrale de la cornea. Acta Ophtalmol. 49: 946, 1971. 9. Sugar, H. S., and Banks, T. L. : Fuchs' heterochromic cyclitis associated with facial hemiatrophy. Am. J. Ophthalmol. 57:627, 1964. 10. Moura, R. A. : Progressive facial hemia trophia. Am. J. Ophthalmol. 55 :635, 1963.
MECHANICAL SYRINGE DRIVE FOR V I T R E O U S SURGERY STEVE CHARLES, M.D.,
CHARLES
M C C A R T H Y , AND D A N I E L EICHENBAUM,
M.D.
Bethesda, Maryland Current methods of trans pars plana vitrectomy utilize suction force to imbricate the vitreous humor into the port of the vitrectomy instrument. 1 The surgical assistant provides the suction by manipulating a 5- to 50-ml syringe while observing the surgeon's operative field through a stereoscopic ob servation tube on the operating microscope. This method is inherently simple and there may be a rapid reversal of the suction to dis engage tissue from the cutting tip. Operating the syringe, however, can be fatiguing and can be relatively imprecise at times, depend ing on the internal friction of the syringe. In addition, it is difficult to operate the syringe with one hand while assisting the surgeon with the other hand. Any mechanical replace ment for the present system, therefore, must ideally be operated by one hand, offer rapid reversibility, eliminate fatigue, be autoclavable, and must provide precise, accurate control of suction force. We developed a manually operated, meFrom the Clinical Branch, National Eye Institute (Drs. Charles and Eichenbaum), and the Biomedi cai Engineering and Instrument Branch (Mr. Mc Carthy), National Institutes of Health, Bethesda, Maryland. Reprint request to Steve Charles, M.D., Clinical Branch, National Eye Institute, National Insti tutes of Health, Bldg. 10, Rm. 10N313, 9000 Rockville Pike, Bethesda, MD 20014.
