OCULAR MANIFESTATIONS AND T R E A T M E N T O F HEMIFACIAL ATROPHY R I C H A R D S. M U C H N I C K , M.D.,
S H E R R E L L J. A S T O N ,
AND T H O M A S D. R E E S ,
M.D.,
M.D.
New York, New York
Hemifacial wasting 1 - 3 of the skin, sub cutaneous tissue, muscle, and bone is most often diagnosed as progressive hemifacial atrophy (Romberg's disease) or localized scleroderma. The distinction usually depends on the absence or pres ence, respectively, of skin pigmentation and other inflammatory changes. It is often difficult, if not impossible, to differ entiate between the two. They may be different manifestations of the same disease, 3 ' 4 and the cause is unknown. Similar findings may be seen in hemi facial microsomia (first and second bran chial arch syndrome) and its variants such as Goldenhar's syndrome, 5 , 6 posttraumatic atrophy, and partial lipodystrophy (Barraquer-Simons disease). 3 , 7 Facial at rophy in partial lipodystrophy, however, is always bilateral and involves only adi pose tissue. Progressive hemifacial atrophy usually begins during the first or second decade of life, and occasionally, cases have start ed in middle or old age. The most com mon early sign is a painless cleft or fur row near the midline of the face. This coup de sabre marks the boundary be tween normal and atrophic tissue. The From the Departments of Ophthalmology (Dr. Muchnick), Manhattan Eye, Ear and Throat Hospi tal, and New York Hospital-Cornell Medical Center; the Department of Plastic Surgery (Drs. Aston and Rees), Manhattan Eye, Ear and Throat Hospital; and the Department of Surgery, Institute of Reconstruc tive Plastic Surgery (Drs. Aston and Rees), New York University Medical Center, New York, New York. This work was supported in part by the William Salomon Foundation, the Bienstock Foundation, the Lauder Foundation, and the Mrs. Charles Woody Fund (Dr. Rees). Reprint requests to Richard S. Muchnick, M.D., 111 E. 65th St., New York, NY 10021.
final degree of deformity varies widely. Some patients have marked progression with fulminating atrophy, whereas others have relatively minimal atrophy (micro form). In addition to facial wasting that may include the ipsilateral salivary glands and hemiatrophy of the tongue, varying de grees of ipsilateral or contralateral in volvement of the trunk and extremities occasionally occur. Unilateral involve ment of such organs as the brain, ear, larynx, esophagus, diaphragm, and kid ney, as well as other anomalies and condi tions, have been reported and summa rized. 3 Many ophthalmologic conditions have been described in patients with hemifa cial atrophy. Reported findings2,3 include enophthalmos, rarely exophthalmos, eye lid atrophy, loss of cilia, blepharoptosis, blepharophimosis, pupillary abnormali ties, Homer's syndrome, heterochromia, heterochromic cyclitis, various other in flammatory disorders, muscle pareses, retinal abnormalities, and phthisis. These findings have often been described in single case reports 8 - 1 2 and have thus not provided an overall clinical pattern be cause of the wide variability of manifesta tions. In the past, a wide variety of treatment techniques has been used. 1 The results have been generally poor. The recon struction of facial contour has been at tempted with grafts of fat, dermis and fat, fascia, bone, cartilage, and various alloplastic materials. In severe cases, particu larly when previous or repeated absorp tion of free grafts has occurred, pedicle flaps have been used.
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Fat, dermis and fat, fascia, and other soft tissue grafts are partially absorbed in all patients. These grafts may also partici pate in the underlying wasting process in patients with hemifacial atrophy. Bone grafts have met with some success in bviilding up atrophic maxillae. Synthetic implants such as polyethylene and polyvinyl often extrude. In recent years, we have been treating these patients with fluid silicone injec tions. The results have been extra ordinarily good and in marked contrast to the generally poor results of previous methods of treatment. We describe herein case histories of 11 of the 59 patients presently being fol lowed u p by us. The purpose is to obtain a more cohesive picture of the ocular abnormalities encountered in hemifacial atrophy and to review our experiences with fluid silicone injections.
primary position. This increased to 30 prism diop ters of left hyperphoria in right downgaze and decreased to 7 prism diopters of left hyperphoria in right upgaze. There was moderate underaction of both the left inferior oblique muscle and left superi or oblique muscle. In July 1977 the patient under went a 14-mm left superior oblique muscle tuck. After an initial overcorrection, this reduced the primary position deviation to 1 prism diopter of left hyperphoria and she became asyptomatic. Case 2—A 6-year-old girl (Fig. 1) was first seen by us in late 1974 with a three-year history of severe progressive right hemifacial atrophy involving the forehead, the temporal fossa, the orbit, the soft tissues of the cheek, the maxilla, the mandible, and the ala of the nose. Best corrected visual acuity was 6/6 (20/20) in both eyes with a refraction of R.E.: +2.50 and L.E.: +0.50. There was 6 mm of enoph thalmos. The right pupil was 3 mm, irregular, and poorly reactive. It did not dilate well with cycloplegics. The left pupil was 6 mm and normally reactive. The right eye felt slightly soft but applanation tonometry could not be performed because of inade quate cooperation. The right fundus revealed retinal striae extending in a spoke-like configuration from the fovea. The disk margin was blurred. The left fundus was normal. We believe the fundus changes may have been caused by hypotony or vitreous changes producing contraction of the internal limit ing membrane.
CASE REPORTS
Over the next 27 months the patient received 20.1 ml of fluid silicone injections to the right side of the face. The cosmetic result was good (Fig. 2). No attempt was made, however, to correct the enoph thalmos. Case 3—A 31-year-old woman had a 15-year histo ry of severe progressive right hemifacial atrophy with additional involvement of the right breast and buttock. The orbit, the temporal fossa, the soft tissues of the cheek, the maxilla, and the upper lip were all affected. Hertel exophthalmometry at base 90 was R.E.: 9.5 and L.E.: 13.5. A depressed superi or sulcus and diffuse, marked thinning of the right lower eyelid were noted. Cilia were sparse. Slit-lamp examination revealed a 1-mm central pigment de posit on the endothelium of the right cornea. The irides were brown-green but there were several sectoral patches of blue atrophic iris in the right eye. The largest was nasally and contained an area of transillumihation near the iris root. The heterochromia was first noticed by the patient three or four years previously. The patient was treated with 14.1 ml of fluid silicone injections to the temporal fossa, cheek, and upper lip for nine months with a good result. Case 4—A 15-year-old girl (Fig. 3) had the onset of mild left hemifacial atrophy at age 7 years with progression for three years. A typical coup de sabre deformity of the forehead developed that was treat ed with 8.5 ml of fluid silicone injections during a period of 12 months (Fig. 4). Additionally, she had moderate atrophy of the ala of the nose and mild involvement of the orbit. Best corrected visual acu ity was 6/4.5 (20/15) in both eyes with a refraction of
Case 1—In 1967 a 23-year-old woman had a mild left hemifacial atrophy, primarily involving the soft tissues of the cheek, the maxilla, and the forehead. T h e atrophy had begun at age 10 years and pro gressed for approximately three years. It then stabi lized except for mild increases after each of two pregnancies. She was treated with 11.6 ml of fluid silicone injections over 26 months with a good result. At age 26 years, the patient noted intermittent vertical diplopia that became increasingly frequent and finally constant by age 29 years. A depressed superior sulcus and 1.5 mm of enophthalmos was noted. The left upper eyelid was thin medially with an early coloboma. The left lower eyelid was atroph ic laterally. The left pupil was 0.5 mm larger than the right. There was 25 prism diopters of left hypertropia in the primary position. This increased to 25 to 30 prism diopters in right, upgaze and downgaze. There was moderate overaction of the left inferior oblique muscle and slight to moderate underaction of the left superior oblique muscle. Intraocular pressures were R.E.: 14 mm Hg and L.E.: 10 mm Hg. In late November 1973, the patient underwent a 10-mm left inferior oblique muscle recession. This reduced her primary position deviation to 4 prism diopters of left hyperphoria. She was markedly improved symptomatically and remained so until early 1977 when she began to note increasing asthenopia and recurrent diplopia. By June 1977 there was 13 prism diopters of left hyperphoria in the
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Fig. 1 (Muchnick, Aston, and Rees). Case 2. Se vere right hemifacial atrophy involving forehead, temporal fossa, orbit, soft tissues of cheek, maxilla, mandible, and ala of nose.
R.E.: - 1 . 0 0 + 0.75 x 75 degrees and L.E.: +0.50. There was 1.5 mm of left enophthalmos and a defect in the superior orbital rim medially. A slight lag of the left inferior rectus was noted. Applanation pres sures were R.E.: 12 mm Hg and L.E.: 9 mm Hg. Case 5—A 23-year-old man first consulted us in March 1975 with a history of severe left hemifacial atrophy beginning at age 10 years and stabilizing after four to five years. He had moderately severe involvement of the soft tissues of the cheek, the maxilla, and the ear, and severe involvement of the upper lip, the soft tissues of the chin, the mandible, and the tongue. Additionally, the patient had severe atrophy of the soft tissues of the left buttock and right flank. The orbital area was relatively spared, with mild left lower eyelid atrophy and loss of cilia being the only manifestation. He has received 22.9 ml of fluid silicone injections for 30 months and is still receiving treatment. Case 6—A 14-year-old girl (Fig. 5) had a four-year history of severe progressive left hemifacial atrophy involving the temporal fossa, the soft tissues of the cheek, the maxilla, and the upper lip. A biopsy revealed scleroderma. There was 1 mm of enoph thalmos, and a depressed superior sulcus. The globe
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Fig. 2 (Muchnick, Aston, and Rees). Case 2. Pa tient has received 20.1 ml of fluid silicone injections for 27 months.
was 1 mm lower than its fellow. The left pupil was slightly larger than the right. Applanation pressures were R.E.: 11 mm Hg and L.E.: 9 mm Hg. She was treated with 25.0 ml of fluid silicone injections to the temporal fossa, cheek, and upper lip for 38 months with a good result (Fig. 6). Case 7—A 15-year-old boy developed severe in flammatory left hemifacial atrophy with skin discol oration at age 4 years. It was diagnosed as scleroder ma and progressed until age 11 years (Fig. 7). He was one of the most severely affected patients we have seen, with almost complete atrophy of the soft tissues of the left side of the face. Only the forehead and nose were spared. Additionally, there was se vere atrophy of the maxilla and moderate atrophy of the mandible. Uncorrected visual acuity was 6/6 (20/20) in both eyes with a refraction of R.E.: +0.25 and L.E.: +1.25. There was 1 mm of left blepharoptosis with eyelid fissures measuring R.E.: 9 mm and L.E.: 8 mm. Levator muscle function was R.E.: 13 mm and L.E.: 8 mm. Marked left upper eyelid lag was noted. The left lower eyelid was diffusely atrophic with loss of cilia. Hertel exophthalmometry at base 90 was R.E.: 16 and L.E.: 9. The superior and inferior sulci were markedly depressed. The eyes were orthophoric in the primary position.
Fig. 3 (Muchnick, Aston, and Rees). Case 4. Mild left hemifacial atrophy with coup de sabre deformi ty of forehead. Ala of nose and orbit also show involvement.
Fig. 4 (Muchnick, Aston, and Rees). Case 4. Pa tient has received 8.5 ml of fluid silicone injections to forehead for 12 months.
Fig. 5 (Muchnick, Aston, and Rees). Case 6. Se vere left hemifacial atrophy involving temporal fos sa, orbit, soft tissues of cheek, maxjlla, and upper lip.
Fig. 6 (Muchnick, Aston, and Rees). Case 6. Pa tient has received 25.0 ml of fluid silicone injections for 38 months.
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Fig. 7 (Muchnick, Aston, and Rees). Case 7. Se vere inflammatory left hemifacial atrophy with skin discoloration. Only forehead a n d nose spared.
Fig. 8 (Muchnick, Aston, and Rees). Case 7. Pa tient has received 51.1 ml of fluid silicone injections and is still receiving treatment.
There was moderate underaction of the left inferior oblique muscle and slight underaction of the left superior rectus, left medial rectus, and left lateral rectus muscles. Intraocular pressures were R.E.: 8 mm Hg and L.E.: 5 mm Hg. Indirect ophthalmoscopy revealed a lightly pigmented retinal pigment epithelium and prominent choroidal pattern in the left eye and a normal right eye. He has received 51.1 ml of fluid silicone injections (Fig. 8) since age 11 years and is still in treatment. He has also had an otoplasty. Case 8—A 16-year-old girl had the onset of mod erate right hemifacial atrophy at age 4 years with progression until age 9 years. The atrophy affected all the soft tissues of the face, with bony involve ment of the zygoma and maxilla. She had received 30.0 ml of fluid silicone injections during the past 82 months with significant improvement. The right lower eyelid was diffusely atrophic with marked loss of cilia. The right globe was 4 mm lower and there was 3 mm of enophthalmos. Applanation pressures were R.E.: 10 mm Hg and L.E.: 14 mm Hg. Case 9—A 22-year-old man had the onset of severe left hemifacial atrophy at age 16 years with progres sion for four years. He had diffuse involvement of the soft tissues of the face as well as bony involve ment of the zygoma and maxilla. He had an iliac
bone graft to his left orbital floor shortly before we first saw him. This improved intermittent vertical diplopia that he had been experiencing for about four years. Best corrected visual acuity was R.E.: 6/4.5 (20/15) and L.E.: 6/7.5-(20/25-) with a refrac tion of R.E.: - 5 . 7 5 + 0.75 x 85 degrees and L.E.: - 4 . 0 0 + 2.50 x 80 degrees. There was 5 mm of enophthalmos, and a markedly depressed superior sulcus. The globe was 3 mm lower. The right pupil was 4 mm and normally reactive. The left was 8 mm and fixed. The left pupil constricted with 0.25% pilocarpine; the right did not. A slight left hypophoria in the primary position was noted. This increased to 20 prism diopters of left hypotropia in upgaze. There was moderate restriction of elevation in the left eye and slight restriction of adduction and abduction. Intraocular pressures were R.E.: 12 mm Hg and L.E.: 8 mm Hg. The fundi revealed early myopic changes. He received 10.0 ml of fluid sili cone injections for 12 months with a good result. Case 10—A 7-year-old boy recently had a threeyear history of severe progressive right hemifacial atrophy. There was near total atrophy of the subcu taneous tissue of the cheek, as well as severe in volvement of the temporal fossa, the zygoma, and the maxilla. Moderate involvement of the tongue and mandible was noted. The orbital area was
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spared; the results of the eye examination were normal. He has not yet received any treatment. Case 11—An 11-year-old girl had the onset of severe left hemifacial atrophy at age 3 years with progression until age 8 years. She had severe in volvement of the soft tissues of the cheek and chin, the ala of the nose, and the upper lip. Milder involvement of the forehead, the zygoma, and the maxilla was noted. One millimeter of enophthalmos and slightly depressed superior and inferior sulci were evident. The right lower eyelid was slightly thin laterally. The left iris was slightly greener than the right. She was treated with 9.5 ml of fluid silicone injections for 22 months with significant improvement. DISCUSSION
The 11 patients in this series were se lected to show the various ocular abnor malities encountered in hemifacial atro phy. Although previous studies 3 , 1 3 indi cated that just over 10% of the patients had ocular findings, a preliminary screen ing of our 59 patients suggests a 35 to 40% incidence of such abnormalities. Whether this represents a true difference, is a result of our being referred more severely af fected patients, or a more careful ocular evaluation is uncertain. There were seven women and four men in this series. This agrees with Rogers' 1 finding that the disease occurs more frequently in women in a ratio of 3:2. He reviewed 670 previously reported cases. In our series the left side was affected in seven patients and the right side in four patients. Rogers found a slight predilection for the left side. The difference however, was not statistically significant. The patients in this series show the variable expression of hemifacial atrophy. They range from mild relatively diffuse involvement (Case 1) to moderate diffuse (Case 8) to severe diffuse (Case 9) to localized (Case 4) involvement. The other patients show still other degrees of in volvement. The patients also exhibit the variable degree of ocular abnormality. They range from no involvement (Case 10) to mild (Cases 5, 6, and 11) to moder
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ate (Cases 1, 4, and 8) to severe (Cases 2, 3, 7, and 9) involvement. The most common manifestation of oc ular involvement was enophthalmos. It was present in nine of the ten patients with ocular findings and ranged from 1 mm (Cases 6 and 11) to 7 mm (Case 7). Enophthalmos is the result of loss of orbital fat. 14 Changes in the eyelids were also com mon. Six patients (Cases 1, 3, 5, 7, 8, and 11) had varying degrees of lower eyelid atrophy. One patient (Case 1) had upper eyelid involvement as well. This apparent predilection for the lower eyelid has not, to our knowledge, been previously ob served. The changes ranged from mild localized atrophy (Case 11) to more ad vanced localized atrophy forming an early coloboma 12 (Case 1) to diffuse marked loss of entire eyelid substance and cilia (Cases 3, 7, and 8). One patient (Case 7) had mild blepharoptosis with decreased levator muscle function and eyelid lag on downgaze. This may be caused by involvement of the levator muscle in the atrophy. 9 , 1 5 Flattening of the maxilla occurred in all but the most mildly affected patient (Case 4). The zygoma was similarly involved in four of the more severely affected patients (Cases 8, 9, 10, and 11). This bony in volvement contributed to flattening of the cheek. In three patients (Cases 6, 8, and 9), the maxillary involvement progressed to inferior orbital rim and floor defects severe enough to cause the globe to be lower than its fellow. One of these pa tients (Case 9) required an iliac bone graft to the orbital floor. One patient (Case 4) had a superior orbital rim defect. Visual acuity was uniformly good. Only one patient (Case 9) with ocular involvement had a visual acuity as low as 6/7.5 (20/25). Several patients with severe involvement (Cases 2, 3, and 7) had visual acuity of 6/6 (20/20). Thus, despite occa sional reports of visual loss from
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phthisis 1 3 ' 1 6 and fundus abnormalities, 1 0 the prognosis for vision seems to be good. Four patients (Cases 2, 4, 7, and 9) were more hyperopic or less myopic in the affected eye. In two patients (Cases 1 and 6) the pupil on the affected side was slightly larger than on the unaffected side, but with normal reactions. One severely af fected patient (Case 9) had a fixed dilated pupil that constricted with 0.25% pilocarpine. Another (Case 2) had a miotic, irreg ular, poorly reactive pupil. These proba bly represent a tonic pupil and an Argyll Robertson pupil, respectively. Previous reports have also indicated that pupillary abnormalities are common and varied. Lindemann 1 3 described pu pillary disturbances in 11 of 19 patients with ocular involvement. A dilated pupil on the affected side more often than a miotic pupil was noted. Tonic pupils and Argyll Robertson pupils have been previ ously described. 1 7 Homer's syndrome, re portedly common, 1 5 ' 1 8 did not appear in our patients. The different pupillary ab normalities are probably caused by dif ferent lesions of the sympathetic or parasympathetic nervous system, or both. Iris abnormalities were noted in two patients. In one (Case 11) the iris on the affected side was slightly greener than on the unaffected side. In the other (Case 3) patches of sectoral iris atrophy were noted. In both, pupillary reactions were normal and there was no iritis. Heterochromia 1 8 and sectoral iris atrophy 9 have been previously found in hemifacial atro phy. Sugar and Banks 1 1 reported a case of heterochromic cyclitis. Additionally, they reviewed two cases of heterochromia and 12 cases of heterochromic cyclitis. They suggested that both the heterochromic cyclitis and the hemifacial atrophy may be the result of neurovascular or neurotrophic changes resulting from distur bances of the sympathetic nervous sys tem.
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Five of the seven patients with signifi cant orbital involvement (more than 1 mm of enophthalmos) had intraocular pres sures at least 3 mm Hg lower on the affected side than on the unaffected side. The child (Case 2) in whom intraocular pressure could not be measured had a soft eye on palpation. This relative hypotony has not, to our knowledge, been previous ly reported although rare instances of phthisis have. 13 ' 16 The origin of the hy potony is not clear. Hypotony, like het erochromic cyclitis, may be the result of neurovascular or neurotrophic changes. Two patients (Cases 1 and 9) had sig nificant vertical motility disturbances that required surgery. Another two (Cases 4 and 7) had milder motility disturbances that did not cause diplopia or require treatment. Limitation of ocular move ments has been previously reported as a common finding. 13 It may be secondary to mechanical restrictions, involvement of the muscles in the atrophy, or cranial nerve pareses. Two patients with severe ocular in volvement had ipsilateral fundus abnor malities. One patient (Case 2) had retinal striae extending in a spoke-like configura tion from the fovea and a blurred disk margin. These changes may have been caused by hypotony or a vitreous abnor mality producing contraction of the inter nal limiting membrane. The other (Case 7) had a lightly pigmented retinal pig ment epithelium. Fundus changes appear to be uncommon although a wide variety have been reported and reviewed. 2 ' 9 ' 10 We could not find any previous reports on findings similar to the ones we observed. Fluid silicone injection has become the major modality in our treatment of hemi facial atrophy. Earlier results have been previously reported. 7 ' 19 The treatment re mains investigational. The technique in volves the use of small volume injections repeated many times at two- to four-week intervals. The patients receive 0.5 to
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2.0 ml of high viscosity medical grade silicone subcutaneously to the affected area of the face. The fluid silicone is injected aseptically through a 26- or 27gauge needle. While the injection is being made with moderate pressure the needle is continuously moved through the sub cutaneous space to insure an even spread. The needle is aspirated frequently to make sure that it is not in the lumen of a small vessel. After the injection, the treat ed area is lightly but thoroughly mas saged to disperse the silicone further. This technique results in a fine droplet dispersion of the silicone in the subcuta neous tissues. 20 Mild fibroplasia is stimu lated and the silicone becomes fixed in a fine reticular fibrous network. Thus, the small volume of individual injections, the massage, and the two- to four-week inter vals between injections all help to elimi nate the migration or drifting that was a problem in early fluid silicone treatment. The use of medical grade silicone has eliminated granuloma formation. Com plications have been limited to an occa sional hematoma and occasional tissue firmness. The results have been generally excel lent with marked improvement of facial contour, normal animation, and no tissue firmness. The amount of fluid silicone injected in the 11 patients in this series ranged from 8.5 ml (Case 4) to 51.1 ml (Case 7). The latter was given for 48 months. No injections have been made into the eyelids because of possible dan ger to the globe. Similarly, no attempt has been made to correct enophthalmos. We do not believe that a safe, effective proce dure is presently available. Other adjunct procedures have been performed as re quired in individual patients. These have included strabismus surgery, otoplasty, and iliac bone grafts. The bone grafts have been to the orbital floor to improve the position of the globe and to the anteri
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or maxilla to further improve facial con tour. SUMMARY
Enophthalmos, flattening of the maxilla that may progress to inferior orbital rim and floor defects, eyelid atrophy, and slight relative hypotony occurred in pa tients with hemifacial atrophy. Less com mon manifestations included pupillary and iris abnormalities, vertical muscle imbalances, and retinal changes. The prognosis for vision was good. Fluid sili cone injection was the major modality in treatment and the results were generally excellent. ACKNOWLEDGMENT
Medical grade silicone fluid, dimethylpolysiloxane, MDX4-4011, was supplied by Dow Corning Corporation under a Food and Drug Administration investigational permit in a program sponsored by the American Society of Plastic and Reconstructive Surgeons.
REFERENCES 1. Rogers, B. O.: Progressive facial hemiatrophy. Romberg's disease. A review of 772 cases. In Broadbent, T. R. (ed.): Transactions of the Third Interna tional Congress of Plastic Surgery. Amsterdam, Excerpta Medica, 1964, p. 681. 2. Walsh, F. B., and Hoyt, W. F.: Clinical Neuro-ophthalmology, vol. 1. Baltimore, Williams and Wilkins, 1969, pp. 970-974. 3. Rees, T. D.: Facial atrophy. Clin. Plast. Surg. 3:637, 1976. 4. Wartenberg, R.: Progressive facial hemiatro phy. Arch. Neurol. Psychiatr. 54:75, 1945. 5. Gorlin, R. J., Jue, K. L., Jacobsen, U., and Goldschmidt, E.: Oculoauriculovertebral dysplasia. J. Pediatr. 63:991, 1963. 6. Gorlin, R. J., Pindborg, J. J., and Cohen, M. M.: Syndromes of the Head and Neck. New York, McGraw-Hill, 1976, pp. 546-552. 7. Rees, T. D., and Coburn, R. J.: Silicone treat ment of partial lipodystrophy. J.A.M.A. 230:868, 1974. 8. Shumway, E. A.: Association of optic neuritis, facial paralysis and facial hemiatrophy. Arch. Ophthalmol. 13:8, 1935. 9. Segal, P., Jablonska, S., and Mrzyglod, S.: Ocular changes in linear scleroderma. Am. J. Ophthalmol. 51:807, 1961. 10. Moura, R. A.: Progressive facial hemiatrophia. Report of a case showing ocular and neuro
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ophthalmologic changes. Am. J. Ophthalmol. 55:635, 1963. 11. Sugar, H. S., and Banks, T. L.: Fuchs' heterochromic cyclitis. Associated with facial hemiatrophy (scleroderma en coup de sabre). Am. J. Ophthalmol. 57:627, 1964. 12. Smith, B., and Guberina, C.: Coloboma in progressive hemifacial atrophy. Am. J. Ophthalmol. 84:85, 1977. 13. Lindemann, H. O.: Interessante befunde bei hemiatrophia facialis progressiva. Albrecht von Graefes Arch. Klin. Ophthalmol. 142:409, 1940. 14. Gorlin, R. J., Pindborg, J. J., and Cohen, M. M.: Syndromes of the Head and Neck. New York, McGraw-Hill, 1976, pp. 341-344. 15. Archambault, L. S., and Fromm, N. K.: Pro gressive facial hemiatrophy. Report of 3 cases. Arch. Neurol. Psychiatr. 27:529, 1932.
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16. Lauber, H.: Ein fall von hemiatrophia facialis progressiva mit beteiligung des auges. Z. Augenheilkd. 57:492, 1925. 17. Francois, J.: Heredity in Ophthalmology. St. Louis, C. V. Mosby, 1961, p p . 616-618. 18. Franceschetti, A., and Koenig, H.: L'importance du facteur heredodegeneratif dans Fhemia trophie faciale et progressive. Etude des complica tions oculaires dans ce syndrome. J. Genet. Hum. 1:27, 1952. 19. Rees, T. D., Ashley, F. L., and Delgado, J. P.: Silicone fluid injections for facial atrophy. A ten-year study. Plast. Reconstr. Surg. 52:118, 1973. 20. Rees, T. D., Ballantyne, D. L., and Haw thorne, G.: Silicone fluid research. A follow-up summary. Plast. Reconstr. Surg. 46:50, 1970.
S H E R R E L L J. A S T O N ,
AND T H O M A S D. R E E S ,
M.D.,
M.D.
New York, New York
Hemifacial wasting 1 - 3 of the skin, sub cutaneous tissue, muscle, and bone is most often diagnosed as progressive hemifacial atrophy (Romberg's disease) or localized scleroderma. The distinction usually depends on the absence or pres ence, respectively, of skin pigmentation and other inflammatory changes. It is often difficult, if not impossible, to differ entiate between the two. They may be different manifestations of the same disease, 3 ' 4 and the cause is unknown. Similar findings may be seen in hemi facial microsomia (first and second bran chial arch syndrome) and its variants such as Goldenhar's syndrome, 5 , 6 posttraumatic atrophy, and partial lipodystrophy (Barraquer-Simons disease). 3 , 7 Facial at rophy in partial lipodystrophy, however, is always bilateral and involves only adi pose tissue. Progressive hemifacial atrophy usually begins during the first or second decade of life, and occasionally, cases have start ed in middle or old age. The most com mon early sign is a painless cleft or fur row near the midline of the face. This coup de sabre marks the boundary be tween normal and atrophic tissue. The From the Departments of Ophthalmology (Dr. Muchnick), Manhattan Eye, Ear and Throat Hospi tal, and New York Hospital-Cornell Medical Center; the Department of Plastic Surgery (Drs. Aston and Rees), Manhattan Eye, Ear and Throat Hospital; and the Department of Surgery, Institute of Reconstruc tive Plastic Surgery (Drs. Aston and Rees), New York University Medical Center, New York, New York. This work was supported in part by the William Salomon Foundation, the Bienstock Foundation, the Lauder Foundation, and the Mrs. Charles Woody Fund (Dr. Rees). Reprint requests to Richard S. Muchnick, M.D., 111 E. 65th St., New York, NY 10021.
final degree of deformity varies widely. Some patients have marked progression with fulminating atrophy, whereas others have relatively minimal atrophy (micro form). In addition to facial wasting that may include the ipsilateral salivary glands and hemiatrophy of the tongue, varying de grees of ipsilateral or contralateral in volvement of the trunk and extremities occasionally occur. Unilateral involve ment of such organs as the brain, ear, larynx, esophagus, diaphragm, and kid ney, as well as other anomalies and condi tions, have been reported and summa rized. 3 Many ophthalmologic conditions have been described in patients with hemifa cial atrophy. Reported findings2,3 include enophthalmos, rarely exophthalmos, eye lid atrophy, loss of cilia, blepharoptosis, blepharophimosis, pupillary abnormali ties, Homer's syndrome, heterochromia, heterochromic cyclitis, various other in flammatory disorders, muscle pareses, retinal abnormalities, and phthisis. These findings have often been described in single case reports 8 - 1 2 and have thus not provided an overall clinical pattern be cause of the wide variability of manifesta tions. In the past, a wide variety of treatment techniques has been used. 1 The results have been generally poor. The recon struction of facial contour has been at tempted with grafts of fat, dermis and fat, fascia, bone, cartilage, and various alloplastic materials. In severe cases, particu larly when previous or repeated absorp tion of free grafts has occurred, pedicle flaps have been used.
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Fat, dermis and fat, fascia, and other soft tissue grafts are partially absorbed in all patients. These grafts may also partici pate in the underlying wasting process in patients with hemifacial atrophy. Bone grafts have met with some success in bviilding up atrophic maxillae. Synthetic implants such as polyethylene and polyvinyl often extrude. In recent years, we have been treating these patients with fluid silicone injec tions. The results have been extra ordinarily good and in marked contrast to the generally poor results of previous methods of treatment. We describe herein case histories of 11 of the 59 patients presently being fol lowed u p by us. The purpose is to obtain a more cohesive picture of the ocular abnormalities encountered in hemifacial atrophy and to review our experiences with fluid silicone injections.
primary position. This increased to 30 prism diop ters of left hyperphoria in right downgaze and decreased to 7 prism diopters of left hyperphoria in right upgaze. There was moderate underaction of both the left inferior oblique muscle and left superi or oblique muscle. In July 1977 the patient under went a 14-mm left superior oblique muscle tuck. After an initial overcorrection, this reduced the primary position deviation to 1 prism diopter of left hyperphoria and she became asyptomatic. Case 2—A 6-year-old girl (Fig. 1) was first seen by us in late 1974 with a three-year history of severe progressive right hemifacial atrophy involving the forehead, the temporal fossa, the orbit, the soft tissues of the cheek, the maxilla, the mandible, and the ala of the nose. Best corrected visual acuity was 6/6 (20/20) in both eyes with a refraction of R.E.: +2.50 and L.E.: +0.50. There was 6 mm of enoph thalmos. The right pupil was 3 mm, irregular, and poorly reactive. It did not dilate well with cycloplegics. The left pupil was 6 mm and normally reactive. The right eye felt slightly soft but applanation tonometry could not be performed because of inade quate cooperation. The right fundus revealed retinal striae extending in a spoke-like configuration from the fovea. The disk margin was blurred. The left fundus was normal. We believe the fundus changes may have been caused by hypotony or vitreous changes producing contraction of the internal limit ing membrane.
CASE REPORTS
Over the next 27 months the patient received 20.1 ml of fluid silicone injections to the right side of the face. The cosmetic result was good (Fig. 2). No attempt was made, however, to correct the enoph thalmos. Case 3—A 31-year-old woman had a 15-year histo ry of severe progressive right hemifacial atrophy with additional involvement of the right breast and buttock. The orbit, the temporal fossa, the soft tissues of the cheek, the maxilla, and the upper lip were all affected. Hertel exophthalmometry at base 90 was R.E.: 9.5 and L.E.: 13.5. A depressed superi or sulcus and diffuse, marked thinning of the right lower eyelid were noted. Cilia were sparse. Slit-lamp examination revealed a 1-mm central pigment de posit on the endothelium of the right cornea. The irides were brown-green but there were several sectoral patches of blue atrophic iris in the right eye. The largest was nasally and contained an area of transillumihation near the iris root. The heterochromia was first noticed by the patient three or four years previously. The patient was treated with 14.1 ml of fluid silicone injections to the temporal fossa, cheek, and upper lip for nine months with a good result. Case 4—A 15-year-old girl (Fig. 3) had the onset of mild left hemifacial atrophy at age 7 years with progression for three years. A typical coup de sabre deformity of the forehead developed that was treat ed with 8.5 ml of fluid silicone injections during a period of 12 months (Fig. 4). Additionally, she had moderate atrophy of the ala of the nose and mild involvement of the orbit. Best corrected visual acu ity was 6/4.5 (20/15) in both eyes with a refraction of
Case 1—In 1967 a 23-year-old woman had a mild left hemifacial atrophy, primarily involving the soft tissues of the cheek, the maxilla, and the forehead. T h e atrophy had begun at age 10 years and pro gressed for approximately three years. It then stabi lized except for mild increases after each of two pregnancies. She was treated with 11.6 ml of fluid silicone injections over 26 months with a good result. At age 26 years, the patient noted intermittent vertical diplopia that became increasingly frequent and finally constant by age 29 years. A depressed superior sulcus and 1.5 mm of enophthalmos was noted. The left upper eyelid was thin medially with an early coloboma. The left lower eyelid was atroph ic laterally. The left pupil was 0.5 mm larger than the right. There was 25 prism diopters of left hypertropia in the primary position. This increased to 25 to 30 prism diopters in right, upgaze and downgaze. There was moderate overaction of the left inferior oblique muscle and slight to moderate underaction of the left superior oblique muscle. Intraocular pressures were R.E.: 14 mm Hg and L.E.: 10 mm Hg. In late November 1973, the patient underwent a 10-mm left inferior oblique muscle recession. This reduced her primary position deviation to 4 prism diopters of left hyperphoria. She was markedly improved symptomatically and remained so until early 1977 when she began to note increasing asthenopia and recurrent diplopia. By June 1977 there was 13 prism diopters of left hyperphoria in the
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Fig. 1 (Muchnick, Aston, and Rees). Case 2. Se vere right hemifacial atrophy involving forehead, temporal fossa, orbit, soft tissues of cheek, maxilla, mandible, and ala of nose.
R.E.: - 1 . 0 0 + 0.75 x 75 degrees and L.E.: +0.50. There was 1.5 mm of left enophthalmos and a defect in the superior orbital rim medially. A slight lag of the left inferior rectus was noted. Applanation pres sures were R.E.: 12 mm Hg and L.E.: 9 mm Hg. Case 5—A 23-year-old man first consulted us in March 1975 with a history of severe left hemifacial atrophy beginning at age 10 years and stabilizing after four to five years. He had moderately severe involvement of the soft tissues of the cheek, the maxilla, and the ear, and severe involvement of the upper lip, the soft tissues of the chin, the mandible, and the tongue. Additionally, the patient had severe atrophy of the soft tissues of the left buttock and right flank. The orbital area was relatively spared, with mild left lower eyelid atrophy and loss of cilia being the only manifestation. He has received 22.9 ml of fluid silicone injections for 30 months and is still receiving treatment. Case 6—A 14-year-old girl (Fig. 5) had a four-year history of severe progressive left hemifacial atrophy involving the temporal fossa, the soft tissues of the cheek, the maxilla, and the upper lip. A biopsy revealed scleroderma. There was 1 mm of enoph thalmos, and a depressed superior sulcus. The globe
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Fig. 2 (Muchnick, Aston, and Rees). Case 2. Pa tient has received 20.1 ml of fluid silicone injections for 27 months.
was 1 mm lower than its fellow. The left pupil was slightly larger than the right. Applanation pressures were R.E.: 11 mm Hg and L.E.: 9 mm Hg. She was treated with 25.0 ml of fluid silicone injections to the temporal fossa, cheek, and upper lip for 38 months with a good result (Fig. 6). Case 7—A 15-year-old boy developed severe in flammatory left hemifacial atrophy with skin discol oration at age 4 years. It was diagnosed as scleroder ma and progressed until age 11 years (Fig. 7). He was one of the most severely affected patients we have seen, with almost complete atrophy of the soft tissues of the left side of the face. Only the forehead and nose were spared. Additionally, there was se vere atrophy of the maxilla and moderate atrophy of the mandible. Uncorrected visual acuity was 6/6 (20/20) in both eyes with a refraction of R.E.: +0.25 and L.E.: +1.25. There was 1 mm of left blepharoptosis with eyelid fissures measuring R.E.: 9 mm and L.E.: 8 mm. Levator muscle function was R.E.: 13 mm and L.E.: 8 mm. Marked left upper eyelid lag was noted. The left lower eyelid was diffusely atrophic with loss of cilia. Hertel exophthalmometry at base 90 was R.E.: 16 and L.E.: 9. The superior and inferior sulci were markedly depressed. The eyes were orthophoric in the primary position.
Fig. 3 (Muchnick, Aston, and Rees). Case 4. Mild left hemifacial atrophy with coup de sabre deformi ty of forehead. Ala of nose and orbit also show involvement.
Fig. 4 (Muchnick, Aston, and Rees). Case 4. Pa tient has received 8.5 ml of fluid silicone injections to forehead for 12 months.
Fig. 5 (Muchnick, Aston, and Rees). Case 6. Se vere left hemifacial atrophy involving temporal fos sa, orbit, soft tissues of cheek, maxjlla, and upper lip.
Fig. 6 (Muchnick, Aston, and Rees). Case 6. Pa tient has received 25.0 ml of fluid silicone injections for 38 months.
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Fig. 7 (Muchnick, Aston, and Rees). Case 7. Se vere inflammatory left hemifacial atrophy with skin discoloration. Only forehead a n d nose spared.
Fig. 8 (Muchnick, Aston, and Rees). Case 7. Pa tient has received 51.1 ml of fluid silicone injections and is still receiving treatment.
There was moderate underaction of the left inferior oblique muscle and slight underaction of the left superior rectus, left medial rectus, and left lateral rectus muscles. Intraocular pressures were R.E.: 8 mm Hg and L.E.: 5 mm Hg. Indirect ophthalmoscopy revealed a lightly pigmented retinal pigment epithelium and prominent choroidal pattern in the left eye and a normal right eye. He has received 51.1 ml of fluid silicone injections (Fig. 8) since age 11 years and is still in treatment. He has also had an otoplasty. Case 8—A 16-year-old girl had the onset of mod erate right hemifacial atrophy at age 4 years with progression until age 9 years. The atrophy affected all the soft tissues of the face, with bony involve ment of the zygoma and maxilla. She had received 30.0 ml of fluid silicone injections during the past 82 months with significant improvement. The right lower eyelid was diffusely atrophic with marked loss of cilia. The right globe was 4 mm lower and there was 3 mm of enophthalmos. Applanation pressures were R.E.: 10 mm Hg and L.E.: 14 mm Hg. Case 9—A 22-year-old man had the onset of severe left hemifacial atrophy at age 16 years with progres sion for four years. He had diffuse involvement of the soft tissues of the face as well as bony involve ment of the zygoma and maxilla. He had an iliac
bone graft to his left orbital floor shortly before we first saw him. This improved intermittent vertical diplopia that he had been experiencing for about four years. Best corrected visual acuity was R.E.: 6/4.5 (20/15) and L.E.: 6/7.5-(20/25-) with a refrac tion of R.E.: - 5 . 7 5 + 0.75 x 85 degrees and L.E.: - 4 . 0 0 + 2.50 x 80 degrees. There was 5 mm of enophthalmos, and a markedly depressed superior sulcus. The globe was 3 mm lower. The right pupil was 4 mm and normally reactive. The left was 8 mm and fixed. The left pupil constricted with 0.25% pilocarpine; the right did not. A slight left hypophoria in the primary position was noted. This increased to 20 prism diopters of left hypotropia in upgaze. There was moderate restriction of elevation in the left eye and slight restriction of adduction and abduction. Intraocular pressures were R.E.: 12 mm Hg and L.E.: 8 mm Hg. The fundi revealed early myopic changes. He received 10.0 ml of fluid sili cone injections for 12 months with a good result. Case 10—A 7-year-old boy recently had a threeyear history of severe progressive right hemifacial atrophy. There was near total atrophy of the subcu taneous tissue of the cheek, as well as severe in volvement of the temporal fossa, the zygoma, and the maxilla. Moderate involvement of the tongue and mandible was noted. The orbital area was
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spared; the results of the eye examination were normal. He has not yet received any treatment. Case 11—An 11-year-old girl had the onset of severe left hemifacial atrophy at age 3 years with progression until age 8 years. She had severe in volvement of the soft tissues of the cheek and chin, the ala of the nose, and the upper lip. Milder involvement of the forehead, the zygoma, and the maxilla was noted. One millimeter of enophthalmos and slightly depressed superior and inferior sulci were evident. The right lower eyelid was slightly thin laterally. The left iris was slightly greener than the right. She was treated with 9.5 ml of fluid silicone injections for 22 months with significant improvement. DISCUSSION
The 11 patients in this series were se lected to show the various ocular abnor malities encountered in hemifacial atro phy. Although previous studies 3 , 1 3 indi cated that just over 10% of the patients had ocular findings, a preliminary screen ing of our 59 patients suggests a 35 to 40% incidence of such abnormalities. Whether this represents a true difference, is a result of our being referred more severely af fected patients, or a more careful ocular evaluation is uncertain. There were seven women and four men in this series. This agrees with Rogers' 1 finding that the disease occurs more frequently in women in a ratio of 3:2. He reviewed 670 previously reported cases. In our series the left side was affected in seven patients and the right side in four patients. Rogers found a slight predilection for the left side. The difference however, was not statistically significant. The patients in this series show the variable expression of hemifacial atrophy. They range from mild relatively diffuse involvement (Case 1) to moderate diffuse (Case 8) to severe diffuse (Case 9) to localized (Case 4) involvement. The other patients show still other degrees of in volvement. The patients also exhibit the variable degree of ocular abnormality. They range from no involvement (Case 10) to mild (Cases 5, 6, and 11) to moder
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ate (Cases 1, 4, and 8) to severe (Cases 2, 3, 7, and 9) involvement. The most common manifestation of oc ular involvement was enophthalmos. It was present in nine of the ten patients with ocular findings and ranged from 1 mm (Cases 6 and 11) to 7 mm (Case 7). Enophthalmos is the result of loss of orbital fat. 14 Changes in the eyelids were also com mon. Six patients (Cases 1, 3, 5, 7, 8, and 11) had varying degrees of lower eyelid atrophy. One patient (Case 1) had upper eyelid involvement as well. This apparent predilection for the lower eyelid has not, to our knowledge, been previously ob served. The changes ranged from mild localized atrophy (Case 11) to more ad vanced localized atrophy forming an early coloboma 12 (Case 1) to diffuse marked loss of entire eyelid substance and cilia (Cases 3, 7, and 8). One patient (Case 7) had mild blepharoptosis with decreased levator muscle function and eyelid lag on downgaze. This may be caused by involvement of the levator muscle in the atrophy. 9 , 1 5 Flattening of the maxilla occurred in all but the most mildly affected patient (Case 4). The zygoma was similarly involved in four of the more severely affected patients (Cases 8, 9, 10, and 11). This bony in volvement contributed to flattening of the cheek. In three patients (Cases 6, 8, and 9), the maxillary involvement progressed to inferior orbital rim and floor defects severe enough to cause the globe to be lower than its fellow. One of these pa tients (Case 9) required an iliac bone graft to the orbital floor. One patient (Case 4) had a superior orbital rim defect. Visual acuity was uniformly good. Only one patient (Case 9) with ocular involvement had a visual acuity as low as 6/7.5 (20/25). Several patients with severe involvement (Cases 2, 3, and 7) had visual acuity of 6/6 (20/20). Thus, despite occa sional reports of visual loss from
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phthisis 1 3 ' 1 6 and fundus abnormalities, 1 0 the prognosis for vision seems to be good. Four patients (Cases 2, 4, 7, and 9) were more hyperopic or less myopic in the affected eye. In two patients (Cases 1 and 6) the pupil on the affected side was slightly larger than on the unaffected side, but with normal reactions. One severely af fected patient (Case 9) had a fixed dilated pupil that constricted with 0.25% pilocarpine. Another (Case 2) had a miotic, irreg ular, poorly reactive pupil. These proba bly represent a tonic pupil and an Argyll Robertson pupil, respectively. Previous reports have also indicated that pupillary abnormalities are common and varied. Lindemann 1 3 described pu pillary disturbances in 11 of 19 patients with ocular involvement. A dilated pupil on the affected side more often than a miotic pupil was noted. Tonic pupils and Argyll Robertson pupils have been previ ously described. 1 7 Homer's syndrome, re portedly common, 1 5 ' 1 8 did not appear in our patients. The different pupillary ab normalities are probably caused by dif ferent lesions of the sympathetic or parasympathetic nervous system, or both. Iris abnormalities were noted in two patients. In one (Case 11) the iris on the affected side was slightly greener than on the unaffected side. In the other (Case 3) patches of sectoral iris atrophy were noted. In both, pupillary reactions were normal and there was no iritis. Heterochromia 1 8 and sectoral iris atrophy 9 have been previously found in hemifacial atro phy. Sugar and Banks 1 1 reported a case of heterochromic cyclitis. Additionally, they reviewed two cases of heterochromia and 12 cases of heterochromic cyclitis. They suggested that both the heterochromic cyclitis and the hemifacial atrophy may be the result of neurovascular or neurotrophic changes resulting from distur bances of the sympathetic nervous sys tem.
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Five of the seven patients with signifi cant orbital involvement (more than 1 mm of enophthalmos) had intraocular pres sures at least 3 mm Hg lower on the affected side than on the unaffected side. The child (Case 2) in whom intraocular pressure could not be measured had a soft eye on palpation. This relative hypotony has not, to our knowledge, been previous ly reported although rare instances of phthisis have. 13 ' 16 The origin of the hy potony is not clear. Hypotony, like het erochromic cyclitis, may be the result of neurovascular or neurotrophic changes. Two patients (Cases 1 and 9) had sig nificant vertical motility disturbances that required surgery. Another two (Cases 4 and 7) had milder motility disturbances that did not cause diplopia or require treatment. Limitation of ocular move ments has been previously reported as a common finding. 13 It may be secondary to mechanical restrictions, involvement of the muscles in the atrophy, or cranial nerve pareses. Two patients with severe ocular in volvement had ipsilateral fundus abnor malities. One patient (Case 2) had retinal striae extending in a spoke-like configura tion from the fovea and a blurred disk margin. These changes may have been caused by hypotony or a vitreous abnor mality producing contraction of the inter nal limiting membrane. The other (Case 7) had a lightly pigmented retinal pig ment epithelium. Fundus changes appear to be uncommon although a wide variety have been reported and reviewed. 2 ' 9 ' 10 We could not find any previous reports on findings similar to the ones we observed. Fluid silicone injection has become the major modality in our treatment of hemi facial atrophy. Earlier results have been previously reported. 7 ' 19 The treatment re mains investigational. The technique in volves the use of small volume injections repeated many times at two- to four-week intervals. The patients receive 0.5 to
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2.0 ml of high viscosity medical grade silicone subcutaneously to the affected area of the face. The fluid silicone is injected aseptically through a 26- or 27gauge needle. While the injection is being made with moderate pressure the needle is continuously moved through the sub cutaneous space to insure an even spread. The needle is aspirated frequently to make sure that it is not in the lumen of a small vessel. After the injection, the treat ed area is lightly but thoroughly mas saged to disperse the silicone further. This technique results in a fine droplet dispersion of the silicone in the subcuta neous tissues. 20 Mild fibroplasia is stimu lated and the silicone becomes fixed in a fine reticular fibrous network. Thus, the small volume of individual injections, the massage, and the two- to four-week inter vals between injections all help to elimi nate the migration or drifting that was a problem in early fluid silicone treatment. The use of medical grade silicone has eliminated granuloma formation. Com plications have been limited to an occa sional hematoma and occasional tissue firmness. The results have been generally excel lent with marked improvement of facial contour, normal animation, and no tissue firmness. The amount of fluid silicone injected in the 11 patients in this series ranged from 8.5 ml (Case 4) to 51.1 ml (Case 7). The latter was given for 48 months. No injections have been made into the eyelids because of possible dan ger to the globe. Similarly, no attempt has been made to correct enophthalmos. We do not believe that a safe, effective proce dure is presently available. Other adjunct procedures have been performed as re quired in individual patients. These have included strabismus surgery, otoplasty, and iliac bone grafts. The bone grafts have been to the orbital floor to improve the position of the globe and to the anteri
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or maxilla to further improve facial con tour. SUMMARY
Enophthalmos, flattening of the maxilla that may progress to inferior orbital rim and floor defects, eyelid atrophy, and slight relative hypotony occurred in pa tients with hemifacial atrophy. Less com mon manifestations included pupillary and iris abnormalities, vertical muscle imbalances, and retinal changes. The prognosis for vision was good. Fluid sili cone injection was the major modality in treatment and the results were generally excellent. ACKNOWLEDGMENT
Medical grade silicone fluid, dimethylpolysiloxane, MDX4-4011, was supplied by Dow Corning Corporation under a Food and Drug Administration investigational permit in a program sponsored by the American Society of Plastic and Reconstructive Surgeons.
REFERENCES 1. Rogers, B. O.: Progressive facial hemiatrophy. Romberg's disease. A review of 772 cases. In Broadbent, T. R. (ed.): Transactions of the Third Interna tional Congress of Plastic Surgery. Amsterdam, Excerpta Medica, 1964, p. 681. 2. Walsh, F. B., and Hoyt, W. F.: Clinical Neuro-ophthalmology, vol. 1. Baltimore, Williams and Wilkins, 1969, pp. 970-974. 3. Rees, T. D.: Facial atrophy. Clin. Plast. Surg. 3:637, 1976. 4. Wartenberg, R.: Progressive facial hemiatro phy. Arch. Neurol. Psychiatr. 54:75, 1945. 5. Gorlin, R. J., Jue, K. L., Jacobsen, U., and Goldschmidt, E.: Oculoauriculovertebral dysplasia. J. Pediatr. 63:991, 1963. 6. Gorlin, R. J., Pindborg, J. J., and Cohen, M. M.: Syndromes of the Head and Neck. New York, McGraw-Hill, 1976, pp. 546-552. 7. Rees, T. D., and Coburn, R. J.: Silicone treat ment of partial lipodystrophy. J.A.M.A. 230:868, 1974. 8. Shumway, E. A.: Association of optic neuritis, facial paralysis and facial hemiatrophy. Arch. Ophthalmol. 13:8, 1935. 9. Segal, P., Jablonska, S., and Mrzyglod, S.: Ocular changes in linear scleroderma. Am. J. Ophthalmol. 51:807, 1961. 10. Moura, R. A.: Progressive facial hemiatrophia. Report of a case showing ocular and neuro
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ophthalmologic changes. Am. J. Ophthalmol. 55:635, 1963. 11. Sugar, H. S., and Banks, T. L.: Fuchs' heterochromic cyclitis. Associated with facial hemiatrophy (scleroderma en coup de sabre). Am. J. Ophthalmol. 57:627, 1964. 12. Smith, B., and Guberina, C.: Coloboma in progressive hemifacial atrophy. Am. J. Ophthalmol. 84:85, 1977. 13. Lindemann, H. O.: Interessante befunde bei hemiatrophia facialis progressiva. Albrecht von Graefes Arch. Klin. Ophthalmol. 142:409, 1940. 14. Gorlin, R. J., Pindborg, J. J., and Cohen, M. M.: Syndromes of the Head and Neck. New York, McGraw-Hill, 1976, pp. 341-344. 15. Archambault, L. S., and Fromm, N. K.: Pro gressive facial hemiatrophy. Report of 3 cases. Arch. Neurol. Psychiatr. 27:529, 1932.
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16. Lauber, H.: Ein fall von hemiatrophia facialis progressiva mit beteiligung des auges. Z. Augenheilkd. 57:492, 1925. 17. Francois, J.: Heredity in Ophthalmology. St. Louis, C. V. Mosby, 1961, p p . 616-618. 18. Franceschetti, A., and Koenig, H.: L'importance du facteur heredodegeneratif dans Fhemia trophie faciale et progressive. Etude des complica tions oculaires dans ce syndrome. J. Genet. Hum. 1:27, 1952. 19. Rees, T. D., Ashley, F. L., and Delgado, J. P.: Silicone fluid injections for facial atrophy. A ten-year study. Plast. Reconstr. Surg. 52:118, 1973. 20. Rees, T. D., Ballantyne, D. L., and Haw thorne, G.: Silicone fluid research. A follow-up summary. Plast. Reconstr. Surg. 46:50, 1970.
