CRANIOFACIAL RECONSTRUCTION: OCULAR MANAGEMENT OF ORBITAL HYPERTELORISM* BY Lois A. Lloyd, MD HYPERTELOISM IS DEFINED AS AN INCREASED DISTANCE BETVEEN ANY PAIRED organs. Orbital hypertelorism, an indicator of craniofacial anomaly,
produces a significant cosmetic facial defect (Figure 1). DIAGNOSIS
Orbital hypertelorism, once called "ox-eyed" by Grieg,l may be confused with canthus inversus, blepharophimosis, flattening of the nasal root, or epicanthal folds. Only accurate measurement of the interorbital distance on the skin surface and roentgenographically can prove hypertelorism. NORMAL INTERORBITAL DISTANCE
Currarino and Silverman2 measured the distance between the right and left orbital dacryon in skull roentgenograms of 250 normal children between birth and 12 years ofage. The average readings are shown in Table I. Above the age of 2 years the range is from 18 to 26 millimeters. Between 3 and 12 years a difference of up to five mm (25%) is insignificant. Growth curve graphs have been published for normal intercanthal distances with percentile standards in Figure 2.3 Roentgenographic methods of measuring hypertelorism are difficult if the dacryon is masked by ethmoidal air cells and basal tomograms are not available. Similarly magnified photographs fail to demonstrate the facial midline accurately making photographic intercanthal measurements unreliable. *From the Department of Ophthalmology, The Hospital for Sick Children, Toronto,
Ontario, Canada. TR. AN1.
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FIGURE 1
Child with a craniofacial anomaly showing orbital hypertelorism.
The measurement for the degree of hypertelorism can be determined by measuring with calipers the distance between the inner canthi.4 (Figure 3A and 3B) The result is expressed as the canthal index.5 The Inner Canthal Distance The Outer Canthal Distance
TABLE 1: NORMAL INTERORBITAL DISTANCE
Year 1 2 3 5
7 10 12
Millimeters 10.5 20.5 21 22 23 25 26
Hypertelorism
125
INTER CANTHAL DISTANCE
97%
mm
PERCENTILE
3.575% 50%
25% 3%
6 9 12 15 18 21 2 4 MONTHS AGE FIGURE
6
8
14
YEARS 2
Normal intercanthal distances with percentile standards.
Values over 40 are considered hyperteloric. We have found the average normal child to have an index of 36 or less.6 Clinical measurements alone, however, are an inadequate criteria for recommending surgery. The relationship between the interorbital distance and the width of the skull, that is, the general proportions of the child's head and face, his social acceptance by parents, teachers, and friends as well as his success in maintaining binocular vision are the real indications for cosmetic surgery.7 Tessier8 classified hypertelorism into three degrees by interorbital roentgenographic measurements (Table II). It is the third degree which identifies the gross facial disfigurement for which surgical treatment is indicated.
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FIGURE 3
A: The distance between outer canthi (Ex-Ex), inner canthi (En-En) and length of the palpebral fissures (Ex-En). B: Diagram showing the level of the palpebral fissures - the distance between the two lines is the measurement of the dislocation of the palpebral fissures in the frontal plane.
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TABLE II: HYPERTELORISM
Degree
Interorbital Distance (mm)
I. Slight II. Moderate III. Severe
30 to 34 35 to 39 40+
CAUSE
Orbital hypertelorism has been ascribed to arrested development of the first branchial arch, producing a deficiency in the midline which allows the brain to herniate forward and downward and to intrude between the orbits to prevent their forward growth during embryonic and fetal life. Tessier9 has postulated 14 facial clefts, many of them associated with well known congenital syndromes (Figure 4). These syndromes include anomalies such as hair lip, cleft palate, and bifid nose in the vertical and oblique planes. Some cases of hypertelorism are associated with craniostenosis, decreasing the volume of the cranial vault, occasionally increasing intracranial pressure and sometimes producing deformities of Crouzon's or Apert's syndromes ("Croupert's" syndrome). Hypertelorism seldom exists by itself. Associated cranial anomalies include encephaloceles deforming the malar, sphenoidal, and frontal bones; brachycephaly, stretching the supraorbital arch and flattening the supraorbital rims; or gigantic frontal bone pneumatization and widened ethmoidal cells displacing the orbits laterally. Besides hypertelorism, the ocular findings include antimongoloid slants of the palpebral fissures, ptosis and colobomata of the eyelids, and displacement of the globes vertically or obliquely as well as laterally. Strabismus is frequent and often associated with some mechanical limitation of the ocular movements. Visual disorders include a decrease in acuity, colour vision, and visual fields. Ophthalmoscopic changes consist of papilledema, optic atrophy, or congenital retinal anomalies. CLINICAL MATERIALS AND METHODS
Since 1971, preoperative examinations of 63 patients in the clinics at The Hospital for Sick Children, Toronto, have been carried out. Many types of congenital anomalies have been noted by a team of specialists (Table III). We develop a topographical map of the face, plan the exact surgery to be done to correct the defects present, record results and complications of the surgery, and supply support to the patient throughout his ordeal.
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FIGURE 4
Craniofacial clefts 0 to 14 as postulated by Tessier.9
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TABLE III: CRANIOFACIAL RECONSTRUCTION TEANM
Plastic surgeon Neurosurgeon Neuro-oph thalmologist Neuro-otologist Dentist Radiologist Psychiatrist
Psychologist Geneticist Anthropologist Medical photographer Speech Therapist Social worker Cosmetologist
The medical illustrator projects the possible changes by imposing an overlay on a photograph of the patient. Of tremendous help to the planning is a styrofoam model of the patient's face (Figure 5). The contours outlined from stereophotographs of the face are transferred to terraced models done to actual scale. The model can be cut in all dimensions and the parts moved to correct the facial deformity. The special roentgenograms required are listed in Table JV.7 THE OCULAR EXAMINATION
Besides the clinical assessment of the orbital and periorbital bony deformities the following observations are made: external measurements (mm) of the inner canthal distance, the outer canthal distance, the pal-
FIGURE 5
Photographic overlay and styrofoam model of a patient's face prior to reconstrucive craniofacial surgery.
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TABLE IV: NEURORADIOGRAPHY
Skull views Basal Lateral Towne
Facial views Caldwell Lateral Stereo/Waters (25% & 301%)
Orbital Tomograms Basal AP Lateral As indicated Cerebral arterioyam
Pneumoncephlogram Radio-isotope study of cranial structures
pebral fissures - their length, width and shape, the interpupillary distance, the vertical displacements of the palpebral fissures and exophthalmous (Hertel and Luedde exophthalmometer). The ocular examination includes visual acuity, refraction (cycloplegic), colour vision (Hardy-RandRittler plates or Ishihara test) and visual fields. The extraocular muscles are examined for ductions, strabismus, nystagmus, fusion and stereopsis. The fundus is examined by direct and indirect ophthalmoscopy. Once plans are complete, the social and psychological factors considered, correction is undertaken. OPERATIV'E PROCEDURE
A combined intracranial and extracranial approach is made by the neurosurgeon and the plastic surgeon. A scalp incision from ear to ear is made by the neurosurgeon. The skin is turned forward over the face. A frontal bone flap is raised so that the frontal lobes can be retracted from the orbital roofs. The bony forehead can then be advanced or recessed as necessary at this stage. The plastic surgeon separates the orbital soft tissues from the bony orbits by subperiostial dissection using an incision through the lower fornices and proceeding as far back as the superior orbital fissures. The apex of the orbits and orbital nerves remain untouched. The incisions through the bony orbital walls anterior to the superior orbital fissures allow displacement of the orbits as a bony box-like unit in any direction - medially, upwards, downwards, or obliquely (Figure 6). The predetermined width of the nasal bone and ethmoidal cells is removed. A glabellar part of the frontal bone and the anterior cranial fossa anterior to the cribiform plate is left intact. The reconstructed bony walls are wired together through the supraorbital ridges and to the frontal bone. Bone chips from the patient's own iliac crest fill any bony gaps in the orbital walls. The medial canthal ligaments are also wired to each other by drilling through the anterior lacrimal crests and the soft
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FIGURE 6
Skull to show the possible directions of orbital displacement in reconstructive craniofacial surgery.
tissues are similarly wired. Excess skin and tissue over the roof of the nose and glabellar areas are resected and the upper canthal fold is corrected if necessary. Complete reconstruction takes place in a single operation. In patients with a recessed midface, the entire midfacial skeletal block may be freed, advanced and rotated downward to repair maxillary malocclusion. The dentist wires the mandibular arch to the frontal bone to maintain the condyle in the glenoid fossa. Hypotensive anesthesia is used. One hundred and fifty percent replacement of the patient's blood volume may be required. A tracheotomy is necessary. The procedure, originally 12 hours, now takes 10 hours or less. The child remains in the intensive care unit for one week. The
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TABLE V: CRANIOFACIAL ANOMALIES
Syndrome Crouzon's Apert's Multiple congenital anomalies Total
Number of Cases
Hypertelorism
Exorbitism
11 12 40 63
11 12 26 49
11 12 16 39
orthodontist controls the position of the intermaxillary fixation for 6 to 8 weeks. Ocular surgery to correct muscular imbalance usually follows in about 6 months. RESULTS
Since 1971, 63 patients have been referred to The Hospital for Sick Children, Toronto, for assessment of craniofacial anomaly. Their ages ranged from 5 months to 33 years. There was a preponderance of females 38 females, 25 males. Hypertelorism was present in 49 patients. The other patients have remained in the craniofacial study because of their interesting ocular congenital anomalies (Table V). Exorbitism in 39 patients was defined as a clinical sign when the Hertel or Luedde exophthalmometer measurement from the lateral orbital margin to the front of the cornea was greater than 12 mm.9 Other anomalies of the orbits included an anti-mongoloid slant, dystopia, and colobomata. Of the 10 patients with ptosis, surgery had been performed in one by a fascial sling operation and in another by a levator resection prior to craniofacial reconstructive surgery. No strabismus could be elicited in 21 patients, but among the remaining 42, exotropia, the commonest type, occurred in 28 (Table VI). V patterns were very common. There was slight apparent paresis of abduction, and sometimes of elevation and depression or adduction associated with mechanical obstruction to muscle movements from orbital deformities. Overacting inferior oblique muscles and slight underaction of superior oblique muscles occurred frequently. Esotropia was found TABLE VI: CRANIOFACIAL ANOMALIES
Syndrome Crouzon's Apert's Multiple cranial anomalies Total
Esotropia 1 3 7 11
Strabismus Exotropia Hypertropia 7 1 8 2 13 3 28
Orthophoria 2 1 18 21
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TABLE VII: CRANIOFACIAL ANOMALIES
Number of Cases Visual field defects Homonymous hemianopia Upper field defect Central scotoma Fundus changes Congenital anomalies Optic atrophy Normal
1 1 1
16 14 33
in 11 patients preoperatively. Vertical imbalances as a main muscular imbalance occurred in three patients. Of the 63 patients, 21 were orthophoric, only three of these in the "Croupert's" group of patients. Among the patients in whom stereopsis could be assessed before operation it was present in ten patients and absent in nine. Nystagmus was present in 14 patients. In some a fine shimmering movement was noted during the fundus examination; others had a jerky component with wider swings on lateral gaze. Latent nystagmus occurred in four patients. Corneal ulceration with conjunctivitis was present in two patients prior to reconstructive surgery. Both had a decreased corneal sensation and required tarsorraphy. Mild conjunctivitis, frequently seen, was easily controlled by antibiotics.
Only three patients had abnormal visual fields (Table VII). One was a homonymous hemianopia in a young girl with dilated lateral ventricles. A unilateral upper visual field defect and a central scotoma were found in one patient. Approximately one half of the patients had normal fundi. Optic atrophy, usually bilateral, was common (14 patients). Also frequent were congenital anomalies of the fundi (16 patients). Some had congenital anomalous vessels branching at the optic disc and some glial proliferation over the nerve head resembling early papilledema but without hemorrhages or exudates. Excess pigment was TABLE VIII: CRANIOFACIAL ANOMALIES
Number of Cases Vision Normal Unilateral amblyopia Bilateral amblyopia Refractive errors Emmetropia Hyperopia Myopia Astigmation
38 16 9 18 16 6 6
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TABLE IX: EFFECT OF RECONSTRUCTIVE SURGERY ON STABISMUS THE HOSPITAL FOR SICK CHILDREN, 1971 TO 1974
Number of Strabismus Orthophoria Exotropia Esotropia Hypertropia Tot
Cases 5 16 8 2 31
Improved Worse
Esotropia
Exotropia
2
4 1
2 1
6
5
3
6
No Change 3 0 5 1 9
2
Insufficient Follow-up 4 1 1 6
often seen at the borders of the optic discs. A chorioretinal coloboma was present in one child. Visual acuity was normal in both eyes in slightly more than half of the patients (38) (Table VIII). Unilateral amblyopia occurred in 25% (16 patients); bilateral decrease in vision between 20/40 and 20/100 was present in nine patients. In the very young children vision could only be estimated by pupillary reflexes to light or to the child's ability to fix on and follow a light. Similarly colour vision tests were only reliable in older children where co-operation was good enough to use the HardyRand-Rittler series of test plates or the Ishihara charts. Normal colour vision was found in all patients so tested. Nearly all these children had been considered retarded. Results of intelligence testing were falsely low because of the lack of experience imposed by extremely sheltered environments. Ratings improved with repeated testings.
OCULAR RESULTS OF RECONSTRUCTIVE SURGERY
Of the 63 patients, 31 have so far undergone craniofacial reconstruction. Those operated on ranged from 2 to 33 years of age (average 12.2 years). Eleven had Crouzon's and 12 Apert's syndrome, that is, half of them had TABLE X: OCULAR EFFECTS OF RECONSTRUCTIVE SURGERY THE HOSPITAL FOR SICK CHILDREN 31 PATIENTS, 1971 TO 1974
Number of Cases (pre op) Improved
Hypertelorism Exorbitism Nystagmus No stereopsis Vision
Normal Decreased
20 23 10 5
17 23
14 17
Worse 1
No Change 2
-
-
-
-
-
-
10 5
-
-
1
13 17
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135
"Croupert's" type of craniofacial deformity. The others had multiple craniofacial anomalies which could not be readily classified. The longest follow-up was 3% years after surgery. Postoperative intelligence testing has shown that the majority (28) in fact, have normal intelligence and are able to live useful lives. Although all patients operated upon were cosmetically improved, the hyperteloric state persisted in some. Exorbitism was improved by surgery in all patients. No corneal damage has resulted from this surgery. An increase in astigmatism was noted in one patient following surgery. The other refractive errors remained essentially unchanged. Surgery has decreased the vision of only one patient. (Tables IX and X)
DISCUSSION
In 400 cases in which Tessier has performed this operation throughout Europe and the United States, he has reported a 2% mortality and 5% infection rate, and 4 cases of blindness.9 One adult in our series suffered serious complications of the operation. She developed pseudomonas osteomyelities of the face and skull and lost vision in one previously normal eye to the degree that she now has only 20/40 vision in that eye. This loss has been ascribed to a drop in blood pressure during anesthesia inducing orbital edema and ocular ischemia in a proptotic eye. Notably, diplopia has not been a major problem postoperatively. It occurred in a 19-year-old boy whose strabismus changed from exotropia to esotropia. He has bilaterally overacting inferior oblique muscles but no ocular muscle paresis. His diplopia has been intermittant and is gradu-
ally subsiding. Tearing, a frequent complaint may be the result of the anti-mongoloid slant of the eyes due to the orbital dystopia, or it may result from nasolacrimal obstruction. The site of the lacrimal obstruction, if present, can be demonstrated by a lacrimal flow nuclear scan requiring just one drop of radionuclide in the conjunctival sac. Although all members of the team play an essential role in the examination and treatment of each patient, their findings are sometimes at odds. Dentists feel, that the optimum time for surgery is age 12 years when the dentition has been completed. Psychologists suggest that some children and their parents cannot accept such drastic surgery before the age of 8 years. However, early surgery may prevent the social problems encountered by children with grotesque disfigurement which make them recluses, rejected by parents, peers and teachers. Ophthalmologists
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Lloyd
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FIGURE 7
Preoperative appearance of a girl with a craniofacial anomaly showing orbital hypertelorism, ptosis and right esotropia.
urge that survey be undertaken early to allow normal development of binocular vision. In the fourth year of this study all but one of the patients so far have improved cosmetically, psychologically and economically. Institutionalized patients can now be adopted; uneducated children are becoming educated and independent (Figures 7 and 8). This multifacet approach brings up the philosophical problem of genetic counselling of the patients. The future holds a real problem for them. Until now, they never married or reproduced. Genetically, Crouzon's and Apert's syndromes carry a 50% recurrence risk. Will our
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137
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8
Postoperative appearance of the girl in Figure 7 showing corrected orbital hypertelorism, slight ptosis, and esotropia.
Lloyd 138 patients feel that they should have the right to subject half of their children to the possibility of this extensive surgical reconstruction which they themselves have undergone? CONCLUSION
Children with hypertelorism and exorbitism due to a number of cranial anomalies are disfigured members of society, open to ridicule and abuse. By well chosen radical surgery on the bones of the head and face, it is possible to improve their appearance significantly. This correction entails assessment and continued care by a large team involving several specialties. The procedures that are done are not without hazard but the results appear to greatly reward the endeavour. REFERENCES
1. Grieg DM: Hypertelorism: a hitherto undifferentiated craniofacial deformity. Edinburgh MedJ 31:560, 1924. 2. Currarino G, Silverman FN: Orbital hypotelorism, arhinencephaly, and trigonocephaly. Ra4liology 74:206, 1960. 3. Feingold M, Bossert WH: Normal values for selected physical parameters, an aid to syndrome delineation. Birth Defects 10: 1974. 4. Farkas LG, Lindsay WK: Morphology of the orbital region in adults following the cleft lip-palate repair in childhood. Am J Phys Anthropol 37:65, 1972. 5. Gorlin RJ, Pindborg JJ, McKusick VA (eds): Syndromes of the Head and Neck. Hypertelorism, New York, McGraw Hill Co, 1974, p 293. 6. Personal Communication: Dr. N. Rudd, The Hospital for Sick Children, Department of Genetics. 7. Munro I: Orbito-cranio-facial surgery. The team approach. Plast Reconstr Surg 55:170, 1975. 8. Tessier P: Orbital hypertelorism 1. Successive surgical attempts. Material and Methods. Causes and mechanisms. ScandJ Plast Reconstr Surg 6:135, 1972. 9. Tessier P: Personal Communication. Chicago meeting of Craniofacial reconstruction Teams, November, 1974.
DISCUSSION DR BYRON SMITH. I wish to congratulate Dr Lloyd, upon her preparation, and presentation of a number of detailed clinical findings in preoperative and postoperative cases of orbital hypertelorism. She has emphasized that the condition is a manifestation of any number of congenital malformation patterns or syndromes. The surgical treatment of orbital hypertelorism is the most radical of all cosmetic surgical procedures practiced by the medical profession, at this time. In the cases presented by Dr Lloyd, it was reiterated that optimal management necessitates the involvement of various specialites.
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139
The oculorotary disturbances are varied in direction and extent. In our series the deviations were unchanged by the cranial surgery in some of the patients. In others, there were changes. Some of them improved following cranial surgery. As in other intracranial conditions, muscle surgery is usually delayed for six months following cranial surgery. I wish to ask Dr Lloyd, if she has found any predictable changes in the muscle pattern following the cranial surgery? Also, has she any explanations for the changes in deviation coincident to cranial surgery? Dr Lloyd advised that the improvement following surgery was measured by the canthal indices. In our series, we have followed the postoperative course, by tabulating the changes in the vertical, horizontal, and sagittal planes by roentgenography. It is my impression that both the bony and soft tissue changes are worthy of tabulation. Dr Lloyd has added a great deal to the details of ocular findings in orbital hypertelorism. I hope that some of Dr Lloyd's publications upon this subject will eventually appear in the general plastic surgery literature. DR GOODWIN BREININ. Mr President. Mr Secretary. I very much enjoyed Doctor Lloyd's presentation and would like to amplify some of the eye findings from a study that is going on at the center for craniofacial anomalies at New York University. About 80 such procedures have been done which can be described as massive surgery. The extraocular findings in these patients are of considerable interest. [Slide] Orbital translocation, can be done in the horizontal, vertical, or in the sagittal direction; and we have reported on some 36 of these patients, most of whom show exotropia preoperatively. One of the lessons we have learned is that a child who is scheduled for such a procedure should not have extraocular muscle surgery done beforehand because the muscle balance may change significantly afterwards. I think Doctor Lloyd very clearly brought out that point. We found a remarkable trend toward esotropization; although most of the patients were initially exotropic, following surgery they become esotropic or less exotropic. [Slide] One can see in the midface hypoplasia group (CrouzonApert syndrome) that again we have a tendency toward exotropia; and that change, while not marked following surgery, again was in the direction of decreased exotropia. [Slide] Here is a young girl who exhibits hypertelorism before muscle surgery and before orbital translocation was done. [Slide] Here is the ocular result after the translocation. You can see she has undergone tremendous change of her ocular motor status with a large esotropia developing. [Slide] This is the subsequent repair of the strabismus which was required; and you can see that if she had had any operation beforehand, it would have been that much worse after the translocation surgery but she is thrilled with the present result. [Slide] Here we see the typical Apert syndrome with a V pattern; and, in general, we have noted a marked tendency toward V exotropia and sometimes V esotropia. [Slide] Here we can see in the various groups that V patterns are very much more frequent than any other type. Occasionally on A pattern may occur. These findings are possibly related to the fact that the superior oblique trochlea is disinserted during this procedure. In fact, all the soft tissue of the orbit is
Lloyd 140 held on a pedicle between the apex of the orbit, the optic foramen, and the nasolacrimal duct; and it just loosely sits there while bone is removed. This produces a characteristic overaction of the inferior obliques. In addition, we have frequently found postoperative ptosis, particularly laterally, which may be a consequence of the way of surgery is done; and finally, we also find that epiphora, dacryostenosis, and dacryocystitis are quite common, but may be very satisfactorily treated with dacryocystorhinostomy and/or conjunctival dacryocystorhinostomy. The procedure of orbital translocation, pioneered by Tessier in France and Converse in the United States, is clearly an important advance in treating these very distressing problems in bizarre looking patients. Even though they may not be beautiful, these children are materially improved psychologically by this surgery. Many of them, do indeed, think they are beautiful. I believe it is a wonderful advance in which ophthalmology has a very important part to play. I congratulate Dr Lloyd on her presentation of this significant work. DR DAVID KNOX. I have a limited experience. The management of these disorders is accomplished mainly by plastic surgeons and neurosurgeons. I do, however, have some fairly firm feelings. I think the Aperts and Crouzon's Syndromes have the risk of hereditary continuance, but some of the midline fusion problems do not have any evidence that they are hereditarily continued. During the surgery I think it is important that an ophthalmologist be there because the general surgical interns, assisting plastic and neurosurgeons, are used to holding on to retractors and pulling vigorously for periods of 5, 10, and 20 minutes without relaxing. I felt that I was there to protect the eye from the other surgeons because their use of the retractor could occlude the retinal vessels. The final point is the same that Dr Breinin made. At the time of initial surgery it may be necessary to do a dacryocystorhinostomy to establish or maintain patency of lacrimal drainage into the nose. Thank you. DR Lois A. LLOYD. May I thank all of those who were so kind to me in their remarks. I would like to include one point about the lacrimal drainage. I omitted it in the paper but we now use a radioisotope drop (a radionucleotide in the conjunctival sac), take x-rays and we can tell exactly, with no trauma to the child, where the lacrimal obstruction exists. Many of these children have dystropic misshapened orbits. We think it is usually a lack of drainage without any real obstruction. Therefore, nasolacrimal surgery might not be indicated in some of these patients. It is best to know why they are tearing. They tear because they have an anti-mongoloid slant to their eyes, and the tears are not draining in the proper direction in many of these children. As far as costs, we think the operation in Canada costs about $2,000 for each patient. This is for the whole procedure including follow-up studies. I realize this is less than it would be in the States. We think this is cheaper than keeping these children institutionalized for the rest of their lives. I do share some of the worries that have been mentioned. The muscle changes are probably also related to the misshapened orbits. I am not so sure about the trochlea being removed because, as you know, in many traumatic cases trochleas are removed without fourth nerve palsies occurring. Thank you very much.
produces a significant cosmetic facial defect (Figure 1). DIAGNOSIS
Orbital hypertelorism, once called "ox-eyed" by Grieg,l may be confused with canthus inversus, blepharophimosis, flattening of the nasal root, or epicanthal folds. Only accurate measurement of the interorbital distance on the skin surface and roentgenographically can prove hypertelorism. NORMAL INTERORBITAL DISTANCE
Currarino and Silverman2 measured the distance between the right and left orbital dacryon in skull roentgenograms of 250 normal children between birth and 12 years ofage. The average readings are shown in Table I. Above the age of 2 years the range is from 18 to 26 millimeters. Between 3 and 12 years a difference of up to five mm (25%) is insignificant. Growth curve graphs have been published for normal intercanthal distances with percentile standards in Figure 2.3 Roentgenographic methods of measuring hypertelorism are difficult if the dacryon is masked by ethmoidal air cells and basal tomograms are not available. Similarly magnified photographs fail to demonstrate the facial midline accurately making photographic intercanthal measurements unreliable. *From the Department of Ophthalmology, The Hospital for Sick Children, Toronto,
Ontario, Canada. TR. AN1.
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FIGURE 1
Child with a craniofacial anomaly showing orbital hypertelorism.
The measurement for the degree of hypertelorism can be determined by measuring with calipers the distance between the inner canthi.4 (Figure 3A and 3B) The result is expressed as the canthal index.5 The Inner Canthal Distance The Outer Canthal Distance
TABLE 1: NORMAL INTERORBITAL DISTANCE
Year 1 2 3 5
7 10 12
Millimeters 10.5 20.5 21 22 23 25 26
Hypertelorism
125
INTER CANTHAL DISTANCE
97%
mm
PERCENTILE
3.575% 50%
25% 3%
6 9 12 15 18 21 2 4 MONTHS AGE FIGURE
6
8
14
YEARS 2
Normal intercanthal distances with percentile standards.
Values over 40 are considered hyperteloric. We have found the average normal child to have an index of 36 or less.6 Clinical measurements alone, however, are an inadequate criteria for recommending surgery. The relationship between the interorbital distance and the width of the skull, that is, the general proportions of the child's head and face, his social acceptance by parents, teachers, and friends as well as his success in maintaining binocular vision are the real indications for cosmetic surgery.7 Tessier8 classified hypertelorism into three degrees by interorbital roentgenographic measurements (Table II). It is the third degree which identifies the gross facial disfigurement for which surgical treatment is indicated.
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FIGURE 3
A: The distance between outer canthi (Ex-Ex), inner canthi (En-En) and length of the palpebral fissures (Ex-En). B: Diagram showing the level of the palpebral fissures - the distance between the two lines is the measurement of the dislocation of the palpebral fissures in the frontal plane.
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TABLE II: HYPERTELORISM
Degree
Interorbital Distance (mm)
I. Slight II. Moderate III. Severe
30 to 34 35 to 39 40+
CAUSE
Orbital hypertelorism has been ascribed to arrested development of the first branchial arch, producing a deficiency in the midline which allows the brain to herniate forward and downward and to intrude between the orbits to prevent their forward growth during embryonic and fetal life. Tessier9 has postulated 14 facial clefts, many of them associated with well known congenital syndromes (Figure 4). These syndromes include anomalies such as hair lip, cleft palate, and bifid nose in the vertical and oblique planes. Some cases of hypertelorism are associated with craniostenosis, decreasing the volume of the cranial vault, occasionally increasing intracranial pressure and sometimes producing deformities of Crouzon's or Apert's syndromes ("Croupert's" syndrome). Hypertelorism seldom exists by itself. Associated cranial anomalies include encephaloceles deforming the malar, sphenoidal, and frontal bones; brachycephaly, stretching the supraorbital arch and flattening the supraorbital rims; or gigantic frontal bone pneumatization and widened ethmoidal cells displacing the orbits laterally. Besides hypertelorism, the ocular findings include antimongoloid slants of the palpebral fissures, ptosis and colobomata of the eyelids, and displacement of the globes vertically or obliquely as well as laterally. Strabismus is frequent and often associated with some mechanical limitation of the ocular movements. Visual disorders include a decrease in acuity, colour vision, and visual fields. Ophthalmoscopic changes consist of papilledema, optic atrophy, or congenital retinal anomalies. CLINICAL MATERIALS AND METHODS
Since 1971, preoperative examinations of 63 patients in the clinics at The Hospital for Sick Children, Toronto, have been carried out. Many types of congenital anomalies have been noted by a team of specialists (Table III). We develop a topographical map of the face, plan the exact surgery to be done to correct the defects present, record results and complications of the surgery, and supply support to the patient throughout his ordeal.
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Lloyd
FIGURE 4
Craniofacial clefts 0 to 14 as postulated by Tessier.9
Hypertelorism
129
TABLE III: CRANIOFACIAL RECONSTRUCTION TEANM
Plastic surgeon Neurosurgeon Neuro-oph thalmologist Neuro-otologist Dentist Radiologist Psychiatrist
Psychologist Geneticist Anthropologist Medical photographer Speech Therapist Social worker Cosmetologist
The medical illustrator projects the possible changes by imposing an overlay on a photograph of the patient. Of tremendous help to the planning is a styrofoam model of the patient's face (Figure 5). The contours outlined from stereophotographs of the face are transferred to terraced models done to actual scale. The model can be cut in all dimensions and the parts moved to correct the facial deformity. The special roentgenograms required are listed in Table JV.7 THE OCULAR EXAMINATION
Besides the clinical assessment of the orbital and periorbital bony deformities the following observations are made: external measurements (mm) of the inner canthal distance, the outer canthal distance, the pal-
FIGURE 5
Photographic overlay and styrofoam model of a patient's face prior to reconstrucive craniofacial surgery.
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130
TABLE IV: NEURORADIOGRAPHY
Skull views Basal Lateral Towne
Facial views Caldwell Lateral Stereo/Waters (25% & 301%)
Orbital Tomograms Basal AP Lateral As indicated Cerebral arterioyam
Pneumoncephlogram Radio-isotope study of cranial structures
pebral fissures - their length, width and shape, the interpupillary distance, the vertical displacements of the palpebral fissures and exophthalmous (Hertel and Luedde exophthalmometer). The ocular examination includes visual acuity, refraction (cycloplegic), colour vision (Hardy-RandRittler plates or Ishihara test) and visual fields. The extraocular muscles are examined for ductions, strabismus, nystagmus, fusion and stereopsis. The fundus is examined by direct and indirect ophthalmoscopy. Once plans are complete, the social and psychological factors considered, correction is undertaken. OPERATIV'E PROCEDURE
A combined intracranial and extracranial approach is made by the neurosurgeon and the plastic surgeon. A scalp incision from ear to ear is made by the neurosurgeon. The skin is turned forward over the face. A frontal bone flap is raised so that the frontal lobes can be retracted from the orbital roofs. The bony forehead can then be advanced or recessed as necessary at this stage. The plastic surgeon separates the orbital soft tissues from the bony orbits by subperiostial dissection using an incision through the lower fornices and proceeding as far back as the superior orbital fissures. The apex of the orbits and orbital nerves remain untouched. The incisions through the bony orbital walls anterior to the superior orbital fissures allow displacement of the orbits as a bony box-like unit in any direction - medially, upwards, downwards, or obliquely (Figure 6). The predetermined width of the nasal bone and ethmoidal cells is removed. A glabellar part of the frontal bone and the anterior cranial fossa anterior to the cribiform plate is left intact. The reconstructed bony walls are wired together through the supraorbital ridges and to the frontal bone. Bone chips from the patient's own iliac crest fill any bony gaps in the orbital walls. The medial canthal ligaments are also wired to each other by drilling through the anterior lacrimal crests and the soft
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131
FIGURE 6
Skull to show the possible directions of orbital displacement in reconstructive craniofacial surgery.
tissues are similarly wired. Excess skin and tissue over the roof of the nose and glabellar areas are resected and the upper canthal fold is corrected if necessary. Complete reconstruction takes place in a single operation. In patients with a recessed midface, the entire midfacial skeletal block may be freed, advanced and rotated downward to repair maxillary malocclusion. The dentist wires the mandibular arch to the frontal bone to maintain the condyle in the glenoid fossa. Hypotensive anesthesia is used. One hundred and fifty percent replacement of the patient's blood volume may be required. A tracheotomy is necessary. The procedure, originally 12 hours, now takes 10 hours or less. The child remains in the intensive care unit for one week. The
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TABLE V: CRANIOFACIAL ANOMALIES
Syndrome Crouzon's Apert's Multiple congenital anomalies Total
Number of Cases
Hypertelorism
Exorbitism
11 12 40 63
11 12 26 49
11 12 16 39
orthodontist controls the position of the intermaxillary fixation for 6 to 8 weeks. Ocular surgery to correct muscular imbalance usually follows in about 6 months. RESULTS
Since 1971, 63 patients have been referred to The Hospital for Sick Children, Toronto, for assessment of craniofacial anomaly. Their ages ranged from 5 months to 33 years. There was a preponderance of females 38 females, 25 males. Hypertelorism was present in 49 patients. The other patients have remained in the craniofacial study because of their interesting ocular congenital anomalies (Table V). Exorbitism in 39 patients was defined as a clinical sign when the Hertel or Luedde exophthalmometer measurement from the lateral orbital margin to the front of the cornea was greater than 12 mm.9 Other anomalies of the orbits included an anti-mongoloid slant, dystopia, and colobomata. Of the 10 patients with ptosis, surgery had been performed in one by a fascial sling operation and in another by a levator resection prior to craniofacial reconstructive surgery. No strabismus could be elicited in 21 patients, but among the remaining 42, exotropia, the commonest type, occurred in 28 (Table VI). V patterns were very common. There was slight apparent paresis of abduction, and sometimes of elevation and depression or adduction associated with mechanical obstruction to muscle movements from orbital deformities. Overacting inferior oblique muscles and slight underaction of superior oblique muscles occurred frequently. Esotropia was found TABLE VI: CRANIOFACIAL ANOMALIES
Syndrome Crouzon's Apert's Multiple cranial anomalies Total
Esotropia 1 3 7 11
Strabismus Exotropia Hypertropia 7 1 8 2 13 3 28
Orthophoria 2 1 18 21
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TABLE VII: CRANIOFACIAL ANOMALIES
Number of Cases Visual field defects Homonymous hemianopia Upper field defect Central scotoma Fundus changes Congenital anomalies Optic atrophy Normal
1 1 1
16 14 33
in 11 patients preoperatively. Vertical imbalances as a main muscular imbalance occurred in three patients. Of the 63 patients, 21 were orthophoric, only three of these in the "Croupert's" group of patients. Among the patients in whom stereopsis could be assessed before operation it was present in ten patients and absent in nine. Nystagmus was present in 14 patients. In some a fine shimmering movement was noted during the fundus examination; others had a jerky component with wider swings on lateral gaze. Latent nystagmus occurred in four patients. Corneal ulceration with conjunctivitis was present in two patients prior to reconstructive surgery. Both had a decreased corneal sensation and required tarsorraphy. Mild conjunctivitis, frequently seen, was easily controlled by antibiotics.
Only three patients had abnormal visual fields (Table VII). One was a homonymous hemianopia in a young girl with dilated lateral ventricles. A unilateral upper visual field defect and a central scotoma were found in one patient. Approximately one half of the patients had normal fundi. Optic atrophy, usually bilateral, was common (14 patients). Also frequent were congenital anomalies of the fundi (16 patients). Some had congenital anomalous vessels branching at the optic disc and some glial proliferation over the nerve head resembling early papilledema but without hemorrhages or exudates. Excess pigment was TABLE VIII: CRANIOFACIAL ANOMALIES
Number of Cases Vision Normal Unilateral amblyopia Bilateral amblyopia Refractive errors Emmetropia Hyperopia Myopia Astigmation
38 16 9 18 16 6 6
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134
TABLE IX: EFFECT OF RECONSTRUCTIVE SURGERY ON STABISMUS THE HOSPITAL FOR SICK CHILDREN, 1971 TO 1974
Number of Strabismus Orthophoria Exotropia Esotropia Hypertropia Tot
Cases 5 16 8 2 31
Improved Worse
Esotropia
Exotropia
2
4 1
2 1
6
5
3
6
No Change 3 0 5 1 9
2
Insufficient Follow-up 4 1 1 6
often seen at the borders of the optic discs. A chorioretinal coloboma was present in one child. Visual acuity was normal in both eyes in slightly more than half of the patients (38) (Table VIII). Unilateral amblyopia occurred in 25% (16 patients); bilateral decrease in vision between 20/40 and 20/100 was present in nine patients. In the very young children vision could only be estimated by pupillary reflexes to light or to the child's ability to fix on and follow a light. Similarly colour vision tests were only reliable in older children where co-operation was good enough to use the HardyRand-Rittler series of test plates or the Ishihara charts. Normal colour vision was found in all patients so tested. Nearly all these children had been considered retarded. Results of intelligence testing were falsely low because of the lack of experience imposed by extremely sheltered environments. Ratings improved with repeated testings.
OCULAR RESULTS OF RECONSTRUCTIVE SURGERY
Of the 63 patients, 31 have so far undergone craniofacial reconstruction. Those operated on ranged from 2 to 33 years of age (average 12.2 years). Eleven had Crouzon's and 12 Apert's syndrome, that is, half of them had TABLE X: OCULAR EFFECTS OF RECONSTRUCTIVE SURGERY THE HOSPITAL FOR SICK CHILDREN 31 PATIENTS, 1971 TO 1974
Number of Cases (pre op) Improved
Hypertelorism Exorbitism Nystagmus No stereopsis Vision
Normal Decreased
20 23 10 5
17 23
14 17
Worse 1
No Change 2
-
-
-
-
-
-
10 5
-
-
1
13 17
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135
"Croupert's" type of craniofacial deformity. The others had multiple craniofacial anomalies which could not be readily classified. The longest follow-up was 3% years after surgery. Postoperative intelligence testing has shown that the majority (28) in fact, have normal intelligence and are able to live useful lives. Although all patients operated upon were cosmetically improved, the hyperteloric state persisted in some. Exorbitism was improved by surgery in all patients. No corneal damage has resulted from this surgery. An increase in astigmatism was noted in one patient following surgery. The other refractive errors remained essentially unchanged. Surgery has decreased the vision of only one patient. (Tables IX and X)
DISCUSSION
In 400 cases in which Tessier has performed this operation throughout Europe and the United States, he has reported a 2% mortality and 5% infection rate, and 4 cases of blindness.9 One adult in our series suffered serious complications of the operation. She developed pseudomonas osteomyelities of the face and skull and lost vision in one previously normal eye to the degree that she now has only 20/40 vision in that eye. This loss has been ascribed to a drop in blood pressure during anesthesia inducing orbital edema and ocular ischemia in a proptotic eye. Notably, diplopia has not been a major problem postoperatively. It occurred in a 19-year-old boy whose strabismus changed from exotropia to esotropia. He has bilaterally overacting inferior oblique muscles but no ocular muscle paresis. His diplopia has been intermittant and is gradu-
ally subsiding. Tearing, a frequent complaint may be the result of the anti-mongoloid slant of the eyes due to the orbital dystopia, or it may result from nasolacrimal obstruction. The site of the lacrimal obstruction, if present, can be demonstrated by a lacrimal flow nuclear scan requiring just one drop of radionuclide in the conjunctival sac. Although all members of the team play an essential role in the examination and treatment of each patient, their findings are sometimes at odds. Dentists feel, that the optimum time for surgery is age 12 years when the dentition has been completed. Psychologists suggest that some children and their parents cannot accept such drastic surgery before the age of 8 years. However, early surgery may prevent the social problems encountered by children with grotesque disfigurement which make them recluses, rejected by parents, peers and teachers. Ophthalmologists
136
Lloyd
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FIGURE 7
Preoperative appearance of a girl with a craniofacial anomaly showing orbital hypertelorism, ptosis and right esotropia.
urge that survey be undertaken early to allow normal development of binocular vision. In the fourth year of this study all but one of the patients so far have improved cosmetically, psychologically and economically. Institutionalized patients can now be adopted; uneducated children are becoming educated and independent (Figures 7 and 8). This multifacet approach brings up the philosophical problem of genetic counselling of the patients. The future holds a real problem for them. Until now, they never married or reproduced. Genetically, Crouzon's and Apert's syndromes carry a 50% recurrence risk. Will our
Hypertelorism
137
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8
Postoperative appearance of the girl in Figure 7 showing corrected orbital hypertelorism, slight ptosis, and esotropia.
Lloyd 138 patients feel that they should have the right to subject half of their children to the possibility of this extensive surgical reconstruction which they themselves have undergone? CONCLUSION
Children with hypertelorism and exorbitism due to a number of cranial anomalies are disfigured members of society, open to ridicule and abuse. By well chosen radical surgery on the bones of the head and face, it is possible to improve their appearance significantly. This correction entails assessment and continued care by a large team involving several specialties. The procedures that are done are not without hazard but the results appear to greatly reward the endeavour. REFERENCES
1. Grieg DM: Hypertelorism: a hitherto undifferentiated craniofacial deformity. Edinburgh MedJ 31:560, 1924. 2. Currarino G, Silverman FN: Orbital hypotelorism, arhinencephaly, and trigonocephaly. Ra4liology 74:206, 1960. 3. Feingold M, Bossert WH: Normal values for selected physical parameters, an aid to syndrome delineation. Birth Defects 10: 1974. 4. Farkas LG, Lindsay WK: Morphology of the orbital region in adults following the cleft lip-palate repair in childhood. Am J Phys Anthropol 37:65, 1972. 5. Gorlin RJ, Pindborg JJ, McKusick VA (eds): Syndromes of the Head and Neck. Hypertelorism, New York, McGraw Hill Co, 1974, p 293. 6. Personal Communication: Dr. N. Rudd, The Hospital for Sick Children, Department of Genetics. 7. Munro I: Orbito-cranio-facial surgery. The team approach. Plast Reconstr Surg 55:170, 1975. 8. Tessier P: Orbital hypertelorism 1. Successive surgical attempts. Material and Methods. Causes and mechanisms. ScandJ Plast Reconstr Surg 6:135, 1972. 9. Tessier P: Personal Communication. Chicago meeting of Craniofacial reconstruction Teams, November, 1974.
DISCUSSION DR BYRON SMITH. I wish to congratulate Dr Lloyd, upon her preparation, and presentation of a number of detailed clinical findings in preoperative and postoperative cases of orbital hypertelorism. She has emphasized that the condition is a manifestation of any number of congenital malformation patterns or syndromes. The surgical treatment of orbital hypertelorism is the most radical of all cosmetic surgical procedures practiced by the medical profession, at this time. In the cases presented by Dr Lloyd, it was reiterated that optimal management necessitates the involvement of various specialites.
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The oculorotary disturbances are varied in direction and extent. In our series the deviations were unchanged by the cranial surgery in some of the patients. In others, there were changes. Some of them improved following cranial surgery. As in other intracranial conditions, muscle surgery is usually delayed for six months following cranial surgery. I wish to ask Dr Lloyd, if she has found any predictable changes in the muscle pattern following the cranial surgery? Also, has she any explanations for the changes in deviation coincident to cranial surgery? Dr Lloyd advised that the improvement following surgery was measured by the canthal indices. In our series, we have followed the postoperative course, by tabulating the changes in the vertical, horizontal, and sagittal planes by roentgenography. It is my impression that both the bony and soft tissue changes are worthy of tabulation. Dr Lloyd has added a great deal to the details of ocular findings in orbital hypertelorism. I hope that some of Dr Lloyd's publications upon this subject will eventually appear in the general plastic surgery literature. DR GOODWIN BREININ. Mr President. Mr Secretary. I very much enjoyed Doctor Lloyd's presentation and would like to amplify some of the eye findings from a study that is going on at the center for craniofacial anomalies at New York University. About 80 such procedures have been done which can be described as massive surgery. The extraocular findings in these patients are of considerable interest. [Slide] Orbital translocation, can be done in the horizontal, vertical, or in the sagittal direction; and we have reported on some 36 of these patients, most of whom show exotropia preoperatively. One of the lessons we have learned is that a child who is scheduled for such a procedure should not have extraocular muscle surgery done beforehand because the muscle balance may change significantly afterwards. I think Doctor Lloyd very clearly brought out that point. We found a remarkable trend toward esotropization; although most of the patients were initially exotropic, following surgery they become esotropic or less exotropic. [Slide] One can see in the midface hypoplasia group (CrouzonApert syndrome) that again we have a tendency toward exotropia; and that change, while not marked following surgery, again was in the direction of decreased exotropia. [Slide] Here is a young girl who exhibits hypertelorism before muscle surgery and before orbital translocation was done. [Slide] Here is the ocular result after the translocation. You can see she has undergone tremendous change of her ocular motor status with a large esotropia developing. [Slide] This is the subsequent repair of the strabismus which was required; and you can see that if she had had any operation beforehand, it would have been that much worse after the translocation surgery but she is thrilled with the present result. [Slide] Here we see the typical Apert syndrome with a V pattern; and, in general, we have noted a marked tendency toward V exotropia and sometimes V esotropia. [Slide] Here we can see in the various groups that V patterns are very much more frequent than any other type. Occasionally on A pattern may occur. These findings are possibly related to the fact that the superior oblique trochlea is disinserted during this procedure. In fact, all the soft tissue of the orbit is
Lloyd 140 held on a pedicle between the apex of the orbit, the optic foramen, and the nasolacrimal duct; and it just loosely sits there while bone is removed. This produces a characteristic overaction of the inferior obliques. In addition, we have frequently found postoperative ptosis, particularly laterally, which may be a consequence of the way of surgery is done; and finally, we also find that epiphora, dacryostenosis, and dacryocystitis are quite common, but may be very satisfactorily treated with dacryocystorhinostomy and/or conjunctival dacryocystorhinostomy. The procedure of orbital translocation, pioneered by Tessier in France and Converse in the United States, is clearly an important advance in treating these very distressing problems in bizarre looking patients. Even though they may not be beautiful, these children are materially improved psychologically by this surgery. Many of them, do indeed, think they are beautiful. I believe it is a wonderful advance in which ophthalmology has a very important part to play. I congratulate Dr Lloyd on her presentation of this significant work. DR DAVID KNOX. I have a limited experience. The management of these disorders is accomplished mainly by plastic surgeons and neurosurgeons. I do, however, have some fairly firm feelings. I think the Aperts and Crouzon's Syndromes have the risk of hereditary continuance, but some of the midline fusion problems do not have any evidence that they are hereditarily continued. During the surgery I think it is important that an ophthalmologist be there because the general surgical interns, assisting plastic and neurosurgeons, are used to holding on to retractors and pulling vigorously for periods of 5, 10, and 20 minutes without relaxing. I felt that I was there to protect the eye from the other surgeons because their use of the retractor could occlude the retinal vessels. The final point is the same that Dr Breinin made. At the time of initial surgery it may be necessary to do a dacryocystorhinostomy to establish or maintain patency of lacrimal drainage into the nose. Thank you. DR Lois A. LLOYD. May I thank all of those who were so kind to me in their remarks. I would like to include one point about the lacrimal drainage. I omitted it in the paper but we now use a radioisotope drop (a radionucleotide in the conjunctival sac), take x-rays and we can tell exactly, with no trauma to the child, where the lacrimal obstruction exists. Many of these children have dystropic misshapened orbits. We think it is usually a lack of drainage without any real obstruction. Therefore, nasolacrimal surgery might not be indicated in some of these patients. It is best to know why they are tearing. They tear because they have an anti-mongoloid slant to their eyes, and the tears are not draining in the proper direction in many of these children. As far as costs, we think the operation in Canada costs about $2,000 for each patient. This is for the whole procedure including follow-up studies. I realize this is less than it would be in the States. We think this is cheaper than keeping these children institutionalized for the rest of their lives. I do share some of the worries that have been mentioned. The muscle changes are probably also related to the misshapened orbits. I am not so sure about the trochlea being removed because, as you know, in many traumatic cases trochleas are removed without fourth nerve palsies occurring. Thank you very much.
