Aesthetic Improvement Resulting from Craniofacial Surgery in Craniosynostosis Syndromes Douglas K. Ousterhout ~, Karin Vargervik 2
1 Dept. of Surgery (Plastic) (Chairman: St. ])Aathes, M.D.) University of California, San Francisco, USA and
2 Dept. of Growth and Development, Craniofacial Anomalies Clinic (Director: Prof K Vargervik, D D S ) University of California, San Francisco, USA
Introduction
The functional improvements achieved by Le Fort III ad- vancement in individuals with craniosynostosis syndromes (Crouzon's, Pfeiffer's, and Apert's) are well established. With such surgery there is improvement in eye protection from below, opening of the nasopharynx, and establish- ment of an improved occlusion. Aesthetics are related to form and as a more normal form is obtained with cranio- facial reconstructive surgery, an improvement in appear- ance should result, allowing a better self image with asso- ciated psychological benefits. Objective studies on facial aesthetics resulting from improving the bony position and associated soft tissue drape in a series of such patients have not been found in the literature. Facial aesthetics are difficult to define but aberrations from the range of normal are rather easily detected subjec- tively as are improvements from surgical procedures. Many factors play a role including skin colour, thickness, contour, subcutaneous tissue, neuromuscular function, etc., but these factors are difficult to define. Other factors, such as tissue angles, planes, contours and proportions have been described in normals and standards have been developed. The purpose of this study was to determine cer- tain points, planes, and angles, pre- and postoperatively and to compare these values with normal standards in or- der to evaluate improvements in facial aesthetics.
Materials and Methods
The lateral cephalograms of 21 consecutive patients with craniofacial dysostosis syndromes (Crouzon's, Pfeiffer's, and Apert's) were analyzed. The same 21 consecutive pa- tients were previously studied for stability of their post- operative midfacial advancement in all cases at least one year postoperatively (Ousterhout et aL, 1986). The present study included a comparison of a group of preoperative points and planes with normals as well as a similar comparison of the same points and planes on postopera- tive cephalograms (again all at least one year after surg- ery).
Summary
A consecutive series of 21 patients with craniosynosto- sis syndromes (9 Crouzon's, 6 Pfeiffer's, 6 Apert's) was evaluated for reconstructive postoperative aesthetic improvement resulting from craniofacial surgery (Le- Fort III, sliding genioplasty, nasal septal reconstruction and cranioplasty). The same consecutive series of pa- tients had been previously evaluated for stability of Le Fort III advancement. All the patients were assessed at least one year post operatively. Stability of the ad- vancement was confirmed. In the present study, var- ious midline bony and soft tissue profile measure- ments were obtained from lateral cephalograms. These measurements were compared to similar measure- ments (standards) in normals. "Normal" standards and "normal" appearance imply harmonious and aestheti- cally pleasing facial features. This study showed mar- kedly abnormal positions of the selected bony and soft tissue landmarks preoperatively compared to the nor- mal but following surgery the measurements studied approached or were similar to the normal standards. Based on these measurements, the patient's appearance improved significantly following reconstructive craniofacial surgery. Suggestions for additional surg- ery based on these studies are made.
Key-Words
Craniofacial surgery - Craniosynostosis syndromes - Crouzon's syndrome - Apert's Syndrome - Pfeiffer's syndrome - Aesthetic result - Follow up
The bony and soft tissue areas chosen for study were on the facial profile: shape and size of the nose, position of the anterior nasal spine in relation to the anterior cranial base, menton and condylion, supradentale to sella, angle of the functional occlusal plane as related to the cranial base, length of the mandible, and the height of various segments of the mid and lower face. These shapes and positions can generally be easily obtained from lateral cephalograms. Farkas (1981), Burlington (McNamara, 1984), and Beh- rents (1985), standards were used as the basis for the com- parison. The vertical height of the orbits, while important in such a study, unfortunately cannot be reliably obtained from lateral cephalograms. Orbitale is very difficult to de- fine both in the pre- and postoperative cephalogram partly because it is a paired structure and not in the midline. Soft tissue cheek position is one of the most important con- tours on the face, but cannot be evaluated on head films. Cheek position is best evaluated by clinical examination, but as this is a subjective evaluation, it is not included in this study. Additionally, height of the upper face (the fore- head) could not be reliably evaluated as the point trichion (frequently used for such evaluations) cannot be deter- mined from the cephalograms. The various landmarks, planes, and angles chosen were se- lected on the basis that they seemed to best represent the bony and soft tissue positions and proportions to be stud- ied. As they were midline structures, except for condylion,
190 J. Cranio-Max.-Fac. Surg. 15 (1987) D. K. Ousterhout, K. Vargervik
Co
6
Fig. 1 The cephalometric bony and soft tissue profile points and planes are shown. These are from Behrents, Burlington, and Par- kas.
they were judged to be more reliably represented on the radiographs than non-midline structures where variable distances from the midline can cause significant variations in projection. The cephalometric bony distances and planes evaluated were (Fig. I): a) Nasion-anterior nasal spine (N-ANS). b) Anterior nasal spine-menton (ANSi -Me). c) Sella-Supradentale (S-Sd). d) Condylion-anterior nasal spine (Cd-ANSii). e) Condylion-pogonion (Cd-Pg). f) Angle of the functional occlusal plane as it relates to
the line sella-nasion (FOP-SN). As nasion is altered with the Le Fort III surgery, determina- tion of the line sella-nasion in the postoperative cephalo- gram was established by superimpositioning the long-term postoperative lateral cephalogram tracings on the preoper- ative tracing. Anterior nasal spine was measured at the point where it is three millimetres thick, except in the mea- surement of nasion-anterior nasal spine where the tip was used. This difference in determining the point anterior na- sal spine was based on the different methods used to esta- blish Burlington and Behrents (1985) standards. The re- maining cephalometric points were in the usual position as described in a number of cephalometric publications. Soft tissue profile and proportions are an important aspect of facial aesthetics. Certain measurements evaluating ante- rior facial soft tissue profiles can be obtained from the same cephalograms in which bony relationships were ob- tained. The particular measurements included in this study obtained from lateral cephalograms and utilizing Farkas (1981) standards were as follows (Fig. 1): a) SV-21 morphological height of the face (nasion-men-
ton).
b) SV-22 physiognomical height of the upper face (nasion- stomion).
c) SV-23 height of the lower face (subnasale-menton). d) SV-24 height of the lower third of the face (stomion-
menton). e) SV-25 length of the nose (nasion-subnasale). f) SV-26 length of the nasal bridge (nasion-pronasale). All of the above six measurements were corrected for the nine per cent magnification occurring in our cephalomet- ric radiographic procedures. In this study there were 3 of Oriental, 1 of Latin, and 17 of European origin. In the Apert's group there were 4 males and 2 females, all 6 Pfeiffer's were females, and of the 9 Crouzon's 6 were males, and 3 were females. The stan- dards used are based solely on those of European origin. As ours was such a variable (3 syndromes) and small groups of patients, it was our opinion that statistical com- parison of our findings with a normal group would be of reduced reliability. Therefore this study is designed only to demonstrate a trend, a pre- and postoperative mean of cer- tain measurements. No attempt was made to show statisti- cal significance.
Results
The results of the various evaluations and comparisons are shown in Tables II through V. In these tables, the mean of the evaluations from this study are compared with the mean in normal standards. Figures 2 through 7 show se- lected subjects who have had Le Fort III advancements and recommendations for improving the aesthetic result are given in the legends. The suggestions for additional surg- ery in individual cases or collectively, are based on the dif- ferences from the normal means. These suggestions in the individual cases are confirmed by the subjective evaluation of the patient (or their photograph).
Discussion and Conclusions
This study was designed to evaluate the aesthetic results following facial surgery in a group of craniosynostosis pa- tients, to determine if our results showed as much of an aesthetic improvement on an objective basis as they showed on a subjective one. We chose to use as our basis of normal the Burlington, Farkas (1981), and Behrents (1985) standards. Unfortunately all of these cephalometric standards for bony position are based on lateral views. While the frontal view is extremely important, since no sa- tisfactory frontal cephalometric standards are known to us, this important area of evaluation was not included. Ad- ditionally the cheek position and orbital height, obviously of considerable importance in any aesthetic evaluation, could not be evaluated. Orbitale was not considered a reli- ably reproducible point in this study. This was especially true in our postoperative films as the infraorbital rim was augmented in all of the patients with a split rib bone graft at the time of their Le Fort III operation. There are no standards known to us for cheek position. This study was preceeded by an evaluation of the stability of our midfacial advancements evaluating the same 21 consecutive patients (ten males, eleven females). In this prior study we found that the midfacial advancements were stable at a minimum of one year postoperatively, us- ing our technique for obtaining bone graft ossification
Aesthetic Improvement Resulting from Craniofacial Surgery J. Cranio-Max.-Fac. Surg. 15 (1987) 191
Fig. 2 a Figl 2 b
Fig. 2 23-year-old (preoperat ive) with Crouzon's Syndrome. Operat ions: Le Fort III and I, nasal septal reconstruct ion, cranioplasty, a: preoperative, b: 3years postoperat ively. Result: Very good.
(Vargervik, 1983). All of these patients were adults at the time of their postoperative evaluations, therefore all of these patients preceeded current infant craniosynostosis procedures (Hoffman and Mohr, 1976), described when these individuals were at least six years old. Because of this their skulls showed significant variation in shape, primari- ly brachycephaly and turricephaly. Often there was con- siderable skull irregularity from previous strip craniecto- mies, occasionally without complete bony coverage of the dura. Within this group of patients there was considerable variation in preoperative facial shape secondary to numer- ous factors including: syndromal variations, habits (such as mouth breathing), various approaches to preoperative or- thodontics (completed by a variety of orthodontists), he- reditary familial influences, etc.
The postoperative cephalometric studies have shown a general improvement in the horizontal position of the bony midface. In completing the Le Fort surgery, the max- illa was brought to the best possible occlusal relationship with the mandible. The mandibles were smaller in the fe- males and at the mean in the males, compared with the normal. Because the anterior nasal spine generally was even further retruded than supradentale in both males and females, the distance from the condyle to the anterior na- sal spine continued to be markedly short in the postopera- tive state even though better than preoperatively. The length sella supradentale, however, was normal postopera- tively even though markedly retruded preoperatively (Table 1). Some mandibular length increase did occur with maturity.
Table 1 Average bony changes with midfacial advancement (Burlington and Behrents Standards)
Females (11) Males (10)
Measurement Preop Mm. Postop. Mm. Preop Mm. Postop Mm. mean from mean from mean from mean from in mm normal in mm normal in mm. normal in ram. normal
Tab le3 Average profile result with Le Fort Ill and I compared with those with Le Fort Ill operations alone
Female Male
Le Fort Ill Le Fort Ill + I Le Fort III Le Fort ill + I
Measurement Postop, Mm. Postop Mm, Postop. Mm, Postop. Mm. mean from mean from mean from mean from in mm, normal in mm. normal in mm. normal in mm, mean (8 pts) mean (3 pts) mean (7 pts) mean (3 pts) mean
Some of these patients were still in mid-adolescence at the time of their Le Fort surgery but all were mature at the time of their final cephalometric evaluation and mandibu- lar growth had ceased. The functional occlusal plane as related to the line sella- nasion was normal on average both pre- and postopera- tively, but it did increase approximately 3 degrees in both sexes with surgery. This is the result of the midface tipping forward as it is advanced in order to maintain occlusion and not create an anterior open bite. The morphological height of the face (Table 2, SV-21, na- sion to gnathion) tended to be longer in the preoperative patient than normal and remained so postoperatively. Where was this increased height located? The physiognom- ical height of the upper face (SV-22, nasion-stomion) was normal in the preoperative patient but became longer than normal in the postoperative patient. SV-23 (subnasale- menton) was normal postoperatively. The height of the lower third of the face (SV-24, stomion-gnathion) was ab- normally long in the preoperative condition but improved in the postoperative. This improvement was primarily due to a better occlusion and correction in many of their aper- tognathia. In some cases the improvement was additional- ly helped by the sliding genioplasty. The distance anterior nasal spine to menton is excessively long (Table 1) but the distance SV-23 and 24, both measuring lower facial height (Table 3), are only slightly increased beyond normal. This would indicate that the soft tissue drape is not exactly fol-
lowing the bony configuration change, consistent with the markedly retrodisplaced anterior nasal spine. This may al- so point to a high floor of the nose, higher than the nostril floor, but particularly an increased height of maxillary alveolar base. This may be secondary, at least in part, to a chronic mouth breathing condition (Harvold et al., 1981; Ousterhout et al., 1983). The distance from nasion to the anterior nasal spine showed considerable change with the LeFortlII advancement. Preoperatively the distance was extremely short but changed to normal length in the males and near normal in the females (Table 1). This is particu- larly interesting in that the size of the nose (SV-25 and SV- 26) did not change nearly so much, but more in the males than females. We felt that this was especially important in the aesthetic improvement seen in the midface postopera- tively in these patients. Therefore the abnormal facial height (SV-21) incorporates a change from the lower face to the midface with the facial surgery in the collective series. The nose was evaluated by two measurements. The length of the nose (SV-25, nasion-subnasale) was increased by the Le Fort III surgery in both the male and female with the fi- nal length being longer than the normal mean. The length of the nasal bridge (SV-26, nasion-pronasale) was shorter preoperatively than normal in both sexes but was im- proved to a normal length postoperatively. While not mea- sured, it was obvious that the columellar-labial angle im- proved considerably with surgery changing from an ab-
Aesthetic improvement Resulting from Craniofacial Surgery J. Cranio-Max.-Fac. Surg. 15 (1987) 193
Fig.3a Fig.3b
Fig.3c Fig.3d
Fig.3 17-year-old (preoperative) with Crouzon's Syndrome. Operation: Le Fort III. a: preoperative, b: six years postoperative- ly, c: preoperative occlusion, d: post opera- tive occlusion. Result: Good but would benefit from sliding genioplasty and nasal septal reconstruction.
normal acute angle to a more normal angle (i.e., slightly greater than a right angle). Some of this improvement in columellar-labial angle was related to the nasal surgery the majority of the patients also had. Six of the twenty-one patients also had a simultaneous LeFortI with their LeFortlII operations (three males, three females). These were compared with the others of the same sex who had only a LeFortIII operation (Table 3). These results would indicate that in general a Le Fort i in addition to the Le Fort III operation, was help- ful to the final facial profile height. This was most evident in the females. Anterior nasal spine-menton height was im- proved more in those who additionally had a LeFortI procedure. The lower facial height (SV-24, stomion-gnath- ion) was improved more in the Le Fort III and I group than in the Le Fort III alone group. This may be related to a more abnormal preoperative occlusion in those requiring the LeFortI operation, especially any existing aperto- gnathia. Le Fort I surgery may have facilitated an increased autorotation of the mandible towards the closed position. However, the measurements obtained did not give proof of this. Fourteen of these patients (8 females and 6 males) under- went a methylmethacrylate cranioplasty for reconstruc-
tion of the forehead (Ousterhout et al., 1980). In these cases the position of the glabellar notch, representing na- sion, are modified as the supraorbital rims and glabellar areas were reconstructed. Before the cranioplasty, the nose frequently appeared very long (Fig. 4) but its appearance was improved with the forehead surgery. Nasion-subna- sale (SV-25) and nasion-pronasale (SV-26) were compared by sex in those with and without a cranioplasty (Table 4). The results of this comparison showed that the vertical height of the nose (SV-25) was improved towards normal, the mean, if a cranioplasty was additionally used. The postoperative dorsal length of the nose (SV-26) was nor- mal whether a cranioplasty was utilized or not. The above was the same for both sexes. These results are better than in the collective average for SV-25 (Table 2) where the na- sal length, nasion-subnasale, was longer than normal. Therefore the cranioplasty was not only helpful in improv- ing forehead aesthetics (see next paragraph) but additional- ly the nose as well (Figs. 2 and 6). While no objective measurements were completed to eval- uate forehead contour and height, clinically the appear- ance was improved by the cranioplasty (Figs. 2, 5, and 7). The majority of those needing a cranioplasty eventually had the procedure, only a few refusing the additional
194 ]. Cranio-Max.-Fac. Surg. 15 (1987) D. K. Ousterhout, K. Vargervik
Fig.4a Fig.4b
1 40 0 "
Fig.4c
Fig.4d Fig.4e Fig.4f
Fig.4 14-year-old (preoperative) with Crouzon's Syndrome. Ope- rations: Le Fort III and i. a: preoperative, b: four years postoperative- ly, c: preoperative tracing, f: postoperative tracing, d: preoperative
179 0 ~
occlusion, e: postoperative occlusion. Result: Fair, primary prob- lem, midface too long. Would benefit from sliding genioplasty nasal septal reconstruction, and cranioplasty.
Table4 Nasal profile length with and without cranioplasty
Females Males
Measurement Postop. Mm. Postop. Mm. Postop. Mm. Postop. Mm. without from with from without from with from cranioplasty normal cranioplasty normal cranioplasty normal cranioplasty normal mean mean mean mean mean mean mean mean in mm. in mm. in mm. in mm. (3 pts) (8 pts) ~' (4 pts.) (6 pts.)
Aesthetic Improvement Resulting from Craniofacial Surgery J. Cranio-Max.-Fac. Surg. 15 (1987) 195
Fig.5a Fig.5b
Fig. 5 16-year-old (preoperative) with Pfeiffer's Syndrome. Operations: Le Fort III, nasal septal reconstruction, cranioplasty, a: preoperative, b: two years postoperatively. Result: Good, would benefit from sliding genioplasty and secondary rhinoplasty.
Fig. 6 a Fig. 6 b
Fig.6 17-year-old (preoperative) with Pfeiffer's Syndrome. Operations: Le Fort Ill, sliding genioplasty, nasal septal recon- struction, cranioplasty, a: preoperative, b: three years postoperatively. Result: Very good.
operation (Figs. 3 and 4). The cranioplasty procedure has never been done simultaneously with the midfacial proce- dures but was always done as the last major operation. Only four patients underwent a sliding genioplasty, pri- marily to advance the chin but also to reduce its vertical height. This study only evaluated vertical lower facial height (SV-24, Table 5). The subjective improvement noted was confirmed. The subjective improvement in the anteri-
¢*
or chin prominence was also obvious but not evaluated (Fig. 6). The advantages of a sliding genioplasty on profile appearance are well recognized. There is, however, a parti- cular problem with vertical chin reduction in that the soft
tissue profile does not follow the bony repositioning on a 1:1 basis. Chin improvement occurred even without a sliding genioplasty (SV-24, Table 2) (Fig. 2), probably as a result of improvement in the occlusion with the resulting autorotation of the mandible towards a more closed posi- tion swinging the chin forward. Within this consecutive series of patients the overall results were pleasing but with better results in some patients than in others. While the subjective appearance was improved in all and pleasing in most, the objective evaluation, when compared with normals, leaves in certain areas, room for improvement, primarily the vertical height of the midface.
196 J. Cranio-Max.-Fac. Surg. 15 (1987) D. K. Ousterhout, K. Vargervik
Fig,7a
Fig.Tc
Fig.7b
200 9 ;:'44 9 I
Fig. 7 20-year-old (preoperative) with Apert's Syndrome. Operations: Le Fort III and I, nasal septal reconstruction, cranio- plasty, a: preoperative, b: four years post- operatively, c: preoperative tracing, d: post- operative tracing. Result: good, would ben- efit from sliding genioplasty.
Fig.Td
In general the Le Fort t procedure was completed with the LeFort I I I because of marked increase in the maxillary alveolar base height or because there was a rotational discrepancy between the upper midface (infraorbital rims) and the lower midface (maxillary alveolar arch). It was a
surprise to see that in general the facial aesthetics were better in this group than the Le Fort III only group. A significant limitation on the aesthetic result was that several patients did not want any further surgery follow- ing the midfacial advancement. Our personal preference is
Table5 Profile change with sliding genioplasty
Female Male (3 patients) (1 patient)
Measurement Preop. Mm. Postop. Mm. Preop. Mm. Postop. Mm. mean from mean from mean from mean from in mm. normal in mm. ,~ normal in mm. normal in mm. normal
mean mean mean mean
SV-24 52.0 + 6.8 48.3 + 3.1 52.3 + 2.2 49.5 - 0.6
Aesthetic Improvement Resulting from Craniofacial Surgery J. Cranio-Max.-Fac. Surg. 15 (1987) 19 7
to do the genioplasty as a secondary procedure with the rhinoplasty after the LeFort l I I (and I) are stable. If we could have predicted that the patient would not have wanted any further surgery, we would have completed a sliding genioplasty at the time of the midfacial advance- ment in the vast majority of these individuals. A very difficult problem, not addressed by this study, is that of the soft tissue drape changes with midfacial surg- ery. The soft tissue in these patients is also not always nor- real. The Apert's patients frequently have acne problems, but in addition, the skin often seems to be thick with in- creased subcutaneous tissue and glandular components in the non-Apert's as well as the Apert's patients. It was not our intention in this study to evaluate aesthetics on a statistical basis. Rather, it was to relate aesthetics to normal size and shape and to evaluate whether our surgery changed the face sufficiently towards the normal. It was our opinion that this evaluation has shown a significant change from the preoperative deformity towards a more normal postoperative shape, size, and appearance. If the normal is more aesthetically pleasing, and we suggest it is, then this extensive surgery has been helpful in improving the aesthetic results, an aesthetic result approaching in many ways "normal" appearance. What seems obvious from this study is that these faces and skulls are very abnormal and even with improvement in shape and appearance towards normal, normal probably cannot be reached but in a few. These are a mixed group of patients and deformities. The surgery must therefore be variable depending upon the patients and their presenting deformity. Planning for the surgery must be based not on- ly on objective studies (cephalometrics, dental study mod- els, CT scans, etc.) but also on subjective evaluations, i.e., looking at the patient and determining in the minds eye where the deformities lie and what can and should be completed, and the sequence of operations (if more than one will be necessary) to obtain the best result. The indi- vidual variations in operations can be extremely signifi- cant. Small differences in contour (shape of the nose, chin and forehead) can cause a significant variation in the aes- thetic result. In conclusion, the post operative appearance of these se- verely deformed patients is the result of an attempt to make the patient normal by correcting the underlying skeletal foundation (normal bony position tending to give
a normal appearance). The best result occurs by developing a harmonious relationship between many factors including the chin, occlusion, nasal contour and length, overall verti- cal height of the various segments of the face, cheek posi- tion, orbital relationships, forehead contour, etc., in asso- ciation with the overall size of the craniofacial skeleton and soft tissue drape. Obviously a complex problem. All of us, patients and professionals, owe a tremendous of gratitude to Dr. Paul Tessier. He, more than any other sur- geon, most clearly sees these above complex relationships. Congratulations, thank you, and a Happy Birthday.
References
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Farkas, L.G.: Anthropometry of the head and face in medicine. El- sevier, New York (1981) 293
Hoffman, H.J., G. Mohr: Lateral canthal advancement of the supra- orbital margin: A new technique in the treatment of coronal syn- ostosis. J. Neurosurg. 45 (1976) 376
Harvold, E.P., B. S. Tomer, K. Vargervik, et al.: Primate experiments on oral respiration. Am. J. Orthod. 79 (1981) 359
McNamara, J. A.: A method of cephalometric evaluation. Am. J. Or- thod. 86 (1984) 449-469
Ousterhout, D.K., S. Baker, I. Zlotolow: Methylmethacrylate oniay implants in the treatment of forehead deformities secondary to craniosynostosis. J. max.-fac. Surg. 8 (1980) 228-233
Ousterhout, D.K., S.Clark, K.Vargervik: Midface position after LeFortlII procedures. Accepted for publication, Cleft Palate (1986)
Ousterhout, D.K., K. Vargervik, A. Miller: Nasal airway as it relates to the timing of the mid and lower facial osteotomies. Ann. Plast. Snrg. 11 (1983) 175-182
Vargervik, K.: Chapters 7 and 8 in: E.P. Harvold (ed.): Treatment of hemifacial microsomia. Liss, New York (1983) 247
Dr. D.K. Ousterhout Dept. of Surgery (Plastic) Univ. of California San Francisco, California, USA 94143 and 490 Post Street San Francisco, California, USA 94102
