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Twenty Year Experience in Maxillocraniofacial Surgery

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Twenty Year Experience in Maxillocraniofacial Surgery An Evaluation of Early Surgery on Growth, Function and Body Image JOSEPH E. MURRAY, M.D., JOHN B. MULLIKEN, M.D., LEONARD B. KABAN, D.M.D., M.D., MYRON BELFER, M.D. We have analyzed 404 patients with a wide variety of maxillary and craniofacial deformities. These do not include head and neck cancer patients as generally defined. Satisfaction has been high as judged by the patients, surgeons and psychiatrists. The complication rate has been significant, approximately 30%, the most common being infection or loss of bone grafts. As experience accumulated, the concept of earlier operative inter- vention has emerged as an aid in unlocking growth potential, diminishing secondary deformity and improving the develop- ment of body image. FORMERLY MAXILLOCRANIOFACIAL surgery dealt with stable, end-stage deformities in the adult. As experience accumulated results suggested that opera- tions in childhood and even in the neonatal age group would minimize the degree of deformity and lead to better anatomic correction and improved function. This report of 404 patients with congenital and acquired defects supports the concept of performing early sur- gical correction. The benefits of early operation on growth and function are not surprising; however, the favorable improvement in body image has been un- anticipated, gratifying dividend. Surgery of the face, cranium, orbit, mouth and jaw has evolved into a subspecialty of plastic surgery and draws in addition on the techniques and expertise of neurosurgery, ophthalmology, oral surgery and dentistry. The full team includes otolaryngology, anesthesia, radiology, speech and hearing, nursing, social sciences and a coordinator. The psychiatrist fills an ex- panding role in the team because the complex patient, physician and family relationship which regulates the psychosocial adjustments changes with growth, devel- opment and surgical intervention. Presented at the Annual Meeting of the American Surgical Association, April 26-28, 1979, Hot Springs, Virginia. Reprint requests: Joseph E. Murray, M.D. Supported in part by the Harry Doehla Foundation, Inc., the Massachusetts Cosmetologists Association, Inc., and the Brigham Surgical Group Foundation, Inc. From the Division of Plastic Surgery, Harvard Medical School and School of Dental Medicine, Children's Hospital Medical Center and Peter Bent Brigham Hospital, Boston, Massachusetts Our previous report1" discussed the preoperative evaluation, anesthesia, surgical technique, results and complications. This report updates our experience with special emphasis on early operation to unlock potential growth, minimize secondary deformity, and enhance the development of body image. Clinical Material Four hundred and four patients are evaluated (Table 1). The congenital deformities comprise the largest group. The incidence of trauma requiring craniofacial surgery has increased threefold over the past five years. We are not including conventional head and neck neoplasms in our craniofacial clinic; the tumors seen are usually of mesenchymal origin, located about the cranium, orbits and nasal cavities and occur in a young age group. Of all the patients studied 65% received operations with a total of 385 operations in the 264 patients. Analysis of the congenital group indicates that the four major categories encompassed by earlier cranio- facial surgery, i.e., craniofacial dysostoses, hyper- telorism, Treacher Collins and hemifacial microsomia are increasing in number slowly and consistently. How- ever, the inclusion of isolated jaw deformities and secondary cleft lip and palate deformities expanded our series (Table 2). Surgical Techniques The techniques have been described previ- ously.2-4'6'9-18 Recent technical improvements have in- cluded advancement of the frontal-orbital complex at the time of the initial operation for craniosynostoses during the first weeks or months of life,5'7 multiple seg- 0003-4932/79/0900/0320 $01. 10 C J. B. Lippincott Company 320
Transcript
Page 1: Twenty Year Experience in Maxillocraniofacial Surgery

Twenty Year Experience in Maxillocraniofacial Surgery

An Evaluation of Early Surgery on Growth, Function and Body Image

JOSEPH E. MURRAY, M.D., JOHN B. MULLIKEN, M.D., LEONARD B. KABAN, D.M.D., M.D., MYRON BELFER, M.D.

We have analyzed 404 patients with a wide variety of maxillaryand craniofacial deformities. These do not include head andneck cancer patients as generally defined. Satisfaction hasbeen high asjudged by the patients, surgeons and psychiatrists.The complication rate has been significant, approximately 30%,the most common being infection or loss of bone grafts. Asexperience accumulated, the concept of earlier operative inter-vention has emerged as an aid in unlocking growth potential,diminishing secondary deformity and improving the develop-ment of body image.

FORMERLY MAXILLOCRANIOFACIAL surgery dealtwith stable, end-stage deformities in the adult. As

experience accumulated results suggested that opera-tions in childhood and even in the neonatal age groupwould minimize the degree of deformity and lead tobetter anatomic correction and improved function.This report of404 patients with congenital and acquireddefects supports the concept of performing early sur-gical correction. The benefits of early operation ongrowth and function are not surprising; however, thefavorable improvement in body image has been un-anticipated, gratifying dividend.

Surgery of the face, cranium, orbit, mouth and jawhas evolved into a subspecialty of plastic surgery anddraws in addition on the techniques and expertise ofneurosurgery, ophthalmology, oral surgery and dentistry.The full team includes otolaryngology, anesthesia,radiology, speech and hearing, nursing, social sciencesand a coordinator. The psychiatrist fills an ex-panding role in the team because the complex patient,physician and family relationship which regulates thepsychosocial adjustments changes with growth, devel-opment and surgical intervention.

Presented at the Annual Meeting of the American SurgicalAssociation, April 26-28, 1979, Hot Springs, Virginia.

Reprint requests: Joseph E. Murray, M.D.Supported in part by the Harry Doehla Foundation, Inc., the

Massachusetts Cosmetologists Association, Inc., and the BrighamSurgical Group Foundation, Inc.

From the Division of Plastic Surgery,Harvard Medical School and School of Dental Medicine,

Children's Hospital Medical Center andPeter Bent Brigham Hospital, Boston, Massachusetts

Our previous report1" discussed the preoperativeevaluation, anesthesia, surgical technique, results andcomplications. This report updates our experience withspecial emphasis on early operation to unlock potentialgrowth, minimize secondary deformity, and enhancethe development of body image.

Clinical Material

Four hundred and four patients are evaluated (Table1). The congenital deformities comprise the largest group.The incidence of trauma requiring craniofacial surgeryhas increased threefold over the past five years. We arenot including conventional head and neck neoplasms inour craniofacial clinic; the tumors seen are usually ofmesenchymal origin, located about the cranium,orbits and nasal cavities and occur in a young age group.Of all the patients studied 65% received operations witha total of 385 operations in the 264 patients.

Analysis of the congenital group indicates that thefour major categories encompassed by earlier cranio-facial surgery, i.e., craniofacial dysostoses, hyper-telorism, Treacher Collins and hemifacial microsomiaare increasing in number slowly and consistently. How-ever, the inclusion of isolated jaw deformities andsecondary cleft lip and palate deformities expandedour series (Table 2).

Surgical Techniques

The techniques have been described previ-ously.2-4'6'9-18 Recent technical improvements have in-cluded advancement of the frontal-orbital complexat the time of the initial operation for craniosynostosesduring the first weeks or months of life,5'7 multiple seg-

0003-4932/79/0900/0320 $01. 10 C J. B. Lippincott Company

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MAXILLOCRANIOFACIAL SURGERY 321TABLE 1. Craniofacial Patients 1959-1979

Patients Patients Per CentStudied Operated Operated

Congenital 349 224 64Acquired 55 40 71tumor 28trauma 27

Total 404 264* 65

* Three hundred and eighty-five operations in 264 patients.

mental osteotomies for unilateral orbital deformity andvarieties of maxillary and mandibular advancementsand setbacks with and without segmental osteotomies.The operations fall into four general categories in degreeof magnitude (Table 3). The most extensive operationsare those involving craniotomy with exposure of thebrain and those with facio-orbital osteotomies and inter-position bone grafts. These long procedures may requirecombined neurosurgical participation during portionsof the procedure. These operations usually are for cor-rection of hypertelorism, and midface stenosis seen inCrouzon's or Apert's syndromes; or for intracranialapproach to tumor resection and trauma cases. Thenext most extensive operations are those requiring facio-orbital dissections, osseous contouring, and onlay bonegrafting: examples are correction of Treacher Collinsdeformity. Without an osteotomy, morbidity in theseprocedures is less, but the full dissection of both orbitalcontents constitutes a very real hazard. Next in mag-nitude are the operations of the maxilla and /or mandiblerequiring osteotomy with or without bone grafts. Al-though the duration ofanesthesia and operation may beshorter in this category than in the previous two,critical complications can occur. Wheneverjaw osteot-omies, either segmental or complete are performed, anincreased hazard is introduced by the need for inter-

TABLE 2. Congenital Deformities 1959-1979

Craniofacial dysostosisCraniosynostosisCrouzon'sApert'sOther

HypertelorismTreacher CollinsHemifacial MicrosomiaOther

congenital tumors (vascular, neurofibroma,encephalocele)

isolated jaw deformities (prognathism, maxillarydeformity, chin)

late cleft lip & palate deformitiesfacial cleftsfacial atrophymiscellaneous

Total

1226194

61

231438

213

14

1083151243

TABLE 3. Magnitude of Operation

++++ Craniotomy, facio-orbital osteotomy, interposition bonegraft

+++ Facio-orbital dissection, osseous contouring, onlaybone graft

++ Maxillary and/or mandibular osteotomy with orwithout bone graft

+ Soft tissue dissection and repair

maxillary fixation requirements with airway hazardsconstituting a major source of complication. The leastextensive operations are those with soft tissue dissectionsand repairs such as, secondary repair of oronasal com-munications or nerve deficit.

Psychiatric Testing

In semistructured interviews the psychiatrist focuseson the stages in body image development. These inter-views are supplemented by the Draw-A-Person exercise,the Piers-Harris evaluation with both mother and child,and the Sears test. These provide objective assessmentsof self concept.

Results

The aim of the treatment is to allow the patient toenter the "mainstream" of life. Correction of anatomyand function are merely means to that end. In this lightwe have considered our operative results from threeviewpoints: patient, surgeon, and psychiatrist (Table 4).As in other areas of plastic surgery, patient satisfactionis higher than the surgeon's anatomic assessment. Itwas a surprise, however, to find the psychiatric evalua-tion surpassing the surgeon's impression of the degreeofpatient contentment. Several patients with incompleteanatomic reconstruction achieved almost completesocial integration and significant personality im-provement.

Complications

The overall incidence of complications was 30%(Table 5). The most severe complication was an un-

TABLE 4. Assessment of Operative Results

Per Cent Per Cent Per Cent NumberGood Average Poor of Patients

Patient andpsychiatrist 88 8 4 60*

Patient and surgeon 80 13 6 200tSurgeon's anatomicassessment 70 22 8 208t

* Interview of 60 consecutive patients following operations of+++ and ++++ magnitude.

t Postoperative clinic notes by surgeons.

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Ann. Surg. * September 1979MURRAY AND OTHERS

36

20

714

TABLE 5. Complications in 264 Operated Patients

DeathBlindness

unilateral completeunilateral partial

Hemorrhagegreater than ½i BVoperation stopped

Airway -prolonged intubation or tracheostomyInfection-I&D or antibioticsBone graft lossminormajor

Central nervous systemOtherhematoma, wound dehiscencetrismusdonor siteflap loss

Total

* Unexplained respiratory arrest 36 hours postextubation.

explained respiratory arrest 36 hours following endo-tracheal extubation in a patient who had had a Grade+ + operation, i.e., a segmental osteotomy and genio-plasty. She had had an incidental dilatation and curet-tage for a gynecological condition utilizing the sameanesthesia. Unilateral blindness occurred in twopatients. In both instances orbital swelling was notedin the final stage of the operation and immediatedecompression was performed with the ophthalmologistin attendance. In one patient, improvement seemed tooccur for 36 hours only to be followed by relapse at

FIG. la. Postoperative x-ray of skull in a year-old patient who hadcoronal strip craniectomies in infancy. Note flatness of foreheadand supraorbital regions, with secondary distortion of occipitalregion.

48 hours. In the other patient the unilateral orbitalswelling was decompressed immediately but visionnever returned.Airway problems occurred in operations which re-

quired intermaxillary fixation. Bone graft loss of majordegree was noted in 14 patients. There were six centralnervous system complications with leakage of cerebro-spinal fluid; all corrected by secondary surgical repair.

Current Concepts of Treatment

6 Early operative correction, performed as safety6 allows, is based on three guiding concepts: unlocking4 potential growth, minimizing secondary deformities,4 and aiding the development of body image.

81(30%) Unlocking of Growth Potential

Excision of prematurely closed cranial sutures im-proves the skull shape and allows the brain to grow.Failure to correct the synostosis of the frontoethmoid,frontosphenoid and the sphenozygomatic suture linesresults in a furrowed forehead deformity and may con-tribute to underdevelopment of the midthird ofthe face.Conventional coronal craniectomies allow adequatedevelopment of the cranial vault; however, there maybe restriction ofgrowth in the frontal area and secondarydistortion of the occipital bones (Fig. la). The forehead

FIG. lb. Early craniectomy failed to prevent typical grooved fore-head in this 8-year-old with Apert's type craniosynostosis.

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MAXILLOCRANIOFACIAL SURGERY 323

stage is vastly more complex than surgery performed inthe first few months of life. The late operation oftenrequires craniotomy and transposition of frontoparietalbone segments along with osteotomies of the orbitsand frontonasal complex. Bone grafts are required tostabilize the osteotomized segments and to improvecontour (Fig. 3c). Unlocking growth potential on the af-fected side allows normal growth stimulation in adjacentareas by the expanding brain acting as a "functionalmatrix."8 In summary, early operation is easier,shorter, and more effective.

Minimizing Secondary Distortion

Secondary deformity may be produced in ways otherthan by overgrowth compensating for a restricted site.Any mass can distort adjacent growth. This is seen incongenital tumors such as vascular malformations,neurofibromas or encephaloceles. Secondary and eventertiary effects are produced with a frontoethmoidalencephalocele (Figs. 4a and b). As the encephaloceleextrudes through the frontonasal defect, distortion ofadjacent tissues can be produced. The eyebrows aredisplaced upward and laterally and orbital hypertelorismis present. This congenital mass acted as a wedge in

FIG. 2a. Preoperative view of a 6-week-old infant shows obliqueconcavities in frontotemporal areas, a result of synostosis of thefrontoethmoid, frontosphenoid, and frontozygomatic sutures. . ... _]

deformity with grooving of the brows bilaterally in apatient with coronal synostosis who had conventionalcoronal craniectomy is shown in Figure lb. In contrast,the concave, oblique forehead grooves in an infantprior to early intervention (Fig. 2a) have been correctedby extended craniectomy including release of the ap-propriate frontal orbital sphenoid and zygomaticsutures in addition to standard coronal craniectomy(Fig. 2b). The forehead advancement is maintained in amore normal position by the developing brain. This wi:-Xadditional osteotomy adds less than 30 minutes to theoperative time because the bones are soft and malleable _and can be osteotomized more easily than in the olderchild or adult. The postoperative result is far superior tothat which could be obtained by secondary operations bat a later date. The contrast between the results of earlyand late correction can be seen by comparing Figureslb and 2b.Failure to unlock growth in a unilateral coronal

synostosis may produce an increasing deformity as ad-jacent areas with normal open suture lines passivelyexpand. The constriction of growth in one area of thecranium thus acts as a tether or lock and the normal /tissues develop around it in a distorted way. Bossing oftissues develop around it in a distorted way. Bossing of

FIG. 2b. One year later the correction of the forehead and brow bythe normal area and displacement of adjacent struc- extended craniectomies is seen. This correction will be maintainedtures may result (Figs. 3a and b). Correction at a late by normal brain growth.

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Ann. Surg. * September 1979

FIG. 3a. Severe distortion of forehead, orbits an(uncorrected unilateral synostoses.

the middle of the face, and gradually caused elongationand lowering of the maxilla and mandible. It appearsas if a horizontal disjunction had occurred at the levelof the equators of the orbits. X-rays (Figs. 4c and 4d)show the distortion of the orbits with the orbital proc-esses of the frontal bone displaced superiorly, thenasal bones displaced inferiorly, and a large open con-duit between the two into which the glial contents haveherniated. At operation it was noted that the brain hadassumed the shape of the defect. Correction consistedof resection of the protruding encephalocele and repairof the dura defect with reinforcement by a bone graft.Bone grafts were also used to reconstruct the nose, theroofs and medial walls of the orbit. Soft tissue resectioncompleted the correction (Figs. 4e and 4f). Good resto-ration of the contour of the forehead was obtained alongwith repositioning the eyebrows. There is still minimalflatness ofthe bridge ofthe nose and the major secondarydefect, elongation of the face, was not completely cor-rectable. This will be extremely difficult to correct ifthe patient ever does desire it for it would require anintrusive, horizontal osteotomy at the level of the infra-orbital vessels.The concept of minimizing secondary deformity

applies most critically at early age when growth potentialis maximal and deformity is minimal.

w. ~~~~~~~~~~~~~~~~~~pdnoseresultsfrom ,j

:e: Sle ..~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~...i~~~~~~~~~k: Zx|w :.

54

FIG. 3c. Correction of forehead, orbit and nose required intracranial

FIG. 3b. The tethering effect centered in the righead areas is accentuated by overgrowth of unrhead. Note obliquity of nose and midface.

yht brow and fore-*estricted left fore-

and facial exposure, with transposition of calvarial segments, inter-position and onlay bone grafts, plus orbito-fronto-nasal osteotomies.This complex operation could have been avoided by earlier surgicalcorrection.

324 MURRAY AND OTHERS

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MAXILLOCRANIOFACIAL SURGERY 325

the decision to undergo operation; the operativeexperience; the immediate postoperative period andthe reintegration stage.The deformity which most strikingly exemplifies the

development and changes in body image is hemifacialmicrosomia, the first and second branchial arch syn-drome. Always asymmetric, the deformity may beminimal at first but as growth of the normal side occursthe defect becomes more obvious. The deformity has avariety of physical manifestations focused either on theexternal ear or mandible, or both. Secondary involve-ment of the zygoma, orbita, maxilla and cranial nervesoccurs frequently. If uncorrected the deformity willprogress in the adult stage to a constriction of theentire facial skeleton with gross distortion of the oc-clusal plane and shortening of the vertical height ofthe face.

Until recently surgical correction was focused on theexternal ear deformity. Currently with the applicationof craniofacial techniques correction of the bonydeformity has become possible. Now the skeletal de-formity takes first priority in repair with external earreconstruction and soft tissue repairs performed later.Correction of the adult deformity requires a complex

FIG. 4a. Late result of uncorrected frontoethmoidal encephalocelereveals secondary elevation of brows, lateral displacement of orbits,and vertical elongation of the central 1/3 of the face.

Aiding the Development of Body ImageBody image is a psychological concept and not merely

a function of objective appearance. Often changes inbody image are confused with physical change followingsurgical intervention. Change in body image, however,involves a complicated process occurring over time andis a result of psychological development of the child,the surgical procedures, and the societal and familialfactors. Craniofacial operations produce rapid anddramatic change in objective appearance and thus allowopportunity to study the evolution ofbody image over acompressed time span.Body image development can be viewed in four stages;

cognitive growth, perception of body stimuli, the stimulifrom the environment in the form ofcomparison and theresponse of others. Because the child starts to define hisbody image far earlier than school age, earlier surgicalcorrection is more likely to allow its normaldevelopment. Surgical intervention may produceobjective physical change without a corresponding rapidchange in body image. The modification ofan established FIG. 4b. Lateral view shows herniated encephalocele and strikingbody image can also be analyzed in four components: expansion in nasofrontal region.

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Ann. Surg. * September 1979MURRAY AND OTHERS

symmetrical, a squared figure (Fig. 5d). This seems tobe objective evidence of an alteration in his responseto sensory stimuli, both from himself and others.Parenthetically, his anatomic improvement, althoughsignificant, was not dramatic and was by no meanscommensurate with his marked improvement on hisself-image. This patient is a good example of patient'ssatisfaction exceeding that of the surgeon's. It alsoillustrates the critical nature ofthe psychiatric evaluation.

Discussion

Surgical decision making is always evolving. Thevariety of deformities and the intricacies of adaptiveresponses and the improvement in surgical techniqueshave changed some of our fundamental premises.Formerly reconstructive surgeons were content torestore anatomy and bodily function expecting to im-

FIG. 4c. A-P x-ray reveals large defect in forehead and lateral dis-placement of medial walls of orbits.

operation involving osteotomies of the mandible andmaxilla, often combined with genioplasty, onlay bonegraft to the zygoma and occasional orbital osteotomies.12The complexity of the repair in the adult is in itself astimulus to seek more effective, earlier intervention.There has always been reluctance to operate on grow-

ing patients for fear of disturbing growth. However, thesedeformities become worse spontaneously with con-tinued growth of the normal side so earlier interventiondoes not produce a more severe end result.A striking change in body image occurred in an 11-

year-old patient with hemifacial microsomia operatedupon for the first time (Figs. 5a and b). As part of hisregular preoperative psychiatric interview and work-uphe was asked to draw the picture of "a man," not asketch of himself, but merely his perception of anindividual man. His operation was major involvingbilateral mandibular osteotomies with elongation ofbothrami, creation of a temporomandibular joint, rotationgenioplasty, and correction of an open bite. As part ofregular postoperative psychiatric testing the "Draw-A-Person" test was repeated. His preoperative draw-ing showed an asymmetrical, amorphous, fluid figure,with uneven face, mouth, shoulders and extremities(Fig. 5c). The postoperative drawing was box-like,

FIG. 4d. Lateral x-ray shows frontal bone displaced upward (upperarrow) and nasal bones downward (lower arrow) revealing widecommunication through which the encephalocele herniates.

326

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MAXILLOCRANIOFACIAL SURGERY 327

stages of its evolution is warranted. The first step, thechild's cognitive development, influences a child'sperception of his body and defines the limit of his bodyimage. This analysis is complicated because patientswho look dull and retarded frequently are considered soregardless of their true intellectual ability. Manypatients with normal intelligence have been rescuedfrom custodial institutions and restored to the"mainstream" following surgical correction. Societytends to underestimate the intellectual ability ofphysically disfigured people and intellectual assessmenthas not always been carried out appropriately.A second stage influencing body image development

is theperception ofstimuli by the patient himself. Theseperceptions can be influenced by the congenitalanomaly. Congenital orofacial and upper extremityanomalies result in relative sensory deprivation.Children denied the sense of oral gratification andstimulation from grasp develop a sense of bodily in-competence in association with their functionalimpairment.

FIG. 4e. After resection of encephalocele and repair of dural defectwith fascia. Forehead, orbits and nose were repaired with bonegrafts. Note correction of brows and medial canthi.

prove automatically the psychological adjustment ofthe patient. Patient satisfaction is our primary aim.Often a patient with minimal correction displays a dis-proportionate satisfaction. Conversely, striking surgicalcorrection of the deformity may not lead to patientsatisfaction but instead may precipitate more criticalintrospective analysis of the residual defects. Thisoccurs more often following treatment for cancer ortrauma, for these born-normal patients compare them-selves to their original state rather than to their im-mediate preoperative condition.

Realization of the benefits of early operation hasevolved slowly. Previously we were hesitant for fear ofdisturbing "growth centers." Retrospective and pro-spective studies indicate that growth disturbances arenot accentuated by operation. Therefore early inter-vention, with unlocking growth potential and preventionof secondary deformation, is supported by our ex-perience. Optimal function influences growth favorably.8In addition, early operation improves the developmentof body image.The body image concept occupies an important role

in indications for surgery; therefore, analysis of theFIG. 4f. Lateral view reveals excellent correction of forehead andbrow, but elongation of nasofrontal angle still persists.

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328 MURRAY AND OTHERS

.&' ;; t _lowing acquired deformities supports the concept thatimpairment may be minimized by early operative inter-vention.The change in body image following operation

demonstrates its adaptability and this occurs in fourcomponents. First, there is a complex decision toundergo surgery. The timing of this decision is based onsocial awareness, self-recognition, parental and peer

.. _!2' _ pressure, emotional state, and the surgeon's assessment._ , -e The second phase, the operative experience, provides

concrete evidence that a change in physical appearancehas taken place. The reality of intervention is nowacknowledged. Prior to intervention there is always the

A.ilil_ f t -;opportunity to indulge in fantasies. The third phase, theimmediate postoperative period, involves physicalpain, increased rather than decreased distortion ofphysical appearance and re-examination of the decision

_]! ^ >4-1and expectations. This postoperative phase is a psy-chological crisis with an opportunity to lay bettergroundwork for subsequent integration. Disruptionof the previous psychological defense often leads to

..1

FIG. 5a. AP view of 11 year old with bilateral asymmetncal hemi-facial microsomia reveals open bite, deviation of chin to right,oblique occlusal plane, and soft tissue deficiency of right side offace.

The third element in body image formation is stimu-lation from the environment in the form of compari-sons to others. The child compares his body with thoseof his parents and peers noting similarities and dif- / yferences. This sense of differentness increases as the A, '$patient gets older and usually is not verbalized.The fourth element in body image development is the

response of others. Parents are often disappointed innot having the idealized child. The responses fromsociety tend to isolate the youngster or present him witha negative view of his deformity. Parents and young-sters often react by identifying the deformity as some-thing "cute" or "special" or may equate deformitieswith a sense of "badness" in relation to other family _ ..

members.Children with lesser intelligence focus more on func-

tional limitations as a defense mechanism and denyany cosmetic significance to their deformity. Theseyoungsters become more appreciative postoperatively,because they are freed of their defenses. Any deformity, FIG. 5b. This is a postoperative view following bilateral mandibular

congenyinhibit normal psycho- osteotomies with elongation of both rami, construction of a temporo-congenital or acquired, may Inhlblt normal psycho- mandibular joint, rotation genioplasty and correction of open bite.logical growth. The fact that inhibition develops fol- Occlusal plane is level.

Ann. Surg. * September 1979

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MAXILLOCRANIOFACIAL SURGERY 329

introspection with acknowledgement of the previouslyexisting deformity. During the fourth or reintegrationphase, the psychological defenses reorganize: withincreased intellectual freedom, a changing of socialpriorities, and increased interpersonal relationships.This phase may last from six weeks to six monthspostoperatively.Change in body image does not necessarily correlate

with the degree of anatomic improvement. In addition,intellectual function is often improved despite the ab-sence of any organic change. Consequently childrenperform better at home; and in school some childrencan be transferred from the specialized programs to theregular classroom. Following trauma or tumor thepatient often denies the deformity until the operativecorrection has been performed. Depression, with-drawal, and eventual alteration in behaviour may beseen in the patient with posttraumatic deformity.

This type of patient is especially vulnerable duringthe late postoperative period.Our craniofacial team's overview of child develop-

ment is illustrated in Figure 6. The normal milestones oc-cur at infancy, school age, adolescence, and adulthood.

FIG. 5c. This sketch was produced preoperatively by the paientin Figure 5a when tested by standard "Draw-a-Person" test in whichhe is asked to draw a picture of "a man."

FIG. 5d. Same test when repeated 6 weeks postoperatively, re-veals striking change in body image.

At each level psychological function exerts its influencein specific ways. For example, a deformed infant, whendeprived may drift further and further from the "main-stream" as a sense of loss and disruption of parentalbonding exerts its influence. The parents, expectingthe idealized child, have a difficult adjustment to makeat birth. During infancy, there is a normal pattern ofincreasing sense of competence with sensory inputfrom hands, mouth, eyes, ears, and nose. Congenitaldeformity may lead to a sense of incompetence,isolation, and eventual psychological constriction.At school age peer interaction, cognitive growth and

mastery of skills assume increasing importance. Nowthe deformed child is again handicapped and thwarted.Peer isolation begins, lowered cognitive performanceand a failed sense of mastery becomes a subconsciouspart of his very being.

During adolescence when consolidation of identity,heterosexuality, career goals and resolution of conflictare major problems, even in the most physically en-dowed, the deformed youngster becomes further andfurther separated from his peers. There is uncertainsense of identity, diminished career goals, a feeling ofseclusion and failure which often envelopes the patient.Whereas the normal adult has a family, career

satisfaction, peer acceptance and a sense of complete-ness, the deformed adult has a sense of isolation, dimin-ished achievement, incomplete feeling, conflict andhostility.

Surgical intervention at any phase tends to shift thedeformed patient toward normal pathways. In contrasta defect from trauma or tumor occurring in a normalperson tends to shift development away from the

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330 MURRAY AND OTHERS Ann. Surg. * September 1979

BODY IMAGE DEVELOPMENT

NORMAL e DISTORTED

PARENTAL PARENTALBONDING BIRT FAILURE

COMPETENCE INFANCY INCOMPETENCE

IPTERACTION SCHOOL ISOLATIONnVrEfCTIONAGE

IDENTITY ADOLESCENCE CONSTRICTION

CAREER ADULT SEC LUSIONFAMILY S

FIG. 6. The contrasting pathways of development between normaland distorted diverge more with each milestone of infancy, schoolage, adolescence, and adulthood. Note progressive constrictionleading to isolation and seclusion in the deformed person.

"'mainstream." Our thesis is that earliest possiblecorrection of deformity makes the journey easier,shorter and more complete.

AcknowledgmentsWe would like to acknowledge the Departments of Neurosurgery,

Radiology, Ophthalmology, Otolaryngology, Dentistry, Anesthesiaand Social Services.

References1. Belfer, M. D., Harrison, A. M. and Murray, J. E.: Body

Image and the Process of Reconstructive Surgery. Am. J.Dis. Child., (in press).

2. Converse, J. M., Ransohoff, J., Mathews, E. S., et al.:Ocular Hypertelorism and Pseudohypertelorism. Plast.Reconstr. Surg., 45:1, 1970.

3. Converse, J. M., Wood-Smith, D. and McCarthy, J. G.:Report on a Series of 50 Craniofacial Operations. Plast.Reconstr. Surg., 55:283, 1975.

4. Edgerton, M. T., Jane, J. A., Berry, F. A. and Marshall,K. A.: New Surgical Concepts Resulting fraom Cranio-orbito-facial Surgery. Ann. Surg., 182:228, 1975.

5. Hanson, J. W., Sayers, M. P., Knopp, L. M., et al.:Subtotal Neonatal Calvariectomy for Severe Craniosynostosis.J. Pediatr., 91:257, 1977.

6. Marchac, D.: Radical Forehead Remodeling for Cranio-stenosis. Plast. Reconstr. Surg., 61:823, 1978.

7. McCarthy, J. G., Coccaro, P. J., Epstein, F. and Converse,J. M.: Early Skeletal Release in the Infant with Cranio-facial Dysostosis. Plast. Reconstr. Surg., 62:335, 1978.

8. Moss, M. L.: Twenty Years of Functional Cranial Analysis.Am. J. Orthod. 61:479, 1972.

9. Munro, 1. R.: Orbito-cranio-facial Surgery: The TeamApproach. Plast. Reconstr. Surg., 55:170, 1975.

10. Murray, J. E. and Swanson, L. T.: Mid-face Osteotomy andAdvancement for Craniosynostosis. Plast. Reconstr. Surg.,41:299, 1968.

11. Murray, J. E., Swanson, L. T., Strand, R. D. and Hricko,G. M.: Evaluation of Craniofacial Surgery in the Treatmentof Facial Deformities. Ann. Surg., 182:240, 1975.

12. Murray, J. E., Kaban, L. B. and Mulliken, J. B.: Cranio-facial Abnormalities. In Ravitch, M. M., Welch, K. J., et al.(eds.) Pediatric Surgery, Chicago, Yearbook MedicalPublishers, Inc., 1979, pp. 233-248.

13. Ortiz-Monasterio, F., Del Campo, F. and Carrillo, A.:Advancement of the Orbits and the Midface in One Piece,Combined with Frontal Repositioning, for Correction ofCrouzon's Deformities. Plast. Reconstr. Surg., 61:507, 1978.

14. Tessier, P.: Osteotomies totales de la face; syndrome deCrouzon; syndrome d'Apert; oxycephalies, scaphocephalies,turricephalies. Ann. Chir. Plast. 12:273, 1967.

15. Tessier, P.: Relationship of Craniostenoses to Cranio-facial Dysostoses and to Faciostenoses. Plast. Reconstr. Surg.,48:224, 1971.

16. Tessier, P.: The Definitive Plastic Surgical Treatment of theSevere Facial Deformities of Craniofacial Dysostosis.Plast. Reconstr. Surg., 48:419, 1971.

17. Tessier, P.: Experiences in the Treatment of Orbital Hyper-telorism. Plast. Reconstr. Surg., 53:1, 1974.

18. Whitaker, L. A., Schut, L. and Randall, P.: CraniofacialSurgery: Present and Future. Ann. Surg., 184:558, 1976.

DISCUSSION

DR. BRADFORD CANNON (Boston, Massachusetts): DuringWorld War II, he contributed to the rehabilitation of wounded per-sonnel with severe injuries to the extremities and hands. His nextinterest was focused on the problems of the failing kidney, to thesolution of which problems he has made most significant contribu-tions, both in the techniques of kidney transplantation, and alsoin the discovery, control and use of the immunosuppressive agentsused in prolonging the survival of such kidneys.

Dr. Murray is one of a handful of world leaders in maxillocranio-facial surgery. As Dr. Murray has told us, the significance of theseprocedures to the growing child speaks for itself.For myself, I enjoy papers such as this, in which I cannot only see,

but almost touch and feel the procedure and the result. Dr. Murray'spaper struck me as one to be enjoyed because the results can bevisualized.

Dr. Murray, your follow-up study is a most enlightening report,and an inspiration to all of us, aware as we must be that every indi-vidual is concerned about his or her appearance. You have shown usways in which some of the most seriously deformed can be helped.

DR. MILTON T. EDGERTON (Charlottesville, Virginia): At Charlot-tesville, we have some modest differences in technical approachesto the patient with craniofacial deformities, but we would agree"right down the line" with the major points of Dr. Murray's thesistoday.

I realize that most of you are not dealing daily with these patients,but I suspect all of you are interested from time to time in catching up


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