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Osteotomies and bone grafts in treatment of developmental facial deformities
Osteotomies and Bone Grafts in Treatment of Developmental Facial Deformities David W. Furnas, MD, Irvine, California Bruce M. Achauer, MD, Irvine, California Renato F. Rubertl, MD, Nairobi, Kenya Don Ft. DeFeo, MD, Irvine, California
Recent advances in the surgical correction of developmental deformities of the facial skeleton have been rapid and dramatic. Testier [1,2] built upon the pioneering success of Gillies and Millard [3] and developed approaches from within the cranial vault to supplement external approaches to the facial skeleton. Their technics are replete with innovations designed to dismantle the deformed facial skeleton, augment it with bone grafts, reassemble it, and fix it by internal and external means to obtain improved facial contours and function. Others [4-101 have added their ideas and experience to the pool of knowledge in craniofacial surgery. Improvements in the design of bone-cutting instrument8 have greatly facilitated the execution of intricate facial osteotomies. The technical advances stimulated by craniofacial surgery have created simultaneous progress in management of deformities of all the parts of the facial skeleton, whether a cranial component is present or not. Craniofacial osteotomies of the upper face and anterior cranial fossa are designed for safe and direct treatment of deformities of the orbits, frontal area, and interorbital 8pace, particularly of craniofacial dysostoses, hypertelorism (Figure l), orbital dystopia (Figure 2), and the defect8 associated with plagiocephaly. Subcranial osteotomies sometimes suffice where the approach skirts the upper orbit and ethmoid areas. Osteotomies for deformities of the midface alone are designed upon a multitude of variations and extensions of LeFort’s fracture line8 coupled with bone grafts. Facial stenosis (Figure 3), mandibulofacial dySO8tOBiS (Figure 4), hemifacial microsomia, and
maxi&y protrusion or retrusion (with or without clefts) are among the problem8 in this group. Deformities of the mandible such as prognathism (Figure 5), retrognathism, and laterognathism are treated with osteotomies performed through either external or intraoral approaches. The surgeon has a wide choice among the technics described for these deformities [ I1 1. Summary
Recent developments in facial and craniofacial osteotomies have opened up new frontiers in the correction of developmental deformities of the face and jaws. Clinical examples are illustrated. Acknowledgment: We acknowledge the help of A. Michael Wood, FRCS, Director General, African Medical and Research Foundation (incorporating the East African Flying Doctors’ Service). References 1.
2. 3. 4. 5.
6.
7.
6. 9. From the DIVLS~IXIof Plastk Sugwy; Unlvwrity of Calltomla. Irvine. lrvifm, California. Reprint requests should be a&bssed to David W. Funas. MD, DMakn of Plastic Swgety. UnlversRy of Callfcunla. Irvine, Irvine, CallfOmia 92717. Presented at the Fwty-Seventh Annual Meeting of the Paclfic Coast Surgical Association, Monterey. Califcmia. February 15-18. 1976.
258
10. 11. 12.
Tessief P: Relationship of craniostenoses to cfanlofacial dy.3os&e6,andtofacbstenoses.P&sfReumsfrSlrg48:224. 1971. Tessbr P, Guiot G. Derome P: Crbital hypertebrlsm. &and J Pk3s.tRewnstr Surg 7: 39,1973. Gill&s H. Millard DR: Prlnclpbs and Art of Plastic Surgery, vol II. Boston, Little. Brown, 1957. p 551. converse Jh4, wood-&M D. McCarthy JG, Coccaro PJ: Craniofacial surgery. C//n Rast mg 1: 499. 1974. Murray JE, Swanson LT. Strand RD. Hrbko GM: Evaluation of cranbfacial surgery In the treatment of facial daformitbs. Ann Surg 162: 240,1975. Edgerton MT. Jane JA, Berry FA, Marshall KA: New sugbal concepts resulting from cranl&itc+facial surgery. Ann Swg 162: 226,1975. Ortiz4onestwio F: A mobile unit for the detection and care of craniofacial anomalies. Plest Reconstr Swg 55: 166. 1975. slqpy: theteamapfmach. Pk4.d Mmro IR: Orbmconsfr Surg 55: 170.1975. Salyer KE. et al: Difficulties and problarns to be solved in the approach to cranbfacial malfmnatbns. Birth defects: orlginal articles series. 11: 315, 1975. Whttaker LA, Randall P: The developing field of craniofaclal sugary. Pedialrics 54: 571,1974. Hinds EC, Kent JN: Surgical Treatment of Developmental Jaw Defcmnities. St. Louis, CV Mosby, 1972. Converse JM, Horowitz SL. Valauri AJ. Montandon D: The
lheAmukanJwmalolSuqmy
Developmental Facial Deformities
Fipre 1. A, hyperteiorism wtth midline craniofacial cleft In a tourteen year old girl. Repaired parasagittai cieR of 1~118and a medan cleti of the lip. 6, cephaiometry shows a 44 mm Interorbital distance ( vs a normal interorbital distance of 25 mm). C, intomrbUai space has been diminished to the correct size by T-r’s plocedure, moving the functtonai o&its as bkcks. The ciett nose was sinwttaneoosly treated by mkfiine excision, x-plasty, and placement of a bone graft. 0, tacial appearance two months postoperatively.
treatment of nasomaxillary hypoplasia. Plasr Reconstr Surg 45: 527, 1970. DiSCU2UiiCWl
John M. Coin (LOSAngeles, CA): Little has been written about the complications of orbitocraniofacial operations. I would like to share with you some data from our series of nineteen craniofacial operations at Children’s Hospital of Los Angeles. Average operating time was 7 hours (range, 4.5 to 11 hours). Average blood loss was 2,000 cc (range, 300 to 3,000
vohma 132. Au&w! 1076
cc). Our youngest patient was eight months old, our oldest nineteen years. Our series includes the four major groups amenable to orbitocraniofacial operations: hypertelorism (7 patients), the Crouzon-Apert spectrum of deformities (5 patients), orbitofacial clefts (2 patients), and orbitofrontal dystopias (5 patients). The Crouzon’s group provides the greatest technical challenge since the operation must correct simultaneously defects of orbital volume, abnormal intermaxillary relationships, and profile deformities. All but one of our com-
259
Furnas et al
Figure 2. A, otbltal dystqMa ( vertkal displacement) associated wlth repaired oculooral clefts and a ckft palate in an ei#teen yew old boy. 6, dystopla of left orbtt improved by nwvlng the left functknal orbit upward by means of cranlofaclal osteotomles (early postoperative pkWe). 7heleftkweflMhasbeenbuM up w&h a medlan forehead flap. Future prom&es will bn9uda et&k tp correct the nose and the right lower lid.
Figure 3. A, devekptmntal facial stenadswnhretnIsknofmkk#ethhrt of face. 6, facial advancement by means of Converse osteotomks [ 12 ] and bone grafts.
plications were in this group. There were no deaths. We have had no cerebral complications or blindness. There were two postoperative infections. In one patient a bone graft was partially lost. One bone graft has nearly completely resorbed without infection. Our most serious complication was necrosis of the premaxilIa, possibly due to an anomalous blood supply, in a child with an exceptionally severe Crouzon’s deformity. One patient bled massively postoperatively from the internal maxillary artery or its branches. Bleeding was controlled by repeated packing. This complication has not been reported before. In two early patients the lacrimal
duct was torn during the operation. Both injuries were recognized and repaired. The levator palpebrae muscle is particularly susceptible to injury or hematoma formation during the periorbital dissection. Ptosis may also be due to chronic stretching of the muscle in an effort to protect the cornea in patients with exophthalmos. Two patients with Crouzon’s disease had ptosis postoperatively and secondary ptosis repairs were required. Edgerton et al (&and J Plast Reconstr Surg 8: 164, 1974) reported seventeen craniofacial operations in patients less than six years of age. There was one death and
The Amwksn
Journal 04 Sugary
Developmental
Facial Deformities
Fiwre 4. A, two year o&t #I wlth mandlbulokclal dysostosk ( Treachsr-CoMw syn&me). Pat&nt has anthno~kld lateral canthl, cokbomas of lower /Ids, clefl zycpmat/c arches with absent zy#nna!k bodies (plus underdevekped /a w, cleft palate, and bilateral mkrotta). (Rep&ted wtth permkskn of pub&her from Furnas DW: Problems In plannlngj mkrotla. Symposium on R&o&ntctkn of the Aurkk [ Tanzer UC, Edgetion MT, ed]. CV My, 1874.) 6, t&ee years a&r com9&on by means of nwv@ t+ lateral cant/U upward via bkotvnal lndokrrs and by tilling the clefts and bulhitng up the facial sk&Mon with bone and cartlkge~arrShumtfbe(pksckftpaiate repalr).
F&o-e 5. A, eleven year ohl girl wnlt
fancy. Patknt can neither ripesk undMMddJ&nWCheW.-hl3S devekymd
a permanent proonethk deformHy w/th an -fbb wlds open bite. 8,’ tfwee years after a two-stage reductkn of the .torrgve, suprahyokf dksectkn, 8nd vertkal mandNlar osteotomks wltlr coronokkctomies. Patiwtt can ckee her mouth. She had hdJ range of mandkbulsr movement and IntelllgAble speech.
another patient suffered permanent brain damage, suggesting that serious risks are greater in the younger age group. Doctor Furnas uses spinal drainage. We have avoided its use and have had no cerebral complications. Perhaps our neurosurgeon, Doctor Frank Anderson, who is in the audience, would wish to comment on this. We are aware of one death, in another state from tonsillar herniation, which can be directly attributed to difficulties with the management of spinal drainage postoperatively. The author5 alluded to evidence indicating that facial growth is not affected by these operations. Although there
volulno 132. Att!Jusi1976
is much evidence to confirm this view, there is a disturbing report by Hogeman and Willmar (&and J Plast Reconstr Surg 8: 169, 1974) which describes retarded growth and relapse of proptosis four year5 after operation in a ten year old patient with Crouzon’s disease. Finally, I would like to suggest, and I am certain the authors will agree with me, that these operations should only be done in major centers so that experienced teams may be developed. The analogy with heart surgery is a strong one and I feel certain that time will show a strong correlation between the level of team activity and outcome in craniofacial surgery.
261
Furnas et al
Frank M. Anderson (Los Angeles, CA): I appreciate being asked to make comments on the presentations by the authors and Doctor Coin. I want to say a little about the neurosurgical aspects of the midface deformities of the Crouzon type. This complicated process seems to be caused by prenatal or early postnatal closure of several joints in the base of the skull, including the sphenojd articulations. The cause of this process remains unknown. Some of us in neurosurgery have, for several years, resected portions of the lateral sphenoid region when freeing the frontal bones for correction of unicoronal and bicoronal synostosis in infants up to six months of age. In at least some patients, this approach has apparently allowed normal development of the midface and prevented serious deformities. Concerning the matter of preoperative withdrawal of spinal fluid, as mentioned by Doctor Goin; retraction of the brain during more involved frontal procedures is simpler if approximately 60 cc of spinal fluid is removed by lumbar puncture just before operation; intravenous mannitol is equally effective. It is my belief that the corrective skull and facial procedures discussed here are very important for the well being and function as well as the psychic status of the pa-
2%
tient. The operations should not be disparagingly to as “only cosmetic.”
referred
David W. Furnas (closing): I will remember what John Goin has said about the tonsillar herniation with spinal drainage. That is a good point. As far as facial growth is concerned, I think in bypertelorism the evidence is that we are not interfering with it. We are not sure. This surgery has not been going on for a long time, but the point Doctor Goin made about Crouxon’s is quite well taken and we might well be interfering with growth. I think this whole area is being assessed gradually. We don’t have any final answers. The patient’s age for Crouzon’s operations we cannot say for sure. I did not mean to belittle the neurosurgeon at all. It is the neurosurgeon and anesthesiologist who come to these patients while we indulge in our arts and crafts, and we appreciate this. One final comment. I think it is a most interesting area of endeavor that Doctor Anderson has commented on-the possibility of preventing the Crouzon’s deformity by appropriate sutural work at an early age. This is another area in which work is just beginning.
The Amerlcan Journal of !%rgwy
Recent advances in the surgical correction of developmental deformities of the facial skeleton have been rapid and dramatic. Testier [1,2] built upon the pioneering success of Gillies and Millard [3] and developed approaches from within the cranial vault to supplement external approaches to the facial skeleton. Their technics are replete with innovations designed to dismantle the deformed facial skeleton, augment it with bone grafts, reassemble it, and fix it by internal and external means to obtain improved facial contours and function. Others [4-101 have added their ideas and experience to the pool of knowledge in craniofacial surgery. Improvements in the design of bone-cutting instrument8 have greatly facilitated the execution of intricate facial osteotomies. The technical advances stimulated by craniofacial surgery have created simultaneous progress in management of deformities of all the parts of the facial skeleton, whether a cranial component is present or not. Craniofacial osteotomies of the upper face and anterior cranial fossa are designed for safe and direct treatment of deformities of the orbits, frontal area, and interorbital 8pace, particularly of craniofacial dysostoses, hypertelorism (Figure l), orbital dystopia (Figure 2), and the defect8 associated with plagiocephaly. Subcranial osteotomies sometimes suffice where the approach skirts the upper orbit and ethmoid areas. Osteotomies for deformities of the midface alone are designed upon a multitude of variations and extensions of LeFort’s fracture line8 coupled with bone grafts. Facial stenosis (Figure 3), mandibulofacial dySO8tOBiS (Figure 4), hemifacial microsomia, and
maxi&y protrusion or retrusion (with or without clefts) are among the problem8 in this group. Deformities of the mandible such as prognathism (Figure 5), retrognathism, and laterognathism are treated with osteotomies performed through either external or intraoral approaches. The surgeon has a wide choice among the technics described for these deformities [ I1 1. Summary
Recent developments in facial and craniofacial osteotomies have opened up new frontiers in the correction of developmental deformities of the face and jaws. Clinical examples are illustrated. Acknowledgment: We acknowledge the help of A. Michael Wood, FRCS, Director General, African Medical and Research Foundation (incorporating the East African Flying Doctors’ Service). References 1.
2. 3. 4. 5.
6.
7.
6. 9. From the DIVLS~IXIof Plastk Sugwy; Unlvwrity of Calltomla. Irvine. lrvifm, California. Reprint requests should be a&bssed to David W. Funas. MD, DMakn of Plastic Swgety. UnlversRy of Callfcunla. Irvine, Irvine, CallfOmia 92717. Presented at the Fwty-Seventh Annual Meeting of the Paclfic Coast Surgical Association, Monterey. Califcmia. February 15-18. 1976.
258
10. 11. 12.
Tessief P: Relationship of craniostenoses to cfanlofacial dy.3os&e6,andtofacbstenoses.P&sfReumsfrSlrg48:224. 1971. Tessbr P, Guiot G. Derome P: Crbital hypertebrlsm. &and J Pk3s.tRewnstr Surg 7: 39,1973. Gill&s H. Millard DR: Prlnclpbs and Art of Plastic Surgery, vol II. Boston, Little. Brown, 1957. p 551. converse Jh4, wood-&M D. McCarthy JG, Coccaro PJ: Craniofacial surgery. C//n Rast mg 1: 499. 1974. Murray JE, Swanson LT. Strand RD. Hrbko GM: Evaluation of cranbfacial surgery In the treatment of facial daformitbs. Ann Surg 162: 240,1975. Edgerton MT. Jane JA, Berry FA, Marshall KA: New sugbal concepts resulting from cranl&itc+facial surgery. Ann Swg 162: 226,1975. Ortiz4onestwio F: A mobile unit for the detection and care of craniofacial anomalies. Plest Reconstr Swg 55: 166. 1975. slqpy: theteamapfmach. Pk4.d Mmro IR: Orbmconsfr Surg 55: 170.1975. Salyer KE. et al: Difficulties and problarns to be solved in the approach to cranbfacial malfmnatbns. Birth defects: orlginal articles series. 11: 315, 1975. Whttaker LA, Randall P: The developing field of craniofaclal sugary. Pedialrics 54: 571,1974. Hinds EC, Kent JN: Surgical Treatment of Developmental Jaw Defcmnities. St. Louis, CV Mosby, 1972. Converse JM, Horowitz SL. Valauri AJ. Montandon D: The
lheAmukanJwmalolSuqmy
Developmental Facial Deformities
Fipre 1. A, hyperteiorism wtth midline craniofacial cleft In a tourteen year old girl. Repaired parasagittai cieR of 1~118and a medan cleti of the lip. 6, cephaiometry shows a 44 mm Interorbital distance ( vs a normal interorbital distance of 25 mm). C, intomrbUai space has been diminished to the correct size by T-r’s plocedure, moving the functtonai o&its as bkcks. The ciett nose was sinwttaneoosly treated by mkfiine excision, x-plasty, and placement of a bone graft. 0, tacial appearance two months postoperatively.
treatment of nasomaxillary hypoplasia. Plasr Reconstr Surg 45: 527, 1970. DiSCU2UiiCWl
John M. Coin (LOSAngeles, CA): Little has been written about the complications of orbitocraniofacial operations. I would like to share with you some data from our series of nineteen craniofacial operations at Children’s Hospital of Los Angeles. Average operating time was 7 hours (range, 4.5 to 11 hours). Average blood loss was 2,000 cc (range, 300 to 3,000
vohma 132. Au&w! 1076
cc). Our youngest patient was eight months old, our oldest nineteen years. Our series includes the four major groups amenable to orbitocraniofacial operations: hypertelorism (7 patients), the Crouzon-Apert spectrum of deformities (5 patients), orbitofacial clefts (2 patients), and orbitofrontal dystopias (5 patients). The Crouzon’s group provides the greatest technical challenge since the operation must correct simultaneously defects of orbital volume, abnormal intermaxillary relationships, and profile deformities. All but one of our com-
259
Furnas et al
Figure 2. A, otbltal dystqMa ( vertkal displacement) associated wlth repaired oculooral clefts and a ckft palate in an ei#teen yew old boy. 6, dystopla of left orbtt improved by nwvlng the left functknal orbit upward by means of cranlofaclal osteotomles (early postoperative pkWe). 7heleftkweflMhasbeenbuM up w&h a medlan forehead flap. Future prom&es will bn9uda et&k tp correct the nose and the right lower lid.
Figure 3. A, devekptmntal facial stenadswnhretnIsknofmkk#ethhrt of face. 6, facial advancement by means of Converse osteotomks [ 12 ] and bone grafts.
plications were in this group. There were no deaths. We have had no cerebral complications or blindness. There were two postoperative infections. In one patient a bone graft was partially lost. One bone graft has nearly completely resorbed without infection. Our most serious complication was necrosis of the premaxilIa, possibly due to an anomalous blood supply, in a child with an exceptionally severe Crouzon’s deformity. One patient bled massively postoperatively from the internal maxillary artery or its branches. Bleeding was controlled by repeated packing. This complication has not been reported before. In two early patients the lacrimal
duct was torn during the operation. Both injuries were recognized and repaired. The levator palpebrae muscle is particularly susceptible to injury or hematoma formation during the periorbital dissection. Ptosis may also be due to chronic stretching of the muscle in an effort to protect the cornea in patients with exophthalmos. Two patients with Crouzon’s disease had ptosis postoperatively and secondary ptosis repairs were required. Edgerton et al (&and J Plast Reconstr Surg 8: 164, 1974) reported seventeen craniofacial operations in patients less than six years of age. There was one death and
The Amwksn
Journal 04 Sugary
Developmental
Facial Deformities
Fiwre 4. A, two year o&t #I wlth mandlbulokclal dysostosk ( Treachsr-CoMw syn&me). Pat&nt has anthno~kld lateral canthl, cokbomas of lower /Ids, clefl zycpmat/c arches with absent zy#nna!k bodies (plus underdevekped /a w, cleft palate, and bilateral mkrotta). (Rep&ted wtth permkskn of pub&her from Furnas DW: Problems In plannlngj mkrotla. Symposium on R&o&ntctkn of the Aurkk [ Tanzer UC, Edgetion MT, ed]. CV My, 1874.) 6, t&ee years a&r com9&on by means of nwv@ t+ lateral cant/U upward via bkotvnal lndokrrs and by tilling the clefts and bulhitng up the facial sk&Mon with bone and cartlkge~arrShumtfbe(pksckftpaiate repalr).
F&o-e 5. A, eleven year ohl girl wnlt
fancy. Patknt can neither ripesk undMMddJ&nWCheW.-hl3S devekymd
a permanent proonethk deformHy w/th an -fbb wlds open bite. 8,’ tfwee years after a two-stage reductkn of the .torrgve, suprahyokf dksectkn, 8nd vertkal mandNlar osteotomks wltlr coronokkctomies. Patiwtt can ckee her mouth. She had hdJ range of mandkbulsr movement and IntelllgAble speech.
another patient suffered permanent brain damage, suggesting that serious risks are greater in the younger age group. Doctor Furnas uses spinal drainage. We have avoided its use and have had no cerebral complications. Perhaps our neurosurgeon, Doctor Frank Anderson, who is in the audience, would wish to comment on this. We are aware of one death, in another state from tonsillar herniation, which can be directly attributed to difficulties with the management of spinal drainage postoperatively. The author5 alluded to evidence indicating that facial growth is not affected by these operations. Although there
volulno 132. Att!Jusi1976
is much evidence to confirm this view, there is a disturbing report by Hogeman and Willmar (&and J Plast Reconstr Surg 8: 169, 1974) which describes retarded growth and relapse of proptosis four year5 after operation in a ten year old patient with Crouzon’s disease. Finally, I would like to suggest, and I am certain the authors will agree with me, that these operations should only be done in major centers so that experienced teams may be developed. The analogy with heart surgery is a strong one and I feel certain that time will show a strong correlation between the level of team activity and outcome in craniofacial surgery.
261
Furnas et al
Frank M. Anderson (Los Angeles, CA): I appreciate being asked to make comments on the presentations by the authors and Doctor Coin. I want to say a little about the neurosurgical aspects of the midface deformities of the Crouzon type. This complicated process seems to be caused by prenatal or early postnatal closure of several joints in the base of the skull, including the sphenojd articulations. The cause of this process remains unknown. Some of us in neurosurgery have, for several years, resected portions of the lateral sphenoid region when freeing the frontal bones for correction of unicoronal and bicoronal synostosis in infants up to six months of age. In at least some patients, this approach has apparently allowed normal development of the midface and prevented serious deformities. Concerning the matter of preoperative withdrawal of spinal fluid, as mentioned by Doctor Goin; retraction of the brain during more involved frontal procedures is simpler if approximately 60 cc of spinal fluid is removed by lumbar puncture just before operation; intravenous mannitol is equally effective. It is my belief that the corrective skull and facial procedures discussed here are very important for the well being and function as well as the psychic status of the pa-
2%
tient. The operations should not be disparagingly to as “only cosmetic.”
referred
David W. Furnas (closing): I will remember what John Goin has said about the tonsillar herniation with spinal drainage. That is a good point. As far as facial growth is concerned, I think in bypertelorism the evidence is that we are not interfering with it. We are not sure. This surgery has not been going on for a long time, but the point Doctor Goin made about Crouxon’s is quite well taken and we might well be interfering with growth. I think this whole area is being assessed gradually. We don’t have any final answers. The patient’s age for Crouzon’s operations we cannot say for sure. I did not mean to belittle the neurosurgeon at all. It is the neurosurgeon and anesthesiologist who come to these patients while we indulge in our arts and crafts, and we appreciate this. One final comment. I think it is a most interesting area of endeavor that Doctor Anderson has commented on-the possibility of preventing the Crouzon’s deformity by appropriate sutural work at an early age. This is another area in which work is just beginning.
The Amerlcan Journal of !%rgwy