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Congenital bilateral zygomatico-mandibular fusion with mandibular hypoplasia
Brirish Journal oj Plastic Surgery ( I996), 49. 25 I-253 0 1996 The British Association of Plastic Surgeons
Congenital bilateral zygomatico-mandibular hypoplasia
fusion with mandibular
S. Kamata, K. Satoh, T. Uemura and T. Onizuka
Depurtment of Plastic and Reconstructive Surgery, Showa University School of Medicine, Tokyo, Japan S UMMAR Y. We report a very rare case of congenital bilateral zygomatico-mandibular fusion with mandibular hypoplasia. Soon after birth, the patient underwent tracheotomy because of upper airway obstruction. At the age of one and a half years, reconstruction of the temporomandibular joint and lengthening of the ramus with a costochondral graft were performed. Only a few cases of congenital bony syngnathia revealed in this case have been described in the literature.
Congenital mandibular hypoplasia, such as that seen in the Robin sequence or hemifacial microsomia, is relatively common, but a case lacking temporomandibular joints and condyles as in severe types of hemifacial microsomia, is seldom encountered. We present a very rare case of congenital bilateral zygomatico-mandibular fusion with severe limitation of mouth opening.
Discussion
Case report A 3982 g boy was born in October 1992 with severe limitation of mouth opening. The pregnancy and delivery were normal. There were no relevant features in the family history. As mandibular hypoplasia and upper airway obstruction were noted soon after birth, he was admitted to the neonatal intensive care unit and underwent tracheotomy to obtain an adequate airway. No other abnormal findings were noticed, except for bilateral low-set ears. Four months later, he was discharged from hospital with the tracheotomy. He could hardly open his mouth and masticate because of severetemporomandibular joint ankylosis. He was fed via a gastric tube. At the age of one and a half years, three-dimensional CT scanning revealed that limitation of mouth opening was caused by zygomatico-mandibular fusion (Figs 1, 2). The hypoplastic mandible was found to be fused with the zygomas, part of the maxilla and part of the temporal bones. The temporomandibular joints, mandibular condyles and coronoid processeswere missing. The right zygomatic arch and part of the left zygomatic arch were absent. The left auditory meatus was present but the right external auditory meatus was absent. In April 1994, he had release of the bilateral zygomatico-mandibular fusion, reconstruction of the temporomandibular joints and lengthening of the mandibular rami with costochondral grafts, and reconstruction of the right zygomatic arch with a costal graft. The operation lasted about five hours and was uneventful. Unfortunately, the patient had a cardiac arrest soon after the operation. In spite of intensive care, he died about 24 hours after the operation. Since permission to conduct an autopsy was not given, the precise of cause of death could not be determined. 251
Congenital mandibular hypoplasia is often observed in the Robin sequence, craniofacial microsomia and mandibulofacial dysostosis.’ Congenital and acquired temporomandibular joint abnormalities occasionally induce joint ankylosis, resulting in limitation of mouth opening and occasional airway obstruction in newborns and infants. 2~4A P lasia of the temporomandibular joint and mandibular condyle is rarely observed in severe types of craniofacial microsomia; several successful reconstructions using costochondral grafts have been described. 1.5-7 However, zygomatico-mandibular fusion with mandibular hypoplasia as in our case is difficult to find in the literature. There have been numerous reports on fibrous syngnathia or fusion of the jaws. Sternberg et al.* reported a case of congenital fibrous alveolar fusion with incomplete cleft lip, cleft palate, and micrognathia. X-ray examination showed a small mandible, underdevelopment of the ascending rami, and poorly defined temporomandibular joints. Separation of the complete alveolar fusion caused gradual opening of the mouth. Verdi et al.’ and Haramis et al.” reported cleft palate and congenital lateral alveolar synechia syndrome consisting of bilateral fibrous interalveolar synechiae and isolated cleft palate. The treatment consisted of simple division of the bilateral synechial bands with scissors, which easily improved the patients’ mouth opening. Each of their patients was discharged from hospital after an uneventful course. Bony syngnathia is less common than fibrous syngnathia. Burket l1 described a case of congenital unilateral bony alveolar fusion with left facial herniatrophy. X-ray examination showed a poorly defined temporomandibular joint on that side. Operative examination revealed fusion of the left alveolar processes from a point located 1 cm from the midline to the retromolar area. Pettersson12 and Hogginsi3 reported cases of bony fusion of the jaws with congenital aglossia and cleft palate. The solid bony masses were found to connect the hard palate with the mandible. Salleh14
British Journal of Plastic Surnerv
Fig. 2
Fig. I Figure l-Preoperative frontal view at the age of 18 months. The patient could hardly open his mouth due to severe temporomandibular joint ankylosis. Tracheal cannula in the tracheotomy can be seen. Figure 2-Frontal view of three-dimensional CT scan demonstrating zygomatico-mandibular fusion with mandibular hypoplasia. The temporomandibular joints, mandibular condyles, and coronoid processes are missing.
reported a case of bilateral bony alveolar fusion with micrognathia, while Miskinyar” reported unilateral bony alveolar fusion with mild micrognathia. Goodacre and WallaceI reported a case of congenital bilateral bony alveolar fusion of a 39-year-old woman with mandibular hypoplasia. Although she could hardly open her mouth, no treatment was attempted in view of the patient’s age and the way in which she had become adapted to her disability. These cases had limitation of mouth opening due to alveolar fusion, but were different from our case. They did not have bony fusion of the temporomandibular joints; hence separation of the mandible and maxilla readily improved ankylosis of the temporomandibular joints and mouth opening. Sawhneyi7 reported 70 cases of bony ankylosis of the temporomandibular joint followed trauma. He classified his cases into four types by pathological severity which influenced the treatment. He reconstructed the temporomandibular joint using an acrylic cylinder. However, these cases should also be differentiated from our case because the bony fusion was only at the temporomandibular joints. Our case had complete fusion of the zygoma and mandible in one piece. We have found it difficult to find a similar case of such extensive bony fusion between the mandible and maxilla in the literature. Simpson and Maves18 reported a case of congenital fusion of the gums and jaws that was similar to ours. Their case had continuous bony fusion of the mandible and maxilla involving the ascending ramus of the mandible and the zygomatic arch. Additionally, Hochstetter” also pre-
sented a photograph of a skull recovered from an Austrian ossuary showing bony fusion of the mandible and maxilla. These congenital syngnathia rarely occur as isolated events and are often associated with various other anomalies. The most frequent associations are cleft palate and mandibular hypoplasia. We have cited nine cases of bony syngnathia, including the present case. Cleft palate was present in four of these cases and mandibular hypoplasia in five cases. This combination of anomalies is thought to derive from maldevelopment of the first branchial arch. Embryologically, the maxilla, palatine bone, and zygoma develop from the maxillary process of the first branchial arch, while the mandible and anterior part of the auricle develop from the mandibular process of the same arch. The second arch provides the posterior part of the auricle, while the first branchial groove furnishes the external auditory meatus. Maldevelopment of the first and second branchial arches results in hypoplasia of the maxilla, mandible, and auricle.4 Hemifacial microsomia and bilateral microsomia occur because of maldevelopment of the first and second branchial arches. Munro6 classified hemifacial microsomia into several types and stated that the zygomatic arch and mandibular condyle were missing in severe types. Converse et al.” reported bilateral facial microsomia and involvement of macrostomia and mandibular condylar hypoplasia in severe forms. Congenital temporomandibular joint ankylosis due to zygomatico-mandibular fusion seen in our case is a new category of facial skeletal
Congenital zygomatico-mandibular fusion anomaly and should be differentiated from these previously reported varieties of congenital facial skeletal deformities. The pathogenesis remains unknown in these deformities, but some developmental defect has occurred in the first branchial arch region in which separation of the mandible, maxilla, and zygoma failed to occur. Although the exact mechanism is obscure, some environmental factor may be the cause of this rare skeletal deformation.
253
II. 12. 13. 14.
15.
References I. McCarthy JG, Grayson BH, Coccaro PJ, Wood-Smith D. Craniofacial microsomia. In: McCarthy JG, ed. Plastic Surgery. Philadelphia: WB Saunders, 1990: 30544100. 2. Cosman B, Crikelair GF. Mandibular hypoplasia and the late development of glossopharyngeal airway obstruction. Plast Reconstr Surg 1972; 50: 57339. 3. Quinn GW, Pickrell KL. Mandibular hypoplasia and airway interference. Endangerment of life and impairment of health. Laryngeal-pharyngeal obstruction. NC Dent J 1978; 61: 19~24. 4. McCarthy JG, Kawamoto HK, Grayson BH. Colen SR, Coccaro PJ, Wood-Smith D. Surgery of the jaws. In: McCarthy JG, ed. Plastic Surgery. Philadelphia: WB Saunders. 1990: 11888474. 5. Munro IR. One-stage reconstruction of the temporomandibular joint in hemifacial microsomia. Plast Reconstr Surg 1980: 66: 6999710. 6. Munro IR. Treatment of craniofacial microsomia. Clin Plast Surg 1987; 14: 177-86. 7. Pensler JM. Christopher RD, Bewyer DC. Correction of micrognathia with ankylosis of the temporomandibular joint in childhood. Plast Reconstr Surg 1993; 91: 7999805. 8. Sternberg N. Sagher U. Golan J, Eidelman AI, Ben-Hur N. Congenital fusion of the gums with bilateral fusion of the temporomandibular joints. Plast Reconstr Surg 1983: 72: 385 7. 9. Verdi GD. O’Neal B. Cleft palate and congenital alveolar synechia syndrome. Plast Reconstr Surg 1984; 74: 684-686. 10. Haramis HT. Apesos J. Cleft palate and congenital lateral
16. 17.
18. 19.
20.
alveolar synechia syndrome: case presentation and literature review. Ann Plast Surg 1995; 34: 424430. Burket LW. Congenital bony temporomandibular ankylosis and facial hemiatroohv. JAMA 1936: 106: 1719-22. Pettersson G. Aglossia’congenita with bony fusion of the jaws. Report of one case. Acta Chir Stand 1961; 122: 93-5. Hoggins GS. Aglossia congenita with bony fusion of the jaws. Br J Oral Surg 1969; 7: 63-5. Salleh NM. Congenital partial fusion of the mandible and maxilla. Report of the case. Oral Surg Oral Med Oral Pathol 1965: 20: 74-6. Miskinyar SA. Congenital mandibulo-maxillary fusion. Plast Reconstr Surg 1979; 63: 120~~1. Goodacre TE. Wallace AF. Congenital alveolar fusion. Br J Plast Surg 1990; 43: 20339. Sawhney CP. Bony ankylosis of the temporomandibular joint: follow-up of 70 patients treated with arthroplasty and acrylic spacer interposition. Plast Reconstr Surg 1986; 77: 29940. Simpson JR, Maves MD. Congenital syngnathia or fusion of the gums and jaws. Otol Head Neck Surg 1985; 93: 9669. Hochstetter F. tiber zwei Falle epithelialer syngnathie bei menschlichen Keimlingen. Dtsch Med Wochenschr 1948: 108812. Converse JM, Wood-Smith D, McCarthy JG, Coccaro PJ, Becker MH. Bilateral facial microsomia. Diagnosis, classification, treatment. Plast Reconstr Surg 1974; 54: 413-23.
The Authors Satoshi Kamata MD, Senior Resident Kaneshige Satoh MD, Assistant Professor Tetsuji Uemura MD, Assistant Lecturer Takuya Onizuka MD, Professor Department of Plastic and University School of Medicine, Tokyo. 142, Japan. Correspondence
to Dr Satoshi
Paper received 18 May Accepted 30 November
Reconstructive l-5-8, Hatanodai,
Kamata.
1995. 1995, after
revision.
Surgery, Showa Shinagawa-ku,
Congenital bilateral zygomatico-mandibular hypoplasia
fusion with mandibular
S. Kamata, K. Satoh, T. Uemura and T. Onizuka
Depurtment of Plastic and Reconstructive Surgery, Showa University School of Medicine, Tokyo, Japan S UMMAR Y. We report a very rare case of congenital bilateral zygomatico-mandibular fusion with mandibular hypoplasia. Soon after birth, the patient underwent tracheotomy because of upper airway obstruction. At the age of one and a half years, reconstruction of the temporomandibular joint and lengthening of the ramus with a costochondral graft were performed. Only a few cases of congenital bony syngnathia revealed in this case have been described in the literature.
Congenital mandibular hypoplasia, such as that seen in the Robin sequence or hemifacial microsomia, is relatively common, but a case lacking temporomandibular joints and condyles as in severe types of hemifacial microsomia, is seldom encountered. We present a very rare case of congenital bilateral zygomatico-mandibular fusion with severe limitation of mouth opening.
Discussion
Case report A 3982 g boy was born in October 1992 with severe limitation of mouth opening. The pregnancy and delivery were normal. There were no relevant features in the family history. As mandibular hypoplasia and upper airway obstruction were noted soon after birth, he was admitted to the neonatal intensive care unit and underwent tracheotomy to obtain an adequate airway. No other abnormal findings were noticed, except for bilateral low-set ears. Four months later, he was discharged from hospital with the tracheotomy. He could hardly open his mouth and masticate because of severetemporomandibular joint ankylosis. He was fed via a gastric tube. At the age of one and a half years, three-dimensional CT scanning revealed that limitation of mouth opening was caused by zygomatico-mandibular fusion (Figs 1, 2). The hypoplastic mandible was found to be fused with the zygomas, part of the maxilla and part of the temporal bones. The temporomandibular joints, mandibular condyles and coronoid processeswere missing. The right zygomatic arch and part of the left zygomatic arch were absent. The left auditory meatus was present but the right external auditory meatus was absent. In April 1994, he had release of the bilateral zygomatico-mandibular fusion, reconstruction of the temporomandibular joints and lengthening of the mandibular rami with costochondral grafts, and reconstruction of the right zygomatic arch with a costal graft. The operation lasted about five hours and was uneventful. Unfortunately, the patient had a cardiac arrest soon after the operation. In spite of intensive care, he died about 24 hours after the operation. Since permission to conduct an autopsy was not given, the precise of cause of death could not be determined. 251
Congenital mandibular hypoplasia is often observed in the Robin sequence, craniofacial microsomia and mandibulofacial dysostosis.’ Congenital and acquired temporomandibular joint abnormalities occasionally induce joint ankylosis, resulting in limitation of mouth opening and occasional airway obstruction in newborns and infants. 2~4A P lasia of the temporomandibular joint and mandibular condyle is rarely observed in severe types of craniofacial microsomia; several successful reconstructions using costochondral grafts have been described. 1.5-7 However, zygomatico-mandibular fusion with mandibular hypoplasia as in our case is difficult to find in the literature. There have been numerous reports on fibrous syngnathia or fusion of the jaws. Sternberg et al.* reported a case of congenital fibrous alveolar fusion with incomplete cleft lip, cleft palate, and micrognathia. X-ray examination showed a small mandible, underdevelopment of the ascending rami, and poorly defined temporomandibular joints. Separation of the complete alveolar fusion caused gradual opening of the mouth. Verdi et al.’ and Haramis et al.” reported cleft palate and congenital lateral alveolar synechia syndrome consisting of bilateral fibrous interalveolar synechiae and isolated cleft palate. The treatment consisted of simple division of the bilateral synechial bands with scissors, which easily improved the patients’ mouth opening. Each of their patients was discharged from hospital after an uneventful course. Bony syngnathia is less common than fibrous syngnathia. Burket l1 described a case of congenital unilateral bony alveolar fusion with left facial herniatrophy. X-ray examination showed a poorly defined temporomandibular joint on that side. Operative examination revealed fusion of the left alveolar processes from a point located 1 cm from the midline to the retromolar area. Pettersson12 and Hogginsi3 reported cases of bony fusion of the jaws with congenital aglossia and cleft palate. The solid bony masses were found to connect the hard palate with the mandible. Salleh14
British Journal of Plastic Surnerv
Fig. 2
Fig. I Figure l-Preoperative frontal view at the age of 18 months. The patient could hardly open his mouth due to severe temporomandibular joint ankylosis. Tracheal cannula in the tracheotomy can be seen. Figure 2-Frontal view of three-dimensional CT scan demonstrating zygomatico-mandibular fusion with mandibular hypoplasia. The temporomandibular joints, mandibular condyles, and coronoid processes are missing.
reported a case of bilateral bony alveolar fusion with micrognathia, while Miskinyar” reported unilateral bony alveolar fusion with mild micrognathia. Goodacre and WallaceI reported a case of congenital bilateral bony alveolar fusion of a 39-year-old woman with mandibular hypoplasia. Although she could hardly open her mouth, no treatment was attempted in view of the patient’s age and the way in which she had become adapted to her disability. These cases had limitation of mouth opening due to alveolar fusion, but were different from our case. They did not have bony fusion of the temporomandibular joints; hence separation of the mandible and maxilla readily improved ankylosis of the temporomandibular joints and mouth opening. Sawhneyi7 reported 70 cases of bony ankylosis of the temporomandibular joint followed trauma. He classified his cases into four types by pathological severity which influenced the treatment. He reconstructed the temporomandibular joint using an acrylic cylinder. However, these cases should also be differentiated from our case because the bony fusion was only at the temporomandibular joints. Our case had complete fusion of the zygoma and mandible in one piece. We have found it difficult to find a similar case of such extensive bony fusion between the mandible and maxilla in the literature. Simpson and Maves18 reported a case of congenital fusion of the gums and jaws that was similar to ours. Their case had continuous bony fusion of the mandible and maxilla involving the ascending ramus of the mandible and the zygomatic arch. Additionally, Hochstetter” also pre-
sented a photograph of a skull recovered from an Austrian ossuary showing bony fusion of the mandible and maxilla. These congenital syngnathia rarely occur as isolated events and are often associated with various other anomalies. The most frequent associations are cleft palate and mandibular hypoplasia. We have cited nine cases of bony syngnathia, including the present case. Cleft palate was present in four of these cases and mandibular hypoplasia in five cases. This combination of anomalies is thought to derive from maldevelopment of the first branchial arch. Embryologically, the maxilla, palatine bone, and zygoma develop from the maxillary process of the first branchial arch, while the mandible and anterior part of the auricle develop from the mandibular process of the same arch. The second arch provides the posterior part of the auricle, while the first branchial groove furnishes the external auditory meatus. Maldevelopment of the first and second branchial arches results in hypoplasia of the maxilla, mandible, and auricle.4 Hemifacial microsomia and bilateral microsomia occur because of maldevelopment of the first and second branchial arches. Munro6 classified hemifacial microsomia into several types and stated that the zygomatic arch and mandibular condyle were missing in severe types. Converse et al.” reported bilateral facial microsomia and involvement of macrostomia and mandibular condylar hypoplasia in severe forms. Congenital temporomandibular joint ankylosis due to zygomatico-mandibular fusion seen in our case is a new category of facial skeletal
Congenital zygomatico-mandibular fusion anomaly and should be differentiated from these previously reported varieties of congenital facial skeletal deformities. The pathogenesis remains unknown in these deformities, but some developmental defect has occurred in the first branchial arch region in which separation of the mandible, maxilla, and zygoma failed to occur. Although the exact mechanism is obscure, some environmental factor may be the cause of this rare skeletal deformation.
253
II. 12. 13. 14.
15.
References I. McCarthy JG, Grayson BH, Coccaro PJ, Wood-Smith D. Craniofacial microsomia. In: McCarthy JG, ed. Plastic Surgery. Philadelphia: WB Saunders, 1990: 30544100. 2. Cosman B, Crikelair GF. Mandibular hypoplasia and the late development of glossopharyngeal airway obstruction. Plast Reconstr Surg 1972; 50: 57339. 3. Quinn GW, Pickrell KL. Mandibular hypoplasia and airway interference. Endangerment of life and impairment of health. Laryngeal-pharyngeal obstruction. NC Dent J 1978; 61: 19~24. 4. McCarthy JG, Kawamoto HK, Grayson BH. Colen SR, Coccaro PJ, Wood-Smith D. Surgery of the jaws. In: McCarthy JG, ed. Plastic Surgery. Philadelphia: WB Saunders. 1990: 11888474. 5. Munro IR. One-stage reconstruction of the temporomandibular joint in hemifacial microsomia. Plast Reconstr Surg 1980: 66: 6999710. 6. Munro IR. Treatment of craniofacial microsomia. Clin Plast Surg 1987; 14: 177-86. 7. Pensler JM. Christopher RD, Bewyer DC. Correction of micrognathia with ankylosis of the temporomandibular joint in childhood. Plast Reconstr Surg 1993; 91: 7999805. 8. Sternberg N. Sagher U. Golan J, Eidelman AI, Ben-Hur N. Congenital fusion of the gums with bilateral fusion of the temporomandibular joints. Plast Reconstr Surg 1983: 72: 385 7. 9. Verdi GD. O’Neal B. Cleft palate and congenital alveolar synechia syndrome. Plast Reconstr Surg 1984; 74: 684-686. 10. Haramis HT. Apesos J. Cleft palate and congenital lateral
16. 17.
18. 19.
20.
alveolar synechia syndrome: case presentation and literature review. Ann Plast Surg 1995; 34: 424430. Burket LW. Congenital bony temporomandibular ankylosis and facial hemiatroohv. JAMA 1936: 106: 1719-22. Pettersson G. Aglossia’congenita with bony fusion of the jaws. Report of one case. Acta Chir Stand 1961; 122: 93-5. Hoggins GS. Aglossia congenita with bony fusion of the jaws. Br J Oral Surg 1969; 7: 63-5. Salleh NM. Congenital partial fusion of the mandible and maxilla. Report of the case. Oral Surg Oral Med Oral Pathol 1965: 20: 74-6. Miskinyar SA. Congenital mandibulo-maxillary fusion. Plast Reconstr Surg 1979; 63: 120~~1. Goodacre TE. Wallace AF. Congenital alveolar fusion. Br J Plast Surg 1990; 43: 20339. Sawhney CP. Bony ankylosis of the temporomandibular joint: follow-up of 70 patients treated with arthroplasty and acrylic spacer interposition. Plast Reconstr Surg 1986; 77: 29940. Simpson JR, Maves MD. Congenital syngnathia or fusion of the gums and jaws. Otol Head Neck Surg 1985; 93: 9669. Hochstetter F. tiber zwei Falle epithelialer syngnathie bei menschlichen Keimlingen. Dtsch Med Wochenschr 1948: 108812. Converse JM, Wood-Smith D, McCarthy JG, Coccaro PJ, Becker MH. Bilateral facial microsomia. Diagnosis, classification, treatment. Plast Reconstr Surg 1974; 54: 413-23.
The Authors Satoshi Kamata MD, Senior Resident Kaneshige Satoh MD, Assistant Professor Tetsuji Uemura MD, Assistant Lecturer Takuya Onizuka MD, Professor Department of Plastic and University School of Medicine, Tokyo. 142, Japan. Correspondence
to Dr Satoshi
Paper received 18 May Accepted 30 November
Reconstructive l-5-8, Hatanodai,
Kamata.
1995. 1995, after
revision.
Surgery, Showa Shinagawa-ku,