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Hemifacial hypertrophy
Hemifacial hypertrophy Report
of two
cases
h?~su~~~uIlayashi, M.D., D.M.Sc.“, Tokuya Tomioka, D.D.S., D.M.Sc., IIidt(c~u doki, D.D.S., D.M.Sc., Kazunori Nakakuki, D.D.S., and KiTyoshi Illekuru, D.D.S., Tokyo, Japan DEP~~R’I’IRIEXT F’ \(‘I~-LTV
OF ORAL
OF MEDICIXE,
SURGERY, THE
TOKYO
UNIVERSITY
UNIVERSI’TY
BRANCH
HOSPITAL,
OF TOKYO
Two cases of congenital hemifacial hypertrophy are reported-one associated with rlrvi systemici and the other with multiple neuromas on the enlarged side. Cephalogr:~ms indicated precisely the progressive overgrowth of the maxilla and mandible :~nd early development of the teeth on the affected side.
T
he human body generally shows morphologic asymmetry. These common features of asymmetry change with time and circumstance in a definite range. However,, l~cn~il~ypertrophic growth not due to local lesions steadily progresses wit,h regular asymmetrical development, having no relation to time and circumstance. Since the first case report of hemihypertrophy was published by Wagner1 in 1839, many cases have been reported .2 Classification has been proposed by various authors, including Ward and Lerner.3 A convenient practical classification of congenital hemihypertrophy by Rowe” includes complex, simple, and hemifacial hypertrophy. Friedrich reported the first case of hemifacial hypertrophy. We have found forty-six cases subsequently reported in the literature.G, 7 In Japan, only one case report? was found. A case described by Inaba9 as “congenital partial hypert,rophy“ appears to be one of hemifacial hypertrophy. *Associate
750
Professor.
Hemifacial
hypertrophy
751
Fig. 2. Case 1. Findings at first examination. 8, Hemihypertrophy of the left lower face and partial “shark’s mouth” appearance. B, Nevus systemicus on left side of neck. C, Relationship of midline between upper and lower jaws is practically normal. D, Enlargement of jaws and premature eruption of teeth on left side.
CASE REPORTS CASE
1
A boy aged 6 years 8 months was first examined on Aug. 23, 1967, because of congenital asymmetry of the face. No anatomic abnormalities were found in the family history. He was born following a normal pregnancy. His mother stated that the delivery probably required the use of forceps. The patient’s birth weight was 3,900 grams. At birth, facial enlargement and pigmentation of the skin of the face and neck on the left side were present. About a month after birth, the boy experienced frequent high fevers and convulsions. He was operated on for bilateral squint at the age of 4 years. The left facial enlargement increased progressively at the same rate as the boy’s physical growth. The facial asymmetry, according to his mother, had recently become more marked. Physical
examination
There were no abnormal findings in the general physical condition, mental health, and laboratory studies. Bilateral squint was present to a minimal degree. There was a prominent enlargement of the left side of the face, which extended from the margo infraorbitalis to the
752
Hayashi
Oral Surg.
et al.
June, 1973
5.2Y oto)
, ,-- ------I. ,,/ ,i
__ -A i\.,
Fig. 8. Case 1. Cephalograms at first examination L. A, Lateral view. B, Roentgen cephalometric analysis: Profilogram (male). Note downwal .d development of jawbones. C and D, Frontal views showing enlargement of mandibula r ramus, premature development, and eruption of teeth on left side.
Hewifacial
Volume 35 Number 6
basis mandibulae, creating a partial “shark’s mouth” appearance (Fig. were found extending over the cheek and neck on the left side (Fig. Oral
hypertrophy 1, a). Nevi 1, B).
753 systemici
examination
Oral examination revealed enlargement of the maxilla and mandible on the left side without any occlusal displacement of the midline between the anterior incisors of both jaws (Fig. 1, C). No anomaly of the tongue was noted. Dental
flndings
It
was found
that
6 / 456 and 821112457
had already
erupted,
and
112 and
p
had
begun to erupt; d and4 were still present (Fig. 1, D) . Because of dental caries, the lower left first molar had already been extracted. A malocclusion was present. In reference to crown size, no differences were found between the upper right and left first molars and between the lower right and left central and lateral incisors. As for L45 and 1457, it was impossible to compare them with their opposite members because they were as yet unerupted, but their measurement figures seemed to be larger than normal.10 Roentgenographic
examination
Cephalograms revealed that the development of the cranial base was within normal limits, and the downward development of the mandible corresponded to the standard value for an ll- to 12-year-old Japanese boy. The mandible’s forward development was within normal limits (Fig. 2, B and B). A posteroanterior roentgenogram demonstrated enlargement of the left face, particularly in the mandibular ramus and condyloid process. The dentition on the left side was equal to that of a lo- to 13-year-old boy (Fig. 2, C and D). The left maxillary canine was impacted. The crown was facing the buccal side. SECOND
EXAMINATION
Physical
Andlngs
Enlargement Oral
IONE
of the left
YEAR
LATERI
side of the face was more obvious.
flndings
Enlargement of the tongue on the left side was minimal when palpated. Overgrowth of the maxilla and mandible on the left side had progressed slightly (Fig. 3, B), and the midline of the mandible was about 4 mm. to the right of the maxillary midline (Fig. 3, a). A gnathostatic model showed that the occlusal plane was more inferior on the left, and, consequently demonstrated malocclusion. Dental
flndings
All four permanent upper incisors and the lower left canine had erupted since the first examination. The upper left canine had been extracted because of malposition, and the lower left second premolar had been extracted because of dental caries. The upper right lateral incisor was in linguoversion, the upper central incisors were edge to edge, the upper left premolars were elongated, the upper left lateral incisor was distal to the lower left canine, and the upper left first molar occluded with the lower left second molar, which showed a mesial inclination (Fig. 3, a). Roentgenographic
findings
Compared with the findings of the first examination, cephalograms indicated a slight downward and forward development of the mandible. A posteroanterior roentgenogram revealed more progressive enlargement of the left side of the face, particularly in the mandibular ramus and condyloid process. There was a prominent lateral projection of the mandibular condyle. The changing dentition on the left side was equal to that of an ll- to 14-year-old boy, while on the right side it was equal to that of a boy of the patient’s age (Fig. 3, C and D).
754
Hayashi
et al.
Oral Surg. June, 1973
Fig. J. Case 1. Findings at second examination (one year later). a, Gnathostatio model B, Overgrowth of jaws on left side. C, showing displacement of midline and malocclusion. Oblique cephalogramlr (left) showing premature development and eruption of posterior teeth. D, Oblique eephalogram (right) showing normal development and eruption of posterior teeth. CASE 2
A girl aged 5 years 2 months was seen on April 5, 1967, complaining of nontender tumors on the surface of t,hc tongue and the buccal mucosa on the left side. She had masticatory disturbances with malocclusion. No anatomic abnormalities were found in the family history. The patient was the product of a normal pregnancy and delivery. Her birth weight was 2,400 grams. She had suffered frequently from high fevers and convulsions between the ages of 6 months and l’/r, years. The two masses on the tongue and buccal mucosa were noticed when she was 3 years of age, and they had gradually increa.sed in size. Some permanent teeth had begun to erupt in the left mandible at the age of 4 years. Physical
examination
The patient was a poorly nourished and poorly developed child. There were no abnormal findings in the general condition and mental health, and laboratory studies yielded normal results.
Hemifacial
hypertrophy
755
Fig. 4. Case 2. Findings at first examination. A, Displacement of midline. B, Overgrowth of mandible and premature eruption of lower teeth on left side. C, Tumors on surface of tongue and buccal mucosa of left side. D, Section of tumors (silver stain). Note proliferation of curving nerve fascicles.
Oral
examination
When the patient protruded her tongue, it curved slightly to the right. Enlargement was slight in the maxilla and obvious in the mandible on the left side, especially in the alveolar bone area (Fig. 4, B). The midline of the mandible was about 5 mm. to the right of the maxillary midline (Fig. 4, A). Two tumors, both elastic, soft, and polypoid, were present on the tongue and buccal mucosa. The former (about 5 by 5 mm.) touched the lower left deciduous second molar, and the latter (about 5 by 7 mm.) touched the lower left permanent canine (Fig. 4, C). There was also a pea-shaped swelling of the lower lip on the left side.
756
Huyashi
ct nl.
Oral Surg. June, 1973
Wandaal : by Sakamoto)
0 6
Fig. 5. Case 2. Cephalograms at first examination. A, Lateral view. R, Roentgen cephalometric analysis : Profilogram (female) showing downward development of jawbones. C and I), Frontal views showing enlargement, of mandilmlnr ramus and premature development and eruption of lower teeth on left side.
Hentifacial
Volume 35 Number 6 Dental
hypertrophy
757
findings
~
had already erupted, and v were about to erupt (Fig. 4, B). No abnormal tooth eruptions were seen in the maxilla. A malocclusion was present. As to crown size, no difference was found in the deciduous teeth of the two sides of the maxilla. The permanent teeth of both sides of the mandible could not be compared because of incomplete eruption. Roentgenographic
examination
Cephalograms revealed a downward development of the mandible corresponding to the standard value for a 7-year-old Japanese girl. The cranial base was within normal limits (Fig. 5, A and B). In a posteroanterior roentgenogram, the mandible of the left side, (specially in the mandibular ramus, was seen to be enlarged (Fig. 5, C and D). The changing dentition was equal to that of a 9- to lo-year-old girl in the left mandible and to that of a girl of the patient’s age on the right side (Fig. 5, C and D). The roots of the left mandibular first molar proved to be abnormal in shape. Histopathologic
examination
The two masses on the cheek and tongue were excised. Histopathologie examination showed proliferation of the curving nerve fascicles surrounded by thick connective tissue under the mucous membrane (Fig. 4, D). The diagnosis was neuroma. Second Physical
examination tlndings
Enlargement Oral
(one
year
of the lower
later)
half
of the left
face was more obvious
(Fig.
6, a).
findings
The mandible and the fungiform papillae of the tongue showed a continuous tendency for further enlargement on the left side. Curving of the tongue to the right at the time of protrusion was more prominent (Fig. 6, B) . The midline of the mandible was about 7 mm. to the right of the maxillary midline (Fig. 7, a). A gnathostatic model revealed that the occlusnl plane had a tendency to move inferiorly on the left side. A new small mass (about 3 by S mm.) was found on the buccal mucosa touching the lower left first permanent molar (Fig. 6, D). The swelling of the lower lip on the left side had slightly increased in size (about 10 by 10 mm.), causing asymmetry of the lower lip (Fig. 6, C). Dental
Rndings
62 (Fig.
had erupted 7, 23).
Roentgenographic
since the first
examination,
and 1123456 were regular
in arrangement
findings
Compared with the first examination, cephalograms showed a slight downward and forward development of the maxilla and mandible. Further enlargement of the mandible of the left side was found on the posteroanterior roentgenogram. The changing dentition was equal to that of an ll- to 13.year-old girl in the left mandible and of a 7-year-old on the right side (Fig. 7, C and D) .
DISCUSSION
There are many diseases which may cause hemifacial swelling. Hemifacial hypertrophy, however, implies congenital disease with hypertrophy of the soft and hard tissues, so that most cases are discovered soon after birth. In mild cases, it is not uncommon for the symptoms to be overlooked or neglected during childhood. Symptoms of hemifacial hypertrophy have been described by many authors.
758
IIlayashi
et al.
Fig. 6. Case 2. barely demonstrated. ment with prominent Swelling on left side molar.
Oral Burg. June, 1973
Findings at second examination (one year later). A, Hypertrophy is B, Deformity of tongue. Dorsum of tongue on left shows enlargefungiform papillae. When protruded, tongue curved slightly to right. C, of lower lip. D, Small mass on buccal mucosa touched lower left first
‘, 0, ‘. I2 Overgrowth generally includes half of the maxilla and mandible and the surrounding tissues, such as the tongue. There are some incomplete cases in which only the upper half of the faceI or half of the tongue” shows an overgrowth. In roetgenograms, the teeth are seen to have developed earlier or erupted prematurely. A bony overgrowth and an enlarged mandibular canal4 were present on the affected side. These reports, however, may be inaccurate because they are based on ordinary roentgenograms rather than on special-view cephalograms. It is of interest to note that in our first patient the enlargement was prominent in the maxilla and mandible but minimal in the tongue and that the left maxillary canine was impacted. In spite of the enlargement of the maxilla and mandible, an increasing width between the midline of the maxilla and the
Hemifacial
hypertrophy
759
Fig. 7. Case 2. Findings at second examination (one year later). A, Gnathostatic model showing displacement of midline. B, Overgrowth of mandible on left side. C, Oblique cephalogram (left) showing premature development and eruption of lower posterior teeth. D, Oblique cephalogram (right) showing normal development and eruption of posterior teeth. mandible was noted with time. This is caused not by a difference in the developmental rate between maxilla and mandible but by an anatomic difference of bone in form and relationship. In the second case, enlargement of the face was noticed at the age of 4 years with early eruption of the permanent teeth. Enlargement was obvious in the mandible and tongue. The dental arch of the maxilla was slightly enlarged on the left side, with no abnormalities in growth and eruption of teeth. It is not clear whether this change occurred because of hemifacial hypertrophy itself or was due to a compensative state corresponding to an enlarged dental arch of the mandible. In the complications of hemihypertrophy, skin abnormalities are found in a large number of reports (65.8 per cent in Japanese reports of hemihypertrophy) .I4 Anomalies of the fingers and toes, mental retardation, and convulsions
760
Ilcrytrshi
tf t/l.
Oral Burg. June, 1973
\vcrt’ rcl~ortetl. Scvi systemici were found on the cheek and neck of the en13rgctl sic10in c)nr first case. In the second case, there were neuromas on the surface of the toilg’llc~ ilIlt t Ilc I~uc*wl nlucosil 0T tlw enlargrd side. We could not find any reports of mnltil)lc 11cnromas which dcvclopcd on the affected side in hemifacial liypc~rtrophy. In this case, multiple neuromas could he caused by the same factors that cansed the hemifacial hypcrtrophy. They might be nontraumatic ~ic~~ro~ll:~s,‘~~ lti such as “Karlkenrlcurorlla.“‘~ We believe, however, that these are trauiilntic 11~~~711’0111as,~~~ I9 because they are located in areas that are very casil>- t ra umatizcd as a result of malocclusion. It is interesting that in these two cases the patients suffered frequent attacks of fclcr and convulsion in infancy. Treatment of the malocclusion is indicatccl in childhood. The problem of appcarancc sho~~ltl be considered when the patient grows up. SUMMARY
Two cases of congenital hemifacial hypertrophy are reported-one associated with nevi systemici and the other with multiple neuromas on the enlarged side. Cephalograms indicated precisely the progressive overgrowth of the maxilla and mandible and early development of the teeth on the affected side.
REFERENCES
H. : Hypertrophie der rechten Brust und der rechten oberen Extremitlt, 1. Wagner, besonders der Hand der Finger. Med. Jahrb. K. K. Gsterreichschen Staates 19: 378, 1839. (Cited by Ringrose ct al.: i965.) Pediatrics 36: 434, 2. Ringrose, R. E., Jabbour, J. T., and Keele, D. K.: Hemihypertrophy, 1965. 3. Ward, J., and Lerner, H. H.: A Review of the Subject of Congenital Hemihypertrophy and a Complete Case Report, J. Pediatr. 31: 403, 1947. 4. Hypertrophy, ORAI, SURG. 15: 572, 1962 - Rowe, N. H.: Hemifacial 0. Friedrich, N. : Uber congenitalc hxlbsertige Kopfhypertrophie, Virchows Arch. [Pathol. -4nat.l 28: 471, 1863. 6. Hanley, F. .J., Floyd, E., and Parker, D.: Congenital Partial Hemihypertrophy of the Face: Report of Three Cases, J. ORAI, SURG. 26: 136, 1968 7. Deadr, M. J., Silagi, J. L., and Hutton, C. E.: Hemihypertrophies of the Face and Mandil,lc, OKAL SURG. 27: 577, 1969. cs Kondo Susumu, and Kiuchi, Masanno: Congenital Hemifacial hypertrophy; Report of a Case. J~D. J. Oral Sure. 5: 142. 1959. 9. Inada, Minor”: Part&l Gigantism With Extensive Congenital “Naevi vasuIasi”Roentgenographical Study of Bone and Teeth, Monatsschr. Klin. Kinderheilkd. 14: 389. ---7
lQ4c)
-l-“.
10. Kamijo, Yasuhiko: Anatomy of Japanese Permanent Teeth, Tokyo, 1962, Seikoshx. R.: An Evaluation of the Oblique 11. Barber, T. K., Pruzansky, S., and Kindelsperger, Cephalometric Film, J. Dent. Child 28: 94, 1961. 12. Stafne, E. C., and Lovestedt, S. A.: Congenital Hemihypertrophy of the Face (Facial Giantism), ORAL SURG. 15: 184, 1962. 13. Romer, K. H.: Hemihypertrophia faeiei, Dtsch. Zahn-Mund-Kieferhlkd. 13: 245, 1950. 14. Miki, Shigeru, and Fujino, Shigeru: Five Cases of Hemihypertrophy, Ann. Paediatr. Jap. 3: 756, 1957. 15. Michalowski, R.: Multiple fibrillare Neurome der Augenlider, Lippen und Zunge mit Genitalhypoplasie und Gelenkanomalien, Arch. Klin. Exp. Dermatol. 231: 20, 1967. 16. Williams, E. D., and Pollock, D. J.: Multiple Mucosal Neuromata With Endocrine Tumors: A Syndrome Allied to von Recklinghausen’s Disease, J. Pathol. Bacterial. 91: 71, 1966. 17. Thies! W.: Multiple echte tibrillare Neurome (Rankenneurome) der Haut und Schlelmhaut, Arch. Klin. Exp. Dermatol. 218: 561, 1964.
Volume 35 Number 6 18. Swanson, H. H.: Traumatic 19. Robinson, M., and Slavkin, 70: 662, 1965. Reprint requests to : Dr. Susumu Hayashi Department of Oral Surgery Tokyo University Branch Hospital Faculty of Medicine University of Tokyo Tokyo, Japan
Wemifacial
hypertrophy
Neuromas, ORAL SURQ. 14: 317, 1961. H. C.: Dental Amputation Neuromas, J. Am. Dent.
761
ASSOC.
of two
cases
h?~su~~~uIlayashi, M.D., D.M.Sc.“, Tokuya Tomioka, D.D.S., D.M.Sc., IIidt(c~u doki, D.D.S., D.M.Sc., Kazunori Nakakuki, D.D.S., and KiTyoshi Illekuru, D.D.S., Tokyo, Japan DEP~~R’I’IRIEXT F’ \(‘I~-LTV
OF ORAL
OF MEDICIXE,
SURGERY, THE
TOKYO
UNIVERSITY
UNIVERSI’TY
BRANCH
HOSPITAL,
OF TOKYO
Two cases of congenital hemifacial hypertrophy are reported-one associated with rlrvi systemici and the other with multiple neuromas on the enlarged side. Cephalogr:~ms indicated precisely the progressive overgrowth of the maxilla and mandible :~nd early development of the teeth on the affected side.
T
he human body generally shows morphologic asymmetry. These common features of asymmetry change with time and circumstance in a definite range. However,, l~cn~il~ypertrophic growth not due to local lesions steadily progresses wit,h regular asymmetrical development, having no relation to time and circumstance. Since the first case report of hemihypertrophy was published by Wagner1 in 1839, many cases have been reported .2 Classification has been proposed by various authors, including Ward and Lerner.3 A convenient practical classification of congenital hemihypertrophy by Rowe” includes complex, simple, and hemifacial hypertrophy. Friedrich reported the first case of hemifacial hypertrophy. We have found forty-six cases subsequently reported in the literature.G, 7 In Japan, only one case report? was found. A case described by Inaba9 as “congenital partial hypert,rophy“ appears to be one of hemifacial hypertrophy. *Associate
750
Professor.
Hemifacial
hypertrophy
751
Fig. 2. Case 1. Findings at first examination. 8, Hemihypertrophy of the left lower face and partial “shark’s mouth” appearance. B, Nevus systemicus on left side of neck. C, Relationship of midline between upper and lower jaws is practically normal. D, Enlargement of jaws and premature eruption of teeth on left side.
CASE REPORTS CASE
1
A boy aged 6 years 8 months was first examined on Aug. 23, 1967, because of congenital asymmetry of the face. No anatomic abnormalities were found in the family history. He was born following a normal pregnancy. His mother stated that the delivery probably required the use of forceps. The patient’s birth weight was 3,900 grams. At birth, facial enlargement and pigmentation of the skin of the face and neck on the left side were present. About a month after birth, the boy experienced frequent high fevers and convulsions. He was operated on for bilateral squint at the age of 4 years. The left facial enlargement increased progressively at the same rate as the boy’s physical growth. The facial asymmetry, according to his mother, had recently become more marked. Physical
examination
There were no abnormal findings in the general physical condition, mental health, and laboratory studies. Bilateral squint was present to a minimal degree. There was a prominent enlargement of the left side of the face, which extended from the margo infraorbitalis to the
752
Hayashi
Oral Surg.
et al.
June, 1973
5.2Y oto)
, ,-- ------I. ,,/ ,i
__ -A i\.,
Fig. 8. Case 1. Cephalograms at first examination L. A, Lateral view. B, Roentgen cephalometric analysis: Profilogram (male). Note downwal .d development of jawbones. C and D, Frontal views showing enlargement of mandibula r ramus, premature development, and eruption of teeth on left side.
Hewifacial
Volume 35 Number 6
basis mandibulae, creating a partial “shark’s mouth” appearance (Fig. were found extending over the cheek and neck on the left side (Fig. Oral
hypertrophy 1, a). Nevi 1, B).
753 systemici
examination
Oral examination revealed enlargement of the maxilla and mandible on the left side without any occlusal displacement of the midline between the anterior incisors of both jaws (Fig. 1, C). No anomaly of the tongue was noted. Dental
flndings
It
was found
that
6 / 456 and 821112457
had already
erupted,
and
112 and
p
had
begun to erupt; d and4 were still present (Fig. 1, D) . Because of dental caries, the lower left first molar had already been extracted. A malocclusion was present. In reference to crown size, no differences were found between the upper right and left first molars and between the lower right and left central and lateral incisors. As for L45 and 1457, it was impossible to compare them with their opposite members because they were as yet unerupted, but their measurement figures seemed to be larger than normal.10 Roentgenographic
examination
Cephalograms revealed that the development of the cranial base was within normal limits, and the downward development of the mandible corresponded to the standard value for an ll- to 12-year-old Japanese boy. The mandible’s forward development was within normal limits (Fig. 2, B and B). A posteroanterior roentgenogram demonstrated enlargement of the left face, particularly in the mandibular ramus and condyloid process. The dentition on the left side was equal to that of a lo- to 13-year-old boy (Fig. 2, C and D). The left maxillary canine was impacted. The crown was facing the buccal side. SECOND
EXAMINATION
Physical
Andlngs
Enlargement Oral
IONE
of the left
YEAR
LATERI
side of the face was more obvious.
flndings
Enlargement of the tongue on the left side was minimal when palpated. Overgrowth of the maxilla and mandible on the left side had progressed slightly (Fig. 3, B), and the midline of the mandible was about 4 mm. to the right of the maxillary midline (Fig. 3, a). A gnathostatic model showed that the occlusal plane was more inferior on the left, and, consequently demonstrated malocclusion. Dental
flndings
All four permanent upper incisors and the lower left canine had erupted since the first examination. The upper left canine had been extracted because of malposition, and the lower left second premolar had been extracted because of dental caries. The upper right lateral incisor was in linguoversion, the upper central incisors were edge to edge, the upper left premolars were elongated, the upper left lateral incisor was distal to the lower left canine, and the upper left first molar occluded with the lower left second molar, which showed a mesial inclination (Fig. 3, a). Roentgenographic
findings
Compared with the findings of the first examination, cephalograms indicated a slight downward and forward development of the mandible. A posteroanterior roentgenogram revealed more progressive enlargement of the left side of the face, particularly in the mandibular ramus and condyloid process. There was a prominent lateral projection of the mandibular condyle. The changing dentition on the left side was equal to that of an ll- to 14-year-old boy, while on the right side it was equal to that of a boy of the patient’s age (Fig. 3, C and D).
754
Hayashi
et al.
Oral Surg. June, 1973
Fig. J. Case 1. Findings at second examination (one year later). a, Gnathostatio model B, Overgrowth of jaws on left side. C, showing displacement of midline and malocclusion. Oblique cephalogramlr (left) showing premature development and eruption of posterior teeth. D, Oblique eephalogram (right) showing normal development and eruption of posterior teeth. CASE 2
A girl aged 5 years 2 months was seen on April 5, 1967, complaining of nontender tumors on the surface of t,hc tongue and the buccal mucosa on the left side. She had masticatory disturbances with malocclusion. No anatomic abnormalities were found in the family history. The patient was the product of a normal pregnancy and delivery. Her birth weight was 2,400 grams. She had suffered frequently from high fevers and convulsions between the ages of 6 months and l’/r, years. The two masses on the tongue and buccal mucosa were noticed when she was 3 years of age, and they had gradually increa.sed in size. Some permanent teeth had begun to erupt in the left mandible at the age of 4 years. Physical
examination
The patient was a poorly nourished and poorly developed child. There were no abnormal findings in the general condition and mental health, and laboratory studies yielded normal results.
Hemifacial
hypertrophy
755
Fig. 4. Case 2. Findings at first examination. A, Displacement of midline. B, Overgrowth of mandible and premature eruption of lower teeth on left side. C, Tumors on surface of tongue and buccal mucosa of left side. D, Section of tumors (silver stain). Note proliferation of curving nerve fascicles.
Oral
examination
When the patient protruded her tongue, it curved slightly to the right. Enlargement was slight in the maxilla and obvious in the mandible on the left side, especially in the alveolar bone area (Fig. 4, B). The midline of the mandible was about 5 mm. to the right of the maxillary midline (Fig. 4, A). Two tumors, both elastic, soft, and polypoid, were present on the tongue and buccal mucosa. The former (about 5 by 5 mm.) touched the lower left deciduous second molar, and the latter (about 5 by 7 mm.) touched the lower left permanent canine (Fig. 4, C). There was also a pea-shaped swelling of the lower lip on the left side.
756
Huyashi
ct nl.
Oral Surg. June, 1973
Wandaal : by Sakamoto)
0 6
Fig. 5. Case 2. Cephalograms at first examination. A, Lateral view. R, Roentgen cephalometric analysis : Profilogram (female) showing downward development of jawbones. C and I), Frontal views showing enlargement, of mandilmlnr ramus and premature development and eruption of lower teeth on left side.
Hentifacial
Volume 35 Number 6 Dental
hypertrophy
757
findings
~
had already erupted, and v were about to erupt (Fig. 4, B). No abnormal tooth eruptions were seen in the maxilla. A malocclusion was present. As to crown size, no difference was found in the deciduous teeth of the two sides of the maxilla. The permanent teeth of both sides of the mandible could not be compared because of incomplete eruption. Roentgenographic
examination
Cephalograms revealed a downward development of the mandible corresponding to the standard value for a 7-year-old Japanese girl. The cranial base was within normal limits (Fig. 5, A and B). In a posteroanterior roentgenogram, the mandible of the left side, (specially in the mandibular ramus, was seen to be enlarged (Fig. 5, C and D). The changing dentition was equal to that of a 9- to lo-year-old girl in the left mandible and to that of a girl of the patient’s age on the right side (Fig. 5, C and D). The roots of the left mandibular first molar proved to be abnormal in shape. Histopathologic
examination
The two masses on the cheek and tongue were excised. Histopathologie examination showed proliferation of the curving nerve fascicles surrounded by thick connective tissue under the mucous membrane (Fig. 4, D). The diagnosis was neuroma. Second Physical
examination tlndings
Enlargement Oral
(one
year
of the lower
later)
half
of the left
face was more obvious
(Fig.
6, a).
findings
The mandible and the fungiform papillae of the tongue showed a continuous tendency for further enlargement on the left side. Curving of the tongue to the right at the time of protrusion was more prominent (Fig. 6, B) . The midline of the mandible was about 7 mm. to the right of the maxillary midline (Fig. 7, a). A gnathostatic model revealed that the occlusnl plane had a tendency to move inferiorly on the left side. A new small mass (about 3 by S mm.) was found on the buccal mucosa touching the lower left first permanent molar (Fig. 6, D). The swelling of the lower lip on the left side had slightly increased in size (about 10 by 10 mm.), causing asymmetry of the lower lip (Fig. 6, C). Dental
Rndings
62 (Fig.
had erupted 7, 23).
Roentgenographic
since the first
examination,
and 1123456 were regular
in arrangement
findings
Compared with the first examination, cephalograms showed a slight downward and forward development of the maxilla and mandible. Further enlargement of the mandible of the left side was found on the posteroanterior roentgenogram. The changing dentition was equal to that of an ll- to 13.year-old girl in the left mandible and of a 7-year-old on the right side (Fig. 7, C and D) .
DISCUSSION
There are many diseases which may cause hemifacial swelling. Hemifacial hypertrophy, however, implies congenital disease with hypertrophy of the soft and hard tissues, so that most cases are discovered soon after birth. In mild cases, it is not uncommon for the symptoms to be overlooked or neglected during childhood. Symptoms of hemifacial hypertrophy have been described by many authors.
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Fig. 6. Case 2. barely demonstrated. ment with prominent Swelling on left side molar.
Oral Burg. June, 1973
Findings at second examination (one year later). A, Hypertrophy is B, Deformity of tongue. Dorsum of tongue on left shows enlargefungiform papillae. When protruded, tongue curved slightly to right. C, of lower lip. D, Small mass on buccal mucosa touched lower left first
‘, 0, ‘. I2 Overgrowth generally includes half of the maxilla and mandible and the surrounding tissues, such as the tongue. There are some incomplete cases in which only the upper half of the faceI or half of the tongue” shows an overgrowth. In roetgenograms, the teeth are seen to have developed earlier or erupted prematurely. A bony overgrowth and an enlarged mandibular canal4 were present on the affected side. These reports, however, may be inaccurate because they are based on ordinary roentgenograms rather than on special-view cephalograms. It is of interest to note that in our first patient the enlargement was prominent in the maxilla and mandible but minimal in the tongue and that the left maxillary canine was impacted. In spite of the enlargement of the maxilla and mandible, an increasing width between the midline of the maxilla and the
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Fig. 7. Case 2. Findings at second examination (one year later). A, Gnathostatic model showing displacement of midline. B, Overgrowth of mandible on left side. C, Oblique cephalogram (left) showing premature development and eruption of lower posterior teeth. D, Oblique cephalogram (right) showing normal development and eruption of posterior teeth. mandible was noted with time. This is caused not by a difference in the developmental rate between maxilla and mandible but by an anatomic difference of bone in form and relationship. In the second case, enlargement of the face was noticed at the age of 4 years with early eruption of the permanent teeth. Enlargement was obvious in the mandible and tongue. The dental arch of the maxilla was slightly enlarged on the left side, with no abnormalities in growth and eruption of teeth. It is not clear whether this change occurred because of hemifacial hypertrophy itself or was due to a compensative state corresponding to an enlarged dental arch of the mandible. In the complications of hemihypertrophy, skin abnormalities are found in a large number of reports (65.8 per cent in Japanese reports of hemihypertrophy) .I4 Anomalies of the fingers and toes, mental retardation, and convulsions
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\vcrt’ rcl~ortetl. Scvi systemici were found on the cheek and neck of the en13rgctl sic10in c)nr first case. In the second case, there were neuromas on the surface of the toilg’llc~ ilIlt t Ilc I~uc*wl nlucosil 0T tlw enlargrd side. We could not find any reports of mnltil)lc 11cnromas which dcvclopcd on the affected side in hemifacial liypc~rtrophy. In this case, multiple neuromas could he caused by the same factors that cansed the hemifacial hypcrtrophy. They might be nontraumatic ~ic~~ro~ll:~s,‘~~ lti such as “Karlkenrlcurorlla.“‘~ We believe, however, that these are trauiilntic 11~~~711’0111as,~~~ I9 because they are located in areas that are very casil>- t ra umatizcd as a result of malocclusion. It is interesting that in these two cases the patients suffered frequent attacks of fclcr and convulsion in infancy. Treatment of the malocclusion is indicatccl in childhood. The problem of appcarancc sho~~ltl be considered when the patient grows up. SUMMARY
Two cases of congenital hemifacial hypertrophy are reported-one associated with nevi systemici and the other with multiple neuromas on the enlarged side. Cephalograms indicated precisely the progressive overgrowth of the maxilla and mandible and early development of the teeth on the affected side.
REFERENCES
H. : Hypertrophie der rechten Brust und der rechten oberen Extremitlt, 1. Wagner, besonders der Hand der Finger. Med. Jahrb. K. K. Gsterreichschen Staates 19: 378, 1839. (Cited by Ringrose ct al.: i965.) Pediatrics 36: 434, 2. Ringrose, R. E., Jabbour, J. T., and Keele, D. K.: Hemihypertrophy, 1965. 3. Ward, J., and Lerner, H. H.: A Review of the Subject of Congenital Hemihypertrophy and a Complete Case Report, J. Pediatr. 31: 403, 1947. 4. Hypertrophy, ORAI, SURG. 15: 572, 1962 - Rowe, N. H.: Hemifacial 0. Friedrich, N. : Uber congenitalc hxlbsertige Kopfhypertrophie, Virchows Arch. [Pathol. -4nat.l 28: 471, 1863. 6. Hanley, F. .J., Floyd, E., and Parker, D.: Congenital Partial Hemihypertrophy of the Face: Report of Three Cases, J. ORAI, SURG. 26: 136, 1968 7. Deadr, M. J., Silagi, J. L., and Hutton, C. E.: Hemihypertrophies of the Face and Mandil,lc, OKAL SURG. 27: 577, 1969. cs Kondo Susumu, and Kiuchi, Masanno: Congenital Hemifacial hypertrophy; Report of a Case. J~D. J. Oral Sure. 5: 142. 1959. 9. Inada, Minor”: Part&l Gigantism With Extensive Congenital “Naevi vasuIasi”Roentgenographical Study of Bone and Teeth, Monatsschr. Klin. Kinderheilkd. 14: 389. ---7
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10. Kamijo, Yasuhiko: Anatomy of Japanese Permanent Teeth, Tokyo, 1962, Seikoshx. R.: An Evaluation of the Oblique 11. Barber, T. K., Pruzansky, S., and Kindelsperger, Cephalometric Film, J. Dent. Child 28: 94, 1961. 12. Stafne, E. C., and Lovestedt, S. A.: Congenital Hemihypertrophy of the Face (Facial Giantism), ORAL SURG. 15: 184, 1962. 13. Romer, K. H.: Hemihypertrophia faeiei, Dtsch. Zahn-Mund-Kieferhlkd. 13: 245, 1950. 14. Miki, Shigeru, and Fujino, Shigeru: Five Cases of Hemihypertrophy, Ann. Paediatr. Jap. 3: 756, 1957. 15. Michalowski, R.: Multiple fibrillare Neurome der Augenlider, Lippen und Zunge mit Genitalhypoplasie und Gelenkanomalien, Arch. Klin. Exp. Dermatol. 231: 20, 1967. 16. Williams, E. D., and Pollock, D. J.: Multiple Mucosal Neuromata With Endocrine Tumors: A Syndrome Allied to von Recklinghausen’s Disease, J. Pathol. Bacterial. 91: 71, 1966. 17. Thies! W.: Multiple echte tibrillare Neurome (Rankenneurome) der Haut und Schlelmhaut, Arch. Klin. Exp. Dermatol. 218: 561, 1964.
Volume 35 Number 6 18. Swanson, H. H.: Traumatic 19. Robinson, M., and Slavkin, 70: 662, 1965. Reprint requests to : Dr. Susumu Hayashi Department of Oral Surgery Tokyo University Branch Hospital Faculty of Medicine University of Tokyo Tokyo, Japan
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Neuromas, ORAL SURQ. 14: 317, 1961. H. C.: Dental Amputation Neuromas, J. Am. Dent.
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