Progressive Hemifacial Atrophy Treated by Orthodontic Surgery

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1 Oral Science International, November 2005, p Copyright 2005, Japanese Stomatology Society. All Rights Reserved. Progressive Hemifacial Atrophy Treated by Orthodontic Surgery Yasumitsu Kodama, Marta Miyazawa, Jun-ichi Fukuda, Akihiko Iida, Kazuhiro Ono and Ritsuo Takagi Division of Oral and Maxillofacial Surgery, Niigata Graduate School of Medical and Dental Sciences Chief: Professor Ritsuo Takagi Abstract: Progressive hemifacial atrophy PHA is a self-limited atrophy of subcutaneous tissues and less frequently of hard tissues on one side of the face. It is a sporadic, slowly progressing disease whose pathogenesis is still unknown. As a rule the asymmetry caused by PHA usually of soft tissue is treated by volume augmentation that involves free tissue grafting or a pedicled flap transfer. We describe a rare case of hard tissue PHA. The atrophic changes occurred in the left molar tooth, maxilla and mandible and were associated with moderate soft tissue atrophy. The left side of the patient s mouth was higher on the right side, and the occlusal plane was severely inclined in spite of normal occlusion. After no alteration and no progression of the atrophy were established, the patient was treated with orthodontic surgery. To correct the inclined occlusal plane and asymmetry profile, we performed a Le Fort I and intraoral vertical ramus osteotomy. In the 3-year follow-up, there were good occlusal balance and improved symmetrical profile without relapse or recurrence of the atrophy. Thus, orthodontic surgery was effective as a first procedure to treat hard tissue atrophy that appeared with moderate soft tissue atrophy. Key words: Progressive Hemifacial Atrophy PHA, Orthodontic Surgery, Facial Asymmetry, Inclined Occlusal Plane Introduction Progressive hemifacial atrophy PHA was described by Parry in 1825 and Romberg in This disease usually occurs in the skin, fat, muscle, and less frequently in bone 1. Symptoms usually appear within the first two decades with an active progressive phase lasting 2 10 years, and mostly in females 2. When the atrophy occurs in soft tissue, the main treatment is volume augmentation by free tissue grafting or pedicled flap transfer 3. Here we describe a rare case of PHA in hard tissues bone and tooth, which resulted in a severely inclined Received 7/15/05; revised 8/25/05; accepted 8/29/05. Requests for reprints : Yasumitsu Kodama, DDS, PhD, Division of Oral and Maxillofacial Surgery, Niigata Graduate School of Medical and Dental Sciences, Gakko cho-dori, Niigata City, , Japan, Tel: , Fax: , damadama@dent.niigata-u.ac.jp occlusal plane. Therefore the patient was treated surgically by Le Fort I and intraoral vertical ramus osteotomy. Report of a case A 15-year-old boy visited our department with a complaint of chin asymmetry in 1998, which was first noticed when he was 8 years old. Routine biochemical and hematological test results were within normal limits. His face showed no alteration of pigmentation and no stigmata of the scleroderma. His pupil reactions were normal, and he had no evidence of parasympathetic or sympathetic denervation. His chin was shifted to the left side and the left angle of his mouth was higher compared to the right angle. Though he had normal occlusion, his occlusal plane was severely inclined on the left side Fig. 1-A. A panoramic diagraph showed asym-

2 132 Oral Science International Vol. 2, No. 2 Fig. 1 Facial appearance of the 15-year-old boy at first consultation. A Hemifacial asymmetry extended across the left side of the face, which had an inclined occlusal plane and aperture of mouth. B Orthopantomograms. These showed microdontia on the left side, and underdevelopment of maxillary sinus and mandible whose condyle, subcondyle and ramus were small. metry with short root suspected microdontia on the left side, underdevelopment of the maxillary sinus on the left side, and a mandible with smaller condyle, subcondyle and ramus than on the opposite side, which had a normal profile Fig. 1-B. In dentition model assay, there was a slight difference of total crown width upper jaw: rt-29.0 mm, lt-27.5 mm, lower jaw: rt-30.5 mm, lt-29.5 mm between the right and the left in the molar region. Since there was uncertainty regarding whether the disease was congenital or acquired, hemifacial microsomia was suspected initially. After pre-surgical orthodontic treatment, it was observed that the amount of facial hair on the left side of his face was reduced significantly Fig. 2-A, his tongue and upper lip were atrophied on the left side, and the median sulcus of his tongue and philtrum were shifted to the left side Fig. 2-B. Therefore image evaluation using computed tomography was performed. It was observed that soft tissue, where the masseter muscle, parotid gland, and subcutaneous fat are located in the left cheek, was smaller Fig. 2-C. The suppression of volume on 3D-CT imaging was 22,328 mm 3 at the right parotid gland, 19,562 mm 3 at the left parotid gland, 6,988 mm 3 at the right submandibular gland, and 3,874 mm 3 at the left submandibular gland. 3D-CT revealed obvious atrophic interference at the left canine fossa and zygomatic process of the maxilla Fig. 2-D. We confirmed that chin asymmetry did not exist during the nursing period by photographs. The condition was similar to PHA as previously described 4 6. Eventually, PHA associated with mainly hard tissue atrophy was diagnosed. To confirm that the progressive atrophy was interrupted, a physiological examination was performed. Thermography showed no exacerbation or further decrease of thermal difference in the neck and facial region. Electromyography revealed no change in activity at the sternocleidomastoid, masseter, and temporal muscles. Doppler blood-flow monitor revealed no alteration of arterial blood flow sounds at the superficial temporal, facial, and greater palatine arteries. There were no differences between the right and the left in these examinations data not shown. We were confident that there was no further progression of the atrophy. Consequently, the atrophy occurred mainly in hard tissue, and the distance between the first molar apex and horizontal plane on the upper left side was 8 mm shorter than on the upper right side. Because the patient desired improvement of his inclined occlusal plane, we carried out orthodontic surgery Le Fort I and intraoral vertical ramus osteotomy to lower the left side by 3.5 mm and raise the right side by 3.5 mm Fig. 3.

November, 2005 133 Fig. 2 Re-evaluation before orthodontic surgery. A Reduction of facial hair on the left side of his face was observed.

D There were obvious atrophic changes at the left canine fossa and zygomatic process of the maxilla on 3D-CT. Fig. 3 Schema of the operaton.

3 November, Fig. 2 Re-evaluation before orthodontic surgery. A Reduction of facial hair on the left side of his face was observed. B The median sulcus of his tongue was shifted to the left side. C Atrophy of the masseter muscle, parotid gland, and subcutaneous fat under the left cheek were shown on CT imaging. D There were obvious atrophic changes at the left canine fossa and zygomatic process of the maxilla on 3D-CT. Fig. 3 Schema of the operaton. Orthodontic surgery Le Fort I and intraoral vertical ramus osteotomy to lower the left side by 3.5 mm and raise the right side by 3.5 mm at the first molar tooth was selected to correct the inclined occlusal plane. In the 3-year follow-up period after the operation, he had good occlusal balance and the incline of his mouth aperture was improving without relapse of atrophy Fig. 4. Discussion PHA is a slow progressive atrophy of one side of the face, primarily involving the subcutaneous tissues 7. To classify PHA, Inigo 3 analyzed 78 PHA patients who

4 134 Oral Science International Vol. 2, No. 2 Fig. 4 Facial appearance post-operatively at 20 years of age. Balanced occlusal plane and aperture of the mouth without postoperative relapse or recurrence of the atrophy are shown. were treated by microsurgical free tissue transfer. PHA was classified as mild, moderate or severe, as well as on the basis of bone involvement in territories of the trigeminal nerve 8. Mild : atrophy of skin and subcutaneous tissue; involvement of only one of the sensory branches of the trigeminal nerve; and no bone involvement. Moderate: two trigeminal territories affected, without involvement of the osseous orbital region or maxillary and mandibular development. Severe : all three trigeminal territories involved or bone involved. By Inigo s criteria, 35% of PHA patients n 28 were mild, 49% n 38 were moderate, 16% n 12 were severe, and 84% had no bone involvement. However, our case involved hard tissue atrophic changes at the left molar tooth, maxilla, and mandible. Therefore we strongly believed that this was a rare case of hard tissue PHA. Many diseases present as maxillofacial asymmetry such as hemifacial microsomia HFM and PHA. Although acquired slowly progressing atrophy that is not congenital is characteristic of PHA, PHA may resemble HFM because of variability in location and degree. Abnormalities in the wide spectrum of HFM include first and second branchial arch syndrome, craniofacial microsomia, otomandibular dysostosis, oculoauriculovertebral dysplasia, Goldenhar syndrome, and lateral facial dysplasia with classified skeletal, auricular and soft tissue deficiencies 9. The orbit, mandible, ear, nerve, and soft tissue OMENS classification 10, which later become the OMENS-Plus classification 11, further differentiated skeletal components into bone and nerve involvement and placed soft tissue into its own category. Recently, Christopher 9 reported the distribution of phenotypic variability using OMENS-Plus classification in about 71 patients with HFM 61% had abnormally shaped ramus or/and condyle; 81%, ear abnormalities; 36%, extracraniofacial anomalies; and 77%, orbital hypoplasia. In our case, it was conjectured on the basis of the history of the patient s illness that progressive atrophy occurred during the first decade. Furthermore he had a normally formed ramus and condyle on the affected side and no orbital hypoplasia or extracraniofacial anomalies. In view of these various findings and reports, we diagnosed atypical PHA. PHA varies in location and degree. This variability is probably due to the absence of a single comprehensive etiology. While uncertain, the etiology of PHA has been associated with disorder of the connective tissue. In some cases there appears to be a genetic tendency suggesting an autosomal dominant inheritance with poor penetrance 12. PHA usually affects previously healthy patients during the first or early second decade of their life and is more frequent in females 2. If the disease appears during the first decade of life, it may interfere with the development of the skeletal structures on one side of the face. By contrast, when the disease occurs during the second decade, it is less likely to interfere with the overall growth of the facial bones 13. In our case, the patient had been aware of his chin asymmetry since he was 8 years old, and he was anxious about the inclined aperture of his mouth. It is suggested that the progressive hard tissue atrophy occurred during the first decade of his life and began before he was 8 years old. Volume restoration is the main problem in treating PHA. Since secondary or recurrence progressive atrophy has not been previously reported, we judged that the orthodontic surgery to correct this problem should be performed at 20 years old. Several corrective procedures have been proposed and been reported to give good results 8. These include dermal fat graft, silicone

5 November, fluid injections, cartilage and bone graft, pedicled flap graft, and free tissue transfer. The most popular treatment to correct facial asymmetry is pedicle flap grafting and orthodontic surgery provides secondary procedures to achieve better cosmetic and functional results 3. Neurolytic block is another surgical procedure that works well, especially on patients suspected of sympathomimetic symptoms in the atrophied region 14. Some infection cases such as meningoencephalitis 15 and poliomyelitis 16 were treated effectively with an antibiotic or antiviral medication. As far as we know, orthodontic surgery has never been selected as the first procedure to improve an asymmetry caused by PHA. In our case, the atrophy occurred mainly in hard tissue, and the patient underwent orthodontic surgery Le Fort I and intraoral vertical ramus osteotomy when he was 20 years old, to correct his severely inclined occlusal plane and asymmetry profile. In the 3-year follow-up, there were good occlusal balance and improved symmetrical profile without relapse or recurrence of the atrophy. Thus orthodontic surgery was effective as the first procedure in these circumstances i.e., obvious hard tissue atrophy appearing with mild soft tissue atrophy. Refereces 1 Kiskadden W.S., and McGregor M.W.: Report of a case of progressive facial hemiatrophy with pathological changes and surgical treatment. Plast Reconstr Surg , Burton J.L., and Lovell C.R.: Disorders of connective tissue. Textbook of Dermatology 3; , Inigo F., Jimenez-Murat Murat Y., Arroyo O., and Fernandez M.Y.: Restoration of facial contour in Romberg s disease and Hemifacial Microsomia: experience with 118 cases. Microsurgery , Whyman R.A., Doyle T.C.A., and Ferguson M.M.: An unusual case of hemifacial atrophy. Oral Surg Oral Med Oral Pathol , Fayad S., and Steffensen B.: Root resorption in a patient with hemifacial atrophy. J Endod , Trumpy I.G., Haakonsen M., Maehlen J., and Lyberg T.: Progressive facial hemiatrophy associated with an astrocytoma in the cerebellopontine region. J Oral Maxillofac Surg , Bilen N., Efendi H., Apaydin R., Bayramgurler D., Harova G., and Komsuoglu S.: Progressive facial hemiatrophy Parry-Romberg syndrome. Australas J Dermatol , Inigo F., Rojo P., and Ysunza A.: Aesthetic treatment of Romberg s disease: experience with 35 cases. Br J Plast Surg , Poon C.C., Meara J.G., and Heggie A.A.: Hemifacial microsomia: use of OMENS-Plus classification at the Royal Children s Hospital of Melbourne. Plast Reconstr Surg , Vento M.D., Labrie R.A., and Mulliken J.B.: The O.M.E.N.S. classification of hemifacial microsomia. Cleft Palate Craniofac J , Horgan J.E., Padora B.L., Labrie R.A., and Mulliken J.B.: OMENS-Plus: Analysis craniofacial and extracraniofacial anomalies in hemifacial microsomia. Cleft Palate Craniofac J , Fatma M.A., Yasar S., Hidayet E., Ali A., and Rustu E.: Parry-Romberg syndrome associated with Adie s pupil and radiologic findings. Pediatric Neurology , Moss M.L., and Crikelair G.F.: Progressive facial hemiatrophy following cervical sympathectomy in rat. Arch Oral Biol 1 254, Nishihara J., Miyake M., Ogawa T., Ohbayashi Y., Tanizaki A., and Nagahara S.: Stellate ganglion block and de-epithelialized free flap transfer in a patient with progressive hemifacial atrophy. Jpn J Oral Maxillofac Surg , Terstrgge K., Kunath B., Felber S., Speciali J.G., Henkes H., and Hosten N.: MR of brain involvement in progressive facial hemiatrophy Romberg disease : reconsideration of a syndrome. AJNR , Cohen M.M.Jr.: Perspectives on craniofacial asymmetry: Hemi-asymmetries. Int Oral Maxillofac Surg , 1995.

What is Hemifacial Microsomia? By Pravin K. Patel, MD and Bruce S. Bauer, MD Children s Memorial Hospital, Chicago, IL

What is Hemifacial Microsomia? By Pravin K. Patel, MD and Bruce S. Bauer, MD Children s Memorial Hospital, Chicago, IL 773-880-4094 Early in the child s embryonic development the structures destined to