|Year : 2020 | Volume
| Issue : 2 | Page : 200-203
Hemifacial microsomia managed by distraction osteogenesis: A clinicoradiological report
Shikha Dogra1, Vignesh Guptha Raju2, Vimanyu Kataria3
1 Department of Pedodontics and Preventive Dentistry, SGT Dental College and Hospital, Gurgaon, India
2 Department of Pedodontics and Preventive Dentistry, Karpaga Vinayaga Institute of Dental Sciences, Kanchipuram, Tamil Nadu, India
3 Department of Oral and Maxillofacial Surgery, Sudha Rustagi College of Dental Sciences and Research, Faridabad, Haryana, India
|Date of Submission||22-Sep-2019|
|Date of Decision||03-Jan-2020|
|Date of Acceptance||05-May-2020|
|Date of Web Publication||28-Jun-2020|
Dr. Shikha Dogra
3503, Ground Floor, Sector 57, Gurgaon - 122 413, Haryana
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Hemifacial microsomia (HFM) is a congenital malformation in which there is hypoplasia of hard and soft tissues on one side of the face. It is considered the second most common congenital syndrome of the head-and-neck region. The hypoplasia manifests in all the structures derived from the first and second branchial arches and therefore, growth and development of these structures is affected in this condition. The present case is a variant of HFM in a 5-year-old Indian male child, which has been diagnosed based on clinical and radiological findings and managed successfully by surgical technique known as distraction osteogenesis.
Keywords: Congenital syndrome, distraction osteogenesis, hemifacial microsomia
|How to cite this article:|
Dogra S, Raju VG, Kataria V. Hemifacial microsomia managed by distraction osteogenesis: A clinicoradiological report. J Indian Soc Pedod Prev Dent 2020;38:200-3
|How to cite this URL:|
Dogra S, Raju VG, Kataria V. Hemifacial microsomia managed by distraction osteogenesis: A clinicoradiological report. J Indian Soc Pedod Prev Dent [serial online] 2020 [cited 2020 Jul 13];38:200-3. Available from: http://www.jisppd.com/text.asp?2020/38/2/200/288225
| Introduction|| |
Hemifacial microsomia (HFM) is a congenital malformation in which there is hypoplasia of hard and soft tissues on one side of the face. It is primarily a syndrome involving structures derived from the first and second branchial arches leading to underdevelopment of temporomandibular joint, mandibular ramus, masticatory muscles, and ear and defects in facial nerve. HFM was first described by German physicians Carl Ferdin and Von Arlt in 1881. Because this condition has a wide range of phenotypic expression, the syndrome has been known by other names such as otomandibular dysostosis, Goldenhar syndrome, and oculoauriculovertebral syndrome. The incidence of HFM is 1:5000 live births.
| Case Report|| |
A 5-year-old male patient reported with the chief complaint of facial asymmetry and deviation of mouth toward the left side with difficulty in mouth opening. On extraoral examination, marked asymmetry of face was observed with deviation of midline toward the left side [Figure 1]. External ear and eyes were normal. Conductive hearing loss was also observed during otolaryngeal examination. Nerve examination did not reveal any abnormalities. Intraoral examination revealed cleft palate, forwardly placed maxillary anterior teeth, and absence of mandibular deciduous molars on the left side. The patient was clinically diagnosed as HFM of the left side of the face. Three-dimensional (3D) computed tomography revealed abnormalities such as deformed condyle and glenoid fossa, hypoplastic ramus, and elongated coronoid process of the left side [Figure 2]a, [Figure 2]b, [Figure 2]c. Axial computed tomography revealed cleft palate, absence of pterygoid plates on left side [Figure 3]a and b]. Based on the clinical and radiographic findings, a diagnosis of HFM was made. It was graded as HFM type O0M2BE0N0S1 based on the descriptive classification devised by Vento et al. using an alphanumeric coding system. The patient was referred to the department of oral maxillofacial surgery, and distraction osteogenesis of the left side of the mandible was performed [Figure 4]a and [Figure 4]b. Two weeks was allowed for healing, and the distraction device was activated by rotating the transcutaneous pin half a turn each day, hence yielding a distraction of 1 mm each day. A total of 12 mm distraction was advised, and the distraction device was placed in position for an additional 8 weeks to allow for neosteogenesis and stabilization of the joint. The final outcome of distraction was marked improvement in facial asymmetry and mouth opening was increased [Figure 5]. For cleft palate, prosthetic appliances such as an obturator may help to close a fistula or aid in speech [Figure 6]. Further management of the cleft palate with frequent follow-ups was advised to evaluate the skeletal and dental components.
|Figure 1: Preoperative picture showing facial asymmetry and occlusal cant line diverted toward the left side|
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|Figure 2: (a-c) Three-dimensional computed tomography shows hypoplasia of zygomatic arch, deviation of mandible on the left side, and hypoplasia of ramus on the left side|
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|Figure 3: (a and b) Sagittal computed tomography section showing aplasia of condylar head and cleft palate|
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|Figure 4: (a and b) Intraoperative picture showing placement of transmandibular distraction device|
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|Figure 5: Postoperative picture showing improvement in facial asymmetry 1 week after the surgery|
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| Discussion|| |
HFM is the second most common congenital facial anomaly after cleft lip and palate. Males are more commonly affected than females (3:2). Unilaterally, right side is more commonly affected than left side. Our case also manifests HFM in a male patient on the left side. Imaging plays an important role in establishing the diagnosis of HFM. Conventional radiography with the use of panoramic radiographs provides an easy comparison between the malformed and the normal side. Computed tomography can provide both a 3D rendition of the soft tissue of the face and an image of the underlying bone. It helps in establishing the degree of anatomical malformation and the relationship of the deformity to the adjacent craniofacial skeleton.,
Management of HFM necessitates a multidisciplinary approach. The surgery may be done during the growth phase or after the growth phase is over. Distraction osteogenesis is a surgical procedure in which new bone is formed between bone segments that gradually are separated by incremental traction. Mandibular lengthening by gradual distraction is a surgical procedure which corrects the gross mandibular asymmetry and hence should be widely used in cases of HFM. The etiopathogenesis of HFM is not yet determined and incompletely understood. Various authors have theorized possible mechanisms such as deficiency in neural crest cell migration to the region of first and second branchial arches, hemorrhage associated with the formation of stapedial artery during embryogenesis, and mutation of locus termed hfm (B1–B3 on chromosomal 10), leading to mesenchymal disruption. In normal parents, the chances of HFM in the second child getting affected is 1%, while the chances of passing the condition to offspring in the affected parents is 3%.,
The clinical features of HFM are variable, ranging from slight asymmetry of the face to severe underdevelopment of one facial half with orbital implications and a partially formed ear to complete absence of ears. The chin and facial midline are deviated toward the affected side, with shifting of corner of the mouth toward the affected side. Bony abnormalities include unilateral shorter zygomatic arch and hypoplastic maxillary and temporal bone. In most cases reported, there is underdevelopment of condyle, but aplasia of mandibular ramus and/or condyle may also be seen in some cases. The masseter, pterygoid, and temporal muscles are hypoplastic on the affected side. Intraoral features include agenesis of the third molar and second premolar on the affected side, as well as supernumerary teeth, enamel malformations, delay in tooth development and hypoplastic teeth, and cleft lip and palate.
In the present case, the extraoral manifestations were similar to the defined criteria. Intraorally, findings were missing posterior teeth in the mandible left side and cleft palate. Radiographic features which were observed were also suggestive for HFM variant. Differential diagnosis includes Pierre–Robins syndrome, Moebius syndrome, and Treacher–Collins syndrome.,,
Mandibular lengthening by gradual distraction was reported in 1973 by Synder et al., who used an extraoral device in canine study. McCarthy et al. were the first to apply distraction osteogenesis to craniofacial skeleton in children having congenital deformity. Distraction osteogenesis has been mentioned as a reliable and versatile technique as with increase in the volume of hard tissue, it facilitates soft-tissue histogenesis.
Karp and Thorne have mentioned that early elongation of mandible may minimize progressive restriction in mid-face growth by restoration of a normal functional matrix. Karun and Navneet in their study have concluded that distraction osteogenesis is a novel technique for correction of mandibular abnormalities. Being a surgical procedure, distraction osteogenesis possess disadvantages such as scar formation that result from pin tracks in the skin, retention of healing device even during the lag phase and requires frequent follow up of the patient. Through extraoral distraction osteogenesis of mandible done in the left side, our patient has greatly improved functional and esthetic correction.
| Conclusion|| |
Accurate diagnosis plays a vital role while treating patients with HFM because many craniofacial anomalies can be misdiagnosed. Management of patients with HFM depends on the patient's age and severity of the deformity. As discussed, treatment of HFM requires multidisciplinary approach, which includes consultation from a surgeon to determine patients who need early surgery. Furthermore, long-term studies are required for treatment modality such as distraction osteogenesis.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]