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Article

A Proposal for the Classification of Temporomandibular Joint Disc Deformity in Hemifacial Microsomia

by
Xiaochen Xue
1,2,
Zhixu Liu
1,2,
Hongpu Wei
1,2 and
Xudong Wang
1,2,*
1
Department of Oral and Cranio-Maxillofacial Surgery, Shanghai Ninth People’s Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200011, China
2
National Clinical Research Center for Oral Disease, Shanghai Key Laboratory of Stomatology, Shanghai Research Institute of Stomatology, Shanghai 200011, China
*
Author to whom correspondence should be addressed.
Bioengineering 2023, 10(5), 595; https://doi.org/10.3390/bioengineering10050595
Submission received: 4 May 2023 / Revised: 12 May 2023 / Accepted: 12 May 2023 / Published: 16 May 2023
(This article belongs to the Special Issue Application of Bioengineering to Clinical Orthodontics)
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Abstract

:
Hemifacial microsomia (HFM) is the second most common congenital craniofacial disease and has a wide spectrum of symptoms. The classic diagnostic criterion for hemifacial microsomia is the OMENS system, which was later refined to the OMENS+ system to include more anomalies. We analyzed the data of 103 HFM patients with magnetic resonance imaging (MRI) for temporomandibular joint (TMJ) discs. The TMJ disc classification was defined into four types: D0 for normal disc size and shape; D1 for disc malformation with adequate length to cover the (reconstructed) condyle; D2 for disc malformation with inadequate length to cover the (reconstructed) condyle; and D3 for no obvious presence of a disc. Additionally, this disc classification was positively correlated with the mandible classification (correlation coefficient: 0.614, p < 0.01), ear classification (correlation coefficient: 0.242, p < 0.05), soft tissue classification (correlation coefficient: 0.291, p < 0.01), and facial cleft classification (correlation coefficient: 0.320, p < 0.01). In this study, an OMENS+D diagnostic criterion is proposed, confirming the conjecture that the development of the mandibular ramus, ear, soft tissue, and TMJ disc, as homologous and adjacent tissues, is affected to a similar degree in HFM patients.

Graphical Abstract

1. Introduction

Hemifacial microsomia (HFM) refers to a series of complicated and varied symptoms caused by malformation of the first and second branchial arches during embryogenesis, with different names, such as craniofacial microsomia, first and second branchial arch syndrome, otomandibular dysostosis, and auriculobranchiogenic dysplasia [1]. It is the second most common facial congenital defect, following cleft lip/palate, with an incidence estimated at 1 in 3000 to 1 in 5600 live births [1,2,3]. Most hemifacial microsomia cases occur sporadically and unilaterally, with bilateral hypoplasia noted in 5–30% of cases [1]. HFM involves hard tissues such as the upper and lower jaws and related soft tissues and can be manifested as a series of malformations. Eye malformations include strabismus, anophthalmia, microphthalmia, eye asymmetry, blepharoptosis, and exophthalmos. Auricular abnormalities include appendage, preauricular fistula, microtia, ear asymmetry, and external auditory canal atresia. Malformations of the first and second pharyngeal arches include cleft lip and palate, cleft tongue, maxillary and mandibular hypoplasia, and malocclusion [4]. Additionally, HFM patients often have extracranial defects, with neurological abnormalities present in 5% to 15% of patients, cardiac abnormalities present in 14% to 47% of patients, urogenital abnormalities present in 5% to 6% of patients, lung and gastrointestinal abnormalities present in 10% of patients, and skeletal abnormalities present in 40% to 60% of patients [5,6,7,8]. Therefore, a comprehensive evaluation of hearing assessment, speech analysis, psychosocial assessment, and spinal imaging should be performed based on the patient’s symptoms [9].
To better describe and classify the wide spectrum of symptoms, several grading systems have been proposed. The Pruzansky [10] and Pruzansky–Kaban [11] classifications focus on mandible defects: Type I: normal morphologic characteristics of the ramus and condyle but diminished in size. Type II: significant architectural and size distortion of the ramus, condyle, and sigmoid notch; Type IIA: acceptable glenoid fossa anatomy and position with respect to the unaffected side; Type IIB: temporomandibular joint (TMJ) malposition. Type III: gross distortion or complete agenesis of the ramus. Meanwhile, the Lauritzen [12] classification evaluates not only the mandible but also the entire facial area with simple standards: Type I: facial skeleton is complete; TMJ is intact and functional; mandible asymmetrical, creating a lack of fullness on one side of the face; Type IA: mild; Type IB: severe (depending on the degree of asymmetry). Type II: the condylar head is missing with no functional TMJ; zygomatic arch present; adequate support of a reconstructed condyle and fossa construction is not necessary. Type III: condyle is missing, and zygomatic arch is hypoplastic or absent; glenoid fossa is absent or rudimentary and must be constructed. Type IV: in addition to Type III defects, the lateral and inferior orbital rims are grossly recessed posteriorly. Type V: in addition to Type IV defects, the orbit is dystopic and frequently hypoplastic; the neurocranium is asymmetrical with a flat temporal fossa. Meurman [13] and Murray et al. [14] studied ear and soft tissue defects, respectively. The SAT classification was introduced based on these previous grading systems [7]. Then, Vento et al. took a further step and proposed what is currently the most agreed upon “OMENS” classification system [15], which was later refined to “OMENS+” to include more anomalies [5].
In “OMENS+”, each letter stands for a kind of defect, as shown in Table 1. “O” stands for orbital asymmetry, evaluating both orbital size and position. “M” stands for mandibular hypoplasia, evaluating the size and shape of the ramus, condyle, and glenoid fossa. “E” stands for external ear deformity. “N” stands for facial nerve dysfunction. “S” stands for soft tissue deficiency. “+” stands for the presence of associated anomalies, and macrostomia/transverse facial cleft is frequently mentioned and written as “+C” [1,5,9,15,16,17].
Studying magnetic resonance imaging (MRI) of the temporomandibular joint (TMJ) is necessary to evaluate the TMJ’s condition and to make treatment plans for HFM patients. Nebbe et al. first superimposed sagittal MRI to lateral cephalometric radiographs to evaluate the TMJ disc position in 1998 [18], and since then, MRI has gradually become the standard for viewing the cartilaginous disc of the TMJ. When analyzing the TMJ MRI of hemifacial microsomia patients, we found TMJ disc malformation, malposition, and even absence in many cases. Anterior disc displacement with or without reduction could be observed in patients with enough TMJ mobility. Additionally, for patients with little or no mobility of the TMJ, discs are usually in the wrong places compared to normal ones because of the malformation of the glenoid fossa and condyle. In our study, we would like to focus on the disc’s length considering the potential condyle–disc relationship with a condyle or reconstructed condyle. An improper condyle–disc relationship may cause facial asymmetry or malocclusion [19], which leads to less stability of surgical and orthodontic treatment for HFM patients.
In this study, we aimed to fulfill the OMENS+ system with a TMJ disc classification and investigate the correlation between disc and OMENS+C classifications. Our goal is to bring attention to the TMJ disc in hemifacial microsomia patients in order to aid in comprehensive surgery planning and prognosis for the stability of surgical treatment and orthodontic treatment.

2. Materials and Methods

2.1. Study Population

A total of 103 consecutive patients diagnosed with unilateral or bilateral hemifacial microsomia who were referred to the Department of Oral and Craniomaxillofacial Surgery in Shanghai Ninth People’s Hospital were chosen. Medical charts, including age, gender, affected side, family history, photographs, and radiographs—including cephalometric films, panoramic radiographs, computed tomography (CT) with three-dimensional (3D) reconstruction, and TMJ MRI—were reviewed. Patients with incomplete chart documentation of their deformities based on the OMENS+ system were excluded.
Among the 103 patients, there were 46 females and 57 males, aged 5 to 39 years old.

2.2. CT, 3D Reconstruction, and MRI

CT scans were obtained using a 1.25 mm thick slice with a hospital-based spiral scanner (GE Healthcare). The CT data were imported into ProPlan CMF 2.0 (Materialise) for 3D reconstruction [20,21].
MRI scans were obtained using a 1.5 T imager (Signa, General Electric, Milwaukee, WI) with bilateral 3-inch TMJ surface coil receivers [22]. The transection plane was scanned to find the long axis of the condyle, and the sagittal plane was then determined to be perpendicular to this long axis [23].

2.3. Statistical Analysis

For bilateral patients, we defined both affected mandibles and discs as two single samples during statistical analysis. The correlation between orbit, mandible, external ear, facial nerve, soft tissue, facial cleft, and disc classifications was analyzed with the Spearman rank correlation coefficient test (2-tailed).

3. Results

3.1. Classification of the OMENS+C

All 103 patients were evaluated and classified according to the OMENS+C system [1]. We present the mandible classification as an example here. M0: normal mandible; M1: small mandible and glenoid fossa with short ramus (Figure 1a); M2a: abnormally shaped and short ramus (glenoid fossa in acceptable position) (Figure 1b); M2b: abnormally shaped and short ramus (glenoid fossa is inferiorly, medially, and anteriorly displaced with severe hypoplasia of condyle) (Figure 1c); M3: absence of ramus and glenoid fossa (Figure 1d). Besides mandibular defects, the orbital, ear, facial nerve, and soft tissue defects and facial cleft were also evaluated.

3.2. Definition of Disc Classification

Studies have shown that untreated TMJ disc displacement may lead to an unstable maxilla–mandibular complex after orthognathic surgery and cause skeletal relapse related to condylar remodeling and resorption [24,25]. These studies indicated a possible instability of the mandible after surgical repositioning. Therefore, a proper condyle–disc relationship should be expected not only in orthognathic surgery but also in mandibular distraction osteogenesis (MDO) and costochondral grafting (CCG), which are currently the major surgical methods for HFM. The sagittal slice with the largest cross-section of the TMJ disc was chosen to evaluate the disc classification in each patient on the affected side. The evaluation criteria included the shape, length, and presence of the disc. We did not take the preoperative condyle and disc position into consideration, because most HFM patients have condylar deformities and malpositioned discs. Instead, we focused on the potential condyle–disc relationship, which includes the current or reconstructed condyle and the disc’s length.
We defined the disc classification as follows:
D0: Normal disc size and shape (Figure 2). The TMJ disc is marked with a dotted white line in the following figures;
D1: Disc malformation with adequate length to cover the (reconstructed) condyle (Figure 3). A proper condyle–disc relationship could be expected after a certain treatment in this type;
D2: Disc malformation with inadequate length to cover the (reconstructed) condyle (Figure 4). After a certain treatment, an acceptable condyle–disc relationship could be expected in this type;
D3: No obvious presence of disc (Figure 5). No condyle–disc relationship could be observed in this type. Treatment stability may be influenced.

3.3. Sample Distribution

We analyzed the distribution of OMENS+C and disc classifications in 108 samples (Table 2), as we defined both affected sides as two single samples in bilateral HFM patients.
The sample distribution of orbital defects shows that O0 (32.4%) and O2 (30.6%) are the most common types, while O1 (22.2%) and O3 (14.8%) types are less commonly observed. Regarding mandibular defects, M2a (32.4%) and M2b (41.7%) are the most common types, while M1 (13.9%) and M3 (12.0%) samples are fewer in number. Regarding external ear defects, E0 (33.3%), E2 (25.0%), and E3 (30.6%) are all the most common types, while the E1 (11.1%) type is observed less. Regarding facial nerve defects, N0 (77.8%) is the most common type, while N1 (9.3%), N2 (10.2%), and N3 (2.8%) are all observed less. Regarding soft tissue defects, S0 (27.8%), S1 (38.0%), and S2 (28.7%) are all the most common types, while S3 (5.6%) samples are fewer in number. Regarding facial clefts, C0 (72.2%) is the most common type, while C1 (19.4%) and C2 (8.3%) are observed less. Regarding TMJ disc defects, D0 (28.7%), D2 (33.3%), and D3 (22.2%) are the most common types, while D1 (15.7%) samples are fewer in number.
Most of the patients who came to our department were mainly seeking medical advice for malformed or asymmetrical mandibles. This mandible classification distribution is quite reasonable and reveals that the M3 type, the most severe type, is relatively rare in the affected population. Additionally, in the M1 type, a milder type, patients might not notice their mild mandibular malformation or are simply unwilling to seek medical advice with no other symptoms. Thus, M3 and M1 samples are fewer in number than the other two types.

3.4. Correlation between OMENS+C and Disc Classifications

The correlation between OMENS+C and disc classifications among all the included samples was statistically analyzed (Table 3). A positive correlation was found between disc and mandibular defects (correlation coefficient: 0.614, p < 0.01), disc and ear defects (correlation coefficient: 0.242, p < 0.05), disc and soft tissue defects (correlation coefficient: 0.291, p < 0.01), and disc defects and facial clefts (correlation coefficient: 0.320, p < 0.01). These results indicate similar degrees of TMJ disc defects; mandibular defects; and ear, soft tissue defects, and facial clefts in HFM patients. In addition, we also found a positive correlation between orbital and soft tissue defects (correlation coefficient: 0.245, p < 0.05), mandible and soft tissue defects (correlation coefficient: 0.332, p < 0.01), mandible and facial cleft defects (correlation coefficient: 0.332, p < 0.01), and ear and soft tissue defects (correlation coefficient: 0.340, p < 0.01). These results also indicate a similar degree of defects of corresponding components in the OMENS+ system in hemifacial microsomia patients.

4. Discussion

When the OMENS system was first introduced, Vento et al. studied the correlation between the five components in this system and found associations between the mandible and the other four components, between orbit and soft tissue defects, between ear and nerve defects, and between nerve and soft tissue defects [15]. Additionally, there have been a few other studies about the correlation between different components in OMENS or OMENS+ systems. Wan et al. studied the correlation between the OMENS score and hearing loss in 70 HFM patients and found that the ear defect score does not correlate with hearing loss degree or hearing loss type [26]. Two other studies both found significant associations between orbital and mandibular defects and between mandibular and soft tissue defects, but they drew different conclusions when it came to other components [3,27]. There are two possible reasons for these different results. First, there is always bias between different observers when deciding the severity of symptoms, particularly for soft tissue defects, which were originally not clearly defined. CT and 3D reconstruction are always necessary to evaluate soft tissue sufficiency to fulfill surgical plans, even for experienced surgeons. The second possible reason is the special definition of facial nerve defects: N1 stands for upper-branches involvement, N2 for lower-branches involvement, and N3 for all-branches defects [1]. In other classifications, the greater the number, the more severe the symptom. For example, M3 is more severe than M2a. However, in nerve classification, N2 and N1 stand for different types of defects, with no comparison of severity, making it illogical to analyze them in the same way as other classifications. Based on these facts, more detailed classifications should be added to the OMENS+ system, and better ways to evaluate the severity of HFM symptoms are needed. In our study, the disc classification also faced problems of bias between different observers, especially when it comes to D1 and D2 to determine whether the disc length is sufficient to cover the condyle or the reconstructed condyle. Thus, quantitative evaluation in MRI images might be necessary in the refined future version. Nonetheless, a positive correlation between mandible, ear, soft tissue, cleft, and disc classifications was found in this study, and the disc classification is logical and reasonable for revealing the TMJ disc condition for hemifacial microsomia patients, and thus should be accepted as part of the OMENS+D system.
Most of the patients who visited our department were mainly seeking medical advice for malformed or asymmetrical mandibles. Treatment for HFM patients’ mandibular deformities still remains partly controversial. In the past, the degree of hemifacial microsomia deformity was used to guide treatment. Surgical treatment to reconstruct the mandible was used in children with more severe defects, while treatment for children with milder deformities could be variable [17]. Gillies first introduced the use of cartilage and bone from the rib cage to address the hypoplastic mandible in the 1920s [28]. Additionally, in 1990, McCarthy’s group began to perform MDO, and this technique became popular and widely used [29,30]. Nowadays, in order to improve facial symmetry and occlusion, CCG and MDO are the two most commonly performed operations for HFM children, though surgical procedures and surgical timing have not yet reached an agreement [16,17]. For M2b and M3 patients with unstable occlusion, MDO cannot induce the formation of the joint at the right location, and CCG has been the gold-standard technique for these types of deformities. However, soft tissue elongation, better stability, and less invasion are distinct advantages of MDO. Studies also show that the later the surgery is performed, the more stable the result could be [31]. Regarding adult hemifacial microsomia patients with milder mandibular asymmetry, combined orthognathic surgery and orthodontic treatment has been widely accepted [32]. Meanwhile, for some M2b and M3 adult patients, it is necessary to perform MDO to supply enough ramus length and soft tissue before orthognathic surgery. With the help of CT, 3D reconstruction, and 3D printing, virtual surgical planning and customized templates provide more direct and accurate surgical results [20,21]. In further studies, we plan to compare how different disc types affect surgery stability in different surgical plans. More accurate and predictive treatment plans can be expected with the help of TMJ disc classification in hemifacial microsomia patients.
When looking for possible pathogenic genes for HFM, we found many studies, but the wide spectrum of symptoms leads to numerous candidates [17,33]. The duplication of OTX2, for example, was implicated in facial cleft and mandibular hypoplasia but not orbital, ear, or nerve defects [34]. In contrast, 22q11.2 deletion caused both mandibular and ear defects in several cases [35,36] and ocular defects in other cases [37]. The haploinsufficiency of SF3B2 could cause mainly mandibular hypoplasia, and facial cleft and the absence of TMJ also appeared in some cases [38]. In addition, the mutation in TCOF1, VWA1, and 14q32 could also cause HFM symptoms [39,40]. However, regarding the animal model, only the Hfm heterozygous mouse model was established to study hemifacial microsomia. The phenotypes include mandibular hypoplasia and external ear abnormalities, but no detailed mapping of mutation sites or follow-up research has been conducted [41,42]. Hoxa2 is an important gene that determines the development patterns of the first and second branchial arches of mice. Hoxa2 −/− mice and Hoxa2 flox/flox; Wnt1-Cre mice exhibit a trend of transformation from the second branchial arch to the first branchial arch and trigger rapid changes in gene expression patterns, such as Alx4 and Six2; thus, abnormal expressions of Alx4 and Six2 could be detected in the second branchial arch at E10.5 [43,44]. Given the inspiration of animal models, more and more causative genes could be found [45,46], and it might be possible in the further study to narrow down symptoms and the number of patients, such as patients with both M3 and D3, to investigate possible causative genes for certain types of HFM.
There are three theories for the pathogenesis of HFM [33]. The first is disruption of the stapedial artery, leading to hemorrhage and subsequent deformation of the surrounding tissues; the second is abnormal migration of the subpopulation of the cranial neural crest cell migration, leading to dysmorphogenesis; and the third is the interference of Meckel’s cartilage development, which is thought to be a supplement to the first theory. According to the first theory, which was first brought up by Poswillo and verified with a rat model in 1973 [47], similar defects are expected between adjacent developing structures near the hematoma or structures with the same blood supply. This partially explains the results above, where soft tissue defects always correlate with other defects as adjacent tissues during embryogenesis. The second theory, on the other hand, suggests similar defects between structures developed from the same streams of the branchial arches. The first branchial arch gives rise to the maxilla, mandible, zygoma, trigeminal nerve, masticatory muscles, connective tissue, and a minor part of the external ear. The second branchial arch gives rise to the stapes, styloid process, portions of the hyoid bone, facial nerve, facial muscles, and the major part of the external ear. Regardless of which theory is chosen, a certain correlation should be found between the OMENS+ system components. In our study, disc classification can reveal the TMJ disc condition for hemifacial microsomia patients and help with more comprehensive treatment planning, and it should be accepted as an OMENS+D system. In addition, we found a positive correlation between the TMJ disc and mandibular defects, between the TMJ disc and ear defects, between the TMJ disc and soft tissue defects, and between the TMJ disc defect and facial cleft, which confirms the conjecture that homologous and adjacent tissues are affected to a similar degree in HFM patients.
There are several limitations to this study. First, the localization of the MRI study planes was not standardized. Due to the various structures of the TMJ in HMF patients, the long axis of condyles could not be symmetrical on both sides. To address this issue, we followed the routine of the MRI study plane: the transection plane was scanned to find the long axis of the condyle, and the sagittal plane was then determined to be perpendicular to this long axis. Additionally, for those condyles whose long axis could not be identified, the long axis of the ramus was used, and the sagittal plane was determined to be perpendicular to this long axis. Second, CCG and MDO are the two major surgical methods for HFM patients, yet there is no agreement on surgical procedures and timing. Whether this new proposed +D classification could provide support or suggestions in deciding surgical methods is still unknown. Surgical results and long-term follow-up are needed in future studies. Based on the unknown pathogenic gene and study limitations mentioned above, we have primarily planned some future research. On the one hand, we plan to narrow down the included symptoms, such as patients with both M3 and D3, to investigate possible causative genes for this most severe type of HFM using techniques such as a GWAS (Genome-Wide Association Study) or WES (Whole Exome Sequencing). Mouse models should be established for further verification and mechanism investigation. On the other hand, MDO and CCG could be performed on patients of different D classifications in order to investigate if certain surgical methods are more suitable for certain TMJ disc conditions.

5. Conclusions

In conclusion, this essay has proposed a new TMJ disc classification of hemifacial microsomia and has refined the diagnostic criterion as OMENS+D. With the definition of D0, D1, D2, and D3, different TMJ disc conditions could be directly revealed for HFM patients. Additionally, this D classification was proved to be positively correlated with mandibular defects, ear defects, soft tissue defects, and facial clefts, which confirms the conjecture that homologous and adjacent tissues are affected to a similar degree in HFM patients.

Author Contributions

Conceptualization, X.W. and X.X.; methodology, Z.L.; software, H.W.; validation, X.W. and X.X.; formal analysis, X.X.; writing—original draft preparation, X.X.; writing—review and editing, X.W.; visualization, X.X.; supervision, X.W.; funding acquisition, X.W. and Z.L. All authors have read and agreed to the published version of the manuscript.

Funding

This research was funded by the National Natural Science Foundation of China, grant numbers 82071096 and 82001027.

Institutional Review Board Statement

The study was conducted in accordance with the Declaration of Helsinki and approved by the Institutional Review Board (or Ethics Committee) of Shanghai Ninth People’s Hospital (protocol code (2015)76, 2015).

Informed Consent Statement

Informed consent was obtained from all subjects involved in the study.

Data Availability Statement

Not applicable.

Conflicts of Interest

The authors declare no conflict of interest.

References

  1. Gougoutas, A.J.; Singh, D.J.; Low, D.W.; Bartlett, S.P. Hemifacial Microsomia: Clinical Features and Pictographic Representations of the OMENS Classification System. Plast. Reconstr. Surg. 2007, 120, 112e–120e. [Google Scholar] [CrossRef] [PubMed]
  2. Hartsfield, J.K. Review of the etiologic heterogeneity of the oculo-auriculo-vertebral spectrum (Hemifacial Microsomia). Orthod. Craniofac. Res. 2007, 10, 121–128. [Google Scholar] [CrossRef] [PubMed]
  3. Tuin, A.J.; Tahiri, Y.; Paine, K.M.; Paliga, J.T.; Taylor, J.A.; Bartlett, S.P. Clarifying the Relationships among the Different Features of the OMENS+ Classification in Craniofacial Microsomia. Plast. Reconstr. Surg. 2015, 135, 149e–156e. [Google Scholar] [CrossRef] [PubMed]
  4. Bogusiak, K.; Puch, A.; Arkuszewski, P. Goldenhar syndrome: Current perspectives. World J. Pediatr. 2017, 13, 405–415. [Google Scholar] [CrossRef]
  5. Horgan, J.E.; Padwa, B.L.; Labrie, R.A.; Mulliken, J.B. OMENS-Plus: Analysis of Craniofacial and Extracraniofacial Anomalies in Hemifacial Microsomia. Cleft Palate-Craniofac. J. 1995, 32, 405–412. [Google Scholar] [CrossRef]
  6. Pierpont, M.E.M.; Moller, J.H.; Gorlin, R.J.; Edwards, J.E. Congenital cardiac, pulmonary, and vascular malformations in oculoauriculovertebral dysplasia. Pediatr. Cardiol. 1982, 2, 297–302. [Google Scholar] [CrossRef]
  7. David, D.J.; Mahatumarat, C.; Cooter, R.D. Hemifacial microsomia—A multisystem classification. Plast. Reconstr. Surg. 1987, 80, 525–535. [Google Scholar] [CrossRef]
  8. Rollnick, B.R.; Kaye, C.I.; Nagatoshi, K.; Hauck, W.; Martin, A.O.; Reynolds, J.F. Oculoauriculovertebral dysplasia and variants: Phenotypic characteristics of 294 patients. Am. J. Med Genet. 1987, 26, 361–375. [Google Scholar] [CrossRef]
  9. Birgfeld, C.; Heike, C. Craniofacial Microsomia. Clin. Plast. Surg. 2019, 46, 207–221. [Google Scholar] [CrossRef]
  10. Pruzansky, S. Not all dwarfed mandibles are alike. Birth Defects 1969, 1, 120–129. [Google Scholar]
  11. Kaban, L.B.; Moses, M.H.; Mulliken, J.B. Surgical correction of hemifacial microsomia in the growing child. Plast. Reconstr. Surg. 1988, 82, 9–19. [Google Scholar] [CrossRef]
  12. Lauritzen, C.; Munro, I.R.; Ross, R.B. Classification and treatment of hemifacial microsomia. Scand. J. Plast. Reconstr. Surg. 1985, 19, 33–39. [Google Scholar] [CrossRef]
  13. Meurman, Y. Congenital Microtia and Meatal Atresia: Observations and Aspects of Treatment. AMA Arch. Otolaryngol. Neck Surg. 1957, 66, 443–463. [Google Scholar] [CrossRef]
  14. Murray, J.E.; Kaban, L.B.; Mulliken, J.B. Analysis and Treatment of Hemifacial Microsomia. Plast. Reconstr. Surg. 1984, 74, 186–199. [Google Scholar] [CrossRef]
  15. Vento, A.R.; LaBrie, R.A.; Mulliken, J.B. The O.M.E.N.S. classification of hemifacial microsomia. Cleft Palate Craniofac. J. 1991, 28, 68–76. [Google Scholar] [CrossRef]
  16. Birgfeld, C.B.; Heike, C. Craniofacial microsomia. Semin. Plast. Surg. 2012, 26, 91–104. [Google Scholar] [CrossRef]
  17. Brandstetter, K.A.; Patel, K.G. Craniofacial Microsomia. Facial. Plast. Surg. Clin. N. Am. 2016, 24, 495–515. [Google Scholar] [CrossRef]
  18. Nebbe, B.; Major, P.W.; Prasad, N.G.N.; Hatcher, D. Quantitative assessment of temporomandibular joint disk status. Oral Surg. Oral Med. Oral Pathol. Oral Radiol. Endod. 1998, 85, 598–607. [Google Scholar] [CrossRef]
  19. Xie, Q.; Yang, C.; He, D.; Cai, X.; Ma, Z. Is mandibular asymmetry more frequent and severe with unilateral disc displacement? J. Craniomaxillofac. Surg. 2015, 43, 81–86. [Google Scholar] [CrossRef]
  20. Sun, H.; Zhang, J.; Li, B.; Liu, Z.; Shen, S.; Wang, X. Accuracy of a new custom-made bone-supported osteotomy and repositioning guide system for reconstruction of the mandibular ramus using costochondral grafts: A preliminary study. Br. J. Oral Maxillofac. Surg. 2020, 58, 51–56. [Google Scholar] [CrossRef]
  21. Li, B.; Sun, H.; Zeng, F.; Zhang, T.; Wang, X. Accuracy of a CAD/CAM surgical template for mandibular distraction: A preliminary study. Br. J. Oral Maxillofac. Surg. 2018, 56, 814–819. [Google Scholar] [CrossRef] [PubMed]
  22. Hu, Y.K.; Yang, C.; Xie, Q.Y. Changes in disc status in the reducing and nonreducing anterior disc displacement of temporomandibular joint: A longitudinal retrospective study. Sci. Rep. 2016, 6, 34253. [Google Scholar] [CrossRef] [PubMed]
  23. Kitai, N.; Murakami, S.; Takashima, M.; Furukawa, S.; Kreiborg, S.; Takada, K. Evaluation of Temporomandibular Joint in Patients With Hemifacial Microsomia. Cleft Palate Craniofac. J. 2004, 41, 157–162. [Google Scholar] [CrossRef] [PubMed]
  24. Al-Moraissi, E.A.; Wolford, L.M. Does Temporomandibular Joint Pathology with or without Surgical Management Affect the Stability of Counterclockwise Rotation of the Maxillomandibular Complex in Orthognathic Surgery? A Systematic Review and Meta-Analysis. J. Oral Maxillofac. Surg. 2017, 75, 805–821. [Google Scholar] [CrossRef]
  25. Goncalves, J.R.; Cassano, D.S.; Wolford, L.M.; Santos-Pinto, A.; Marquez, I.M. Postsurgical stability of counterclockwise maxillomandibular advancement surgery: Affect of articular disc repositioning. J. Oral Maxillofac. Surg. 2008, 66, 724–738. [Google Scholar] [CrossRef]
  26. Wan, J.; Meara, J.G.; Kovanlikaya, A.; Nelson, M.D.; Don, D. Clinical, Radiological, and Audiological Relationships in Hemifacial Microsomia. Ann. Plast. Surg. 2003, 51, 161–166. [Google Scholar] [CrossRef]
  27. Park, J.U.; Do, T.H.; Kwon, G.Y.; Choi, T.H.; Kim, S. Statistical analysis using the OMENS classification in Oriental patients with hemifacial microsomia: A comparative analysis with Western centers. Ann. Plast. Surg. 2014, 72, 50–55. [Google Scholar] [CrossRef]
  28. Gillies, H.D. Plastic surgery of the face. Lancet 1920, 196, 177–192. [Google Scholar] [CrossRef]
  29. Klein, C.; Howaldt, H.-P. Correction of Mandibular Hypoplasia by Means of Bidirectional Callus Distraction. J. Craniofac. Surg. 1996, 7, 258–266. [Google Scholar] [CrossRef]
  30. McCarthy, J.G.; Stelnicki, E.J.; Mehrara, B.J.; Longaker, M.T. Distraction Osteogenesis of the Craniofacial Skeleton. Plast. Reconstr. Surg. 2001, 107, 1812–1824. [Google Scholar] [CrossRef]
  31. Mommaerts, M.Y.; Nagy, K. Is early osteodistraction a solution for the ascending ramus compartment in hemifacial microsomia? A literature study. J. Cranio-Maxillofac. Surg. 2002, 30, 201–207. [Google Scholar] [CrossRef]
  32. Wang, P.; Wang, Y.; Zhang, Z.; Li, X.; Ye, B.; Li, J. Comprehensive consideration and design with the virtual surgical planning-assisted treatment for hemifacial microsomia in adult patients. J. Cranio-Maxillofacial Surg. 2018, 46, 1268–1274. [Google Scholar] [CrossRef]
  33. Chen, Q.; Zhao, Y.; Shen, G.; Dai, J. Etiology and Pathogenesis of Hemifacial Microsomia. J. Dent. Res. 2018, 97, 1297–1305. [Google Scholar] [CrossRef]
  34. Zielinski, D.; Markus, B.; Sheikh, M.; Gymrek, M.; Chu, C.; Zaks, M.; Srinivasan, B.; Hoffman, J.D.; Aizenbud, D.; Erlich, Y. OTX2 Duplication Is Implicated in Hemifacial Microsomia. PLoS ONE 2014, 9, e96788. [Google Scholar] [CrossRef]
  35. Digilio, M.C.; McDonald-McGinn, D.M.; Heike, C.; Catania, C.; Dallapiccola, B.; Marino, B.; Zackai, E.H. Three patients with oculo-auriculo-vertebral spectrum and microdeletion 22q11.2. Am. J. Med Genet. Part A 2009, 149A, 2860–2864. [Google Scholar] [CrossRef]
  36. Xu, J.; Fan, Y.S.; Siu, V.M. A child with features of Goldenhar syndrome and a novel 1.12 Mb deletion in 22q11.2 by cytogenetics and oligonucleotide array CGH: Is this a candidate region for the syndrome? Am. J. Med. Genet. A 2008, 146A, 1886–1889. [Google Scholar] [CrossRef]
  37. Tan, T.Y.; Collins, A.; James, P.; McGillivray, G.; Stark, Z.; Gordon, C.T.; Leventer, R.J.; Pope, K.; Forbes, R.; Crolla, J.A.; et al. Phenotypic variability of distal 22q11.2 copy number abnormalities. Am. J. Med Genet. Part A 2011, 155, 1623–1633. [Google Scholar] [CrossRef]
  38. Timberlake, A.T.; Griffin, C.; Heike, C.L.; Hing, A.V.; Cunningham, M.L.; Chitayat, D.; Davis, M.R.; Doust, S.J.; Drake, A.F.; Duenas-Roque, M.M.; et al. Haploinsufficiency of SF3B2 causes craniofacial microsomia. Nat. Commun. 2021, 12, 4680. [Google Scholar] [CrossRef]
  39. Wang, Y.; Ping, L.; Luan, X.; Chen, Y.; Fan, X.; Li, L.; Liu, Y.; Wang, P.; Zhang, S.; Zhang, B.; et al. A Mutation in VWA1, Encoding von Willebrand Factor A Domain-Containing Protein 1, Is Associated with Hemifacial Microsomia. Front. Cell Dev. Biol. 2020, 8, 571004. [Google Scholar] [CrossRef]
  40. Su, P.-H.; Liu, Y.-F.; Yu, J.-S.; Chen, J.-Y.; Chen, S.-J.; Lai, Y.-J. Facial asymmetry and clinical manifestations in patients with novel insertion of theTCOF1gene. Clin. Genet. 2012, 82, 460–465. [Google Scholar] [CrossRef]
  41. Naora, H.; Kimura, M.; Otani, H.; Yokoyama, M.; Koizumi, T.; Katsuki, M.; Tanaka, O. Transgenic Mouse Model of Hemifacial Microsomia: Cloning and Characterization of Insertional Mutation Region on Chromosome 10. Genomics 1994, 23, 515–519. [Google Scholar] [CrossRef] [PubMed]
  42. Cousley, R.; Naora, H.; Yokoyama, M.; Kimura, M.; Otani, H. Validity of theHfmTransgenic Mouse as a Model for Hemifacial Microsomia. Cleft Palate-Craniofac. J. 2002, 39, 81–92. [Google Scholar] [CrossRef] [PubMed]
  43. Gendron-Maguire, M.; Mallo, M.; Zhang, M.; Gridley, T. Hoxa-2 mutant mice exhibit homeotic transformation of skeletal elements derived from cranial neural crest. Cell 1993, 75, 1317–1331. [Google Scholar] [CrossRef]
  44. Santagati, F.; Minoux, M.; Ren, S.-Y.; Rijli, F.M. Temporal requirement of Hoxa2 in cranial neural crest skeletal morphogenesis. Development 2005, 132, 4927–4936. [Google Scholar] [CrossRef] [PubMed]
  45. Quiat, D.; Timberlake, A.T.; Curran, J.J.; Cunningham, M.L.; McDonough, B.; Artunduaga, M.A.; DePalma, S.R.; Duenas-Roque, M.M.; Gorham, J.M.; Gustafson, J.A.; et al. Damaging variants in FOXI3 cause microtia and craniofacial microsomia. Genet Med. 2023, 25, 143–150. [Google Scholar] [CrossRef]
  46. Luquetti, D.V.; Heike, C.L.; Zarante, I.; Timms, A.E.; Gustafson, J.; Pachajoa, H.; Porras-Hurtado, G.L.; Ayala-Ramirez, P.; Duenas-Roque, M.M.; Jimenez, N.; et al. MYT1 role in the microtia-craniofacial microsomia spectrum. Mol. Genet. Genomic Med. 2020, 8, e1401. [Google Scholar] [CrossRef]
  47. Poswillo, D. The pathogenesis of the first and second branchial arch syndrome. Oral Surg. Oral Med. Oral Pathol. 1973, 35, 302–328. [Google Scholar] [CrossRef]
Bioengineering 10 00595 g001 550
Figure 1. Definition of mandible classification in OMENS+ system. (a): small mandible and glenoid fossa with short ramus, defined as M1. (b): abnormally shaped and short ramus (glenoid fossa in acceptable position). (c): abnormally shaped and short ramus (glenoid fossa is inferiorly, medially, and anteriorly displaced with severe hypoplasia of condyle), defined as M2b; (d): absence of ramus and glenoid fossa, defined as M3.
Figure 1. Definition of mandible classification in OMENS+ system. (a): small mandible and glenoid fossa with short ramus, defined as M1. (b): abnormally shaped and short ramus (glenoid fossa in acceptable position). (c): abnormally shaped and short ramus (glenoid fossa is inferiorly, medially, and anteriorly displaced with severe hypoplasia of condyle), defined as M2b; (d): absence of ramus and glenoid fossa, defined as M3.
Bioengineering 10 00595 g001
Bioengineering 10 00595 g002 550
Figure 2. Normal disc size and shape. Defined as D0. The TMJ disc is marked in dotted white line.
Figure 2. Normal disc size and shape. Defined as D0. The TMJ disc is marked in dotted white line.
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Bioengineering 10 00595 g003 550
Figure 3. Disc malformation with adequate length to cover the (reconstructed) condyle. Defined as D1.
Figure 3. Disc malformation with adequate length to cover the (reconstructed) condyle. Defined as D1.
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Bioengineering 10 00595 g004 550
Figure 4. Disc malformation with inadequate length to cover the (reconstructed) condyle. Defined as D2.
Figure 4. Disc malformation with inadequate length to cover the (reconstructed) condyle. Defined as D2.
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Bioengineering 10 00595 g005 550
Figure 5. No obvious presence of disc. Defined as D3.
Figure 5. No obvious presence of disc. Defined as D3.
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Table
Table 1. Description of OMENS+ system [1,5,9,15,16,17].
Table 1. Description of OMENS+ system [1,5,9,15,16,17].
O (Orbit)
O0normal orbital size and position
O1abnormal orbital size
O2↓inferior orbital displacement
O2↑superior orbital displacement
O3abnormal orbital size and position
M (Mandible)
M0normal mandible
M1small mandible and glenoid fossa with short ramus
M2aabnormally shaped and short ramus (glenoid fossa in acceptable position)
M2babnormally shaped and short ramus (glenoid fossa is inferiorly, medially, and anteriorly displaced with severe hypoplasia of condyle)
M3absence of ramus and glenoid fossa
E (Ear)
E0normal auricle
E1mild hypoplasia and cupping with presence of all structures
E2absence of external canal with variable hypoplasia of concha
E3malpositioned lobule with absent auricle; lobular remnant typically inferiorly and anteriorly displaced
N (Nerve)
N0no facial nerve involvement
N1temporal and/or zygomatic branch involvement
N2buccal and/or mandibular and/or cervical branch involvement
N3all branches affected
S (Soft tissue)
S0no soft tissue deficiency
S1minimal soft tissue deficiency
S2moderate soft tissue deficiency (between S1 and S3)
S3severe soft tissue deficiency
C (Macrostomia/Cleft)
C0no cleft
C1cleft terminates medially to anterior border of masseter
C2cleft terminates laterally to anterior border of masseter
Table
Table 2. Distribution of samples in OMENS+C classifications and disc classification.
Table 2. Distribution of samples in OMENS+C classifications and disc classification.
Orbitn%
O03532.4%
O12422.2%
O23330.6%
O31614.8%
Total108
Mandiblen%
M11513.9%
M2a3532.4%
M2b4541.7%
M31312.0%
Total108
Earn%
E03633.3%
E11211.1%
E22725.0%
E33330.6%
Total108
Nerven%
N08477.8%
N1109.3%
N21110.2%
N332.8%
Total108
Soft tissuen%
S03027.8%
S14138.0%
S23128.7%
S365.6%
Total108
Cleftn%
C07872.2%
C12119.4%
C298.3%
Total108
Discn%
D03128.7%
D11715.7%
D23633.3%
D32422.2%
Total108
Table
Table 3. Correlation between OMENS+C and disc classification.
Table 3. Correlation between OMENS+C and disc classification.
DiscOrbitMandibleEarNerveSoft Tissue
Orbit−0.016
Mandible0.614 **0.099
Ear0.242 *0.1220.137
Nerve−0.0560.106−0.0250.234
Soft tissue0.291 **0.245 *0.332 **0.340 **0.166
Cleft0.320 **−0.0120.332 **−0.0320.0360.13
* stands for p < 0.05, ** stands for p < 0.01.
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Xue, X.; Liu, Z.; Wei, H.; Wang, X. A Proposal for the Classification of Temporomandibular Joint Disc Deformity in Hemifacial Microsomia. Bioengineering 2023, 10, 595. https://doi.org/10.3390/bioengineering10050595

AMA Style

Xue X, Liu Z, Wei H, Wang X. A Proposal for the Classification of Temporomandibular Joint Disc Deformity in Hemifacial Microsomia. Bioengineering. 2023; 10(5):595. https://doi.org/10.3390/bioengineering10050595

Chicago/Turabian Style

Xue, Xiaochen, Zhixu Liu, Hongpu Wei, and Xudong Wang. 2023. "A Proposal for the Classification of Temporomandibular Joint Disc Deformity in Hemifacial Microsomia" Bioengineering 10, no. 5: 595. https://doi.org/10.3390/bioengineering10050595

APA Style

Xue, X., Liu, Z., Wei, H., & Wang, X. (2023). A Proposal for the Classification of Temporomandibular Joint Disc Deformity in Hemifacial Microsomia. Bioengineering, 10(5), 595. https://doi.org/10.3390/bioengineering10050595

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