Surgical correction of hemifacial microsomia in the growing child

This is a follow-up study of 20 children who had surgical correction of hemifacial microsomia in an effort to improve facial growth and minimize secondary distortion. In group 1 (skeletal types I and IIA), 10 children underwent elongation and lengthening of the mandible. In group 2 (skeletal types IIB and III), 10 children had total construction of a new temporomandibular joint and mandibular ramus with rib grafts and costochondral junction. In both groups, an open bite was created on the affected side to provide space for tooth eruption and downward growth of the middle face. The mean follow-up was 50.9 months (18 to 117 months) in group 1 and 45 months (18 to 50 months) in group 2. Analysis of our early results showed that all patients have had downward growth of the midface on the affected side. All five patients in group 1, followed to complete closure of the surgically created open bite, have maintained a level occlusal plane. In group 2, the one patient followed to completion of facial growth continues to have a level occlusal plane and facial symmetry. Appropriately timed mandibular construction and/or elongation in children with hemifacial microsomia is safe and effective. There is a decrease in secondary deformity on the affected side, and the eventual overall facial growth is optimized. Some group 2 children will need secondary elongation and augmentation of the mandible. Nevertheless, this study demonstrates that early mandibular correction may obviate the need for maxillary and orbital procedures in adulthood.     

Brief communication: bilateral aplasia of the condyles in a 1,400-year-old mandible from Israel

A rare pathological mandible, manifesting bilateral absence of the condyles, is discussed. The pathology was identified as hemifacial microsomia. The mandible, dated to the Byzantine period in Israel, manifests bilateral aplasia of the condyles and extreme shortness, but normal width, of the body. The extremely well-developed coronoid process, the grooved masseter insertion area, and the manifestation of a medial pterygoid tubercle (MPT) suggest hypertrophy of the occlusal muscles. The presence of a large MPT is considered a Neanderthal autapomorphy. Studying the biomechanic forces acting on the deformed mandible in hemifacial microsomia patients may shed light on the mastication process in Neanderthals.     

Analysis and treatment of hemifacial microsomia

Our experience over the past 25 years leads us to conclude that hemifacial microsomia is a progressive skeletal and soft-tissue deformity with the earliest skeletal manifestations in the mandible. We find that not only does the mandibular asymmetry become worse with time, but as the contralateral side grows, the deformed mandible also increases ipsilateral secondary deformation of the maxilla, nose, and orbit. We also find that there is virtually no "catch up" growth on the affected side of the face and that these patients always become more deformed with age. The psychological problems also increase with time and progression of the facial deformity. Based on our experience with correction of end-stage deformities, we now treat these patients at the earliest possible age, as determined by the patient's skeletal classification. We treat the mandible first in the hope that this repositioning in a more physiologic position will unlock the growth potential of the adjacent structures, minimize secondary deformity, and improve function and appearance to the greater benefit of the skeletal and psychological growth of the patient.     

Hemifacial microsomia: a multisystem classification

Variability of deformities in hemifacial microsomia has precluded the general acceptance of any classification based on one reference organ. We present a review of hemifacial microsomia classifications and propose a TNM-style multisystem classification. This alphanumeric coding system, SAT, provides cohesion to existing hemifacial microsomia classifications. The acronym SAT is derived as follows: S = skeletal, A = auricle, and T = soft tissue. There are five levels of skeletal deformity (S1 through S5), four levels of auricular deformity (A0 through A3), and three levels of soft-tissue deformity (T1 through T3). Hence a patient with minimal deformity would be classified S1A0T1, whereas a patient with the most severe deformity would be S5A3T3.     

Surgical treatment of hemifacial microsomia. (First and second branchial arch syndrome).

A review of 17 patients operated upon for unilateral hemifacial microsomia, and followed for up to 18 years, clarifies several principles of management. The management is facilitated by dysplasia predominance grouping, with the major functional deformity dictating the sequence of repair. Our surgical goals are: (1) provision of adequate upper airway; (2) preservation of normal teeth and adequate alveolar bone to achieve proper dental occlusion; (3) restoration of binaural hearing; (4) external ear construction; (5) acceptable facial symmetry; (6) balanced facial animation; and (7) a child with an acceptable self-image.     

Neurovascular free-muscle transfer to treat facial paralysis associated with hemifacial microsomia

Despite the wide spectrum of hemifacial microsomia manifestations, treatment mainly focuses on mandible and ear abnormalities, rather than on facial paralysis. In fact, the surgical treatment of facial paralysis associated with hemifacial microsomia is quite underdeveloped, because the degree of paralysis is frequently incomplete or partial. Timing and type of surgery are also difficult to determine. Neurovascular free-muscle transfer is now a standard procedure for the dynamic smile reconstruction of longstanding facial paralysis. This type of strategy has considerable potential in the treatment of facial paralysis in patients with hemifacial microsomia. We present here our experience with neurovascular free-muscle transfer for smile reconstruction in eight patients with facial paralysis associated with hemifacial microsomia. The age of the patients at the time of surgery ranged from 7 to 28 years old, (average, 13.9 years). Six were male patients and two were female patients. The two-stage method combining gracilis muscle transfer with cross-face nerve grafting was performed in three patients, whereas the one-stage transfer of the latissimus dorsi muscle was performed in five. To construct a natural or near-natural smile, the muscles were transferred into the paralyzed cheek in all except one patient, in whom the latissimus dorsi muscle was transferred into the sublabial area to reconstruct a paralyzed lower lip. A dermal flap segment vascularized with perforating vessels from the latissimus dorsi muscle was simultaneously inserted into the underdeveloped cheek for soft-tissue augmentation in this patient. Muscle contraction was evident in all patients between 4 and 8 months after muscle transfer. Our present series revealed that neurovascular free-muscle transfer is a good option not only for smile reconstruction but also for restoration of the facial contours of patients with hemifacial microsomia. Compared with the two-stage method combining gracilis muscle transfer with cross-face nerve grafting, the one-stage method using the latissimus dorsi muscle has some advantages, including a one-stage operation, a shorter recovery period, and the absence of sequelae that occur after harvesting a sural nerve.     

Hemifacial microsomia: use of the OMENS-Plus classification at the Royal Children's Hospital of Melbourne

Hemifacial microsomia is the most common facial congenital disability after cleft lip and palate, but as yet its pathogenesis remains unknown. Clinical classification systems have evolved over the last 30 years from those classifying only single components of the disorder, to those classifying according to the combination of deformities, to the most recent systems that grade each anatomical component separately, such as the Orbit, Mandible, Ear, Nerve, and Soft tissue (OMENS) system. The aim of the present study was to review the classification of patients with hemifacial microsomia treated by the Melbourne Craniofacial Unit at the Royal Children's Hospital using the OMENS-Plus system of classification and to correlate the findings with data from other centers. Records of patients treated by the craniofacial unit were reviewed and included in the study if adequate clinical records, photographs, and radiographs (anteroposterior, lateral, basal cephalometry, panoramic views) were available. The data were entered into a database file developed for this purpose. Seventy-one patients were identified from the hospital database, of which six were excluded because of incomplete data. Of the 65 patients, there were 31 (48 percent) with right-sided microsomia, 25 (38 percent) with left-sided microsomia, and nine (14 percent) with bilateral microsomia, with an overall male-to-female ratio of 1.2:1. The majority of patients had a normal orbit (77 percent), mildly hypoplastic mandibular ramus-condyle with functioning temporomandibular joint (57 percent with type M1 or M2a), normal facial nerve (76 percent), and mild soft-tissue hypoplasia (73 percent). There was a similar proportion of patients with mild ear anomalies (53 percent with grade 0 or 1) compared with those with more severe anomalies (47 percent with grade 2 or 3). Correlative analysis demonstrated a slight but positive correlation between the severity gradings of the five individual components. The correlation was lowest between the grading of the nerve and ear and that of the mandible and nerve. The data demonstrate the phenotypic variability of hemifacial microsomia and suggest a degree of relationship among the components of hemifacial microsomia. The OMENS-Plus system has provided a major advancement in the classification of hemifacial microsomia. The authors suggest refinements to the grading of the orbit and nerve components.     

Thrombophilia gene mutations in oculoauriculovertebral spectrum

Oculoauriculovertebral spectrum or Goldenhar syndrome is a phenotypically and probably genetically heterogeneous disorder characterized by hemifacial microsomia (unilateral ear abnormalities and ipsilateral mandibular hypoplasia) as well as vertebral anomalies and epibulbar dermoid/lipodermoids. Although most cases of the Goldenhar syndrome are sporadic, both autosomal recessive and dominant inheritance have been reported so far. In this report, we describe the clinical aspects of two familial cases with evidence of autosomal dominant inheritance and a non-familial case, and compare them with the reports in the literature. One of our familial cases was a ten day old female infant of a mother with left hemifacial microsomia. She had multiple bilateral preauricular tags and a "fleshy masse" on her right cheek. The other familial case was a two months old male infant whose father had hemifacial asymmetry. He had unilateral microtia, and abnormal antihelix, a skin tag in the contralateral ear associated with bilateral sensorineural hearing loss. The third case was a sporadic case who was 2 years old boy with preauricular skin tag, right hemifacial microsomia and limbal dermoid at the temporal limbus of the right eye. As there were no other associated defects, the cases we presented here were thought to be mild variations of the Goldenhar spectrum. The most commonly encountered mutations of thrombophilia genes were studied. We believe that the interfamilial and intrafamilial clinical variabilities observed in these cases reinforce the necessity of a careful examination for the whole family with regards to the stigmata of Goldenhar syndrome.     

Surgical management of the anophthalmic orbit, part 1: congenital

Congenital microphthalmos and anophthalmos are rare conditions in which orbital growth is deficient. Hypoplasia of the globe affects the bony orbit (micro-orbitism), the conjunctival sac, and eyelids (microblepharism), and it may be associated with abnormalities of the entire hemifacial skeleton (hemifacial microsomia). In the present article, the authors review a series of 19 patients with microphthalmos (nine had right-sided, one had bilateral, and nine had left-sided microphthalmos) who were treated in the Orbitopalpebral Unit at Hospital Foch over a period of 15 years (follow-up, 5 months to 18 years).Orbital expansion was achieved using spherical implants (n = 13), orbital osteotomies (n = 4), and orbital expanders (n = 2). Both expanders were removed within 6 months because of failure (one infection and one rupture). The current preferred method for orbital expansion is to use serial implants in the growing orbit and osteotomies in cases of late referral or insufficient orbital volume in the older child. The target proportions of the reconstructed orbit are not planned to mirror the healthy side exactly. The inferior orbital rim is kept higher to support the orbital implant, and the orbit is kept shallow to avoid a sunken appearance.Cranial bone grafts were used to augment deficient orbital contours; they were assisted by anterior transposition of the temporalis muscle (n = 5) when additional orbital volume was required. Conjunctival sac reconstruction was achieved by the use of serial conformers placed in the conjunctival sac during the neonatal period, followed by grafts of buccal mucosa and full-thickness skin maintained in place with a tarsorrhaphy for 3 to 6 months. Eyelid reconstruction using local flaps and skin grafts proved to be necessary in cases treated by osteotomy expansion, although reconstruction was not required after expansion using serial solid shapes. The results illustrate an evolution in approach and concepts of reconstruction of the microphthalmic orbit and emphasize the need for an integrated craniofacial approach for this complex deformity.     

Maxillary changes and occlusal traits in crania with artificial fronto-occipital deformation

Artificial fronto-occipital deformation of the cranial vault was typical of pre-Columbian cultures in the central Andean coastal regions. We have studied the influence of this deformation on maxillary and mandibular morphology. Measurements were performed on 86 adult Ancon skulls with anteroposterior deformation. Undeformed skulls from the area of Makatampu (n = 52) were used as the control group. To explore the influence of the deformity on occlusion, the skulls were categorized using the Angle classification and the alignment of the interincisor midline. In the group of deformed skulls, there was an increase in lateral growth of the vault and of the base of the skull (P < 0.001), giving rise to a greater interpterygoid width of the maxilla (P < 0.001), and an increase in the transverse diameter of the palatal vault. The mandible presented an increase in the length of the rami (P < 0.001) and in the intercondylar width, with no alteration of mandibular length. The deformed skulls had normal (class I) occlusion, with no displacement of the midline. The difference in the asymmetry index between the two groups was not statistically significant. Artificial fronto-occipital deformation of the cranial vault provoked compensatory lateral expansion of the base that was correlated with the transverse development of the maxilla and mandible. Occlusion and sagittal intermaxillary position were not affected by the cranial deformity. These results provide evidence of the integration between the neurocranium and the viscerocranium in craniofacial development, and support the hypothesis of a compensatory effect of function.     

Multiple-segment osteotomy in maxillofacial surgery

Multiple-segment osteotomy is defined as an osteotomy that divides the tooth-bearing arch of the maxilla or mandible into three or more segments. Combining large-segment orthognathic surgery and unitooth or small-segment surgery is an effective approach for dealing with a wide range of dentofacial deformities with occlusal problems. The indications for a multiple-segment osteotomy included dentofacial deformities and malocclusions requiring stable correction within a short overall treatment period. From 1991 to 1997, a total of 85 patients had multiple-segment osteotomy orthognathic procedures performed at Chang Gung Memorial Hospital. The indications for surgery were maxillary protrusion/deformity (31 patients), mandibular prognathism (51 patients), and noncleft maxillary retrusion (three patients). The types of osteotomies performed were Le Fort I, anterior segmental osteotomies of the maxilla or the mandible, palatal split, posterior segment, and unitooth or double-tooth segments. Follow-up ranged from 6 months to 7 years; stability was seen in movements, with only three complications (one partial gingival loss and two inferior mental paresthesias). No osteotomized segments were lost. The average overall treatment time was approximately 15 months, including 3 to 6 months of preoperative and 9 to 12 months of postoperative orthodontic treatment. This is at least 6 months shorter than traditional orthognathic surgery. Experience with 85 consecutive patients has shown that the results are good and the procedure is safe, with minimal complications.     

Formation of anterior maxillary teeth in 0-3-year-old children with cleft lip and palate and prenatal risk factors for delayed development

Formation of deciduous and permanent maxillary incisors and canines was studied in 361 children with cleft lip and palate. Altogether, 704 occlusal X rays of the children aged from 2 weeks to 41 months were assessed using a 13-stage scale. The early timing of tooth development was in both dentitions close to that reported for healthy children. However, developmental asymmetry was noted between contralateral teeth on the cleft and noncleft sides. In the unilateral cleft lip and palate group, every tenth cleft-side deciduous central incisor, deciduous canine, and permanent central incisor was delayed compared to the corresponding contralateral tooth. The formation timing was also compared within 11 prenatal and hereditary variables. Risk factors for delayed development were low placental weight, short gestation, mother's use of drugs during pregnancy, first in birth order, cleft with multiple additional malformations, and relatives having malformations other than oral clefts. However, the associations between a risk factor and tooth development were weak.     

The Effects of Maxillary Protraction with or without Rapid Maxillary Expansion and Age Factors in Treating Class III Malocclusion: A Meta-Analysis

We conducted a comprehensive meta-analysis of 12 studies to examine whether maxillary protraction face mask associated with rapid maxillary expansion (FM/RME) could be an effective treatment for Class III malocclusion and to evaluate the effect of timing on treatment response. Patients with a maxillary deficiency who were treated with FM with or without RME were compared with those who had an untreated Class III malocclusion. In both treatment groups, forward displacement of the maxilla and skeletal changes were found to be statistically significant. In addition, posterior rotation of the mandible and increased facial height were more evident in the FM group compared with the control group. However, no significant differences were observed between the early treatment groups and late treatment groups. The results indicated that both FM/RME and FM therapy produced favorable skeletal changes for correcting anterior crossbite, and the curative time was not affected by the presence of deciduous teeth, early mixed dentition or late mixed dentition in the patient.     

Craniofacial changes in hemifacial microsomia

Cephalometry, X-ray cephalometry, and somatoscopy were used in the studies of 65 adult males with a severe unilateral microtia subdivided into three groups: with marked asymmetry, with slight asymmetry, and without any obvious facial asymmetry. The group with marked asymmetries was designated as hemifacial microsomia. In this group the affected side of the face was depressed on the average from above and from below towards the level of the external auditory canal. The center of the anteroposterior reduction was situated in the region in front of the pterygomaxillar fissure. The anteroposterior and vertical facial dimensions on the affected side were reduced most markedly, while the width dimensions showed the slightest changes. Hypoplasia was most severe within the lower face and increased towards the otocephalic centre. The mandibular joint was displaced in an anteroinferior and medial direction. Hypertelorism did not occur, but the orbit on the affected side was smaller in height and was frequently vertically dislocated. The facial profile was unchanged except for retrusion of the chin and increased frequency of bite disorders. The mobility of the mandible was limited. Hemihypoplasia also exerted an influence on structures that were not of branchiogenic origin, e.g., the cranial base (narrowing, asymmetry, and more pronounced curvature), the neurocranium (depression in mastoid and tympanotemporal regions, posterior rotation of the vault), and the frontonasal component (deviation of the nose and premaxilla). The cranial vault and the bottom of the occipital bone showed on the average no asymmetries. The similar character of deviations in slightly affected groups revealed that in spite of the high variability of changes typical for branchiogenic malformations the development of the face in these defects was subjected to certain rules. Marked facial asymmetry occurred only in every fifth patient with a severe degree of microtia, while definite signs of asymmetry were absent in every third patient.     

Correction of severe facial deformity.

A craniofacial team has been developed to corrdinate the treatment of patients with severe facial deformity and to minimize the surgical risks. Two hundred patients have been evaluated in the last 4 years and more than 100 treated by this team to correct orbital hypertelorism, oxycephaly and plagiocephaly with exophthalmos, features of Crouzon''s disease and Apert''s and Treacher Collins syndromes, hemifacial microsomia, and severe lower facial deformity and malocclusion. Surgical principles include extensive subperiosteal stripping of bone, osteotomy as necessary, and repositioning maintained by bone grafts. Postoperative evaluation is being maintained for 5 years or to maturity in younger children. Although many of the results are less than perfect, there has been sufficient improvement, especially psychologic and functional, to warrant continuation of the surgical program under closely controlled conditions.     

Roentgencephalometric measurements in trisomy 8 mosaicism: report of three cases

Roentgencephalometric anomalies in three cases of Warkany syndrome (trisomy 8 mosaicism) are described. These include asymmetry of the mandible with a wide gonial angle and a high and narrow symphysis; SNA (anteroposterior position of maxilla) and SNB (anteroposterior position of mandible) values indicate a backward position of the mandible. Other findings point to a disturbance in the vertical growth of the facial skeleton. These measurements may explain at least part of the facial phenotype and may aid in diagnosis, especially in those cases with an uncertain clinical diagnosis and "normal" karyotype in peripheral blood lymphocytes.     

Canine role in dental wear patterns: Macaca nemestrina

A study of variables and patterns in dental wear among 30 individuals in a colony of Macaca nemestrina shows that consideration of age and sex are crucial for understanding differential wear degrees on molar occlusal planes. With advanced age, this species of non-human primate undergoes obliteration of initial morphological characteristics through the gradual erosion of enamel. Wear gradients are differential from PM1-M3 in both sexes. It appears that there is a functional relationship between degrees of occlusal plane wear and the degree of wear on the canines, and that females show a greater degree of wear changes relative to males of equivalent age because of initial differences in canine length and robusticity due to sexual dimorphism. It appears that there is a direct relationship between occlusal wear plane changes and the degree of wear on the canines, with advanced differential wear showing up in individuals in whom years of maxillary canine honing, canine damage, and the normal wear of mastication has reduced dimensions of unworn permanent canines. Other considerations included in this study are the honing functions of the deciduous first mandibular molars and molar cusp height relative to canine function.     

The influence of incompetent lip seal on the growth and development of craniofacial complex

Abnormal orofacial functions in the period of growth and development can cause morphological anomalies of the craniofacial complex. The aim of this study was to determine the correlation between open mouth posture and morphology of craniofacial complex. The shape, size and relationships of skeletal parts of craniofacial complex were determined by analysis of lateral cephalograms in the sample of 84 children--45 girls and 39 boys (aged 8.96 +/- 0.66 years). The sample was divided into two groups--lip competence and lip incompetence group. Differences in cephalometric values between observed groups were found. The values of inclination of lower central incisors (angle ILi/NB), interbasal angle (NL/NSL), angle between occlusal and mandibular plane and anterior lower facial height were significantly higher in the group with open mouth posture. It can be concluded that lip incompetence plays an important role in growth and development of craniofacial complex.     

Mandibular growth after distraction in patients under 48 months of age

Distraction osteogenesis is an effective technique for reconstruction of the congenitally deficient mandible. However, the age at which it is best performed remains under discussion. Distraction performed at an early age, while possibly allowing the face to develop with a more normal functional matrix, may entail a higher rate of complications. Additionally, it is possible that subsequent asymmetric growth of the mandible may necessitate serial distraction. To address this issue, the clinical records and cephalometric radiographs of all patients less than 48 months of age undergoing mandibular distraction at New York University Medical Center between August of 1989 and August of 1997 were examined. There was a total of 14 patients ranging in age from 19 months to 43 months. Nine patients had a diagnosis of unilateral craniofacial microsomia, three had Treacher Collins syndrome, one had Nager syndrome, and one had bilateral developmental micrognathia. The average amount of distraction was 27 mm (range, 23 to 39 mm) in unilateral cases and 24 mm in bilateral cases (range, 15 to 31 mm). The period of clinical follow-up averaged 32.6 months (range, 12 to 92 months). All patients showed significant improvement in craniofacial appearance, and in four patients, long-term tracheostomy tubes were removed. There were two major complications. In one patient with craniofacial microsomia, there was a relapse in the early postretention phase related to the presence of a dentigerous cyst. This required removal of the cyst and repeat distraction. In the patient with Nager syndrome, a coronoid ankylosis developed requiring surgical release. There were no other major complications. The scars required revision in only two of the patients. Cephalometric analysis of the patients in the study revealed a differential in the rate of growth between the affected and the unaffected side in all cases of craniofacial microsomia. The affected side always grew at a slower rate than the contralateral side after the distraction process was complete. This led to a progressive asymmetry of the rami, clinically expressed by some degree of facial asymmetry and an occlusal cant. For this reason, secondary distraction was required in one patient and is planned in a second. Initial overcorrection of the patient would seem to minimize the likelihood that secondary distraction will be necessary. Distraction osteogenesis for reconstruction of the mandible in this subset of young patients was a safe and effective technique for improving the craniofacial skeletal form and appearance, with minimal associated morbidity. Longer follow-up is necessary to assess the full impact of growth in these cases.