The oral manifestations of Apert syndrome.

As part of an ongoing study of 119 patients with the Apert syndrome, extensive data were available for the analysis of oral manifestations, including mouth shape, lip posture, palatal morphology, dental anomalies, and malocclusion. Findings included a characteristic trapezoidal-shaped mouth. Cleft soft palate or bifid uvula was found in approximately 75%. A Byzantine-arch shaped palate was recorded in almost all patients. Dental anomalies included severely delayed eruption, ectopic eruption, and shovel-shaped incisors. Malocclusion tended to be severe with mesial molar occlusion, mandibular overjet, anterior and posterior crossbites, and severe crowding of teeth. The oral manifestations of Apert syndrome are compared and contrasted with those of Crouzon syndrome.     

Lip-nasal aesthetics following Le Fort I osteotomy

Forty-one patients undergoing Le Fort I osteotomy for superior and/or anterior repositioning of the maxilla were prospectively studied for changes in soft-tissue morphology of the nasomaxillary region. Nasal parameters studied were changes in interalar rim width and nasal tip projection. It was observed that alar rim width increases with anterior and/or superior repositioning of the maxilla, but increases in nasal tip projection occur only when there is an anterior vector of maxillary movement. These nasal changes could not be quantitatively correlated to magnitude of maxillary movement. Lip changes studied were the horizontal displacement at the vermilion border and subnasale versus that of the incisal edge and point A, respectively, when the maxilla is sagittally advanced and the vertical shortening of the lip versus that of the incisal edge when the maxilla is shortened. Using linear regression analysis, horizontal displacement of the upper lip at the vermilion border was 0.82 +/- 0.13 mm for every 1 mm of maxillary advancement at the incisal edge (p less than 0.001) and 0.51 +/- 0.13 at the subnasale for every 1 mm of maxillary advancement at point A (p less than 0.001). Eighty percent of patients undergoing maxillary intrusive procedures had lip shortening ranging from 20 to 50 percent of the vertical maxillary reduction. Surprisingly, no statistically significant correlation could be demonstrated for lip shortening versus extent of vertical maxillary reduction. Previous literature in disagreement with these findings is discussed. Guidelines for treatment planning utilizing these data are suggested.     

A model for fetal cleft lip repair in lambs.

Fetal wounds heal without inflammation and scar formation. This phenomenon may, in the future, be applicable to human cleft lip and palate repair. However, extensive experimental work must first be done to document the benefits of in utero repair. We developed a large animal model for creation and repair of a complete cleft lip and alveolus using fetal lambs. The cleft lip and alveolus deformity was created in eight 75-day-gestation fetuses (term = 145 days) and either repaired in three layers or left unrepaired. There were four sham-operated fetuses, and all animals were alive at harvest. Repaired, unrepaired, and control fetuses were harvested at 7, 14, 21, and 70 days following surgery. The unrepaired fetuses demonstrated a complete cleft lip and alveolus with an oronasal fistula. The maxilla was asymmetrical, with the greater segment deviated toward the cleft and with decreased anterior maxillary width. In contrast, repaired cleft lip and alveolus animals showed no scar, normal thickness of the lip, and a symmetrical maxilla. Histologic analysis of the repaired wounds showed evidence of tissue regeneration without scar formation. The results of this preliminary study indicate that the fetal lamb cleft lip and alveolus model is technically feasible with an excellent survival rate. Healing occurs without scar formation. In the repaired animals, the maxilla was symmetrical. This model will be used to document facial growth following in utero repair of a cleft lip and alveolus.     

Improved primary surgical and dental treatment of clefts

The improved combination of surgical and dental teamwork in the primary treatment of clefts presented here is consistent with principles. In fact, this is a staged design for correction of classic clefts of the lip and palate that, based on biological principles, facilitates the continuance of the failed embryonic "migrations" toward a normal end point. Positioning of the alveolar segments, dissection of mucoperiosteum out of the cleft, and union of mucoperiosteum across the alveolar and anterior hard palate cleft make it possible to create a periosteal tunnel across the bony gap and set up a condition conducive to bone formation and eventual tooth eruption in the cleft area. Lip closure by adhesion reduces the tension of the primary lip closure and allows gentle molding until solidification of the arch occurs. Thus a complete cleft has been rendered an incomplete cleft. With a balanced, stabilized maxillary platform, the definitive lip and nose corrections can be carried to completion early (by 2 to 4 years of age). These planned actions bypass a persistent cleft, fistulas, raw areas, malposition of alveolar segments, and probably the necessity for later bone grafting. The only question not totally answered is the effect of this approach on final growth. Although most reports seem to indicate that growth has and will proceed within normal limits, another 10 years of careful follow-up is indicated and, in fact, is in progress.     

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.     

Binderoid complete cleft lip/palate

A small subset of infants with complete cleft lip/palate look different because they have nasolabiomaxillary hypoplasia and orbital hypotelorism. The authors' purpose was to define the clinical and radiographic features of these patients and to comment on operative management, classification, and terminology. The authors reviewed 695 patients with all forms of incomplete and complete cleft lip/palate and identified 15 patients with nasolabiomaxillary hypoplasia and orbital hypotelorism. All 15 patients had complete labial clefting (5 percent of 320 patients with complete cleft lip/palate), equally divided between bilateral and unilateral forms. The female-to-male ratio was 2:1. Of the seven infants with unilateral complete cleft lip/palate, one had an intact secondary palate and all had a hypoplastic septum, small alar cartilages, narrow basilar columella, underdeveloped contralateral philtral ridge, ill-defined Cupid's bow, thin vermilion-mucosa on both sides of the cleft, and a diminutive premaxilla. Of the eight infants with bilateral complete cleft lip, one had an intact secondary palate. The features were the same as in patients with unilateral cleft, but with a more severely hypoplastic nasal tip, conical columella, tiny prolabium, underdeveloped lateral labial elements, and small/mobile premaxilla. Central midfacial hypoplasia and hypotelorism did not change during childhood and adolescence. Intermedial canthal measurements remained 1.5 SD below normal age-matched controls. Skeletal analysis (mean age, 10 years; range, 4 months to 19 years) documented maxillary retrusion (mean sagittal maxillomandibular discrepancy, 13.7 mm; range, 3 to 17 mm), absent anterior nasal spine, and a class III relationship. The mean sella nasion A point (S-N-A) angle of 74 degrees (range, 65 to 79 degrees) and sella nasion B point (S-N-B) angle of 81 degrees (range, 71 to 90 degrees) were significantly different from age-matched norms ( = 0.0007 and = 0.004, respectively). The ipsilateral central and lateral incisors were absent in all children with unilateral cleft, whereas a single-toothed premaxilla was typically found in the bilateral patients. Several modifications were necessary during primary nasolabial repair because of the diminutive bony and soft-tissue elements. All adolescent patients had Le Fort I maxillary advancement and construction of an adult nasal framework with costochondral or cranial graft. Other often-used procedures were bony augmentation of the anterior maxilla; cartilage grafts to the nasal tip and columella; and dermal grafting to the median tubercle, philtral ridge, and basal columella. Infants with complete unilateral or bilateral cleft lip/palate in association with nasolabiomaxillary hypoplasia and orbital hypotelorism do not belong on the holoprosencephalic spectrum because they have normal head circumference, stature, and intelligence, nor should they be referred to as having Binder anomaly. The authors propose the term cleft lip/palate for these children. Early recognition of this entity is important for counseling parents and because alterations in standard operative methods and orthodontic protocols are necessary.     

中华鲎虫外部形态结构的初步观察

中华鲎虫体由头、躯干和尾3部分组成.头部具1对复眼和1个无节幼体眼,复眼后有1个颈感器.第1触角细小、棒状,第2触角退化.口器由上唇、大颚和2对小颚组成,上唇为头部的突起物,大颚和2对小颚为附肢,第2小颚的皮肤皱褶延长扩大形成头胸甲.雌体躯干部由36个体节组成,雄体39个体节,前30节具附肢,共57对.雌体躯干部后6个体节无附肢,雄体躯干部后9个体节无附肢.尾节分叉,细长多节,具刚毛,左右两根尾鞭的分节不对称.     

The "levator septi nasi muscle" and its clinical significance

It is strange that all textbooks of anatomy describe the depressor septi nasi muscle singly, without an antagonist. Incidentally, in 1986, a small rod of soft tissue was found between the medial crura of the two alar cartilages during a rhinoplastic operation with the external approach technique of Anderson and Ries. From 1990 through 1995, anatomic dissections of the nasolabial region under 3.5x loupe magnification were performed on 14 Chinese formalin-preserved cadavers, one fresh Chinese cadaver, and one fresh American white female cadaver. The small soft-tissue rod was found in every one of the dissected cadavers, and it was seen to be a pair of muscles. Each one of these paired small muscles arose from the aponeurosis on the dorsum of the nose and inserted into the muscular substance of the upper lip at the base of the columella and to the anterior spine of the maxilla. Histologic examinations of these muscles stained with hematoxylin and eosin and Masson trichrome showed that they were striated muscles. According to its origin and insertion, this newly found muscle was called the "levator septi nasi." Its clinical significance in cleft lip deformity and its relations to the orbicularis oris muscle, the dermocartilaginous ligament of Pitanguy, and the nasal superficial musculoaponeurotic system of Letourneau and Daniel are all discussed.     

Peripheral calcifying cystic odontogenic tumour of the maxillary gingiva

ABSTRACT: BACKGROUND: Odontogenic tumors are lesions that are derived from remnants of the components of the developing tooth germ. The calcifying cystic odontogenic tumor or calcifying odontogenic cyst is a benign cystic neoplasm of odontogenic origin that is characterized by an ameloblastoma-like epithelium. Calcifying cystic odontogenic tumor may be centrally or peripherally located, and its ghost cells may exhibit calcification, as first described by Gorlin in 1962. Most peripheral calcifying cystic odontogenic tumors are located in the anterior gingiva of the mandible or maxilla. CASE PRESENTATION: Authors report a rare case of a peripheral calcifying cystic odontogenic tumor of the maxillary gingiva. A 39-year-old male patient presented with a fibrous mass on the attached buccal gingiva of the upper left cuspid teeth. It was 0.7-cm-diameter, painless and it was clinically diagnosed as a peripheral ossifying fibroma. After an excisional biopsy, the diagnosis was peripheric calcifying cystic odontogenic tumor. The patient was monitored for five years following the excision, and no recurrence was detected. CONCLUSIONS: All biopsy material must be sent for histological examination. If the histological examination of gingival lesions with innocuous appearance is not performed, the frequency of peripheral calcifying cystic odontogenic tumor and other peripheral odontogenic tumors may be underestimated.     

Total reconstruction of the "end-stage" cleft lip and palate deformity

The purpose of this paper is to present a 5-year experience using a comprehensive surgical approach to reconstruct what we have chosen to call the "end-stage cleft lip and palate deformity." The deformity consists of varying degrees of midface retrusion, malocclusion, nasal deformity, and lip deformity. Most of the patients afflicted had unacceptable upper lip anatomy characterized by tightness and lack of cupid's bow and bulk. All had severe palatal scarring with resulting arch collapse and severe malocclusion. Most had had multiple surgical attempts to improve nasal aesthetics using standard rhinoplasty techniques with little or no improvement. The procedure involves splitting the upper lip with incisions extending into the upper buccal sulcus and rim of the nose allowing wide skeletalization of the maxilla and osteocartilagenous nasal skeleton. LeFort I or II maxillary advancement, nasal reconstruction, and upper lip modification (with Abbé flap if indicated) are done. The jaws are placed in intermaxillary fixation for 6 to 8 weeks. This comprehensive approach has been used in 16 patients, aged 15 to 29 years, with follow-up of up to 5 years. Excellent functional and aesthetic improvement has occurred in all patients, and complications have been minimal.     

Midfacial duplication: a rare malformation sequence

A child with duplicate maxilla is presented, along with a review of the literature of similar cases. Partial duplications of the maxilla are frequently accompanied by cleft lip and palate, multiple uvulae, hypertelorbitism, and intracranial anomalies with hydrocephalus. Theories of the morphopathogenesis for this malformation sequence are discussed.     

Duplication of stomatodeal structures: report of three cases with literature review and suggestion for classification

Three cases of duplications of stomatodeal structures are reported. One had an accessory mouth that could move simultaneously with his mouth at sternal notch. Another had a teeth-bearing bony mass at left maxilla with excessive upper lip and a false pouch. The third had an excessive upper lip, upper jaw with teeth, and hard and soft palate. The literature is reviewed, and a classification is suggested: type I, a duplicated mouth; type II, duplication of maxilla-upper lip or mandible-lower lip complex; and type III, centrally located, poorly developed lip-jaw duplication.     

Primary reconstruction of complex midfacial defects with combined lip-switch procedures and free flaps.

Free flaps are generally the preferred method for reconstructing large defects of the midface, orbit, and maxilla that include the lip and oral commissure; commissuroplasty is traditionally performed at a second stage. Functional results of the oral sphincter using this reconstructive approach are, however, limited. This article presents a new approach to the reconstruction of massive defects of the lip and midface using a free flap in combination with a lip-switch flap. This was used in 10 patients. One-third to one-half of the upper lip was excised in seven patients, one-third of the lower lip was excised in one patient, and both the upper and lower lips were excised (one-third each) in two patients. All patients had maxillectomies, with or without mandibulectomies, in addition to full-thickness resections of the cheek. A switch flap from the opposite lip was used for reconstruction of the oral commissure and oral sphincter, and a rectus abdominis myocutaneous flap with two or three skin islands was used for reconstruction of the through-and-through defect in the midface. Free flap survival was 100 percent. All patients had good-to-excellent oral competence, and they were discharged without feeding tubes. A majority (80 percent) of the patients had an adequate oral stoma and could eat a soft diet. All patients have a satisfactory postoperative result. Immediate reconstruction of defects using a lip-switch procedure creates an oral sphincter that has excellent function, with good mobility and competence. This is a simple procedure that adds minimal operative time to the free-flap reconstruction and provides the patient with a functional stoma and acceptable appearance. The free flap can be used to reconstruct the soft tissue of the intraoral lining and external skin deficits, but it should not be used to reconstruct the lip.     

Behavioral correlates of tooth eruption in Madagascar lemurs

Observations on the sequence and timing of gingival tooth eruption are reported for six species of Madagascar lemurs. Complete sequences of eruption were obtained for the deciduous dentition, and partial to complete sequences were recorded for the permanent dentition. In Cheirogaleus medius and in four species of the genus Lemur, the deciduous teeth erupt in front-to-back sequence, with the toothcomb emerging near birth as an integrated complex. In Propithecus verreauxi the same pattern is exhibited, but the small peglike lower canine and dp₃ erupt last. Eruption of the permanent dentition in Lemur species takes place in two distinct stages. In the first stage the upper incisors, toothcomb, and first two molars penetrate the gingiva. After an interval of 3 to 4 months, the remaining permanent teeth erupt. Deciduous premolars erupt when young animals are being weaned. The eruption of the deciduous toothcomb appears unrelated to feeding or grooming behavior. In L. catta and L. fulvus, the first stage of permanent tooth eruption occurs at approximately 6 months of age, when the growth rate slows down and (in wild populations) the rainy season is ending. This suggests that eruption of the anterior molars is timed to coincide with a shift from a more frugivorous to a more folivorous dietary regime, which occurs during the dry season. No further tooth eruption occurs until approximately 1 year of age, when the growth rate increases and the rainy season returns for wild populations. Thus, the second wave of permanent tooth eruption in these species again appears linked to changing climatic conditions which lead to a shift in dietary preferences.     

Growth status of children treated for unilateral cleft lip and palate

Cephalometric distances, angles, and proportions were evaluated for 32 children 5 to 8 years of age treated for unilateral cleft lip and palate. The children were age and sex matched with untreated controls with normal skeletal relationships. The unilateral cleft lip and palate sample was treated by the same surgeon and orthodontist using the same techniques and appliances. Measures of overall facial proportions, facial convexity, and prognathism were not significantly different between the two groups. The primary group differences pertain to the posterior aspect of the maxilla, which is vertically short in the unilateral cleft lip and palate sample. Horizontally, the maxilla of the unilateral cleft lip and palate children was significantly longer, producing a steeper palatal plane. In addition, the zygoma and orbits of unilateral cleft lip and palate children were somewhat retruded; the posterior cranial base and total mandibular length also were longer in the unilateral cleft lip and palate children.     

Dental Eruption Sequences in Fossil Colobines and the Evolution of Primate Life Histories

Unlike other catarrhines, colobines show early molar eruption relative to that of the anterior dentition. The pattern is variable, with Asian genera (Presbytina) showing a greater variability than the African genera (Colobina). The polarity of early relative molar eruption, as well as the degree to which it is related to phylogeny, are unclear. Schultz (1935) suggested that the trend reflects phylogeny and is primitive for catarrhines. More recently, however, researchers have proposed that life history and dietary hypotheses account for early relative molar eruption. If the colobine eruption pattern is primitive for catarrhines, it implies that cercopithecines and hominoids converged on delayed relative molar eruption. Alternatively, if the colobine condition is derived, factors such as diet and mortality patterns probably shaped colobine eruption patterns. Here we update our knowledge on eruption sequences of living colobines, and explore the evolutionary history of the colobine dental eruption pattern by examining fossil colobine taxa from Eurasia (Mesopithecus) and Africa (Kuseracolobus aramisi and Colobus sp.) and the basal cercopithecoid Victoriapithecus macinnesi. We scored specimens per Harvati (2000). The Late Miocene-Early Pliocene Mesopithecus erupts the second molar early relative to the incisors, while the Early Pliocene Kuseracolobus aramisi does not. These results demonstrate that the common colobine tendency for early molar eruption relative to the anterior dentition had appeared by the Late Miocene, and that some of the diversity observed among living colobines was already established in the Late Miocene/Early Pliocene. We discuss the implications of these results for phylogenetic, life history, and dietary hypotheses of dental development.     

Sequences and timing of dental eruption in Bolivian captive-born squirrel monkeys (Saimiri sciureus)

Squirrel monkeys, colony-born from Bolivian parents, were studied to establish the sequences and timing of eruption for deciduous and permanent teeth. Infants were born with a naked gingiva, and in only one monkey was di₁ present at birth. The eruption of the lower deciduous teeth preceded their upper counterparts with the exception of di2, dc, and dpm2. No significant differences were found between the right and left quadrants of the mandible and maxilla. No significant sexual differences were found in the age of eruption. By the age of 14 weeks, all deciduous teeth had erupted. The sequence of eruption of the replacement teeth was different from that of milk teeth. The differences lie in the delayed eruption of canine teeth and in the inverted sequence, from the back to the front, of the premolar series. Significant sexual differences were found in total eruption (TE) for PM₃ and I² (P < 0.05) and highly significant differences (P < 0.01) in TE and initial eruption (IE) for C¹, females being more precocious than males. The age at which monkeys completed dental eruption was highly variable, 103–119 weeks for males and 89–112 weeks for females. Differences were found when our results were compared with those of Long and Cooper [1968] for Colombian squirrel monkeys.     

Platyrrhine dental eruption sequences

To determine dental eruption sequences of extant platyrrhines, 367 mandibles and maxillae of informative juvenile specimens from all 16 genera were scored for presence of permanent teeth including three intermediate eruption stages following Harvati (Am J Phys Anthropol 112 (2000) 69-85). The timing of molar eruption relative to that of the anterior dentition is variable in platyrrhines. Aotus is precocious, with all molars erupting in succession before replacement of any deciduous teeth, while Cebus is delayed in M2-3 eruption relative to I1-2. Callitrichines have a distinct tendency toward delayed canine and premolar development. Platyrrhine eruption sequences presented here show some evidence of conformity to Schultz's Rule, with relatively early replacement of deciduous dentition in "slower"-growing animals. The relationship of dental eruption sequences to degree of folivory, body mass, brain mass, and dietary quality is also examined. The early eruption of molars relative to anterior teeth in Pithecia, Chiropotes, and Cacajao, in comparison to genera such as Ateles, Lagothrix, and Alouatta, showing relatively later eruption of the molars, appears to be consistent with current phylogenetic hypotheses. Schultz (Am J Phys Anthropol 19 (1935) 489-581) postulated early relative molar eruption as the primitive dental eruption schedule for primates. The extremely early molar eruption of Aotus versus Callicebus (where both incisors erupt before M2 and M3, with M3 usually last) may lend support to the status of Aotus as a basal taxon. The early relative molar eruption of the fossil platyrrhine species Branisella boliviana is also consistent with this hypothesis (Takai et al.: Am J Phys Anthropol 111 (2000) 263-281).     

Cephalometric and anthropomorphic observations of Binder's syndrome: a study of 19 patients

Binder's syndrome (maxillonasal dysplasia) is a disorder characterized by nasomaxillary hypoplasia. To ascertain the extent of underdevelopment of the midfacial skeleton and soft tissues, 19 of 29 patients with Binder's syndrome were retrospectively evaluated, both with cephalometry and anthropometry. Ten females and nine males were placed collectively into three age groups: 6 years, 10 years, and 16 to 17 years. Cephalometric measurements disclosed a short anterior cranial base (S-N), a normal length of the vertical maxilla (SE-PNS), a decreased horizontal maxilla (PNS-A, Co-A), a recessed orbitale (SNO), and a high-normal mandibular length (Co-Gn). Anthropometry revealed a large nasofrontal angle, acute nasal inclination and nasolabial angle, decreased nasal prominence (Sn-Prn), a decreased columellar length (C-Sn), and a normal vertical nose (N-Sn) and upper lip (Sn-Sto) length.