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Effects of fetus weight, dam strain, dam weight, and litter size on the craniofacial morphogenesis of CL/Fr mouse fetuses affected with cleft lip and palate.

OBJECTIVE: This study examined the factors related to the morphogenesis of the craniofacial complex of the CL/Fr mouse fetus affected with CLP based on the findings of a lateral cephalogram. DESIGN: Embryo transfer experiments were performed to determine the effect of the fetus weight, dam strain, dam weight, and litter size on the Intra-uterine craniofacial morphogenesis of CL/Fr mouse fetuses. On the 18th gestational day, each pregnant dam that had received CL/Fr mouse embryos was laparotomized to remove the transferred fetuses that had developed in the uteri of the cleft lip and palate (CLP)-susceptible CL/Fr strain dam and the CLP-resistant C57BL strain dam. A cephalometric observation of the craniofacial morphology of each fetus was subsequently performed. RESULTS: Based on a multiple regression analysis, the standardized partial regression coefficients of the affected fetus weight, the dam weight, and the litter size on the maxillary size of the affected CL/Fr fetus were 0.71 (p < .01), 0.03, and -0.07. According to a least-squares analysis of variance, the dam strain effect in addition to the effect of the affected fetus weight on the maxillary size and the cranial size of the affected fetuses was significant (p < .01 for cranial size, p < .05 for maxillary size) and close to a significant level (p = .09) for the mandibular size of the affected fetuses. The adjusted maxillary size and cranial size after statistically eliminating the effects of the affected fetus weight, dam weight, and litter size on each original craniofacial size of the affected fetuses that had developed in the CL/Fr dam strain were also significantly smaller than those of the affected fetuses that had developed in the C57BL dam strain. CONCLUSIONS: The present results indicate that the craniofacial growth of the CL/Fr mouse fetus affected with CLP increased in proportion to the fetus weight. The dam strain effect, in addition to the effect of the affected fetus weight, could thus not be ignored when the etiology of the spontaneous CLP was examined, while the uterine environment, provided by the CL/Fr strain dam, retarded the intra-uterine craniofacial growth of the affected fetuses. It was therefore concluded that the dam strain effect, as well as the effect of the affected fetus weight, both play an important role on the craniofacial morphogenesis of the CL/Fr strain of the affected fetuses that developed in both strain dams.

Analysis of Variance↗

Association of distinct craniofacial features in nonsyndromic cleft lip and palate family members.

OBJECTIVE: To assess the relationship of distinct craniofacial features among family members with cleft lip and palate (CLP). METHODS: Lateral cephalometric and anthropometric measurements of the members of 28 Costa Rican families with CLP were analyzed. The distinct craniofacial features in subjects with CLP were identified by comparing their craniofacial measurements with published normative values. The presence of these features was assessed in the unaffected family members. Regression coefficients were computed to evaluate the association of the distinct craniofacial measurements between parents and their offspring with and without CLP. RESULTS: The male and female subjects with CLP were characterized by shortened head (HL), anterior cranial base (S-N), and palatal length (ANS-PNS, A-PNS) measurements as well as hyperdivergent angle of S-N plane to palatal plane (<SN-PP). All unaffected family members also had shortened S-N and ANS-PNS measurements and mothers of affected daughters also shortened HL. Mothers had a significant association in S-N, ANS-PNS, and <SN-PP measurements with their affected daughters (p <.05 for all), and fathers had a significant association in A-PNS measurement with their affected sons (p <.05). None of the associations of the distinct craniofacial measurements were significant between mothers and their unaffected daughters or between fathers and their unaffected sons. CONCLUSION: Unaffected family members of subjects with CLP also have some distinct craniofacial features, however in lesser degree. Mothers contribute more to their affected daughters' and fathers to their affected sons' distinct craniofacial morphology.

Adolescent↗

Craniofacial muscle pain: review of mechanisms and clinical manifestations.

Epidemiologic surveys of temporomandibular disorders (TMD) have demonstrated that a considerable proportion of the population--up to 5% or 6%--will experience persistent pain severe enough to seek treatment. Unfortunately, the current diagnostic classification of craniofacial muscle pain is based on descriptions of signs and symptoms rather than on knowledge of pain mechanisms. Furthermore, the pathophysiology and etiology of craniofacial muscle pain are not known in sufficient detail to allow causal treatment. Many hypotheses have been proposed to explain cause-effect relationships; however, it is still uncertain what may be the cause of muscle pain and what is the effect of muscle pain. This article reviews the literature in which craniofacial muscle pain has been induced by experimental techniques in animals and human volunteers and in which the effects on somatosensory and motor function have been assessed under standardized conditions. This information is compared to the clinical correlates, which can be derived from the numerous cross-sectional studies in patients with craniofacial muscle pain. The experimental literature clearly indicates that muscle pain has significant effects on both somatosensory and craniofacial motor function. Typical somatosensory manifestations of experimental muscle pain are referred pain and increased sensitivity of homotopic areas. The craniofacial motor function is inhibited mainly during experimental muscle pain, but phase-dependent excitation is also found during mastication to reduce the amplitude and velocity of jaw movements. The underlying neurobiologic mechanisms probably involve varying combinations of sensitization of peripheral afferents, hyperexcitability of central neurons, and imbalance in descending pain modulatory systems. Reflex circuits in the brain stem seem important for the adjustment of sensorimotor function in the presence of craniofacial pain. Changes in somatosensory and motor function may therefore be viewed as consequences of pain and not factors leading to pain. Implications for the diagnosis and management of persistent muscle pain are discussed from this perspective.

Afferent Pathways↗

Craniofacial morphology and growth in the ferret: effects from alteration of masticatory function.

INTRODUCTION: Our hypothesis is that any effects on craniofacial growth and morphology induced by altering the masticatory function will vary according to the species involved. The general aim of this thesis was to test the possible effects of altering the masticatory function on craniofacial growth and morphology, as well as features of masticatory muscles in ferrets. To better elucidate these mechanisms involved, the anatomy of the skull, craniofacial growth pattern and development of occlusion in the ferret were investigated to serve as baseline data. MATERIALS AND METHODS: The description of the anatomy and the radiographic features of the skull was based on observations of 100 skulls of adult ferrets of both sexes. The craniofacial growth pattern was studied in 16 young ferrets of both sexes using serial cephalograms taken on 5 occasions according to age: 25 days, 35 days, 55 days, 80 days and 300 days. To follow the development of occlusion, these animals underwent dental examinations every other day. Forty male ferrets were divided into two groups from the age of 5 weeks. One group was fed hard pellets (hard-diet group), and the other the same diet but softened with water (soft-diet group). After six months, specimens of masticatory muscles were dissected and analysed histochemically, and cephalograms were analysed cephalometrically. RESULTS: The ferret skull is relatively elongated with a short facial region, being longer and broader in males than in females. The viscero- and neurocranium follow an orderly pattern of expansive growth. The growth of the mandible is mainly characterized by an anteroposterior body elongation, and enlargement of the coronoid process. Craniofacial growth in ferrets ceases earlier in female than in male animals. The deciduous teeth erupted between the 19th and 31st postnatal day, and exfoliated approximately between days 51 and 76. The time of eruption of the permanent teeth ranged from 42 to 77 days. The female ferrets were generally ahead of the males regarding the eruption age of permanent teeth. The alteration of the masticatory function by feeding the animals a soft diet caused a hard palate plane more distant from the cranial base plane, smaller inter-frontal and inter-parietal widths, and a slenderer zygomatic arch, as well as a generally shorter and narrower coronoid process. Variations of most variables are greater in animals fed on soft diet. The mean cross-sectional area of type I and type II fibres in the temporalis and the masseter, and type II fibres in the digastricus, as well as the number of capillaries per fibre were significantly smaller in the soft-diet animals. CONCLUSION: Altered masticatory function has induced changes in masticatory muscles and certain effects on craniofacial growth in ferrets. These changes are not the same as those reported in other species (e.g. rats). It appears that reduced masticatory function leads to smaller fibres in the elevators and transverse skull dimensions. The different effects are presumably related to the differences in craniofacial anatomy and growth pattern, as well as the masticatory system, in the various species studied previously.

Animals↗

[A cephalometric study on the similarity of craniofacial morphology between children and their parents].

This study was performed to estimate genetic influences on craniofacial form and to obtain a method of analysing the characteristics in a child as well as the individual growth prediction in each child. Posteroanterior and lateral cephalographs were used to estimate the morphological similarities of craniofacial form between 150 children and their parents. Subjects consisted of 38 boys and 112 girls who were estimated to mature according to the bone maturity index (B.M.I.) on the hand radiographs and who were older than 13 years chosen from among 3,500 orthodontic patients registered at the University Hospital associated with the Faculty of Dentistry, Kyushu University. The correlation analysis in family, estimation of the sexual linkage, the multi-regression analysis and estimation of the heritability of craniofacial characters were computed. The differences in correlations were also tested between prepuberty and postpuberty craniofacial forms in another 100 children with orthodontic treatment. The results are as follows: 1. The craniofacial form of children was highly correlated with that of their parents. 2. The genetic effects of determining the craniofacial form of offspring by the father were equivalent to those by the mother, but daughters were more affected by parents than were sons. 3. The proportions of multi-regression functions ranged from 3.7 to 34.0 percent. These values are too low to predict the growth of a child. 4. The calculated heritabilities on craniofacial form were from 0.286 to 0.962, with the majority being more than 0.600. 5. The coefficients of correlation between the children and their parents increased with growth, regardless of their orthodontic treatment.

Adolescent↗

Perspectives on craniofacial growth.

This article underscores the importance of the growth process in the production of normal and abnormal craniofacial morphology. Although much has been learned over the past century, we still have only a limited appreciation of the 3D changes that occur in the skull during postnatal growth. We have also stressed that the current cooperation between reconstructive surgeons and radiologists is producing a substantive amount of data that could be used to further our understanding of the postnatal growth process. At The Johns Hopkins University we are putting our efforts toward the collection and organization of a large data base of craniofacial images that will allow us to study questions about the role growth plays in mediating the results of reconstructive surgery. Our ultimate goal is to provide information about the future appearance of craniofacial patients based on empirically derived growth patterns. In a recent article Dufresne and Richtsmeier proposed that the results of surgical correction may be predicted based on the etiology of the craniofacial condition, and not necessarily the degree or character of the dysmorphology. The authors hypothesize that a categorization or classification of craniofacial dysmorphology can be proposed, based on the response of individuals to surgery, and that such a classification reflects real differences in etiology. Hence, a poor response to surgery reflects a condition that includes a growth disorder, whereas cases that respond positively to reconstructive surgery are those in which the growth process is not part of the dysmorphology. In the latter cases, a dysmorphic face is surgically transformed into an acceptable morphology, and normative growth vectors maintain or improve postoperative facial appearance. Thus, Dufresne and Richtsmeier13 suggest that this divergence in the response to surgery among patients relates directly to the role of the growth process in various types of dysmorphologies. Using our tools and the data base we are forming, we envision a markedly different preoperative consultation for future craniofacial patients. When a patient is evaluated, images from the data base with a similar diagnosis will be used to calculate a growth pattern for the proper age interval. The patient's scan is then "grown" according to the appropriate growth pattern. The parents and patient can evaluate this new image and see what their child's skull will look like 2, 3, or 4 years from the present, both with and without reconstructive surgery. In addition, our tools will statistically compare the simulated (or "grown") skull to samples of craniofacial images of normal and affected children of an age/sex/ethnic population that matches the patient. This testing will determine whether growth results in the patient becoming more normal, or more different from normal, with time.(ABSTRACT TRUNCATED AT 400 WORDS)

Child↗

The effect of dam strain on the craniofacial morphogenesis of CL/Fr mouse fetuses.

The embryo transfer technique and cephalometry were used to investigate the effect of dam strain in intrauterine craniofacial growth and the severity of cleft lip and palate (CLP) in a CLP-susceptible CL/Fr strain of embryos. The CL/Fr strain of embryos at early blastocyst stage was transferred to the same dam strain and to the CLP-resistant C57BL dam strain. On the 18th gestational day, each dam was laparotomized to take out the fetuses. The spontaneous incidence of CLP in the fetuses was checked and a cephalometric observation of the craniofacial complex of each fetus was done just after laparotomy. The dorsoventral craniofacial size of the unaffected fetuses and the severity of CLP i the affected ones were compared between both dam strains. The following results were obtained: 1) The overall craniofacial sizes of the unaffected fetuses observed in the CL/Fr dam strain were significantly smaller than those seen in the C57/BL dam strain. Those of the affected fetuses observed in the CL/Fr dam strain were smaller than those seen in the C57BL dam strain although the interstrain difference was not significant. 20 The dam strain had a highly significant effect on the craniofacial size of the unaffected fetuses. 3) The CLP frequency in the CL/Fr dam strain was significantly higher than that in the C57BL dam strain. 4) The severity of CLP in the affected fetuses observed in the CL/Fr dam strain was significantly more serious than that seen in the C57BL dam strain. These results indicated that the CLP-susceptible CL/Fr dam strain retarded the intrauterine craniofacial growth of the fetuses and that the cleft condition in the affected fetuses observed in the CL/Fr dam strain was more seriously affected than that seen in the CLP-resistant C57BL dam strain. Thus, it can be concluded that the effect of the dam strain played an important role in the craniofacial morphogenesis of the CL/Fr strain of mouse fetuses that developed from the embryo transferred to the CL/Fr and C57BL dam strains along with the genotype of embryos.

Animals↗

A longitudinal study of the postnatal maternal effect on the craniofacial growth of mouse offspring by cross-nursing.

A cross-nursing experiment was conducted to examine the nursing dam strain effect on the postnatal growth of a newborn mouse offspring by simultaneously using larger DDD strain mice and smaller C57BL strain ones. A periodical cephalometric observation of the postnatal craniofacial growth of the offspring was longitudinally made from birth up to the 100th day of life in addition to measuring the offspring weight. According to multivariate statistical analyses, the following results were obtained: 1) The mean body weight of the DDD offspring cross-nursed by the C57BL dam was significantly lighter than that of the DDD offspring self-nursed by the DDD dam throughout the whole experimental period except at birth and on the 100th day. 2) The mean body weight of the C57BL offspring cross-nursed by the DDD dam was significantly heavier than that of the C57BL offspring self-nursed by the C57BL dam from the 10th day up to the 30th day. 3) There was a significant positive relationship between the offspring weight and the offspring craniofacial size throughout the entire experimental period in the C57BL offspring and from the 10th day up to the 40th day in the DDD offspring. 4) The nursing dam strain effect on the craniofacial size of the DDD offspring was also significant on the 30th, 60th, 80th, and 100th days. The adjusted craniofacial size of the DDD offspring cross-nursed by the C57BL dam was significantly smaller than that of the DDD offspring self-nursed by the DDD dam on the 30th, 60th, 80th, and 100th days. 5) The nursing dam strain effect on the craniofacial size of the C57BL offspring was not significant throughout the whole experimental period. The adjusted craniofacial size of the C57BL offspring cross-nursed by the DDD dam was almost the same as that of the C57BL offspring self-nursed by the C57BL dam throughout the whole experimental period. 6) The fat content of the DDD dam tended to be higher than that of the C57BL dam and the interstrain difference was closed to a significant level (P = 0.06) on the 20th day. The moisture content of the DDD dam tended to be lower than that of the C57BL dam and the interstrain difference was also significant on the 7th day (P < 0.001). Based on these findings, it can thus be concluded that the nursing dam strain effect played an important role in the postnatal somatic growth of the DDD and the C57BL offspring and the craniofacial growth and the DDD offspring.

Animals↗

A longitudinal observation of the postnatal craniofacial growth in artificial monozygotic twin mice.

An artificial monozygotic twin mouse produced by bisecting a 16-cell stage embryo with a fine glass-needle and then transferring a pair of demi-embryos together into the right uterus of a pregnant recipient female mouse after a 24 hr in-vitro cultivation allowed us to examine the postnatal growth changes in monozygotic twin mice. With regard to the preimplantational growth process of the embryo, we investigated the success rate of bisecting an embryo into paired demi-embryos, the developmental ability of the bisected demi-embryo up to the early blastocyst, and the production rate of such monozygotic twin mice. The items examined in the postnatal growth process of the monozygotic twin mouse were the growth curve of the twin weight and the craniofacial size of the monozygotic twin mouse by cephalometric observations at the 3rd, 10th, 21st, 42nd, 70th, and 100th postnatal days, respectively. The following results were thus obtained. 1) The success rate of bisecting the 16-cell stage embryos into a paired demi-embryos was 86.1% (2,528/2,936). As a result, 2,301 of 2,528 paired demi-embryos could develop up to blastocysts after 24 hr of in-vitro cultivation (91.0%). 2) In addition 42 of the 394 (10.7%) recipient female mice, which had been transferred as paired demi-embryos and delivered a litter, resulted in 31 singleton mice (7.9%) or 11 paired monozygotic twin mice (2.8%), respectively. 3) The weight of each twin increased rapidly up to the 42nd day but thereafter only increased gradually up to the 100th day. The within-pair difference among each paired monozygotic twin mouse could be observed from after the 21st day until the 100th day. 4) Both the dorsoventral craniofacial size of each twin mouse and the lateral craniofacial size of each monozygotic twin mouse increased rapidly up to the 42nd day and thereafter gradually reached a plateau by the 100th day. Almost no within-pair difference was observed among each paired monozygotic twin mouse after weaning up to the 100th day. Based on these results, it is thus concluded that the craniofacial growth of each paired monozygotic twin mouse more closely resembled the growth pattern than that of the somatic growth, which thus indicated that the craniofacial growth was more greatly controlled by the genetic effect than the somatic growth. Furthermore, these findings thus suggest that an embryo bisection is an essential for producing artificial monozygotic twin mice from paired demi-embryos. Therefore, artificial monozygotic twin mice are considered to be a useful animal model for examining the effect of genetic-environment interaction on either the prenatal dento-craniofacial morphogenesis or the postnatal craniofacial growth in mice.

Animals↗

Airway management in children with major craniofacial anomalies.

OBJECTIVES: Delineation of clinical characteristics affecting the airway in a cohort of craniofacially deformed children. What factors differ between patients requiring and those not requiring surgical airway intervention? What factors predispose to the need for tracheotomy? When can decannulation be expected if tracheotomy is required? What interventions aid decannulation? STUDY DESIGN: Five-year retrospective chart review at tertiary center. METHODS: Two hundred fifty-one patients met the following entry criteria: enrollment in the New York University Institute of Reconstructive and Plastic Surgery's Craniofacial Clinic and admission to Tisch Hospital in Manhattan for surgery from 1990 to 1994. Hospital, clinic, and departmental office records were reviewed. All patients had major craniofacial bony anomalies and underwent administration of general anesthesia at least once. RESULTS: Nearly 20% of all children required tracheotomy (47/251). Craniofacial synostosis patients (Crouzon, Pfeiffer, or Apert syndrome) had the highest rate of tracheotomy (48% [28/59]). Mandibulofacial dysostoses patients (Treacher Collins or Nager syndrome) had the next highest rate (41% [28/59]). Patients with oculo-auriculo-vertebral sequence were less likely to undergo tracheotomy (22% [9/41]). Children with craniosynostosis rarely required a surgical airway, unless there was marked associated facial dysmorphism (1% [1/72]). The duration of cannulation was related to the age at tracheotomy in a bimodal distribution. Generally, tracheotomies required before age 4 years remained for several years, whereas those placed after age 4 were removed after several weeks. The presence of a cleft palate correlated with reduced risk for tracheotomy, but the presence of a ventriculoperitoneal shunt correlated with an increased risk for tracheotomy. Procedures selectively used to improve the airway included midface advancement, mandibular expansion, tonsillectomy and adenoidectomy, uvulopalatopharyngoplasty, anterior tongue reduction, and endoscopic tracheal granuloma excision. CONCLUSIONS: The likelihood for surgical airway management is related to specific craniofacial diagnosis. The length of tracheal cannulation is greatest for infants and young children who manifest severe airway compromise, often because of nasal obstruction in combination with other anatomic factors. Early tracheotomy is advocated for these patients to promote optimal growth and development. Choanal atresia is often misdiagnosed in these infants; nasal obstruction is actually secondary to midface retrusion. Staged surgical interventions can allow eventual successful decannulation in nearly all cases of craniofacial syndromes.

Adolescent↗

Recent advances in craniofacial morphogenesis.

Craniofacial malformations are involved in three fourths of all congenital birth defects in humans, affecting the development of head, face, or neck. Tremendous progress in the study of craniofacial development has been made that places this field at the forefront of biomedical research. A concerted effort among evolutionary and developmental biologists, human geneticists, and tissue engineers has revealed important information on the molecular mechanisms that are crucial for the patterning and formation of craniofacial structures. Here, we highlight recent advances in our understanding of evo-devo as it relates to craniofacial morphogenesis, fate determination of cranial neural crest cells, and specific signaling pathways in regulating tissue-tissue interactions during patterning of craniofacial apparatus and the morphogenesis of tooth, mandible, and palate. Together, these findings will be beneficial for the understanding, treatment, and prevention of human congenital malformations and establish the foundation for craniofacial tissue regeneration.

Animals↗

Lambdoid stenosis (posterior plagiocephaly) and craniofacial asymmetry: long-term outcomes.

We recently reviewed our series of craniofacial cases involving lambdoid stenosis (posterior plagiocephaly) and positional deformation. We now have 22 cases (who underwent surgery) with greater than 1 year follow up (range 1-7 years). We were impressed by the potential severity of the craniofacial deformity that can occur in what is often considered a positional deformation caused either by intrauterine conditions or by postnatal positioning. To decide which children were candidates for craniofacial reconstruction, we reviewed our cases and determined what we considered were appropriate criteria for craniofacial surgery. Among the 22 patients selected for surgery, 16 patients had changes in facial characteristics secondary to skull base and petrous ridge deformation, which often led to mandibular malalignment. In addition, 11 children were found to have developed scolosis of the face, similar to that seen in anterior plagiocephaly. The long-term severity of these selected craniofacial deformities may be markedly reduced if these children are operated on early with craniofacial reconstruction techniques. The removal of the lambdoid sutures, which appears to apply a twisting or torque effect on the facial structures and skull base, assisted in the children's further normal cranial development. In the children that were operated on before 1 year of age, most of the facial asymmetry was corrected. In addition, we observed that several of the children showed correction of the malignment of the ears, a common finding in these cases. Three-dimensional reconstructions have been extremely helpful in documenting the abnormalities of the skull base and sutural patterns and in planning the surgical approaches. All the patients had either a Marchac transposition or a bandeau/forehead type reconstruction. There were no long-term complications, and the esthetic results were considered good to excellent.

Cephalometry↗

Congenital craniofacial anomalies of ophthalmic importance.

Congenital craniofacial abnormalities frequently require ophthalmic evaluation and surgical management. Called upon to perform as part of the craniofacial team managing the often severely deformed craniofacial patient, the ophthalmologist must bring a basic knowledge of craniofacial syndromes and developmental anatomy, as well as clinical acumen to help preserve or improve ocular and adnexal function. As an introduction to this area of ophthalmology, the clinical features, classification, appropriate facial embryology, assessment and surgical considerations of the various congenital craniofacial abnormalities are reviewed. The expanding availability of craniofacial surgeons and surgical teams along with improved surgical results will ultimately require an increasing involvement by many more ophthalmologists in the evaluation and management of these congenital abnormalities.

Craniofacial Dysostosis↗

Clinical applications of Norian SRS (calcium phosphate cement) in craniofacial reconstruction in children: our experience at Hospital La Paz since 2001.

PURPOSE: The reconstructive surgeon has been searching for an ideal material to use in the reconstruction of craniofacial deformities. Although bone graft presents most of the ideal properties, its limited availability and donor-site morbidity makes its use compromised in some patients, especially in pediatric patients. In the search of this ideal material, calcium phosphate cements have shown promising results in patients with craniofacial deformities, because of its unlimited availability, avoidance of donor-site morbidity, and long-term maintenance. MATERIALS AND METHODS: We report our experience at the Pediatric Division of Maxillofacial Surgery Department, Hospital La Paz, Madrid, since 2001, in the use of Norian SRS (calcium phosphate cement; Norian, Cupertino, CA) for the treatment of secondary craniofacial deformities. Clinical charts are retrospectively reviewed, paying attention to gender, age, pathologic conditions, previous surgeries, and the clinical applications of this kind of calcium phosphate cement. Complications are also reviewed. RESULTS: Five patients have been treated for different craniofacial malformations, using Norian SRS as a part of the surgical procedure. The main indication was frontal and cranial irregularities. Temporal hollowing was also reconstructed with the calcium phosphate cement. All patients, except for 1, recuperated successfully. One patient suffered a wound dehiscence with subsequent infection of the material. CONCLUSION: Norian SRS offers craniofacial surgeons an option in the treatment of pediatric patients with diverse craniofacial malformations, although special attention must be paid to the risk of infections in selected patients.

Bone Cements↗

Craniofacial distraction osteogenesis: a review of the literature: Part 1: clinical studies.

A review of the literature dealing with distraction osteogenesis (DO) of the craniofacial skeleton, provided by a PUBMED search (National Library of Medicine, NCBI; revised 3 April 2000) from 1966 to December 1999 was conducted. Key words used in the search were distraction, lengthening, mandible, mandibular, maxilla, maxillary, midface, midfacial, monobloc, cranial, craniofacial and maxillofacial. This search revealed 285 articles. One hundred and nine articles were clinically orientated and were analysed in detail in this study. The type of distraction, indications, age, type of surgery, distraction rates and rhythms, latency and contention periods, amount of lengthening, follow-up period, relapse, complications and the nature of the distraction device were analysed. This review revealed that 828 patients underwent DO of the craniofacial skeleton; 579 underwent mandibular DO, 129 maxillary DO, 24 simultaneous mandibular and maxillary DO and 96 midfacial and/or cranial DO. Craniofacial DO has proven to be a major advance for the treatment of numerous congenital and acquired craniofacial deformities. Treatment protocols and success criteria for craniofacial DO are suggested on the basis of these results. There is still, however, a lack of sufficient data, especially on follow-up and relapse, so that treatment strategies have to be validated by long-term studies in the future.

Adolescent↗

Hand abnormalities associated with craniofacial syndromes.

One in approximately every 626 newborns has a congenital anomaly of the upper limb. Frequently, patients with craniofacial syndromes may have associated hand anomalies. In this article, we try to answer why certain craniofacial syndromes have associated hand anomalies through an examination of the common factors in development and maldevelopment of these two very complex structures. In general, we conclude that the common factors responsible for patients with craniofacial syndromes that have associated hand anomalies are the common time course of development of these two structures and certain mutations of genes controlling limb and craniofacial development. This article attempts to elucidate the sequence and crucial factors responsible for proper limb growth, as we understand it today. The most common craniofacial syndromes that include craniosynostosis and upper extremity anomalies are presented. These are Apert's, Saethre-Chotzen, Pfeiffer's, and Carpenter's syndromes. As we discuss each of these syndromes, basic principles regarding the surgical correction of the associated hand anomalies are described. We hope that this overview serves to give the pediatrician and the craniofacial specialist general guidelines for what to look for and expect in the hands and upper extremities of children under their care.

Acrocephalosyndactylia↗

Adult psychological functioning of individuals born with craniofacial anomalies.

This study represents an initial investigation into the adult psychological functioning of individuals born with craniofacial disfigurement. A total of 24 men and women born with a craniofacial anomaly completed paper and pencil measures of body image dissatisfaction, self-esteem, quality of life, and experiences of discrimination. An age- and gender-matched control group of 24 non-facially disfigured adults also completed the measures. As expected, craniofacially disfigured adults reported greater dissatisfaction with their facial appearance than did the control group. Craniofacially disfigured adults also reported significantly lower levels of self-esteem and quality of life. Dissatisfaction with facial appearance, self-esteem, and quality of life were related to self-ratings of physical attractiveness. More than one-third of craniofacially disfigured adults (38 percent) reported experiences of discrimination in employment or social settings. Among disfigured adults, psychological functioning was not related to number of surgeries, although the degree of residual facial deformity was related to increased dissatisfaction with facial appearance and greater experiences of discrimination. Results suggest that adults who were born with craniofacial disfigurement, as compared with non-facially disfigured adults, experience greater dissatisfaction with facial appearance and lower self-esteem and quality of life; however, these experiences do not seem to be universal.

Adult↗

Tissue expansion in the reconstruction of Tessier craniofacial clefts: a series of 17 patients.

Tessier craniofacial clefts are among the most surgically challenging examples of craniofacial dysmorphology. These clefts are characterized by hypoplasia of soft-tissue and skeletal elements throughout the three-dimensional extent of the cleft. Whereas bone grafting and craniofacial osteotomies have been successful toward correcting the underlying skeletal abnormalities, the ultimate success of these reconstructions has been limited by the deficiency of skin and soft tissue. This deficiency demands reconstruction ideally with tissue of like texture, consistency, and, especially in the face, color. Craniofacial tissue expansion was used toward reconstructing these facial clefts with like-quality tissue, allowing for tension-free reconstruction after osteotomy and bone grafting. Seventeen patients with Tessier craniofacial clefts underwent preoperative craniofacial soft-tissue expansion in the surgical management of their clefts. Tissue expansion was used in the primary correction of facial clefts in eight patients, with nine patients undergoing expansion before secondary surgery. In this series, tissue expansion has evolved as an important element in overcoming the skin and soft-tissue deficiency associated with these clefts, allowing for tension-free closure and improved aesthetic results in these surgically challenging patients.

Adolescent↗