bluuebunny/crossref_metadata_2025 huggingface dataset:
The Tessier Number 5 Cleft with Associated Extremity Anomalies
Authors: Gökalan Kara İnci , Öçsel Hakan
Published: October 2018

Objective:

Oblique facial clefts are rare congenital deformities. They constitute 0.22% of all facial malformations. In this case report, an oblique facial cleft defined as Tessier number 5 is presented. The number 5 cleft of Tessier is one of the most rare congenital facial clefts; review of the literature revealed 20 patients. This case was also demonstrated with associated limb anomalies, suggesting that the patient may have been affected by the amnion rupture sequence. The teratology of these malformations is discussed, and attention is drawn to the amniotic rupture sequence as a possible cause.
The Faith of a Coronal Suture Grafted onto Midline Synostosis Inducing Dura and Deprived from Tensile Stress
Authors: Mommaerts Maurice Y. , Staels Patrick F. J. , Casselman Jan W.
Published: October 2018

Objective:

To discuss possible reasons for the synostosis of a coronal suture that was transplanted onto synostosis inducing dura in a scaphocephalic human cranium.

Design:

Case report.

Setting:

Supraregional teaching hospital, center for craniofacial anomalies.

Patient:

A bathmocephalic boy, followed from age 7½ to 26 months.

Intervention:

Radical synostosectomy, radial osteotomies in the parietal bone with outward fracturing of the barrel staves, and left-sided coronal suture transplantation onto the midline was undertaken at the age of 11 months.

Methods:

Computer tomography and clinical follow-up.

Results:

The sutural graft, initially deprived from tensile stress and quickly exposed to the anomalous dura, turned synostotic in one year.

Conclusions:

Both cell signaling and biomechanical theories on calvarial morphogenesis, sutural development, and synostosis can apply. An animal experiment is recommended to test which hypothesis prevails.
Vestibuloplasty after Secondary Alveolar Bone Grafting
Authors: Iino Mitsuyoshi , Fukuda Masayuki , Murakami Kaho , Horiuchi Toshikatsu , Niitsu Kouta , Seto Kanichi
Published: October 2018

Objective:

This paper introduces a surgical technique for vestibuloplasty after secondary alveolar bone grafting of patients with cleft lip and palate (CLP). This paper also reports on the patients who underwent this modified vestibuloplasty.

Surgical Procedure:

The vestibuloplasty technique described in this paper consists of: (1) reduction of submucosal scar tissue of the upper lip, (2) V-Y plasty of the superficial mucosa, (3) placement of horizontal mattress sutures between nostril floor skin and freed marginal mucosa, (4) application of artificial skin to cover the exposed periosteal surface, and (5) use of a removable retention splint.

Conclusion:

This surgical procedure appears to be very useful for patients with CLP. The technique enables the surgeon to obtain an adequate sulcus depth around the graft area. In addition, this technique releases the mucosal scar contraction and improves the shape and mobility of the upper lip.
Children of the Philippines: Attitudes toward Visible Physical Impairment
Authors: Harper Dennis C. , Peterson David B.
Published: October 2018

Objective:

This pilot study was designed to evaluate children's attitudes and understanding of physical disabilities with special reference to those with craniofacial anomalies in the Philippines. Children with and without craniofacial anomalies were studied.

Design:

This was a two-group correlational design with additional statistical assessment of subgroup differences. Each group was interviewed and information obtained on a standard disability preference task, attributions for playmate choice, and frequency of contact with disabilities. Parents completed a structured interview.

Setting:

Participants were 122 children recruited from Negros, Philippines.

Participants:

Fifty-four children with craniofacial anomalies (aged 7 to 12 years) were enrolled in the study, and 68 children without any disabilities were recruited from a local school in Bacolod City, Negros, Philippines.

Main Outcome Measures:

Participants completed a picture-ranking interview of specific physical disabilities and provided their reasons for their play choices and their contact with physical disabilities.

Results:

The Kendall W correlation was significant for the children with craniofacial anomalies and for those without physical disabilities. Both groups reported lower preferences for disabilities that interfere with play and social interactions. Children depicted with facial anomalies received lower preference, compared with other physical disabilities. Children with craniofacial anomalies who have experienced surgical repair reported more positive rankings for the child depicted with a facial cleft. Sex differences in disability preference were noted.

Conclusions:

Children in the Philippines with and without craniofacial differences revealed similarities in preferences to children in several Western (United States) and non-Western countries. Children depicted with facial anomalies received lower preference than other visible physical differences. Children reported both positive and negative explanations for their disability play preferences. Facial differences may result in illogical and negative explanations for social avoidance among children. Similar reactions are noted in other parts of the world.
Facial Anthropometry in Cleft Patients: A Historical Appraisal
Authors: Vegter Florine , Hage J. Joris
Published: October 2018

Objective:

To present a historical appraisal of the use of anthropological and cephalometrical facial soft tissue measurements in cleft patients.

Design:

The McDowell Indexes and a Medline search were used to trace references up to 1999. Also, references listed with chapters and articles on facial clefts were searched for anthropometrical studies. Twenty-six retrieved articles and book chapters on soft tissue anthropometry and 12 cephalometric publications on soft tissue measurements on radiographs and plaster casts of cleft patients were reviewed.

Results and Conclusions:

Since 1931, the facial soft tissue appearance of cleft patients has been evaluated by means of anthropometric and cephalometric techniques. Not all of the older studies were performed in a statistically correct fashion. Many of the conclusions of the studies overlap despite differences in technique of assessment. Most studies demonstrate the deficient growth of the maxilla and the deformities of the facial profile in cleft patients.
Cranial Base Growth and Morphology in Second-Trimester Normal Human Fetuses and Fetuses with Cleft Lip
Authors: Sherwood Todd F. , Mooney Mark P. , Sciote James J. , Smith Timothy D. , Cooper Gregory M. , Siegel Michael I.
Published: October 2018

Objective:

The present radiographic study describes the size and shape of the cranial base from the sagittal aspect for a sample of 77 second-trimester “normal” control fetuses (n = 61) and fetuses (n = 16) exhibiting isolated, unilateral clefts of the lip (CL), ranging in fertilization age from 10 to 22 weeks.

Methods:

Fetuses were placed in a cephalostat, and standardized, lateral head radiographs were taken. The radiographs were traced, and 15 cephalometric landmarks were identified and digitized for analysis. Growth curves for cranial base lengths, angles, and areas were compared between control and CL groups. Also, cranial base triangles were constructed and shape comparisons were made using tensor biometric analysis.

Results:

No significant differences (p > .05) in regression line slopes were noted for any comparisons between the control and CL samples. Tensor biometric analysis also revealed no significant differences in the shapes of various cranial base triangles between the control and CL samples.

Conclusion:

This report presents second-trimester baseline growth curves for various cranial base components in CL human fetal specimens, and these data suggest that CL fetuses may also be used as an appropriate control sample for prenatal growth comparison studies of cleft lip and palate and cleft palate.
Endocranial Vascular Patterns in a Familial Rabbit Model of Coronal Suture Synostosis
Authors: Burrows Annie M. , O'Loughlin Valerie D. , Mooney Mark P. , Smith Timothy D. , Wolfgang Losken H. , Siegel Michael I.
Published: October 2018

Objective:

The present study investigates the potential relationship between craniosynostosis and any changes in endocranial vasculature. The hypothesis that crania from rabbits with familial, nonsyndromic coronal suture synostosis and crania from rabbits with experimental immobilization of the coronal suture are associated with altered form of the middle meningeal vessels and dural venous sinuses is tested.

Design:

Silicone rubber endocasts from 14 adult New Zealand white rabbits (Oryctolagus cuniculus) with familial nonsyndromic coronal suture synostosis (five with bilateral coronal suture synostosis and nine with unilateral coronal suture synostosis) were made to assess middle meningeal vessel and dural venous sinus form. For comparative purposes, endocasts were made from 25 rabbits with normal, patent coronal sutures and 10 rabbits with experimental immobilization of the coronal suture. Impressions of the dural venous sinuses were assessed for depth and width. The area of the confluens of sinuses was also assessed. Impressions of the middle meningeal vessels were assessed for depth, width, and degree of convolution. For width of the dural venous sinuses and area of the confluens of sinuses, comparisons among groups were made with a one-way analysis of variance (ANOVA). For depth of the dural venous sinuses and impressions of the middle meningeal vessels, comparisons among groups were made using a Kruskal-Wallis one-way ANOVA.

Results:

Crania with familial coronal suture synostosis had significantly (p < .05) reduced posterior dural venous sinus dimensions when compared with both crania from rabbits with experimental immobilization of the coronal suture and rabbits with normal coronal sutures. Crania with both coronal suture synostosis and experimental immobilization had significant increases in dimensions of the middle meningeal vessels relative to normal crania. In addition, casts from rabbits with unicoronal suture synostosis showed marked asymmetry in the dural venous sinuses.

Conclusions:

These results support the hypothesis that craniosynostosis is associated with alterations in endocranial vasculature. These changes are most likely a secondary response to synostosis rather than a causal factor and may reflect increased intracranial pressure, decreased intracranial volume, and local accumulations and reductions of cerebrospinal fluid in the posterior region of the skull and immediately deep to the coronal suture.
The Dental Health and Caries-Related Microflora in Children with Craniosynostosis
Authors: Mustafa Duaa , Lucas Victoria S. , Junod Pablo , Evans R. , Mason Carol , Roberts Graham J.
Published: October 2018

Objective:

To compare levels of dental caries, bacterial dental plaque, gingivitis, enamel defects, and caries-related microflora in children with and without craniosynostosis.

Study group:

Fifty-seven children with craniosynostosis and their matched controls.

Outcome measures:

The decayed, missing, and filled teeth and surfaces in both the deciduous (dmfs and dmft) and the permanent dentition (DMFS and DMFT). The plaque and gingivitis scores and developmental enamel defects were also recorded. The caries-related microflora was sampled using an alginate swab and the prevalence of Streptococcus mutans and Lactobacillus and Candida species were recorded.

Results:

The dmfs (p < .02) and dmft (p < .01) were significantly greater in the control children. The plaque score for the deciduous dentition only (p < .02) and also the gingivitis score for the permanent teeth only (p < .008) in the craniosynostosis group were significantly greater. The total aerobic bacterial count (p < .004), anaerobic count (p < .002), and Candida count (p < .05) were significantly greater in the control group. The proportion of S. mutans both as a percentage of the total anaerobic count (p < .04) and the total streptococcal count (p < .05) was significantly greater in the control group.
Displacement of the Mandible in a Child with Congenital Unilateral Temporomandibular Joint Ankylosis Treated with Two-Stage Condylar Replacement: A Long-Term Study with the Aid of Roentgen Stereometric Analysis
Authors: Wittbjer Jan , Sarnäs Karl-Victor , Rune Bodil
Published: October 2018

Objective:

To monitor mandibular displacement in three dimensions with a high degree of accuracy before, during, and after treatment.

Design:

Prospective roentgen stereometric analysis from age 3 years 7 months to age 13 years 8 months.

Setting:

Center for Craniofacial Anomalies and Department of Maxillofacial Surgery, Malmö University Hospital, Malmö, Sweden.

Patient:

Girl with unilateral congenital temporomandibular joint (TMJ) ankylosis and marked facial asymmetry.

Interventions:

Epipharyngeal inspection at age 2 years 6 months and insertion of implants under general anesthesia. Resection of the affected right condyle at age 5 years 4 months, replacement of the ramus-condyle complex with a custom made titanium prosthesis at age 5 years 5 months, and replacement of the artificial condyle by a costochondral graft at age 9 years 7 months. Roentgen examinations were performed at intervals from age 3 years 7 months to age 13 years 8 months. Body length was measured annually.

Main Outcome Measures:

Stereo roentgenograms were digitized by the Department of Orthopedic Surgery, Malmö University Hospital.

Results:

Unexpected lowering of the mandible occurred before treatment. Each procedure had a distinct effect on the direction of mandibular displacement. There was little or no pathological involvement of the functional matrix. Growth of the costochondral graft exceeded growth of the unaffected TMJ. Anterior displacement as well as rotation and tilting of the mandible to the unaffected side accelerated after the age of body peak height velocity.

Conclusions:

Accurate monitoring of mandibular displacement may contribute to a rationale for clinicians’ decision on treatment protocol and reveal compensatory differential growth.
Three-Dimensional Analysis of Morphological Changes in the Maxilla of Patients with Cleft Lip and Palate
Authors: Braumann Bert , Keilig Ludger , Bourauel Christoph , Jäger Andreas
Published: October 2018

Objective

Three-dimensional (3-D) morphological changes in the maxilla of patients with cleft lip and palate (CLP) have been recorded, mainly using two-dimensional cast analyses. Although these seem to be insufficient, no standardized 3-D method has been developed until now. In this study, accuracy, precision, and validity of a newly developed 3-D digital computer-aided procedure to visualize and metrically analyze the growth of the edentulous maxilla of infants with CLP have been evaluated.

Patients

The method was applied to 10 infants with complete unilateral CLP.

Interventions

Consecutive casts of the maxilla (1 week and 3, 6, and 12 months) of each patient were optically measured with a 3-D laser scanner. Following digitizing, the casts were computer reconstructed, aligned, and superimposed using specialized computer software. The distances between the surfaces were measured. Additionally, the surfaces were segmented perpendicular to the alveolar crest, the reference points being C1, C1′, C2, C2′, and I. The volumes of the resulting segments were determined and compared with one another.

Results

The newly developed analysis enables a visualization of the extent and direction of morphological changes in the maxilla of infants with CLP. With this method it is possible to quantify these changes of the volume of defined alveolar segments.

Conclusions

The 3-D analysis developed is an ideal tool for the examination of 3-D morphological changes in the edentulous maxilla of patients with CLP. The results will serve as the starting point for a longitudinal study on the efficacy of different methods, not only of presurgical infant orthopedics but also of surgical procedures.
Alveolar Bone Grafting in Patients with Complete Clefts: A Comparative Study between Secondary and Tertiary Bone Grafting
Authors: Dempf Rupert , Teltzrow Thomas , Kramer Franz-Josef , Hausamen Jarg-Erich
Published: October 2018

Objective

This investigation evaluated the results of alveolar bone grafting in patients with complete clefts, comparing secondary alveolar bone grafting (during the mixed stage of dentition) versus tertiary bone grafting (after completion of the second stage of dentition).

Design

This was a retrospective study. Of 140 osteoplasties, which all were operated according to the same standardized surgical technique, a clinical and roentgenological follow-up investigation was carried out in a collective of 85 osteoplasties. Clinically we searched for oronasal fistulae, assessed the periodontal status, determined the extent of the gingival attachment in the area of the osteoplasty, and searched for vertical growth disturbances in the area of the osteoplasty. Roentgenologically the height of the alveolar bone in the former clefted area was ascertained.

Setting

Records were obtained from a clinical and radiological study of the Department of Oral and Maxillofacial Surgery of the Medical University of Hannover (Germany).

Interventions

All patients were operated with the same standardized surgical method. The osteoplastic bridging of the alveolar cleft was performed via a vestibular gingival marginal incision and exclusively by grafting of cancellous bone from the iliac crest.

Results

The best results of alveolar bone grafting in cases of secondary osteoplasty were obtained when the lateral incisor or canine had grown into the transplant and had led to a functional stress of the transplanted bone. Approximately good results were to be found in tertiary osteoplasty when the transplanted bone had been stressed functionally through a dental implant. Comparing the secondary with the tertiary osteoplasty, there was a trend of lower resorption in secondary osteoplasty.

Conclusions

Secondary osteoplasty should represent an integral component of any concept for the comprehensive treatment of patients with cleft lip and palate.
Ultrasound Diagnosis of Craniosynostosis
Authors: Miller Carolyn , Losken H. Wolfgang , Towbin Richard , Bowen A'delbert , Mooney Mark P. , Towbin Alex , Faix Richard S.
Published: October 2018

Objective

To retrospectively study prenatal ultrasound images of patients with craniosynostosis to determine the extent to which prenatal diagnosis is possible.

Method

Prenatal ultrasound images of 19 patients with postnatally diagnosed metopic or coronal suture craniosynostosis were retrospectively reviewed. The 26 ultrasound examinations obtained were compared with normal images and tables of gestation.

Results

It was not possible to diagnose craniosynostosis in the first trimester. In the second trimester, Kleeblattschädel was diagnosed at 20.5 weeks. A multilobular shape to the skull and diastasis of the frontotemporal suture was identified. In a second child with Kleeblattschädel, the cephalic index was above normal 86.4 (normal range 70 to 86), and the head circumference to abdominal circumference was increased. In the third trimester, the head shape deformation was more obvious. Brachycephaly diagnosis was made in the second trimester. In Crouzon syndrome the hypertelorism was identified at 19.9 weeks. Plagiocephaly was diagnosed at 21.4 weeks. In trigonocephaly the reduced cephalic index was noted at 18.8 weeks. In the third trimester, the deformity was diagnosed in all cases.

Conclusion

No diagnosis of craniosynostosis was made in the first trimester. In the second trimester, it was possible to diagnose Kleeblattschädel, tri-gonocephaly, brachycephaly (bilateral coronal suture craniosynostosis), and plagiocephaly (unilateral coronal suture craniosynostosis) in nine of the examinations. In the third trimester and at term, it was possible to diagnose previously listed conditions from six of the seven examinations obtained. Klee-blattschädel was suspected during original examination. A total of 15 examinations of 26 were correctly diagnosed during this investigation.
Validity of the HfmTransgenic Mouse as a Model for Hemifacial Microsomia
Authors: Cousley Richard , Naora Hiroyuki , Yokoyama Minesuke , Kimura Minoru , Otani Hiroki
Published: October 2018

Objective

Our comprehension of hemifacial microsomia (HFM) has been hindered by its diverse phenotype and unclear etiopathogenesis. The conventional view has been that HFM's facial defects result from embryonic hemorrhages in the region of the first and second branchial arches. A more recent model based on a transgenic mutation of a locus termed Hfm (B1 to B3 on chromosome 10) appears to provide an insight into HFM causation. This study investigated the validity of this model by examining the Hfm craniofacial phenotype and histological development of the embryonic head (E13.5 to 17.5).

Results

The results confirmed that although the loss-of-function mutation was transmitted in an autosomal dominant manner, the penetrance rate was significantly reduced and only Hfm heterozygotes were viable. The observations here extend the Hfm phenotype beyond microtia and jaw asymmetry to include structural and positional anomalies affecting the external auditory meatus, middle ear, cranial base, maxilla, and pharyngeal structures. Temporo-mandibular joint (TMJ) development and palatal shelf fusion were also affected in a small number of cases. In addition, some Hfmembryos displayed a novel finding: transposition of the developing inner ear between the tubotympanic recess and cranial base.

Conclusions

These craniofacial features, especially the ear anomalies and facial asymmetry indicate that the Hfm transgenic mouse represents a useful model for the HFM-microtia spectrum. In particular, it supports the hypothesis that at least a proportion of HFM anomalies has a genetic causation mediated via mesenchymal disruptions and possibly embryonic hemorrhages.
Functional Analysis of Cleft Lip and Palate Patients with Modified Rakosi Method
Authors: Akcam M. Okan , Altiok Tunç , Özdiler F. Erhan
Published: October 2018

Objective

The aim of this study was to investigate the mandibular functions of patients with cleft lip and palate (CLP) by means of modified Rakosi Functional Analysis and to test whether there was a risk of tendency to temporo-mandibular joint disorder when compared with Angle Class I non-CLP patients.

Design

Fifteen patients with unilateral CLP with a mean age of 12.2 ± 1.91 years and randomly selected 15 Angle Class I orthodontic patients with a mean age of 10.5 ± 2.01 years as a control group. None of the subjects had complaint of symptoms of temporomandibular joint disorder in their histories. All subjects were evaluated using modified Rakosi Functional Analysis method in accordance with a scoring system of the findings.

Main outcome Measures

Intra- and extracapsular clinical functional surveys were taken. A Mann-Whitney U test was used to evaluate the differences between the CLP and non-CLP groups.

Results and Conclusion

There was a significant difference (p < .01) between the distributions of the total scores of the two groups. A significant difference (p < .05) in right lateral movement was also observed between the two groups. However, there were no significant but borderline differences in clicking, crepitus, and maximum mouth opening findings between the two groups. Because these results do not explain the reason for the aforementioned differences, further studies seem essential.
Airway Obstruction following Palatoplasty: Analysis of 247 Consecutive Operations
Authors: Antony Anuja K. , Sloan Gerald M.
Published: October 2018

Objective and Methods

Between February 1987 and September 1997, 247 patients underwent primary repair of a cleft of the secondary palate by one surgeon, using the double-opposing Z-plasty (Furlow) technique. This retrospective study reviews perioperative and postoperative airway compromise among these patients. The purposes of this study were to identify factors associated with airway obstruction following palatoplasty and to analyze the management of those patients. Although infants experiencing airway problems following Wardill-Kilner and Von Langenbeck palatoplasty have been described, airway complications in a group of Furlow repair patients has not been previously reported.

Results

Fourteen patients (5.7%) had airway problems. The average age of these patients was 18 months, which was not significantly different from those without airway problems. Airway obstruction occurred as late as 48 hours after the completion of surgery. Twelve of the 14 patients had severe airway compromise requiring continued postoperative intubation, reintubation, or tracheostomy (one). There were no deaths. Thirteen of the 14 patients with postoperative airway problems (93%) had other congenital anomalies in addition to clefting, a named congenital disorder, or both. Seven of those 13 had Pierre Robin sequence. In contrast, only 40 of the 233 patients without airway problems (17%) had additional congenital anomalies or named disorders. Presence of other congenital anomalies was associated with a significantly increased risk of airway obstruction (p = .005).

Conclusion

Patients with cleft palate with the Pierre Robin sequence or other additional congenital anomalies had an increased risk of airway problems following palatoplasty. Awareness of this risk permits identifying those patients prior to surgery so that they can be monitored and managed appropriately, minimizing the likelihood of major complications or death.
Beyond Easy Answers: Prenatal Diagnosis and Counseling during Pregnancy
Authors: Strauss Ronald P.
Published: October 2018

Objective

The advancing sophistication and availability of prenatal diagnostic technologies, such as transvaginal ultrasound, chorionic villus sampling, amniocentesis, and alpha feto-protein testing, have increased the medical capacity to detect genetic and congenital conditions during pregnancy. This paper raises many social and ethical questions about how families, craniofacial teams, and society respond when a prenatal diagnosis is made and considers the ethical and social issues around counseling, managing information, and making decisions.

Design

Ethical and sociological analysis.

Setting

Implications examined on the societal, health professional, and family level.

Results

Families and health professionals often manage ambiguity, uncertainty, and complex decision making in facing a prenatal diagnosis. Embedded in parental and clinical decisions are values about children with birth defects. Families are making decisions about whether to bear or abort an affected fetus on the basis of their perceptions of the impairment and on their expectation of the burden involved for the family and the child. On a broader, societal level, pressures to conform and minimize human differences are apparent in bio-medical interventions, the Human Genome Project, advertising and media images, and social pressures to normalize disabilities. How society deals with prenatal diagnosis will impact upon social values; moral, legal, and ethical perspectives; and on health policy.

Conclusion

Prenatal diagnostic technologies raise complex ethical, family, policy, and legal issues that have broad implications for the lives of children born with special health care needs, including children with cleft lip and palate.
Prenatal Diagnosis of Cleft Lip and Palate: Detection Rates, Accuracy of Ultrasonography, Associated Anomalies, and Strategies for Counseling
Authors: Jones Marilyn C.
Published: October 2018

Objective

As ultrasound becomes more widely utilized in pregnancy and imaging technology improves, cleft lip and palate will become more commonly identified in prenatal life. In efforts to meet the needs for information regarding cause and management, pregnant women and their partners are increasingly referred to cleft and craniofacial treatment programs. This group of patients provides unique challenges to professionals unfamiliar with the issues inherent to this population. Information regarding the extent of the defect and the absence of associated abnormalities is usually incomplete. Treatment teams may be uncomfortable with the possibility that couples may choose not to continue a pregnancy on the basis of what they hear. Currently between 14% and 25% of cleft lip, with or without cleft palate, is detected antenatally. About 12% of presumably isolated clefts will be one feature in a broader pattern of malformation. This article reviews the current status of ultrasound in the detection of clefts during pregnancy and outlines a strategy for counseling based on the author's experience in both a prenatal diagnosis program and a cleft-craniofacial treatment team.
Dental Development of Permanent Lateral Incisor in Complete Unilateral Cleft Lip and Palate
Authors: Ribeiro Luciana Lourenço , Neves Lucimara Teixeira Das , Costa Beatriz , Gomide Marcia Ribeiro
Published: October 2018

Objective

The objective of this retrospective study was to verify whether the root development of the permanent lateral incisor on the cleft side was delayed, compared with its contralateral tooth in subjects with complete unilateral cleft lip and palate.

Setting

Craniofacial Anomalies Rehabilitation Hospital (HRAC)-University of São Paulo-Bauru, Brazil.

Patients

A sample of 98 panoramic radiographs of HRAC patients with complete unilateral cleft lip and palate was selected, regardless of sex and race.

Main outcome Measure

Panoramic radiographs were analyzed and the root development stage of the cleft-side permanent lateral incisor was compared with its contralateral tooth.

Results

Overall, the permanent cleft-side lateral incisor was significantly delayed in root development in relation to the noncleft side contralateral tooth (p < .05). However, no significant differences were observed between boys and girls.

Conclusions

Root development of the permanent cleft-side lateral incisor was delayed, compared with its contralateral tooth. The cleft itself may play an important role in this delay because the results from boys and girls were very similar.
Modifying the Two-Stage Cleft Palate Surgical Correction
Authors: Chait Laurence , Gavron Gerald , Graham Cathy , Noik Errol , De Aguiar Gavin
Published: October 2018

Objective

This paper reports the experience with a two-stage approach to surgical correction of the complete cleft palate, wherein timing of the second stage is dependent on the judgment of the speech pathologist and the orthodontist together with the surgeon.

Patients

Of a total of 35 patients having complete unilateral clefts a sample of 22 were available for postsurgical assessment. The first-stage repair of the palate was carried out at an average age of 10.7 months (range 6 to 17 months), and the second-stage repair of the residual cleft was completed at an average age of 32.7 months (range 26 to 34 months).

Interventions

The first-stage repair of the soft palate defect involved mobilizing two short posteriorly based flaps, which extend onto the posterior quarter of the hard palate thus including up to 1 cm of mucoperiosteum. Careful freeing of the muscle is followed by an intravelar veloplasty. The later closure of the residual cleft involved turnover hinge flaps and small mucoperiosteal flaps.

Results

Eighty-seven percent of the sample had good to excellent speech as assessed by the Great Ormond Street screening method. Only two patients showed evidence of recessive maxillae with Class III malocclusions.

Conclusions

A two-stage surgical closure of the palate using this procedure would appear to confer several valuable advantages to the patient. These include favorable outcomes for speech in the large majority of cases and minimal adverse effects on the growth of the midface region.
The Correlation between Nasalance and a Differentiated Perceptual Rating of Speech in Dutch Patients with Velopharyngeal Insufficiency
Authors: Keuning Kornelis H.D.M. , Wieneke George H. , Van Wijngaarden Hans A. , Dejonckere Philippe H.
Published: October 2018

Objective:

The correlation between the nasalance score and the perceptual rating of several aspects of speech of speakers with velopharyngeal insufficiency (VPI) by six speech-language pathologists was evaluated.

Procedure:

The overall grade of severity, hypernasality, audible nasal emission, misarticulations, and intelligibility were rated on visual analog scales. Speech samples with a normal distribution of phonemes (normal text [NT]) and those free of nasal consonants (denasal text [DT]) of 43 patients with VPI were used. Mean nasalance scores were computed for the speech samples, and Spearman correlation coefficients were computed between the mean nasalance score and the five parameters of the differentiated rating.

Setting:

The Institute of Phoniatrics, Utrecht University Hospital, The Netherlands.

Results:

The correlation coefficient between the mean nasalance and the perceptual rating of hypernasality ranged among judges from .31 to .56 for NT speech samples and .36 to .60 for DT speech samples. Only small differences were found between speech pathologists with and without expertise in cleft palate speech. The rating of the overall grade of severity appeared to correlate quite well with the rating of the intelligibility (rNT = .77, rDT = .79). Lower correlation coefficients, ranging from .34 to .71, were found between overall grade of severity and hypernasality, audible nasal emission, and mis-articulations.

Conclusions:

A low correlation between the nasalance and the perceptual rating of hypernasality was found. The parameter overall grade of severity appeared to be determined mainly by the parameter intelligibility. Expertise in rating of cleft palate speech does not guarantee a high correlation between instrumental measurement and perceptual rating.
Spectral Analysis of Prespeech Sounds (Spontaneous Cries) in Infants with Unilateral Cleft Lip and Palate (UCLP): A Pilot Study
Authors: Wermke Kathleen , Hauser Christine , Komposch Gerda , Stellzig Angelika
Published: October 2018

Objective:

The objectives of the present study were: (1) to analyze the cry features of infants with cleft lip and palate (UCLP) by means of spectral analysis, (2) to describe changes of the acoustic parameters from birth until 9 months of age, and (3) to compare these data with existing cry data of infants without cleft (control group).

Design:

The study was designed on a interdisciplinary, prospective, and longitudinal basis.

Setting:

Interdisciplinary study: (1) Institute of Anthropology at the Humboldt-University, Berlin; (2) Heidelberg University Hospital: Interdisciplinary Cleft Palate and Craniofacial Center.

Patients and Method:

The cry parameters of five patients with complete unilateral cleft lip, alveolar ridge, and hard and soft palate were analyzed from birth to 9 months of age. The patients were treated with the same protocol. At the age of 24 months, sensomotor development was assessed using the KIPHARD test. Perceptual judgment of speech, performed after 36 months of life, included nasal resonance, nasal emission of air, articulation disorders, and speech intelligibility.

Main Outcome Measure:

The cry parameters of fundamental frequency (F0), pitch period perturbation quotient (PPQ), and cry duration (Tsam) were analyzed.

Results:

Contrary to the expectation that laryngeal parameters are not affected by vocal tract malformations, differences of cry parameters were found between the patients with UCLP and the noncleft group. Particularly, the F0 and its short-time variability (PPQ) were affected.

Conclusions:

The preliminary results of this study showed that F0 and PPQ of spontaneous cries are influenced in patients with UCLP, and a cry analysis might become a noninvasive tool for early detection of an at-risk status for neuromuscular development and prediction of an at-risk status for later speech and language acquisition in infants with cleft lip and palate. Future research strategies are outlined.
Incidence and Severity of Obstructive Sleep Apnea following Pharyngeal Flap Surgery in Patients with Cleft Palate
Authors: Liao Yu-Fang , Chuang Ming-Lung , Chen Philip K.T. , Chen Ning-Hung , Yun Claudia , Huang Chiung-Shing
Published: October 2018

Objective:

To investigate the incidence and severity of obstructive sleep apnea (OSA) associated with pharyngeal flap surgery in patients with cleft palate at least 6 months postoperatively and to determine whether age or the flap width had an effect on them. The hypothesis tested in this study was that the severity of OSA associated with pharyngeal flap surgery is greater in children than in adults.

Subjects:

Ten adults, six men and four women, with a mean age of 28.0 years at pharyngeal flap (adult group). Twenty-eight children, 13 boys and 15 girls, with a mean age of 6.3 years at pharyngeal flap (child group).

Design:

A prospective analysis.

Main Outcome Measures:

An overnight polysomnographic study was used to determine the incidence and severity of OSA 6 months after pharyngeal flap.

Results:

The incidence of OSA following pharyngeal flap was high but not significantly different between these two groups (90% in adults and 93% in children, p = 1.000). When OSA was stratified into different levels of severity according to the values of respiratory disturbance index, there were noticeable differences between these two groups (p = .022). In the adult group, eight patients (89%) had mild OSA and 1 patient (11%) had moderate to severe OSA. In the child group, 11 patients (42%) were found to have mild OSA, and 15 patients (58%) had moderate to severe OSA. No relation was found between the flap width and the incidence (p = .435 in adults and .640 in children) or the severity (p = .325 in adults and .310 in children) of OSA in each group.

Conclusions:

Six months following pharyngeal flap surgery, more than 90% of the patients with cleft palate still had OSA. The severity of OSA associated with pharyngeal flap surgery tended to be greater in children than in adults. The flap width was unrelated to the incidence and severity of OSA, no matter in adults or in children.
Occlusal Relationship in Patients with Bilateral Cleft Lip and Palate during the Mixed Dentition Stage: Does Neonatal Maxillary Arch Configuration Predetermine Outcome?
Authors: Perlyn Chad A. , Brownstein Jeffrey N. , Huebner Donald V. , Marsh Jeffrey L. , Nissen Richard J. , Pilgram Thomas
Published: October 2018

Objective:

To determine whether there is any correlation between initial maxillary arch dysmorphology patterns in complete bilateral cleft lip/palate (BCLP) and the subsequent occlusal relationship in early mixed dentition.

Design:

This retrospective study evaluated 41 consecutive patients with complete BCLP who were managed by a single tertiary cleft team between 1983 and 1992. All patients had bilateral synchronous lip adhesions with placement of a passive alveolar molding appliance at 4 to 8 weeks of age; definitive modified Manchester lip repair at 6 to 8 months of age; and a one-stage “two-flap” palatoplasty at 14 months of age. Neither gingivoperiosteoplasty nor early alveolar bone grafting was performed. Four patients required early surgical premaxillary setback because of adverse social reactions to a very protrusive premaxilla. All patients (7 to 9 years) were assessed prior to initiation of first-phase orthodontics (maxillary expansion) in anticipation of alveolar bone grafting. Cleft team records were reviewed to determine initial maxillary arch dysmorphology, treatment, and occlusal relationships in early mixed dentition stage. The initial maxillary dysmorphology was classified using a new grouping based on orientation of the vomer, the intercanine distance, and its symmetry. Patterns of mixed dentition occlusion were then compared with group type, and predication tables were generated. Because midface retrusion is the major dentoskeletal morbidity in cleft patients, angle Class I and II occlusions were designated as “favorable” and Class III as “unfavorable.”

Results:

In early mixed dentition, approximately three-fourths of patients with complete BCLP had favorable occlusion and one-fourth unfavorable occlusion. The occlusal status seemed unrelated to the initial maxillary arch dysmorphology, the use of early passive alveolar molding appliance, or surgical premaxillary setback.

Conclusion:

Patterns of variation within the initial dysmorphology of the maxillary arch in infants with complete BCLP cannot be used to predetermine subsequent molar occlusal relationships in the mixed dentition.
Toward Pathogenesis of Apert Cleft Palate: FGF, FGFR, and TGFβ Genes Are Differentially Expressed in Sequential Stages of Human Palatal Shelf Fusion
Authors: Britto Jonathan A. , Evans Robert D. , Hayward Richard D. , Jones Barry M.
Published: October 2018

Objective:

Critical cellular events at the palatal medial edge epithelium (MEE) occur in unperturbed mammalian palatogenesis, the molecular control of which involves a number of growth factors including transforming growth factor β3 (TGFβ3). Apert syndrome is a monogenic human disorder in which cleft palate has been significantly correlated to the fibroblast growth factor receptor (FGFR) 2-Ser252Trp mutation. We report the relative expression of these genes in human palatogenesis.

Methods:

The expression of the IgIIIa/b and IgIIIa/c transcript isoforms of FGFR2 and the proteins FGFR1, FGFR2, and FGFR3 was studied in situ throughout the temporospatial sequence of human palatal shelf fusion and correlated with the expression of TGFβ3. In addition, the immunolocalization of the ligand FGFs 2, 4, and 7 was undertaken together with the intracellular transcription factor STAT1, which is activated by FGFR signaling.

Results:

FGFRs are differentially expressed in the mesenchyme and epithelia of fusing palatal shelves, in domains overlapping those of their ligands FGF4 and FGF2 but not FGF7. Coexpression is seen with TGFβ3, which is implicated in MEE dynamics and FGF and FGFR upregulation, and STAT1, an intracellular transcription factor that mediates apoptosis.

Conclusions:

The coregulation of molecules of the FGFR signaling pathway with TGFβ3 throughout the stages of human palatal fusion suggests their controlling influence on apoptosis and epitheliomesenchymal transdifferentiation at the MEE. Experimental evidence links FGFR2-IgIIIa/b loss of function with palatal clefting, and these correlated data suggest a unique pathological mechanism for Apert cleft palate.
Midline Skeletodental Morphology in Holoprosencephaly
Authors: Kjær Inger , Keeling Jean W. , Hansen Birgit Fischer , Becktor Karin B.
Published: October 2018

Objective:

The purpose of the present study was to analyze histologically the midline structures in a human fetus with holoprosencephaly and a single median maxillary central incisor.

Methods:

A human male fetus, CRL 137 mm, postconceptional age 18 weeks, with a diagnosis of holoprosencephaly, the cebocephalic type (one nostril), and alobar brain development, was investigated. After radiography, the cranial midline structures were examined histologically.

Results:

The histological examination revealed partial absence of the intermaxillary suture (in the region anterior to the central incisor), absence of the internasal suture, and partial absence of the metopic suture (caudal part). A single midline nasal bone was observed instead of two bilateral nasal bones, and a midline maxillary central incisor was found instead of two bilateral incisors. A short nasal septum, absence of crista galli, and partial absence of cartilaginous tissue anterior to the sella turcica in the region of the presphenoid bone were recorded.

Conclusions:

The study shows that in the affected fetus, there were malformations of midline structures anterior to the sella turcica comprising suture development and cartilage development.
Prenatal and Perinatal Factors Associated with Isolated Oral Clefting
Authors: Wyszynski Diego F. , Wu Tianxia
Published: October 2018

Objective:

To describe medical service utilization and maternal morbid conditions of women who carry offspring with oral clefts (OCs), to describe maternal and offspring complications during birth, and to evaluate postnatal characteristics of their newborns with isolated OCs.

Methods:

Two thousand four hundred thirty-seven patients with isolated OCs and 4871 unaffected matched controls meeting inclusion criteria were selected from the U.S. Natality database for 1997. Matching variables were mother's and father's race and child's race, sex, county of birth, and month of birth. Patients and controls were compared in terms of maternal demographic characteristics, gestational complications, physical characteristics of the newborns, maternal exposure to potential risk factors, and adequacy of prenatal care.

Results:

Although the quality of care was very good in both groups, low gestational age, low birth weight, and low 5-minute Apgar score are more frequent among newborns with OCs than in unaffected controls. Mothers of offspring with OCs are at increased risk, compared with mothers of controls, of having hydramnios or oligohydramnios, eclampsia, and abruptio placenta. Obstetric procedures, such as amniocentesis, electronic fetal monitoring, induction of labor, tocolysis, and ultrasound, and repeat cesarean deliveries are more frequent among mothers of patients than those of controls. At birth, new-borns with isolated OCs are at risk of having hyaline membrane disease and of requiring assisted ventilation, independently of gestational age.

Conclusion:

The results of this large population-based, case-control study suggest that the presence of an OC in the offspring is associated with increased risk for prenatal and perinatal complications in the mother.
A Comparison of Three Methods of Repairing the Hard Palate
Authors: Pigott R. W. , Albery E. H. , Hathorn I. S. , Atack N. E. , Williams A. , Harland K. , Orlando A. , Falder S. , Coghlan B.
Published: October 2018

Objective

To compare growth, speech, and nasal symmetry outcomes of three methods of hard palate repair.

Patients

Consecutive available records of children born with unilateral bony complete cleft lip and palate over the period 1972 to 1992.

Interventions

Identical management of lip, nose, alveolus, and soft palate. Hard palate repair by Cuthbert Veau (CV) from 1972 to 1981, von Langenbeck (vL) from 1982 to 1989, or medial Langenbeck (ML) from 1989 to 1991.

Outcome Measures

For growth: GOSLON yardstick or 5-year model index. For speech: articulation test. Nasal anemometry. For nasal symmetry: Coghlan computer-based assessment. All these measures were developed during the period of data collection but not for this project.

Results

There was a strong trend toward more favorable anteroposterior maxillary growth with the change from CV to vL to ML techniques. This fell short of statistical significance because of the small sample size. There was a significant reduction in cleft-related articulation faults (p = .01) considered to be related to improved arch form. In the absence of improved rates of velopharyngeal insufficiency or nasal symmetry, increased surgical experience was discounted as a significant contribution to improved growth and articulation outcomes.

Conclusions

Reduced periosteal undermining and residual exposed palatal shelf from CV to vL to ML improved incisor relationships and articulation.