bluuebunny/crossref_metadata_2025 huggingface dataset:
Early Maxillary Orthopedics in a Child with an Oblique Facial Cleft
Authors: Stellzig Angelika , Basdra Efthimia K. , Mühling Jochen , Komposch Gerda
Published: October 2018

Objective

Oblique facial clefts are extremely rare. In view of their variabilty, surgical treatment cannot be standardized. To date, early maxillary orthopedics in the treatment of oblique facial clefts have not been reported. The case described here presents a newborn child with a Tessier 3 cleft. Because of the enormous width of the alveolar cleft, a narrowing of the segments was regarded as prerequisite for definitive lip closure. To bring the segments together, an actively working plate with a forward pull was designed.

Conclusion

After a period of 4 weeks, proper alignment and proximity of the segments were achieved, so that definitive lip closure and plastic reconstruction of the remaining soft tissue defect could be easier performed.
Freeman-Sheldon Syndrome: Case Management from Age 6 to 16 Years
Authors: Ohyama Kimie , Susami Takafumi , Kato Yoshiyuki , Amano Hiromi , Kuroda Takayuki
Published: October 2018

Objective

This is the first description of long-term follow-up of a case of Freeman-Sheldon syndrome. Microstomia was treated with a mouth expander for 2 to 3 hours per day before active orthodontic treatment. Separate impressions were necessary in each quadrant of both upper and lower jaws because of limited mouth opening.

Conclusion

Orthodontic treatment improved the patient's Class II malocclusion, which was accompanied by crowding and a deep bite.
Velopharyngeal Incompetence as the Presenting Symptom of Malignant Brainstem Tumor
Authors: Lefaivre Jean-François , Cohen Steven R. , Riski John E. , Burstein Fernando D.
Published: October 2018

Objective

When a patient presents with velopharyngeal incompetence (VPI) without an obvious structural or neurologic cause, the clinician is faced with a diagnostic challenge. We present an 11-year-old male with a long history of VPI who had been referred to our institution for evaluation and treatment.

Results

Detailed clinical examination and work-up revealed a malignant brain-stem tumor. The presenting symptoms of breathiness associated with VPI had been overlooked by several different clinicians in the past. The patient successfully underwent a sphincter pharyngoplasty.

Conclusions

A careful neurologic examination with special attention to the cranial nerves is necessary to identify subtle neurologic deficits and avoid delay in diagnosis. Differential diagnosis of neurogenic VPI is discussed.
Maternal Cigarette Smoking and Oral Clefts: A Meta-analysis
Authors: Wyszynski Diego F. , Duffy David L. , Beaty Terri H.
Published: October 2018

Objective

A meta-analysis was performed to estimate the association between maternal cigarette smoking and the risk of having a child with a nonsyndromic oral cleft (NSOC).

Design

Studies published from 1966 through 1996 were retrieved using MEDLINE, Current Contents, bibliographies, and other sources. MEDLINE and Current Contents search terms included “oral clefts,” “cigarette smoking,” “birth defects,” and “congenital malformations.” Cohort and case-control studies that enrolled oral cleft patients [cleft lip with or without cleft palate (CL/P), cleft palate (CP), or both] and controls, and presented information on maternal cigarette exposure during pregnancy were included in the analysis. Descriptive and outcome data from each study were independently abstracted by two authors.

Results

The overall odds ratio of the 11 studies satisfying criteria was 1.29 [95% confidence interval (CI), 1.18 to 1.42] for CL/P and 1.32 (95% CI: 1.10 to 1.62) for CP, Indicating a small increased risk of having a child with either a CL/P or a CP for mothers who smoke during the first trimester of the pregnancy.

Conclusions

These analyses suggest a small but statistically significant association between maternal cigarette smoking during the first trimester of gestation and increased risk of having a child with a CL/P or CP.
Embryologic Features of Term Fetuses and Newborns in CL/Fr Mice with Special Reference to Cyanosis
Authors: Kadowaki Shingo , Sakamoto Michiko , Kamiishi Hiroshi , Tanimura Takashi
Published: October 2018

Objective

The objective of this study was to clarify the embryologic features of CL/Fr mice in relation to cyanosis.

Method

Orofacial examinations were conducted on 18-day-old term fetuses and newborns. Except for open eyelids in a few cases, all of the external abnormalities in this strain were cleft lip and/or palate (CLP).

Results

The CLP incidence in term fetuses was 28.4%. The body and placental weights were not different between CLP(+) and CLP(-) fetuses. Several types of CLP were found; the most frequent was bilateral complete CLP (60%) followed by left complete (16%). Bilateral complete CL, which is the most severe CL type, was associated with poorly developed secondary palate and wide cleft. In newborns, 39.4% of CLP(+) mice showed cyanosis, where no CLP(-) mice had such signs. The body weight of cyanotic mice with CLP was significantly lighter than that of noncyanotic mice with CLP. Cyanotic mice had severe types of CLP and unelevated palatal shelves.

Conclusion

We suggest that mechanical airway obstruction may be one of the causes of cyanosis in newborn CL/Fr mice.
Development of Infants and Toddlers with Clefts from Birth to Three Years of Age
Authors: Neiman Gary S. , Savage Hallie E.
Published: October 2018

Objective

The purpose of this study was to use caregiver report measures to describe the developmental status of infants and toddlers with clefts.

Method

Developmental assessment data were obtained on 186 infants and toddlers with cleft lip (n = 48), cleft palate (n = 46), and cleft lip/palate (n = 92) at one of the following age categories: 5 months (n = 47), 13 months (n = 46), 25 months (n = 47), and 36 months (n = 46). Developmental assessment measures used were the Kent Infant Developmental Scale and the Minnesota Child Development Inventory, both caregiver reports. Data were analyzed in separate 2-between ANOVAs (age x cleft type) for each developmental domain according to developmental assessment measure. Further, results were examined relative to the normative sample.

Results

The ANOVA results indicated that at 5 months, lower motor and self-help developmental quotients (DQs) were evident compared to the 13-month-old level. When compared to the normative sample, the 5-month-old infants exhibited ‘at-risk/delayed’ development on the motor, self-help, and cognitive domains, and as reflected on their full-scale scores, depending on the cleft type. Infants at 13 and 25 months were within normal limits in all developmental domains, with the exception of the 13-month-old infants with cleft palate, who demonstrate ‘at-risk’ development in the motor domain. At 36 months of age, all toddlers demonstrated significantly lower developmental performance in the fine motor, gross motor, and expressive language domain compared to the 25-month-old toddlers. Toddlers with cleft palate exhibit ‘at-risk/delayed’ development in the expressive language domain at 36 months.

Conclusion

Data are discussed relative to the events surrounding team management of clefts, Including surgery, middle-ear problems, and feeding difficulty.
Satisfaction with Facial Appearance among Subjects Affected by a Cleft
Authors: Thomas Peter T. , Turner Sarah R. , Rumsey Nicky , Dowell Tom , Sandy Jonathan R.
Published: October 2018

Objective

This study examined the satisfaction of patients with clefting and their parents with facial appearance and how this alters with age. The relationship between satisfaction with appearance and psychosocial functioning was also examined.

Design

Prospective

Setting

Subjects were recruited for the study from nine hospital-based clinics.

Patients, Participants

All subjects had some type of cleft and were 10, 15, or 20 years of age. In all, 111 subjects with clefting and 62 parents were included.

Main Outcome Measures

Facial appearance was rated on a subjective ordinal scale of 1 to 7; psychosocial adjustment was measured with the Childhood Experience Questionnaire.

Results

Self-satisfaction with appearance among the 10- and 15-year-old subjects correlated with their psychosocial adjustment (p = .027). The 20-year-old subjects were, on average, significantly more satisfied with their appearance than the 10- and 15-year-olds (p = .009 and p = .012, respectively). However, some 20-year-old subjects remained greatly dissatisfied with aspects of their facial appearance. Subjects with visible anomalies were significantly more dissatisfied with their appearance than subjects with invisible anomalies (p = .035). The 15-year-old subjects were identified as being significantly more dissatisfied with appearance than their parents (p = .005).

Conclusions

Subjects affected by a cleft with visible Impairments are more dissatisfied with their facial appearance than are subjects with invisible impairments. Satisfaction with facial appearance among 10- and 15-year-old subjects with a cleft may be associated with their self-reported levels of psychosocial functioning. Measuring self-satisfaction with appearance may help to identify subjects at risk from adjustment problems.
Mandibular and Maxillary Asymmetry in Individuals with Unilateral Cleft Lip and Palate
Authors: Laspos Christodoulos P. , Kyrkanides Stephanos , Tallents Ross H. , Moss Mark E. , Subtelny J. Daniel
Published: October 2018

Objective

This study was conducted to evaluate the degree of maxillary and mandibular asymmetry in the verticle and transverse planes seen in posteroanterior cephalometric radiographs relative to chronologic age in postoperative complete UCLP patients compared to controls.

Method

Mandibular and nasomaxillary asymmetry was retrospectively studied in complete unilateral cleft lip and palate (UCLP) and noncleft individuals (controls) by means of posteroanterior cephalometric analysis. All the UCLP patients available (total 40) and randomly selected noncleft controls (total 142) were Included in the study. The UCLP patients had undergone lip and palate reconstruction in Strong Memorial Hospital, University of Rochester, Rochester, New York, and orthodontic treatment in the Department of Orthodontics, Eastman Dental Center, Rochester, New York. The controls were selected based on the age that treatment was initiated and were treated in the department for various malocclusions; none had undergone maxillary expansion or surgical treatment. The asymmetry assessed on mixed longitudinal records of the patients with UCLP was analyzed relative to three chronologic age groups and compared to the controls. In addition, mandibular asymmetry was correlated to maxillary asymmetry in UCLP individuals to investigate possible growth patterns between the two jaws.

Results

Mandibular asymmetry in UCLP individuals was found to increase with growth and time and peaked at post-pubertal growth-spurt stages. The cleft subjects were more asymmetric than controls in all stages of growth. Mandibular asymmetry followed the affected maxilla closely, indicating a parallel growth pattern of the jaws.

Conclusion

The unilateral cleft lip and palate patients manifested asymmetry of the mandible. This asymmetry develops in a parallel pattern with the affected maxilla, suggesting that early evaluation and treatment of the anomalies in the nasomaxillary skeleton as well as in the mandible is necessary when treating unilateral cleft lip and palate individuals.
The Jaw Index: New Guide Defining Micrognathia in Newborns
Authors: Van Der Haven Ibo , Mulder J. Wiebe , Van Der Wal Karel G.H. , Hage J. Joris , De Lange-De Klerk Elly S.M. , Haumann Th. J.
Published: October 2018

Objective

Newborns with glossoptosis due to micrognathia can suffer from life-threatening respiratory distress and severe feeding difficulties. These characteristic features are found in newborns with Pierre Robin sequence. In the relevant literature, no concensus concerning diagnosis for these childern can be found. A guide defining micrognathia can be helpful in the diagnosis of glossoptosis and possible airway obstruction.

Method

The jaw index is obtained in newborns by measuring three facial dimensions. In a series of 100 healthy neonates the standard measures were defined.

Results

The average jaw index in children with Pierre Robin sequence differs significantly from the reference group and was found to be over 3.6 times the normal value.

Conclusions

Micrognathia can be defined with the Jaw index.
Robin Sequence: Obstructive Sleep Apnea following Pharyngeal Flap
Authors: Abramson David L. , Marrinan Eileen M. , Mulliken John B.
Published: October 2018

Objective

We reviewed 24 children with Robin sequence who underwent cleft palate repair.

Method

All patients were 5 years of age or older at the time of review, allowing for accurate assessment of speech in relation to velopharyngeal function. All infants had palatal closure between 9 and 14 months of age, either V-Y repair (n = 16) or von Langenbeck repair (n = 8).

Results

Only 1 of 16 children who had V-Y repair had borderline velopharyngeal dysfunction (VPD). For reasons that are unclear, in the von Langenbeck repair group, six of eight children had VPD, and four of six underwent pharyngeal flap. Three additional patients with nonsyndromic Robin sequence had palatoplasty and subsequent pharyngeal flap. Six of the combined total of seven children with nonsyndromic Robin sequence developed obstructive sleep apnea and required flap take-down.

Conclusion

Since conventional pharyngeal flap for VPD in nonsyndromic Robin sequence children resulted in a high incidence of obstructive sleep apnea, alternative management should be considered: modification of the standard pharyngeal flap, palatal lengthening (V-Y or double-opposing Z-plasty), or construction of a speech bulb.
Relationship between Lower-Lip Fistulae and Cleft Lip and/or Palate in Van der Woude Syndrome
Authors: Onofre Mirian Aparecida , Brosco Heli Benedito , Taga Rumio
Published: October 2018

Objective

This study was conducted to evaluate the relationship between fistulae of the lower lip and cleft lip and/or palate in patients with Van der Woude syndrome.

Methods

The medical records of 11,000 patients with cleft lip and/or palate registered at the Cleft Lip-Palate Research and Rehabilitation Hospital, University of São Paulo, Bauru were reviewed. Of these patients, 133 (1.2%) presented with Van der Woude syndrome.

Results

Of the 133 patients, 88 (66.2%) exhibited full clefts, 22 (16.5%) only cleft lip, and 23 (17.3%) only cleft palate. The lower-lip fistulae observed in these 133 patients were bilateral symmetric in 66 (49.7%), bilateral asymmetric in 42 (31.6%), microform In 19 (14.3%), median in 5 (3.8%), and unilateral in 1 (0.7%).

Conclusion

This population sample appears to exhibit the previously published tendency for bilateral, unilateral, or mixed-type congenital fistulae to be associated with cleft lip with or without cleft palate, while so-called microforms or conic elevations are almost exclusively associated with cleft palate.
Location of Alveolar Clefts Relative to the Incisive Fissure
Authors: Lisson Jörg A. , Kjær Inger
Published: October 2018

Objective:

The purpose of this study was to investigate, in a normal human fetal sample, the development of the anterior area of the palate. Emphasis has been placed on the bilateral epithelial fusion lines between the soft-tissue palatal shelves and the premaxillary mucosa, and on the relationship between these fusion lines and the locations of the incisive fissures and of the tooth buds. The goal was to evaluate how the location of clefts in cleft lip and palate (CLP) patients interferes with tooth buds that are often affected in clefting.

Design:

Nineteen maxillas of human fetuses underwent histologic examination after serial horizontal sectioning. Superimpositions of tracings from photographs of the histologic sections yielded information on the maxillary morphology at different levels.

Results:

After elevation and fusion of the soft-tissue palatal shelves, the palatal fusions were Y-shaped. During subsequent osseous palate development, including formation of the incisive fissure and intermaxillary suture, the Y-shaped epithelial fusions disintegrated. The locations of the anterior arms of the epithelial Y and of the incisive fissure did not coincide.

Conclusions:

The incisive fissure is likely not the location of clefts in CLP. The location of anterior palatal clefting in CLP patients follows the original course of the epitheilal fusion between the palatal shelves and the premaxillary mucosa found in the region of the lateral incisors.
Maxillary Growth following Atelocollagen Implantation on Mucoperiosteal Denudation of the Palatal Process in Young Rabbits: Implications for Clinical Cleft Palate Repair
Authors: Fujioka Masaki , Fujii Tohru
Published: October 2018

Objective:

The implantation of atelocollagen matrix on the denuded surface of palatal bone following cleft palate repair has been used because it enhances wound healing. This study was performed to determine whether the beneficial effect of atelocollagen matrix implantation on the prevention of scar tissue contraction also inhibits the scar's interference with the growth of maxillary bone.

Method:

Fifty New Zealand White rabbits (aged 4 weeks) underwent palatal mucoperiosteal denudation, and atelocollagen matrix was implanted on the left palatal process. The opposite side was left open as a control.

Results:

Histopathologically, the implantation side exhibited early infiltration of mononuclear cells and fibroblasts, and better growth of connective tissue strands and epithelium. in addition, the formation of rete ridges were seen that were similar to the normal mucosa. The bone of the atelocollagen-implanted side was covered with regenerated periosteum-like layers, but that of the control side was lined by granulation tissue, suggesting the existence of continuous inflammation on the periosteal region. When the animals reached adulthood (aged 24 weeks), the areas of scars and palatal processes, palatal shelf width, molar teeth incline, and bone mineral contents were measured and compared between sides. The atelocollagen-applied scars showed less contraction, the area and width of atelocollagen-implanted palatal processes showed more satisfactory growth, and the dental arch deformity was suppressed in comparison with the control side.

Conclusions:

Our results suggest that the use of atelocollagen matrix on the denuded bone surface following cleft palate repair decreases the scar's effect on maxillary growth.
Application of Thiamine in Preventing Malformations, Specifically Cleft Alveolus and Palate, during the Intrauterine Development of Rats
Authors: Bienengräber Volker , Fanghänel Jochen , Malek Fathi A. , Kundt Günther
Published: October 2018

Objective:

Animal experiments were conducted to test the reproducibility of previously documented antiteratogenic effects of thiamine on cleft formation in the craniofacial system.

Design:

Thirteen gravid Wistar rats carrying 98 fetuses were given the hydrazine derivative procarbacine (200 mg/kg BW) on the fourteenth day postconception (PC) to induce malformations, chiefly cleft alveolus and palate (day of determining presence of sperm was called the first day PC). Seven of the treated gravid rats carrying 48 fetuses were additionally given a daily dose of 200 mg/kg thiamine from the thirteenth to the nineteenth day PC.

Outcome Measures:

A comparative analysis of the fetuses in both experimental groups was conducted externally and, for the skeleton, macroscopically using special staining techniques; the heads were analyzed using successional histologic sections; bodies were examined stereomicroscopically using the razor-blade sectioning technique.

Results:

In 12 of the 16 parameters evaluated, no statistically significant differences were found between experimental groups. in some cases, we even observed an amplifying effect of thiamine on the development of malformations in the rat strain used in our study.

Conclusions:

Because several previous authors have repeatedly described treatment with thiamine as one of the sufficient prophylactic measures in slowing the development of viscerocranial malformations, especially cleft afveolus and palate, it is of utmost importance that the timing of treatment and dosage of thiamine be taken into consideration not only in animal experiments but also when applying results to humans.
Elevated Levels of Transforming Growth Factors Beta 2 and Beta 3 in Lambdoid Sutures from Children with Persistent Plagiocephaly
Authors: Lin Kant Y. , Nolen Amber A. , Gampper Thomas J. , Jane John A. , Opperman Lynne A. , Ogle Roy C.
Published: October 2018

Objective:

To analyze the pertinent history and physical findings specific to the subset of patients with a progressive posterior skull deformity, requiring surgery to correct their deformity.

Patients:

Since the Academy of Pediatrics issued its recommendation on supine positioning of infants to prevent sudden infant death syndrome (SIDS) in 1992, 73 children have presented to the University of Virginia Craniofacial Anomalies Clinic with posterior-skull deformities. The majority were successfully managed with conservative therapy, but in six patients, the deformity was severe and persistent, requiring surgical correction. All six children were older (7.5–12 mo), presenting with more severe morphologic appearances and a higher incidence of associated neurodevelopmental delay. Three had family backgrounds of isolated craniosynostosis.

Methods:

Characteristics of these patients were examined to determine why they may have differed from those that responded to conservative management. Immunohistochemical staining of their lambdoid sutures was performed.

Results:

Significantly increased staining for TGF-ß2 and TGF-ß3, potent stimulators of bone cell growth and differentiation, was seen in all ‘affected’ sutures from the flattened side of the skull, compared to unaffected sutures from the protruding side of the skull—a pattern similar to that seen during normal bony obliteration of calvarial sutures.

Conclusion:

The majority of patients with posterior plagiocephaly associated with positioning responded to conservative management, while a small subset of patients with persistent posterior skull deformation required surgical intervention. A genetic basis for the latter patients’ persistent plagiocephaly, rather than positioning, cannot be ruled out. Genetics, prolonged external pressure against the sutures, or a combination of these factors may lead to permanently raised levels of growth factors in ‘affected’ sutures.
Breast Feeding for Cleft Lip and Palate Patients, Using the Hotz-Type Plate
Authors: Kogo Mikihiko , Okada Gen , Ishii Shouichirou , Shikata Megumi , Iida Seiji , Matsuya Tokuzo
Published: October 2018

Objective:

Oral-cavity feeding movements were analyzed during sucking and used to modify a Hotz-type plate to facilitate sucking in infants with cleft lip and palate.

Design:

Sucking movements were analyzed using lateral view cine radiography as three adults fed from a bottle. A Hotz-type plate was then modified to better isolate the oral cavity and to occlude the oral airway. The plate was then utilized indirect breast feeding by 10 babies.

Outcome Measures:

Sucking rate, amount of milk taken, and duration of use of the plate were assessed.

Results:

Wearing this plate, four babies with cleft lip and palate could suck their mother's breast, drinking about 22 g/trial.

Conclusions:

Although supplemental bottle feeding was required to provide enough nourishment, this is the first step to reaching ideal breast feeding situation for these patients.
Titanium Mesh and Bone Dust Calvarial Patch during Cranioplasty
Authors: O'Broin Eoin S. , Morrin Martina , Breathnach Eamonn , Allcutt David , Earley Michael J.
Published: October 2018

A technique of filling calvarial detects using bone dust reinforced with titanium Micro Mesh (Leibinger®) was investigated using plain x-ray films and computed tomography (CT) in seven consecutive patients (age range, 7–21 years). The aim of our study was to assess whether, in the presence of the titanium, bone dust harvested with a power burr promotes persistent ossification that is comparable with adjacent bone. The mesh was localized by standard skull plain x-ray films, and orthogonal CT scans were obtained at between 9 and 18 months post-operatively. An ultrahigh-resolution algorithm was used to detect neoossification on either side of the Micro Mesh (1-mm collimation, 330-mA and 120 KV(p) at the center of 400 HU, and window width of 2000 Hu). The mesh induced minimal streak artifact. Virtually no new bone formation was seen. It appears that bone dust was completely reabsorbed in this patient group even in the presence of semi-rigid fixation.
The Lambda Flap for Secondary Cleft Lip Repair
Authors: Tange Ichiro
Published: October 2018

Objective:

A modified Abbe flap of the lambda figure type, designed by the author and used before or after secondary cleft lip repair in 146 consecutive cases since 1990 is described.

Design:

This series consisted of 71 cases with unilateral deformity and 75 cases with bilateral deformity at adolescent or adult ages. The technical details of this method and representative cases with the results are shown. The philtrum is created by incising the two branches of the lambda flap obliquely at 45 degrees to the skin surface in the lower lip, then matching them in the central recipient bed of the deficient upper lip.

Results and Conclusions:

The resultant upper lip is not only full and slack, but also attractive with an acute cubic contour of the philtrum. Furthermore, the residual scar at the donor site is concealed in the mentolabial fold.
Self-Perceived Facial Appearance and Psychosocial Adjustment in Preadolescents with Craniofacial Anomalies
Authors: Pope Alice W. , Ward Johanna
Published: October 2018

Objective:

To identify aspects of psychosocial adjustment related to the self-perceived facial appearance of preadolescents with craniofacial abnormalities.

Design:

Concurrent relationships were evaluated using a within-group correlational design.

Participants:

Participants were 24 patients, aged 11 to 13, of a major craniofacial center and their parents who were contacted by telephone and agreed to participate.

Main Outcome Measures:

Self-report and parent-report questionnaires assessing psychosocial adjustment.

Results:

Self-perceived facial appearance was positively correlated with global self-worth, self-perceived social acceptance, and number of same-sex close friends, and negatively correlated with loneliness, parent-rated social problems, and parental advice/support and concern (all p's < .05 or better).

Conclusions:

Dissatisfaction with facial appearance was associated with peer relationship problems and low global self-esteem, but not with other aspects of self-concept or other types of adjustment problems.
Psychological Research of Children with Craniofacial Anomalies: Review, Critique, and Implications for the Future
Authors: Broder Hillary L.
Published: October 2018

No abstract available.
Mandibular Asymmetry in Noncleft and Unilateral Cleft Lip and Palate Individuals
Authors: Laspos Christodoulos P. , Kyrkanides Stephanos , Tallents Ross H. , Moss Mark E. , Subtelny J. Daniel
Published: October 2018

Objective:

The purpose of this study was to retrospectively investigate mandibular asymmetry in unilateral cleft lip and palate individuals (UCLP) in relation to chronologic age and in relation to lower facial asymmetry.

Design:

The longitudinal records of 34 UCLP individuals and 142 controls treated in the Department of Orthodontics, Eastman Dental Center, Rochester, NY, were included in the study. Posteroanterior and oblique cephalometric radiographs were analyzed for lower facial asymmetry and mandibular asymmetry, respectively. Mandibular asymmetry in UCLP was analyzed relative to three age groups (6–10, 11–14, and 15 or greater) and compared to controls. Moreover, mandibular asymmetry was analyzed relative to lower facial asymmetry.

Results:

UCLP individuals showed no significant differences in mandibular asymmetry compared to controls. In addition, no significant correlation was found between mandibular asymmetry and lower facial asymmetry in UCLP.

Conclusions:

The degree of mandibular asymmetry in UCLP appears not to be the major contributing factor to the lower facial asymmetry noted on these individuals. Possible cranial-base/temporal-region anomalies may be involved in unilateral cleft lip and palate and be responsible of the asymmetry noted in the lower facial skeleton.
Craniofacial Morphology of Conotruncal Anomaly Face Syndrome
Authors: Kitano Ichiko , Park Susam , Kato Kogo , Nitta Naotsugu , Takato Tsuyoshi , Susami Takafumi
Published: October 2018

Objective and Design:

The conotruncal anomaly face syndrome (CTAF) comprises congenital heart disease and dysmorphic face, and is frequently associated with cleft palate or hypernasality. There have been many discussions about the overlap with velocardiofacial syndrome (VCF). The aim of this study was to clarify the craniofacial characteristics of CTAF patients by clinical examination, and photogrammetric and cephalometric analyses, and to clarify the differences compared to published data on VCF.

Results:

The facial features of CTAF included hypertelorism, small palpebral fissures, upward slanting of palpebral fissures, bloated eye lids, low nasal bridge, small mouth, open mouth at rest, and malformed auricles. Cephalometric features included bialveolar protrusion, small gonial angle, backward rotation of the mandibular ramus, and labial inclination of the maxillary incisors. An acute cranial base angle was also noted. These results differed from those of VCF. There were, however, no obvious pathognomonic findings for the differential diagnosis between CTAF and VCF.

Conclusions:

Considering these findings, use of CATCH 22, the inclusive classification of cardiac anomalies, cleft palate, and dysmorphic face may be of value for the clinical understanding in these patients.
Comparative Survey of Osteotomized and Nonosteotomized BCLP Patients
Authors: Lisson Jörg A. , Tränkmann Joachim
Published: October 2018

Objective:

At Hannover Medical School, treatment of BCLP patients was revised and updated in 1980. The objective of the present study was to evaluate the differences in treatment outcome between BCLP patients treated after the revised concept including infant orthopedics, and BCLP patients who received osteotomy in addition to surgical and orthodontic treatment during childhood.

Patients:

Nine of 48 BCLP patients born between 1980 and 1983 received surgical and orthodontic treatment according to the Hannover concept. They were compared to 9 of 68 adolescent and adult patients from Hannover without this protocol, who underwent maxillary osteotomy and consecutive orthodontic treatment.

Main Outcome Measures:

Comparison of the two groups was made at the end of active orthodontic treatment by cast analysis and lateral cephalometrics to evaluate sagittal, transverse, and vertical changes.

Results and Conclusions:

No patient treated using the revised protocol showed characteristics of skeletal angle class III at any stage of investigation. No indication for osteotomy was found in this group. All patients with osteotomy had skeletal angle class III resulting from insufficient midfacial growth. Sagittal and vertical skeletal relations were successfully improved by osteotomy.
Sagittal Position of the Left and Right Maxillary Segment in Children with Cleft Lip and Palate
Authors: Ras Frank , van Aalten Laura , Janse Arina , Mobers Shell , Prahl-Andersen Birte
Published: October 2018

Objective:

The purpose of the present study was to investigate left-right differences in the sagittal position of the maxillary segments in children with cleft lip and palate.

Method:

The sample consisted of children with operated cleft lip or cleft lip and alveolus [CL/CLA (n=16) mean age, 9.3 yr], operated unilateral cleft lip and palate [UCLP (n=27) mean age, 9.1 yr], and operated bilateral cleft lip and palate [ [BCLP(n = 17) mean age, 9.5 yr]. Computed tomography (CT) horizontal slices of the maxilla were obtained and used to determine the sagittal position of the left and right segment of the maxilla in relation to the mandibular rami and the cranial base. Significant effects were analyzed with multivariate analyses of variance (MANOVA).

Results and Conclusions:

It was concluded that, in contrast to children having CL/CLA or UCLP, children with BCLP showed left-right differences in the sagittal position of the maxillary segments. The segment on the left side was more posteriorly positioned compared to the right side. Because the same results were obtained in relation to the mandibular rami as well as in relation to the cranial base, it can be assumed that the position of these rami are not affected by the different types of oral clefts.
Testing for Interaction between Maternal Smoking and TGFA Genotype among Oral Cleft Cases Born in Maryland 1992–1996
Authors: Beaty Terri H. , Maestri Nancy E. , Hetmanski Jacqueline B. , Wyszynski Diego F. , Vanderkolk Craig A. , Simpson Jennifer C. , McIntosh Iain , Smith E. Anne , Zeiger Joanna S. , Raymond Gerald V. , Panny Susan R. , Tifft Cynthia J. , Lewanda Amy F. , Cristion Connie A. , Wulfsberg Eric A.
Published: October 2018

Objective:

Infants born in Maryland between June 1992 and June 1996 were used in a case-control study of nonsyndromic oral clefts to test for effects of maternal smoking and a polymorphic genetic marker at the transforming growth factor alpha (TGFA) locus, both of which have been reported to be risk factors for these common birth defects.

Design and Setting:

Cases were infants with an oral cleft ascertained through three comprehensive treatment centers, with additional ascertainment through a registry of birth defects maintained by the Maryland Health Department. Controls were healthy infants. Medical history information on infants and mothers were collected, along with DNA samples Patients, Participants: Among 286 cases contacted (72% ascertainment), there were 192 nonsyndromic isolated oral clefts (106 M; 86 F) available for this case-control study.

Main Outcome Measures:

The largest group of 149 Caucasian nonsyndromic cases and 86 controls was used to test for association with maternal smoking and genotype at the Taq1 polymorphism in TGFA.

Results:

While this modest sample had limited statistical power to detect gene-environment interaction, there was a significant marginal Increase In risk of having an oral cleft If the mother smoked (odds ratio = 1.75, 95%CI = 1.01 to 3.02). We could not demonstrate statistical interaction between maternal smoking and TGFA genotype in this study, however, and the observed increase in the C2 allele among cases was not statistically significant.

Conclusions:

We could not confirm either the reported association between oral clefts and TGFA genotype or its interaction with maternal smoking. However, these data do show an increased risk if the mother smoked during pregnancy, and this effect was greatest among infants with a bilateral cleft and no close family history of clefts.
Formation and Enlargement of the Paranasal Sinuses in Normal and Cleft Lip and Palate Human Fetuses
Authors: Smith Timothy D. , Siegel Michael I. , Mooney Mark P. , Burrows Annie M. , Todhunter John S.
Published: October 2018

Objective:

Comparisons of paranasal sinus morphology between humans with and without cleft lip and palate (CLP) have yielded conflicting opinions regarding size differences. Although postnatal samples have been investigated, no studies have compared paranasal sinus volumes between cleft and noncleft human fetuses.

Method:

The nasal cavities of 20 ‘normal’ and 9 CLP human fetuses (8–21 weeks’ postmenstrual age) were examined to assess prenatal volumetric changes of the maxillary sinuses, anterior and posterior ethmoidal air cells, and sphenoidal sinuses. Lengths and volumes of right and left maxillary and sphenoidal sinuses were calculated from histologically prepared sections using a computer reconstruction technique, and regression equations were generated to assess the enlargement rates.

Results:

All paranasal sinuses were found among both normal and CLP specimens in the same locations and in similar age ranges. However, greater shape asymmetry was noted for all sinuses in CLP compared to normal specimens. In the normal sample, results indicated significant (p < .05) correlations between right or left maxillary sinus length (R2 = 0.49, 0.54) and volume (R2 = 0.67, 0.68), and increasing postmenstrual age, but no significant (p > .05) correlations were observed for right or left sphenoidal sinus length or volumes and postmenstrual age. Maxillary sinus length changes were best described by second-order polynomial regression equations, and volume changes were best described by logarithmic equations. When individual right or left sinuses of CLP specimens were compared to the mean of the normal sample, one maxillary sinus was significantly (p < .05) larger, and 9 maxillary sinuses were not significantly (p > .05) different. Sphenoidal sinus lengths and volumes of CLP specimens were within the same range compared to these dimensions for the normal sample.

Conclusion:

Results on normal specimens indicate that maxillary sinuses exhibit second-trimester length and volume increases, whereas sphenoidal sinuses are more variable. This study suggests a similar timing of sinus formation in normal and CLP fetuses, but shape asymmetries are frequently detected among CLP specimens. In particular, the sphenoidal sinuses may be altered in shape and size by adjacent, hypertrophic cartilaginous structures in CLP fetuses. These results indicate that the maxillary sinuses of CLP fetuses are not deficient in size compared to noncleft fetuses.
Examination of Craniofacial Morphology in 10-Month to 5-Year-Old Children with Cleft Lip and Palate
Authors: Casal Carmen , Rivera Alejandro , Rubio Germán , Sentís-Vilalta Joan , Alonso Alfonso , Gay-Escoda Cosme
Published: October 2018

Objective:

The purpose of this study was to assess craniofacial growth In children from 10 months to 5 years of age with cleft lip and/or palate and to develop a systematic method of cephalometric measurements.

Design:

A case-control study.

Setting:

Craniofacial unit of a teaching hospital for children.

Patients:

A consecutive series of the first 22 patients with cleft lip and/or palate who underwent early reconstructive treatment [isolated cleft lip (CL) 6; isolated cleft palate (CP) 7; unilateral cleft lip and palate (UCLP) 7; and bilateral complete cleft lip and palate (BCLP) 2] (mean age, 27.9 months) and 22 age- and sex-matched noncleft children.

Interventions:

Lateral cephalometric headfilms of the children were taken using a pediatric cephalostat.

Main Outcome Measures:

Cephalometric landmarks were measured according to Ricketts cephalometry.

Results:

As compared with controls, CL patients had a lingual position and inclination of maxillary and mandibular incisors, an increase of interincisal angle and a decrease of incisor overjet, an increase in facial convexity, and a decrease in facial depth and mandible body length. In CP patients, palatal plane inclination and mandible arch were significantly reduced. In UCLP patients, there was a decrease in molar relation and incisor overbite, an increase in interincisal angle, reduced position and inclination of maxillary incisors and inclination of mandibular incisors, an increase in facial convexity, and lower facial height.

Conclusions:

Based on the absence of midface growth reduction, these short-term results suggest a tendency toward normal maxillomandibular growth.
Effect of Temporary Closure of Oronasal Fistulae on Levator Veli Palatini Muscle Activity
Authors: Tachimura Takashi , Hara Hisanaga , Koh Hideyasu , Wada Takeshi
Published: October 2018

Objective:

The objective of this study was to clarify electromyographically the effects of closing an oronasal fistula on levator muscle activity and oral air pressure in patients with velopharyngeal incompetence and in those with adequate velopharyngeal function.

Subjects:

Five patients with adequate velopharyngeal function and six patients with velopharyngeal incompetence were studied. All subjects had an oronasal fistula at the anterior third portion of the hard palate in spite of primary palatal closure using palatal push-back operation.

Outcome Measures:

The smoothed electromyographic activity of the levator veli palatini muscle was measured with the fistula closed with a cotton swab dipped in saline and with the fistula left open.

Results:

Under the closed fistula condition, oral air pressure was greater than that observed under the open fistula condition irrespective of velopharyngeal function. Levator veli palatini muscle activity was significantly lower in magnitude under the condition of closure than under the open condition in the patients with adequate velopharyngeal function, whereas in those with velopharyngeal incompetence, it was not significantly changed.

Conclusions:

The results suggest that velopharyngeal function is affected by temporary closure of an oronasal fistula, and that the magnitude of the effect is greater for subjects with adequate velopharyngeal function than for subjects with velopharyngeal incompetence.
Neonatal Feeding of Infants Born with Cleft Lip and/or Palate: Parental Perceptions of their Experience in South Wales
Authors: Oliver Richard G. , Jones Gail
Published: October 2018

Objective:

This paper describes the responses of 100 parents of children born with cleft lip and/or palate in South East Wales to a postal questionnaire survey of their experiences of the services available to help with the feeding of their children in the immediate postnatal period.

Results and Conclusions:

Prior to birth, the majority of mothers intended to breast feed their child; after birth, the majority of infants were bottle fed. The majority of mothers felt that they had been a pan of the decision to change methods. Advice on feeding was obtained from a variety of sources, both in hospital and on discharge. Mothers of cleft children who were first born in the family were less critical of the support offered in hospital than were mothers whose cleft child was a second or subsequent child. Isolated clefts of the palate were associated with a high proportion of feeding via nasogastric tube both on discharge from hospital and at home. The major criticism of the service offered to parents and their children was the poor quality of advice on feeding their children, both in hospital and on discharge. A variety of mechanisms for service improvement are suggested.
Management of Congenital Buccopharyngeal Membrane
Authors: Bent John P. , Klippert Frederick N. , Smith Richard J. H.
Published: October 2018

Objective:

To describe our experience in treating congenital buccopharyngeal membranes and reflect on lessons learned managing this rare anomaly.

Design:

Case series of two patients followed 3 and 6 years, respectively.

Setting:

Two different academic tertiary-care medical centers.

Patients:

Two patients with congenital buccopharyngeal membranes.

Intervention:

Multiple pharyngoplasties, including the use of lasers, rotated mucosal flaps, and oropharyngeal stents.

Results:

The first patient has had her tracheotomy removed, but she still uses a gastrostomy tube and has an expressive speech delay. The second patient can eat well but has persistent speech and airway problems, largely due to his micrognathia.

Conclusions:

Centrally dehiscent buccopharyngeal membranes are rare and require carefully planned treatment. Associated anomalies worsen the prognosis. We propose a set of recommendations to assist in the management of this difficult problem.
Resonance Imaging of the Eustachian Tube Cartilage in Microtia
Authors: Imai Yoshinor , Matsuo Kiyoshi , Imai Naomi
Published: October 2018

Objective

The purpose of this study was to determine whether external and internal defects in microtia are related.

Method

Magnetic resonance images of the eustachian tube cartilage were evaluated for 20 patients who had unilateral microtia. Nineteen patients were classified as Grade 2, and one was classified as Grade 3. The Grade 3 patient also had unilateral facial palsy.

Results

On T1-, T2-, and proton-density-weighted images, the eustachian tube cartilage was clearly identified as a pair of straight lines with low signal intensity. There was no evidence of hypoplasia of the eustachian tube cartilage on the microtic side in any Grade 2 patient, but hypoplasia was evident on the microtic side of the patient classified as Grade 3.

Conclusion

These findings are consistent with the view that impairment of embryonic development before 6 weeks results in injury to the immature pri-mordium and malformation of both the external and middle ear. In contrast, injuries that occur at a later fetal age (i.e., after 3 months) do not appear to cause middle ear malformations.
Objective, Noninvasive Evaluation of Velopharyngeal Function in Cleft and Noncleft Patients
Authors: Kunkel Martin , Wahlmann Ulrich , Wagner Wilfried
Published: October 2018

Objective

The purpose of this study was to investigate a new diagnostic method that provides an approach to noninvasive, objective measurement of velopharyngeal movement by acoustic determination of epipharyngeal volume changes with velopharyngeal muscle function.

Design

This was a case control study, using consecutive samples.

Setting

This study took place at the Cleft Palate Rehabilitation Center of the University of Mainz, Germany.

Patients

Subjects were 29 consecutive cleft lip and palate (CLP) patients and 31 controls (21 patients with dysgnathia and 10 healthy volunteers).

Intervention

A series of transnasal acoustic measurements (pressure wave: 55 dB for 2 milliseconds) of epipharyngeal volume were performed with the pharyngeal muscles relaxed in end-expiration and while the velopharyngeal orifice was closed, with the difference in volume representing maximal pharyngeal movement.

Results

Cleft palate patients yielded significantly lower values of velopharyngeal movement (6.5 cm3) than did the control group (8.0 cm3)(p < .05; Mann-Whitney U test). Overlapping ranges of values were measured for the C(L)P and control groups. The least mobility (4.75 cm3) was measured in patients who had undergone pharyngeal flap surgery. Different patterns of restriction were observed in patients with and without a pharyngeal flap.

Conclusion

Acoustic pharyngometry may provide access to noninvasive quantitative measurement of velopharyngeal movement and a better understanding of the pattern of movement in C(L)P-patients. We expect it to be a helpful tool in objectively monitoring the progress of logopedic therapy.