bluuebunny/crossref_metadata_2025_split huggingface dataset:
The Origin and Development of the Upper Lateral Incisor and Premaxilla in Normal and Cleft Lip/Palate Monkeys Induced with Cyclophosphamide
Authors: Wei Xin , Senders Craig , Owiti G. O. , Liu Xiaoming , Wei Zhen-Nian , Dillard-Telm Lisa , Mcclure Harold M. , Hendrickx Andrew G. 
Published: October 2018

Objective Cleft lip/palate (CLP) is a common human congenital defect in which the maxillary lateral incisors are often absent, malformed, and malpositioned. The present study was designed to examine the origin of the upper primary lateral incisor relative to the medial nasal process (MNP) and maxillary process (MP) fusion area and to the premaxillary/maxillary (incisive) suture in monkeys. Method Scanning electron microscopy, histology, skeletal staining, and drying techniques were used to study facial development in embryo and fetal monkey specimens. A teratogenic dose of cyclophosphamide was administered to pregnant monkeys prior to fusion of the MNP and MP and fetuses were examined for CLP. Results Formation of the anterior maxilla involved fusion of the MNP and MP at stages 14–18. At stages 18–20, the palatal portion of the MNP had formed the medial and lateral incisive mounds. By stage 22, the upper primary lateral incisor has formed within the MP, lateral to the MNP/MP fusion area and to the ossifying premaxilla. Ossification of the premaxilla begins in the MNP and subsequently spreads laterally across the MNP/MP fusion area into the MP. Accordingly, the lateral incisor undergoes a complex positional shift (mainly medial) relative to the incisive suture both prenatally and postnatally and is finally located medial to the suture. Examination of the cyclophosphamide-induced CLP fetuses showed that the lateral incisor is located lateral to the alveolar cleft and does not shift medial to the incisive suture. Conclusion Understanding the origin of the lateral incisor (the tooth closest to the cleft) and the shift after its formation provides clues to high incidence of malformations and ectopia of this incisor in cleft patients.
Congenital Heart Disease in a Patient with Microform Cleft Lip
Authors: Grech Victor , Lia Alexander , Mifsud Anton 
Published: October 2018

Objective Microform cleft lip (MCL), also known as congenital healed cleft lip, is a rare anomaly that is thought to be a spontaneously healed variant of the more common clefting of lip and palate. Although conventional clefting is known to be associated with congenital heart disease (CHD), MCL has not been similarly associated. We present one patient with MCL and CHD in the form of coarctation of the aorta, an association not previously described. Conclusion As is done in patients with cleft lip, cleft palate, or both, patients with MCL should be carefully examined for evidence of CHD and referred to a pediatric cardiologist if deemed advisable.
Nasometric Values for Normal Nasal Resonance in the Speech of Young Flemish Adults
Authors: Van Lierde K. M. , Wuyts F. L. , De Bodt M. , Van Cauwenberge P. 
Published: October 2018

Objective The purpose of this study was to obtain normative nasalance scores for adult subjects speaking the Flemish language. Additional objectives of the study were to determine if speaker sex played a role in differences in nasalance scores and if significantly different nasalance scores existed for Flemish compared with other languages or dialects. Design Nasalance scores were obtained while young Flemish adults read three standard nasalance passages. These passages were an oronasal passage (a text that contained the same approximate percentage of nasal consonants as found in the standard Dutch speech), an oral passage (a text that excluded nasal consonants), and a nasal passage (a reading text loaded with nasal consonants). Participants Subjects included 58 healthy young Flemish adults with normal oral and velopharyngeal structure and function, normal hearing levels, normal voice characteristics, and normal resonance and articulation skills. Methods The Nasometer (model 6200) was used to obtain nasalance scores for the three reading passages. These three reading passages were designed specifically for use with the nasometer. The nasalance data were analyzed for sex dependence, using Student's t test for each reading passage. This same test was used for comparison of our data with data of other languages. Results Normative nasalance data were obtained for the oronasal text (33.8%), the oral text (10.9%), and the nasal text (55.8%). Female speakers exhibited significantly higher nasalance scores than male speakers on the passages containing nasal consonants (normal text, p = .001; nasal text, p = .042). Furthermore, statistically significant cross-linguistic nasality differences were observed. The English and Spanish languages were found to have more nasalance than the Flemish language. For the North Dutch and Flemish languages, this cross-linguistic phenomenon was absent. Conclusion These normative nasalance scores for normal young adults speaking the Flemish language provide important reference information for Flemish cleft palate teams. Sex-related differences and cross-linguistic differences were shown.
Clinical Experience with Infants with Robin Sequence: A Prospective Study
Authors: Marques Ilza Lazarini , de Sousa Telma Vidotto , Carneiro Araken Fernando , Barbieri Marco Antonio , Bettiol Heloisa , Pereira Gutierrez Manoel Romeu 
Published: October 2018

Objective To study the clinical course of patients with Robin sequence (RS) during the first 6 months of life. Design A longitudinal prospective study of children with RS. Setting Hospital de Reabilitação de Anomalias Craniofaciais, Universidade de São Paulo, Bauru-SP, Brazil, 1997 and 1998. Patients Sixty-two children were studied from hospital admission to 6 months of age. Thirty-three (53.2%) presented with probable isolated RS (PIRS), 25 (40.3%) presented with syndromes or other malformations associated with RS, and 4 (6.5%) presented with RS with neurological involvement. Interventions The type of respiratory tract obstruction was defined by nasopharyngoscopy. The patients with type 1 and type 2 obstruction underwent nasopharyngeal intubation (NPI), and glossopexy was indicated in patients with type 1 obstruction who did not show clinical improvement with this procedure. Tracheostomy was indicated in patients with type 2 obstruction who did not show a good course after NPI, in patients with type 1 obstruction who did not show good course after glossopexy, and in patients with type 3 and type 4 obstruction. Results Prone position treatment (PPT) or NPI was the definitive treatment in 25 cases (75.8%) of PIRS and in 13 cases (52%) of syndromes or other malformations. Among the children with type 1 obstruction, 24 (51.1%) were submitted exclusively to PPT and 12 (25.5%) to NPI. With the type 2 groups, only one (12.5%) received PPT, and three (37.5%) were treated exclusively with NPI. All 15 infants treated exclusively with NPI (24.4%) presented with good weight, length, and neuromotor development. Conclusions Most patients with PIRS and type 1 obstruction improved without surgical intervention. NPI should be the initial treatment in all patients with RS with type 1 and type 2 obstruction who present with important respiratory and feeding difficulties.
Normative Values of Craniofacial Measurements in Idiopathic Benign Macrocephalic Children
Authors: Evereklioglu Cem , Yakinci Cengiz , Er Hamdi , Doganay Selim , Durmaz Yasar 
Published: October 2018

Objective: To determine normal standard values at each age for head circumference; inner canthal distance; outer canthal distance; near interpupillary distance; distant [far] interpupillary distance; canthal index; and circumference-interorbital index specific for the age, sex, and race in 7- to 16-year-old idiopathic benign macrocephalic male and female children. Methods: The measurements of head circumference were obtained in 8736 children (4591 boys and 4145 girls) in the city center of Malatya. From these, macrocephalic children (boys = 103, girls = 93) were chosen and invited to our clinic for detailed examination. Mean values for each parameter were obtained at each age from 7 to 16 years. Results: The mean age for male and female children were 11.87 ± 2.87 and 11.72 ± 2.75 years, respectively. The overall mean values for head circumference, inner canthal distance, outer canthal distance, near interpupillary distance, distant interpupillary distance, canthal index and circumference-interorbital index in boys were found to be 57.43 ± 1.46 cm, 31.90 ± 2.41 mm, 89.29 ± 4.34 mm, 58.79 ± 2.97 mm, 62.10 ± 3.23 mm, 35.73 ± 2.24, and 5.56 ± 0.44, respectively. In girls, these values were 56.57 ± 1.24 cm, 31.45 ± 2.65 mm, 87.71 ± 4.11 mm, 58.16 ± 3.46 mm, 61.26 ± 3.66 mm, 35.84 ± 2.31, and 5.56 ± 0.43, respectively. Conclusion: Normal values of craniofacial measurements for idiopathic benign macrocephalic healthy children are useful in early identification of some craniofacial syndromes, congenital or posttraumatic telecanthus, epicanthus, and hypo-hypertelorism and of planning surgical intervention. We suggest that the comparison of craniofacial dimensions of macrocephalic healthy children must be performed with normal standards specific for age as well as sex and race.
La Maladie de Grisel: Spontaneous Atlantoaxial Subluxation
Authors: Meek M. F. , Hermens R. A. E. C. , Robinson P. H. 
Published: October 2018

Objective: “La maladie de Grisel” (Grisel's syndrome) is a spontaneously occurring atlantoaxial subluxation with torticollis. We present a case of atlantoaxial subluxation occurring in a 20-year period of pharyngoplasty surgery. The occurrence of a “spontaneous” atlantoaxial subluxation after oral cavity or pharynx operations is rare. Because some neck pain and stiffness are commonly seen after these kinds of operations, we would like to draw attention to this unusual complication. Symptoms associated with a torticollis after an operation in the oral cavity or pharynx requires additional investigation to exclude this rare complication. A review of the available literature concerning etiology and treatment of la maladie de Grisel is presented.
Intracranial Migration of Halo Fixation Pins: A Complication of Using an Extraoral Distraction Device
Authors: Le Bach T. , Eyre James M. , Wehby Monica C. , Wheatley Michael J. 
Published: October 2018

Objective: Distraction osteogenesis is a well-accepted technique in the treatment of patients with hypoplastic craniofacial components. Complications of distraction osteogenesis are well described in the literature. We describe a complication of using an external distraction device in a 9-year-old girl with Pfeiffer. Intervention and Results: A modified Lefort III osteotomy was performed for maxillary hypoplasia with application of an external distraction halo device by a pediatric neurosurgeon. A postoperative computed tomography (CT) scan showed 0.5-cm skull penetration of the cranial pins. The pins were repositioned and the patient was followed up on a regular basis until discharge from the hospital. At 3-week follow-up, a CT scan of the head showed migration of the pins 1.5 cm intracranially. The halo was removed and repositioned at a different site. No detectable neurological sequelae from the pin penetration were noted. The patient developed cellulitis at the site of the penetration and was admitted to the hospital for a course of intravenous antibiotics. There were no other complications, and the rest of her treatment course proceeded as planned. A review of the literature on complications of halo usage as well as suggestions for their management in association with distraction osteogenesis is described.
Morphological Characterization of the Levator Veli Palatini Muscle in Children Born with Cleft Palates
Authors: Lindman Rolf , Paulin Gunnar , Stål Per S. 
Published: October 2018

Objective: The aim of this study was to analyze, morphologically and biochemically, one of the soft palate muscles, the levator veli palatini (LVP), in children born with cleft palate. Subjects and Methods: Biopsies were obtained from nine male and three female infants in connection with the early surgical repair of the hard and soft palate. Samples from five adult normal LVP muscles were used for comparison. The muscle morphology, fiber type and myosin heavy chain (MyHC) compositions, capillary supply, and content of muscle spindles were analyzed with different enzyme-histochemical, immunohistochemical, and biochemical techniques. Results: Compared with the normal adult subjects, the LVP muscle from the infantile subjects with cleft had a smaller mean fiber diameter, a larger variability in fiber size and form, a higher proportion of type II fibers, a higher amount of fast MyHCs, and a lower density of capillaries. No muscle spindles were observed. Moreover, one-third of the biopsies from the infantile subjects with cleft LVP either lacked muscle tissue or contained only a small amount. Conclusions: The LVP muscle from children with cleft palate has a different morphology, compared with the normal adult muscle. The differences might be related to different stages in maturation of the muscles, changes in functional demands with growth and age, or a consequence of the cleft. The lack of contractile tissue in some of the cleft biopsies offers one possible explanation to a persistent postsurgical velopharyngeal insufficiency in some patients, despite a successful surgical repair.
Change in Levator Veli Palatini Muscle Activity of Normal Speakers in Association with Elevation of the Velum Using an Experimental Palatal Lift Prosthesis
Authors: Tachimura Takashi , Nohara Kanji , Fujita Yoshinori , Hara Hisanaga , Wada Takeshi 
Published: October 2018

Objective: The purpose of this study was to examine whether mechanical elevation of the velum can directly affect the levator veli palatini muscle (LVP) activity using normal speakers as subjects. Design: Each subject was instructed to produce /mu/, /u/, /pu/, /su/, and /tsu/ in a speech task and to blow with maximum possible effort. Smoothed electromyographic activity of the LVP was recorded with an experimental palatal lift prosthesis (PLP) both in place and removed. Participants: Four normal speakers were used as subjects. Results: LVP activity for all tasks was significantly smaller (p < .001; Student's t test) with the PLP in place than without the PLP for all subjects. An analysis of variance (p < .001) clarified that activity ranges of the LVP were significantly different between the removal and placement conditions for all the subjects. Conclusion: The result was similar to that previously obtained for patients with velopharyngeal incompetence wearing a PLP. It is possible that the decrease in the LVP activity in association with placement of a PLP is caused by the direct effect of mechanical elevation, which decreases the distance the velopharyngeal mechanisms must travel for complete closure of the velopharynx.
Nasal Morphology and Shape Parameters as Predictors of Nasal Esthetics in Individuals with Complete Unilateral Cleft Lip and Palate
Authors: Russell K. A. , Waldman S. D. , Tompson B. , Lee J. M. 
Published: October 2018

Objective: The purpose of this study was to assess the ability of shape parameters of nasal morphology to predict esthetics in individuals with complete unilateral cleft lip and palate (CUCLP). Methods: This retrospective study involved 28 patients with repaired CUCLP. Nostril morphology was analyzed using nose casts and a video-imaging technique. Calculated shape parameters included area, perimeter, centroid, angle of the principal axis, major and minor moments of area, anisometry, bulkiness, lateral offset, and three-dimensional internostril angles. Esthetics was assessed using a panel of six orthodontists who rated nasal esthetics from frontal, lateral, basal, and three-quarters view slides and from nose casts. Correlations between esthetics and the shape parameters were completed using the entire group as well as using two statistically determined subsets: those with the best and those with the worst esthetics. Results: Nasal esthetics was related to only the perimeter and bulkiness parameter ratios. Symmetry of the perimeters between the right and left nostrils positively correlated with better esthetics using the entire sample group while symmetry of bulkiness between the right and left nostrils positively correlated with better esthetics using both the entire sample group and the best and worst subsets. Conclusions: Only perimeter and bulkiness showed positive correlations with nasal esthetics. The group of parameters used to assess nostril morphology had neither significant correlation with—nor predictive power for—esthetics. Thus, an assessment of the entire nasal surface topography in three dimensions needs to be completed and assessed with respect to predictability of nasal esthetics.
The Oral Health of Children with Clefts of the Lip, Palate, or Both
Authors: Chapple J. R. , Nunn J. H. 
Published: October 2018

Objective: The purpose of this study was to assess the prevalence of dental caries, developmental defects of enamel, and related factors in children with clefts. Design: This cross-sectional prevalence study used standard dental indices for assessment. Setting: Children underwent a dental examination under standard conditions of seating and lighting in the outpatient department of a dental hospital as part of an ongoing audit to monitor clinical outcomes. Participants: Ninety-one children aged 4, 8, and 12 years were included in the study. Outcome Measurements Dental caries were assessed by use of the decayed, missing, and filled index for primary teeth (dmft); Decayed, Missing, and Filled index for permanent teeth (DMFT) according to the criteria as used in the national survey of children's dental health in the United Kingdom (O'Brien, 1994). Developmental defects were assessed using the modified Developmental Defects of Enamel Index (Clarkson and O'Mullane, 1989). Dental erosion was assessed using the criteria derived for the national survey of children's dental health (O'Brien, 1994). Results: Caries prevalence increased with age; 63% of patients at 4 years and 34% at 12 years were caries free. The mean dmft for the 4-year-olds was 1.3 with a mean DMFT for the 12-year-olds of 1.8. All the 4-year-olds had evidence of erosion of enamel in the primary teeth (incisors and first molars) and 56% of the 12-year-olds had erosion of permanent teeth (incisors and first permanent molars). Developmental defects of enamel became more prevalent with age, with at least one opacity in 56% of 4-year-olds and 100% of 12-year-olds. Hypoplasia was not found in the primary dentition but affected permanent teeth in 38% of 8-year-olds and 23% of the 12-year-olds. Conclusion: This study has shown that dental disease is prevalent in these patients. These assessments not only provide a baseline on oral health parameters in young people with clefts but underline the need for a more aggressive approach to prevention of oral disease to optimize clinical outcome.
Associations between Severity of Clefting and Maxillary Growth in Patients with Unilateral Cleft Lip and Palate Treated with Infant Orthopedics
Authors: Peltomäki Timo , Vendittelli Bruno L. , Grayson Barry H. , Cutting Court B. , Brecht Lawrence E. 
Published: October 2018

Objective: The purpose of this study was to examine possible associations between severity of clefting in infants and maxillary growth in children with complete unilateral cleft lip and palate. Design: This was a retrospective study of measurements made on infant maxillary study casts and maxillary cephalometric variables obtained at 5 to 6 years of follow-up. Setting: The study was performed at the Institute of Reconstructive Plastic Surgery of New York University Medical Center, New York, New York. Patients: Twenty-four consecutive nonsyndromic unilateral complete cleft lip and palate patients treated during the years 1987 to 1994. Interventions: All the patients received uniform treatment (i.e., presurgical orthopedics followed by gingivoperiosteoplasty to close the alveolar cleft combined with repair of the lip and nose in a single stage at the age of 3 to 4 months). Closure of the palate was performed at the age of 12 to 14 months. Results: Infant maxillary study cast measurements correlated in a statistically significant manner with maxillary cephalometric measurements at age 5 to 6 years. Conclusions: The results demonstrate the large variation in the severity of unilateral cleft lip and palate deformity at birth. Patients with large clefts and small arch circumference, arch length, or both demonstrated less favorable maxillary growth than those with small clefts and large arch circumference or arch length at birth.
Craniofacial Morphology, Dentition, and Skeletal Maturity in Four Siblings with Seckel Syndrome
Authors: Kjær Inger , Hansen N. , Becktor K. B. , Birkebæk N. , Balslev T. 
Published: October 2018

Objective: To describe the craniofacial morphology, dentition, and hand maturity in four siblings with Seckel syndrome. Patients: Two boys and two girls, with Seckel syndrome. The children studied showed extreme growth retardation, severe microcephaly, bird-headed profile with receding chin, prominent nose, mental retardation, and extremely delayed skeletal maturation. The growth hormone axis and pituitary thyroid function was normal. Methods: Skeletal and dental development were investigated from radiographic material, and a cephalometric analysis was performed from profile radiographs. Results: The craniums were remarkably small with an extremely short anterior cranial base (−4.3 to −5.5 standard units) and maxillary length (−3.8 to −4.7 SU). Differences in the morphology of the sella turcica were observed in girls and boys. Tooth maturity progressed normally. Tooth agenesis and tooth malformations were observed. Taurodontic root morphology was observed only in the girls. The approximate skeletal maturity showed retardation from 4 years 3 months to 4 years 11 months. Malformations of the hand-wrist skeleton occurred in the epiphyseal ossification centers of the middle phalangeal bone in the second, third, and fourth finger and in the distal phalangeal bone in the fifth finger. The epiphyseal ossification centers were lacking in the middle and distal phalangeal bones of the fifth finger. Conclusion: The underlying gene defect in the affected children seemingly affects bone development and growth but not dental maturation and eruption.