Clefts Of Secondary Palate Research Articles

Tissue‐specific requirement for TGF‐β signaling during craniofacial development

Members of the transforming growth factor‐β (TGF‐β) superfamily mediate a wide range of biological activities, including cell proliferation, differentiation, extracellular matrix formation, and induction of homeobox genes, suggesting that TGF‐β signaling is important in pattern formation and organogenesis. During craniofacial development, TGF‐β is expressed in the craniofacial epithelium and the cranial neural crest (CNC) derived mesenchyme, suggesting tissue‐specific functions in regulating craniofacial development. To investigate the tissue‐specific requirement of TGF‐β signaling, we generated animal models with epithelim or CNC‐derived mesenchyme specific inactivation of Tgfbr2 gene and investigated the molecular mechanism of TGF‐β signaling in regulating the fate of craniofacial epithelial or the CNC mesenchyme cells, respectively. Using mice with conditional inactivation of Tgfbr2 in the epithelium, we show that TGF‐β signaling is specifically required for regulating palate fusion and skin development. At the cellular level, TGF‐β signaling is critical for mediating apoptosis of MEE cells during palatal fusion. On the other hand, conditional Tgfbr2 gene ablation in the CNC resulted in complete cleft secondary palate, calvaria agenesis, and other skull defects with complete phenotype penetrance. Significantly, disruption of the TGF‐β signaling does not adversely affect CNC migration. Cleft palate in Tgfbr2 mutant mice results from a cell proliferation defect within the CNC‐derived palatal mesenchyme. The midline epithelium of the mutant palatal shelf remains functionally competent to mediate palatal fusion once the palatal shelves are placed in close contact in vitro. Our data suggests that TGF‐β IIR plays a critical, cell autonomous role in regulating the fate of MEE and CNC cells during palatogenesis. To explore the molecular mechanism of TGF‐β signaling in regulating craniofacial development, we analyzed downstream target genes affected by altered TGF‐β signaling in either the epithelium or the CNC‐derived mesenchyme. Comprehensive analysis reveals specific TGF‐β signaling network in regulating craniofacial development. Collectively, our study demonstrates that TGF‐β signaling plays an essential role in cell fate determination of the craniofacial epithelium and the CNC‐derived mesenchyme and provides a model for the study of abnormal craniofacial development.

Mouse genetic models of cleft lip with or without cleft palate

Nonsyndromic cleft lip and palate (CLP) is among the most common human birth defects. Transmission patterns suggest that the causes are "multifactorial" combinations of genetic and nongenetic factors, mostly distinct from those causing cleft secondary palate (CP). The major etiological factors are largely unknown, and the embryological mechanisms are not well understood. In contrast to CP or neural tube defects (NTD), CLP is uncommon in mouse mutants. Fourteen known mutants or strains express CLP, often as part of a severe syndrome, whereas nonsyndromic CLP is found in two conditional mutants and in two multifactorial models based on a hypomorphic variant with an epigenetic factor. This pattern suggests that human nonsyndromic CLP is likely caused by regulatory and hypomorphic gene variants, and may also involve epigenetics. The developmental pathogenic mechanism varies among mutants and includes deficiencies of growth of the medial, lateral or maxillary facial prominences, defects in the fusion process itself, and shifted midline position of the medial prominences. Several CLP mutants also have NTD, suggesting potential genetic overlap of the traits in humans. The mutants may reflect two interacting sets of genetic signaling pathways: Bmp4, Bmpr1a, Sp8, and Wnt9b may be in one set, and Tcfap2a and Sox11 may be in another. Combining the results of chromosomal linkage studies of unidentified human CLP genes with insights from the mouse models, the following previously unexamined genes are identified as strong candidate genes for causative roles in human nonsyndromic CLP: BMP4, BMPR1B, TFAP2A, SOX4, WNT9B, WNT3, and SP8.

Cleft Deformities in Zimbabwe, Africa

In the African country of Zimbabwe, a variety of socioeconomic factors have contributed to a lack of specialty care and resources for the indigent population. Although cleft lip and palate has a lower incidence in Africa (0.67 per 1000 births) than in Latin America or Asia, access to reconstructive surgery is often difficult to obtain. A surgical team worked with Zimbabweans at the Harare Central Hospital, Harare, to perform cleft surgery for 39 patients. We review the epidemiology of cleft deformities in Africa, our experience with 39 patients with cleft lip and palate, and the techniques used to address 2 patients with midfacial clefts. To our knowledge, this retrospective case review and epidemiologic literature review is the first review of cleft care in Zimbabwe. Poverty in Zimbabwe, caused in part by the highest inflation rate in the world, has contributed to the emigration of a large number of specialists to other countries. In addition, the health care system is overwhelmed by a high prevalence rate of human immunodeficiency virus (25%), leading to a drastically reduced parental life expectancy (mean life expectancy, 36 years). Primary and secondary cleft lip and palate repairs were completed without complications. Children requiring care beyond the scope of this mission were referred to the Republic of South Africa. The cooperation among the Zimbabwean administration, physicians, and nurses was integral to the organization and successful execution of this reconstructive surgical mission. Ultimately, until the socioeconomic conditions improve in Zimbabwe, training and continuing education of local physicians are imperative to advance the care of children with cleft lip and palate.

Derivation of a mouse model for conditional inactivation of Pax9

Pax9 is required for the formation of a variety of organs during mouse development. The function of Pax9 at postnatal stages is unknown since homozygosity of the null allele (Pax9(lacZ)) causes neonatal lethality. Recently, we have generated a hypomorphic Pax9 allele, Pax9(neo), which contains a removable neomycin resistance cassette (neo) and loxP sites flanking the first two exons of Pax9. Here we show that FLP-mediated in vivo excision of neo generates phenotypically normal Pax9(flox) mice. Crossing Pax9(flox) mice to PGK-Cre mice leads to efficient recombination of loxP sites and neonatal lethality in the resulting Pax9(del/del) offspring. Inactivation of Pax9 using Wnt1-Cre mice causes cleft secondary palate and tooth agenesis and reveals that the Pax9 expressing mesenchymal cells of the nose, palate, and teeth are derived from neural crest cells. The conditional Pax9 allele will be a valuable tool to study Pax9 function in specific tissues of adult mice.

Sonographic assessment of normal fetal palate using three‐dimensional imaging: a new technique

The aim of this study was to describe a novel three-dimensional (3D) ultrasound rendering technique to examine the normal fetal posterior palate and to assess its correspondence with the real fetal anatomy. A prospective longitudinal study was conducted from January to October 2005 and included 100 fetuses in a low-risk population. Fetal ultrasound examinations were performed at 17, 22, 27 and 32 weeks' gestation to determine the normal 3D ultrasound view of the fetal palate at different gestational ages. The ultrasound scans were performed using the strict anterior axial plane of the starting reconstruction volume and the underside 3D view of the fetal palate. The 3D view of the fetal palate was compared with the normal anatomical view of the fetal palate obtained by surgical fetopathological examination of fetuses at the same gestational ages. The sonographic visualization rates of seven defined anatomical landmarks of the fetal palate were computed for each gestational age. The visualization rates across gestational ages were compared by use of the Cochrane Q test. The reliability of detection of each anatomical landmark across gestational ages was determined by Cronbach's Alpha. In all cases a 3D ultrasound view of the fetal maxilla and secondary palate was obtained at each period of gestation and corresponded well to the fetal anatomical specimens. The seven defined anatomical landmarks of the fetal palate were identified in 42-100% of cases. The visualization rates across gestational ages were significantly different in five of these anatomical landmarks. These differences can be explained by different developmental processes of these anatomical structures. The overall reliability of visualization across the gestational ages for the anatomical landmarks was medium to very high (0.73-0.96), except for the landmark interpalatal suture which was low (0.48). This technique of anterior axial 3D view reconstruction of the fetal palate seen by an underside view can provide unique diagnostic information on the integrity of the secondary palate. This innovative, simple and rapid technique may become the reference technique in ultrasound investigation of the fetal palate, and should be of value in diagnosing isolated secondary cleft palate or palatal involvement when cleft lip and alveolus are diagnosed.

A novel technique for visualization of the normal and cleft fetal secondary palate: angled insonation and three‐dimensional ultrasound

To evaluate the effectiveness of a novel approach to the visualization of the fetal secondary palate using three-dimensional (3D) ultrasound. Sonographic examinations were performed in normal fetuses and in one fetus with cleft lip and palate. To avoid acoustic shadowing from the alveolar ridge, the secondary palate was insonated at a 45 degrees angle in the sagittal plane, and 3D ultrasound was used to reconstruct axial and coronal planes. The secondary palate was successfully visualized in 10 of 15 normal fetuses, both in the axial and coronal planes. In the fetus with cleft lip and palate the lesion of the secondary palate was clearly demonstrated, particularly in the coronal plane. Angled insonation and 3D ultrasound allow clear visualization of normal and cleft secondary palate.

Temporal and spatial expression of Pax9 and Sonic hedgehog during development of normal mouse palates and cleft palates in TGF- β3 null embryos

Transforming growth factor-β ( TGF- β3) gene disruption causes cleft secondary palate. Pax9 and Sonic hedgehog ( Shh) genes are involved in the patterning of vertebrate embryonic tissues, including the facial skeleton. We investigated the expression of Pax9 and Shh genes during normal mouse palate development and in the developing cleft palates of TGF- β3 null embryos. Whole mount in situ hybridization was conducted with use of Pax9 and Shh riboprobes for TGF- β3 null, heterozygous and wild type mice at E12.5–E16.5. Histological analysis was processed by section in situ hybridization. In the wild type, Pax9 and Shh were expressed in the palate between E12.5–E15.5. Shh expression in the secondary palate was restricted to the rugae and the soft palate. Pax9 expression was predominantly in the palatal medial edge between E14.5 and E15.5. These patterns suggest that Shh and Pax9 may have different functions during palate development. In TGF- β3 null mice, both genes expression patterns in the palate were different to those in wild type mice. In TGF- β3 null mice, Pax9 expression was much reduced in the palatal medial edge at the critical time of palatal fusion (E14.5–E15.5). Shh expression in the palates of TGF- β3 null mice was reduced throughout E12.5–E15.5, whilst Shh expression in heterozygous did not appear down regulated compared with the wild type. These results indicate that Pax9 and Shh expression are altered when the TGF- β3 gene is deleted and suggest that Pax9 and Shh may be involved in the TGF- β3 regulation of normal palatal fusion.

Real-time Magnetic Resonance Imaging Aids Prenatal Diagnosis of Isolated Cleft Palate

Cleft of the secondary palate without cleft lip is difficult to visualize sonographically. This study was performed to assess the utility of sonography, standard magnetic resonance (MR) imaging, and real-time MR imaging in the diagnosis of isolated cleft palate. We prospectively assessed 5 fetuses at risk for isolated cleft palate on the basis of family history, micrognathia, or both, using sonography and standard and real-time single-shot fast spin echo MR sequences. Written informed consent was obtained under our Institutional Review Board-approved Health Insurance Portability and Accountability Act-compliant protocol. Images were assessed for confidence in a diagnosis of cleft or normal palate. Prenatal and postnatal diagnoses were compared. In 3 fetuses, micrognathia was visualized by sonography and MR imaging with standard and real-time sequences. One fetus at 19 weeks had a wide cleft of the entire secondary palate, and another fetus at 33 weeks had a cleft of the soft palate; these defects were seen only with real-time MR imaging. One 35-week gestational age fetus had a cleft soft palate that was visualized on standard and real-time MR imaging. Two fetuses with no abnormalities had the normal midline secondary palate seen only on real-time MR imaging. In all fetuses, real-time images were helpful in assessing the secondary palate because the entire midline naso-oropharynx could be visualized. Real-time MR imaging allows for rapid assessment of the midline structures, providing accurate diagnosis of isolated cleft palate.

A Case of Mitochondrial Myopathy and Cleft Palate

To present a child born with both mitochondrial myopathy and cleft palate, outlining medical complications of this dual diagnosis-the first reported case in English literature. A preterm Caucasian boy presented to the cleft palate clinic at birth for evaluation of a secondary cleft palate. Multiple anomalies were also identified, including rib fusions, ventricular septal dyskinesis, and mild hypotonia. Initial chromosome studies were unremarkable. After a multidisciplinary preoperative clearance at 11 months old, the infant underwent palatoplasty and muscle biopsy for evaluation of hypotonia. Upon extubation, he became apneic and unresponsive to anticholinesterase reversals. He was reintubated. The next morning, he was extubated uneventfully and was discharged home on postoperative day 2 (POD2). On POD7, he returned with signs of bronchitis, thereby prompting immediate reevaluation and treatment by his primary pediatrician. On POD13, he presented to an outlying emergency department with cyanosis and poor respiratory drive. Before intubation, he received a depolarizing muscle relaxation; cardiac arrest ensued and resuscitation was unsuccessful. Pathology from the muscle biopsy and postmortem examination ultimately supported mitochondrial myopathy with dilated cardiomyopathy. Mitochondrial myopathies are rare disorders suspected in patients demonstrating developmental delays, hypotonia, cardiomyopathy, conduction system defects, or genetic mutations. In these patients, postoperative complications can be lethal, especially when compounded (e.g., anesthetic drug reactions, respiratory compromise, surgical stress, and postoperative infections). General discussion and specific recommendations are provided.

Distinct functions for Bmp signaling in lip and palate fusion in mice

Previous work suggested that cleft lip with or without cleft palate (CL/P) is genetically distinct from isolated cleft secondary palate (CP). Mutations in the Bmp target gene Msx1 in families with both forms of orofacial clefting has implicated Bmp signaling in both pathways. To dissect the function of Bmp signaling in orofacial clefting, we conditionally inactivated the type 1 Bmp receptor Bmpr1a in the facial primordia, using the Nestin cre transgenic line. Nestin cre; Bmpr1a mutants had completely penetrant, bilateral CL/P with arrested tooth formation. The cleft secondary palate of Nestin cre; Bmpr1a mutant embryos was associated with diminished cell proliferation in maxillary process mesenchyme and defective anterior posterior patterning. By contrast, we observed elevated apoptosis in the fusing region of the Nestin cre; Bmpr1a mutant medial nasal process. Moreover, conditional inactivation of the Bmp4 gene using the Nestin cre transgenic line resulted in isolated cleft lip. Our data uncover a Bmp4-Bmpr1a genetic pathway that functions in lip fusion, and reveal that Bmp signaling has distinct roles in lip and palate fusion.

The cellular and molecular etiology of the cleft secondary palate in Fgf10 mutant mice

Mammalian palatogenesis depends on interactions between the stomodium-derived epithelium and the cranial neural crest-derived ectomesenchyme. Fibroblast growth factor 10 (FGF10) is a mesenchymal signaling factor that guides the morphogenesis of multiple organs through tissue–tissue interactions. This is consistent with widespread agenesis and dysgenesis of organs observed in Fgf10 −/− mice. In this study, we report the presence of a wide-open cleft secondary palate in Fgf10 homozygous null mutant mice. Fgf10 transcripts were detected in the palatal mesenchyme from E11.5 to E13.5 during normal palatogenesis and were enriched in the anterior and middle portions of the palatal shelves. In Fgf10 −/− embryos, histological analyses revealed aberrant adhesion of the palatal shelves with the tongue in the anterior and fusion with the mandible in the middle and posterior beginning at E13.5, which could prevent normal elevation of the palatal shelves leading to a cleft palate. TUNEL and BrdU assays demonstrated significant levels of apoptosis in the medial edge epithelium (MEE) but unaltered cell proliferation in mutant palatal shelves. At the molecular level, we show that Fgf10 is epistatic to Jagged2 and Tgfβ3 in the developing palate. Notably, the expression of Jagged2 is downregulated throughout the palate epithelium in Fgf10 mutants while Tgfβ3 is misexpressed in the palatal epithelium at the oral side. Our results demonstrate that mesenchymally expressed Fgf10 is necessary for the survival of MEE cells and for the normal expression of Jagged2 and Tgfβ3 in the palatal epithelium during mammalian palatogenesis.

Poster 10: Posterior palate repositioning by distraction osteogenesis in clefts

Poster 10: Posterior palate repositioning by distraction osteogenesis in clefts

A similarity function to evaluate the orthodontic condition in patients with cleft lip and palate

The objective of this work is the modeling of a similarity function adapted to the medical environment using the logical-combinatorial approach of pattern recognition theory, and its application to compare the orthodontic conditions of patients with cleft-primary palate and/or cleft-secondary palate congenital malformations. The variables in domains with no a priori algebraic or topological structure are objects whose similarity or difference is evaluated by comparison criteria functions. The range of these functions is an ordered set normalized into the unit interval, and they are designed to allow differentiation and non-uniform treatment of the object-variables. The analogy between objects is formalized as a similarity function that stresses the relations among the comparison criteria and evaluates the partial descriptions (partial similarity/difference) or total descriptions (total similarity/difference) of the objects. For the orthodontic problem we defined a set of 12 variables featuring the unilateral/bilateral fissures, the conditions of maxilla, premaxilla, mandible and patient's bite. The comparison criteria (logical for malocclusion, fuzzy for maxillary collapse unilateral/anteroposterior and for overbite, and Boolean for protrusive/retrusive premaxilla conditions) were assigned a relevance factor based on the orthodontist accumulated knowledge and experience. The modeling of the similarity function and its effectiveness in comparing orthodontic conditions in patients are illustrated by the study of four clinical cases with different clefts. The results through similarity are close to the expected ones. Moreover evaluated at different moments it allows to assess the effect of treatment in a single patient, hence providing valuable auxiliary criteria for medical decision making as to the patient's rehabilitation. We include the potential extension of the methodology to other medical disciplines such as speech therapy and reconstructive surgery.

Wnt1-Cre-mediated deletion of AP-2α causes multiple neural crest-related defects

The AP-2α transcription factor is required for multiple aspects of vertebrate development and mice lacking the AP-2α gene (tcfap2a) die at birth from severe defects affecting the head and trunk. Several of the defects associated with the tcfap2a-null mutation affect neural crest cell (NCC) derivatives including the craniofacial skeleton, cranial ganglia, and heart outflow tract. Consequently, there is considerable interest in the role of AP-2α in neural crest cell function in development and evolution. In addition, the expression of the AP-2α gene is utilized as a marker for premigratory and migratory neural crest cells in many vertebrate species. Here, we have specifically addressed how the presence of AP-2α in neural crest cells affects development by creating a conditional (floxed) version of tcfap2a which has subsequently been intercrossed with mice expressing Cre recombinase under the control of Wnt1 cis-regulatory sequences. Neural crest-specific disruption of tcfap2a results in frequent perinatal lethality associated with neural tube closure defects and cleft secondary palate. A small but significant fraction of mutant mice can survive into adulthood, but have retarded craniofacial growth, abnormal middle ear development, and defects in pigmentation. The phenotypes obtained confirm that AP-2α directs important aspects of neural crest cell function. At the same time, we did not observe several neurocristopathies affecting the head and heart that might be expected based on the phenotype of the AP-2α-null mouse. These results have important implications for the evolution and function of the AP-2 gene family in both the neural crest and the vertebrate embryo.

Conditional inactivation of Tgfbr2 in cranial neural crest causes cleft palate and calvaria defects.

Cleft palate and skull malformations represent some of the most frequent congenital birth defects in the human population. Previous studies have shown that TGFbeta signaling regulates the fate of the medial edge epithelium during palatal fusion and postnatal cranial suture closure during skull development. It is not understood, however, what the functional significance of TGFbeta signaling is in regulating the fate of cranial neural crest (CNC) cells during craniofacial development. We show that mice with Tgfbr2 conditional gene ablation in the CNC have complete cleft secondary palate, calvaria agenesis, and other skull defects with complete phenotype penetrance. Significantly, disruption of the TGFbeta signaling does not adversely affect CNC migration. Cleft palate in Tgfbr2 mutant mice results from a cell proliferation defect within the CNC-derived palatal mesenchyme. The midline epithelium of the mutant palatal shelf remains functionally competent to mediate palatal fusion once the palatal shelves are placed in close contact in vitro. Our data suggests that TGFbeta IIR plays a crucial, cell-autonomous role in regulating the fate of CNC cells during palatogenesis. During skull development, disruption of TGFbeta signaling in the CNC severely impairs cell proliferation in the dura mater, consequently resulting in calvaria agenesis. We provide in vivo evidence that TGFbeta signaling within the CNC-derived dura mater provides essential inductive instruction for both the CNC- and mesoderm-derived calvarial bone development. This study demonstrates that TGFbeta IIR plays an essential role in the development of the CNC and provides a model for the study of abnormal CNC development.

Incidence of cleft lip and palate in the northeast of Mexico: a 10-year study.

In Latin American countries, studies on the incidence of cleft lip and palate are not widely available. A 10-year retrospective study was carried out to determine the incidence of cleft lip and palate at the Autonomous University of Nuevo Leon's University Hospital. The study included data from patients who attended the plastic surgery outpatient clinic from January 1990 to December 1999. The author reviewed 10,843 files from which 376 patients were selected to identify the following variables: time of the year in which the first consultation took place, gender, birthplace, type of cleft, age of both parents, and medications taken during pregnancy. The highest incidence was found in patients aged 1 to 6 months. Ninety-four patients had a primary palate cleft; 76 had a secondary palate cleft; and 206 had primary and secondary palate clefts. The gender distribution of the 206 patients with primary and secondary clefts was 127 boys and 79 girls. The mean parent age was 29.5 years (father) and 25.7 years (mother). The incidence of cleft lip and palate in the cited hospital was 1.1:1000 births. Clefts of the left side occurred more often than of the right. Boys were affected more commonly than girls.

Use of an onplant as orthodontic anchorage

Considerable limitations in the clinical application of orthodontic treatment can result from the lack of teeth suitable for anchorage. If prosthetic rehabilitation is necessary, endosseous implants can be first used as orthodontic anchorage and then loaded by a prosthetic superstructure. However, in a plan of exclusive orthodontic treatment, alveolar bone is not always available, and an orthodontic osseointegrated anchorage system might be necessary. We report the use of an onplant for palatal anchorage to extrude the unerupted horizontal maxillary first molars in a 12-year-old white girl with tooth aplasia and secondary cleft palate. After a healing period of 5 months, the onplant remained stable under indirect elastic tension of approximately 160 g applied for 17 weeks, and the maxillary first molars were successfully extruded. (Am J Orthod Dentofacial Orthop 2002;122:566-70)

Rescue of cleft palate in Msx1-deficient mice by transgenic Bmp4 reveals a network of BMP and Shh signaling in the regulation of mammalian palatogenesis.

Cleft palate, the most frequent congenital craniofacial birth defects in humans, arises from genetic or environmental perturbations in the multi-step process of palate development. Mutations in the MSX1 homeobox gene are associated with non-syndromic cleft palate and tooth agenesis in humans. We have used Msx1-deficient mice as a model system that exhibits severe craniofacial abnormalities, including cleft secondary palate and lack of teeth, to study the genetic regulation of mammalian palatogenesis. We found that Msx1 expression was restricted to the anterior of the first upper molar site in the palatal mesenchyme and that Msx1 was required for the expression of Bmp4 and Bmp2 in the mesenchyme and Shh in the medial edge epithelium (MEE) in the same region of developing palate. In vivo and in vitro analyses indicated that the cleft palate seen in Msx1 mutants resulted from a defect in cell proliferation in the anterior palatal mesenchyme rather than a failure in palatal fusion. Transgenic expression of human Bmp4 driven by the mouse Msx1 promoter in the Msx1(-/-) palatal mesenchyme rescued the cleft palate phenotype and neonatal lethality. Associated with the rescue of the cleft palate was a restoration of Shh and Bmp2 expression, as well as a return of cell proliferation to the normal levels. Ectopic Bmp4 appears to bypass the requirement for Msx1 and functions upstream of Shh and Bmp2 to support palatal development. Further in vitro assays indicated that Shh (normally expressed in the MEE) activates Bmp2 expression in the palatal mesenchyme which in turn acts as a mitogen to stimulate cell division. Msx1 thus controls a genetic hierarchy involving BMP and Shh signals that regulates the growth of the anterior region of palate during mammalian palatogenesis. Our findings provide insights into the cellular and molecular etiology of the non-syndromic clefting associated with Msx1 mutations.

Comparison of Craniofacial Morphology in Patients With Bilateral Complete Cleft of Primary Versus Secondary Palate

Abstract Objective: To identify and compare the craniofacial morphology of patients with bilateral complete cleft of primary palate (BCCPP) and bilateral complete cleft of secondary palate (BCCSP). In addition, to evaluate the differential effects of lip repair and palatal repair on the craniofacial morphology in cleft patients. Subjects: Twenty-four children, 10 boys and 14 girls, mean age of 5.8 years, with BCCPP (BCCPP group). Twenty-five children, 10 boys and 15 girls, mean age of 5.6 years, with BCCSP (BCCSP group). Design: Retrospective analysis. Main Outcome Measures: Cephalometric analysis was used to determine the craniofacial morphology at about 5 years of age. Results: Compared with the BCCSP group, the BCCPP group demonstrated longer maxillary length, more protruded maxilla, more favorable jaw relation, more severely retroclined maxillary incisors, and larger overjet. Conclusions: The subjects with BCCPP had craniofacial characteristics that differed significantly from those with BCCSP. It cou...

Comparison of Craniofacial Morphology in Patients with Bilateral Complete Cleft of Primary versus Secondary Palate

To identify and compare the craniofacial morphology of patients with bilateral complete cleft of primary palate (BCCPP) and bilateral complete cleft of secondary palate (BCCSP). In addition, to evaluate the differential effects of lip repair and palatal repair on the craniofacial morphology in cleft patients. Twenty-four children, 10 boys and 14 girls, mean age of 5.8 years, with BCCPP (BCCPP group). Twenty-five children, 10 boys and 15 girls, mean age of 5.6 years, with BCCSP (BCCSP group). Retrospective analysis. Cephalometric analysis was used to determine the craniofacial morphology at about 5 years of age. Compared with the BCCSP group, the BCCPP group demonstrated longer maxillary length, more protruded maxilla, more favorable jaw relation, more severely retroclined maxillary incisors, and larger overjet. The subjects with BCCPP had craniofacial characteristics that differed significantly from those with BCCSP. It could also be speculated that palatal repair had more adverse effect on the growth of the maxilla in length; however, that repair influenced less the anteroposterior position of maxillary incisors than lip repair did.