Craniofacial Morphology in Patients with Kallmann's Syndrome with and without Cleft Lip and Palate

1997 ◽  
Vol 34 (5) ◽  
pp. 417-424 ◽  
Author(s):  
Kirsten Mølsted ◽  
Inger Kjær ◽  
Aleksander Giwercman ◽  
Søren Vesterhauge ◽  
Niels Erik Skakkebæk
Keyword(s):  
Luteinizing Hormone ◽  
Cleft Lip ◽  
Cleft Lip And Palate ◽  
Hypogonadotropic Hypogonadism ◽  
Craniofacial Morphology ◽  
Endocrine Treatment ◽  
Anterior Cranial Base ◽  
Kallmann's Syndrome ◽  
Maxilla And Mandible ◽  
Kallmann’S Syndrome

Objective: Kallmann's syndrome is characterized by the association of hypogonadotropic hypogonadism and anosmia or hyposmia. The principal endocrine defect of hypogonadotropic hypogonadism is a failure to secrete luteinizing hormone-releasing hormone (LHRH), resulting in underdevelopment of the pituitary gonadotropes and an inability to synthesize and release luteinizing hormone and follicle-stimulating hormone. The purpose of the present investigation was to describe the dentition and the craniofacial morphology in patients diagnosed with Kallmann's syndrome. Design: The sample consisted of 11 patients, 2 of whom also had bilateral cleft lip and palate. Radiographic investigations, including cephalometry, were performed. Comparisons were made to normal individuals and to cleft lip individuals without Kallmann's syndrome. Results: Dentition: tooth agenesis occurred more frequently in patients with Kallmann's syndrome. Craniofacial morphology: Increased mandibular inclination and mandibular angulation were seen in Kallmann patients. When clefting also occurred, extreme retrognathism of both maxilla and mandible was seen, a deviation which seemingly worsened during growth. The anterior cranial base and the sphenoid bone showed an altered morphology in one of the patients with Kallman's syndrome. Conclusions: An early diagnosis of Kallmann's syndrome is very important because the prognosis for endocrine treatment thereby improves, and therefore, it is recommended that the sense of smell be evaluated in patients with the craniofacial morphology described.

Craniofacial Morphology in Patients with Kallmann's Syndrome with and without Cleft Lip and Palate

1997 ◽  
Vol 34 (5) ◽  
pp. 417-424 ◽  
Author(s):  
Kirsten Mølsted ◽  
Inger Kjær ◽  
Aleksander Giwercman ◽  
Søren Vesterhauge ◽  
Niels Erik Skakkebæk
Keyword(s):  
Cleft Lip ◽  
Cleft Lip And Palate ◽  
Craniofacial Morphology ◽  
Kallmann's Syndrome ◽  
Kallmann’S Syndrome

Three-Dimensional Analysis of the Pharyngeal Airway Volume and Craniofacial Morphology in Patients With Bilateral Cleft Lip and Palate

2020 ◽  
pp. 105566562094698
Author(s):  
Wenying Kuang ◽  
Jie Zheng ◽  
Shaolin Li ◽  
Shiyu Yuan ◽  
Hong He ◽  
...  
Keyword(s):  
Cleft Lip ◽  
Cleft Lip And Palate ◽  
Three Dimensional ◽  
Tertiary Hospital ◽  
Craniofacial Morphology ◽  
Class I ◽  
Oropharyngeal Airway ◽  
Pharyngeal Airway ◽  
Bilateral Cleft Lip ◽  
Maxilla And Mandible

Objective: This study aimed to determine the correlations between the craniofacial morphology and pharyngeal airway volume in patients with complete bilateral cleft lip and palate (BCLP). Design: Retrospective study. Setting: Tertiary hospital. Participants: Twenty-seven patients with complete BCLP and 27 class I control patients, aged 10 to 14 years. Main Outcome Measure: The pharyngeal airway volume and craniofacial morphology were evaluated using cone-beam computed tomography. Measurements were compared between groups and any correlations were identified. Results: A significantly smaller total pharyngeal airway volume (TPV), oropharyngeal airway volume, and upper (UOPV) and lower (LOPV) oropharyngeal airway volume were found in patients with BCLP than in class I control patients, with no difference in the nasopharyngeal volume between groups. Furthermore, the craniofacial morphology measurements of N-Me, S-Go, Or-C, Ptm-C, Me-C, Co-Go, Go-Me, Ptm-Or, N-S-Ar, and Ar-Go-Me significantly differed between the BCLP and control groups (all P < .05). Multiple regression analysis indicated that Ptm-C and Me-C; Ptm-C, Or-C, and Me-C; and Me-C explained 20.3%, 38.9%, and 17.1% of the variations in TPV ( P = .025), UOPV ( P = .002), and LOPV ( P = .018), respectively. Conclusions: Total pharyngeal airway volume, TPV, OPV, UOPV, and LOPV were significantly smaller in patients with BCLP than in class I controls. In patients with BCLP, the maxilla showed inhibited sagittal development and a retrograde position; moreover, the pharyngeal airway volume was weakly associated with the position of the maxilla and mandible relative to the coronal plane.

Craniofacial Morphology in Twins with Cleft Lip and Palate

1996 ◽  
Vol 33 (2) ◽  
pp. 96-103 ◽  
Author(s):  
Tuula Laatikainen ◽  
Reijo Ranta ◽  
Rolf Nordström
Keyword(s):  
Cleft Palate ◽  
Normative Data ◽  
Cleft Lip ◽  
Cleft Lip And Palate ◽  
Cranial Base ◽  
Craniofacial Morphology ◽  
Mandibular Angle ◽  
Gonial Angle ◽  
Maxilla And Mandible ◽  
Single Birth

The craniofacial morphology of 11 pairs of monozygotic (MZ) and 28 pairs of dizygotic (DZ) Finnish twins, discordant or concordant for cleft of the lip (CL), unilateral cleft lip and palate (UCLP), or cleft palate only (CP) were investigated by means of lateral cephalometric radiographs. The results were compared to those of age-, sex-, and cleft-type matched single-birth cleft subjects, and also with normative data from the Nordic population. The co-twins with no cleft lip or palate (NONC) showed only slightly more obtuse gonial and steeper mandibular angles compared to normative data. Twins with CL, UCLP or CP had a more retrusive mandible, a wider cranial base and mandibular angle, and a wider angle between the maxilla and mandible than did the single-birth cleft subjects. Comparison of the noncleft twin group with the CL, UCLP, and CP twin groups for the CL twins showed no significant differences. For the UCLP twins, a more retrusive and down- and backward rotation of both jaws, a wider gonial angle, and a wider cranial base angle was seen. The CP twins had their maxillae slightly retrusive, the down- and backward rotation of both jaws was apparent, and the gonial angle was more obtuse. A comparison between the noncleft MZ and noncleft CDZ twins showed no significant differences. The MZ CP twins had a more retrusive mandible and more down- and backward rotation of both jaws than did DZ CP twins. It thus can be suggested that twinning itself does not seem to have an effect on maxillofacial morphology, but the features of the mandibular structure, the cranial base angulation, and the inclination of the jaws are at least partly genetically induced.

scholarly journals Craniofacial Analysis May Indicate Co-Occurrence of Skeletal Malocclusions and Associated Risks in Development of Cleft Lip and Palate

2020 ◽  
Vol 8 (1) ◽  
pp. 2 ◽  
Author(s):  
Denise K. Liberton ◽  
Payal Verma ◽  
Konstantinia Almpani ◽  
Peter W. Fung ◽  
Rashmi Mishra ◽  
...  
Keyword(s):  
Cleft Lip ◽  
Cleft Lip And Palate ◽  
Morphological Changes ◽  
Principal Component ◽  
Craniofacial Morphology ◽  
Geometric Morphometric ◽  
Dentofacial Deformities ◽  
Dentofacial Deformity ◽  
Maxilla And Mandible ◽  
The Impact

Non-syndromic orofacial clefts encompass a range of morphological changes affecting the oral cavity and the craniofacial skeleton, of which the genetic and epigenetic etiologic factors remain largely unknown. The objective of this study is to explore the contribution of underlying dentofacial deformities (also known as skeletal malocclusions) in the craniofacial morphology of non-syndromic cleft lip and palate patients (nsCLP). For that purpose, geometric morphometric analysis was performed using full skull cone beam computed tomography (CBCT) images of patients with nsCLP (n = 30), normocephalic controls (n = 60), as well as to sex- and ethnicity- matched patients with an equivalent dentofacial deformity (n = 30). Our outcome measures were shape differences among the groups quantified via principal component analysis and associated principal component loadings, as well as mean shape differences quantified via a Procrustes distance among groups. According to our results, despite the shape differences among all three groups, the nsCLP group shares many morphological similarities in the maxilla and mandible with the dentofacial deformity group. Therefore, the dentoskeletal phenotype in nsCLP could be the result of the cleft and the coexisting dentofacial deformity and not simply the impact of the cleft.

Association of Distinct Craniofacial Features in Nonsyndromic Cleft Lip and Palate Family Members

2003 ◽  
Vol 40 (4) ◽  
pp. 397-402 ◽  
Author(s):  
Marja R. Perkiomaki ◽  
Young-Jooh Yoon ◽  
Ross H. Tallents ◽  
Ingrid Barillas ◽  
Roberto Herrera-Guido ◽  
...  
Keyword(s):  
Cleft Lip ◽  
Cleft Lip And Palate ◽  
Family Members ◽  
Costa Rican ◽  
Craniofacial Morphology ◽  
Normative Values ◽  
Anterior Cranial Base ◽  
Craniofacial Features ◽  
Relationship Of ◽  
The Relationship

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

Craniofacial Morphology in Twins with Cleft Lip and Palate

1996 ◽  
Vol 33 (2) ◽  
pp. 96-103 ◽  
Author(s):  
Tuula Laatikainen ◽  
Reijo Ranta ◽  
Rolf Nordström
Keyword(s):  
Cleft Lip ◽  
Cleft Lip And Palate ◽  
Craniofacial Morphology

Effects of Fetus Weight, Dam Strain, Dam Weight, and Litter Size on the Craniofacial Morphogenesis of CL/Fr Mouse Fetuses Affected with Cleft Lip and Palate

1997 ◽  
Vol 34 (4) ◽  
pp. 325-330 ◽  
Author(s):  
Kazuaki Nonaka ◽  
Yasunori Sasaki ◽  
Yoshihisa Watanabe ◽  
Ken-ichi Yanagita ◽  
Minoru Nakata
Keyword(s):  
Litter Size ◽  
Cleft Lip ◽  
Cleft Lip And Palate ◽  
Craniofacial Morphology ◽  
Strain Effect ◽  
Craniofacial Growth ◽  
Lateral Cephalogram ◽  
Mouse Fetus ◽  
Mouse Fetuses ◽  
Craniofacial Morphogenesis

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

Repair of Giant Anterior Skull Base Encephalocele Containing Intralesional Eloquent Brain: Technical Note

2021 ◽  
Author(s):  
Andrew J Kobets ◽  
Richard J Redett ◽  
Jonathan M Walsh ◽  
Joseph Lopez ◽  
Melike Guryildirim ◽  
...  
Keyword(s):  
Airway Obstruction ◽  
Skull Base ◽  
Cleft Lip ◽  
Cleft Lip And Palate ◽  
Neural Tissue ◽  
Anterior Skull Base ◽  
Csf Rhinorrhea ◽  
Hernia Sac ◽  
Live Births ◽  
Anterior Cranial Base

Abstract BACKGROUND Encephaloceles are herniations of intracranial neural tissue and meninges through defects in the skull. Basal encephaloceles are rare anterior skull base defects incident in 1 in 35,000 live births. Sphenoethmoidal encephaloceles are even more uncommon, with an incidence of 1 in 700,000 live births. Anterior skull base encephaloceles may be life-threatening in infants, presenting as airway obstruction and respiratory compromise. They can also present with cerebrospinal fluid (CSF) rhinorrhea, purulent nasal drainage, or meningitis. OBJECTIVE To report a novel technique for repairing a giant sphenoethmoidal encephalocele containing eloquent neural tissue. METHODS A 16-mo-old girl presented with progressive airway obstruction from a giant sphenoethmoidal encephalocele that filled her oral cavity. She had multiple congenital anomalies including agenesis of the corpus callosum and cleft lip and palate. Computed tomography showed complete absence of the bony anterior cranial base, and magnetic resonance imaging demonstrated the presence of the pituitary gland and hypothalamus in the hernia sac. RESULTS We repaired the encephalocele using a combined microsurgical and endoscopic multidisciplinary approach working through transcranial, transnasal, and transpalatal corridors. The procedure was completed in a single stage, during which the midline cleft lip was also repaired. The child made an excellent neurological and aesthetic recovery with preservation of pituitary and hypothalamic function, without evidence of CSF fistula. CONCLUSION The authors describe a novel multidisciplinary technique for treating a giant sphenoethmoidal encephalocele containing eloquent brain. The cleft lip was also repaired at the same time. The ability to work through multiple corridors can enhance the safety and efficacy of an often-treacherous operative endeavor.

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