scholarly journals Rare Expression Of Van Der Wound Syndrome: A Case Report Revisited

2017 ◽  
Vol 3 (2) ◽  
pp. 61-65
Author(s):  
Kristaninta Bangun ◽  
Nurina Widayanti ◽  
Gentur Sudjatmiko
Keyword(s):  
Cleft Lip ◽  
Cleft Lip And Palate ◽  
Mouth Opening ◽  
Careful Examination ◽  
Van Der Woude Syndrome ◽  
Lower Lip ◽  
Genital Abnormality ◽  
Craniofacial Syndrome ◽  
Oral Abnormalities ◽  
Left Eyelid

Background: Van der Woude syndrome (VWS) is a rare developmental malformation, characterized by pits in the lower lip. Van der Woude syndrome is an autosomal dominant craniofacial syndrome with various expression: lower lip pits, cleft lip with or without cleft palate, syngnathia, hypoodontia, and ankyloglossia. Extra-oral abnormalities findings can be found: syndactily, corpus callosum dysgenesis, megacolon, ventricular septal defect and genital abnormality. Methods: We reported a case of 5-month-old male with rare expression of VWS: bilateral cleft lip and palate, syngnathia, lower lip pits, ptosis of upper left eyelid and macropenis. Results: We perform surgery to release the fibrous band to achieve satisfactory maximum mouth opening. Next we perform cheiloplasty and lower lip pit removal. Conclusion: Proper surgical intervention in VWS patients can improve feeding and prevent further temporomandibular complications. Careful examination of patients with cleft lip and lower lip pit should be done to avoid misdiagnosis.

Prenatal diagnosis and management of Van der Woude syndrome

2016 ◽  
Vol 5 (1) ◽  
pp. 61-63
Author(s):  
Karla Ferreres García ◽  
Beatriz Berenguer ◽  
Luis Ortiz Quintana ◽  
Elena De Tomás Vicente ◽  
Ricardo Fernández Pérez-Pacheco ◽  
...  
Keyword(s):  
Cleft Lip ◽  
Autosomal Dominant ◽  
Cleft Lip And Palate ◽  
Ultrasound Examination ◽  
Craniofacial Anomalies ◽  
Dominant Inheritance ◽  
Van Der Woude Syndrome ◽  
Lower Lip ◽  
Postnatal Diagnosis ◽  
Variable Penetrance

Abstract We report the postnatal diagnosis of Van der Woude syndrome (VWS) in a foetus found to have an isolated right cleft lip and palate by ultrasound examination. After prenatal genetic counselling, the parents declined further evaluation by amniocentesis. At delivery, the infant was also found to have labial pits in the lower lip in addition to the cleft lip and palate identified by ultrasound consistent with VWS. Although VWS is rare, its autosomal dominant inheritance and variable penetrance should prompt additional modalities to more thoroughly evaluate the extent of other organ system and more extensive craniofacial anomalies.

scholarly journals Functional Outcomes of Cleft Lip Surgery. Part II: Quantification of Nasolabial Movement

2007 ◽  
Vol 44 (6) ◽  
pp. 607-616 ◽  
Author(s):  
Carroll-Ann Trotman ◽  
Julian J. Faraway ◽  
H. Wolfgang Losken ◽  
John A. van Aalst
Keyword(s):  
Cleft Lip ◽  
Cleft Lip And Palate ◽  
Alveolar Bone ◽  
Mouth Opening ◽  
Upper Lip ◽  
Vertical Movements ◽  
Lower Lip ◽  
Lower Jaw ◽  
Lip Movement ◽  
The Asymmetry

Objective: To explore nasolabial movements in participants with repaired cleft lip and palate. Design: A parallel, three-group, nonrandomized clinical trial. Subjects: Group 1 = 31 participants with a cleft lip slated for revision surgery (revision), group 2 = 32 participants with a cleft lip who did not have surgery (nonrevision), and group 3 = 37 noncleft control participants. Methods: Three-dimensional movements were assessed using a video-based tracking system that captured movement of 38 landmarks placed at specific sites on the face during instructed maximum smile, cheek puff, lip purse, mouth opening, and natural smile. Measurements were made at two time points at least 1 week and no greater than 3 months apart. Summary measurements were generated for the magnitude of upper lip, lower lip, and lower jaw movements and the asymmetry of upper lip movement. Separate regression models were fitted to each of the summary measurements. Results: Lateral movements of the upper lip were greater than vertical movements. Relative to the noncleft group, the revision and nonrevision groups demonstrated 6% to 28% less upper lip movements, with the smiles having the most restriction in movement and greater asymmetry of upper lip movement. Having an alveolar bone graft further increased the asymmetry, while a bilateral cleft lip decreased the asymmetry. Lower jaw movement caused a small increase in upper lip movement. Conclusions: The objective measurement of movement may be used as an outcome measure for cleft lip surgery.

Congenital Lower Lip Pits (Van der Woude Syndrome): Report of a Case

2006 ◽  
Vol 7 (1) ◽  
pp. 134-140 ◽  
Author(s):  
Zuhal Kirzioğlu ◽  
Münciye Semra Özay Ertürk
Keyword(s):  
Cleft Lip ◽  
Autosomal Dominant ◽  
Cleft Lip And Palate ◽  
Dental Treatment ◽  
Surgical Removal ◽  
Supernumerary Tooth ◽  
Autosomal Dominant Disorder ◽  
Van Der Woude Syndrome ◽  
Lower Lip ◽  
Syndrome Report

Abstract Van der Woude syndrome (VWS) is a rare autosomal dominant disorder that is characterized by a cleft lip and palate with congenital lip pits. This is a report of a case of VWS with sinuses in the lower lip, a cleft in the upper lip, and a supernumerary tooth in the maxilla. The main characteristics of this disorder are discussed. Dental treatment of the patient was performed, but the surgical removal of the sinus was rejected by the parents. This case report brings this condition to the attention of dentists and surgeons and emphasizes lip pits may not always be identical in appearance. Citation Kirzioğlu Z, Ertürk MSÖ. Congenital Lower Lip Pits (Van der Woude Syndrome): Report of a Case. J Contemp Dent Pract 2006 February;(7)1:134-140.

Soft-Tissue Profile Changes during Widening and Protraction of the Maxilla in Patients with Cleft Lip and Palate Compared with Normal Growth and Development

1993 ◽  
Vol 30 (5) ◽  
pp. 454-468 ◽  
Author(s):  
Rolf S. Tindlund ◽  
Per Rygh
Keyword(s):  
Soft Tissue ◽  
Cleft Lip ◽  
Cleft Lip And Palate ◽  
Lower Lip ◽  
Maxillary Protraction ◽  
Transverse Expansion ◽  
Short Period ◽  
Soft Tissue Profile ◽  
Lateral Cephalograms ◽  
Maxillary Deficiency

During the last 15 years, cleft lip and palate (CLP) patients with maxillary deficiency in the care of the Bergen CLP team have undergone an interceptive orthopedic treatment phase during the deciduous and mixed dentition period. The present study includes 68 patients who received maxillary transverse expansion by use of a modified quad-helix appliance and 98 cases given maxillary protraction by a facial mask. All cases were treated until an acceptable normal occlusion was attained. Lateral cephalograms were taken immediately before and after the active treatment periods. Sagittal changes of the soft-tissue profile during transverse expansion and protraction were analyzed separately for unilateral complete cleft lip and palate (UCLP) patients and bilateral complete cleft lip and palate (BCLP) patients. The soft-tissue profiles of the groups were compared to growth changes of noncleft age-matched children (NORM group). During the short period of maxillary transverse expansion (mean period, 3.5 months) no significant change of the soft-tissue profile was found, except in the protrusion of the lower lip in the BCLP group. During the period of maxillary protraction (mean periods, 12 months in the UCLP group and 15 months in the BCLP group) the soft-tissue profile improved significantly by reducing the characteristic tendency towards a concave profile in CLP patients with maxillary deficiency. Significant Increases of the sagittal maxillomandlbular lip relation (angle SS-N-SM: mean Increase, 2.5 degrees) and the Holdaway-angle (H-angle: mean increase, 3.0 degrees) were found to be similar in the UCLP and BCLP groups. However, the use of different reference lines for evaluation of treatment effects upon the soft-tissue profile resulted in conflicting findings suggesting that anteriorly situated reference lines are more suitable for the evaluation of CLP patients. Thus, the esthetic line (E.line) indicated a favorable position of the lips after treatment; the subspinale-pogonlon line (ss.pg) revealed an Improved soft-tissue profile; the soft-tissue-facial line (N.PG) showed a retruded nose and upper lip; whereas basal references such as the nasion-sella line (NSL) and the occlusal-line perpendicular (OLP) mainly showed major differences between the CLP groups and the NORM groups.

A Cephalometric Evaluation of Lower Lip in Patients with Unilateral Cleft Lip and Palate

2004 ◽  
Vol 41 (5) ◽  
pp. 485-489 ◽  
Author(s):  
T. Ufuk Toygar ◽  
M. Okan Akçam ◽  
Ayça Arman
Keyword(s):  
Cleft Lip ◽  
Cleft Lip And Palate ◽  
Normal Subjects ◽  
Middle Part ◽  
Class I ◽  
Control Group ◽  
Upper Lip ◽  
Lower Lip ◽  
Normal Individuals ◽  
Lateral Cephalograms

Objective The aim of this study was to evaluate cephalometrically the lower lip position and area of patients with unilateral cleft lip and palate (UCLP) comparatively with Class I skeletodental normal subjects. Patients Lateral cephalometric and hand-wrist radiographs obtained from 24 patients with UCLP (mean age 12.86 years), along with 20 normal individuals (mean age 12.33 years) used as a control group, were examined. Design In addition to standard cephalometric dentofacial variables, lower lip area (superior, middle, inferior) was also measured using a digital planimeter on the lateral cephalograms. Results The superior and middle part of the lower lip areas were significantly smaller (p < .05) in the UCLP group, compared to the control group. The inferior and total lower lip areas of patients with UCLP were found to be significantly smaller than controls. The labiomental angle was also smaller (38.79 degrees). Conclusions The results suggest that the lower lip of patients with UCLP is smaller, retruded, and curved, with a deep labiomental sulcus, compared with normal individuals during puberty. Therefore, practitioners should focus not only on the upper lip of patients with UCLP but also should consider the lower lip as it was found distinct from normal individuals during puberty.

Cleft Palate and Congenital Synechiae Syndrome: A Case Report

2004 ◽  
Vol 41 (2) ◽  
pp. 206-207 ◽  
Author(s):  
Siun M. Murphy ◽  
Suzanne Rea ◽  
Eleanor McGovern ◽  
Paddy Fleming ◽  
David Orr
Keyword(s):  
Case Report ◽  
Soft Tissue ◽  
Cleft Lip ◽  
Cleft Lip And Palate ◽  
Mouth Opening ◽  
Baby Girl ◽  
Early Management ◽  
Secondary Palate ◽  
Normal Feeding ◽  
Oral Development

Objective A 1-day-old baby girl with a cleft of the secondary palate and a soft tissue band connecting the upper and lower jaws and preventing mouth opening was referred to the cleft lip and palate team by her pediatrician. This case represents a further example of an interesting but rare anomaly known as congenital alveolar synechia syndrome that requires early management to allow normal feeding and oral development.

scholarly journals Congenital double lip pits

2016 ◽  
Vol 28 (1) ◽  
Author(s):  
Retno Widayanti ◽  
Melita Sylvyana ◽  
Endang Syamsudin ◽  
Rizki Diposarosa
Keyword(s):  
Cleft Lip ◽  
Cleft Lip And Palate ◽  
Early Stage ◽  
Fistulous Tract ◽  
Surgical Excision ◽  
Surgical Removal ◽  
Lower Lip ◽  
Stage Of Development ◽  
Developmental Disturbances ◽  
Bilateral Cleft Lip

Introduction : Lip pits are unusual congenital anomalies affecting the lip, first described by DeMurquay in 1845. Lip pits can encounter aesthetic problem thus the patient ask for surgical overcome. Case Report : A 4-year-old girl patient came with double lower lip pits that present since birth with bilateral cleft lip and palate. The pits made a mucous accumulation occurs during mealtimes and crying, and felt aesthethic discomfort. The surgery was performed by simple excision combined with split-lip advancement technique under general anaesthesia and the excised pits was then analized for histopatological structures. The patient has no aesthetic defect after surgery. Discussion : Congenital lip pits are developmental anomalies that occur as an isolated defect or either in association with other developmental disturbances. It happens due to notching of lip at an early stage of development with fixation of tissues of the base of the notch or from a failure of complete union of embryonic lateral sulci of the lip. Lip pits can be shallow or deep, and may be associated with accessory salivary glands. The treatment is usually surgical excision with removal of entire fistulous tract. Conclusion : Surgical removal of lip pits is commonly for cosmetic purpose. It must be treated wisely because lips are essential part of someones face.

Lip Movement in Patients with a History of Unilateral Cleft Lip

2001 ◽  
Vol 38 (5) ◽  
pp. 468-475 ◽  
Author(s):  
Christel A. W. Rutjens ◽  
Paul H. M. Spauwen ◽  
Pascal H. H. M. van Lieshout
Keyword(s):  
Cleft Lip ◽  
Relative Phase ◽  
Cleft Lip And Palate ◽  
General Increase ◽  
Lower Lip ◽  
History Of ◽  
Lip Movement ◽  
Cleft Lip Repair ◽  
Palate Repair ◽  
The Stability

Objective: The influence of a repaired cleft lip on the stability of coordination between upper and lower lip in nonspeech and speech tasks was investigated. Design: First, we looked at the effects of a secondary cleft lip repair in three individuals. Second, we compared subjects with a history of repaired unilateral cleft lip and subjects with no history of cleft lip (controls). Lip coordination was measured using continuous estimates of relative phase. Participants: Subjects were nine children and adolescents with a primary unilateral cleft lip and palate repair and 4 participants without cleft matched for age across different age categories. Results: In general, the averaged relative phase angle (RPA) angle values were smaller than 180 degrees, indicating an upper lip lead for lip closure. Controls showed a tendency toward a more symmetric type of coordination (close to 180 degrees), compared with subjects with a repaired unilateral cleft lip. The controls also showed less variation in coordination between the lips. For the more complex speech tasks, a general increase in variability of the RPA values for all subjects was observed, most likely suggesting a more flexible type of coordination. Regarding the effect of a secondary cleft lip repair, only one of the three patients showed a clearly less symmetric and less stable type of coordination, compared with preoperation results. Conclusions: There appear to be differences in lip coordination between speakers without and speakers with a repaired unilateral cleft lip. Furthermore, it seems that the stability of lip coordination tends to increase with age.

Rare and Multiple Hypodontia in Van der Woude Syndrome: Case Report

2021 ◽  
pp. 105566562110582
Author(s):  
Aline Cristina da Silva Trevizan ◽  
Andréa Guedes Barreto Gonçales ◽  
Bruna Stuchi Centurion Pagin ◽  
Otávio Pagin ◽  
Lucimara Teixeira das Neves
Keyword(s):  
Case Report ◽  
Cleft Lip ◽  
Alveolar Bone ◽  
Detailed Examination ◽  
Permanent Teeth ◽  
Missing Teeth ◽  
Van Der Woude Syndrome ◽  
Lower Lip ◽  
The Family

Van der Woude syndrome (VWS) is a rare syndrome of genetic etiology, commonly occasioned by mutations in the IRF6 gene and that causes disorders in craniofacial development. VWS is characterized by the presence of paramedian fistulas in the lower lip and cleft lip and / or cleft palate. Although some dental phenotypes have been reported in this syndrome, multiple and rare hypodontias were not described. Through this case report, we present a case of Van der Woude Syndrome (VWS) with rare and multiple hypodontia in which clinical data and radiographic exams were evaluated. The patient presented hypodontia of eight permanent teeth (lateral incisors, second premolars and second molars). So, when the dentist recognizes multiple and/or rare hypodontias, for an accurate diagnosis, detailed examination of the lower lip is indicated, as well as a survey of the family history and referral for genetic counseling, since the syndrome presents high penetrance. The patient is expected to be rehabilitated to have a good quality of life. Rehabilitation in these cases requires alveolar bone graft, orthodontics and prosthesis to replace missing teeth.

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