Congenital lower lip pits (Van der Woude syndrome): presentation of 10 cases.

The objective of the present study was to evaluate the glands of wall of congenital fistulae of the lower lip with the transmission electron microscope in order to characterize their microstructural pattern. Thin section of Araldite resin embedded congenital fistulae of the lower lip of four patients with Van der Woude syndrome from the Hospital de Reabilitação de Anomalias Craniofaciais da Universidade de São Paulo, Bauru, SP, were analyzed with a transmission electron microscope. The results showed that the glands were mostly made by typical mucous acini exhibiting, with certain frequency, myoepithelial cells surrounding them. In some of lobules, a few acini smaller than the typical mucous, showed granules of moderate electron density or containing a dense core or exhibiting small dense spherule and predominance granular material. These granules resemble to described recently by others in various human minor salivary glands. We concluded that glands associated with congenital fistula of lower lip of patients with Van der Woude syndrome, in spite of being located in vermilion border of the lip, showed at the transmission electron microscope characteristics of labial minor salivary gland, i.e, are mostly mucous with a few seromucous units, while typical seromucous demilunes are not present.

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Congenital Fistulae of the lower Lip in Van der Woude Syndrome: A Histomorphological Study

The Cleft Palate-Craniofacial Journal ◽

10.1597/1545-1569_1999_036_0079_cfotll_2.3.co_2 ◽

1999 ◽

Vol 36 (1) ◽

pp. 79-85 ◽

Cited By ~ 1

Author(s):

Mirian Aparecida Onofre ◽

Heli Benedito Brosco ◽

João Urias Brosco ◽

Rumio Taga

Keyword(s):

Van Der Woude Syndrome ◽

Lower Lip

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Prenatal diagnosis and management of Van der Woude syndrome

Case Reports in Perinatal Medicine ◽

10.1515/crpm-2015-0037 ◽

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.

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Congenital Lower Lip Pits (van der Woude Syndrome): What Pathologists Need to Know

Pediatric and Developmental Pathology ◽

10.2350/13-02-1302-oa.1 ◽

2013 ◽

Vol 16 (5) ◽

pp. 343-347

Author(s):

Rong Fan ◽

Roberto L. Flores ◽

Philip R. Faught ◽

Jingmei Lin

Keyword(s):

Van Der Woude Syndrome ◽

Lower Lip

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FURTHER ASPECTS OF VAN DER WOUDE SYNDROME (CONGENITAL LOWER LIP PITS)

Pediatric Dermatology ◽

10.1111/j.0736-8046.2004.21125.x ◽

2004 ◽

Vol 21 (1) ◽

pp. 92-92 ◽

Cited By ~ 5

Keyword(s):

Van Der Woude Syndrome ◽

Lower Lip

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Congenital Lower Lip Pits (Van der Woude Syndrome): Presentation of 10 Cases

Pediatric Dermatology ◽

10.1111/j.1525-1470.1998.tb01391.x ◽

2009 ◽

Vol 15 (6) ◽

pp. 443-445 ◽

Cited By ~ 2

Author(s):

Eduardo Nagore ◽

José M. Sánchez-Motilla ◽

M. Isabel Febrer ◽

Gabriel Serrano ◽

Juan Bonillo ◽

...

Keyword(s):

Van Der Woude Syndrome ◽

Lower Lip

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Lower Lip Pits in a Patient With van der Woude Syndrome

Journal of Craniofacial Surgery ◽

10.1097/scs.0b013e3181edc528 ◽

2010 ◽

Vol 21 (5) ◽

pp. 1380-1381 ◽

Cited By ~ 6

Author(s):

Shahram Baghestani ◽

Naser Sadeghi ◽

Majid Yavarian ◽

Hekmat Alghasi

Keyword(s):

Van Der Woude Syndrome ◽

Lower Lip

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

The Cleft Palate-Craniofacial Journal ◽

10.1177/10556656211058265 ◽

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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