Case Reports

Van der woude syndrome: A case report and review

Nyoman Ayu Anggayanti , Endang Sjamsudin, Farah Asnely Putri

Nyoman Ayu Anggayanti
Departement of Oral and Maxillofacial Surgery, School of Dentistry, Faculty of Medicine, Universitas Udayana, Denpasar, Indonesia.. Email: ayu.anggayanti@unud.ac.id

Endang Sjamsudin
Departement of Oral and Maxillofacial Surgery, Faculty of Dentistry, Universitas Padjadjaran, Bandung, Indonesia

Farah Asnely Putri
Departement of Oral and Maxillofacial Surgery, Faculty of Dentistry, Universitas Padjadjaran, Bandung, Indonesia
Online First: December 01, 2020 | Cite this Article
Anggayanti, N., Sjamsudin, E., Putri, F. 2020. Van der woude syndrome: A case report and review. Indonesia Journal of Biomedical Science 14(2): 118-121. DOI:10.15562/ijbs.v14i2.269


Introduction: The prevalent autosomal disorder, with or without cleft palate and lip, is Van der Woude Syndrome (VWS). The condition has been known for its lower lip pits. Interferon Regulatory Factor 6 (IRF6) gene mutations are causing a range of cases of VWS. The furrows caused an accumulation of mucous and felt esthetic pain during meals and crying.

Purpose: To determine VWS causes, prevalence, treatment, symptoms, and diagnosis.

Case report: A six-month-old baby was with lip pits, a bilateral cleft lip and palate since birth. The extraoral exam showed bilateral pit swellings with dome form on the lower vermilion boundary laterally to the midline. Simultaneous operations with the Nordhoff technique and simple excision were conducted combined with a breakthrough procedure under general anesthesia to correct bilateral cleft lips. The patient showed better clinical condition after the seventh-day post-surgery without infection sign, and bilateral cleft in lower lip pit closed in a month after surgery.

Conclusion: VWS is generally not known and not always diagnosed. It is peculiar that the cleft palate and lip phenomenon is routinely integrated into the same pedigree. The patient with a lip pit can be thoroughly examined, for example, by revealing the secret shape of the cleft. In this case, the surgical repair was handled simultaneously to both functional and esthetic aspects to achieve satisfying results.

References

Tehranchi A., Behnia H., Nadjmi N., et al. Multidsiciplinary management of a patient with van der Woude syndrome: A case report. International Journal of Surgery Case Reports. 2017;30:142–147.

Bertin H., Diallo-Hornez, Isidor B., et al. Surgical management of lower lip pits in Van der Woude syndrome. J Stomatol Oral Maxillofac Surg. 2018;119:67–70.

Retno Widayanti, et al. Congenital double lip pits: A case report. Padjadjaran Journal of Dentistry. 2016;28(1):62-67.

Baghestani S., Sadeghi N., Yavarian M., et al. Lower lip pits in a patient with van der Woude syndrome. J Craniofac Surg. 2010;21:1380-1381.

Lam AK., David DJ., Townsend GC, et al., Van der Woude syndrome: dentofacial features and implications for clinical practice. Australian Dental Journal. 2010;55: 51–58.

Trishala.A et al., Van Der Woude Syndrome – A Review. J. Pharm. Sci. & Res. 2016;8(6):495-497.

Krauel L., Jose P., Munoz E., Gean E., et al. Van der Woude syndrome and lower lip pits treatment. J. Oral Maxillofac Surg. 2008;66:589-592.

Hasan S., Ishrat Khan N., Van der Woude syndrome-a case report. Recent Research in Science and Technology. 2011;3(12):53-57 50.

Ziai MN., Bensin AG., et al., Congenital lip pits and van der Woude syndrome. The journal of craniofacial surgery. 2008;16(5).


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