Surgical Treatment of Lip Pits In The Van Der Woude Syndrome

Abstract

glands in the lip pits. As extra-oral manifestations, congenital heart defect, accessory nipple and Hischsprung disease can be found. It has an incidence of 1 in 75,000 to 100,000 [1-4,6]. Therefore, the aim of this article is to describe the clinical findings of VWS through a report of a case in addition to reviewing the etiology, treatment of oral lesions as well as the management of the patient and family, taking into consideration the hereditary factor. Case History Seventeen-year-old female patient was evaluated with the complaint of pit-shaped injury located in semi lower lip mucosa, since birth, and there was no salivary secretion. During the intra oral examination, bifid uvula was noted, others mucous membranes showed normal aspects of coloration and texture and teeth were in excellent conditions of hygiene and conservation. It was asymptomatic and the patient complained predominantly about aesthetic. Good general health condition was noted and the medical history was unchanged (Figure 1A). After what was mentioned above, a presumptive diagnosis of VWS was made. During anamnesis a similar familiar history was not described, but the patient was referred to a clinical physician for cardiac evaluation, where a normal cardiac standard was found. After planning, surgical correction was carried out of the labial pits through navicular incision along the lower lip semi mucosa (Figure 1B), followed by excision. After controlling hemostasis, layered closure was performed (Figure 1C). Histopathological examination showed fragment of mucosa coated by parakeratinized stratified squamous epithelium. Lamina propria consists of dense connective tissue, exhibiting moderate mononuclear inflammatory infiltrate predominantly lymphocytic near the epithelium. In the sub mucosa region mature adipocytes were observed (Figure 2). Postoperative follow-up showed good cosmetic results and good patient satisfaction (Figure 1D). The patient was counseled regarding the hereditary characteristics of the syndrome and the need for monitoring and early approach in any cases of lips and palate clefts of the descendants.