Surgical Treatment of Lip Pits In The Van Der Woude Syndrome

نویسندگان

  • Rafael Santiago de Almeida
  • Willian Caetano Rodrigues
  • Willian Morais de Melo
  • Juliana Seo
  • Hitoshi Shinohara
چکیده

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. Introduction

برای دانلود رایگان متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Congenital lip pits and van der Woude syndrome.

Van der Woude syndrome is an autosomal dominant disease characterized by lower lip pits with or without cleft lip and/or cleft palate. The lip pits commonly have salivary glands that drain into them, which leads to salivary flow from the lip pits. Lip pits may be associated with submucosal palatal cleft, velopharyngeal insufficiency, or genitourinary or cardiovascular anomalies. The pits are tr...

متن کامل

The Van der Woude syndrome (dominantly inherited lip pits and clefts).

A patient with symmetrical lower lip pits with or without cleft lip or palate is most likely to be the carrier of a dominant gene that causes the syndrome of lip pits and clefts or Van der Woude syndrome. Familial occurrence of lower lip pits and clefts was first described by Demarquay in 1845.1 Watanabe et a12 reviewed some 100 cases in 1951, and Van der Woude delineated the syndrome in 1954.3...

متن کامل

Congenital lower lip pits (Van der Woude syndrome).

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 re...

متن کامل

Cleft lip and palate, lower lip pits, and limb deficiency defects.

Cleft lip or palate and lower lip pits are typical features of the autosomal dominantly inherited Van der Woude syndrome. Limb defects have not been reported in this syndrome so far. A girl with a unilateral complete cleft lip and palate, bilateral lower lip pits, and amniotic deformities of all four limbs is reported and the possibility of chance occurrence of cleft lip and palate, lower lip p...

متن کامل

Van der Woude syndrome- a syndromic form of orofacial clefting

Van der Woude Syndrome is the most common form of syndromic orofacial clefting, accounting for 2% of all cases, and has the phenotype that most closely resembles the more common non-syndromic forms. The syndrome has an autosomal dominant hereditary pattern with variable expressivity and a high degree of penetrance with cardinal clinical features of lip pits with a cleft lip, cleft palate, or bo...

متن کامل

[Van der Woude syndrome (report of a case)].

SummaryA rare case of Van Der Woude Syndrome, which is characterized by pits in the lower lip and bilateral cleft of the lip and cleft palate is presented. A multidisciplinary approach to treatment produced an aesthetically pleasing and functional outcome.

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

عنوان ژورنال:

دوره   شماره 

صفحات  -

تاریخ انتشار 2017