Neuromodulator for the Treatment of Congenital Unilateral Lower Lip Palsy.

INTRODUCTION:: Congenital unilateral lower lip palsy is an infrequently encountered condition that manifests as lower lip asymmetry during smiling, laughing, and crying. Treatment options are not well characterized. METHODS:: The authors present the case of a 51-year-old woman who was referred for surgical intervention for facial paralysis. Physical examination demonstrated a symmetric face at rest that became asymmetric when smiling. The asymmetry, evident by inappropriate inferior displacement of the lower lip, was secondary to unilateral contraction and presence of the depressor labii inferioris. The depressor anguli oris was symmetric bilaterally. Her presentation was consistent with congenital unilateral lower lip palsy. RESULTS:: Lidocaine was injected into the depressor labii inferioris on the side of the face that demonstrated unilateral presence and contraction. This resulted in symmetry of the smile and lower lip without untoward effect. Onabotulinum toxin A was thereafter injected into the depressor labii inferioris. In-office treatment with botulinum toxin injection resulted in a 4-month improvement in smile symmetry. CONCLUSION:: Chemodenervation is a safe and minimally invasive method to improve smile symmetry and lower lip position in cases of congenital unilateral lower lip palsy.

Congenital Midline Upper Lip Sinuses: 3 Rare Cases.

Congenital sinuses of the upper lip are rare congenital malformations. There have been only 40 cases described in the literature. We report 3 cases of congenital midline upper lip sinus in Caucasian children. Two of those lesions were associated with other anomalies (complete cleft palate and hemifacial macrosomia or submucous cleft palate with bifid uvula). The pathophysiology remains unexplained yet. Congenital upper lip sinuses can be considered as possible microforms of cleft-lips. Associated anomalies are frequent and must be sought. The treatment is a full excision of the sinus tract and of the skin around the punctum in order to avoid risks of recurrence.

Diseases of the lips.

Heath care providers should be comfortable with normal as well as pathologic findings in the lips, because the lips are highly visible and may display clinical manifestations of local, as well as systemic inflammatory, allergic, irritant, and neoplastic alterations. Fortunately, the lips are easily accessible. The evaluation should include a careful history and physical examination, including visual inspection, as well as palpation of the lips and an examination of associated cervical, submandibular, and submental nodes. Pathologic and microscopic studies, as well as a review of medications, allergies, and habits, may further highlight possible etiologies. Many lip conditions, including premalignant changes, are relatively easy to treat, when the abnormalities are detected early; however, advanced disease and malignancies are challenging for both the patient and clinician. Treatment should be focused on eliminating potential irritants or allergens and treatment of the primary dermatosis. In this paper we review physiologic variants as well as pathologic conditions of the lips.

Congenital Midline Sinus of the Upper Lip.

An extremely rare patient of congenital midline sinus of the upper lip in a 2-year-old boy is presented. The sinus was located on the midline of the philtrum just below the base of the columella. Surgical removal of the sinus was performed via an extra- and inraoral approach. Histopathological examination of the resected sinus revealed it to be lined by keratinized stratified squamous epithelium with sebaceous glands and hair follicles. Three postulates can account for the formation of the congenital midline sinus of the upper lip: the fusion theory, the merging theory, and the invagination theory. However, the etiology of this rare abnormality remains obscure.

Congenital labial mucocele: rare presentation of a common disease.

Oral mucoceles are common lesions characterized by accumulation of mucus following rupture of a minor salivary gland duct. However, congenital mucoceles are a rare and distinctive oral condition observed in newborns. This case report details the features of a congenital labial nodule diagnosed as congenital mucocele. These lesions are rare in the oral cavity and should be diagnosed in the delivery room, but many cases are referred for further evaluation. Management is simple, and recurrence is not expected.

[Median congenital fistula of the upper lip associated with Goldenhar syndrome: Report of a case]. / Fistule congénitale médiane de la lèvre supérieure associée à un syndrome de Goldenhar : à propos d'un cas.

AIM OF THE STUDY: We present the case of a patient with Goldenhar syndrome associated with congenital fistula in the middle of philtrum at the upper lip. PATIENTS AND METHODS: The patient was supported from birth for Goldenhar syndrome. Several procedures were performed in childhood to correct a macrostomia and mandibular hypoplasia right. At 14 years old, she has a rhinoplasty to correct a complex nasal malformation. During the procedure, a hole in the upper central incisor inter-space is found. It is extended by a fistula which runs through the front palate towards the vomer rail at the base of the partition. This fistula is blind behind the vomer and can be completely resected. Pathological examination of the resection reveals a squamous lining. RESULTS: The median fistula of the upper lip are extremely rare : less than 30 cases reported in the literature. The unusual run of the fistula and the recent appearance of a pituitary syndrome in this patient makes us look for a continuity between it and Rathke's pocket by computed tomography imaging type. CONCLUSION: The association of Goldenhar syndrome and median congenital fistula of the upper lip was never yet described in the literature. The association with hypopituitarism was suspected with the posterior extension of the malformation, not confirmed by the scanner, but strongly suspected jointly by the neurosurgical and plastic surgery team.

[Labial recurrent swelling revealing median congenital upper-lip fistula]. / Tuméfaction labiale récidivante révélatrice d'une fistule congénitale médiane de la lèvre supérieure.

Midline upper-lip fistulas are an extremely rare variant of congenital facial malformations. Less than 30 cases have been reported in the literature since 1970. We report the case of a 2 and a half-year-old girl presenting with a median congenital blind fistula of the upper lip, without any relation with the oral cavity. A recurrent swelling of the upper lip was the main symptom. Complete surgical excision of the cyst or of the fistulous tract must be obtained to avoid recurrence.

[Child care management in maxillofacial humanitarian mission]. / Prise en charge de l'enfant en mission humanitaire maxillofaciale.

Our practice in a humanitarian (or crisis) context differs from what we experience in daily practice. There are several reasons for this. First, the diseases encountered are sometimes unfamiliar, such as sequelae of noma, or the presentation of familiar diseases may be unusual, such as facial malformations seen at a late stage. Secondly, these missions take place in developing countries, and consequently, evaluation and anticipation of possible malnutrition should be considered, especially because facial diseases themselves may be responsible for nutritional problems. Lastly, conditions are often difficult, occurring in an unusual environment, and we sometimes have to face communication and equipment problems. The goal of our work, based on a 15-year experience (in Bamako and Mopti with the Association "Santé et Développement", and in Ouagadougou with the organization "Les enfants du noma") and the analysis of literature, is to point out these features and maybe to be helpful to others.

Congenital midline sinus of the upper lip.

A congenital lip sinus is a rare condition that has been reported to occur in both the upper and lower lips, either in isolation or in association with congenital deformities such as a cleft lip and palate in Van der Woude syndrome. The prevalence of lower lip sinuses has been estimated to be about 0.00001% of the white population. Upper lip sinuses are even more uncommon. To date, there have been several case reports of upper lip sinuses and fistulas, but no similar cases have been described in Singapore. We herein report a case of congenital upper lip sinus presenting as a recurring upper lip abscess and review the current literature on this condition.

An alternative technique using a gutta percha points and blue methylene to excision of congenital fistula of lower lip in patient with Van der Woude syndrome.

Van der Woude syndrome is a rare disorder of craniofacial development, characterized by the triad (congenital lip fistula, cleft lip and/or cleft palate). Discomfort caused by spontaneous or induced drainage of saliva/mucus when pressure is applied or during a meal as well as poor aesthetics match is one of the main complaints of patients with congenital lip fistula. This paper aims to describe an alternative technique for excision of the congenital fistula of lower lip in patient with Van der Woude syndrome, using gutta percha points and methylene blue for better identify the the fullest extent of the fistula. This approach aims to facilitate a dissection of fistula tissues providing better preservation of the tissues around the fistula, faster healing and better aesthetics lip of the patient.

A case of congenital midline fistula of the upper lip.

Congenital fistulas of the lip are commonly found in the lower lip and accompany cleft lip. They are seen as a symptom of Van der Woude syndrome, which is predominantly hereditary. In contrast, congenital fistulas of the upper lip are rare. A number of hypotheses have been proposed to explain the pathogenesis of fistulas of the upper lip, including fusion failure of facial prominences and absence of mesoblasts, suggesting a relationship between this condition and the development of cleft lip. The pathogenesis of this disorder has been attracting attention. We report the case of a 5-year-old girl with congenital fistula of the upper lip.

Van der Woude syndrome: dentofacial features and implications for clinical practice.

BACKGROUND: Van der Woude syndrome (VWS) is the most common clefting syndrome in humans. It is characterized by the association of congenital lower lip fistulae with cleft lip and/or cleft palate. VWS individuals have a high prevalence of hypodontia. Although caused by a single gene mutation, VWS has variable phenotypic expression. This study aimed to describe the range of clinical presentations in 22 individuals with VWS to facilitate its diagnosis. METHODS: A retrospective study of 22 patients with a diagnosis of VWS was undertaken at the Australian Craniofacial Unit (ACFU) in Adelaide. Three extended families with affected members were included in the study cohort. RESULTS: The overall prevalence of lip pits in this study cohort was 86%. Cleft phenotypes included bilateral cleft lip and palate (32%); unilateral cleft lip and palate (32%); submucous cleft palate (23%); and isolated cleft hard and soft palate (9%). Missing permanent teeth were reported in 86% of affected individuals. CONCLUSIONS: Submucous cleft palate in VWS may go undiagnosed if the lower lip pits are not detected. Associated hypodontia and resultant malocclusions will also require management by a dental team.

Congenital bilateral lower lip pits associated with fistulae of the minor salivary glands: case report of the principal Van der Woude syndrome's trait.

BACKGROUND: Van der Woude syndrome (VWS; OMIM 119300) is an autosomal dominant developmental malformation presenting with bilateral lower lip pits related to cleft lip, cleft palate, and other malformations in about half the patients. These congenital lip pits appear clinically as a defect in the vermilion border of the lip. They are commonly related to fistulae of minor salivary glands with or without excretion. Usually the cause of cosmetic defects, VWS is preferably treated through simple excision. This report aims to describe a case of VWS involving a patient who presented with isolated congenital bilateral lower lip pits related to fistulae of the minor salivary glands, which were treated successfully through simple excision. CASE REPORT: A 5-year-old girl with a diagnosis of congenital bilateral lip pits associated with fistulae of minor salivary glands was treated successfully through simple excision. The diagnosis was confirmed by clinical examination, sialography, and scintigraphy. A good aesthetic result was observed after 2 years of follow-up evaluation. CONCLUSION: Physicians must be aware of VWS because, although underreported and frequently not diagnosed, it is the most common cleft syndrome.

Surgical correction of congenital lower lip sinuses in Van der Woude syndrome.

OBJECTIVE: To review the clinical outcome for patients with Van der Woude syndrome undergoing surgical excision of congenital lip sinuses. DESIGN: Retrospective chart review. SETTING: Multidisciplinary cleft-craniofacial team within a tertiary care hospital. PATIENTS, PARTICIPANTS: Seventeen patients with Van der Woude syndrome were identified from hospital records as having cleft lip and/or palate surgery performed at the University of Iowa, and six of these patients underwent simple surgical excision of lower lip sinuses. MAIN OUTCOME MEASURES: Incidence of postoperative complications and number of surgeries required per patient for correction of lower lip defect. RESULTS: All six patients undergoing surgical excision of lip sinuses had postoperative mucocele formation. These uniformly required one or two further surgeries for correction. There were no other complications associated with the procedures and no patient had a pre- or postoperative whistling deformity. Cosmetic outcome was thought to be satisfactory in all cases. CONCLUSIONS: Despite the overall improvement in cosmetic appearance following simple surgical excision of congenital lower lip sinuses, the high rate of mucocele formation and repeat surgery has led to cautious preoperative counseling regarding the risks and benefits of the procedure.

Congenital unilateral lower lip palsy and eventration of diaphragm.

Congenital unilateral lower lip palsy is a rare but well-known limited variation of congenital unilateral facial palsy. We report a three-month-old boy with diaphragmatic eventration and isolated lower lip palsy, a combination that to our knowledge, has not been described before. Probable causes of this combination of multiple congenital malformations, in this case, could be due to nonrandom and heterogeneous mutations. The diaphragmatic eventration was treated successfully.