Fluoroscopic and CT fistulography of the first branchial cleft.

We present an unusual case of a complete first branchial cleft fistula communicating between the external auditory canal and the skin near the angle of the mandible. CT and fluoroscopic fistulography were used to establish the presence and course of the tract and to assist in surgical planning. The embryology and classification of first branchial cleft anomalies are discussed, with emphasis on the impact of imaging.

Cheek fistula from the ectopic salivary gland: a variant of the oculo-auriculo-vertebral spectrum.

OBJECTIVES/HYPOTHESIS: This study aimed to investigate the diagnosis and management of a distinct developmental deformity syndrome characterized by congenital cheek fistula, ectopic accessory parotid gland, and preauricular appendage. STUDY DESIGN: Retrospective study. METHODS: We analyzed the medical records, radiologic and histopathologic findings, and follow-up data for seven patients (four males) with a congenital cheek salivary fistula. Computed tomography, fistulography, and sialography had been performed for diagnosis. Surgical treatment effect was evaluated. RESULTS: The mean age of the patients was 8.1 years (range, 2-16 years). The distinctive clinical feature was a congenital skin orifice lateral to the commissure with saliva discharge during eating. The cheek fistulae were accompanied by ipsilateral preauricular appendages in all seven patients. The skin orifice connected to an ectopic gland anterior to the masseter and inferior to Stensen's duct. Parotid sialography demonstrated an intact Stensen's duct in all cases. Hypoplasia of the ipsilateral mandible could be observed in five cases. Excision of the ectopic gland, skin orifice, and fistula was performed in five cases resulting in optimal treatment outcomes with no recurrent or adverse events. CONCLUSIONS: A congenital saliva-discharging fistula with an ectopic accessory parotid gland, ipsilateral preauricular appendage, and mandibular hypoplasia constitutes a rare developmental syndrome. Surgical excision can effectively treat congenital cheek salivary fistula. LEVEL OF EVIDENCE: 4.

Surgical excision of a congenital lateral fistula of the upper lip. An intra-oral approach.

A new technique for removal of a congenital lateral upper lip fistula is described, using an intra-oral approach. Previous reports of removal of such a congenital malformation have used an extra-oral approach. The technique described allows for excellent visualization of the fistula without cutaneous lip incision. Using this approach, good aesthetic results and complete removal of the fistula and associated mucous glands can be obtained.

Congenital fistula of the palate.

Four cases of congenital fistula of the palate are presented. All four patients had a fistula which was situated in the vault with a bifid uvula, submucous separation of the palatal muscles, deformities of the palatal plates and unilateral cleft lip. Velopharyngeal incompetence appeared in primarily treated children. The aetiology and surgical treatment of the congenital defect are discussed.

Quick hardening polymers in the surgical treatment of congenital neck fistulae.

A method of filling up congenital fistulae of the neck by quick hardening polymers, immediately before the operation, is presented. Two cases are reported.

Oronasal fistulas in cleft palate patients and their influence on speech.

The size, site, and influence on speech of oronasal fistulas were studied in 12 patients with unilateral and 32 with bilateral cleft lip and palate. There were more and larger fistulas in the bilateral group. The series was divided into: those with fistulas that affected speech (group A, n = 18) and those with fistulas that did not affect their speech (group B, n = 26). Group A had significantly larger fistulas than group B, but there were no differences in the sites of the fistulas, either between the bilateral and unilateral groups or between groups A and B. Most fistulas were located in the region of the incisive foramen or in the hard palate. Judgements by listeners and analyses by the NORAM instrument were made of the speech of 12 of the patients in group A before and after temporary covering of the fistulas. Significant differences in hypernasality, according to both listener's judgments and instrumental analyses were found. This finding is further evidence that an oronasal fistula can influence and contribute to velopharyngeal dysfunction.

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.

Branchial anomalies in children.

BACKGROUND: Branchial cleft anomalies are the second most common head and neck congenital lesions seen in children. Amongst the branchial cleft malformations, second cleft lesions account for 95% of the branchial anomalies. This article analyzes all the cases of branchial cleft anomalies operated on at Great Ormond Street Hospital over the past 10 years. METHODS: All children who underwent surgery for branchial cleft sinus or fistula from January 2000 to December 2010 were included in this study. RESULTS: In this series, we had 80 patients (38 female and 42 male). The age at the time of operation varied from 1 year to 14 years. Amongst this group, 15 patients had first branchial cleft anomaly, 62 had second branchial cleft anomaly and 3 had fourth branchial pouch anomaly. All the first cleft cases were operated on by a superficial parotidectomy approach with facial nerve identification. Complete excision was achieved in all these first cleft cases. In this series of first cleft anomalies, we had one complication (temporary marginal mandibular nerve weakness. In the 62 children with second branchial cleft anomalies, 50 were unilateral and 12 were bilateral. In the vast majority, the tract extended through the carotid bifurcation and extended up to pharyngeal constrictor muscles. Majority of these cases were operated on through an elliptical incision around the external opening. Complete excision was achieved in all second cleft cases except one who required a repeat excision. In this subgroup, we had two complications one patient developed a seroma and one had incomplete excision. The three patients with fourth pouch anomaly were treated with endoscopic assisted monopolar diathermy to the sinus opening with good outcome. CONCLUSION: Branchial anomalies are relatively common in children. There are three distinct types, first cleft, second cleft and fourth pouch anomaly. Correct diagnosis is essential to avoid inadequate surgery and multiple procedures. The surgical approach needs to be tailored to the type of anomaly of origin of the anomaly. Complete excision is essential for good outcomes.

Congenital lacrimal sac fistula: intraoperative visualization by polyvinyl siloxane cast.

We report the intraoperative use of polyvinyl siloxane impression material to demonstrate the anatomy of the lacrimal sac, canaliculi, and lacrimal duct in a case of congenital lacrimal sac fistula. A 1-week-old boy was examined for tearing since birth. Examination revealed a left congenital lacrimal sac fistula. After a failed surgery to close the fistula with silicone intubation at 6 months of age, the patient underwent endonasal dacryocystorhinostomy performed at 14 months of age, aided by intraoperative injection of polyvinyl siloxane (trade name Reprosil) to mark and protect the nasolacrimal sac and facilitate endonasal visualization. A polyvinyl siloxane cast demonstrated the anatomy of the accessory canaliculus causing nasolacrimal duct obstruction. Postoperatively, the epiphora resolved and the fistula remained closed. The polyvinyl siloxane cast provides a 3-dimensional "ex vivo " model of the lacrimal sac, upper duct, and canalicular anatomy, and can be used in dacryocystorhinostomy surgery to identify and protect the lacrimal sac.

Simultaneous occurrence of a congenital lateral upper lip sinus and congenital gingival cyst: a case report and discussion of pathogenesis.

The simultaneous occurrence of a congenital lateral sinus of the upper lip and of a congenital gingival cyst in a newborn boy is reported. The sinus extended to a depth of 10 mm and was lined by a parakeratinized squamous epithelium. The gingival cyst appeared as a 1 cm palatal enlargement lingual to the maxillary alveolar ridge and demonstrated a thin epithelial lining with localized plaquelike thickenings and occasional clear cells with vacuolated cytoplasm. There was no familial history of congenital deformities, and no additional abnormalities were noted. These two epithelium-lined structures show a clear topographic and morphologic relationship, and they may share a common embryologic origin. It is suggested that they derive from epithelium remaining subsequent to the union of the maxillary process and medial nasal process mesoderms.

Velopharyngeal inadequacy in the absence of overt cleft palate.

Velopharyngeal inadequacy in the absence of overt cleft palate may be due to any one, of any combination, of the following: intraorally visible stigmata associated with submucous defects (any combination of bifid uvula, muscular diastasis of the soft palate, bony defect of the hard palate); "occult" anatomical defects of the levator palatini or musculus uvulae, detectable only by nasopharyngoscopy or by operative dissection; anatomic disproportion between the size of the nasopharynx and the length of the hard and/or soft palate; mechanical interference with motion of the velopharyngeal system occurring as a result of scarring or contracture, and possibly as a result of interposition of the upper poles of the faucial tonsils between the velum and the posterior pharyngeal wall; a wide variety of neuromotor deficits, either congenital or acquired, causing reduced and/or incoordinated movement of the velopharyngeal musculature; a learning error of unknown origin which results in velopharyngeal inadequacy only on specific phonemes with all other pressure consonants emitted orally. Submucous defects of the secondary palate do not necessarily produce velopharyngeal inadequacy. Thus, our estimates of both the incidence of submucous defects and of the frequency of genes for clefting in any given population are undoubtedly low. Finally, "stress velopharyngeal inadequacy" in wind instrument players has been linked to a variety of anatomic findings and is not necessarily accompanied by velopharyngeal inadequacy in speech. This paper will review the historic aspects of velopharyngeal inadequacy and will discuss and analyze the causes outlined above.

Bilateral congenital lacrimal anlage ducts (lacrimal fistula) in a Patient With the VACTERL association.

PURPOSE: To present a case of bilateral congenital lacrimal anlage ducts in a patient with the VACTERL (vertebral anomalies, anal atresia, cardiac malformations, tracheoesophageal fistula, renal anomalies, and limb anomalies) association. METHODS: Case report. RESULTS: A 19-year-old man presented with intermittent facial swelling. Examination revealed bilateral lacrimal anlage ducts. The patient underwent excision of the ducts with silicone intubation. CONCLUSIONS: We add the VACTERL association to the list of systemic conditions associated with lacrimal anlage ducts.