Standardization for the reporting of nasopharyngoscopy and multiview videofluoroscopy: a report from an International Working Group.

A multidisciplinary International Working Group of scientists was assembled to address the question of standardizing reporting techniques for multiview videofluoroscopy and nasopharyngoscopy, the generally accepted standards for direct observation of the velopharyngeal valve. This report is a first attempt to propose standards while seeking feedback from the readership in order to further develop a common methodology.

Congenital absence of the nasal columella.

Three cases of congenital absence of the nasal columella are presented: one male and twin females. The male also had bilateral congenital cataracts. The nose and the septum were otherwise well developed and appeared normal. The missing segment involved the medial crura of the alar cartilages with their tegumental mantle. There was no deformity of the upper lip or alveolus. Two procedures for reconstruction of the columella that were used in these cases are described in detail. The embryonic pathogenesis of this deformity is suggested.

Sphenoethmoidal cephalocele with cleft palate: transpalatal versus transcranial repair. Report of two cases.

Two cases of sphenoethmoidal encephalocele with cleft palate are reported in detail. The encephaloceles had prolapsed into the nose and nasopharynx, and protruded into the mouth. Previous examples of this entity are reviewed. Such patients have a typical facial appearance: hypertelorism, median cleft lip, and bifid nose of varied severity. Ocular findings include coloboma of the optic nerve or anophthalmia. The prolapsed cerebral tissue varies from nonfunctional glia elements to vital structures of the hypothalamus-pituitary area. Agenesis of the corpus callosum has been reported consistently. Transcranial access to the bone defect, particularly its posterior portion, is difficult because of distorted cerebral anatomy and abnormal vasculature. The fragility of the prolapsed cerebral tissue makes it difficult to preserve the cerebral tissue intact and to reposition it into the cranium. When there is a cleft palate, it is possible to repair the encephalocele extracranially through a transoral, transpalatal approach, preserving and repositioning the content of the sac. Dural closure and obliteration of the bone defects are other essential steps of the operation. Both patients reported here were successfully operated on by the transoral, transpalatal route, at the age of 3 months and 4 years, respectively. They were followed for several years and had comprehensive endocrinological work-ups. One patient exhibited some pituitary deficiency requiring substitution therapy.

The role of lateral pharyngeal wall movement in pharyngeal flap surgery.

A total of 202 patients with pharyngeal flaps were assessed with nasopharyngoscopy and multiview fluoroscopy to determine the role of lateral pharyngeal wall movement postoperatively. Variations in the construction of flaps resulted in three categories: namely, a long narrow flap with a high insertion, a short broad flap with a low insertion, and an intermediate-size flap that is inserted in a position somewhere between the first two. It was found that in all cases where there was no evidence of velopharyngeal insufficiency, the sole determiner of velopharyngeal closure was the medical excursion of the lateral pharyngeal walls to the sides of the flap. In flap failures, the causes for velopharyngeal insufficiency were inappropriate degree, level, and symmetry of the lateral pharyngeal wall motion. The success of pharyngeal flap surgery depends largely on the preoperative assessment of the velopharyngeal mechanism and the choice of a type of pharyngeal flap that will best assist closure of the velopharyngeal port during speech.

Velopharyngeal insufficiency due to hypoplasia of the musculus uvulae and occult submucous cleft palate.

With the help of nasopharyngoscopy, it was possible to delineate specific morphologic changes in the palates of patients with velopharyngeal insufficiency, without an overt cleft and without the triad of symptoms of submucous cleft palate, visible through the oral cavity. Such malformations are part of the broad spectrum of the faulty midline mesodermal fusion of the palate. This anomaly is aptly called occult submucous cleft palate, because it can only be detected by viewing the functioning palate from the nasal surface. The musculus uvulae is either absent or deficient and is frequently associated with some degree of muscular diastasis that does not involve the oral surface. Like the cleft of the secondary palate, the submucous cleft palate often occurs as part of a generalized syndrome of multiple malformations.

Localized cerebritis following an esthetic rhinoplasty.

We report a case of Wernicke's sensory aphasia, caused by a localized cerebritis of the left temporal lobe. The condition developed in the second week after an esthetic rhinoplasty. There was an associated small abscess in the right inner canthal area.

A comprehensive study of pharyngeal flap surgery: tailor made flaps.

Three methods of pharyngeal flap surgery were analyzed with multiview videofluoroscopy and nasopharyngoscopy at least six months post-operatively. It was found that, by varying the type of insertion of the flap into the palate, post-operative flap width could be "tailored" to the size of the gap in the velopharyngeal sphincter. The value of varying flap width according to gap size was assessed by analyzing speech results in 60 patients specifically assigned for narrow, moderate, or wide flaps. Results show a marked improvement in the effectiveness of pharyngeal flap surgery when operations are prescribed according to the degree of lateral pharyngeal wall motion seen pre-operatively.

A new syndrome involving cleft palate, cardiac anomalies, typical facies, and learning disabilities: velo-cardio-facial syndrome.

This report describes a pattern of similarities among 12 patients which are felt to represent a newly recognized congenital malformation syndrome. The symptoms shown most consistently by the 12 patients were overt or submuscous clefts of the secondary palate, ventricular septal defects, typical facies, and learning disabilities. Other symptoms were noted with varying frequency. The occurrence of velopharyngeal insufficiency in all twelve patients reflected poor motion in the lateral pharyngeal walls, thus necessitating specific forms of treatment. Treatment was often dependent on the extent of cardiac lesions.

The lateral transhelical approach for correction of deformities of the external ear.

Additional experience with the chondrocutaneous flap in the repair of auricular defects showed that (i) the entire medial surface of the auricle can be easily and rapidly exposed through this approach, and (ii) the transhelical scar on the lateral surface of the ear remains inconspicuous. The skin of the lateral surface of the ear is similar to that of the eyelids and forms flat, smooth scars which do not hypertrophy. Furthermore, these scars are hidden under the curl of the helix. Through this approach, correction of the protrusion can be accomplished by any method or combination of methods which call for manipulation on the medial surface of the auricle. The scar is remote from the site of manipulation of the cartilage. An additional advantage of this procedure is that it can be combined easily with a small reduction in the size of the scapha.

Spheno-pharyngeal meningocele and cleft palate. Case report with 12-year follow-up.

A rare congenital anomaly consisting of a spheno-pharyngeal meningocele and cleft palate is presented. The repair of the meningocele was accomplished through an intra-oral transpalatal approach. A primary pharyngeal flap was used to accomplish the repair of the cleft palate. Fortunately, there were no important anomalies of the central nervous system. Twelve years later, the patient functions normally and speaks well. The only remaining symptom of midline craniofacial dysmorphia is a mild hypertelorism.

Injuries of the great auricular nerve in rhytidectomy.

The great auricular nerve is most frequently injured in the course of rhytidectomy. Frequency of such injuries is many times higher than that of all other nerves combined. Estimate of such frequency cannot be obtained because many surgeons pay little attention to this complication. Patients often accept the discomfort of anesthesia of the external ear as a normal sequel of the operation, although an occasional neurotic patient may complain of the sensation of having his ear cut off. If the proximal end of the cut nerve becomes attached to the skin flap, neuroma will cause a trigger point on the lateral part of the neck. This may lead to a bizarre complaint of migraine-like pain on the side of the face. Two patients with neuroma of the great auricular nerve were seen, 1 and 2 years, respectively, after rhytidectomy. Diagnosis was confirmed by an operation. Repair of this nerve, either at the time of the operation or several years later, has a very favorable prognosis.The anatomy of this nerve and the precautions necessary to avoid injury during rhytidectomy will be discussed.

Speech results after Millard island flap repair in cleft palate and other velopharyngeal insufficiencies.

Twenty-four patients were evaluated for voice quality after primary palatal repair by the Millard island flap procedure. In patients with overt cleft palate, acceptable speech was obtained in 71 percent. In patients with velopharyngeal insufficiency without an overt cleft, the success rate was 60 percent. Although we consider this method reliable and useful, we have no reason to believe that it offers substantial advantages over other established procedures. We suggest that the main reason for our failures to achieve velopharyngeal competence and acceptable voice quality after a repair is the inherent hypoplasia of palatal musculature.