The crumpled-ear deformity.

The common characteristics of the crumpled-ear deformity, the steps for surgical correction, and the authors' experience are described in this article. Commonly, the ear has a folded-over appearance; a normal length and width when unfurled; and wrinkling of the skin and cartilage of the helical rim, scapha, antihelix, and concha. Principles of correction of this deformity include (1) superficial scoring of the concave segments of the cartilage to open the wrinkled segments, (2) creating the antihelical fold by a mattress-suture technique, (3) repositioning of the helical rim, and (4) repositioning of the prominent ear lobe, if present. A total of 12 crumpled ears were evaluated in six patients, three of whom underwent surgery during their teenage years. No complications have been observed. A new classification of an ear deformity has been proposed based on morphology that to the authors' knowledge has not been described in the literature. Identification of the common features of the deformity allows for precise correction using already well-defined techniques.

Management of secondary turricephaly in craniofacial surgery.

In children with syndromic craniofacial disorders, such as Crouzon and Apert syndromes, who are managed surgically, a difficult problem that can occur is secondary turricephaly. One of the more widely accepted theories as to why this deformity occurs is that a lack of skull base growth results from fusion of the basal and facial sutures. Despite initial adequate forehead and orbital bandeau advancement, many of these patients require subsequent procedures, which do not always correct the characteristics deformity. We have identified a subset of 11 syndromic children who developed this characteristic deformity of turricephaly after primary reconstruction, 6 of whom required either secondary or tertiary procedures. Only 5 patients had a good outcome with a mean follow up of 4.5 years (range 1-8 years). Our surgical methods, and our rationale for the timing of surgery are discussed, and the literature on the management of this problem is reviewed.

The use of magnetic resonance angiography prior to pharyngeal flap surgery in patients with velocardiofacial syndrome.

Twenty consecutive patients with velocardiofacial syndrome underwent magnetic resonance angiography (MRA) to determine if abnormalities of the neck arteries would contraindicate pharyngeal flap surgery. All 20 patients were found to have anomalies to the carotid arteries, vertebral arteries, medially placed internal carotids, low carotid bifurcations, and tortuous or kinked internal carotids. The internal carotids were found to be almost directly under the mucous membrane of the pharynx in two patients. In these two patients, the arteries were close to the pharyngeal midline at the base of the first cervical vertebra and might easily be severed during the raising of a pharyngeal flap. Hypoplastic vertebral arteries also were found. One patient had an extra neck vessel. The anomalies of the internal carotids did not have a strong correlation with endoscopically observed pulsations in the position affected the location of the internal carotids did not have a strong posterior pharyngeal wall. It also was found that head position affected the location of the internal carotid arteries when they were located close to the pharyngeal mucous membrane. The information provided in the MRA studies allowed assessment of the arterial anomalies in relation to the flap donor site so that the patients in the sample who underwent pharyngeal flap surgery using a short superiorly based flap had no major bleeding complications.

Free fibula flap mandible reconstruction for oral obstruction secondary to giant fibrous dysplasia.

Fibrous dysplasia is a disorder of bone that may be associated with endocrinopathies and skin pigmentation. The pathologic, proliferative expansion and distortion of the skeleton is of unknown etiology. Craniofacial involvement that includes the mandible can exhibit gigantic disproportions and dysfunction. Treatment has evolved to include more aggressive strategies of resection and sophisticated reconstructive techniques. The reported case is noteworthy for the unrelenting growth of craniofacial fibrous dysplasia in an adult female with endocrinopathies, progressing to oral obstruction that required urgent treatment utilizing immediate free bone-flap reconstruction. The free fibula flap was employed to restore mandibular continuity after palliative subtotal mandibulectomy. Bony healing to dysplastic tissue occurred in the remaining mandibular segment. This case illustrates that fibrous dysplasia has the capacity for virulent regrowth subsequent to conservative resection. Defects following radical surgery for giant fibrous dysplasia of the mandible can be reconstructed with a microsurgical bone-flap technique.

Treatment of asymmetric velopharyngeal insufficiency with skewed pharyngeal flap.

Twenty-two patients, with hypernasal speech and asymmetric velopharyngeal insufficiency (VPI) identified preoperatively by multi-view video-fluoroscopy and nasopharyngoscopy, were managed with superiorly based pharyngeal flaps skewed to the side with reduced lateral pharyngeal wall movement. Patient age ranged from 5 to 58 years. The etiology of the VPI included cleft palate with or without cleft lip, neurogenic VPI, velocardiofacial syndrome, tumor resection or iatrogenic causes, submucous cleft palate, neurofibromatosis, and hemifacial microsomia. Follow-up, at 1 year and thereafter, showed resolution of VPI in all but two patients. An auxiliary flap to augment the primary flap was added on the side of diminished lateral pharyngeal wall motion which corrected the residual VPI. Three patients developed hyponasality. One was a child whose symptoms improved with time and growth. Two were adults, but the hyponasal resonance was mild and required no further intervention. The advantage of skewing flaps is that at least one port functions adequately for ease in respiration and for drainage of secretions, thus reducing the risk of nasal obstruction. One open port also allows access for nasoendotracheal intubation should anesthetic be required for future operations.

Asterion region synostosis.

Posterior plagiocephaly historically has been associated with synostosis of the lambdoid suture. The incidence, diagnosis, and modes of treatment for stenosis of the lambdoid suture remain controversial. Commonly, the lambdoid suture is found to be open both on radiographic examination and at the time of surgery. The study reports on nine patients who presented with unilateral posterior plagiocephaly and who were found to have open lambdoid sutures, but a stenosed region of the asterion. The area of involvement included the distal-most lambdoid suture, the parietomastoid, occipitomastoid, and proximal squamosal sutures. Positional molding or torticollis was ruled out in all patients. All the patients showed progressive involvement of the skull base, including anterior shifts of the ipsilateral ear, compensatory ipsilateral frontal bossing and malar protrusion. Stenosis of the asterion was diagnosed with three-dimensional computed tomography scans, corroborated at the time of surgery and confirmed histologically. Surgical correction involved resection of the affected asterion and reconstruction using a bandeau-technique, barrel staves of the occipital bone and bone graft transposition. This approach provided excellent esthetic results in all patients.

Monobloc correction of external nasal deviations.

Nine patients (7 men, 2 women) with external nasal deviation underwent corrective procedures using a monobloc nasal osteotomy technique. The deformities ranged from mild to severe. Eight patients had post-traumatic deviations, whereas 1 had a unilateral cleft nasal deformity. For this monobloc technique, osteotomies were performed at unequal levels to correct the height difference, no periosteal undermining was performed, and septal dissection was undertaken only after monobloc repositioning. There was no need for grafts or microplate fixation. Minimum follow-up was 8 months. All patients had improvement in their external deviation, 1 patient was mildly undercorrected, and only 1 patient (cleft nasal) required a radical submucosal resection.

Pharyngeal flap revisions: flap elevation from a scarred posterior pharynx.

Twenty-one consecutive patients who had earlier superiorly based pharyngeal flap surgery and persistent velopharyngeal insufficiency were seen between 1976 and 1991. Patients were divided into two treatment groups, depending on the results of videofluoroscopic and nasopharyngoscopic assessment. The first group consisted of 18 patients who had bilateral port insufficiency and required a complete reconstruction of a new superiorly based pharyngeal flap that was elevated from a scarred posterior pharyngeal wall. After an average follow-up of 6.2 years, 15 patients had normal resonance, 2 patients had improvement but continued hypernasality, and 1 patient was hyponasal. The second group consisted of 3 patients who had "patch" flaps to a unilateral port insufficiency. Postoperatively, all 3 of those patients had normal resonance. Indications for the decision to "redo" or patch flaps are described.

Achondroplasia: pre- and postsurgical considerations for midface advancement.

Achondroplasia, the most common type of dwarfism, involves several physical characteristics that can cause pre- and intraoperative respiratory problems. The characteristics include rib-cage deformities, midface hypoplasia, choanal stenosis, muscular hypotonia, foramen magnum compression, and cervical abnormalities. Specific pre- and postoperative strategies for dealing with achondroplastic dwarfs are discussed.

Distraction of the frontal bone outside the cranial plane: a rabbit model.

Distraction of the frontal bone outside the cranial plane using the Ilizarov principle was performed in 17 22-week-old New Zealand white rabbits. Five rabbits had frontal bone osteotomy only and were in the control group; 5 rabbits were placed in the sham control group and had frontal bone osteotomy plus application of a customized headgear appliance; and 7 rabbits were placed in the experimental group, which underwent frontal bone osteotomy application of the headgear and distraction. The frontal bone was elevated 1 mm every other day for a period of 8 weeks, and the animals were then killed. Cephalometry was performed both preoperatively and at the end of the 8-week period. Histological examination of the skulls was also performed. The experimental group showed a significantly elevated frontal bone compared to the sham control group (p < 0.05). Callous bone filled the distracted segment, which united the frontal bone with the cranial plane. Therefore, frontal bone advancement by distraction osteogenesis is possible using this rabbit model.

Refinements of the tongue flap for closure of difficult palatal fistulas.

The posteriorly based tongue flap can be very useful to close difficult palatal fistulas, especially because the palatal sling prevents dehiscence of the tongue flap. However, special techniques may need to be employed with very large palatal fistulas or severely scarred palates. This technique has been used successfully in 5 patients. A detailed case report is presented, for which refinements of the tongue flap technique was required.

Craniofacial, limb, and abdominal anomalies in a distinct syndrome: relation to the spectrum of Pfeiffer syndrome type 3.

Presented are 2 patients with abnormal craniofacial region, limbs, and abdomen, features that may be consistent with Pfeiffer syndrome, type 3. Both patients had bicoronal and bisphenoidal synostosis, extreme exophthalmic midface hypoplasia, and hydrocephalus. The limbs had a fixed flexion deformity of the elbows with broad thumbs which were radiopalmarly deviated; the toes were broad with a varus deformity and syndactyly toes 2-5. Both patients developed bowel obstruction secondary to midgut malrotation, and one of the patients had prune belly syndrome. Review of the literature disclosed an additional patient who, in retrospect, had Pfeiffer syndrome type 3 and midgut malrotation. These patients suggest that intestinal malrotation with or without prune belly syndrome may be a common component of this entity.

The frontal tiara.

A technique for the construction of a frontal bar and a forehead plate as a single unit is described. This technique could be used in any procedure in which a frontal bandeau is advanced and the forehead needs to be remodeled. By using microscrew fixation, the bulkiness of miniplates and wires is eliminated.

Solitary calvarial plasmacytoma.

One patient with a solitary plasmacytoma of the frontal bone was treated with complete surgical resection. To reconstruct the large calvarial defect, a split-thickness calvarial graft was harvested from the parietal area and secured in place with miniplates. Solitary plasmacytomas of the calvaria are not common. Clinical presentation, diagnostic criteria, therapeutic approaches, and prognosis are discussed.

Glossopexy for upper airway obstruction in Robin sequence.

A modified glossopexy is described with results of the operation in 24 patients who were specifically selected based on nasopharyngoscopic examination of the upper airway. Only patients with documented glossoptosis on endoscopy were selected for glossopexy. The procedure is designed to use two points of attachment for the tongue, one at the mandibular alveolus and the other at the lower lip. The genioglossus is also released to lengthen the tongue. All patients had resolution of their upper airway obstruction. There was only one partial dehiscence. The glossopexies were all released at the time of palate repair, usually before the first birthday.

Pharyngeal flap surgery: postoperative complications.

Using a protocol specifically designed to decrease the risk factors for postoperative morbidity and upper airway obstruction, we have essentially eliminated major complications after pharyngeal flap surgery while maintaining excellent speech results. The protocol includes inserting a short pharyngeal flap into a tissue "sandwich" and keeping an NP tube in place for 48 hours postoperatively. The complications reduced by use of this protocol include apnea and other upper respiratory complications, as well as bleeding. In addition, postoperative discomfort is decreased with the current protocol. Using this approach, pharyngeal flap surgery is highly effective in improving speech and is associated with low morbidity.

Pharyngeal flap surgery in adults.

The elimination of hypernasal speech in patients with cleft palate following pharyngeal flap surgery in childhood is well established. However, pharyngeal flaps in adults have been considered to yield more modest results. This study reports on 20 adult patients with cleft palate-related hypernasality who underwent pharyngeal flap surgery. Normal nasal resonance was achieved in 15 cases, hyponasality occurred in 3 cases, and hypernasality persisted in 2 cases. However, speech intelligibility was not always dramatically improved. Indications and outcome were found to be highly dependent on preoperative articulation.

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.

The tongue flap: placement and fixation for closure of postpalatoplasty fistulae.

A tongue flap is frequently used for closure of fistulae following cleft palate repair. Early tongue flap dehiscence is a troublesome complication. While the tongue flap is a very effective means of functionally obliterating the transpalatal oronasal opening, the final appearance of the repair leaves much to be desired. More often than not, the tongue tissue appears bulky and unnatural in the roof of the mouth. A method is described that overcomes the problem of flap detachment during the early postoperative period by suspending and supporting the tongue pedicle with a palatal sling. On peroral view, the repaired area has a better appearance when the tongue flap lines the nasal side rather than the oral side as in conventional procedures.

Ear reduction with or without setback otoplasty.

Ear reduction has been performed occasionally for aesthetic considerations. This series is comprised of 8 patients representing 7 bilateral reductions and 1 unilateral reduction for a total of 15 ears. Historically, surgery for macrotia consisted of resections of full-thickness wedges from the periphery of the ear. The surgical defect was repaired by directly approximating the wound edges. Additional removal of triangular or crescent-shaped segments from adjacent sides of the wound prevented the cupping of the reconstructed ear. Secondary deformities were not uncommon consequences. The most distressing were cruciform scars that defaced the lateral surface of the ear. These were frequently exacerbated by the uneven coaptation of the underlying cartilages. The current technique places the incisions at strategic locations where the scars that form are less conspicuous. The initial steps are essentially identical with the lateral transhelical approach to otoplasty for protruding ears. In some patients, both procedures have been combined during the same operative session.