Role of free-tissue transfer in the treatment of recalcitrant palatal fistulae among patients with cleft palates

Recurrent palatal fistulae present a particularly vexing problem for patients with cleft lips and palates and their surgeons. When primary closure fails, conventional wisdom and the standard of care suggest local flap techniques for defect closure. For the large majority of patients, this approach is successful. There is, however, a small subset of patients who undergo multiple surgical procedures in unsuccessful attempts to close recalcitrant fistulae, particularly at the anterior, densely scarred, hard palate. In this setting, repair calls for the introduction of well-vascularized pliable tissue to close the defect and to avoid hampering further palatal growth. Local muscle flaps and oral axial pattern flaps have been advocated and used successfully. However, those approaches have their own drawbacks, such as multiple surgical interventions, patient compliance, and intraoral scarring. In an effort to avoid the problems associated with local flaps, distant microvascular tissue transfers were investigated. During a 6-year period, six free-tissue transfers were performed as a primary means of treating recalcitrant palatal fistulae. Three dorsalis pedis flaps and three osseous angular scapular flaps were used. The conditions of all patients improved, with five patients achieving complete long-term closure of the palatal defect. This experience indicates that modern microvascular techniques have reached a level of success commensurate with that of other flap techniques; therefore, it is concluded that free-tissue transfer should be considered as a primary means of addressing these difficult cleft problems.     

Predictors of velopharyngeal insufficiency in cleft palate orthognathic surgery

The purpose of this study was to appraise the value of preoperative speech assessments, nasopharyngoscopy, and surgical models as predictors of velopharyngeal deterioration after a Le Fort I maxillary advancement in cleft patients. This retrospective study involved a series of 26 cleft patients (16 unilateral complete and nine bilateral complete cleft lips and palates, and one isolated complete cleft palate) who had Le Fort I maxillary advancements between March 1, 1993, and February 7, 1996. The 13 male patients and 13 female patients ranged in age from 15.3 to 46 years (mean age, 19.5 years). Four of these patients had previously undergone pharyngeal flap surgery. Eleven patients had palatal fistulas and one had a bifid uvula that was repaired at the time of orthognathic surgery. Patients with perceived hypernasal speech preoperatively all had hypernasality after advancement (nine of nine). Velopharyngeal insufficiency was observed in two of the 16 whose resonance preoperatively was within normal limits. Speech assessment, therefore, predicted accurately the postoperative status in 23 of 26 patients. Twelve patients had preoperative nasopharyngoscopy that indicated a high risk for velopharyngeal insufficiency (borderline or inadequate closure). Nine of these patients had postoperative velopharyngeal insufficiency. Two of the 14 patients not judged at risk by nasopharyngoscopy developed velopharyngeal insufficiency. Therefore, 21 of the 26 patients were accurately predicted by nasopharyngoscopy. Scoping detected borderline velopharyngeal insufficiency in one patient who was not detected by speech alone. The combined predictive value of speech and scope identified all but one patient who would develop postoperative velopharyngeal insufficiency. The degree of anteroposterior movement determined from surgical models was not predictive of the outcome. Patients with hypernasal speech preoperatively continue to have hypernasal speech after Le Fort I advancement. Preoperative perceptual speech assessment by specially trained speech-language pathologists is an excellent test for predicting postoperative velopharyngeal insufficiency status. Nasopharyngoscopy is an invasive and resource-dependent test that should be assessed with respect to cost effectiveness. In this series, only one patient's risk was more accurately predicted using nasopharyngoscopy than by speech assessment alone.     

Free forearm flap for closure of difficult oronasal fistulas in cleft palate patients.

There were four patients with palatal clefts who had been operated on many times previously but who still had large oronasal defects due to wound disruption. Moreover, there was considerable scar in the residual palatal tissue, which was contracted in the anteroposterior dimension. These patients were treated with a radial forearm flap transfer. The technical aspects of this reconstruction are emphasized, especially methods to enhance primary healing and to facilitate in setting the flap. Three of the patients were successfully reconstructed with one operation. The fourth had a small area of dehiscence anteriorly that was later closed with advancement of the flap tissue. There were no other complications. With the replacement of healthy tissue, the palate could be pushed further back to achieve better repair of the muscle. This would contribute to better speech function. In every patient, nasal regurgitation was eliminated, and speech quality improved significantly. The radial forearm flap is ideal for intraoral use, providing thin, hairless skin with a long, large-caliber vascular pedicle. It can reconstruct defects in one stage with well-vascularized tissue and minimal dissection of the palate. In a select group of cleft palate patients, this free-tissue transfer should be considered to achieve closure of large oronasal fistulas in patients with dense scar.     

Snail family members and cell survival in physiological and pathological cleft palates

Palate fusion is a complex process that involves the coordination of a series of cellular changes including cell death and epithelial to mesenchymal transition (EMT). Since members of the Snail family of zinc-finger regulators are involved in both triggering of the EMT and cell survival, we decided to study their putative role in palatal fusion. Furthermore, Snail genes are induced by transforming growth factor beta gene (TGF-beta) superfamily members, and TGF-beta(3) null mutant mice (TGF-beta(3)-/-) show a cleft palate phenotype. Here we show that in the wild-type mouse at the time of fusion, Snail is expressed in a few cells of the midline epithelial seam (MES), compatible with a role in triggering of the EMT in a small subpopulation of the MES. We also find an intriguing relationship between the expression of Snail family members and cell survival associated to the cleft palate condition. Indeed, Snail is expressed in the medial edge epithelial (MEE) cells in TGF-beta(3)-/-mouse embryo palates, where it is activated by the aberrant expression of its inducer, TGF-beta(1), in the underlying mesenchyme. In contrast to Snail-deficient wild-type pre-adhesion MEE cells, Snail-expressing TGF-beta(3) mutant MEE cells survive as they do their counterparts in the chick embryo. Interestingly, Slug is the Snail family member expressed in the chick MEE, providing another example of interchange of Snail and Slug expression between avian and mammalian embryos. We propose that in the absence of TGF-beta(3), TGF-beta(1) is upregulated in the mesenchyme, and that in both physiological (avian) and pathological (TGF-beta(3)-/-mammalian) cleft palates, it induces the expression of Snail genes promoting the survival of the MEE cells and permitting their subsequent differentiation into keratinized stratified epithelium.     

Primary hyperparathyroidism: the surgical problems. A study of 1,300 operated patients

In this short presentation the surgical management and then the possible vicissitudes of primary hyperparathyroidism are successively summarized: negative investigations, and the persistence or postoperative recurrence of hyperparathyroidism. Results obtained in a series of 1,300 patients who underwent surgical treatment confirm that a single cervicotomy procedure, without risk for the patient, ensures definitive cure of this disease in 95% of cases.     

Evaluation of velopharyngeal closure by CT scan and endoscopy

Computerized tomography (CT) and endoscopy were used for the objective evaluation of velopharyngeal closure. In 19 patients with cleft palates and 9 normal subjects, CT scans of the velopharynx were made both at rest and during vowel phonation with a scanning time of 3.0 seconds and slicing width of 3 mm. At the same time, endoscopic observations of the velopharynx through the nose were carried out both at rest and during phonation. CT scan during phonation clearly demonstrated the mobility of the velopharynx, i.e., elevation of the soft palate, inward movement of the lateral pharyngeal walls, and protrusion of the posterior pharyngeal wall, in a single picture. Its disadvantage is exposure to x-rays and a rather complicated procedure. However, endoscopy is simple with no exposure to x-rays, but its disadvantage is occasional incomplete visualization because of the dead angle created by the elevated soft palate. Thus the combined use of CT and endoscopy can help to determine a rational choice of treatment for cleft palates.     

Development of articulation before delayed hard-palate closure in children with cleft palate: a cross-sectional study

The development of articulation before surgical closure of the hard palate was compared in 75 preschool children with cleft lip and palate and 40 preschool children born without clefts. The children were aged 2 years to 5 years 11 months. The patients had significantly poorer articulation skills than the controls at each age level. Substitutions were the most frequent error, and they did not decrease with age in the patients. Fistula size and a history of speech therapy were significant factors in the articulation error scores only in 5-year-olds. No advantage in articulation proficiency was found for those who had worn a prosthesis to occlude the hard-palate defect.     

Optimal timing of cleft palate closure

Treatment objectives for the cleft palate patient--normal speech, normal maxillofacial growth, and normal hearing--are closely related. Controversy about the timing of cleft palate surgery is directed at the need for early palatoplasty for improved speech and hearing versus delayed hard palate repair for undisturbed facial growth. This controversy as to the value of early versus delayed closure continues into the present. The authors present an updated argument regarding this controversy along with a comprehensive literature review. They also present a logical algorithm based on the literature and their personal experience.     

A prospective evaluation of the prevalence of submucous cleft palate in patients with isolated cleft lip versus controls.

Although there is an established relationship between cleft lip and overt cleft palate, the relationship between isolated cleft lip and submucous cleft palate has not been investigated. To test the hypothesis that patients with isolated cleft lip have a greater association with submucous cleft palate, a double-armed prospective trial was designed. A study group of 25 consecutive children presenting with an isolated cleft lip, with or without extension through the alveolus but not involving the secondary palate, was compared with a control group of 25 children with no known facial clefts. Eligible patients were examined for the presence of physical criteria associated with classic submucous cleft palate, namely, (1) bifid uvula, (2) absence of the posterior nasal spine, and (3) zona pellucida. Nasoendoscopy was subsequently performed just after induction of general anesthesia, and the findings were correlated with digital palpation of the palatal muscles. Patients who did not satisfy all three physical criteria and in whom nasoendoscopy was distinctly abnormal relative to the control group were classified as having occult submucous cleft palate. Classic submucous cleft palate was found in three study group patients (12 percent), all of whom had flattening or a midline depression of the posterior palate and musculus uvulae on nasoendoscopy and palpable diastasis of the palatal muscles under general anesthesia. An additional six study group patients (24 percent) had similar nasoendoscopic criteria and palpable diastasis of the palatal muscles; they were classified as having occult submucous cleft palate. No submucous cleft palate was identified in the control group. Seventeen patients in the study group had an alveolar cleft with a 53 percent (9 of 17) prevalence of submucous cleft palate. In the present study, classic submucous cleft palate in association with isolated cleft lip was 150 to 600 times the reported prevalence in the general population. All children with an isolated cleft lip should undergo peroral examination and speech/resonance assessment no later than the age of 3 years. Any child with an isolated cleft lip with velopharyngeal inadequacy or before an adenoidectomy should be assessed by flexible nasal endoscopy to avoid missing an occult submucous cleft palate.