Acute obstructive sleep apnea as a complication of sphincter pharyngoplasty.

This report describes postoperative airway compromise following sphincter pharyngoplasty (SP) for treatment of post-palatoplasty velopharyngeal dysfunction. A retrospective review of 58 SPs performed for post-palatoplasty velopharyngeal dysfunction, on 30 male, and 28 female patients, over a 5-year study period was undertaken at a tertiary referral academic institution (Washington University School of Medicine), at the St. Louis Children's Hospital, Cleft Palate and Craniofacial Deformities Institute. Eight patients were identified who had the following inclusion criteria: overt perioperative and/or postoperative airway dysfunction, identifiable syndromes, or microretrognathia. Items reviewed were patient demographic factors, associated medical problems, genetics evaluations, nasendoscopic characteristics of velopharyngeal closure, anesthetic evaluation of the patients, and the incidence and severity of perioperative complications. Particular attention was paid to factors contributing to the airway obstruction. Of the eight subjects with perioperative and/or postoperative upper airway dysfunction following SP, five patients had Pierre Robin sequence/micrognathia, while three patients had a history of perinatal respiratory and/or feeding difficulties without micrognathia or an identified genetic disorder. All but two episodes of airway dysfunction resolved within 3 days postoperatively. These patients were discharged home with apnea monitors; both were readmitted with recurrent airway dysfunction. Continuous positive airway pressure (CPAP) was utilized successfully in all instances, and no patients required take-down of the SP to relieve airway dysfunction. CPAP is an effective, noninvasive treatment strategy for management of iatrogenically induced apnea following SP, without sacrificing the surgical benefit of improved speech intelligibility.

Long-term studies of metopic synostosis: frequency of cognitive impairment and behavioral disturbances.

Although the occurrence of cognitive impairment and behavioral disturbances in patients with metopic synostosis has been described, the incidence of this dysfunction has not been established. The records of 36 consecutive children with metopic synostosis followed at one craniofacial center from 1978 to 1993 were reviewed and parental questionnaires were completed to establish the frequency of mental retardation, learning disabilities, and behavioral problems associated with this synostosis. Documentation of syndromes, abnormal karyotype, and central nervous system anomalies also was done. The study group consisted of 27 males and 9 females. The average age at most recent follow-up was 7 years and 1 month (range 6 months to 22 years). Two patients had chromosomal abnormalities (9p syndrome and trisomy 21). On the basis of CT and MRI scans, intracranial anomalies were identified for only one patient having an absent corpus callosum. Thirty-two of the study patients had adequate information for longitudinal assessment. Twenty patients have normal development without apparent disability. Of these, those of school age are at appropriate grade level. Eight patients have mild to moderate learning disabilities or behavioral problems, including attention deficit/hyperactivity disorder and impaired language development. Four patients have significant mental impairment. Impaired cognitive development was not limited to children with abnormal karyotype or central nervous system anomaly. Cognitive and behavioral abnormalities occur in at least a third of patients with metopic synostosis. The, at times, subtle nature of these abnormalities mandates longitudinal developmental and neurologic evaluation for infants with metopic synostosis.

Do palatal lift prostheses stimulate velopharyngeal neuromuscular activity?

The purpose of this investigation was to evaluate the ability of palatal lift prostheses to stimulate the neuromuscular activity of the velopharynx. Nasendoscopic evaluations were audio-videotaped preprosthetic and postprosthetic management for 25 patients who underwent placement of a palatal lift prosthesis for velopharyngeal dysfunction (VPD). These audio-videotapes were presented in blinded fashion and random order to three speech pathologists experienced in assessment of patients with VPD. They rated the tapes on the following parameters: VP gap size, closure pattern, orifice estimate, direction and magnitude of change, and qualitative descriptions of the adequacy of VP closure during speech. VP closure for speech was unchanged in 69% of patients and the number of patients rated as improved or deteriorated was nearly identical at about 15%. Postintervention gap shape remained unchanged in 70% of patients. The extent of VP orifice closure during speech remained unchanged in 57% of patients. Articulations that could impair VP function improved in 30% of patients, deteriorating in only 4%. Results of this study neither support the concept that palatal lift prostheses alter the neuromuscular patterning of the velopharynx, nor provide objective documentation of the feasibility of prosthetic reduction for weaning.

Revision of the failed sphincter pharyngoplasty: an outcome assessment.

This paper reports results of surgical management of failed sphincter pharyngoplasties that were performed for velopharyngeal dysfunction. Revisional surgery consisted of tightening of the sphincter pharyngoplasty port or reinsertion of sphincter pharyngoplasty flaps following dehiscence. We critique the anatomic abnormalities associated with unacceptable vocal resonance and nasal air escape following sphincter pharyngoplasty and analyze the effect of sphincter pharyngoplasty revision on ultimate speech outcome. The results of initial sphincter pharyngoplasty surgery were evaluated in 46 patients with velopharyngeal dysfunction. Nine (20 percent) of these patients were considered surgical failures because of persistent hypernasality and/or nasal turbulence on perceptual speech evaluation at least 3 months postoperatively. These patients underwent sphincter pharyngoplasty revision and form the basis of this report. All patients who failed sphincter pharyngoplasty initially underwent both preoperative and postoperative perceptual speech evaluations, lateral phonation radiographic studies with still reference views, and flexible nasendoscopic studies. Evaluations of upper airway status were conducted by the same experienced otolaryngologist. Following sphincter pharyngoplasty revision, 7 of 9 (78 percent) patients demonstrated resolution of velopharyngeal dysfunction, and to some degree, all patients managed with revision became hyponasal. The primary cause of failure was partial or complete flap dehiscence; a secondary cause was hypotonicity of the velopharyngeal mechanism. Failure was not correlated with the level of insertion of the pharyngoplasty flaps with respect to the point of attempted velopharyngeal contact. Sphincter pharyngoplasty is an effective means of management for velopharyngeal dysfunction in many patients. The objective of removing the stigmata of velopharyngeal dysfunction without causing upper airway obstruction may not be realistic in some patients with microretrognathia (i.e., Pierre Robin sequence), in whom anatomic constraints predispose to flap dehiscence. Problems with surgical technique contributing to failure appear to be related to experience of the surgeon, and improvement in outcome can be anticipated as the "learning curve" is overcome.

Management of the hypodynamic velopharynx.

Velopharyngeal dysfunction (VPD) resulting from an adynamic or hypodynamic velopharynx is an unusual pathology that poses vexing management problems for the Cleft Palate team. Correction of VPD has the potential for airway compromise. Endoscopically, this pathology is recognized by a large velopharyngeal (VP) gap size, which demonstrates little or no dynamic activity of the posterior or lateral pharyngeal walls nor of the velum in response to speech tasks or connected speech. Because of a paucity of literature defining the entity, a retrospective review of 175 patients who were treated for VPD at our center was undertaken. Analysis of management failures revealed an unexpected concentration of patients with hypodynamic or paretic VP mechanisms as documented by nasendoscopic assessments. A subpopulation of 41 (23%) patients with this characteristic was studied to define the patients at risk, to determine etiologic factors, and to critique intervention outcome among various surgical and nonsurgical managements. Results showed that the phenomenon of VP hypodynamism occurred more frequently in patients with submucous cleft palate (p = .014) and with VPD in association with malformation syndromes (p = .009) than in patients in other diagnostic categories. Conversely, VPD not associated with clefting occurred with greater frequency in the nonhypodynamic group than in the hypodynamic group (p = .002). Composite (surgical and prosthetic) primary management failure occurred in 42%. Between one and three procedures were necessary to achieve an acceptable speech result. We present a management algorithm and provide data regarding realistic expectations for various treatment outcomes in patients with this complex disorder, which have not, to date, been previously described.

Practical application of flexible fiberoptic nasopharyngoscopy for evaluating velopharyngeal function.

Flexible fiberoptic nasopharyngoscopy increasingly is recognized as a valuable tool for assessing velopharyngeal function. There are, however, few reports in the literature describing a method for routine use of nasoendoscopy, the outcome of such evaluations, the criteria for referral, and a mechanism for communicating findings. Results from a 2-year clinical nasoendoscopy data base are presented. Ninety-eight patients were referred for evaluation of velopharyngeal function during speech. Flexible fiberoptic nasopharyngoscopic evaluations were conducted in 85 patients. Patients ranged in age from 3 years to adult. Eighty-nine percent of the evaluations were completed successfully on a first attempt. A standard format has been designed to communicate the results of nasoendoscopic examinations to primary and tertiary care providers. Nasoendoscopy has become an integral part of our cleft team care and is used routinely for decisions regarding palatal management, fabrication and fitting of palatal prostheses, and treatment of resonance disorders following pharyngeal flap surgery.