Eileen M. Marrinan

Velopharyngeal function in nonsyndromic cleft palate: relevance of surgical technique, age at repair, and cleft type.

OBJECTIVE The goal of this study was to determine the relative importance of surgical technique, age at repair, and cleft type for velopharyngeal function. DESIGN This was a retrospective study of patients operated on by two surgeons using different techniques (von Langenbeck and Veau-Wardill-Kilner [VY]) at Childrens Hospital, Boston, MA. PATIENTS We included 228 patients who were at least 4 years of age at the time of review. Patients with identifiable syndromes, nonsyndromic Robin sequence, central nervous system disorders, communicatively significant hearing loss, and inadequate speech data were excluded. MAIN OUTCOME MEASURE Need for a pharyngeal flap was the measure of outcome. RESULTS Pharyngeal flap was necessary in 14% of von Langenbeck and 15% of VY repaired patients. There was a significant linear association (p = .025) between age at repair and velopharyngeal insufficiency (VPI). Patients with an attached vomer, soft cleft palate (SCP), and unilateral cleft lip/palate (UCLP) had a 10% flap rate, whereas those with an unattached vomer, hard/soft cleft palate (HSCP), and bilateral cleft lip/palate (BCLP) had a 23% flap rate (p = .03). Age at repair was critical for the unattached-vomer group (p = .03) but was not statistically significant for the attached-vomer group (p = .52). CONCLUSIONS Surgical technique was not a significant variable either in aggregate or for the Veau types. Patients in the earliest repair group (8-10 months) were the least likely to require a pharyngeal flap. Early repair was more critical for HSCP and BCLP patients. There was no correlation between velopharyngeal insufficiency and Veau hierarchy. The attached vomer/levator muscle complex may be a more important predictor of surgical success than the anatomic extent of cleft.

Palatoplasty outcomes in nonsyndromic patients with cleft palate: a 29-year assessment of one surgeon's experience.

The primary objective of cleft palate repair is velopharyngeal competence without fistula. The reported incidence of fistula and velopharyngeal insufficiency (VPI) is variable. Our purpose was to assess the senior surgeons 29-year palatoplasty experience with respect to incidence of fistula and VPI. Our hypotheses were that VPI is related to (1) age at palatoplasty, (2) cleft palate type, and (3) VPI and palatal fistula incidence decrease with the surgeons experience. We reviewed the records of all children with cleft palate treated by the senior author between 1976 and 2004. Cleft palate was categorized according to Veau. Palatoplasty was performed on 449 patients, using a 2-flap technique with muscular retropositioning. The mean age at palatoplasty was 11.6 ± 4.9 months (range, 7.0-46.4 months). The incidence of palatal fistula was 2.9%, and velopharyngeal sufficiency was found in 85.1% of patients. We found a significant association between age at palatoplasty and VPI (P = 0.009, odds ratio, 1.06 [95% confidence interval, 1.02-1.10]). Velopharyngeal insufficiency was also associated with the Veau hierarchy (P = 0.001). Incidence of VPI was independent of surgeon experience (P = 0.2). In conclusion, the incidence of palatal fistula was low. Velopharyngeal insufficiency was associated with increasing age at palatoplasty and with the Veau hierarchy.

Submucous Cleft Palate and Velopharyngeal Insufficiency: Comparison of Speech Outcomes Using Three Operative Techniques by One Surgeon

Objective Our purpose was to compare speech outcomes among three primary procedures for symptomatic submucous cleft palate (SMCP): two-flap palatoplasty with muscular retropositioning, double-opposing Z-palatoplasty, or pharyngeal flap. Design Retrospective review. Setting Tertiary hospital. Patients, Participants All children with SMCP treated by the senior author between 1984 and 2008. Interventions One of three primary procedures: two-flap palatoplasty with muscular retropositioning, double-opposing Z-palatoplasty, or pharyngeal flap. Main outcome Measures Speech outcome and need for a secondary operation were analyzed among procedures. Success was defined as normal or borderline competent velopharyngeal function. Failure was defined as persistent borderline insufficiency or velopharyngeal insufficiency with recommendation for a secondary operation. Results We identified 58 patients with SMCP who were treated for velopharyngeal insufficiency. We found significant differences in median age at operation among the procedures (p < .001). Two-flap palatoplasty with muscular retropositioning (n = 24), double-opposing Z-palatoplasty (n = 19), and pharyngeal flap (n = 15) were performed at a median of 2.5, 3.6, and 9.5 years, respectively. There were significant differences in success among procedures (p = .018). Normal or borderline competent function was achieved in 6/20 (30%) patients who underwent two-flap palatoplasty, 10/15 (67%) following double-opposing Z-palatoplasty, and 11/12 (92%) following pharyngeal flap. Among patients treated with palatoplasty, success was independent of age at operation (p = .16). Conclusions Double-opposing Z-palatoplasty is more effective than two-flap palatoplasty with muscular retropositioning. For children older than 4 years, primary pharyngeal flap is also highly successful but equally so as a secondary operation and can be reserved, if necessary, following double-opposing Z-palatoplasty.

Predictors of velopharyngeal insufficiency after Le Fort I maxillary advancement in patients with cleft palate.

PURPOSE Approximately 25% to 40% of patients with cleft lip/palate develop maxillary retrusion that requires Le Fort I osteotomy. Maxillary advancement brings the soft palate forward, and this may cause velopharyngeal insufficiency (VPI). The goal of this study was to identify predictors that place patients with repaired cleft palate at risk of developing VPI after Le Fort I advancement. MATERIALS AND METHODS This was a retrospective study of nonsyndromic patients with cleft lip/palate who had a Le Fort I osteotomy between 2000 and 2008. Charts were reviewed and data were collected on patient characteristics, preoperative speech assessments, and nasopharyngoscopic reports. Pre- and postoperative cephalometric radiographs were used to measure maxillary advancement and to assess the structure of the velopharynx. Simple logistic regression analysis examined the association between each predictive variable and postoperative VPI, as indicated by need for pharyngeal flap. Predictors with P ≤ .10 were included in the multivariate regression model. In both the univariate and the multivariate analyses, P ≤ .05 was considered statistically significant. RESULTS Univariate analysis showed a significant association between preoperative soft palatal length and need for a pharyngeal flap (P = .005). By multivariate analysis, both preoperative soft palatal length and postoperative pharyngeal depth were associated with need for pharyngeal flap (P = .003 and P = .030). CONCLUSION This study shows that a short soft palate is associated with VPI after Le Fort I osteotomy. Assessment of palatal length and pharyngeal depth on cephalometric radiographs is helpful in predicting postoperative VPI and need for a pharyngeal flap in patients with cleft palate after maxillary advancement.

Velopharyngeal Insufficiency: Diagnosis and Management

Purpose of reviewJournal articles relevant to the diagnosis and treatment of velopharyngeal insufficiency were reviewed. All studies ascertained by PubMed search were included. Recent findingsStudies reported on the application of magnetic resonance scanning, reliability tests of the International Working Group diagnostic protocol, the use of nasometry, and techniques designed to assess the function of the velopharyngeal mechanism. Treatment studies focused on outcomes in small samples of cases and complication rates from pharyngeal flap. One study discussed ineffective speech therapy procedures. SummaryThere were relatively few studies this past year. Those that were published were hindered by small and heterogeneous sample sizes and occasionally by inappropriate methods for assessing outcomes. None of the findings will have a major impact on the current state-of-the-art for diagnosis of velopharyngeal insufficiency. The speech therapy study has a very important message that should be taken to heart by all clinicians involved in the management of children with clefts and craniofacial disorders.

Speech outcome after palatal repair in nonsyndromic versus syndromic Robin sequence.

Background: The authors’ purpose was to document speech outcome after cleft palate repair in patients with syndromic versus nonsyndromic Robin sequence. They also report the results of secondary correction of velopharyngeal insufficiency using a superiorly based pharyngeal flap or double-opposing Z-palatoplasty. Methods: Charts of patients with Robin sequence and cleft palate between 1980 and 2007 were reviewed. Data collected included date of birth, sex, syndrome/association, cleft palatal type (Veau class I or II), age at palatoplasty, incidence of palatal fistula, postoperative speech assessment, videofluoroscopic results, need for secondary operation for velopharyngeal insufficiency, and type of secondary operation (pharyngeal flap or double-opposing Z-palatoplasty). Results: The authors identified 140 patients with Robin sequence who had palatal closure. Postoperative speech evaluation was available for 96 patients (69 percent). A syndrome or association was identified in 42 patients (30 percent). Primary palatoplasty was successful in 74 patients (77 percent); speech was characterized as competent and competent to borderline competent. The authors found a significantly higher incidence of velopharyngeal insufficiency following palatal repair for syndromic (38 percent) than nonsyndromic Robin sequence (16 percent). (p = 0.039). In patients with velopharyngeal insufficiency, competent or borderline competent speech was determined after double-opposing Z-palatoplasty (two of five patients) or pharyngeal flap (eight of 10 patients). Conclusions: The rate of velopharyngeal insufficiency in syndromic Robin sequence is significantly greater than in nonsyndromic Robin sequence. The authors prefer pharyngeal flap for velopharyngeal insufficiency in patients with Robin sequence, whether syndromic or nonsyndromic, without retrognathism or signs/symptoms of obstructive sleep apnea. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, II.

Language and Literacy Development in Individuals with Velo­‐Cardio­‐Facial Syndrome

Velo-cardio-facial syndrome (VCFS) is a genetic disorder caused by a microdeletion of chromosome 22q11.2. Although there is some variability, VCFS is associated with a characteristic physical, behavioral, and cognitive phenotype. This review article focuses on aspects of language and literacy development in VCFS, describing what is known and offering avenues for future research. The most consistently reported language and literacy findings in the VCFS population include reading, spelling, phonological processing, and auditory verbal rote memory abilities as areas of relative strength. Receptive language abilities are noted to be relatively stronger than expressive language abilities in individuals with VCFS. Speech disorders are very common in VCFS, and the most common compensatory articulation substitution in VCFS is the glottal stop.

Uvular malformation in the presence of deformational plagiocephaly.

Background:Deformational plagiocephaly is cranial asymmetry caused by external forces on the skull. Deformational plagiocephaly is seen in 5% to 48% of healthy newborns. Incomplete uvular fusion, in contrast, is one of many uvular malformations. The incidence of all degrees of incomplete uvular fusion is approximately 1% in healthy children. Bifid uvula is a malformation that is often considered a microform cleft palate or a marker for submucous cleft palate. Methods:This is a retrospective study of patients with deformational plagiocephaly seen at the Upstate Cleft and Craniofacial Center between January 1, 2006, and September 30, 2011. Patients were identified by the International Classification of Diseases, Ninth Revision code for plagiocephaly. Seventy-nine patients were excluded with craniosynostosis and syndromic diagnoses. One hundred forty-six patients with deformational plagiocephaly were included in the study. Data were collected for sex, age at presentation, parity, multiple births, delivery, oligohydramnios, cephalohematoma, uterine abnormalities, fetal position, and intrauterine growth restriction. Clinical findings were collected including location of cranial flattening and uvular malformations. Results:Twenty-four of 146 patients with deformational plagiocephaly had incomplete fusion of the uvula ranging from complete bifid uvula to a notched uvular tip (16.4%). This association was statistically significant (odds ratio, 18; 95% confidence interval, 11.1–28.9). Most patients (62.3%) were male. We recorded primiparity (44.5%), multiple births (17.1%), vacuum-assisted delivery (6.2%), cesarean section (36.3%), oligohydramnios (4.1%), uterine abnormalities (2.1%), abnormal fetal position (3.4%), and intrauterine growth restriction (1.4%). Ten of the 24 patients with plagiocephaly and uvular malformation were seen for an initial consultation only in our chart system. Of the remaining 14 patients with follow-up, none had recorded signs or symptoms of velopharyngeal insufficiency. Conclusions:The incidence of incomplete uvular fusion in infants with deformational plagiocephaly is 16.4%, which is significantly higher than the approximate 1% incidence reported in the general population. This is the first report of uvular malformation in the presence of deformational plagiocephaly.

Anatomic Severity, Midfacial Growth, and Speech Outcomes in Van der Woude/Popliteal Pterygium Syndromes Compared to Nonsyndromic Cleft Lip/Palate.

Objective To summarize the clinical characteristics and surgical and speech outcomes for patients with Van der Woude/popliteal pterygium syndromes (VWS/PPS) and to compare them with a historic cohort of patients with nonsyndromic cleft lip/cleft palate (CL/P) Design Retrospective chart review. Setting Tertiary care center. Patients All patients with VWS/PPS seen at Boston Childrens Hospital from 1979 to 2012: 28 patients with VWS (n = 21)/PPS (n = 7) whose mean age was 17.3 ± 10.4 years, including 18 females (64%) and 10 males (36%); 18 patients (64%) had a family history of VWS/PPS. Main Outcome Measures Cleft type, operative procedures, speech, and midfacial growth. Data were compared with historic cohorts of patients with nonsyndromic CL/P treated at one tertiary care center. Results There were 24 patients (86%) with CP±L, Veau types I (n = 4,17%), II (n = 4, 17%), III (n = 5, 21%), and IV (n = 11, 46%). Nine patients (38%) had palatal fistula after palatoplasty. Fourteen of 23 (61%) patients with CL/P age 5 years or older had midfacial retrusion, and 10 (43%) required a pharyngeal flap for velopharyngeal insufficiency. Fishers exact test demonstrated higher frequencies of Veau type IV CP±L (P = .0016), bilateral CL±P (P = .0001), and complete CL±P (P < .0001) in VWS/PPS compared with nonsyndromic patients. Incidences of midfacial retrusion (P = .0001), palatal fistula (P < .0001), and need for pharyngeal flap (P = .0014) were significantly greater in patients with VWS/PPS. Conclusions Patients with VWS/PPS have more severe forms of labiopalatal clefting and higher incidences of midfacial retrusion, palatal fistula, and velopharyngeal insufficiency following primary repair as compared with nonsyndromic CL/P.