Complex Care of Individuals with Cleft Lip and Cleft Palate or Velopharyngeal Insufficiency

Objective: To investigate the impact of cleft width and cleft type on the need for secondary surgery and velopharyngeal competence from a longitudinal perspective. Design: Retrospective, longitudinal study. Setting: A single multidisciplinary craniofacial team at a university hospital. Patients: Consecutive patients with unilateral or bilateral cleft lip and palate and cleft palate only (n = 313) born from 1984 to 2002, treated with 2-stage palatal surgery, were reviewed. A total of 213 patients were included. Main Outcome Measures: The impact of initial cleft width and cleft type on secondary surgery. Assessment of hypernasality, audible nasal emission, and glottal articulation from routine follow-ups from 3 to 16 years of age. The assessments were compared with reassessments of 10% of the recordings. Results: Cleft width, but not cleft type, predicted the need for secondary surgery, either due to palatal dehiscence or velopharyngeal insufficiency. The distribution of cleft width between the scale steps on a 4-point scale for hypernasality and audible nasal emission differed significantly at 5 years of age but not at any other age. Presence of glottal articulation differed significantly at 3 and 5 years of age. No differences between cleft types were seen at any age for any speech variable. Conclusions: Cleft width emerged as a predictor of the need for secondary surgery as well as more deviance in speech variables related to velopharyngeal competence during the preschool years. Cleft type was not related to the need for secondary surgery nor speech outcome at any age.

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Treatment of Asymmetric Velopharyngeal Insufficiency with Skewed Pharyngeal Flap

The Cleft Palate-Craniofacial Journal ◽

10.1597/1545-1569_1994_031_0287_toaviw_2.3.co_2 ◽

1994 ◽

Vol 31 (4) ◽

pp. 287-294 ◽

Cited By ~ 8

Author(s):

Ravelo V. Argamaso ◽

Gerald J. Levandowski ◽

Karen J. Golding-Kushner ◽

Robert J. Shprintzen

Keyword(s):

Cleft Palate ◽

Cleft Lip ◽

Tumor Resection ◽

Velocardiofacial Syndrome ◽

Velopharyngeal Insufficiency ◽

Lateral Pharyngeal Wall ◽

Pharyngeal Wall ◽

Wall Movement ◽

Hypernasal Speech

Twenty-two patients, with hypernasal speech and asymmetric velopharyngeal insufficiency (VPI) identified preoperatively by multi-view videofluoroscopy 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 latrogenic 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.

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Is Isolated Palatal Anomaly an Indication to Screen for 22q11 Region Deletion?

The Cleft Palate-Craniofacial Journal ◽

10.1597/1545-1569_2003_040_0176_iipaai_2.0.co_2 ◽

2003 ◽

Vol 40 (2) ◽

pp. 176-179 ◽

Cited By ~ 5

Author(s):

Orit Reish ◽

Yehuda Finkelstein ◽

Ronit Mesterman ◽

Ariela Nachmani ◽

Baruch Wolach ◽

...

Keyword(s):

Cleft Palate ◽

Cleft Lip ◽

Cleft Lip And Palate ◽

Young Patients ◽

Velocardiofacial Syndrome ◽

Clinical Findings ◽

Velopharyngeal Insufficiency ◽

Facial Dysmorphism ◽

22Q11 Deletion ◽

22Q11 Region

Objective Velocardiofacial syndrome (VCFS) is the most common multiple anomaly disorder associated with palatal clefting. Cytogenetic hemizygous deletion of 22q11 region is found in 80% of patients. The frequency of 22q11 deletion in patients presenting with isolated palatal anomalies has not been fully assessed. Our objective was to determine the frequency of the deletion in patients with isolated palatal anomalies. Design Patients were referred because of velopharyngeal insufficiency because of isolated congenital palatal anomalies. Diagnosis of palatal anomalies was confirmed by videonasopharyngoscopy, multiview videofluoroscopy and cephalometry. Other clinical findings suggestive of VCFS were sought, and subjects with these characteristics were excluded from the study. Peripheral blood samples from all patients were analyzed cytogenetically utilizing fluorescent in situ hybridization for the 22q11 region. Results Thirty-eight patients aged 3 to 31 years were included in the study. Nine had cleft palate, 7 cleft lip and palate, 10 overt and 11 occult submucous cleft palate, and 1 had a deep nasopharynx. No deletion of 22q11 region was detected in any of the evaluated patients. Conclusions A routine screening for the 22q11 deletion in older children and adults presenting with an isolated palatal anomaly may not be required. Because other signs related to VCFS such as facial dysmorphism and behavioral or psychiatric disorders may evolve at an older age, young patients should be followed up and reevaluated for additional relevant symptoms that may lead to deletion evaluation. In light of the fact that the current literature is inconsistent, the relative small size of this study and the significant consequences of missed 22q11.2 deletion, more information is needed before definitive recommendations can be made.

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Velopharyngeal Function in Nonsyndromic Cleft Palate: Relevance of Surgical Technique, Age at Repair, and Cleft Type

The Cleft Palate-Craniofacial Journal ◽

10.1597/1545-1569_1998_035_0095_vfincp_2.3.co_2 ◽

1998 ◽

Vol 35 (2) ◽

pp. 95-100 ◽

Cited By ~ 46

Author(s):

Eileen M. Marrinan ◽

Richard A. Labrie ◽

John B. Mulliken

Keyword(s):

Surgical Technique ◽

Cleft Palate ◽

Cleft Lip ◽

Velopharyngeal Insufficiency ◽

Significant Variable ◽

Muscle Complex ◽

Cleft Lip Palate ◽

Pharyngeal Flap ◽

Velopharyngeal Function ◽

Repair Group

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 Children's 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.

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Speech Intelligibility of Patients with Cleft Lip and Palate after Placement of Speech Prosthesis

The Cleft Palate-Craniofacial Journal ◽

10.1597/06-061.1 ◽

2007 ◽

Vol 44 (6) ◽

pp. 635-641 ◽

Cited By ~ 29

Author(s):

João Henrique Nogueira Pinto ◽

Giseleda Silva Dalben ◽

Maria Inês Pegoraro-Krook

Keyword(s):

Cleft Palate ◽

Speech Intelligibility ◽

Cleft Lip ◽

Speech Therapy ◽

Cleft Lip And Palate ◽

Velopharyngeal Insufficiency ◽

Cross Sectional ◽

Before And After ◽

Dental Needs

Objective: To evaluate the speech intelligibility of patients with clefts before and after placement of a speech prosthesis. Design: Cross-sectional. Setting: Hospital for Rehabilitation of Craniofacial Anomalies, University of São Paulo (HRAC/USP), Bauru, Brazil. Patients: Twenty-seven patients with unoperated cleft palate or operated cleft palate presenting with velopharyngeal insufficiency (VPI) after primary palatoplasty, treated with speech prosthesis, aged 8 to 63 years. Interventions: Patients were fitted with palatopharyngeal obturators or pharyngeal bulbs, suitable to their dental needs. Five speech-language pathologists blindly evaluated speech samples of the patients with and without the prosthesis. Main Outcome Measures: Classification of speech samples according to a scoring system developed for speech intelligibility problems: 1 (normal), 2 (mild), 3 (mild to moderate), 4 (moderate), 5 (moderate to severe), and 6 (severe). Results were evaluated by the calculation of means of all judges for each patient in both situations. Results: The judges presented significant agreement (W = .789, p < .01). Speech intelligibility was significantly better after placement of the prosthesis for both unoperated patients (Z = 1.93, p = .02) and operated patients with VPI after primary palatoplasty (Z = 1.78, p = .03). Conclusions: Speech intelligibility may be improved by rehabilitation of patients with cleft palate using a speech prosthesis. Speech therapy is needed to eliminate any compensatory articulation productions developed prior to prosthetic management.

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Provision and Utilization of Team- and Community-Based Operative Care for Patients With Cleft Lip/Palate in North Carolina

The Cleft Palate-Craniofacial Journal ◽

10.1177/1055665620946565 ◽

2020 ◽

Vol 57 (11) ◽

pp. 1298-1307

Author(s):

Elliot Le ◽

Peter Shrader ◽

Hayden Bosworth ◽

Jillian Hurst ◽

Benjamin Goldstein ◽

...

Keyword(s):

North Carolina ◽

Cleft Palate ◽

Cleft Lip ◽

Alveolar Bone ◽

Velopharyngeal Insufficiency ◽

Multidisciplinary Teams ◽

Cross Sectional ◽

Operative Care ◽

Alveolar Bone Grafting ◽

Community Providers

Objective: To characterize operative care for cleft lip and/or palate (CL/P) based on location (ie, from American Cleft Palate Craniofacial Association [ACPA]–approved multidisciplinary teams or from community providers). Design: Cross-sectional analysis of Healthcare Cost and Utilization Project State Inpatient Database and State Ambulatory Surgery & Services Database databases for North Carolina from 2012 to 2015. Setting/Patients and Main Outcome Measures: Clinical encounters for children with CL/P undergoing operative procedures were identified, classified by location as “Team” versus “Community,” and characterized by demographic, geographic, clinical, and procedural factors. A secondary evaluation reviewed concordance of team and community practices with an ACPA guideline related to coordination of care. Results: Three teams and 39 community providers performed a total of 3010 cleft-related procedures across 2070 encounters. Teams performed 69.7% of total volume and performed the majority of cleft procedures, including cleft lip repair, palate repair, alveolar bone grafting, and correction of velopharyngeal insufficiency. Community locations principally offered myringotomy and rhinoplasty. Team care was associated with higher guideline concordance. Conclusions: American Cleft Palate Craniofacial Association –approved team-based care accounts for the majority of cleft-related care in North Carolina; however, a substantial volume of cleft-related procedures was provided by community providers, with 3 providers accounting for the vast majority of community cases.

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Outcomes in Pharyngoplasty: A 10-Year Experience

The Cleft Palate-Craniofacial Journal ◽

10.1597/04-115.1 ◽

2006 ◽

Vol 43 (2) ◽

pp. 222-225 ◽

Cited By ~ 32

Author(s):

Landon S. Pryor ◽

James Lehman ◽

Michael G. Parker ◽

Anna Schmidt ◽

Lynn Fox ◽

...

Keyword(s):

Cleft Palate ◽

Cleft Lip ◽

Cleft Lip And Palate ◽

Revisional Surgery ◽

Retrospective Chart Review ◽

Surgical Revision ◽

Velopharyngeal Insufficiency ◽

Significant Difference ◽

Potential Risk Factors ◽

Palate Repair

Objective The outcomes of 61 patients who underwent a pharyngoplasty for velopharyngeal insufficiency were reviewed to determine potential risk factors for reoperation. Design This was a retrospective chart review of 61 consecutive patients over approximately 10 years (1993 to 2003). Variables analyzed included gender, cleft type, age at the time of pharyngoplasty, length of time between palate repair and pharyngoplasty, and associated syndromes. Participants Of the 61 patients, 20 (34%) had a unilateral cleft lip and palate, 5 (8%) had a bilateral cleft lip and palate, 13 (21%) had an isolated cleft palate, 7 (11%) had a submucous cleft palate, and 16 (26%) were diagnosed with noncleft velopharyngeal insufficiency. Results Of the 61 patients, 10 (16%) required surgical revision. No statistically significant difference was found among gender, cleft type, age at the time of pharyngoplasty, the length of time between palate repair and pharyngoplasty, and associated congenital syndromes, with respect to the need for surgical revision (p > .05). Of the surgical revisions, 50% (5) were performed for a pharyngoplasty that was placed too low. Conclusions Because 50% of the pharyngoplasty revisions had evidence of poor velopharyngeal closure and associated hypernasality resulting from low placement of the sphincter, the pharyngoplasty needs to be placed at a high level to reduce the risk for revisional surgery. The pharyngoplasty is a good operation for velopharyngeal insufficiency with an overall success rate of 84% (51 of 61) after one operation and greater than 98% (60 of 61) after two operations.

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Applying the ICF-CY to Specialist Speech-Language Pathologists’ Practice With Toddlers With Cleft Palate Speech

The Cleft Palate-Craniofacial Journal ◽

10.1177/1055665620918799 ◽

2020 ◽

Vol 57 (9) ◽

pp. 1105-1116 ◽

Cited By ~ 2

Author(s):

Anna Cronin ◽

Sharynne McLeod ◽

Sarah Verdon

Keyword(s):

Young Children ◽

Cleft Palate ◽

Cleft Lip ◽

The United States ◽

Velopharyngeal Insufficiency ◽

International Classification Of Functioning ◽

Speech Language Pathologists ◽

Cross Sectional ◽

Cleft Palate Speech ◽

Public Clinics

Objective: To evaluate how the practice of specialist speech-language pathologists (SLPs) working with young children with cleft palate ± cleft lip (CP±L) maps onto the International Classification of Functioning, Disability, and Health – Children and Youth version (ICF-CY) and consider the functionality of the categories of the ICF-CY for this specialist area of practice. Design: Cross-sectional, qualitative study. Setting: Semistructured face-to-face interviews were conducted with SLPs working in tertiary-level hospitals, universities, and public clinics. Participants: Six specialist SLPs with 17 to 39 years of experience working with young children with CP±L as researchers and clinicians in Australia, Brazil, Denmark, Ireland, New Zealand, and the United States. Main Outcome Measure(s): Specialists’ practices were captured using in-depth, semistructured interviews. Data collected were analyzed by directed content analysis applying the ICF-CY as a coding schema. Results: In total, 4077 data points were coded. Most mapped onto Body Structures (684, 16.8%), Body Functions (906, 22.2%), and Environmental Factors (1626, 39.9%) with less emphasis on Activities and Participation (560, 13.7%). A “best fit” approach was taken to topics that did not map exactly onto categories of the ICF-CY (eg, velopharyngeal insufficiency [VPI]); however, there was not always an ideally suitable category available. Conclusions: The current study revealed strengths and challenges in categorizing practice within the ICF-CY for children with CP±L, including collaboration with parents and significant others, specificity around speech, language, and articulation, and the different types of VPI. Therefore, future discussion around how best to use the framework with children with CP±L is needed.

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Communicating With a Cleft Palate Team: Improving Coordination of Care Across Treatment Settings

Perspectives on Speech Science and Orofacial Disorders ◽

10.1044/ssod25.1.35 ◽

2015 ◽

Vol 25 (1) ◽

pp. 35-38 ◽

Cited By ~ 2

Author(s):

Scott Dailey ◽

Kris Wilson

Keyword(s):

Best Practices ◽

Cleft Palate ◽

Cleft Lip ◽

Speech Disorders ◽

Therapeutic Interventions ◽

Velopharyngeal Insufficiency ◽

Coordination Of Care ◽

Critical Aspect ◽

Complex Needs ◽

Collaboration And Communication

Patients with cleft palate with and without cleft lip, and/or velopharyngeal insufficiency (VPI) present with complex speech disorders that require both surgical and therapeutic interventions. Because of their complex needs, best practices indicate that individuals should be followed by a Cleft Palate Team in order to provide care in a coordinated fashion. Collaboration between SLPs in the community and SLPs on a Cleft Palate Team is a critical aspect to ensuring optimal speech outcomes for patients with clefts/VPI. Collaboration and communication between the SLPs may take different avenues and different timelines based on the needs of the patient and resources available.

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Quality of Life in Patients With Velopharyngeal Insufficiency in West China

The Cleft Palate-Craniofacial Journal ◽

10.1177/10556656211034107 ◽

2021 ◽

pp. 105566562110341

Author(s):

Lidan Lu ◽

Aipiziguli Yakupu ◽

Yanhui Wu ◽

Xiangnan Li ◽

Pengxin Zhang ◽

...

Keyword(s):

Quality Of Life ◽

Cleft Palate ◽

Cleft Lip ◽

Mixed Model ◽

Linear Mixed Model ◽

Cleft Lip And Palate ◽

Generalized Linear Mixed Model ◽

Velopharyngeal Insufficiency ◽

Caregiver Report

Objective This study aimed to investigate the quality of life (QOL) of patients with cleft lip and palate and velopharyngeal insufficiency (VPI) in relation to sex, age, age at initial cleft lip surgery, and age at initial cleft palate surgery. Design This is a cross-sectional study. Setting The study was conducted in a tertiary medical center. Participants The participants were caregivers of 72 patients with cleft lip and palate and VPI aged 4 to 20 years. Main Outcome Measure(s) Participants completed the Chinese version of the caregiver report of the VPI Effects on Life Outcomes (VELO) questionnaire. The Mann–Whitney U test was used to evaluate the patients’ sex, age, age at initial cleft lip repair, and age at initial cleft palate repair in relation to VELO total score and domains. Spearman correlation analysis was completed including all study variables. Associations between the study variables and the VELO total score were tested using a generalized linear mixed model. Results In the univariate analysis, patients’ age and age at initial cleft palate surgery influenced the QOL of patients with VPI. There were no differences in the VELO total score or domains based on sex or age at first cleft lip surgery. In the generalized linear mixed model, patients older than 8 years had higher VELO total scores. Conclusions By caregiver report, the QOL of patients under age 8 years with VPI was lower than older patients. In addition, the caregiver impact domain was higher for parents of children who had their initial cleft palate surgery at age 2 years or younger.

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