Submucous cleft with a congenital palatal perforation

Submucous cleft palate is a congenital disorder resulting in velopharyngeal insufficiency. Nasal regurgitation is one of the symptoms of submucous cleft palate. This study aims to investigate a relationship between submucous cleft palate and a history of nasal regurgitation in patients during infancy. This retrospective study includes patients diagnosed with submucous cleft palate at our hospital for 20 years, from 1998 to 2017. Based on the clinical records of these patients, we investigated sex, age at referral to our facility, age at surgery, presence of nasal regurgitation, method of surgery, association of syndromes and mental retardation, and transition of velopharyngeal function. There were fifty-seven patients referred to our facility. Patients with episodes of nasal regurgitation were referred to clinics later than those without nasal regurgitation. The timing of surgery was also the same. The velopharyngeal function of patients with nasal regurgitation was worse than that of those without nasal regurgitation. A history of nasal regurgitation cannot predict submucous cleft palate. However, patients with submucous cleft palate with nasal regurgitation had worse velopharyngeal function than those without nasal regurgitation. An increased understanding of the relationship between submucous cleft palate and nasal regurgitation and further study will predict submucous cleft palate and determine a good timing for surgical interventions.

Congenital palatal fistula (CPF) is the rarest phenotypic variation of cleft palate, commonly associated with submucous cleft palate (SCP). Isolated palatal fistula without SCP has been reported only rarely. We report here the case of a 4-year-old child, with a 10 mm × 5 mm CPF, presenting with nasal regurgitation and speech abnormalities. The patient had unilateral palatal muscular malposition without classical features of SCP. The repair was performed using turnover flaps and Bardach's two-long flap palatoplasty and intravelar veloplasty. Healing occurred without any complications.

Background:Although cleft lip and cleft palate are among the most common congenital malformations, the presence of an isolated congenital palatal fistula along with a submucous cleft is very rare. This appears as an oval-shaped, full-thickness fenestration in the palatal midline that does not fully extend anteriorly or posteriorly, accompanied by the findings of a submucous cleft. Because of the uncommon nature of this entity, there is controversy about its etiology, diagnosis, and management.Methods:Two cases of children with congenital palatal fistulae and a submucous cleft palate are presented who were treated in different settings by different surgeons. Cases are discussed along with a thorough review of the available literature.Results:Patient 1 presented at 4 years of age with “a hole in the palate” since birth and abnormal speech. His palatal fistula and submucous cleft were repaired with a modified von Langenbeck technique in Ethiopia. At a 2-year follow-up, the palate remained closed, but hypernasal speech persisted. Patient 2 was a 1-year-old presenting with failure to thrive and nasal regurgitation, who underwent a Furlow palatoplasty in the United States with good immediate results. She was unfortunately lost to follow-up.Conclusions:A congenital fenestration of the palate is rare. Reports reveal suboptimal speech at follow-up, despite various types of repair, especially when combined with a submucous cleft. Available literature suggests that repair should not focus on fistula closure only but instead on providing adequate palate length to provide good velopharyngeal function, as in any cleft palate repair.

To describe the clinical course and management of a patient with submucous cleft palate who developed myasthenia gravis (MG) as an adult and suffered recurrent hypernasality. Few reports have described MG patients undergoing pharyngeal flap surgery for velopharyngeal incompetence, and these have described only slight speech improvement in such patients. Case report. The patient underwent primary pushback palatoplasty and superiorly based pharyngeal flap surgery for submucous cleft and short palate at age 7. Hypernasality showed major improvement after initial surgery. At age 19, the patient developed MG that triggered the recurrence of velopharyngeal incompetence. After MG was treated, revision pushback palatoplasty was performed for velopharyngeal incompetence when the patient was 24 years old. Preoperatively and postoperatively, the patient was evaluated by the same speech-language-hearing therapists, each with at least 5 years of clinical experience in cleft palate speech. After the second pushback palatoplasty, hypernasality and audible nasal air emission during speech decreased to mild. Primary pushback palatoplasty and pharyngeal flap surgery were performed for the submucous cleft palate. Revision pushback palatoplasty improved velopharyngeal inadequacy induced by MG. Decreased perceived nasality positively influenced the patient's quality of life. Combined pushback palatoplasty and pharyngeal flap surgery is thus an option in surgical treatment for velopharyngeal inadequacy to close the cleft and the velopharyngeal orifice in cases of cleft palate and MG.

Objective: Submucous cleft palate (SMCP) is a congenital condition associated with abnormal development of the soft palate musculature. In a proportion of cases, this results in velopharyngeal insufficiency (VPI), the treatment for which includes pharyngeal flap surgery, pharyngoplasty, and palate reconstruction. The aim of this paper is to determine whether there is superiority of one or more types of surgical procedure over the others in improving speech in patients with VPI secondary to SMCP. Methodology: Nine databases, including MEDLINE and EMBASE, were searched between inception and January 2013 to identify articles published relating to the surgical management of SMCP. Only studies that reported outcome measures for postoperative speech were included in the systematic review. Results: Twenty-six studies analyzing the outcomes of surgery for VPI in patients with SMCP met the inclusion criteria. In these studies, speech outcomes were measured either in a binary fashion (i.e., normal speech or evidence of VPI) or using scales of VPI severity. Of the 26 studies, only two utilized blinded speech assessment, and 12 included both preoperative and postoperative speech assessment. Conclusions: The review found little evidence to support any specific surgical intervention. This is in large part due to the inclusion of mixed etiologies within study populations and the lack of unbiased validated preoperative and postoperative speech assessment. Further methodologically rigorous studies need to be conducted to provide a secure evidence base for the surgical management of SMCP.

A retrospective study was undertaken to assess speech outcomes in patients undergoing Furlow palatoplasty. Since 1994, the authors have used the position of the levator veli palatini musculature to determine type of surgical intervention recommended for the management of velopharyngeal insufficiency. Furlow palatoplasty has been used in patients with clinical evidence of sagittally oriented levator veli palatini musculature. Forty-eight patients who underwent a Furlow palatoplasty between June of 1994 and August of 1998 were included. All patients underwent preoperative and postoperative perceptual speech analyses to describe velopharyngeal insufficiency severity, nasal air emissions, and resonance, and preoperative nasendoscopy to assess velopharyngeal gap size and palatal and lateral pharyngeal wall movement. Other patient characteristics considered included gender, age at time of surgery, previously repaired cleft palate, submucous cleft palate, and syndrome diagnosis. Speech outcomes were determined on the basis of postoperative perceptual speech analyses and were categorized in one of three ways: (1) complete resolution of velopharyngeal insufficiency, (2) substantial improvement of velopharyngeal insufficiency, and (3) audible residual velopharyngeal insufficiency. Complete resolution of velopharyngeal insufficiency was defined as normal resonance and an absence of nasal air emissions. Substantial improvement of velopharyngeal insufficiency was defined as an improvement of at least two categories in velopharyngeal insufficiency severity in those patients without complete resolution. Audible residual velopharyngeal insufficiency refers to patients with postoperative velopharyngeal insufficiency severity ratings of mild, moderate, or severe. The male:female ratio in the study was 27:21. Twelve patients were syndromic; three had velocardiofacial syndrome. The median age at surgery was 6.5 years (range, 2 to 22 years). The average duration of follow-up was 14.7 months (range, 1.3 to 58.6 months). Postoperatively, the severity of velopharyngeal insufficiency was rated as none in 19 of the 48 patients (39.6 percent), minimal in eight (16.7 percent), mild in six (12.5 percent), moderate in nine (18.75 percent), and severe in six (12.5 percent). Substantial improvement was seen in seven of the 29 patients without complete resolution. There was a significant association between male gender and complete resolution of velopharyngeal insufficiency (p < 0.05). Presence of syndrome and female gender was associated with audible residual velopharyngeal insufficiency (p < 0.05). The main complication was palatal fistula (two cases). In conclusion, most patients who underwent a Furlow palatoplasty had a complete resolution or substantial improvement of velopharyngeal insufficiency postoperatively, and there were few surgical complications.

BackgroundThe submucous cleft palate (SMCP) is a type of cleft palate that may result in velopharyngeal insufficiency (VPI). Palate muscles completely separate oral and nasal cavities by closing off the velopharynx during functional processes such as speech or swallow. Also, hypernasality may arise from anatomical or neurological abnormalities in these functions. Treatments of this issue involve a combination of surgical intervention, speech aid, and speech therapy. This case report demonstrates successfully treated VPI resulted from SMCP without any surgical intervention but solely with speech aid appliance and speech therapy.Case presentationA 13-year-old female patient with a speech disorder from velopharyngeal insufficiency that was caused by a submucous cleft palate visited to our OMFS clinic. In the intraoral examination, the patient had a short soft palate and bifid uvula. And the muscles in the palate did not contract properly during oral speech. She had no surgical history such as primary palatoplasty or pharyngoplasty except for tonsillectomy. And there were no other medical histories. Objective speech assessment using nasometer was performed. We diagnosed that the patient had a SMCP. The patient has shown a decrease in speech intelligibility, which resulted from hypernasality. We decided to treat the patient with speech aid (palatal lift) along with speech therapy. During the 7-month treatment, hypernasality measured by a nasometer decreased and speech intelligibility became normal.ConclusionsSurgery remains the first treatment option for patients with velopharyngeal insufficiencies from submucous cleft palates. However, there were few reports about objective speech evaluation pre- or post-operation. Moreover, there has been no report of non-surgical treatment in the recent studies. From this perspective, this report of objective improvement of speech intelligibility of VPI patient with SMCP by non-surgical treatment has a significant meaning. Speech aid can be considered as one of treatment options for management of SMCP.

INTRODUCTION: Patients with 22q deletion syndrome are at increased risk of submucous cleft palate and velopharyngeal insufficiency (1–4). The authors' aim is to evaluate speech outcomes following primary Furlow palatoplasty or pharyngeal flap for correction of velopharyngeal insufficiency in submucous cleft palate patients with and without 22q deletion syndrome. METHODS: Records of submucous cleft palate patients who underwent primary surgery between 2001 and 2010 were reviewed. Data included 22q deletion syndrome diagnosis, age at surgery, procedure, preoperative nasopharyngoscopy and nasometry, speech outcomes, complications and secondary surgery rates. The primary study outcome was a perceptual assessment of resonance within normal limits. Secondary outcomes included the proportions of 22q deletion syndrome and nonsyndromic patients who (1) improved postoperatively with regards to perceptual assessment; (2) experienced a complication following surgery; or (3) required revision surgery for persistent velopharyngeal insufficiency. RESULTS: Seventy-eight submucous cleft palate patients were identified. Twenty-three patients had 22q deletion syndrome. Fewer 22q deletion syndrome patients obtained normal resonance on perceptual assessment compared to nonsyndromic patients (74 percent versus 88 percent). A similar difference existed based on postoperative nasometric scores. Among 22q deletion syndrome patients, similar success rates were achieved with Furlow palatoplasty and pharyngeal flap. No difference in the proportion improved postoperatively was noted between 22q deletion syndrome and nonsyndromic groups. One complication was experienced per group. More revision surgeries were indicated in the 22q deletion syndrome group (17 percent) compared to nonsyndromic group (4 percent). Median times to normal resonance for 22q deletion syndrome and nonsyndromic patients were 150 weeks and 34 weeks, respectively (Figure 1). Adjusting for multiple variables, 22q deletion syndrome patients were 3.6 times less likely to develop normal resonance.Figure 1: Kaplan-Meier analysis comparing unadjusted probabilities of developing normal resonance on perceptual speech assessment for 22q deletion (red) and nonsyndromic (blue) patients. Censored data are depicted by open circles. Median times to normal resonance for 22q deletion syndrome and nonsyndromic patients were 150 weeks and 34 weeks, respectively.CONCLUSION: Careful selection of Furlow palatoplasty or pharyngeal flap for primary repair of submucous cleft palate is highly effective in 22q deletion syndrome patients and yields results approaching those of nonsyndromic patients.

It is uncommon for a palatal fistula to be detected in individuals who have not undergone surgery, and only sporadic cases have been reported. It is even more difficult to find cases of acquired palatal fistula in patients with submucous or incomplete cleft palate. Herein, we present 2 rare cases of this phenomenon. Case 1 was a patient with submucous cleft palate who acquired a palatal fistula after suffering from oral candidiasis at the age of 5 months. Case 2 was a patient with incomplete cleft palate who spontaneously, without trauma or infection, presented with a palatal fistula at the age of 9 months.

Submucous cleft palate (SMCP) is a subgroup of cleft palate that can present as velopharyngeal insufficiency due to muscle displacement. The pharyngeal flap and Furlow palatoplasty have been introduced to correct SMCP patient with velopharyngeal insufficiency. However, pharyngeal flap and Furlow palatoplasty can occur various complications. We consider the overlapping intravelar veloplasty (IVVP) can overcome these complications. Therefore, we present the speech outcomes of overlapping IVVP for the treatment of patients with SMCP. We retrospectively reviewed 12 patients with SMCP underwent overlapping IVVP between April 2016 and October 2018. The patients who underwent speech evaluation, nasometry, and nasoendoscopy before and after surgery and who were followed up for >18 months were enrolled in this study. The average age of the patients was 5 years (range, 3-11 years) and the postoperative follow-up period ranged from 18 to 24 months (mean, 20 months). The preoperative perceptual speech evaluation was moderate and the postoperative evaluation was normal ( P < 0.01). The preoperative and postoperative nasalance scores obtained using a nasometer were 37.00 and 12.50, respectively, ( P < 0.01). Preoperative and postoperative velopharyngeal movements were grades 3 and 0, respectively, ( P < 0.01). Our study showed that overlapping IVVP could provide successful correction of velopharyngeal insufficiency in patients with SMCP, including relatively old patients.

Submucous cleft palate (SMCP) is a rare form of cleft palate involving abnormal muscle development in the soft palate. Diagnosis is often delayed, with many cases identified after age 4. Symptoms like hypernasality and velopharyngeal insufficiency (VPI) may appear, though some remain asymptomatic. SMCP diagnosis typically relies on Calnan's triad: bifid uvula, hard palate notch, and zona pellucida. Wiskott-Aldrich syndrome (WAS) is a rare X-linked immune disorder marked by thrombocytopenia, eczema, and infections. Early diagnosis is key to effective treatment. Due to the rarity of both SMCP and WAS, we present a unique case and suggest careful management for optimal outcomes. A one-year and 10-month-old boy withWASwas referred to our cleft center for evaluation of a congenital palatal fistula. There was no history of trauma or surgery, but a 9 × 7 mm midline fistula was observed, along with a diagnosis ofSMCP. After hematopoietic stem cell transplantation (HSCT) and continued immunosuppressive therapy, a palatal plate was created to assist feeding. At age 5, a cleft palate repair using a modified Bardach technique was performed. Postoperative healing was successful without complications. This case highlights multidisciplinary management in a patient with both SMCP and WAS.

One hundred and twenty-one cleft children (67 with isolated cleft lip (CL), 32 with isolated submucous cleft palate (SMCP), and 22 with combined cleft lip and submucous cleft palate (CL + SMCP)) were compared retrospectively from lateral cephalograms taken at a mean age of 6.2 years (range 5.5-7.9). None of the children had had their palates repaired or been operated on to treat velopharyngeal insufficiency (VPI). The children with CL + SMCP and CL had similar morphology. They had greater maxillary length, greater maxillary and mandibular prominence, less vertical growth pattern, less deep nasopharyngeal airways, and thinner upper lips than those with isolated SMCP. After 6 years of age one patient with CL + SMCP, none with CL, and 16 with SMCP needed operations for VPI. This small series suggests that children with CL + SMCP and SMCP have different morphology. Although CL + SMCP is a combination of two types of clefts, it seems to be associated with similar morphology to CL.

Objective: To determine which surgical technique offers the lowest rate of velopharyngeal insufficiency (VPI) without the need for further operative intervention, in pediatric patients with nonsyndromic submucous cleft palate (SMCP). Methods: This systematic review and meta-analysis included articles reporting on nonsyndromic pediatric patients treated surgically during childhood for SMCP, with data on postoperative speech outcomes and/or recommendations for secondary surgery. Main outcome measures included rates of unfavorable speech outcomes defined as persistent VPI requiring secondary surgery and speech outcome data. Results: 15 articles met our inclusion criteria, reporting on 383 children who underwent surgical treatment; 343 patients were included in studies reporting recommendations for secondary surgery. There was 1 randomized comparative trial, 4 comparative studies, and 10 single cohort studies. Eight articles used validated speech assessment tools. Our model showed the proportion of patients recommended for secondary surgery varied between techniques, ranging from 0.0% (CI 0.0, 1000) in pharyngeal flap to 17.8% (CI 8.9, 32.5) in straight line repair techniques, but there was no statistically significant difference between treatments (P = .33). Speech improvement ranged from 44.4% to 100%, with 9 studies recommending secondary surgery for some of their patient series. Conclusions: Although not of statistical significance, pharyngeal flap yields the lowest rate of reoperation as a primary technique for pediatric patients with nonsyndromic SMCP. Delayed repair age inherent to SMCP may render operations that rely on a functional levator muscle with less favorable outcomes. The absence of standardized surgical techniques, speech outcomes, speech therapy, and assessment make comparative analysis and recommendation difficult. We advocate for standardized speech assessment tools to improve future quantitative assessment of cleft surgery outcomes and a randomized controlled trial to better elucidate the preferred first-line technique.

Submucous cleft palate (SMCP) is a common congenital malformation of the soft palate which may present as velopharyngeal insufficiency (VPI), which can affect the quality and intelligibility of speech. Surgical techniques, which can be used to reconstruct these structural or anatomical defects and to correct velopharyngeal insufficiency, include palatal repair and procedures that rearrange the muscle attachments of the soft palate. To provide reliable evidence regarding the effectiveness of surgical interventions to treat velopharyngeal insufficiency and improve speech in patients with submucous cleft palate. We searched the Cochrane Oral Health Group Trials Register (to 21st December 2006); Cochrane Developmental, Psychosocial and Learning Problems Group Trials Register (on 12th March 2007); the Cochrane Central Register of Controlled Trials (CENTRAL) (The Cochrane Library 2006, Issue 4); MEDLINE (from 1966 to 21st December 2006); EMBASE (from 1980 to 21st December 2006); and CINAHL, ERIC, PsycINFO (on 7th March 2007). Randomised controlled trials comparing surgical interventions to correct velopharyngeal insufficiency in submucous cleft palate. Limited data from one included trial precluded pooling of data, and only a descriptive summary is presented. This review included one trial, involving 72 participants aged 4 to 7 years with submucous cleft palate associated velopharyngeal insufficiency, which compared minimal incision palatopharyngoplasty (MIPP) to MIPP with additional velopharyngeal surgery, either pharyngeal flap (32) or sphincter pharyngoplasty (3). The trial provided no information about post-operative speech assessment, very limited data on any instrumental assessments and there were no reports of obstructive sleep apnoea or other adverse effects after the interventions. Complete closure occurred in 32 (86%) of the participants in the MIPP group and in 31 (89%) in the additional treatment group, P > 0.05. After eliminating the nine patients with residual velopharyngeal insufficiency, the post-operative gap size during closure was 7.4 +/-3.2% in the MIPP group and 8 +/-4.1% in the additional intervention group (P > 0.5). The trial provided some weak and unreliable evidence that there was no significant difference in the effectiveness of minimal incision palatopharyngoplasty versus the same procedure performed simultaneously with an individually tailored pharyngeal flap or sphincter pharyngoplasty for correcting velopharyngeal insufficiency associated with submucous cleft palate.

Submucous cleft palate is a congenital malformation with specific clinical and anatomical features. It can be present with or without velopharyngeal insufficiency. Surgical treatment of this malformation is indicated only when velopharyngeal insufficiency has been demonstrated. This article compares two modalities of surgical treatment for submucous cleft palate. The first includes a minimal incision palatopharyngoplasty, as described in a previous report. The second combines the first technique with additional individualized velopharyngeal surgery (individualized pharyngeal flap or sphincter pharyngoplasty) performed simultaneously. The individualized part of the procedure was selected and performed according to the findings of videonasopharyngoscopy and multiview videofluoroscopy, as reported previously. Two hundred and three patients with submucous cleft palate were studied from 1990 to 1999. Videonasopharyngoscopy and multiview videofluoroscopy demonstrated velopharyngeal insufficiency in 72 patients, who were randomly divided into two groups. Those in group 1 (n = 37) underwent a minimal incision palatopharyngoplasty. Patients in group 2 (n = 35) also underwent that procedure but simultaneously received individualized pharyngeal flap or sphincter pharyngoplasty, according to the findings of videonasopharyngoscopy and multiview videofluoroscopy. The median age of the patients from both groups was not significantly different (p > 0.5). The frequency of residual velopharyngeal insufficiency after palatal closure was not significantly different in both groups of patients (14 percent versus 11 percent; p > 0.5). The mean size of the gap at the velopharyngeal sphincter during speech was not significantly different in both groups of patients before surgery (23 percent versus 22 percent; p > 0.5). After the surgical procedures, there was a nonsignificant difference between both groups of patients in mean residual size of the gap in cases of velopharyngeal insufficiency (7 percent versus 8 percent; p > 0.5). It seems that minimal incision palatopharyngoplasty is a safe and reliable procedure for palatal closure in patients with submucous cleft palate. The use of additional individualized velopharyngeal surgery performed simultaneously did not seem to decrease the frequency of residual velopharyngeal insufficiency. Moreover, the residual size of the gap at the velopharyngeal sphincter was not significantly reduced when an additional surgical procedure was performed simultaneously with palatal closure.