Dandy Walker Syndrome with Tessier 7 Cleft-a Rare Case Report and a Surgical Note on the Use of Vermilion Flap and Lazy W-Plasty

The Dandy-Walker syndrome is a malformation of the brain that involves the mal-development of the cerebellum, associated with a cystic enlargement of this area, and frequently hydrocephalus. This malformation occurs in ~1 in 30,000 babies. It is seen mostly in females. Developmental anomalies like cleft lip, cleft palate, and cardiac malformation, orthopaedic and urinary structural abnormalities may also occur in 30% of the individuals. We report a case of Dandy Walker syndrome with Tessier 7 facial cleft with paramedian cleft palate in a 6 month old child. Surgical methods used to correct this anomaly include commissuroplasty, myoplasty of the orbicularis oris, and closure of the cleft cheek. Authors report a vermilion square flap technique that combines a lower lip mucocutaneous vermilion border flap with a lazy W-plasty to ensure a natural commissure and skin closure.     

Craniofacial morphological outcome following treatment with three different surgical protocols for complete unilateral cleft lip and palate: a premilinary study

This study compared craniofacial morphology between three groups of children with complete unilateral cleft lip and palate, treated with different surgical protocols. The study included 66 10-year-old children (42 boys and 20 girls) with a complete unilateral cleft lip and palate (22 patients in each of the three groups). Children aged 7 months underwent one-stage surgery, performed by a single surgeon. During surgery, the soft and hard palate and the lip underwent correction. The difference between the groups depended on the hard palate closure. Group I patients had the mucoperiosteal flap elevated on both sides of the cleft. Group II patients had the mucoperiosteal flap elevated on the non-cleft side, and had only a minimal 2–3 mm mucoperiosteal flap elevated on the cleft side. Group III patients had mucoperiostium elevated from the septum vomer to create a single-layered caudally pedicled flap, and had only a minimal 2–3 mm palatal flap elevated on the cleft side. Craniofacial morphology was defined using lateral cephalometric analysis. Significant craniofacial morphological differences were identified between groups I, II and III. Group III demonstrated the most favourable morphology. This indicates that the technique of hard palate closure has significant influence on craniofacial growth and development.     

Early hard palate closure using a vomer flap in unilateral cleft lip and palate: effects on cleft width

Objectives

Although no universal consensus exists on treatment of cleft palates, early hard palate closure is commonly performed. The aim of the present study was to determine the influence of a vomer flap for early hard palate closure on residual palatal cleft width in patients with a unilateral complete cleft lip and palate (UCLP).

Materials and methods

Forty-seven UCLP patients were retrospectively divided into two groups. Group A consisted of 25 patients who underwent early lip closure and simultaneous hard palate closure using a vomer flap. Group B included 22 patients who had lip closure only at first surgery. Palatal cleft widths of both groups were measured at two time points and were compared using the Mann–Whitney U test to examine the influence of vomerplasty in this very early stage.

Results

No significant difference of baseline characteristics between the groups was found, and comparison of age at the time of surgeries was not significantly different. Mean age at the time of vomerplasty was 4.0 months. After the first surgery, a significantly greater total cleft width reduction of 5.0 mm average was found in group A compared to only 1.5 mm reduction in group B. This reduction took place after an average of 7.1 and 7.0 months, respectively.

Conclusions

Lip closure accompanied by early hard palate closure using a vomer flap is associated with a significant postoperative reduction of the residual cleft when compared to lip closure only.

Clinical relevance

This study shows another great advantage of performing early hard palate closure using a vomer flap.     

Vomer flap for hard palate repair is related to favorable maxillary growth in unilateral cleft lip and palate

Objective

Vomer flap repair is assumed to improve maxillary growth because of reduced scarring in growth-sensitive areas of the palate. Our aim was to evaluate whether facial growth in patients with unilateral cleft lip and palate was significantly affected by the technique of hard palate repair (vomer flap versus two-flap).

Materials and methods

For this retrospective longitudinal study, we analyzed 334 cephalometric radiographs from 95 patients with nonsyndromic complete unilateral cleft lip and palate who underwent hard palate repair by two different techniques (vomer flap versus two-flap). Clinical notes were reviewed to record treatment histories. Cephalometry was used to determine facial morphology and growth rate. The associations among facial morphology at age 20, facial growth rate, and technique of hard palate repair were assessed using generalized estimating equation analysis.

Results

The hard palate repair technique significantly influenced protrusion of the maxilla (SNA: β?=??3.5°, 95 % CI?=??5.2-1.7; p?=?0.001) and the anteroposterior jaw relation (ANB: β?=??4.2°, 95 % CI?=??6.4-1.9; p?=?0.001; Wits: β?=??5.7 mm, 95 % CI?=??9.6-1.2; p?=?0.01) at age 20, and their growth rates (SNA p?=?0.001, ANB p?<?0.01, and Wits p?=?0.02).

Conclusions

The results suggest that in patients with unilateral cleft lip and palate, vomer flap repair has a smaller adverse effect than two-flap on growth of the maxilla. This effect on maxillary growth is on the anteroposterior development of the alveolar maxilla and is progressive with age. We now perform hard palate closure with vomer flap followed by soft palate closure using Furlow palatoplasty.

Clinical relevance

These findings may improve treatment outcome by modifying the treatment protocol for patients with unilateral cleft lip and palate.     

单侧完全性唇腭裂患儿唇裂修复同期硬腭裂隙封闭的临床观察

目的　探讨单侧完全性唇腭裂患儿时唇裂修复同期硬腭裂隙封闭的可行性及临床效果。方法　47例年龄为3·0~7·5月龄的单侧唇腭裂患儿在唇裂修复同期行硬腭裂隙封闭,分析手术时间、术中出血、术后恢复、创口愈合及腭部裂隙变化情况。结果　所有患儿的手术均顺利完成。手术时间与单纯唇裂修复术相比平均延长13 min , 术中出血平均增加5 ml,术后恢复好,无创口感染及裂开。至患儿9~18月龄二期手术时腭部裂隙比行单纯唇裂修复术平均小0·28 cm,使二期手术时软腭后退充分,腭咽闭合良好。结论　单侧完全性唇腭裂患儿唇裂修复同期行硬腭裂隙封闭是安全和可行的。     

Speech evaluation and dental arch shape following pushback palatoplasty in cleft palate patients: Supraperiosteal flap technique versus mucoperiosteal flap technique.

BACKGROUND: The aim of this study was to evaluate and compare the maxillary dental arch shape and speech of cleft palate patients following pushback palatoplasty using either the supraperiosteal flap technique or the mucoperiosteal flap technique. PATIENTS: Sixty-two patients (29, cleft palate only; 33, unilateral cleft lip, alveolus and palate) operated on by the supraperiosteal technique and 47 patients (23, cleft palate only; 24 unilateral cleft lip, alveolus and palate) by the mucoperiosteal technique were reviewed in this study. Study design: Dental arch shape and speech proficiency at preschool and school age were evaluated in all patients. RESULTS: Dental arch shapes were classified as U type (good dental arch shape) and V type (narrow dental arch shape). In cleft palate only patients, U type was observed in 90% of the supraperiosteal group and 83% of the mucoperiosteal group. In unilateral cleft lip, alveolus and palate patients, U type was observed in 85% of the supraperiosteal group, while only in 33% of the mucoperiosteal group. In cleft palate only patients, normal speech at school age was observed 100% of the supraperiosteal group and 83% of the mucoperiosteal group. In unilateral cleft lip, alveolus and palate patients, normal speech at school age was observed in 97% of the supraperiosteal group and 75% of the mucoperiosteal group. Misarticulation was frequently found in patients with the V type of dental arch shape. CONCLUSION: It is suggested that pushback palatoplasty using the supraperiosteal technique is more advantageous for speech development compared with the mucoperiosteal technique.     

Modified VIP-CT flap in late maxillary alveolar cleft surgery

ObjectiveThis article describes a new application for the modified vascularized interpositional periosteal-connective tissue flap (modified VIP-CT flap) to solve problematic cases of late maxillary alveolar cleft bone grafting.MethodsThis study analysed the ability of the VIP-CT flap to provide coverage of bone grafts in maxillary alveolar cleft surgery. Donor site morbidity was also evaluated.ResultsThirteen patients were operated on using this technique, seven patients were female, the age range was between 12 and 25 years, and all of them were missing at least one permanent anterior tooth adjacent to the cleft. Two patients had bilateral alveolar clefts. In most of the operated patients one VIP-CT flap was used, but in two patients (cases 3 and 4) bilateral VIP-CT flaps were used for treatment of wide alveolar clefts. In case 3 both of the flaps were used for closure of the oral side and in case 4 one flap was used for nasal closure and another for oral side coverage. Two patients had alveolar cleft only with normal palatal anatomy, and the remaining patients had previously operated cleft palates (11 patients).ConclusionVIP-CT flaps can readily be used in adult patients with cleft lip (alveolar only) and in many operated unilateral and bilateral complete cleft lip and palate patients successfully.     

Elongated pharyngeal flap in extensive clefts of the hard and soft palate

In some cases of extensive palatal defects surgical closure may be regarded as unfeasible, and the condition treated with an obturator prosthesis. In such a case the cleft can be closed in one operation by means of a pharyngeal flap elongated through a pharyngotomy according to Bengt Johanson (1966). Eleven patients who had used obturators were operated on between 1957 and 1978. The mean age of the patients was 39 years. All patients were cleft lip and palate or cleft palate cases; two unilateral, five bilateral, and four with an isolated cleft palate. In most of these patients a temporary tracheostomy was performed after which the pharynx was opened through a neck incision. A flap was created which reached the alveolar ridge. For oral closure, mucoperiosteal flaps were used. Anterior palatal fistulas developed in two cases; one closed spontaneously and the other remained as a 3-mm fistula behind the alveolar ridge. Three patients had postoperative transient dysphagia. Phoniatric evaluation showed that two patients had better speech after operation than before with an obturator. Gross speech improvement at this late age should not be expected and is not the primary goal of the procedure. The aim of surgical closure with an elongated pharyngeal flap is to replace the obturator.     

Dento-alveolar development in unilateral cleft lip, alveolus and palate.

BACKGROUND AND OBJECTIVE: Palatal surgery for cleft lip, alveolus and palate is considered to have the most powerful negative impact on maxillary growth. The aim of this study was to compare dento-alveolar development of the permanent dentition and morphology of the palate after surgery in unilateral cleft lip, alveolus and palate patients following two types of palatoplasty: supraperiosteal flap vs mucoperiosteal flap technique.PATIENTS: Thirty-eight patients born between 1976 and 1983 with a complete unilateral cleft of lip, alveolus and palate were studied. Fifteen patients were treated with supraperiosteal flaps (SP group), and the other 23 patients with mucoperiosteal flaps (MP group). In this cross-sectional study, dental casts of stage IV A of Hellman's dental age in each patient were used. METHODS: The following distances were measured: (1). transverse distance C-C', (2). transverse distance M-M', (3). palatal length, (4). palatal height. RESULTS: No statistically differences were seen between the SP and MP groups regarding C-C' and M-M'. However, palatal length and palatal height were significantly greater in the SP than in the MP group. CONCLUSION: The technique that leaves no denuded palatal bone is considered to be advantageous for the development of the alveolar process.     

One-stage closure of uni- and bilateral cleft lip and palate

A preliminary report of an ‘all-in-one’ one-staged closure of all forms of cleft lip and palate during the first year of life. The one-stage repair of complete uni- and bilateral clefts includes the anatomical reconstruction of soft palate, hard palate closure in two layers, alveoloplasty with bone grafting and lip repair. This surgical technique is described and early results presented.     

Sucking efficiency of early orthopaedic plate and teats in infants with cleft lip and palate

Intraoral negative pressure during bottle feeding with two kinds of teats (a regular Nuk and a cleft Nuk) was measured in 7 infants with cleft lip and palate, 8 infants with cleft palate, 2 infants with cleft lip, 4 infants with operated cleft lip and palate and 7 normal infants. Infants with cleft lip and palate or cleft palate were unable to generate negative pressure before cleft lip and palate closure. The presence or absence of an early orthopaedic plate did not make any difference. In infants with unoperated cleft lip and with operated cleft lip and palate, peak negative pressure during feeding differed little from that of normal infants.     

Influence of vomer flap on craniofacial growth in patients with cleft lip and palate: A systematic review

The aim of this review was to evaluate the impact of the vomer flap on craniofacial growth in patients with cleft lip and palate. The review was conducted according to the PRISMA checklist and is registered in the International Prospective Register of Systematic Reviews (PROSPERO — CRD42018095714). Two investigators performed the research using the PubMed/MEDLINE, Embase, and Web of Science databases for studies published until November 2018. The focused question was ‘Does the vomer flap have a lesser impact on craniofacial growth in patients with cleft lip and palate?’. A total of 13 articles was selected for this review, comparing the vomer flap technique with other flap surgery techniques. The outcomes analyzed were: facial development (primary outcome); and the growth of the maxilla and mandible, occlusion, occurrence of fistula, and speech development (secondary outcomes). It was concluded that there is no difference in impact between vomer flap and the other flap surgery techniques on craniofacial development.     

The buccal flap--a useful technique in cleft palate repair?

OBJECTIVE: To evaluate speech quality and oronasal fistula after primary palate repair using a buccal mucosal flap. DESIGN: Retrospective study cohort of patients with cleft palate. SETTING: Primary care center for treatment of craniofacial congenital anomalies. PATIENTS AND METHODS: One hundred fifty-six nonsyndromic patients underwent palatoplasty with the buccal myomucosal flap by the senior surgeon between 1989 and 2002. The preoperative workup, surgical technique, and other factors that might affect the outcome were identical in every case. Oronasal fistula and variables affecting speech quality were analyzed. RESULTS: The most common type of cleft was unilateral cleft lip and palate (43.5%). The median follow-up was 5.8 years (0.4 to 21 years), and the median age at repair was 6.2 months. The overall fistula formation was 3.6%, decreasing progressively: 1989 to 1994: 2.9%, 1995 to 2002: 0.7% (p <.05). Velopharyngeal incompetence (VPI) occurred in 8.8% of the patients, decreasing from 5.3% to 3.5% in the last years. VPI and oronasal fistulae were observed mainly in unilateral and bilateral clefts of the lip and palate. Velopharyngeal adequacy occurred in 91.1% of the children, and resonance was normal in 91.1 %. None of the patients had severe hypernasality or hyponasality. Articulation was normal in 97.9% of the children. Speech quality was good in 89% of the patients. CONCLUSIONS: The technique presented has been effective, with the advantages of palatal closure without tension, good muscular reconstruction, lengthening of the nasal layer, and palatal closure without raw areas. The technique, early repair, and surgeon's skills were the most important variables for good outcomes regarding speech and fistula formation.     

Facial growth following pharyngeal flap surgery: skeletal assessment on serial lateral cephalometric radiographs

The purpose of this study was to evaluate the effect of pharyngeal flap surgery on subsequent facial growth in patients with cleft lip and/or palate. Pharyngeal flap surgery is used in such patients to partially obliterate the velopharyngeal port, reducing hypernasal speech. Thirty-four patients (18 with cleft palate only, 16 with unilateral cleft lip and palate) were selected from the longitudinal growth study of the H.K. Cooper Clinic. Seventeen of these (9 with cleft palate only, 8 with unilateral cleft lip and palate) underwent pharyngeal flap surgery between the ages of 5 and 7 years. The other seventeen patients did not undergo pharyngeal flap surgery and served as a control group for this study. Serial lateral cephalometric radiographs were traced and digitized (ages 3 to 5, preflap; ages 7-10, postflap). Fourteen skeletodental measurements (six angular, six linear, two derived) were taken to determine whether pharyngeal flap surgery may be related to subsequent facial growth changes. The data from the 17 flap patients were compared with control data taken from the other seventeen patients. The groups were matched for sex, cleft type, and similarity of presurgical mandibular growth direction (facial axis angle). Results obtained demonstrate several significant areas of change following flap surgery, including a decrease in facial axis angle, an increase in Frankfort-mandibular plane angle, an increase in incremental gains in lower anterior face height, and increased retroclination of upper and lower incisors in the flap group as compared to their matched controls.     

Primary repair of the alveolar cleft

Nasoalveolar fistula and oropharyngeal fistula of the anterior palatal region are very commonly seen in cases when there are concomitant clefts of the lip and the palate. Absence of adequate tissue in that region complicates the treatment and necessitates new tissue transfers from near or distant tissues. Today, the techniques used for correcting cleft lip cannot successfully solve these 2 problems. In this study, we describe a technique that depends on the principle of using the lip mucosal tissues that remains during the Tennison cleft lip correction technique, with a flap designation, to correct the tissue defect of the cleft between the foramen incisivum and lip and the alveolar region. Twenty-two patients (13 boys and 9 girls), with ages ranging from 3 to 53 months (mean, 24 mo), with unilateral cleft lip and palate underwent surgery with this new technique. In all these patients, clefts in the anterior palatal and alveolar regions were successfully corrected. Fistula was observed in none of these patients in these regions. Through this method, clefts in the anterior palatal and alveolar regions can be corrected during repair of cleft lips.     

Results of randomized controlled trial of soft palate first versus hard palate first repair in unilateral complete cleft lip and palate.

OBJECTIVE: To compare the outcomes for primary repair of unilateral cleft lip and palate, operating on the soft palate first versus the hard palate first. DESIGN: Randomized controlled trial. SETTING: The Regional Cleft Service of West Nepal. PATIENTS: Forty-seven consecutive patients with nonsyndromic unilateral cleft lip and palate, of whom 37 were assessed 4 to 6 years after completing primary surgical repair. INTERVENTIONS: Primary repair of unilateral cleft lip and palate by two differing sequences: (1) soft palate repair, with hard palate and lip repair 3 months later; and (2) lip and hard palate repair, followed by the soft palate repair 3 months later. MAIN OUTCOME MEASURES: Analysis of dental study models, weight gain, and speech recordings. RESULTS: Four to 7 years after completing the cleft closure, there was no significant difference in facial growth between the two types of repair sequencing. Completing posterior repair first had no effect on anterior alveolar gap width. It narrowed the hard palate gap by reducing the intercanine distance. Anterior repair dramatically closed the anterior alveolar gap, and narrowed the intercanine distance. Comparing anterior alveolar gap width with age at first presentation demonstrated that there was no spontaneous narrowing of the cleft in older children. Completing posterior closure first had a weight gain advantage over anterior closure first. Improved oropharyngeal closure, and thus swallowing, is the likely explanation. CONCLUSION: Changing the sequencing of cleft closure has no demonstrable difference in facial growth at 4 to 7 years after completion of the primary surgery.     

Dental arch relationships following palatoplasty for cleft lip and palate repair

Palatal scarring is assumed to be a primary cause of facial growth derangement in cleft lip and palate. Evidence supporting this hypothesis is confounded by the clinical involvement of various surgeons, and therefore definitive conclusions are not possible. In this study, we investigated the dental arch relationship in two groups, Exposed (47 children; 11.2 yrs) and Unexposed (61 children; 11.2 yrs), with a unilateral cleft lip and palate operated on by the same surgeon. The technique of hard palate repair differed between the two groups. In the Exposed group, palatal bone of the non-cleft side only was left denuded, inducing scar formation. In the Unexposed group, a vomerplasty with tight closure of the soft tissues was applied. Three raters graded the dental arch relationship and palatal morphology using the EUROCRAN Index. The dental arch relationship in the Exposed group was less favorable than in the Unexposed group (p = 0.009). Palatal morphology in both groups was comparable (p = 0.323). This study demonstrates that reduction of denuded bony areas on the palate after palatal repair with a vomer flap had a favorable effect on the dental arch relationship. For palatal morphology, no effect of the type of palatal repair was found.     

Dentocraniofacial morphology of 21 patients with unilateral cleft lip and palate: a cephalometric study.

OBJECTIVE: To assess the skeletal and dental craniofacial proportions of unilateral cleft lip and palate patients who were operated upon using the Malek technique, and compare them with a normal group to highlight the effect of surgical correction on craniofacial development during growth. DESIGN: Retrospective. METHODS: The cleft palate was closed using the Malek technique in a single operation at 3 months for 11 patients (complete closure of lip and palate) and in a two-stage operation for 10 patients (soft palate at 3 months, lip and hard palate at 6 months). Comparisons were made with a normal control group. Angular and linear measurements of anterior and posterior dimensions of the upper and lower compartments of the face were measured in the 7th and 12th years. RESULTS AND CONCLUSION: No significant differences were observed between the two groups of palate technique repair, although significant differences were observed between craniofacial dimensions of normal versus cleft lip and palate patients. At a skeletal level, the maxilla and mandible were retrusive relative to the cranial base in the cleft lip and palate group. In fact, there was a backward rotation of the palatal plane with repercussions on the maxillo-mandibular complex position. Furthermore, the maxilla was shorter than in normal patients, whereas the mandible was normally shaped. The upper incisors were retroclined and they locked the lower incisors in linguoversion. There was a posterior skeletal deficit of the respiratory compartment, compensated by more marked posterior maxillary alveolar growth. Facial growth in cleft lip and palate patients followed the same pattern, but was delayed compared with normal patients.     

Microsurgical tissue transfer for rehabilitation of the patient with cleft lip and palate.

OBJECTIVE: Three case reports of microsurgically revascularized tissue transfer for secondary closure of complex oronasal fistulae in cleft lip and palate patients are reported. One scapular and two radial forearm flaps were used in that respect; the scapular flap was transferred without a skin paddle and was left for secondary epithelialization whereas iliac crest bone was transplanted in the two patients with the forearm flaps in a further surgical step. CONCLUSIONS: These microsurgical flaps represent solutions in selected cases of oronasal fistulae in patients with cleft lip and palate with extensive scarring, large defects, or both. Alternative free flaps of the vast spectrum available today, however, also deserve consideration.     

Long-term evidence for favorable midfacial growth after delayed hard palate repair in UCLP patients.

OBJECTIVE: To investigate whether delayed hard palate repair resulted in better midfacial growth in the long term than previously achieved with "conventional" surgical methods of palatal closure. DESIGN AND SETTING: Long-term cephalometric data from patients with unilateral cleft lip and palate were available from two Scandinavian cleft centers. The patients had been treated by different regimens, particularly regarding the method and timing of palatal surgery. Patients were analyzed retrospectively, and one investigator digitized all radiographs. PATIENTS: Thirty consecutively treated subjects from each center, with cephalograms taken at three comparable stages between 10 and 16 years of age. RESULTS AND CONCLUSIONS: Patients whose hard palates were repaired late (early soft palate closure followed by delayed hard palate repair at the stage of mixed dentition) had significantly better midfacial development than patients in whom the hard palate was operated on early with a vomer flap, and then during the second year of life, the soft palate was repaired with a push-back procedure. As the growth advantage in the delayed hard palate repair group was accomplished without impeding long-term speech development, the delayed repair regimen proved to be a good alternative in surgical treatment of patients with unilateral cleft lip and palate.