Clinical outcomes of the furlow z-plasty for primary cleft palate repair

Objective: To review the clinical outcomes following the Furlow Z-plasty for primary cleft palate repair. The primary objective was to determine if the presence of an associated sequence or syndrome (i.e., Pierre Robin sequence), age at palate repair, cleft type, or surgeon experience influenced speech outcomes after a Furlow Z-plasty. Design: The outcomes of 140 patients who underwent palate repair were analyzed retrospectively. Speech evaluations were performed to score the severity of hypernasality, nasal escape, articulation errors, and velopharyngeal insufficiency. Results: The mean age at latest evaluation was 4 years 9 months (age range 2 years old to 12 years old and 4 months). Of the 140 patients, 83% had no evidence of hypernasality, 91% had no presence of nasal escape, and 69% had no articulation errors. Overall, 84% of patients had no evidence of velopharyngeal insufficiency. Secondary posterior pharyngeal flap to correct velopharyngeal insufficiency was required in only 2.1% of patients. The formation of an oronasal fistula occurred in only 3.6% of patients. Nonsyndromic patients with Pierre Robin sequence and syndromic patients did just as well as purely nonsyndromic patients in terms of velopharyngeal insufficiency, hypernasality, and nasal escape. Syndromic patients were more likely to make mild-to-moderate articulation errors. In addition, age at palate repair, cleft type, and surgeon experience had no statistically significant effect on speech results. Conclusions: The Furlow Z-plasty yielded excellent speech results in our patient population with minimal and acceptable rates of fistula formation, velopharyngeal insufficiency, and the need for additional corrective surgery.     

Two-stage palatoplasty using a modified Furlow procedure

A two-stage palatal repair using a modification of Furlow palatoplasty is presented. The authors investigate the speech outcome, fistula formation and maxillary growth. In a prospective, successive cohort study, 40 nonsyndromic patients with wide cleft palate were operated on between March 2001 and June 2006 by a single surgeon. 10 patients in the first cohort underwent a Furlow palatoplasty (control group). In 30 patients in the second cohort a unilateral myomucosal cheek flap was used in combination with a modified Furlow palatoplasty (study group). The hard palate was closed in both groups 9–12 months later. The Bzoch speech quality score was superior in the study group, and the hypernasality was significantly reduced in the study group. Overall fistula formation was 0%. At the time of hard palate reconstruction palatal cleft width was significantly reduced. Relative short-term follow up of maxillary growth was excellent. There were no postoperative haematomas, infections, or episodes of airway obstruction. This technique is particularly encouraging, because of better speech outcome, absence of raw surfaces on the soft palate, no fistula formation, and good maxillary growth. Further follow-up is necessary to determine the long-term effects on facial development.     

Management of cleft palate among patients with Pierre Robin sequence

Pierre Robin Sequence (PRS) is a congenital craniofacial anomaly distinguished by the presence of micrognathia, glossoptosis, and upper airway obstruction. Cleft palate occurs in over 3/4 of patients with PRS. The wide U-shape cleft and airway dysfunction create challenges in clinical management. Currently, disputes exist on the treatment protocol and prognosis of cleft palate management among patients with PRS. This review is focused on the deformity features, intervention timing, technique selection, airway support, and outcome evaluation of cleft palate among patients with PRS, aiming to provide reference to further evolution in the management of PRS-related cleft palate.     

Using a modified nutrition plate for early intervention in a newborn infant with Pierre Robin sequence: A case report.

Pierre Robin sequence is a triad of micrognathia, glossoptosis, and cleft palate that results in upper airway obstruction and feeding problems. It is a serious condition with potentially severe, life-threatening airway obstruction. Approaches for treatment of airway obstruction among neonates with Pierre Robin sequence include prone positioning of the infant, tongue-lip adhesion, mandibular distraction, and tracheostomy. This case report describes a modified nutrition plate and a baby treated using this appliance.     

Furlow palatoplasty to restore velopharyngeal competence following tonsillectomy avulsion of a pharyngeal flap.

OBJECTIVE: We report the successful use of a Furlow palatoplasty to salvage velopharyngeal competence following iatrogenic avulsion of a pharyngeal flap that had been previously established to treat velopharyngeal insufficiency associated with a submucous cleft palate. INTERVENTION: A tonsillectomy, conducted by a surgeon unaffiliated with a cleft palate team, was used to remove enlarged tonsils that had developed after pharyngeal flap surgery and extended into the lateral ports causing nasal obstruction and hypernasality because of mechanical interference with port closure. A posttonsillectomy evaluation revealed avulsion of the pharyngeal flap, which was successfully treated using a Furlow palatoplasty. CONCLUSIONS: To our knowledge, this is the first report of iatrogenic avulsion of a pharyngeal flap caused by tonsillectomy. Based on a review of the literature and this case experience, we would conclude that tonsillectomy should not be regarded as a routine procedure in patients previously treated with a pharyngeal flap. If required, it should be performed by a skilled otolaryngologist, preferably one affiliated with a multidisciplinary cleft palate team who is familiar with pharyngoplasty surgery. Finally, our experience would suggest that the Furlow palatoplasty is sufficiently robust to be used as a secondary salvage procedure to restore velopharyngeal sufficiency following iatrogenic avulsion of a pharyngeal flap.     

Transmandibular K-wire in the management of airway obstruction in Pierre Robin sequence

The Pierre Robin sequence was first described by Pierre Robin in 1923 as a triad of micrognathia, U-shaped cleft palate, and glossoptosis. Although the problems associated with Pierre Robin sequence may be numerous, the most acute problem in affected newborns is upper airway obstruction. The causes of upper airway obstruction are heterogeneous, and treatment is controversial and may include a long stay in an intensive care setting. Most patients could be treated conservatively with prone/lateral positioning and close clinical observation. A more aggressive approach is to hold the tongue forward surgically by a lip-tongue adhesion (glossopexy) technique, and if all else fails, it might be necessary to perform a tracheostomy. The purpose of this article is to report an unconventional technique for the management of airway obstruction in Pierre Robin sequence. A transmandibular K-wire was used in two patients with Pierre Robin sequence to prevent airway obstruction. The author was not the surgeon who placed the K-wire, and in one of the patients, the K-wire was retained for 4 years before being removed by the author. Both patients had surgical intervention to manage the airway problem in the form of lip-tongue adhesion in addition to the transmandibular K-wire. In conclusion, the value of using a transmandibular K-wire in the two cases presented here could not be determined and was questionable.     

Furlow腭成形术对腭咽闭合功能的影响

目的：研究腭裂患儿经Furlow腭成形术后软腭长度,软腭厚度以及腭咽腔深度的变化,探讨Furlow腭成形术在促进腭咽闭合功能中的作用。方法：2002年11月至2006年11月运用Furlow腭成形术完成不完全性腭裂或隐性腭裂患者45例,术前术后测量软腭长度,软腭厚度和腭咽腔深度。采用SPSS10.0软件包进行成对样本检验。P〈0.05定义为有显著统计学差异。结果：术前术后软腭长度;软腭厚度和腭咽腔深度分别做成对样本＂T＂检验,结果P〈0.01,均有显著统计学差异。结论：Furlow腭成形术延长了软腭长度,增加了软腭厚度,并使腭咽腔的深度变窄。对手术后腭咽闭合功能的恢复具有促进作用。     

Longitudinal follow-up of obstructive sleep apnea following Furlow palatoplasty in children with cleft palate: a preliminary report.

OBJECTIVE: To longitudinally investigate the incidence and severity of obstructive sleep apnea (OSA) following Furlow palatoplasty for velopharyngeal insufficiency (VPI) in children with cleft palate. SUBJECTS: Ten children, six boys and four girls, mean age 5.1 years, at Furlow palatoplasty. DESIGN: Prospective analysis. MAIN OUTCOME MEASURES: Overnight polysomnographic studies were used to determine the incidence and severity of sleep apneas 1 day prior to Furlow palatoplasty, 1 week postoperatively, and approximately 3 and 6 months postoperatively. RESULTS: None of the patients suffered OSA prior to Furlow palatoplasty. A high incidence of mild OSA (100%) occurred during the early postoperative period (p <.001) but resolved within 3 months in all but two patients (20%). Only one OSA (10%) persisted 6 months postoperatively. CONCLUSIONS: Furlow palatoplasty for VPI in children with cleft palate might induce temporary and mild OSA.     

Fast and early mandibular osteodistraction (FEMOD) in severe Pierre Robin Sequence

Pierre Robin Sequence (PRS) is a congenital abnormality characterized by mandibular hypoplasia, glossoptosis and often secondary palate cleft.It may be an isolated or part of a most complicated syndrome. The genetic syndrome that most frequently co-occurs is Stickler syndrome characterized by skeletal abnormalities, joint pain, congenital myopia and retinal detachment.The authors describe their fast and early mandibular osteodistraction (FEMOD) protocol in severe cases of PRS airway obstruction.     

Pierre Robin综合征低龄患者行腭裂修复术围手术期的风险评价

目的 评价Pierre Robin综合征低龄患者施行腭裂修复术围手术期的风险，并探讨控制风险的手段。方法 2001年5月-2004年2月北京大学口腔医学院收治Pierre Robin综合征患者共6例，均由同一名颌面外科医师采用von Langenbeck术式进行腭裂修复，围手术期行血氧监测，术前及术后第4天行多导睡眠仪监测。结果 所有患者均在麻醉插管时出现不同程度的低氧血症，只有1例在术后2h内出现了呼吸困难伴严重的低氧血症。睡眠监测结果显示，患者手术后睡眠呼吸紊乱的程度比术前并无明显加重。结论 低龄PierreRobin综合征患者实施腭裂修复术围手术期的风险主要是严重的低氧血症，经过全面的术前风险评价及严格的风险控制，多数患者在低龄阶段由经验丰富的医师进行腭裂修复手术是安全的。     

Pierre Robin sequence with esophageal atresia and congenital radioulnar synostosis.

A wide spectrum of anomalies can be associated with Pierre Robin sequence. This report presents a 3-day-old infant with micrognathia, U-shaped cleft palate, low-set right ear with microtia, glossoptosis, esophageal atresia, and right congenital radioulnar synostosis. The association of congenital radioulnar synostosis and esophageal atresia with Pierre Robin sequence has not been previously described.     

Polysomnographic indications for surgical intervention in Pierre Robin sequence: acute airway management and follow-up studies after repair and take-down of tongue-lip adhesion

The major concern for neonates with Pierre Robin sequence is the stability of the airways. The accepted management has been close clinical observation followed by surgical intervention if the airway was felt to be unstable. Six newborns with this diagnosis were admitted for evaluation in a 7-month period. Each underwent transcutaneous oxygen and carbon dioxide monitoring in a resting state. Each infant then was evaluated with infant polysomnography to ascertain the presence of obstructive apnea. Pulse-oximetry was utilized to document the oxygen saturation of each child during the polysomnography. On the basis of these studies, four of the neonates required a tongue-lip adhesion to stabilize the airway. These four infants were reassessed with polysomnography postoperatively, prior to cleft palate repair, following palatoplasty, and after their tongue-lip adhesion was released. This method of evaluation allows early testing of the stability of the airway in a way that augments and confirms the clinical assessment of the infant, allowing appropriate surgical intervention when necessary. This method of evaluation also allows the safe prediction of airway stability following palatoplasty and release of the tongue-lip adhesion.     

The Furlow Z-plasty in two-staged palatal repair modifications and complications

OBJECTIVES: To report the modifications and complications of the Furlow palatoplasty for two-stage closure of the palate. PATIENTS AND METHODS: Prospective study of a consecutive series of 45 primary closures of the soft palate portion of clefts extending into the hard palate; mean (S.D.) age at repair 12 (2) months; median follow-up 4 years 4 months (range 2 months to 9 years). The hard palatal part of the cleft was closed in 18 patients at the mean age of 3 years 11 months. RESULTS: The main modifications that we made were the use of quilting sutures, lateral V-Y closures, and fibrin glue application, and the omission of lateral releasing incisions. Patients stayed in hospital for a median of 4 days (range 3-8 days). Two patients had postoperative partial obstruction of the airway and were given steroids. In six patients, a smaller portion of the oral layer of the wound broke down; it healed by secondary intention in five, but resulted in partial dehiscence in one. There were no oronasal fistulas in the 18 patients who had delayed closure of the hard palate part of the cleft. Secondary pharyngoplasty was not necessary in any patient. CONCLUSION: Furlow's technique has been modified for use in the two-stage closure of complete cleft palates (with or without cleft lip or alveolus) with an acceptable rate of complications.     

Sommerlad–Furlow modified palatoplasty: A retrospective study

The purpose of this study was to introduce the surgical process of Sommerlad–Furlow modified (S–F) palatoplasty and compare its surgical and functional outcomes with conventional Sommerlad (S) palatoplasty.Patients with non-syndromic cleft palate who had undergone either S–F palatoplasty or S palatoplasty were retrospectively reviewed. Data on the outcomes of velopharyngeal function and postsurgical palatal fistula incidence were collected for all patients. Data for preselected factors, including gender, age at palatoplasty, and cleft type, were also collected. Chi-square tests were conducted.1254 patients were included. The postsurgical velopharyngeal competence (VPC) rate after S–F palatoplasty was significantly higher than after S palatoplasty (total, 70.5% vs 57.9%, p < 0.0001; age ≤ 1, 87.0% vs 69.2%, p < 0.0001; 1 < age ≤ 2, 78.3% vs 69.3%, p = 0.0479). With regard to different types of cleft palate, the postsurgical VPC rates after S–F palatoplasty were all significantly higher than for S palatoplasty in all patients younger than 2 years of age (complete cleft palate, 78.7% vs 62.4%, p = 0.0016; hard and soft palate cleft, 84.4% vs 74.8%, p = 0.0172; submucosal cleft and soft palate cleft, 96.6% vs 68.4%, p = 0.0114). The postoperative fistula rate after S–F palatoplasty was 4.3%.This modified palatoplasty technique provided adequate cleft palate closure, with satisfactory speech outcomes and low fistula rates, while older age at palatoplasty may affect the postsurgical outcomes. Within the limitations of the study it seems that the Sommerlad–Furlow modified technique is an option for cleft palate repair.     

Syndromes 15. Robin sequence

Robin sequence (RS) is a congenital anomaly that includes micrognathia, U-shaped cleft palate, and upper airway obstruction. More than 80% of children with RS have other malformations as a result of associated congenital anomalies. This report briefly reviews some multidisciplinary aspects, including etiopathology, diagnosis and treatment.     

An effect comparison between Furlow double opposing Z-plasty and two-flap palatoplasty on velopharyngeal closure

The aim of this study was to compare velopharyngeal closure between patients who underwent Furlow palatoplasty and two-flap palatoplasty. A retrospective review of 88 patients with incomplete palate cleft was performed. 48 patients (17 males; 31 females) aged 2-28 years received Furlow palatoplasty. 40 patients (17 males; 23 females) aged 2-21 years received two-flap palatoplasty. Velopharyngeal function was categorized as adequate, marginal or inadequate. Complications associated with the operation were documented. Statistically significant differences were not found amongst sex distribution, age at operation, follow-up time, and preoperative speech intelligibility. After primary repairs using Furlow and two-flap palatoplasty, the surgeon's incidence of postoperative palatal fistula was 0%. The complications were not significantly different between the two groups. The authors achieved the lowest reported incidence of postoperative palatal fistulas in primary Furlow palatoplasty. The outcomes of the velopharyngeal closure were better in patients who received Furlow palatoplasty (P<0.05). Furlow palatoplasty was more effective than two-flap palatoplasty in obtaining perfect velopharyngeal closure. A probable explanation may be that Furlow palatoplasty can reposition and overlap the divergent palatal muscle and lengthen the soft palate.     

Comparison of speech outcomes using type 2b intravelar veloplasty or furlow double-opposing Z plasty for soft palate repair of patients with unilateral cleft lip and palate

BackgroundThe aim of this study is to compare speech outcomes, fistula rates, and rates of secondary speech surgeries after palatoplasty using Furlow palatoplasty or type 2b intravelar veloplasty for soft palate repair.Patients and methodsPatients with unilateral cleft lip and palate who had either Furlow palatoplasty or intravelar veloplasty for soft palate repair were retrospectively evaluated for demographic and perioperative variables and speech outcomes. Fistula rate, secondary surgical intervention for improved speech results, and findings of speech assessment were further reviewed for the patients who met the inclusion criteria.ResultsA total of 76 patients, 36 in the Furlow palatoplasty group and 40 in the intravelar veloplasty group, were included in the study. In the speech assessment, nasalance values were statistically similar between the two groups. Also, there was no statistically significant difference between the groups in velopharyngeal motility (p = 0.103). The total rates of secondary surgeries and fistula were statistically similar between the groups (p = 0.347 and 0.105, respectively).ConclusionThe similar outcomes of speech and surgical evaluation between the two groups make the surgeon's preference determinant in the selection of the surgical technique for soft palate repair.     

Preoperative pharyngeal depth is associated with postoperative velopharyngeal function following primary cleft palate repair at the age of five and above

To determine prognostic factors for postoperative velopharyngeal function following the primary cleft palate repair at the age of five and above.This study reviewed patients with cleft palate who had undergone Furlow palatoplasty at age 5 or older from 2009 to 2014. We obtained intraoperative measurements, including velar length, pharyngeal depth, cleft width, maxillary width, cleft palate index (cleft width/maxillary width) and palatopharyngeal ratio (velar length/pharyngeal depth), as well as speech evaluation results at least 1 year after surgery. Logistic regression and retrospective analyses were performed to determine factors associated with speech performance after the primary cleft palate repair.Among the six intraoperative measurements of velopharyngeal morphology, only pharyngeal depth was incorporated into the regression model, and was found to have an inverse association with postoperative velopharyngeal function, Exp (B) 0.883 (95% CI 0.798-0.976). Moreover, a pharyngeal depth greater than 16 mm was significantly associated with a higher risk of postoperative velopharyngeal insufficiency (P < 0.01).Pharyngeal depth is potentially a prognostic indicator for the primary management of cleft palate in older patients. Pharyngoplasty may need to be considered when the pharyngeal depth is large and the patient’s access to surgery is limited.     

Feeding plate for a neonate with Pierre Robin sequence

Pierre Robin Sequence is a congenital condition involving a combination of micrognathia and glossoptosis with or without a cleft palate. Feeding problems are often associated with cleft anamoly which make it difficult for the infant to maintain adequate nutrition. Here we present a case of 58-day-old neonate with Pierre Robin sequence, in whom we contructed a palatal obturtor for feeding.     

A feeding obturator for a preterm baby with Pierre Robin sequence

The article describes a clinical and laboratory technique for the fabrication of a feeding obturator for a baby with Pierre Robin sequence. Emphasis is placed on the direct fabrication of a preliminary custom tray, preventing thermal trauma to the tissues, and overcoming the danger of airway obstruction or foreign body aspiration. The functional problems associated with a cleft palate and various methods to overcome them are also discussed.