Journal of Plastic, Reconstructive & Aesthetic Surgery (2015) 68, 1095e1099
Do patients with isolated Pierre Robin Sequence have worse outcomes after cleft palate repair: A systematic review* Teng Wan a,b, Yang Chen a,b, Guomin Wang a,b,* a Department of Oral and Maxillofacial Surgery, Ninth People’s Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai 200011, PR China b Shanghai Key Laboratory of Stomatology, Shanghai 200011, PR China
Received 15 October 2014; accepted 13 April 2015
KEYWORDS Pierre Robin Sequence; Cleft palate repair; Outcome; Cleft palate-only; Systematic review
Summary Cleft palate repair for Pierre Robin Sequence (PRS) patients has always been a challenge for surgeons and anesthetists. The aim of this systematic review is to investigate the outcome of cleft palate repair for PRS patients compared with cleft palate-only patients. All papers published before October 2014 were searched in the databases PubMed and MEDLINE. Search terms included “Pierre Robin Sequence,” “cleft palate repair,” and “speech result.” Additional studies were identified by hand searching the reference lists of the papers retrieved from electronic search. Two independent reviewers assessed the eligibility of studies for inclusion, extracted the data, and assessed the quality of the studies. Six studies met the inclusion criteria. All but one study had multiple deficiencies in study designs. Four studies assessed the fistula rate of both groups, and all studies assessed some aspect of the speech results. Conflicting results and a lack of high-quality and long-term outcomes of reviewed studies provided no conclusive scientific evidence about whether the outcome of cleft palate repair for PRS patients was better or worse than cleft palate-only patients. Further well-designed, well-controlled, and long-term studies are needed. ª 2015 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.
*
This study has not been presented at any meeting, wholly or in part. * Corresponding author. Department of Oral and Maxillofacial Surgery, Ninth People’s Hospital, School of Medicine, Shanghai Jiao Tong University, No. 639 Zhi Zao Ju Road, Shanghai 200011, PR China. Tel.: þ86 021 23271699 5149; fax: þ86 021 53072423. E-mail address: [email protected] (G. Wang). http://dx.doi.org/10.1016/j.bjps.2015.04.015 1748-6815/ª 2015 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.
1096
T. Wan et al.
Introduction Pierre Robin Sequence (PRS) is a congenital condition characterized by micrognathia, glossoptosis, airway obstruction, and often U-shaped cleft palate (Figure 1). It was first described by the French stomatologist Pierre Robin in 19231 and 1934.2 Infants with this condition usually suffer from airway problems and subsequently feeding difficulties, pulmonary infection, failure to thrive, and sometimes even death. In literature, there are many different names for this condition, such as Pierre Robin Syndrome, complex, or anomalad. Whether cleft palate is an obligatory feature of PRS remains debated. A survey3 of 73 cleft and craniofacial providers yields 14 different definitions of PRS, which shows the variability in the definition of this well-known eponym. Thus, the incidence of the condition varies from one per 8500 to one per 20,000 live births.4 Different diagnoses not only influence epidemiological and outcome studies but also make it very difficult to compare the results of different studies. The fact that PRS can occur isolated or in syndromic form further influences the situation. Most of the studies on PRS have focused on the airway management in the neonatal period.5e7 Little information is available about the outcomes after palatoplasty in this subset of patients with cleft palate. Protocols for the management of PRS vary by surgeons and institutions. The outcome studies of cleft palate repair are few, and they show conflicting results regarding the oronasal fistula rates and long-term speech results. The purpose of this study is to systematically review whether patients with isolated PRS have worse outcomes after cleft palate repair compared with cleft palate-only (CPO) patients. The outcomes include oronasal fistula rate, other complications, and long-term speech results.
Methods Study selection To find the relevant articles appropriate for this review, a search was conducted according to the following search strategy: (1) The electronic database MEDLINE (via PubMed) from 1966 to October 2014 was searched using a combination of the following keywords: “Pierre Robin Sequence,” “cleft palate repair,” and “speech result; ” and (2) the reference lists of all relevant publications were reviewed to identify any publications not already identified using the first search strategies. The inclusion criteria were as follows: 1. Studies that evaluated the outcomes of cleft palate repair for PRS patients versus CPO patients. 2. Only articles in English and Chinese were included.
Figure 1
U-shaped cleft palate in PRS patients.
Data extraction and quality check A standardized data extraction form was used to retrieve information of interest, including author, year of publication, study design, sample size, surgical technique, age at palate repair, age at speech evaluation, methods and measurements (Table 1), and results (fistula rate, resonance, articulation, and need for secondary treatment) (Table 2). The quality of included studies was documented by evaluating its quality using a modified NewcastleeOttawa Scale8 (Table 3). It was developed to assess the quality of nonrandomized studies with its design, content, and ease of use directed to the task of incorporating the quality assessments in the interpretation of meta-analytic results. The extraction of data and quality check were done by two authors independently without blinding. Conflicts between examiners were resolved by discussion of each paper to reach a consensus.
Results We retrieved 59 papers, which we analyzed according to the selection criteria. Six of these papers qualified for the review9e14 (Table 1). All studies are retrospective. All studies reviewed the fistula rate and some aspect of the speech result. The variables used in the six studies are shown in Table 2. The quality of each study is shown in Table 3. All but one study11 had multiple deficiencies in methods.
Fistula rate The exclusion criteria were as follows: 1. Studies that did not have a control group. 2. Cleft palate repair at >3 years of age. 3. Studies involving 3 years Fistula,a need for VPI 14.9 m treatment 14.2 m (12e18 m) >8 years Fistula, resonance, 12.5 m (11e14 m) velopharyngeal function, need for VPI treatment 12 m (6e78 m) 4.75 years Resonance, articulation, velopharyngeal function, need for VPI treatment 13 8.9 years Fistula, resonance, (8 monthse2.4 years) (5.3e11.8 years) velopharyngeal function, 12 m 7.83 years articulation, need for VPI (3 monthse9 years) treatment (5e18.5 years) 12.7 m 34.67 m Fistula, resonance, velopharyngeal function
ND: no data. a No data of the control group.
Resonance Of the six studies reviewed, five studies9,11e14 examined the resonance of both groups. Five studies9,11e14 examined the hypernasality of both groups: one study11 showed increased hypernasality in PRS group, and others found no difference between groups. Three studies9,13,14 examined
hyponasality of both groups; all found no difference between groups. Three studies9,11,12 examined the nasal emission of both groups; one study9 showed increased nasal emission in PRS group. The results of study 513 were the final results after velopharyngeal insufficiency (VPI) treatment, which was different from other studies.
Velopharyngeal function Table 2
Variables in included studies.
Variable
19
210
311
412
513
614
Fistula rate Resonance Hypernasality Hyponasality Nasal emission Velopharyngeal function Articulation Minor Gross Constant intelligibility Need for VPI treatment
X e X X [ X e X X X X Xb
e e e e e e e e e e e [
X e [ e X Y e e e X e Xb
e
X e Xa Xa e Xa e e e e e X
X e X X e X e e e e e e
X e X X X e e e e e
[, increased magnitude in PRS group; Y, decreased magnitude in PRS group; X, no difference between groups. a The results were final speech outcomes after VPI treatment for both groups. b The indication for VPI treatment was not specified in the article.
Five studies9,11e14 examined velopharyngeal (VP) function of both groups: one study11 showed decreased VP function in PRS group, and the other four showed no significant difference between groups. The result of study 513 was the final result after VPI treatment.
Articulation Three studies9,11,12 examined articulation; one9 evaluated minor VP unrelated articulation errors, gross VP unrelated articulation errors, and constant VP-related errors, all of which showed no difference between groups. One study12 evaluated the compensatory articulation errors associated with VPI, and it found no difference between groups.
Intelligibility Only one study9 examined the intelligibility of speech. Both intelligibility in known and unknown contexts showed no significant difference between groups.
1098 Table 3
T. Wan et al. Quality analysis of included studies. 19
Selection
Comparability Exposure
Representativeness of the exposed cohort Selection of the non-exposed cohort Ascertainment of exposure Demonstration that outcome of interest was not present at start of study Study controls for associated syndromes Study controls for age at cleft palate repair Assessment of outcome Was follow-up long enough for outcomes to occur Adequacy of follow up of cohorts
* *
* * *
210
311
412
513
614
* *
* * * *
* * * *
* * * *
* *
* * * * *
*
* *
* *
* * *
*
* * * *
A study can be awarded a maximum of one star for each numbered item within the Selection and Outcome categories. A maximum of two stars can be given for Comparability.
Need for VPI treatment Four studies9e11,13 recorded the need for VPI treatment in both groups. Only one of them showed increased need for VPI treatment. The indications for VPI treatment were not specified in two studies, which makes it hard to compare the results.
Quality analysis All studies but one11 have multiple deficiencies in the study designs. Three9,13,14 of the six studies had relatively small sample size (4 years) to provide convincing results. Four studies10e12,14 had the loss of follow-up rate
Do patients with isolated Pierre Robin Sequence have worse outcomes after cleft palate repair: A systematic review* Teng Wan a,b, Yang Chen a,b, Guomin Wang a,b,* a Department of Oral and Maxillofacial Surgery, Ninth People’s Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai 200011, PR China b Shanghai Key Laboratory of Stomatology, Shanghai 200011, PR China
Received 15 October 2014; accepted 13 April 2015
KEYWORDS Pierre Robin Sequence; Cleft palate repair; Outcome; Cleft palate-only; Systematic review
Summary Cleft palate repair for Pierre Robin Sequence (PRS) patients has always been a challenge for surgeons and anesthetists. The aim of this systematic review is to investigate the outcome of cleft palate repair for PRS patients compared with cleft palate-only patients. All papers published before October 2014 were searched in the databases PubMed and MEDLINE. Search terms included “Pierre Robin Sequence,” “cleft palate repair,” and “speech result.” Additional studies were identified by hand searching the reference lists of the papers retrieved from electronic search. Two independent reviewers assessed the eligibility of studies for inclusion, extracted the data, and assessed the quality of the studies. Six studies met the inclusion criteria. All but one study had multiple deficiencies in study designs. Four studies assessed the fistula rate of both groups, and all studies assessed some aspect of the speech results. Conflicting results and a lack of high-quality and long-term outcomes of reviewed studies provided no conclusive scientific evidence about whether the outcome of cleft palate repair for PRS patients was better or worse than cleft palate-only patients. Further well-designed, well-controlled, and long-term studies are needed. ª 2015 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.
*
This study has not been presented at any meeting, wholly or in part. * Corresponding author. Department of Oral and Maxillofacial Surgery, Ninth People’s Hospital, School of Medicine, Shanghai Jiao Tong University, No. 639 Zhi Zao Ju Road, Shanghai 200011, PR China. Tel.: þ86 021 23271699 5149; fax: þ86 021 53072423. E-mail address: [email protected] (G. Wang). http://dx.doi.org/10.1016/j.bjps.2015.04.015 1748-6815/ª 2015 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.
1096
T. Wan et al.
Introduction Pierre Robin Sequence (PRS) is a congenital condition characterized by micrognathia, glossoptosis, airway obstruction, and often U-shaped cleft palate (Figure 1). It was first described by the French stomatologist Pierre Robin in 19231 and 1934.2 Infants with this condition usually suffer from airway problems and subsequently feeding difficulties, pulmonary infection, failure to thrive, and sometimes even death. In literature, there are many different names for this condition, such as Pierre Robin Syndrome, complex, or anomalad. Whether cleft palate is an obligatory feature of PRS remains debated. A survey3 of 73 cleft and craniofacial providers yields 14 different definitions of PRS, which shows the variability in the definition of this well-known eponym. Thus, the incidence of the condition varies from one per 8500 to one per 20,000 live births.4 Different diagnoses not only influence epidemiological and outcome studies but also make it very difficult to compare the results of different studies. The fact that PRS can occur isolated or in syndromic form further influences the situation. Most of the studies on PRS have focused on the airway management in the neonatal period.5e7 Little information is available about the outcomes after palatoplasty in this subset of patients with cleft palate. Protocols for the management of PRS vary by surgeons and institutions. The outcome studies of cleft palate repair are few, and they show conflicting results regarding the oronasal fistula rates and long-term speech results. The purpose of this study is to systematically review whether patients with isolated PRS have worse outcomes after cleft palate repair compared with cleft palate-only (CPO) patients. The outcomes include oronasal fistula rate, other complications, and long-term speech results.
Methods Study selection To find the relevant articles appropriate for this review, a search was conducted according to the following search strategy: (1) The electronic database MEDLINE (via PubMed) from 1966 to October 2014 was searched using a combination of the following keywords: “Pierre Robin Sequence,” “cleft palate repair,” and “speech result; ” and (2) the reference lists of all relevant publications were reviewed to identify any publications not already identified using the first search strategies. The inclusion criteria were as follows: 1. Studies that evaluated the outcomes of cleft palate repair for PRS patients versus CPO patients. 2. Only articles in English and Chinese were included.
Figure 1
U-shaped cleft palate in PRS patients.
Data extraction and quality check A standardized data extraction form was used to retrieve information of interest, including author, year of publication, study design, sample size, surgical technique, age at palate repair, age at speech evaluation, methods and measurements (Table 1), and results (fistula rate, resonance, articulation, and need for secondary treatment) (Table 2). The quality of included studies was documented by evaluating its quality using a modified NewcastleeOttawa Scale8 (Table 3). It was developed to assess the quality of nonrandomized studies with its design, content, and ease of use directed to the task of incorporating the quality assessments in the interpretation of meta-analytic results. The extraction of data and quality check were done by two authors independently without blinding. Conflicts between examiners were resolved by discussion of each paper to reach a consensus.
Results We retrieved 59 papers, which we analyzed according to the selection criteria. Six of these papers qualified for the review9e14 (Table 1). All studies are retrospective. All studies reviewed the fistula rate and some aspect of the speech result. The variables used in the six studies are shown in Table 2. The quality of each study is shown in Table 3. All but one study11 had multiple deficiencies in methods.
Fistula rate The exclusion criteria were as follows: 1. Studies that did not have a control group. 2. Cleft palate repair at >3 years of age. 3. Studies involving 3 years Fistula,a need for VPI 14.9 m treatment 14.2 m (12e18 m) >8 years Fistula, resonance, 12.5 m (11e14 m) velopharyngeal function, need for VPI treatment 12 m (6e78 m) 4.75 years Resonance, articulation, velopharyngeal function, need for VPI treatment 13 8.9 years Fistula, resonance, (8 monthse2.4 years) (5.3e11.8 years) velopharyngeal function, 12 m 7.83 years articulation, need for VPI (3 monthse9 years) treatment (5e18.5 years) 12.7 m 34.67 m Fistula, resonance, velopharyngeal function
ND: no data. a No data of the control group.
Resonance Of the six studies reviewed, five studies9,11e14 examined the resonance of both groups. Five studies9,11e14 examined the hypernasality of both groups: one study11 showed increased hypernasality in PRS group, and others found no difference between groups. Three studies9,13,14 examined
hyponasality of both groups; all found no difference between groups. Three studies9,11,12 examined the nasal emission of both groups; one study9 showed increased nasal emission in PRS group. The results of study 513 were the final results after velopharyngeal insufficiency (VPI) treatment, which was different from other studies.
Velopharyngeal function Table 2
Variables in included studies.
Variable
19
210
311
412
513
614
Fistula rate Resonance Hypernasality Hyponasality Nasal emission Velopharyngeal function Articulation Minor Gross Constant intelligibility Need for VPI treatment
X e X X [ X e X X X X Xb
e e e e e e e e e e e [
X e [ e X Y e e e X e Xb
e
X e Xa Xa e Xa e e e e e X
X e X X e X e e e e e e
X e X X X e e e e e
[, increased magnitude in PRS group; Y, decreased magnitude in PRS group; X, no difference between groups. a The results were final speech outcomes after VPI treatment for both groups. b The indication for VPI treatment was not specified in the article.
Five studies9,11e14 examined velopharyngeal (VP) function of both groups: one study11 showed decreased VP function in PRS group, and the other four showed no significant difference between groups. The result of study 513 was the final result after VPI treatment.
Articulation Three studies9,11,12 examined articulation; one9 evaluated minor VP unrelated articulation errors, gross VP unrelated articulation errors, and constant VP-related errors, all of which showed no difference between groups. One study12 evaluated the compensatory articulation errors associated with VPI, and it found no difference between groups.
Intelligibility Only one study9 examined the intelligibility of speech. Both intelligibility in known and unknown contexts showed no significant difference between groups.
1098 Table 3
T. Wan et al. Quality analysis of included studies. 19
Selection
Comparability Exposure
Representativeness of the exposed cohort Selection of the non-exposed cohort Ascertainment of exposure Demonstration that outcome of interest was not present at start of study Study controls for associated syndromes Study controls for age at cleft palate repair Assessment of outcome Was follow-up long enough for outcomes to occur Adequacy of follow up of cohorts
* *
* * *
210
311
412
513
614
* *
* * * *
* * * *
* * * *
* *
* * * * *
*
* *
* *
* * *
*
* * * *
A study can be awarded a maximum of one star for each numbered item within the Selection and Outcome categories. A maximum of two stars can be given for Comparability.
Need for VPI treatment Four studies9e11,13 recorded the need for VPI treatment in both groups. Only one of them showed increased need for VPI treatment. The indications for VPI treatment were not specified in two studies, which makes it hard to compare the results.
Quality analysis All studies but one11 have multiple deficiencies in the study designs. Three9,13,14 of the six studies had relatively small sample size (4 years) to provide convincing results. Four studies10e12,14 had the loss of follow-up rate
