Comparison of two surgical protocols for the treatment of unilateral cleft lip and palate: a multidisciplinary systematic review and meta-analysis.

There is still no unanimous agreement on the optimal surgical protocol(s) for the treatment of unilateral cleft lip and palate (UCLP), and a huge variety of protocols are employed by cleft centres across the world. The aim of this systematic review and meta-analysis was to compare reported patient outcomes of the Oslo protocol (and modifications) (OP) and delayed hard palate closure protocols (DHPCP) from a multidisciplinary perspective. A systematic search of multiple databases was conducted until September 2023. Studies reporting any patient outcomes of these protocols were included. Random-effects meta-analyses were performed for evidence synthesis, including comparisons of results between the types of protocol. The quality of evidence was evaluated using the ROBINS-I tool. In total, 62 articles (42 studies) reporting patients with UCLP were reviewed, involving 1281 patients following the OP and 655 following DHPCP. Equally poor long-term sagittal maxillofacial growth was found, and similar results for velopharyngeal insufficiency and nasolabial appearance. In contrast, OP was associated with a lower rate of oronasal fistulas. Disregarding the scarcity of comparable evidence for some domains, the results of this review, overall, favour OP over DHPCP. However, caution should be taken when interpreting the results on velopharyngeal insufficiency and oronasal fistulas, since the possibility of confounding and other biases remains.

Complications in post-bariatric body contouring surgery using a practical treatment regime to optimise the nutritional state.

Background: Post-bariatric body contouring surgery (BCS) treats redundant skin after massive weight loss; however, the complication risk is relatively high (23-70%). Most complications are wound-related, which may be partly due to a poor nutritional status after bariatric surgery. The objective of this observational study was to optimise nutrition preoperatively and assess the prevalence of wound-related complications after BCS. Methods: This prospective cohort study included 140 patients. Patients were treated according to the post-bariatric BCS guideline. Nutritional parameters were collected via pre- and peri-operative blood sampling; any deficiencies were treated. A protein-enriched diet was prescribed by a dietician 4 weeks preoperatively up until closure of all wounds. Complications were recorded using the Clavien-Dindo classification. Univariate and multivariate regression analyses were performed to identify variables associated with wound-related complications. Results: The overall wound-related complication rate was 51%. Most complications were minor, with only 4.3% was considered major. No significant differences in patient characteristics were found between patients with and without complications. Variables indicating an optimised nutritional state were not significantly associated with a decreased risk of complications; the most influential factor was a sufficient post-operative protein intake (OR 0.27, 95% CI 0.07 - 1.02, p = 0.05). Conclusion: The overall wound-related complication rate was in accordance with previous literature; however, major complications were few. This study showed a weak correlation between optimising nutritional state and better outcome after BCS, especially following a protein-enriched diet post-operatively. Therefore, we recommend continuing research on nutrition and wound-related complications, using homogeneous study populations and well-defined complications.

Surgical management in submucous cleft palate patients.

OBJECTIVES: The submucous cleft palate (SMCP) is considered to be the most subtle type of cleft palate. Early detection is important to allow on time intervention by speech therapy and/or surgical repair before the children already develop compensatory speech mechanisms. The purpose of this study was to investigate at what time children with a SMCP present, to determine when children are operated, and to analyze the postoperative outcomes for in SMCP children. PATIENT AND METHODS: Medical records from 766 individuals registered in the cleft registry in the Wilhelmina's Children's' Hospital, Utrecht, were retrospectively reviewed. Inclusion criteria were children diagnosed with SMCP. The following data were collected: age at diagnosis, physical examination, age at surgery, surgical technique, speech therapy pre- and post-surgery, otitis media, secondary cleft surgery, family history, syndromes, and other anomalies. RESULTS: In total, 56 SMCP children were identified. The mean age of diagnosis was 44.0 months (range 0-150, SD = 37.0). In 48 children (85.7%), surgical intervention was performed (Furlow plasty, intravelar veloplasty, pharyngoplasty, or Furlow combined with buccal flap). CONCLUSION: This retrospective study reconfirms that SMCP often presents late, even in a country with a modern healthcare system and adequate follow-up of all newborns by the so-called youth doctors in "children's healthcare centers" up to the age of 4 years old. Almost 86% of patients ultimately needed palate surgery when SMCP was suspected. CLINICAL RELEVANCE: Any child presenting with repeated episodes of otitis media, nasal regurgitation, or speech difficulties should have prompt consideration for SMCP as diagnosis.

Incidence of cleft-related speech problems in children with an isolated cleft lip.

OBJECTIVES: Clinicians agree that children with isolated cleft lip have fewer cleft-associated problems than children with cleft lip and palate. Unfortunately, for isolated cleft lip children, the risk of cleft-associated problems is unknown and maybe underestimated. Often, these children do not get the required follow-up by a multidisciplinary team and thereby not the known benefits in supporting their development. This study examines the incidence of cleft-related speech problems and ear problems in children with isolated cleft lip. MATERIALS AND METHODS: A prospective study was performed on all children born with an isolated cleft lip and treated at the Wilhelmina Children's Hospital in Utrecht between January 2007 and April 2014. Data were collected for sex, date of birth, genetics, cleft lip type, date of cleft lip repair, type of repair, speech/language problems, and ear problems. RESULTS: This study included 75 patients (59% male). The mean age of the children at the moment of speech examination was 32.5 months (SD 6.1). Eighteen of the 75 children (24%) needed speech and language therapy; however, only one child (1.3%) had a cleft-related speech problem. Sixteen of the 75 patients (21%) reported a history of one or more episodes of acute otitis media (AOM)/otitis media with effusion (OME) during the first 6 years. CONCLUSION/CLINICAL RELEVANCE: This is the first prospective study analyzing the incidence of cleft-related speech problems in children with an isolated cleft lip. These children do not have a higher risk of cleft-related speech problems or AOM/OME when compared to the general population. However, children with an isolated cleft do have a higher incidence of speech therapy.

Body Image as a Potential Motivator for Bariatric Surgery: a Case-Control Study.

BACKGROUND: Not every eligible person opts for bariatric surgery. Body image concerns might be a reason to choose surgery. This case-control study evaluated differences in body image between a pre-bariatric surgery population and a weight-matched control group from the general population. We hypothesized that the pre-bariatric group would show less satisfaction with appearance, defined as a discrepancy between evaluating one's appearance as less attractive while attaching more importance to appearance. METHODS: Data from 125 pre-bariatric patients were compared with 125 body weight-matched controls from the general population. The Multidimensional Body-Self Relations Questionnaire-Appearance Scales was used to assess appearance evaluation (AE), appearance orientation (AO), and their discrepancy score. Both groups were compared with norms from the non-body weight-matched general population. RESULTS: The pre-bariatric group had lower AE scores (mean 2.23 ± 0.65 vs. mean 2.54 ± 1.06) and higher AO scores (mean 3.33 ± 0.69 vs. mean 3.04 ± 0.90) than the control group. The discrepancy between AE and AO was larger in the pre-bariatric group (p < 0.001). Compared with the general population, both groups showed lower AE scores (d = - 1.43 and d = - 1.12, p < 0.001) and lower AO scores (d = - 0.23 and d = - 0.58, p < 0.001). CONCLUSIONS: People with morbid obesity have on average less body image satisfaction. The results indicate that part of the motivation of people that choose bariatric surgery may be due to relatively low global appearance evaluation combined with considering appearance more important. Knowledge about motivations can be used to communicate realistic expectations regarding treatment outcome.

The BAPRAS screening tool for reimbursement in a postbariatric population.

INTRODUCTION: Reimbursement of body-contouring surgery (BCS) is a worldwide problem: there is no objective instrument to decide which postbariatric patients should qualify for reimbursement. The British Association of Plastic, Reconstructive and Aesthetic Surgeons (BAPRAS) has developed a screening tool for this purpose. In this study, we used a modified version of this screening tool in a postbariatric population and describe which patients would qualify for reimbursement using this tool. METHODS: In this cross-sectional study postbariatric patients were asked to fill in an online questionnaire based on the BAPRAS screening tool with questions regarding complaints of overhanging skin and medical history. Weight loss data were extracted from a prospective database. The BODY-Q was added to assess patient-reported outcomes. RESULTS: Patients who wanted to undergo BCS (n = 90) had higher screening tool scores and lower BODY-Q scores compared to patients who did not want BCS (n = 24). In total, 25 patients (26%) qualified for reimbursement, these patients had higher weight loss (33.5% versus 29.2%, p = 0.008), lower BMI (27.3 kg/m2 versus 30.4 kg/m2, p = 0.014) and more medical (4.0 versus 2.0, p = 0.004) and psychological complaints (88% versus 61%, p = 0.009). There was a significant, negative correlation between the screening tool scores and almost all BODY-Q scales. CONCLUSIONS: Patients with a desire for BCS have more complaints of excess skin, which negatively impacts their well-being. With the modified BAPRAS screening tool, patients with the best weight (loss) and most medical and psychological complaints of excess skin qualified for referral and reimbursement of BCS.

Internationally adopted children with cleft lip and/or cleft palate: Middle ear findings and hearing during childhood.

OBJECTIVE: Adopted children with cleft lip and/or cleft palate form a diverse group of patients. Due to increased age at palatal repair, adopted children have a higher risk of velopharyngeal insuffiency and poor speech outcome. Delayed palate repair may also lead to longer lasting Eustachian tube dysfunction. Decreased function of the Eustachian tube causes otitis media with effusion and recurrent acute otitis media, which can lead to other middle ear problems and hearing loss. METHODS: One-hundred-and-thirty-two adopted children treated by the Cleft palate team in Wilhelmina Children's Hospital during January 1994 and December 2014 were included. Retrospectively, middle ear findings, the need for ventilation tube insertion and hearing during childhood were assessed. Findings were compared with 132 locally born children with cleft lip and/or cleft palate. RESULTS: Adopted children had a mean age of 26.5 months old when they arrived in our country. After the age of two the total number of otitis media with effusion episodes and the need for ventilation tube placement did not significantly differ among adopted and non-adopted children. Adopted children had significantly more tympanic membrane perforations. Hearing threshold levels normalized with increasing age. Although within normal range, adopted children showed significantly higher pure tone averages than locally born children when they were eight to ten years old. CONCLUSION: In general, adopted patients with cleft lip and/or cleft palate did not have more middle ear problems or ventilation tubes during childhood. However, theyhave more tympanic membrane perforations.

Internationally adopted children with cleft lip and/or palate: A retrospective cohort study.

OBJECTIVE: The treatment approach for internationally adopted children with cleft lip and/or palate differs from locally born children with cleft lip and/or palate. They are older at initial presentation, may have had treatment abroad of different quality, and are establishing new and still fragile relationships with their adoptive parents. The aim of this study was to describe the characteristics and initial care and treatment of this group. METHODS: A retrospective cohort study was performed including all internationally adopted children with cleft lip and/or palate presenting to the cleft team outpatient clinic in the Wilhelmina Children's Hospital between January 1994 and December 2014. Medical records of all patients were reviewed; information concerning demographic characteristics, characteristics at initial presentation, and treatment were obtained. RESULTS: A total number of 132 adopted patients were included: 15% had cleft lip, 7% had cleft palate, and 78% had cleft lip and palate. The average age at the time of adoption was 26.5 months. In most cases, China was the country of origin. Seventy-eight percent had surgery in their country of origin, primarily lip repair. Fistulae in need of revision surgery were found in 8% of the patients. Pharyngoplasty was needed in 48% of the patients. No significant differences were found for mean age at adoption, gender, cleft type, and one- or two-stage palatal closure. CONCLUSION: Internationally adopted children with cleft lip and/or palate are a very diverse group of patients with challenging treatment. These children undergo surgery late and frequently need additional surgery.

Hearing sensitivity in adults with a unilateral cleft lip and palate after two-stage palatoplasty.

OBJECTIVE: To evaluate long-term hearing and middle ear status in patients treated for a unilateral complete cleft lip and palate (UCLP) by two-stage palatoplasty. METHODS: Forty-nine UCLP patients aged 17 years and older were included in this retrospective study. Patients were invited for a multidisciplinary long-term follow-up of their treatment at a tertiary center for craniofacial surgery in the Netherlands. ENT assessment included tympanometry and pure-tone audiometry. Medical files were searched for medical and surgical history. RESULTS: In total, 19.4% of the patients had significant long-term hearing loss (PTA > 20 dB), comprising conductive hearing loss in 21.5% of the patients. In the majority (70%), this hearing loss was more pronounced at higher frequencies. In 25% the high fletcher index showed hearing thresholds above 20 dB. Ventilation tubes were placed at least once in 78.7% of the patients. The frequency of tube insertion was positively correlated with the incidence of reduced tympanic compliance (tympanogram type B) and the need for a pharyngoplasty. CONCLUSION: The present study reports long-term hearing outcomes in UCLP patients with hard palate closure at 3 years of age. Persistent hearing loss was observed in 19.4% of our patients (PTA > 20 dB), mainly at the higher frequencies. Ventilation tube placement was associated with reduced tympanic compliance and higher risk on hearing loss (PTA air conduction). Our high incidence of velopharyngeal incompetence during early childhood, which is likely associated with Eustachian tube dysfunction, might have contributed to these results.

Long-term mid-facial growth of patients with a unilateral complete cleft of lip, alveolus and palate treated by two-stage palatoplasty: cephalometric analysis.

OBJECTIVES: The aim of this study is to evaluate long-term facial growth in adults previously treated for an isolated unilateral complete cleft lip, alveolus and palate by two-stage palatoplasty. MATERIALS AND METHODS: Unilateral cleft lip and palate (UCLP) patients of 17 years and older treated by two-stage palatoplasty were invited for long-term follow-up. During follow-up, lateral cephalograms were obtained (n = 52). Medical history was acquired from their medical files. Outcome was compared to previously published normal values and the Eurocleft study. RESULTS: Soft and hard palate closure were performed at the age of 8 (SD 5.9) months and 3 (SD 2.2) years, respectively. The mean maxillary and mandibular angle (SNA, SNB) were 74.9° (SD 4.2) and 75.8° (SD 3.8). Maxillary and maxillomandibular relationships (SNA, ANB) were comparable to all Eurocleft Centres, except for Centre D. We observed a significantly steeper upper interincisor angle compared to the Eurocleft Centres. CONCLUSIONS: This study describes the long-term craniofacial morphology in adults treated for a UCLP with hard palate closure at a mean age of 3 years. The mean maxillary angle SNA and mandibular angle SNPg were comparable to previous studies both applying early and delayed hard palate closure. The observed upper incisor proclination is likely caused by orthodontic overcorrection in response to the unfavourable jaw relationships. No clear growth benefit of this protocol could be demonstrated. CLINICAL RELEVANCE: The present study shows the long-term craniofacial morphology of UCLP adults after the Utrecht treatment protocol which includes two-stage palate closure.

Otologic and audiologic findings in 22q11.2 deletion syndrome.

Hearing loss is frequently present in the 22q11.2 deletion syndrome. Our aim was to describe the audiologic and otologic features of patients with 22q11.2 deletion syndrome. We conducted a retrospective cohort study in a single tertiary referral center. We reviewed medical files of all patients with 22q11.2 deletion syndrome who visited an otolaryngologist, plastic surgeon or speech therapist, for audiologic or otologic features. Hearing loss was defined as a pure tone average (of 0.5, 1, 2, and 4 kHz) of >20 decibel hearing level. Audiograms were available for 102 of 199 included patients, out of which 163 ears were measured in the required frquencies (0.5-4 kHz). Median age at time of most recent audiogram was 7 years (range 3-29 years). In 62 out of 163 ears (38%), hearing loss was present. Most ears had conductive hearing loss (n = 58) and 4 ears had mixed hearing loss. The severity of hearing loss was most frequently mild (pure tone average of ≤40 decibel hearing level). In 22.5% of ears, otitis media with effusion was observed at time of most recent audiogram. Age was not related to mean air conduction hearing thresholds or to otitis media with effusion (p = 0.43 and p = 0.11, respectively). In conclusion, hearing loss and otitis media are frequently present in patients with 22q11.2 deletion syndrome. Moreover, our results suggest that children with 22q11.2 deletion syndrome remain susceptible for otitis media as they age.

Currently known risk factors for hypertrophic skin scarring: A review.

OBJECTIVE: The study aims to provide an overview of risk factors for hypertrophic scarring. BACKGROUND: Hypertrophic skin scarring remains a major concern in medicine and causes considerable morbidity. Despite extensive research on this topic, the precise mechanism of excessive scarring is still unknown. In addition, the current literature lacks an overview of the possible risk factors in the development of hypertrophic scars. METHODS: PubMed searches were performed on risk factors for hypertrophic scar (HTS) formation. RESULTS: Eleven studies suggesting nine factors associated with HTS formation were found. Studies concerning chemotherapy, age, stretch, infection, and smoking have a moderate to high strength of evidence, but some other factors have not been studied in a convincing manner or are still disputed. CONCLUSIONS: Risk factors for HTS formation are young age, bacterial colonization, and skin subjected to stretch. Chemotherapy, statins, and smoking seem to play a protective role in HTS formation.

Late detection of cleft palate.

Cleft palate only (CPO) is a common congenital malformation, and most patients are diagnosed within the first weeks after birth. Late diagnosis of the cleft palate (CP) could initially result in feeding and growth impairment, and subsequently speech and hearing problems later in life. The purpose of this study is to retrospectively investigate (1) at which age CPO is diagnosed and (2) how the presence of syndromes and other factors relate to the age at diagnosis. The mean age of all children at our centre with CPO included between 1997 and 2014 at diagnosis (n = 271) was 1 year and 4 months. In all, 24.8% (n = 67) was older than 12 months when diagnosed, and 37.3% (n = 101) of all children had been diagnosed >30 days. These findings remain valid when a cut-off point of 14 days is used (44.3% late). Moreover, the grade of the cleft was a determining factor for successful diagnosis; submucous clefts were detected much later on average (89.3% > 30 days; p = .000). Similar results were found using Kaplan-Meier survival analyses. CONCLUSION: CPO is often diagnosed late. Patients diagnosed ≤30 days after birth more often presented with an associated disorder. Early diagnoses became more frequent as the severity of the cleft increased (grades 1-4). Professionals should perform more thorough intra-oral investigations, including manual palpations and visual inspections of the palate; they should be made more aware of the frequent accompanying symptoms. WHAT IS KNOWN: The presence of cleft palate only (CPO) is known to negatively affect feeding, hearing, speech and (social) development. Submucous clefts are often underdiagnosed due to their difficulty to detect. As far as we know the literature shows that symptomatic submucous CPs are often diagnosed at an average age of 4.9 years. WHAT IS NEW: 37.3% respectively of all children with CPO were diagnosed relatively late (>30 days after birth), 24.8% was older than 12 months when diagnosed. Mean age of all children with CPO was 1 year and 4 months. We conclude that midwives and pediatricians should perform more through intra-oral investigations of all new-borns, including both a manual palpation, als well a visual inspection of the palate.

[Interdisciplinary orthodontic surgical treatment of children with cleft lip and palate from 9 to 20 years of age]. / Interdisciplinaire orthodontisch-chirurgische behandeling van schisis van 9 tot 20 jaar.

Cleft lip and palate is a common congenital malformation with a prevalence of 1:600 newborns. Children with orofacial clefts are treated by an interdisciplinary team of specialists while parents and child play a key role in their own care process. The orthodontic and facial orthopedic treatment of a child with a cleft takes many years. Children often get bored of the long treatment and this can cause problems with compliance and oral hygiene. Therefore it is advisable to distinguish 5 well-defined stages in the orthodontic treatment and to attempt to have some 'orthodontics free' time in between. The 3 orthodontic treatment phases between the age of 9 and 20 years consist of orthodontic treatment concerning the closing of the cleft with a bone transplant, the treatment of the permanent dentition and, finally, a possible combined orthodontic surgical treatment at the end of the period of growth. Good interdisciplinary collaboration among the different dental disciplines is essential in this regard.

Prevalence of feeding disorders in children with cleft palateonly: a retrospective study.

OBJECTIVES: The purpose of this study in children with cleft palate only (CPO) is to (1) explore the prevalence of feeding problems on a retrospective basis, (2) investigate rates of nasogastric (NG) feeding, (3) examine the prevalence of associated disorders and/or syndromes (AssD/S), (4) investigate if there are certain risk factors associated with feeding difficulties, NG feeding, and failure of breastfeeding, and (5)investigate the effect of palatoplasty on feeding difficulties. MATERIALS AND METHODS: In total, 90 questionnaires were included in this study. The medical records were reviewed. RESULTS: Feeding difficulties were reported in 67 % (n =60) of all cases. NG feeding was given in 32% (n =28) of all children. Forty-nine children (54 %) have associated malformations. There is no significant relation for gender, gestational age,and birth weight as risk factors for feeding difficulties, NG feeding, and failure of breastfeeding. The severity of the cleft is significantly related to the prevalence of AssD/S. After palatoplasty, feeding difficulties improved in 79 % of the CPO children. CONCLUSIONS AND CLINICAL RELEVANCE: First, our results clearly indicate that children with CPO are at high risk of developing feeding difficulties (67 %); NG feeding is often necessary (32 %). Second, our results also indicate that the more severe the cleft, the more likely the chance for AssD/S. Third, these verity of the cleft is significantly related to the prevalence of AssD/S. Fourth, there is no significant relation for gender, gestational age, and birth weight as risk factors for feeding difficulties, NG feeding, and failure of breastfeeding. Fifth, improvement of feeding difficulties after surgery supports the importance of the soft palate closure in relation to sucking patterns and feeding skills.

Prevalence of feeding disorders in children with cleft palate only: a retrospective study.

OBJECTIVES: The purpose of this study in children with cleft palate only (CPO) is to (1) explore the prevalence of feeding problems on a retrospective basis, (2) investigate rates of nasogastric (NG) feeding, (3) examine the prevalence of associated disorders and/or syndromes (AssD/S), (4) investigate if there are certain risk factors associated with feeding difficulties, NG feeding, and failure of breastfeeding, and (5) investigate the effect of palatoplasty on feeding difficulties. MATERIALS AND METHODS: In total, 90 questionnaires were included in this study. The medical records were reviewed. RESULTS: Feeding difficulties were reported in 67% (n = 60) of all cases. NG feeding was given in 32% (n = 28) of all children. Forty-nine children (54%) have associated malformations. There is no significant relation for gender, gestational age, and birth weight as risk factors for feeding difficulties, NG feeding, and failure of breastfeeding. The severity of the cleft is significantly related to the prevalence of AssD/S. After palatoplasty, feeding difficulties improved in 79% of the CPO children. CONCLUSIONS AND CLINICAL RELEVANCE: First, our results clearly indicate that children with CPO are at high risk of developing feeding difficulties (67%); NG feeding is often necessary (32%). Second, our results also indicate that the more severe the cleft, the more likely the chance for AssD/S. Third, the severity of the cleft is significantly related to the prevalence of AssD/S. Fourth, there is no significant relation for gender, gestational age, and birth weight as risk factors for feeding difficulties, NG feeding, and failure of breastfeeding. Fifth, improvement of feeding difficulties after surgery supports the importance of the soft palate closure in relation to sucking patterns and feeding skills.

Minimal extracorporeal circulation (MECC) does not result in less hypertrophic scar formation as compared to conventional extracorporeal circulation (CECC) with dexamethasone.

INTRODUCTION: Cardiopulmonary bypass surgery is associated with a systemic inflammatory response through the interaction of air, blood and synthetic components in the bypass system and the physical trauma of surgery. An alternative cardiopulmonary bypass system, minimal extracorporeal circulation (MECC), has shown promising results in terms of reducing the inflammatory response. We hypothesized that this system may reduce pathological excessive scarring. To study this assumption, the effects of MECC and the effects of conventional extracorporeal circulation (CECC) with dexamethasone on skin scarring were compared in a standardized wound-healing model. METHODS AND RESULTS: Pre-sternal scars were evaluated prospectively at four and 12 months postoperatively. The height and width of the scars were measured, using a slide caliper and sonography. The scars were scored using the validated Patient and Observer Scar Assessment Scale. Additional risk factors for hypertrophic scar formation were identified by means of a questionnaire. During surgery, MECC was used in 45 patients and CECC/dexamethasone in 42 patients. Four months postoperatively, 22 patients of the MECC group (49%) and 18 patients in the CECC/dexamethasone group (43%) had developed hypertrophic scars. Twelve months postoperatively, the hypertrophic scars in four patients of the MECC group and in two patients of the CECC/dexamethasone group had become normotrophic. In 18 patients of the MECC group (38%) and 16 patients of the CECC group (41%) the scars remained hypertrophic at 12 months. These differences between the two groups were not statistically significant. CONCLUSION: MECC does not reduce hypertrophic scar formation compared with CECC with dexamethasone, but its use is more beneficial than the use of CECC/dexamethasone because of the circulatory and immunological advantages and because treatment with dexamethasone can be omitted.

Classification of contour deformities after massive weight loss: the applicability of the Pittsburgh Rating Scale in The Netherlands.

BACKGROUND: The Pittsburgh Rating Scale is the only validated classification system of skin deformities occurring after massive weight loss. The purpose of this study was to replicate the validation of the Pittsburgh Rating Scale classification and to evaluate its usefulness in the treatment of massive weight-loss patients in The Netherlands. METHODS: Thirteen trained observers applied the Pittsburgh Rating Scale to photographs of 25 patients. These photographs showed the 10 regions of the body for which the Pittsburgh Rating Scale is designed. Six of the observers were medical specialists, three were medical interns in plastic surgery and four observers were specialised nurse practitioners. As a measure of inter-rater agreement we calculated the intraclass correlation with a threshold value of 0.6 for good validity. The observers also answered 11 questions about the scale's usefulness in daily practice. RESULTS: In two consecutive tests the photographs of 10 regions were scored, which resulted in a total of 20 observations per patient. Sixty percent of the intraclass correlation values were below the threshold of 0.6 for good validity. The mean intraclass correlation value was 0.577. CONCLUSIONS: The Pittsburgh Rating Scale could not be validated as a reliable classification system for skin deformities after massive weight loss. The scale however seems to be a good first step in a challenging task. There was no doubt among the observers that a good classification system would be beneficial for adequate treatment. A modified Pittsburgh Rating Scale should include, besides anatomical parameters, functional disability and hygienic impairment scores and perioperative risk factors.

Velopharyngeal dysfunction and 22q11.2 deletion syndrome: a longitudinal study of functional outcome and preoperative prognostic factors.

OBJECTIVE: To describe the effect of time after velopharyngoplasty on outcome and to search for preoperative prognostic factors for residual hypernasality in patients with 22q11.2 deletion syndrome. DESIGN: Retrospective chart review. SETTING: Tertiary hospital. PATIENTS: Patients with 22q11.2 deletion syndrome and velopharyngeal dysfunction who underwent a primary (modified) Honig velopharyngoplasty between 1989 and 2009. MAIN OUTCOME MEASURES: Clinically obtained perceptual and instrumental measurements of resonance, nasalance, and understandability before and after velopharyngoplasty. RESULTS: Data were available for 44 of 54 patients (81% follow-up), with a mean follow-up time of 7.0 years (range, 1.0 to 19.4 years). During follow-up, 24 (55%) patients attained normal resonance and 20 (45%) had residual hypernasality or underwent revision surgery. Mean postoperative nasalance and understandability scores were closer to the norm than mean preoperative scores were (2.0 versus 5.5 standard deviations for the normal passage, 1.3 versus 8.1 standard deviations for the nonnasal passage, and score 2.3 versus 4.1 understandability). Serial measurements revealed that hypernasality only resolved an average of 5 years after surgery, and three patients whose resonance initially normalized later relapsed to hypernasality. Gender, age at surgery, lateral pharyngeal wall adduction, velar elevation, presence of a palatal defect, previous intravelar veloplasty, nasalance, understandability, adenoidectomy, hearing loss, and IQ were not able to predict poor outcome following primary velopharyngoplasty (all p > .05). CONCLUSIONS: In this chart review of patients with 22q11.2 deletion syndrome and velopharyngeal dysfunction, residual hypernasality persisted in many patients after velopharyngoplasty. None of the preoperative factors that were studied had prognostic value for the outcome.

Prenatal ultrasound screening for orofacial clefts.

OBJECTIVES: To evaluate the sensitivity and specificity of ultrasound for detecting prenatal facial clefts in low-risk and high-risk populations. METHODS: This study prospectively followed up a non-selected population, namely all pregnant women who underwent routine second-trimester prenatal ultrasound screening in the Utrecht region during the 2-year period from January 2007 to December 2008. RESULTS: A total of 35 924 low-risk and 2836 high-risk pregnant women underwent ultrasound screening. Orofacial clefts were present in 62 cases, an incidence of 1:624. The distribution of clefts was as follows: 18 (29%) cleft lip, 25 (40%) cleft lip with cleft palate, 17 (27%) cleft palate only, one median cleft and one atypical cleft. Of these, 38 (61%) were unilateral and 23 (37%) were bilateral. Thirty-nine per cent (24/62) had associated anomalies, with most chromosomal defects found in the cleft lip with cleft palate and cleft palate only groups. Cleft lip with or without cleft palate was detected prenatally in 38/43 cases, a sensitivity of 88%. No case of cleft palate only was detected prenatally. There were three false-positive cases, of which two were fetuses with multiple congenital deformities. CONCLUSIONS: Ultrasound screening has a high sensitivity for the detection of cleft lip with and without cleft palate in high-risk and low-risk pregnancies in our region, where well-trained sonographers carry out primary screening. The key to a high sensitivity of prenatal ultrasound is likely to be a combination of excellent training of sonographers, referral to specialized centers when a cleft is suspected, routine visualization of the fetal face and advances in ultrasound techniques.