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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.
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Fisura del Paladar , Insuficiencia Velofaríngea , Adolescente , Niño , Preescolar , Fisura del Paladar/cirugía , Humanos , Lactante , Recién Nacido , Estudios Retrospectivos , Habla , Trastornos del Habla , Colgajos QuirúrgicosRESUMEN
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.
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Labio Leporino , Fisura del Paladar , Pérdida Auditiva , Otitis Media con Derrame , Niño , Preescolar , Labio Leporino/complicaciones , Labio Leporino/epidemiología , Labio Leporino/cirugía , Fisura del Paladar/complicaciones , Fisura del Paladar/epidemiología , Fisura del Paladar/cirugía , Femenino , Humanos , Incidencia , Masculino , Ventilación del Oído Medio , Estudios Prospectivos , HablaRESUMEN
OBJECTIVES: To facilitate the best approach during cleft palate surgery, children are positioned with hyperextension of the neck. Extensive head extension may induce intraoperative cerebral ischemia if collateral flow is insufficient. To evaluate and monitor the effect of cerebral blood flow on cerebral tissue oxygenation, near-infrared spectroscopy has proved to be a valuable method. The aim of this study was to evaluate and quantify whether hyperextension affects the cerebral tissue oxygenation in children during cleft palate surgery. MATERIALS AND METHODS: This prospective study included children (ASA 1 and 2) under the age of 3 years old who underwent cleft palate repair at the Wilhelmina Children's Hospital, in The Netherlands. Data were collected for date of birth, cleft type, date of cleft repair, and physiological parameters (MAP, saturation, heart rate, expiratory CO2 and O2, temperature, and cerebral blood oxygenation) during surgery. The cerebral blood oxygenation was measured with NIRS. RESULTS: Thirty-four children were included in this study. The majority of the population was male (61.8%, n = 21). The mixed model analyses showed a significant drop at time of Rose position of - 4.25 (69-74 95% CI; p < 0.001) and - 4.39 (69-74 95% CI; p < 0.001). Postoperatively, none of the children displayed any neurological disturbance. CONCLUSION: This study suggests that hyperextension of the head during cleft palate surgery leads to a significant decrease in cerebral oxygenation. Severe cerebral desaturation events during surgery were uncommon and do not seem to be of clinical relevance in ASA 1 and 2 children. CLINICAL RELEVANCE: There was a significant drop in cerebral oxygenation after positioning however it is not clear whether this drop is truly significant physiologically in ASA 1 and 2 patients.
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Fisura del Paladar , Procedimientos de Cirugía Plástica , Preescolar , Femenino , Humanos , Masculino , Oxígeno , Estudios Prospectivos , Espectroscopía Infrarroja CortaRESUMEN
Paramount for the generation of auricular structures of clinically-relevant size is the acquisition of a large number of cells maintaining an elastic cartilage phenotype, which is the key in producing a tissue capable of withstanding forces subjected to the auricle. Current regenerative medicine strategies utilize chondrocytes from various locations or mesenchymal stromal cells (MSCs). However, the quality of neo-tissues resulting from these cell types is inadequate due to inefficient chondrogenic differentiation and endochondral ossification, respectively. Recently, a subpopulation of stem/progenitor cells has been identified within the auricular cartilage tissue, with similarities to MSCs in terms of proliferative capacity and cell surface biomarkers, but their potential for tissue engineering has not yet been explored. This study compared the in vitro cartilage-forming ability of equine auricular cartilage progenitor cells (AuCPCs), bone marrow-derived MSCs and auricular chondrocytes in gelatin methacryloyl (gelMA)-based hydrogels over a period of 56 d, by assessing their ability to undergo chondrogenic differentiation. Neocartilage formation was assessed through gene expression profiling, compression testing, biochemical composition and histology. Similar to MSCs and chondrocytes, AuCPCs displayed a marked ability to generate cartilaginous matrix, although, under the applied culture conditions, MSCs outperformed both cartilage-derived cell types in terms of matrix production and mechanical properties. AuCPCs demonstrated upregulated mRNA expression of elastin, low expression of collagen type X and similar levels of proteoglycan production and mechanical properties as compared to chondrocytes. These results underscored the AuCPCs' tissue-specific differentiation potential, making them an interesting cell source for the next generation of elastic cartilage tissue-engineered constructs.
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Condrogénesis/efectos de los fármacos , Cartílago Auricular/citología , Hidrogeles/farmacología , Células Madre/citología , Ingeniería de Tejidos/métodos , Animales , Biomarcadores/metabolismo , Diferenciación Celular/efectos de los fármacos , Células Cultivadas , Fuerza Compresiva , ADN/metabolismo , Módulo de Elasticidad , Matriz Extracelular/efectos de los fármacos , Matriz Extracelular/metabolismo , Regulación de la Expresión Génica/efectos de los fármacos , Glicosaminoglicanos/metabolismo , Caballos , Especificidad de Órganos/efectos de los fármacos , ARN Mensajero/genética , ARN Mensajero/metabolismo , Células Madre/efectos de los fármacos , Factores de TiempoRESUMEN
OBJECTIVES: Throat packs are commonly used to prevent ingestion or aspiration of blood and other debris during cleft lip/palate surgery. However, dislodgement or (partial) retainment after extubation could have serious consequences. The aim of the present study was to investigate the effect of omitting pharyngeal packing during cleft lip/palate surgery on the incidence of early postoperative complications in children. MATERIALS AND METHODS: A retrospective study was performed on all children who underwent cleft lip/palate surgery at the Wilhelmina Children's Hospital. This study compared the period January 2010 through December 2012 when pharyngeal packing was applied according to local protocol (group A) with the period January 2013 till December 2015 when pharyngeal packing was no longer applied after removal from the protocol (group B). Data were collected for sex, age at operation, cleft lip/palate type, type of repair, lateral incisions, length of hospital stay, and complications in the first 6 weeks after surgery. Early complications included wound dehiscence, postoperative bleeding, infection, fever, upper respiratory tract infection (URTI), and lower respiratory tract infection (LRTI). RESULTS: This study included 489 cleft lip/palate operations (group A n = 246, group B n = 243). A total of 39 (15.9%) early complications were recorded in group A and a total of 40 (16.5%) in group B. There were no significant differences (P = 0.902) in complications between the two groups; however, there was a significant difference (P < 0.001) in length of hospital stay between the two groups (group A 3.6 days vs group B 3.2 days). CONCLUSION: Omitting routine placement of throat packs in cleft lip/palate surgery was not associated with an increased early postoperative complication rate. Therefore, the traditional, routine placement of a throat pack during cleft lip/palate surgery can be questioned. CLINICAL RELEVANCE: The traditional, routine placement of a throat pack during cleft lip/palate surgery can be questioned.
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Manejo de la Vía Aérea/instrumentación , Labio Leporino/cirugía , Fisura del Paladar/cirugía , Faringe , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/prevención & control , Adolescente , Niño , Preescolar , Femenino , Humanos , Lactante , Tiempo de Internación/estadística & datos numéricos , Masculino , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
OBJECTIVE: To develop a reliable and easy-to-use method to assess the nasolabial appearance of 18-year-old patients with unilateral cleft lip and palate (CLP). DESIGN: Retrospective analysis of nasolabial aesthetics using a 5-point ordinal scale and newly developed photographic reference scale: the Cleft Aesthetic Rating Scale (CARS). Three cleft surgeons and 20 medical students scored the nasolabial appearance on standardized frontal photographs. SETTING: VU University Medical Center, Amsterdam. PATIENTS: Inclusion criteria: 18-year-old patients, unilateral cleft lip and palate, available photograph of the frontal view. EXCLUSION CRITERIA: history of facial trauma, congenital syndromes affecting facial appearance. Eighty photographs were available for scoring. MAIN OUTCOME MEASURES: The interobserver and intraobserver reliability of the CARS for 18-year-old patients when used by cleft surgeons and medical students. RESULTS: The interobserver reliability for the nose and lip together was 0.64 for the cleft surgeons and 0.61 for the medical students. There was an intraobserver reliability of 0.75 and 0.78 from the surgeons and students, respectively, on the nose and lip together. No significant difference was found between the cleft surgeons and medical students in the way they scored the nose ( P = 0.22) and lip ( P = 0.72). CONCLUSIONS: The Cleft Aesthetic Rating Scale for 18-year-old patients has a substantial overall estimated reliability when the average score is taken from three or more cleft surgeons or medical students assessing the nasolabial aesthetics of CLP patients.
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Actitud del Personal de Salud , Labio Leporino/cirugía , Fisura del Paladar/cirugía , Estética/psicología , Nariz/anomalías , Adolescente , Femenino , Humanos , Masculino , Países Bajos , Evaluación de Resultado en la Atención de Salud , Fotograbar , Reproducibilidad de los Resultados , Estudios Retrospectivos , Estudiantes de Medicina/psicología , Cirujanos/psicologíaRESUMEN
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.
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Labio Leporino/fisiopatología , Labio Leporino/cirugía , Fisura del Paladar/fisiopatología , Fisura del Paladar/cirugía , Desarrollo Maxilofacial/fisiología , Adolescente , Cefalometría , Labio Leporino/diagnóstico por imagen , Fisura del Paladar/diagnóstico por imagen , Femenino , Humanos , Masculino , Adulto JovenRESUMEN
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.
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Fisura del Paladar/diagnóstico , Factores de Edad , Fisura del Paladar/mortalidad , Fisura del Paladar/patología , Parto Obstétrico , Femenino , Edad Gestacional , Humanos , Lactante , Recién Nacido , Estimación de Kaplan-Meier , Masculino , Derivación y Consulta , Estudios RetrospectivosRESUMEN
OBJECTIVE: The aim of this study was to assess the accuracy of prenatal transabdominal ultrasound in determining the oral cleft type. METHODS: A retrospective cohort study was performed on all consecutive cases of orofacial cleft diagnosed by prenatal ultrasound examination in the Wilhelmina Children's Hospital, a tertiary referral hospital, between January 2002 and December 2012. Prenatal findings were compared with postnatal diagnoses. RESULTS: A total of 134 patients were included. The mean gestational age at ultrasound examination was 24 weeks + 5 days. Prenatal diagnosis was in accordance with postnatal findings in 76.9% of the cases (103/134) with regard to the cleft type. Underestimation of the cleft occurred in 19.4% (26/134), whereas in 3.7% (5/134), the extent was overestimated. In distinguishing bilateral from unilateral clefts, no errors were made. CONCLUSION: Prenatal ultrasound is accurate in assessing the types of orofacial clefts in a large majority of the cases and is completely accurate in distinguishing between unilateral and bilateral clefts. This study indicates that it is a reliable technique to assess the cleft type, which is important for counseling future parents. Although, the clinician should be aware of the fact that a cleft palate is easily missed, and subsequently, underestimation of cleft extend is frequent.
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Labio Leporino/diagnóstico por imagen , Fisura del Paladar/diagnóstico por imagen , Errores Diagnósticos/estadística & datos numéricos , Ultrasonografía Prenatal , Diagnóstico Diferencial , Femenino , Estudios de Seguimiento , Edad Gestacional , Humanos , Recién Nacido , Masculino , Embarazo , Estudios RetrospectivosRESUMEN
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.
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Fisura del Paladar/fisiopatología , Ingestión de Alimentos , Niño , Femenino , Humanos , Masculino , Prevalencia , Estudios Retrospectivos , Factores de RiesgoRESUMEN
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.
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Fisura del Paladar/fisiopatología , Ingestión de Alimentos , Humanos , Prevalencia , Estudios RetrospectivosRESUMEN
OBJECTIVE: The aim of the study was to measure the degree of premaxillary protrusion in fetuses with orofacial clefts of various severities. METHODS: The maxilla-nasion-mandible (MNM) angle was measured retrospectively on by multiplanar corrected volumes. Sixty-two fetuses with orofacial clefts and known outcome volumes of the fetal head were available. RESULTS: In 48 of the 62 cases, the MNM angle could be measured [mean gestational age 23 (range, 18-30) weeks]. The mean MNM angle was normal in all nine cases with cleft lip and intact alveolar ridge (15.2°; range, 12.5°- 16.9°). In 24 cases with unilateral cleft lip with or without cleft palate (UCL/P), the mean MNM angle was 20.0° (range, 13.3-26.2°), being above the 95th percentile in 79% (n = 19) and normal in 21% (n = 5). In 14 bilateral cleft lip and palate (BCL/P) cases, the mean MNM angle was 26.5° (range, 19.2°-33.7°) and above the 95th percentile in all cases. There was no difference in MNM angle between isolated clefts and clefts associated with other anomalies. In one case with a Tessier 4 cleft, the MNM angle was above the 95th percentile (25.2°). CONCLUSION: The premaxilla tends to protrude in both BCL/P as UCL/P cases. The degree of protrusion varies greatly, especially in the BCL/P group.
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Labio Leporino/diagnóstico por imagen , Fisura del Paladar/diagnóstico por imagen , Maxilar/diagnóstico por imagen , Ultrasonografía Prenatal , Femenino , Humanos , Maxilar/anomalías , Embarazo , Estudios RetrospectivosRESUMEN
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.
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Cromosomas Humanos Par 22/genética , Trastornos del Habla/fisiopatología , Insuficiencia Velofaríngea/genética , Insuficiencia Velofaríngea/fisiopatología , Adolescente , Niño , Preescolar , Femenino , Eliminación de Gen , Humanos , Estudios Longitudinales , Masculino , Pronóstico , Estudios Retrospectivos , Síndrome , Resultado del Tratamiento , Insuficiencia Velofaríngea/cirugíaAsunto(s)
Antagonistas Adrenérgicos beta/uso terapéutico , Atenolol/uso terapéutico , Hemangioma/tratamiento farmacológico , Neoplasias Cutáneas/tratamiento farmacológico , Cicatrización de Heridas/efectos de los fármacos , Femenino , Hemangioma/patología , Hemangioma/fisiopatología , Humanos , Lactante , Recién Nacido , Masculino , Neoplasias Cutáneas/patología , Neoplasias Cutáneas/fisiopatología , Úlcera Cutánea/tratamiento farmacológico , Úlcera Cutánea/patología , Úlcera Cutánea/fisiopatologíaRESUMEN
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.
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Labio Leporino/diagnóstico por imagen , Fisura del Paladar/diagnóstico por imagen , Cara/diagnóstico por imagen , Ultrasonografía Prenatal , Labio Leporino/embriología , Labio Leporino/epidemiología , Fisura del Paladar/embriología , Fisura del Paladar/epidemiología , Cara/anomalías , Cara/embriología , Femenino , Estudios de Seguimiento , Humanos , Recién Nacido , Masculino , Países Bajos/epidemiología , Valor Predictivo de las Pruebas , Embarazo , Resultado del Embarazo , Diagnóstico Prenatal , Estudios Prospectivos , Medición de Riesgo , Sensibilidad y EspecificidadRESUMEN
Infection after reconstructive surgery for microtia is a technical challenge. This can be a sign of cholesteatoma formation by entrapment of epithelium in the middle or outer ear, specifically when the patient does not respond to first choice antibiotic therapy and debridement. Two patients with microtia presented themselves with severe infections after ear reconstruction. In both cases cholesteatoma was diagnosed as the cause of the infection. After cholesteatoma management an additional surgical procedure was necessary to improve the esthetic outcome. The plastic surgeon should identify possible signs of cholesteatoma after reconstruction of the auricle.
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BACKGROUND: Auricular reconstruction for microtia is most frequently performed using autologous costal cartilage (ACC) or porous polyethylene (PPE) implants. Short-term results are generally promising, but long-term results remain unclear. Long-term outcomes were explored in this systematic review, and minimal reporting criteria were suggested for future original data studies. METHODS: A systematic literature search was conducted in MEDLINE, EMBASE, and the Cochrane Central Register of Controlled Trials from inception through October 14, 2020. Articles on auricular reconstruction in patients with microtia using ACC or PPE were included if postsurgical follow-up was at least 1 year. Outcome reporting was split into separate publications, and results on complications were reported previously. This publication focused on long-term aesthetic, patient-reported, and sensitivity outcomes. RESULTS: Forty-one publications reported on these outcomes. Both materials led to aesthetically pleasing results and high rates of patient satisfaction. ACC frameworks grew similarly to contralateral ears, and the anterior surface of auricles regained sensitivity. Furthermore, postoperative health-related quality of life (HRQoL) outcomes were generally good. Data synthesis was limited due to considerable variability between studies and poor study quality. No conclusions could be drawn on the superiority of either method due to the lack of comparative analyses. CONCLUSION: Future studies should minimally report (1) surgical efficacy measured using the tool provided in the UK Care Standards for the Management of Patients with Microtia and Atresia; (2) complications including framework extrusion or exposure, graft loss, framework resorption, wire exposure and scalp/auricular scar complications and (3) HRQoL before and after treatment using the EAR-Q patient-reported outcome measure (PROM).
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Microtia Congénita/cirugía , Estética , Medición de Resultados Informados por el Paciente , Procedimientos de Cirugía Plástica/métodos , Cartílago Costal/trasplante , Humanos , Satisfacción del Paciente , Complicaciones Posoperatorias , Prótesis e ImplantesRESUMEN
BACKGROUND: Microtia is a rare disorder characterized by malformation or even complete absence of the auricle. Reconstruction is often performed using autologous costal cartilage (ACC) or porous polyethylene implants (PPE). However, the long-term outcomes of both methods are unclear. OBJECTIVE: This systematic review aimed to analyze long-term complications and suggest minimal reporting criteria for future original data studies. METHODS: A systematic literature search was conducted in MEDLINE, EMBASE and the Cochrane Central Register of Controlled Trials from inception through October 14, 2020. Articles on auricular reconstruction in patients with microtia using ACC or PPE were included provided that the follow-up period was at least one year. This publication focused on long-term complications reported in patients with a postoperative follow-up period of at least one year. RESULTS: Twenty-nine publications reported on complications during long-term follow-up. Overall long-term complication rates were not reported. The incidence of individual complications during long-term follow-up was less than 10% after ACC reconstruction and less than 15% in PPE reconstruction. Framework resorption and wire exposure were reported even after an extended follow-up of more than five years after ACC reconstruction, while reports on the extended long-term results of PPE reconstruction are limited. Data synthesis was limited due to heterogeneity and poor study quality. CONCLUSIONS: Future studies should report on long-term complications including framework exposure or extrusion, graft loss, framework resorption, wire exposure and scalp and auricular scar complications. We recommend a surgical follow-up of at least five years.
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Microtia Congénita/cirugía , Procedimientos de Cirugía Plástica/métodos , Complicaciones Posoperatorias , Cartílago Costal/trasplante , Humanos , Prótesis e ImplantesRESUMEN
BACKGROUND: While research has shown that children with single sided deafness have a lower quality of life and developmental outcomes compared to normal hearing peers, little is known about these domains in children with unilateral congenital conductive hearing loss due to aural atresia. OBJECTIVES: This study aims to investigate the hearing-related quality of life, developmental outcomes and educational performance in children and young adults with unilateral conductive hearing loss due to aural atresia. METHODS: Nineteen children and young adults with unilateral aural atresia received a set of five questionnaires. Hearing-related quality of life (SSQ), general quality of life (Kidscreen-27), speech and language development (CCC-2-Nl), educational performance and problems in social-emotional and behavioral domains (CBCL/YSR/ASR) were measured with validated questionnaires. Scores on the questionnaires were compared to their norm scores. Mann-Whitney U tests and independent t-tests were used to identify significant differences between age groups. RESULTS: Mean scores on the SSQ subscales were speech 6.78, spatial 5.00 and quality 6.98. Mean scores on the Kidscreen-27, CCC-2-NL, CBCL/YSR/ASR fell within normal or non-clinical range. A high number of cases needed speech therapy (60.7%) or special measures in class (79.3%) or showed grade repetition (>30%) in primary or secondary school. CONCLUSION: Children and young adults with unilateral conductive hearing loss due to congenital aural atresia showed lower scores regarding hearing-related quality of life compared to normal-hearing peers. The result show similarities with children with single sided deafness. Regarding general quality of life, speech and language development and in social-emotional and behavioral domains the studied children and young adults seem to develop according to norm scores. It is important to observe these children closely as they may need guidance during education to allow them to thrive.
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Audífonos , Pérdida Auditiva Unilateral , Niño , Oído , Audición , Pérdida Auditiva Conductiva/diagnóstico , Pérdida Auditiva Conductiva/etiología , Pérdida Auditiva Unilateral/etiología , Pruebas Auditivas , Humanos , Calidad de Vida , Adulto JovenRESUMEN
Bioengineering of the human auricle remains a significant challenge, where the complex and unique shape, the generation of high-quality neocartilage, and shape preservation are key factors. Future regenerative medicine-based approaches for auricular cartilage reconstruction will benefit from a smart combination of various strategies. Our approach to fabrication of an ear-shaped construct uses hybrid bioprinting techniques, a recently identified progenitor cell population, previously validated biomaterials, and a smart scaffold design. Specifically, we generated a 3D-printed polycaprolactone (PCL) scaffold via fused deposition modeling, photocrosslinked a human auricular cartilage progenitor cell-laden gelatin methacryloyl (gelMA) hydrogel within the scaffold, and cultured the bioengineered structure in vitro in chondrogenic media for 30 days. Our results show that the fabrication process maintains the viability and chondrogenic phenotype of the cells, that the compressive properties of the combined PCL and gelMA hybrid auricular constructs are similar to native auricular cartilage, and that biofabricated hybrid auricular structures exhibit excellent shape fidelity compared with the 3D digital model along with deposition of cartilage-like matrix in both peripheral and central areas of the auricular structure. Our strategy affords an anatomically enhanced auricular structure with appropriate mechanical properties, ensures adequate preservation of the auricular shape during a dynamic in vitro culture period, and enables chondrogenically potent progenitor cells to produce abundant cartilage-like matrix throughout the auricular construct. The combination of smart scaffold design with 3D bioprinting and cartilage progenitor cells holds promise for the development of clinically translatable regenerative medicine strategies for auricular reconstruction.