RESUMEN
OBJECTIVE: The purpose of this study was to categorize and compare outcomes and sequels in 2 groups of patients born with unilateral and bilateral complete cleft lip/palate, having their primary cleft palate repair performed in our hospital, by the same surgical team, during 2 different periods of time, to establish which of the 2 surgical reconstructive strategies employed was more effective to decrease sequels. MATERIAL AND METHODS: This is a randomized clinical trial including a total of 291, nonsyndromic patients, primary assisted in our Hospital, between 2002 and 2013, and operated by the same senior surgeon.Two groups of patients of similar characteristics were treated utilizing 2 different surgical procedures according to the considered period. Isolated palates, syndromic patients, secondary and adult cases were considered as exclusion critters. Surgical data was obtained from medical records, and clinical examinations. All the patients were cautiously evaluated by a team expert to verify results, evolution, and sequels. RESULTS: Statistically significant differences in the total percentage of complications were found between both groups. Group A: 54.85% and Group B: 21.90% (P value < 0.001).Each complication was also considered by groups and estimated as follow: CONCLUSIONS:: Based on the results of our research, we can suggest the Carstens' variant plus the introduced modifications by the authors, as a useful surgical procedure to be utilized in primary complete unilateral or bilateral cleft palate repair to prevent post op common complications.
Asunto(s)
Fisura del Paladar/cirugía , Preescolar , Labio Leporino/cirugía , Femenino , Estudios de Seguimiento , Humanos , Lactante , Masculino , Procedimientos Neuroquirúrgicos , Procedimientos de Cirugía Plástica/métodosRESUMEN
OBJECTIVE: To introduce a different approach for maxillary nerve block (MNB), in cleft palate repair. To reduce the use of opioids during surgery and to prevent frequent respiratory complications by means of an adequate intra and postoperative pain relief. PATIENTS AND METHODS: A prospective clinical trial was planned, to collect scientific evidences between 2 groups of patients with primary cleft palate, receiving surgery in 2 Pediatric centers of Buenos Aires, utilizing a different protocol.Sixty patients undergoing primary cleft palate repair in both hospitals, from January 2017 to July 2018, by senior surgeons and the same expert anesthesiologists' team, were included.Syndromic and secondary cases, and patients whose parents rejected to participate of this study were excluded. The first group called Hospital A included 45 children, the second group identified as Hospital B was formed by 15 patients.A combination of general whit local anesthesia and a bilateral regional MNB, was used in all the patients of the Hospital A. Utilizing an aspirating syringe, children received 0.15âml/kg of lidocaine clorhidrate 2% with epinephrine 1:50.000, under direct vision through the spheno palatine holes, just before surgery. A traditional general anesthesia procedure plus local anesthesia, was utilized in all the patients treated at the Hospital B Medial blood pressure and cardiac frequency parameters were tested during induction, along the surgical procedure and in the immediate post op, to detect any sign of pain (12). After surgery, patient reactivity, airway depression symptoms, time of initial feeding and discharge time, were also monitored (13).This study was approved by the Hospitals Ethics Committees of both hospitals, and is in accordance with the 1975 Helsinki Declaration, as amended in 1983. The parents have signed an informed consent form for all the patients included. RESULTS: Patients of both groups did not show any significant variant in the monitored parameters to detect signals of pain, along the surgery. The rest of controls during and after surgery showed significant differences in favor of the patients of Hospital A. CONCLUSIONS: Bilateral regional MNB, under direct vision trough the spheno palatine holes results an effective, easy, and safe method for pain relief during and after primary cleft palate repair surgeries.The combination of slight general anesthesia with local anesthesia and regional blocks, results a good option to reduce opioids utilization, to prevent neurotoxicity, respiratory depression, sickness, and vomiting facilitating early feeding and patient discharge.
Asunto(s)
Fisura del Paladar/cirugía , Nervio Maxilar , Anestesia Local , Preescolar , Epinefrina , Femenino , Humanos , Lactante , Lidocaína , Masculino , Nervio Maxilar/cirugía , Bloqueo Nervioso/métodos , Nervios Periféricos , Estudios ProspectivosRESUMEN
OBJECTIVE: Evaluate simulation-based comprehensive cleft care workshops as a reproducible model for education with sustained impact. DESIGN: Cross-sectional survey-based evaluation. SETTING: Simulation-based comprehensive cleft care workshop. PARTICIPANTS: Total of 180 participants. INTERVENTIONS: Three-day simulation-based comprehensive cleft care workshop. MAIN OUTCOME MEASURES: Number of workshop participants stratified by specialty, satisfaction with the workshop, satisfaction with simulation-based workshops as educational tools, impact on cleft surgery procedural confidence, short-term impact on clinical practice, medium-term impact on clinical practice. RESULTS: The workshop included 180 participants from 5 continents. The response rate was 54.5%, with participants reporting high satisfaction with all aspects of the workshop and with simulation-based workshops as educational tools. Participants reported a significant improvement in cleft lip (33.3 ± 5.7 vs 25.7 ± 7.6; P < .001) and palate (32.4 ± 7.1 vs 23.7 ± 6.6; P < .001) surgery procedural confidence following the simulation sessions. Participants also reported a positive short-term and medium-term impact on their clinical practices. CONCLUSION: Simulation-based comprehensive cleft care workshops are well received by participants, lead to improved cleft surgery procedural confidence, and have a sustained positive impact on participants' clinical practices. Future efforts should focus on evaluating and quantifying this perceived positive impact, as well reproducing these efforts in other areas of need.
Asunto(s)
Labio Leporino , Fisura del Paladar , Labio Leporino/cirugía , Fisura del Paladar/cirugía , Simulación por Computador , Estudios Transversales , HumanosRESUMEN
INTRODUCTION: A cleft of the primary and/or secondary palate involve almost every function of the face except vision. Today, an isolate cleft palate or a complete cleft lip/palate should not be considered as an adverse condition because surgical repair has reached a highly satisfactory level. Nevertheless, for the average cleft surgeon palatoplasty still remains an enigma. PLANNING THE STRATEGY: Having an observational and an anatomical measurement methodology of some pre op parameters, it would make possible to predict a complicate repair. In that way, the surgeon can select the best closure strategy, minimize surgical aggression, and even prevent the presence of sequels. Palates must be reviewed just before the operation, under general anesthesia, with the patient and the Dingman retractor in place. By using a precision caliper at least 4 parameters should to be considered to select the right strategy. CONCLUSIONS: Since 2009, author has selected the alveolar extension palatoplasty with complete muscle dissection and retro positioning, plus posterior pillars elongation with a hemi-uvula rotation and reconstruction as the procedure of choice for complete primary cleft palate repair. The utilization of the pre op mentioned parameters to identify cleft palate diversity and severity seems to be useful to select the correct strategy to perform cautious surgical procedures.
Asunto(s)
Fisura del Paladar/cirugía , Estudios de Seguimiento , Humanos , Lactante , Procedimientos Neuroquirúrgicos , Procedimientos de Cirugía Plástica , Resultado del Tratamiento , Úvula/cirugíaRESUMEN
INTRODUCTION: Chronic otitis media with effusion (OME) is a recurrent complication, usually found in cleft palate patients.Conductive hearing loss is the result of the Eustachian tube dysfunction caused by the absence of fusion and the altered insertion of the muscles of the secondary palate. It is also the consequence of an ineffective muscular reconstruction after primary cleft palate repair. METHODS: This is a cohort study to compare 4 groups of patients born with isolated cleft lip (ICL), unilateral cleft lip/palate (UCLP), bilateral cleft lip/palate (BCLP), and isolated cleft palate (ICP), received in our hospital between June 2015 to September 2017, operated by the same surgeon, using the same surgical technique and protocol.Complete cleft palate repair was performed, in average, at 10 months, and placement of ventilation tubes, if necessary, was made in the same operatory act.After palate repair, primary or secondary hearing loss was checked, joint to the connection with the type of used ventilation tubes, recurrences and complications also were considered. RESULTS: The study sample was integrated by 69 patients, 2 of 11 patients with ICL (18.18%), 30 of 34 patients with UCLP (88.23%), 17 of 19 patients with BCLP (89.47%), and 4 of 5 patients with ICP (80.00%) were diagnosed with OME requiring ventilation tubes at the time of surgery. It can be established that the average hearing loss in patients with diabolos in the postoperative period is 19.4 db and in those patients with T tubes it is 14.2 db, the difference being statistically significant (Pâ<â0.05). CONCLUSION: Hearing improvement prior to language acquisition is essential for a proper speech development. Early trans tympanic tubes implantation during cleft palate repair contributes to a correct short-term ventilation of the middle ear, being the T tubes the best option.
Asunto(s)
Labio Leporino/cirugía , Fisura del Paladar , Pérdida Auditiva Conductiva , Ventilación del Oído Medio/métodos , Otitis Media con Derrame , Procedimientos de Cirugía Plástica/métodos , Argentina/epidemiología , Audiometría/métodos , Niño , Preescolar , Fisura del Paladar/complicaciones , Fisura del Paladar/diagnóstico , Fisura del Paladar/fisiopatología , Fisura del Paladar/cirugía , Estudios de Cohortes , Femenino , Pérdida Auditiva/etiología , Pérdida Auditiva Conductiva/diagnóstico , Pérdida Auditiva Conductiva/etiología , Humanos , Lactante , Masculino , Otitis Media con Derrame/diagnóstico , Otitis Media con Derrame/etiología , Otitis Media con Derrame/fisiopatología , Otitis Media con Derrame/terapia , Periodo Perioperatorio , Resultado del Tratamiento , Membrana Timpánica/diagnóstico por imagenRESUMEN
Bilateral complete clefts represent the result of an incomplete fusion with all the morphologic components present. It is well known that patients with bilateral cleft lip and palate have typical characteristics such as insufficient medial face development with an orthodontic class III tendency, flat nose and short columella with abnormal nasolabial angle, bilateral oronasal fistulas, alterations in the number and position of the lateral incisors, and agenesis or supernumerary teeth. Successfully solving these cases, results in a difficult challenge and studies showing extended follow-up are not frequent. Bilateral complete clefts, including medial facial dysplasia, are a rare condition, not only difficult to be included in any classification but also of complex solution. These patients require multiple surgical procedures throughout life, and long-term results are often still far from ideal. Due to surgical intervention and diminished intrinsic growth potential, surgical results may change from initially good into a progressively disappointing outcome. However, if the ideal timing and type of surgery are known, in combination with the intrinsic growth potential, these results could be ameliorated. A patient with complete bilateral cleft, presenting hypoplasia of philtrum and premaxilla, flat nose with rudimentary columella and septum, is reported. A description of her interdisciplinary treatment and long-term outcome with an adequate and stable result was observed. Being the intrinsic growth restriction mainly localized in the central mid-face, a protocol oriented to stimulate facial development during growth period could be essential to reduce the number of surgical procedures and prevent sequels. Early referral to a specialized center is mandatory to achieve a correct treatment and result.
Asunto(s)
Labio Leporino/cirugía , Fisura del Paladar/cirugía , Efectos Adversos a Largo Plazo , Nariz , Procedimientos de Cirugía Plástica , Cuidados Posteriores , Cara/anomalías , Cara/cirugía , Femenino , Humanos , Lactante , Labio/cirugía , Efectos Adversos a Largo Plazo/etiología , Efectos Adversos a Largo Plazo/prevención & control , Desarrollo Maxilofacial , Nariz/anomalías , Nariz/cirugía , Enfermedades Nasales/congénito , Fístula Oral/cirugía , Procedimientos de Cirugía Plástica/efectos adversos , Procedimientos de Cirugía Plástica/métodos , Tiempo de TratamientoRESUMEN
: Van der Woude syndrome is the most generic form of syndromic orofacial cleft, present in approximately 2% of all cleft patients. The lower lip pits with or without cleft lip and/or palate is typical of this syndrome. Popliteal pterygium syndrome (PPS), also known as popliteal web syndrome or fasciogenito-popliteal syndrome, was first described by Trelat in 1869, the incidence is approximately 1 in 300,000 live births. The term PPS was coined by Gorlin et al in 1968 based on the most unusual anomaly, the popliteal pterygium. Popliteal pterygium syndrome shares features with van der Woude syndrome but, in addition, is characterized by genital anomalies, syndactyly of fingers and toes, and toenail dysplasia. In some patients, oral or eyelid synechiae are present. Van der Woude syndrome and PPS are autosomal dominantly inherited disorders caused by heterozygous mutations in IRF6. OBJECTIVE:: To report the familial nature of the disease in the mother and son, and to summarize the clinical characteristics, treatment, and outcomes in both patients.
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Anomalías Múltiples , Labio Leporino , Fisura del Paladar , Quistes , Anomalías del Ojo , Dedos/anomalías , Articulación de la Rodilla/anomalías , Labio/anomalías , Deformidades Congénitas de las Extremidades Inferiores , Sindactilia , Anomalías Urogenitales , Femenino , Humanos , MasculinoRESUMEN
OBJECTIVE: To evaluate the effects and durability of results after using dynamic presurgical nasoalveolar remodeling in patients with unilateral and bilateral cleft lips and palates. METHOD: Dynamic presurgical nasoalveolar remodeling technique does not rely on the relatively static force exerted by the orthopedic plate held in place by means of tape or adhesives. The principle behind this procedure is the use of the force generated during suction and swallowing to produce the remodeling effects on the nasal structures. The nasal component acts not only on the nasal structures but also in the lip function by stimulating labial muscle contraction. Direct anthropometry performed before primary reconstruction of the lip and nose and 1 year later posterior to surgery suggests that results are permanent. CONCLUSIONS: When the dynamic presurgical nasoalveolar remodeling protocol is instituted early, it can avoid memory cartilage fixation. Changes incorporated to the nasal extension allowed us to obtain better results, elevating the nasal tip, remodeling the depressed cleft side alar cartilages in unilateral cases, and repositioning and correcting the positional alteration of the nasal cartilages building a new columella in bilateral cases. In addition, comfort and tolerance of patients has been improved, and adjustments needed during follow-up have been simplified, significantly reducing the consultation time and increasing the interval between appointments. The obtained outcome facilitates primary surgical cleft lip and nose reconstruction and improves surgical results.
Asunto(s)
Labio Leporino/terapia , Cartílagos Nasales/anomalías , Deformidades Adquiridas Nasales/terapia , Cuidados Preoperatorios/métodos , Férulas (Fijadores) , Proceso Alveolar/fisiopatología , Estudios de Casos y Controles , Fisura del Paladar/terapia , Deglución/fisiología , Femenino , Humanos , Recién Nacido , Masculino , Cartílagos Nasales/fisiopatología , Cuidados Preoperatorios/instrumentación , Resultado del TratamientoRESUMEN
OBJECTIVE: To present technical modifications to the original presurgical nasal remodeling appliance introduced in 1991. The purpose of the modifications is to improve the cleft nasal deformity before unilateral and bilateral cleft lip repair. METHOD: The principle behind this technique, known as dynamic presurgical nasal remodeling (DPNR), is the use of the force generated during suction and swallowing. A conventional intraoral plate is built with a nasal extension added to the labial vestibular flange. The nasal extension was modified and consists of three components. The palatal plate is left loose in the mouth to generate a discontinuous but controlled impact directed to the affected nasal structures during suction and swallowing. The principle aim of the DPNR technique in unilateral cases is to improve the deformation of nasal structures by straightening the columella, elevating the nasal tip, and remodeling the depressed cleft side alar cartilages. In bilateral cases, the aims are to elongate the columella and to obtain nasal tip projection. CONCLUSIONS: The modifications introduced in the appliance enhance the original DPNR technique and are effective in ameliorating the initial cleft nasal deformity. This facilitates primary surgical cleft lip and nose correction and improves surgical outcomes in patients with complete unilateral and bilateral cleft lip and palate.
Asunto(s)
Labio Leporino/cirugía , Fisura del Paladar/cirugía , Nariz/anomalías , Diseño de Prótesis , Stents , Fenómenos Biomecánicos , Deglución , Humanos , Recién Nacido , Terapia Miofuncional/instrumentación , Nariz/crecimiento & desarrollo , Nariz/patología , Obturadores Palatinos , Cuidados Preoperatorios , Estrés Mecánico , Conducta en la LactanciaRESUMEN
OBJECTIVE: The purpose of this investigation was to study the social environment of families of children with different types of nonsyndromic oral clefts (OC) and to compare these groups with a control population of families of children without clefts. DESIGN: The study compared three nonsyndromic oral cleft groups and the control group using the Moos Family Environment Scale, which examines cohesion, expressiveness, conflict, independence, achievement-orientation, intellectual-cultural orientation, active-recreational orientation, moral-religious emphasis, organization, and control. SETTING: All parents of children with nonsyndromic oral clefts from a large craniofacial clinic in Buenos Aires, Argentina, were identified and were enrolled in this study between June 2000 and August 2001. Control families were ascertained from the pediatrics service of a hospital located in the vicinity of the craniofacial clinic. PARTICIPANTS: One hundred and sixty-five parents were selected, based on having a child with nonsyndromic unilateral cleft lip with or without cleft palate (UCL/P), bilateral cleft lip with or without cleft palate (BCL/P), or isolated cleft palate (ICP). One hundred and eighty control parents with no family history of congenital anomalies were selected, as well. RESULTS: There was no major difference in the social environment of families of children with different types of nonsyndromic oral clefts. When compared with families in the control group, families of children with nonsyndromic oral clefts scored better in all three subdimensions of family relationship, revealed a high level of independence, and showed better structure and organization than control families did; however, families of children with nonsyndromic oral clefts reported participating in fewer recreational activities. CONCLUSIONS: Overall, families of children with nonsyndromic oral clefts displayed a good social environment. Efforts should be focused to involve them in recreational activities.
Asunto(s)
Labio Leporino/psicología , Fisura del Paladar/psicología , Relaciones Familiares , Medio Social , Adaptación Psicológica , Adulto , Argentina , Estudios de Casos y Controles , Niño , Demografía , Femenino , Humanos , Masculino , Modelos Psicológicos , Oportunidad Relativa , Psicometría , Análisis de Regresión , Estrés Psicológico , Encuestas y CuestionariosRESUMEN
Results from a genome-wide screen of 10 multiplex families ascertained through probands with nonsyndromic cleft lip with or without cleft palate (CL/P) in Mexico, Argentina, and the United States yielded suggestive evidence of linkage to chromosomes 2, 6, 17 and 18. Fine mapping excluded all regions except chromosome 2. Subsequent analysis was performed on the original 10 families plus an additional 16 families using 31 markers on chromosome 2. This analysis showed intriguing evidence of linkage to 2q (Zlr=2.26, empirical P-value=0.028 in a chromosome-wide analysis). Transmission disequilibrium tests also revealed evidence of linkage and disequilibrium for two markers in this region (D2S168 and D2S1400 with P-values=0.022 and 0.006, respectively). A subset of these 26 families provided additional evidence for a susceptibility gene for CL/P on 2q, suggesting that further studies of genes in this region are warranted.
Asunto(s)
Cromosomas Humanos Par 2 , Labio Leporino/genética , Fisura del Paladar/genética , Ligamiento Genético , Mapeo Cromosómico , Femenino , Marcadores Genéticos , Predisposición Genética a la Enfermedad , Pruebas Genéticas , Humanos , Desequilibrio de Ligamiento , Escala de Lod , Masculino , Repeticiones de MicrosatéliteRESUMEN
OBJECTIVE: To investigate the attitudes of Argentine parents of children with nonsyndromic oral clefts with respect to prenatal diagnosis, termination of pregnancy, and reproduction and to assess the variables that might influence their responses. DESIGN: One hundred and sixty-five parents of children with oral clefts, ascertained from a craniofacial clinic in the suburbs of Buenos Aires, answered a 151-item semistructured questionnaire. The questionnaire included sections covering sociodemographic information, level of religiousness, characteristics of the child's cleft, parental perception of their child's cleft, pregnancy history, recurrence risk, access to health care, attitudes in regard to abortion, and family environment. RESULTS: Most parents (60%) believe their child's cleft is not a serious condition. None of the respondents would terminate a pregnancy because the ultrasound reveals an oral cleft. Similarly, very few (6.1%) would terminate the pregnancy if there were an early diagnosis of Down syndrome. Half of the respondents believe that abortion should not be an option for any couple expecting a child. CONCLUSIONS: Most respondents do not perceive oral clefts as a severe condition. Parents would not choose to terminate the pregnancy over delivery of such an affected newborn.
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Aborto Inducido/psicología , Actitud , Labio Leporino/psicología , Padres/psicología , Diagnóstico Prenatal/psicología , Adulto , Argentina , Niño , Labio Leporino/diagnóstico , Labio Leporino/diagnóstico por imagen , Labio Leporino/patología , Femenino , Humanos , Reproducción , Índice de Severidad de la Enfermedad , Encuestas y Cuestionarios , Ultrasonografía Prenatal/psicologíaRESUMEN
El presente trabajo tiene como objetivo realizar un análisis de los beneficios y contradindicaciones que podría producir la acción simultánea de modelar la nariz y el alvéolo, previo a la reconstrucción quirúrgica primaria en niños nacidos con fisura labio alveolopalatina uni o bilateral. Se realiza un repaso en los antecedentes del tema, se describe la técnica de impresión y el procedimiento de elaboración de la prótesis y se introducen importantes modificaciones en el componente nasal. Se concluye que existen múltiples veneficios al utilizar este tratamiento, con respecto de las técnicas tradicionales incluidas en la bibliografía
Asunto(s)
Humanos , Recién Nacido , Labio Leporino , Fisura del Paladar , Nariz , Obturadores Palatinos , Proceso Alveolar , Coloides , Materiales de Impresión Dental , Labio , Desarrollo Maxilofacial , Nariz , Aparatos Ortopédicos , Resinas Acrílicas/químicaAsunto(s)
Humanos , Recién Nacido , Lactante , Labio Leporino/terapia , Fisura del Paladar/terapia , Nariz/anomalías , Férulas (Fijadores)Asunto(s)
Humanos , Recién Nacido , Lactante , Fisura del Paladar/terapia , Labio Leporino/terapia , Nariz/anomalías , FerulaRESUMEN
La existencia de un sustituto cutáneo que reemplace las grandes pérdidas por trauma resulta indispensable en al actualidad. En los centros avanzados del mundo se está utilizando con éxito el cultivo de células epiteliales del propio paciente; si bien esto ha permitido salvar muchas vidas de pacientes con grandes superficies corporales quemadas, el método posee aún varios aspectos modificables tendientes a acercarlo a lo ideal. En nuestro país, sin embargo, deberán implementarse primero las condiciones y cambios básicos que permitan su aplicación y posterior desarrollo (AU)