Ilza Lazarini Marques

Clinical experience with infants with Robin sequence: a prospective study.

OBJECTIVE To study the clinical course of patients with Robin sequence (RS) during the first 6 months of life. DESIGN A longitudinal prospective study of children with RS. SETTING Hospital de Reabilitação de Anomalias Craniofaciais, Universidade de São Paulo, Bauru-SP, Brazil, 1997 and 1998. PATIENTS Sixty-two children were studied from hospital admission to 6 months of age. Thirty-three (53.2%) presented with probable isolated RS (PIRS), 25 (40.3%) presented with syndromes or other malformations associated with RS, and 4 (6.5%) presented with RS with neurological involvement. INTERVENTIONS The type of respiratory tract obstruction was defined by nasopharyngoscopy. The patients with type 1 and type 2 obstruction underwent nasopharyngeal intubation (NPI), and glossopexy was indicated in patients with type 1 obstruction who did not show clinical improvement with this procedure. Tracheostomy was indicated in patients with type 2 obstruction who did not show a good course after NPI, in patients with type 1 obstruction who did not show good course after glossopexy, and in patients with type 3 and type 4 obstruction. RESULTS Prone position treatment (PPT) or NPI was the definitive treatment in 25 cases (75.8%) of PIRS and in 13 cases (52%) of syndromes or other malformations. Among the children with type 1 obstruction, 24 (51.1%) were submitted exclusively to PPT and 12 (25.5%) to NPI. With the type 2 groups, only one (12.5%) received PPT, and three (37.5%) were treated exclusively with NPI. All 15 infants treated exclusively with NPI (24.4%) presented with good weight, length, and neuromotor development. CONCLUSIONS Most patients with PIRS and type 1 obstruction improved without surgical intervention. NPI should be the initial treatment in all patients with RS with type 1 and type 2 obstruction who present with important respiratory and feeding difficulties.

Prospective clinical trial comparing outcome measures between Furlow and von Langenbeck Palatoplasties for UCLP.

The goal of this prospective randomized clinical trial was to compare 2 cohorts of standardized cleft patients with regard to functional speech outcome and the presence or absence of palatal fistulae. The 2 cohorts are randomized to undergo either a conventional von Langenbeck repair with intravelar velarplasty or the double-opposing Z-plasty Furlow procedure. A prospective 2 × 2 × 2 factorial clinical trial was used in which each subject was randomly assigned to 1 of 8 different groups: 1 of 2 different lip repairs (Spina vs. Millard), 1 of 2 different palatal repair (von Langenbeck vs. Furlow), and 1 of 2 different ages at time of palatal surgery (9–12 months vs. 15–18 months). All surgeries were performed by the same 4 surgeons. A cul-de-sac test of hypernasality and a mirror test of nasal air emission were selected as primary outcome measures for velopharyngeal function. Both a surgeon and speech pathologist examined patients for the presence of palatal fistulae. In this study, the Furlow double-opposing Z-palatoplasty resulted in significantly better velopharyngeal function for speech than the von Langenbeck procedure as determined by the perceptual cul-de-sac test of hypernasality. Fistula occurrence was significantly higher for the Furlow procedure than for the von Langenbeck. Fistulas were more likely to occur in patients with wider clefts and when relaxing incisions were not used.

Seqüência de Robin: protocolo único de tratamento

Objective: To present a single protocol that might cover both the respiratory and feeding difficulties of neonates and infants with Robin sequence. Sources of data: The article was prepared on the basis of the most recent publications available in bibliographic databases and in books that discuss the treatment of Robin sequence, especially the studies conducted at the Hospital for Rehabilitation of Craniofacial Anomalies of Universidade de Sao Paulo (HRAC/USP). Summary of the findings: We present the morphological and genetic aspects of Robin sequence and concepts about nasopharyngoscopy and its clinical implications; we discuss the treatment of respiratory and feeding difficulties, and we present a single protocol for the treatment of all Robin sequence cases regardless of their severity and complexity. Conclusions: Robin sequence is not only an anatomic obstructive disorder to be treated with surgical procedures, but knowledge about childrenis growth and development must be applied by a multidisciplinary team, since this permits the maintenance of airway permeability and of the ability to feed orally, often without the need of surgical procedures and their risks, especially when applied to neonates and small infants.

Growth impairment of children with different types of lip and palate clefts in the first 2 years of life: a cross-sectional study

OBJECTIVE To analyze the differences in growth impairment according to sex in the 2 first years of life in children with three types of clefts. METHODS This was a cross-sectional study of 881 children (58.9% boys and 41.1% girls) with cleft lip and palate treated at the Craniofacial Anomaly Rehabilitation Hospital, (University of São Paulo, Bauru, SP), Brazil. Age ranged from 1 to 24 months. Three types of clefts were evaluated: isolated cleft lip (181/20.5%), isolated cleft palate (157/17.8%) and cleft lip + palate (543/61.6%). Weight and length measurements and data regarding breast-feeding and socioeconomic level were obtained. Children with weight and length below the 10th percentile of the NCHS reference were considered to have impaired growth. RESULTS Sample distribution according to cleft type and sex was similar to that observed in other epidemiological studies. Breast-feeding was more frequent in the isolated cleft lip group (45.9%) then in the isolated cleft palate (12.1%) or cleft lip + palate group (10.5%). Isolated cleft lip children showed less marked impairment of weight (23.8%) and length (19.3%) compared to the cleft lip + palate group (35.7% and 33.1%, respectively). In the latter group, the proportion of children with weight and length below the 10th percentile was very close to that of the isolated cleft palate group (34.4% and 38.9%). CONCLUSIONS The impairment in weight and length was more severe in cleft lip + palate and isolated cleft palate children and may be attributed to feeding difficulties compared to the isolated cleft lip group.

Nasopharyngoscopy in Robin sequence: clinical and predictive value.

Objective To correlate nasopharyngoscopic findings with clinical manifestations during the first month of life and study the course of respiratory obstruction during the first year in infants with Robin sequence (RS). Design A longitudinal prospective study of children with RS. Setting Hospital de Reabilitação de Anomalias Craniofaciais, University of São Paulo, Bauru-SP, Brazil, 1998 to 2000. Patients Fifty-six children were studied from the age of 1 month to 12 months. Interventions The type of respiratory obstruction was defined by nasopharyngoscopy. Patients for whom glossoptosis was the only mechanism of respiratory obstruction were classified as having mild, moderate, or severe glossoptosis by nasopharyngoscopy and as mild, moderate, or severe cases with respect to the clinical manifestations. Results Forty-two (75%) patients showed respiratory obstruction caused by glossoptosis; seven (43.7%) of these infants with mild clinical manifestations showed moderate glossoptosis during the first month of life and five (31.3%) presented severe glossoptosis; 10 (45.5%) of the infants with severe clinical manifestations showed moderate and 11 (50.0%) severe glossoptosis. At 12 months of age, glossoptosis was mild or absent in 83.3% of the patients, moderate in 14.3% and severe in 2.4%. Conclusions A poor correlation between the severity of glossoptosis and the severity of clinical manifestations was observed for patients with respiratory obstruction caused by glossoptosis during the first month of life, but the correlation between glossoptosis and respiratory distress according to age was statistically significant. Nasopharyngoscopy is not a good method for predicting the severity of the clinical course of respiratory obstruction caused by glossoptosis.

Prejuízo no crescimento de crianças com diferentes tipos de fissura lábio-palatina nos 2 primeiros anos de idade. Um estudo transversal

OBJETIVO: Analisar as diferencas da restricao de crescimento ate o segundo ano de vida, com base no genero, entre criancas com tres tipos de fissuras. METODOS: Estudo transversal com 881 criancas (58,9% meninos e 41,1% meninas) com fissura labial e palatina do Hospital de Reabilitacao de Anomalias Craniofaciais, Universidade de Sao Paulo, Bauru (SP), Brasil, com idades entre 1 a 24 meses. Foram avaliados tres tipos de fissuras: fissura labial (181/20,5%), fissura palatina (157/17,8%) e fissura labial + palatina (543/61,6%). Foram obtidas as medidas de peso e comprimento e os dados acerca do aleitamento materno e do nivel socioeconomico. Criancas com peso e comprimento abaixo do percentil 10 da referencia NCHS foram consideradas como tendo restricao do crescimento. RESULTADOS: A distribuicao da amostra de acordo com o tipo de fissura e genero foi semelhante aquela observada em outros estudos epidemiologicos. O aleitamento materno foi mais frequente no grupo com fissura labial (45,9%) que nos grupos de fissura palatina (12,1%) ou de fissura labial + palatina (10,5%). Os lactentes com fissura labial mostraram menos comprometimento do peso (23,8%) e do comprimento (19,3%) comparados aqueles do grupo com fissura labial + palatina (35,7% e 33,1%, respectivamente), sendo que o ultimo grupo mostrou proporcoes de criancas com peso e comprimento inferiores ao percentil 10 muito proximas as do grupo com fissura palatina (34,4% e 38,9%). CONCLUSOES: O comprometimento do peso e comprimento e mais grave nos lactentes com fissura labial + palatina e com fissura palatina e pode ser atribuido principalmente as dificuldades de alimentacao, em comparacao ao grupo com fissura labial.

Nasopharyngeal Intubation in Robin Sequence: Technique and Management:

Objective: To provide a detailed description of the nasopharyngeal intubation (NPI) technique and photographs, which should be helpful for those who may need to perform it for treating the airway obstruction in Robin sequence. Design: To describe and illustrate the NPI technique and the necessary considerations for its application. Setting: Hospital de Reabilitação de Anomalias Craniofacial of University of São Paulo, Brazil. Result: The NPI procedure involves the use of a whitish, Portex, number 3.0 or 3.5, silicone tube, introduced 8 cm deep into the infants nostril and fixed with Micropore tape. The tube is to be removed at least twice a day for proper hygiene (with running water, detergent, and swabs) and should be changed every 7 days. This procedure is taught to the childrens parents or caretakers by the nurse during hospitalization. Conclusion: The technique is so simple that it can be performed by the parents themselves, allowing continuation of the treatment at home.

Growth of children with isolated Robin sequence treated by nasopharyngeal intubation: importance of a hypercaloric diet.

Objective To compare the growth curves (weight and length) of two groups of infants with isolated Robin sequence (RS) treated with nasopharyngeal intubation (NPI), one group receiving a hypercaloric diet and the other receiving a normal diet for age, and compare the growth rates and duration of NPI between the two groups. Design Prospective longitudinal study. Setting Hospital de Reabilitação de Anomalias Craniofaciais, Universidade de São Paulo, Bauru, Brazil, 1998 to 2000. Patients Twenty-three children with isolated RS treated by prolonged NPI. Interventions Nine children received a milk formula supplemented with 5% to 7% glucose polymers and 3% to 5% medium chain triglycerides (group 1), and 14 children received a milk formula only (group 2). Weight and length were measured at birth and every month up to 6 months of life for the construction of weight and length growth curves. The time of use of the nasogastric tube, age at the beginning of NPI, and duration of NPI were also determined. Results No significant difference in length was observed between the two groups. The mean weight curves obtained for the two groups were below the 50th percentile of the National Center for Health Statistics, but weight gain was higher in group 1. The duration of NPI was shorter in this group. Conclusions The hypercaloric diet led to an improvement in nutritional status, which in turn contributed to improved respiratory conditions in isolated RS.

Otologic and audiologic outcomes with the Furlow and von Langenbeck with intravelar veloplasty palatoplasties in unilateral cleft lip and palate.

Objective Cleft palate increases the risk of chronic middle ear disease and hearing loss. The goal of this report was to determine which of two palate surgeries and which timing of palate surgery were associated with better otologic and audiologic outcomes in children with unilateral cleft lip and palate at 5 to 6 years of age. Design Subjects were randomly assigned to the von Langenbeck with intravelar veloplasty or Furlow palate repair, to palate surgery at 9 to 12 months or 15 to 18 months of age, and to the Spina or Millard lip repair. Setting Centralized, tertiary care craniofacial treatment center. Patients A total of 673 infants with unilateral cleft lip and palate. Interventions Palate and lip were repaired using established techniques. Serial otoscopic and audiometric evaluations were performed. Main Outcome Measures Hearing and otoscopic findings at 5 to 6 years old. Results There were 370 children available for analysis. Hearing and need for tympanostomy tube placement did not differ by palatoplasty, age at palatoplasty, cheiloplasty, or surgeon. Risk of developing cholesteatoma or perforation was higher with Millard cheiloplasty (odds ratio = 5.1, 95% confidence interval = 1.44 to 18.11, p = .012). Type and age at palatoplasty were not significantly associated with either the rate of developing these sequelae or the rate of achieving bilaterally normal hearing and ear examinations. Conclusions Type of palatoplasty did not influence otologic and audiologic outcomes in 5- to 6-year-olds with unilateral cleft lip and palate. The potential influence of lip repair on otologic outcomes warrants further investigation.

Longitudinal Study of Growth of Children with Unilateral Cleft-Lip Palate from Birth to Two Years of Age:

Objective: To study the growth of children with complete unilateral cleft lip and palate (UCLP) from birth to 2 years of age and to construct specific UCLP growth curves. Design: Physical growth was a secondary outcome measure of a National Institutes of Health–sponsored longitudinal, prospective clinical trial involving the University of Florida (United States) and the University of São Paulo (Brazil). Patients: Six hundred twenty-seven children with UCLP, nonsyndromic, both genders. Methods: Length, weight, and head circumference were prospectively measured for a group of children enrolled in a clinical trial. Median growth curves for the three parameters (length, weight, head circumference) were performed and compared with the median for the National Center for Health Statistics (NCHS) curves. The median values for length, weight, and head circumference at birth and 6, 12, 18, and 24 months of age were plotted against NCHS median values and statistically compared at birth and 24 months. Setting: Hospital de Reabilitação de Anomalias Craniofaciais, Universidade de São Paulo, Bauru, Brazil (HRAC-USP). Results: At birth, children of both genders with UCLP presented with smaller body dimensions in relation to NCHS median values, but the results suggest a catch-up growth for length, weight, and head circumference for girls and for weight (to some degree) and head circumference for boys. Conclusions: Weight was the most compromised parameter for both genders, followed by length and then head circumference. There was no evidence of short stature. This study established growth curves for children with UCLP.