Antonio Carlos Fernandes

QI EM PACIENTES COM HIDROCEFALIA E MIELOMENINGOCELE : IMPLICACOES DO TRATAMENTO CIRURGICO

Myelomeningocele occurs in 0.4 for 1000 neonates and is associated with hydrocephalus in 85-90%, and reports on cognition are sparsely found in literature. Forty five children with treated hydrocephalus and myelomeningocele were studied in regard of IQ, and statistically correlated to functional motor level, age of the first shunt, number of revisions of shunt, infection of the shunt and circumference of the head. The medium age was of 7.5 years (3-15 years), 16 males and 29 females. Three (6.6%) had a IQ score >110, 11 (24.4%) had a score between 100-110, 8 between 85-100 (17.7%), 16 (35.5%) between 85-100 (17.7%) and 7 (15.5%) between 50-70. IQ directly correlated with motor level, having better cognitive results the children with minor functional motor disabilities. Cognition was best in children operated until the seven day of life (t 0.0099), with progressive worse results in children operated after the first month of life, no significance was observed in children operated in the period 7 to 31 days (t 0.1013). Worse results were observed in the group of patients with infection of shunts (t 0.0146). Results were progressively worse with reoperations. The best results in relation of the circumference of the head were seen with children in the medium range (t 0.0115); intermediate results were seen in patients between the medium range and-1 SD (t 0.00130) and medium range and + 1SD. The worse results were seen in patients at the extremes of >1SD (t 0.0269) and <1SD (t 0.0042). According to cognitive results the surgical treatment of hydrocephalus have to be done until the first month of life, avoiding reoperations and infections that have unfavorable impact in IQ.

Tratamento cirúrgico da escoliose na amiotrofia espinhal progressiva

OBJETIVO: Descrever os resultados imediatos e tardios da cirurgia para correcao de escoliose em pacientes com amiotrofia espinhal progressiva (AEP). METODO: 14 pacientes com AEP submetidos a cirurgia para escoliose foram selecionados para analise retrospectiva dos prontuarios e radiografias, assim como para exame radiografico e aplicacao de um questionario na consulta final. O tempo medio de seguimento foi de 22 meses. A escoliose media pre-operatoria foi de 78,4° pelo metodo de Cobb. Todos os pacientes apresentavam obliquidade pelvica (media de 25,5°) e 11 apresentavam cifose. A media de idade a epoca da cirurgia foi de 12 anos e 3 meses. Em 12 pacientes foi realizada artrodese via posterior com instrumental de Luque-Galveston, e em 2 com instrumental de Cotrel-Dubousset. RESULTADOS: Foi obtida correcao no pos-operatorio imediato de 64,3% da escoliose, 36,4% da cifose e 70,9% da obliquidade pelvica. As complicacoes foram fistula liquorica e infeccao precocemente em um caso; e tardiamente soltura da amarria de T1, em dois casos. Houve perda media de correcao no final do seguimento de 0,26° da escoliose e de 1,28° da obliquidade pelvica. Os pais e os pacientes referiram boa melhora em relacao aos aspectos esteticos, postura, equilibrio para sentar e cuidados de higiene, assim como das intercorrencias respiratorias. CONCLUSAO: A cirurgia para escoliose na AEP tem impacto satisfatorio quanto a estetica, qualidade de vida e funcao respiratoria dos pacientes, com minima perda da correcao obtida com o tempo e com poucas complicacoes.

Hypothalamic tumor associated with atypical forms of anorexia nervosa and diencephalic syndrome

We report the case of a 10-year-old girl with a mature teratoma in the hypothalamic region. The patient presented a 2-month history of anorexia, psychic disturbances and a 37% loss of body weight. These symptoms had led initially to a diagnosis of major depression and atypical anorexia nervosa. She also presented some signs and symptoms of diencephalic syndrome. This case illustrates the importance of considering a slow-growing mass as a rare but real possibility in the differential diagnosis of anorexia nervosa, mainly in atypical cases.

Avaliação da função motora em crianças com distrofia muscular congênita com deficiência da merosina

Congenital muscular dystrophy (CMD) is a heterogeneous group of disorders characterized by early onset of hypotonia and weakness. Almost 50% of the cases are caused by primary deficiency of a protein named merosin (MD), and present a homogenous phenotype with a severe motor and respiratory involvement. Eleven children with clinical and histological diagnosis of CMD-MD, aged of 3 to 15 years, were studied using the manual muscle testing (Medical Research Council), goniometric analysis, motor ability and day life activities (Barthel index) scales, with the objective to characterize the main motor function limitations. The muscular groups most affected were cervical flexors, paravertebral and proximal portions of limbs. The muscular groups of upper limbs were as affected as the lower limbs, and the extensors were more affected than the flexors groups. All children had severe muscular retractions on the hip, knee and elbow. Other frequent deformities were scoliosis and equinus-varum feet. No children presented the motor ability to walk, stand up and crawl; and all of them were classified as dependents or semi-dependents in the day life activities scale. Our findings confirm the severe and diffuse involvement of skeletal muscle in CMD-MD patients, producing serious motor limitations and deformities.

Surgical treatment for myelodysplastic clubfoot

Objective To analyze the results from surgical treatment of 69 cases of clubfoot in 43 patients with myelodysplasia according to clinical and radiographic criteria, at our institution between 1984 and 2004. Methods This was a retrospective study involving analysis of medical files, radiographs and consultations relating to patients who underwent surgical correction of clubfoot. The surgical technique consisted of radical posteromedial and lateral release with or without associated talectomy. Results The patients’ mean age at the time of the surgery was four years and two months, and the mean length of postoperative follow-up was seven years and two months. Satisfactory results were achieved in 73.9% of the feet and unsatisfactory results in 26.1% (p < 0.0001). Conclusion Residual deformity in the immediate postoperative period was associated with unsatisfactory results. Opening of the Kite (talocalcaneal) angle in feet that only underwent posteromedial and lateral release, along with appropriate positioning of the calcaneus in cases that underwent talectomy, was the radiographic parameter that correlated with satisfactory results.

Tratamento da subluxação e luxação do quadril na paralisia cerebral em pacientes tetraparéticos espásticos, nível motor V, através datenotomia de adutores e psoas

Objective: To assess if adductor and psoas release is effective in prevent hip displacement in quadriplegic Cerebral Palsy pacients, Gross motor V. Methods: A retrospective study with 26 patients who had psoas and adductor release was analyzed. Reimers Index before and after surgery was analysed. We divided the patients into two groups: surgery made in patients above five yars and after five years of age. Results: With a follow up of seven years, most of the patients of both groups had Reimers Index controlled by the surgery proposed. Conclusion: Adductor and psoas release was effective in controlled hip displacement in quadriplegic Cerebral Palsy patients, Gross Motor V. The age at the time of the surgery does not influence in the result.Reimers Index up to 70% and asymmetric hip not had good results.