Madrid 1997 World Down Syndrome Conference Controversial Therapies Workshops
REVIEW OF THE MEDICAL THERAPIES IN DOWN'S SYNDROME
Romano, C.
Department of Pediatrics, Oasi Institute, Via Conte Ruggero, 73, 94018 Troina (EN), (Italy). Phone 39-935-936111. Fax 39-935-653327. E-mail pediat@oasi.en.it.
The innumerable drug therapies established in people affected by Down's syndrome (DS) testify to the complexity of the topic. I would like to separate it in two parts. The first section is made up of the therapies set up as a treatment of DS, all considered unquestionable today. Pituitary extract, glutamic acid and its derivatives, thyroid hormone, 5-hydroxytryptophan, pyridoxine, dimethyl sulfoxide, cell therapy, megadoses of vitamins, and "U" series will be first reviewed. The second section is made up of those therapies used for specific conditions encountered throughout the life of people with DS. The use of zinc sulphate for the treatment of growth delay, immune derangement and hypothyroidism will be commented. The possible therapy of the subclinical hypothyroidism with L-thyroxin will be then addressed. The use of growth hormone will be subsequently reviewed. The last reported treatment will be facial plastic surgery. The final message is that a person with DS must be monitored up with a clinical schedule which encompasses the early diagnosis of the most frequently prone conditions. An early rehabilitation in the areas of swallowing, chewing, speech and motor skills, without overloading the child, remains essential in order to achieve the best quality of life. Experimental treatments may be suggested only within specific research projects.
STATE OF THE ART ABOUT REPAIR AND PLASTIC SURGERY ON D.S
The facial appearance is able to provide a barrier between an individual and his acceptance in society. Some of the facial characteristics in children with DS are:A habitually open mouth; a large, furrowed tongue protruding from the mouth and fissured lips; a flat nasal bridge which causes epicanthal folds; dysplastic ears; a fat neck. There are two ways in which the plastic surgeon can help the children and the parents:
By reducing or removing the handicaps caused by the macroglossia.
By correction of a distorted facial appearance. Approved operations are as follows: Partial resection of an oversized tongue, lifting of a flat nasal bridge, correction of dysplastica ears, correction of a fat neck. All these operations may be carried out in one session, done under general anesthesia. The correction of macroglossia aims to eliminate irritation in eating, drinking and speaking and to enable the mouth to be shut, which was made possible in 60%. 25% can close their mouth during the day, but have them open at night. With a smaller tongue the children can chew thoroughly and drink without drooling. Breathing through the nose leads to less frequent airway diseases in 83%.Of the nearly 400 children who have been operated on since 1978 all were treated for macroglossia. Nearly 90% of the parents of the operated children would recommend this operation to other parents. Although most families are coping well with their children with DS, occasionally the facial stigmata may be so significant that social integration can be eased only by corrections which are offered by plastic surgery.
CIRUGIA ORAL EN EL SINDROME DE DOWN
Lucini, H.
CBA. Argentina Phone/Fax: 051243686
CONCLUSIONES QUIRÚRGICAS
El niño con Síndrome de Down presenta una alteración anatomofuncional muy específica. En él encontramos una deformidad del paladar óseo, una diástasis muscular en el velo (variable de acuerdo al grado del síndrome) que corregimos sin excéresis agresiva (rombo o triángulo) con resultados similares a técnicas ya descritas.Hemos obtenido resultados de mejoría en la articulación de la palabra; del tono muscular de la cara de la deglución.La cirugía mejora la sinergia del velo y labio superior; se comienza a movilizar el platisma, comenzando por la mejilla y ascendiendo hasta llegar a la frente. El labio inferior debe corregirse del mismo modo, pues también presenta una disrrafia, mejorando la protusión del mismo. Creemos que esta cirugía de realizarse en aquellos pacientes cuyo órgano de comunicación presenta alteraciones que así lo requiera; previo rapport con los padres para manifestarles el verdadero alcance de la misma.
ASPECTO FONOAUDIOLÓGICO
Mejora la función protésica fisiológica lingual.
Disminución del Síndrome de empuje lingual.
Presencia de disrafia labial inferior que rompe el equilibrio del espinter labio-yugal.
Mejoramiento de la globalización de la coordinación fonorespiratoria y fonodeglutoria.
Mejoramiento de la dispraxias vegetativas logrando equilibrio mas compensado, durante los periodos de la deglución, sobre todo de alimentos sólidos y líquidos.
Reconstrucción del arco esfinteriano labio yugal como prevención de malas oclusiones.
Prevención de patologías periodontales y de la característica clase tres.
Utilización de ortodoncia comunes para la secuela en malas oclusiones y en alineaciones dentarias.
THE HARMONIOUS OROFACIAL DEVELOPMENT IN A CHILD WITH DS: AN IMPORTANT ELEMENT FOR SOCIAL INTEGRATION
Giuca, M.R., Bargagna, S.*, Marrapese, E.
Universitá degli Studi di Pisa. Istituto di Clinica Odontoiatrica. Via Roma, 67 56100 Pisa (Italia) Fax: 050-555232
The typical facial aspect of the Down person is the result of many factors which disturb the normal maturing of oral and maxilofacial structures. The Authors suggest two different protocols of therapeutic intervention for early correction of alterated craniofacial development in child with the syndrome. The diagnostic criteria, the most common therapeutic means, the aims short term and long-term regabilitative therapy are described. Special attention is given to the orthodontic prevention and treatment of malocclusion related to the syndrome, as well as to the therapy of muscular disfunctions.