Desafios clínicos e psicossociais no tratamento de um paciente com síndrome de Proteus / Clinical and psychosocial challenges in the treatment of a patient with Proteus syndrome

A Síndrome de Proteus é uma rara associação de malformações que podem afetar vários tecidos e órgãos. É caracterizada por macrodactilia bilateral, hipertrofia craniana, anomalias ósseas, escoliose, hamartomas de tecidos moles, nevo verrucoso pigmentar, anormalidades viscerais e outras hipertrofias. Há pouco mais de 200 casos notificados em todo o mundo. O presente artigo relata a evolução clínica de um paciente pediátrico com essa síndrome. A criança apresentou desnutrição grave associada ao extremo gigantismo de membros inferiores. Além disso, apresentou repercussões psicossociais relacionadas à exclusão social. A doença tornou-se mais grave e progrediu como síndrome consumptiva. Finalmente, os pais concordaram com a amputação dos membros inferiores hipertróficos. Um ano após as amputações, a criança estava totalmente reabilitada, protetizada dos membros amputados, com melhora nutricional, além de apresentar notória recuperação psicológica e reinserção social, o que representou melhora significativa da qualidade de vida para o paciente.

Proteus syndrome is a rare combination of malformations that can affect several tissues and organs. It is characterized by bilateral macrodactyly, cranial hypertrophy, bone anomalies, scoliosis, soft-tissue hamartomas, verrucous pigmented nevus, visceral abnormalities and other forms of hypertrophy. Just over 200 cases have been reported worldwide. This article reports on the clinical course of a pediatric patient with this syndrome. The child had severe malnutrition associated with extreme gigantism of the lower limbs and also psychosocial problems related to social exclusion. As the disease progressed it exacerbated and evolved into a wasting syndrome. After several years, the parents agreed to amputation of the hypertrophic lower limbs. One year after the amputations the child had been rehabilitated and had adapted to prostheses, with nutritional improvement and notable psychological recovery and social reintegration, which represented a significant improvement in his quality of life.

Transposition flap for correction of ectopic breast in Poland's syndrome using a pre-molded silicone prosthesis / Retalho de transposição para correção de mama ectópica em síndrome de Poland usando prótese de silicone pré-moldada

The authors report a variation of Poland's syndrome with ectopic right breast. Three surgeries were performed at different times: transposition flap to place the mammary gland in an anatomic position; reduction of the contralateral left breast; and implantation of a pre-molded silicone prosthesis to correct the malformation caused by the absence of the major and minor pectoral muscles. The surgeries were performed at six-month intervals, and the final outcome was new and acceptable positioning of the breasts.

Os autores apresentam uma variante da síndrome de Poland com mama direita ectópica. Foram realizados três tempos cirúrgicos: retalho de transposição para colocação da glândula mamária em posição anatômica; redução da mama contralateral esquerda; e implantação de prótese de silicone pré-moldada para correção do defeito causado pela agenesia dos músculos peitoral maior e peitoral menor. Com intervalo de seis meses entre as cirurgias, o resultado final apresenta novo posicionamento aceitável das mamas.

Begging on the Streets of Addis Ababa: an Impact of Musculoskeletal Disability

Background: This was a 2-year interventional prospective study aimed at determining the frequency and pattern of musculoskeletal disability among beggars of the streets of Addis Ababa. It was part of a continuous multidisciplinary study that was trying to assess causes of street begging and looked for ways to stop it or at least bring it to 'tolerable' proportions. This part of the study mainly focused on treatable/correctable musculoskeletal disabilities leading to begging on streets in the city. It also assessed the degree; duration and reasons for street begging and determined whether correcting treatable musculoskeletal disabilities stopped beggars from begging or not. The study setting was in Addis Ababa city; in collaboration with C.A.R.D.O.S. Ethiopia. Methods: This was an interventional prospective follow-up study on beggars of the streets of Addis Ababa who claimed musculoskeletal disability as their main cause for begging. A location in a sub city was selected for a reason of hosting the largest number of beggars. In collaboration with the local administrator a clinic was opened amidst the busy street and volunteer street beggars with musculoskeletal disability were recruited for the study. Surgical procedures were performed in 61 'patients' and were followed for two years; from April 2007-April 2009. Some beggars refused a clearly beneficial surgery Results: Our survey revealed there are 1;237 street beggars including the outskirts of 'Entoto' mountain. Nearly two-third of the street beggars were males and age ranged from a week to 90. Of the 204 beggars with musculoskeletal disability; 118 were evaluated to clearly benefit from a successful surgical procedure. The commonest diagnosis was leprosy with its complications recorded in 47 of the 204; followed by bone and joint infections; 13.2( 27/204) and complex; unclear congenital anomalies ranked third. Neglected dislocations; mal-united of non-united fractures were observed in twenty (9.8) of the street beggars. Iatrogenic cause was discovered as a cause of disability in six beggars. Sixty one beggars were operated. The ages for operated cases ranged from 12 to 78 years. The duration of begging in beggars selected for surgery was from 4.5 to 56 years. Corrective amputation; Bone grafting and Sequestrectomy were the commonest procedures in respected order. One patient died due to concomitant cardiac illness. From the whole group 68 patients went back to begging while from the operated group only a single patient recently was found begging in one of the streets in Addis. Conclusion: Musculoskeletal disability may lead to begging. Well-funded; multi-sectoral long-term campaign on begging will possibly reduce it to a 'tolerable' level

La minifijación ósea externa en las malformaciones congénitas / The external bony minifixation in congenital malformations

Se realizó un estudio de los distintos procederes quirúrgicos con el sistema de minifijación ósea externa RALCA a 74 pacientes portadores de alguna malformación congénita operados en el CCOI "Frank País" desde 1990 hasta 1999. Se presentan los resultados obtenidos con dicho procedimiento en el difícil tratamiento de estas entidades

Reconstrucción de la pared torácica mediante prótesis sintética moldeada durante el acto operatorio / Abdominal wall of the reconstruction intervening synthetical prothesis during surgery acto

La reparación de la pared torácica consecutiva a resecciones extensas de esqueleto debidas por lo general a neoplasias, debe ser cuidadosamente efectuada con la finalidad de restaurar la rigidez y la estabilidad del torax, previniendo trastornos ventilatorios que de otro modo inevitablemente ocurrirían. A 4 pacientes que habían sido sometidos a la exéresis parcial de varias costillas, 3 de ellos con parte de esternón, se les colocó una prótesis fabricada con malla de marlex y metilmetacrilato. Para confeccionar esa prótesis fue utilizado un molde o matriz de aluminio con la forma y el tamaño de una sección de la pieza operatoria; por consiguiente aquélla se adaptó con bastante precisión a la brecha parietal. Se describe el procedimiento. El resultado funcional fue muy satisfactorio.