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- A 63-Year-Old Woman Presenting with a Synovial Sarcoma of the Hand: a Case ReportPublication . Casal, D; Ribeiro, AI; Mafra, M; Azeda, C; Mavioso, C; Mendes, MM; Mouzinho, MMINTRODUCTION: Synovial sarcoma is a high-grade, soft-tissue sarcoma that most frequently is located in the vicinity of joints, tendons or bursae, although it can also be found in extra-articular locations. Most patients with synovial sarcoma of the hand are young and have a poor prognosis, as these tumors are locally aggressive and are associated with a relatively high metastasis rate. According to the literature, local recurrence and/or metastatic disease is found in nearly 80% of patients. Current therapy comprises surgery, systemic and limb perfusion chemotherapy, and radiotherapy. However, the 5-year survival rate is estimated to be only around 27% to 55%. Moreover, most authors agree that synovial sarcoma is one of the most commonly misdiagnosed malignancies of soft tissues because of their slow growing pattern, benign radiographic appearance, ability to change size, and the fact that they may elicit pain similar to that caused by common trauma. CASE PRESENTATION: We describe an unusual case of a large synovial sarcoma of the hand in a 63-year-old Caucasian woman followed for 12 years by a multidisciplinary team. In addition, a literature review of the most pertinent aspects of the epidemiology, diagnosis, treatment and prognosis of these patients is presented. CONCLUSION: Awareness of this rare tumor by anyone dealing with hand pathology can hasten diagnosis, and this, in turn, can potentially increase survival. Therefore, a high index of suspicion for this disease should be kept in mind, particularly when evaluating young people, as they are the most commonly affected group.
- Abcesso do Espaço de Parona com Origem em Foco Sético Oral: Abordagem MultidisciplinarPublication . Fonseca, L; Ribeiro, L; Pinheiro, J; Silva Guerra, AA infeção do espaço de Parona é uma patologia rara do antebraço. Reportamos um caso de um homem de 55 anos, com dor e impotência funcional na porção distal do antebraço, sem história de trauma, com uma semana de evolução. Associadamente, apresentava história de infeção odontogénica, síncrona com o início das queixas do antebraço, mas com resolução espontânea. A ecografia do punho documentou um abcesso, que foi prontamente drenado e o exame microbiológico demonstrou infeção por Streptococcus constellatus. A avaliação estomatológica revelou agudização sintomática de periodontite crónica no dente 2.7. O doente realizou a eliminação de focos séticos orais durante o internamento e teve alta com evolução favorável. As manifestações do quadro clínico e o agente isolado confirmam a origem odontogénica. Do que pudemos apurar, este é o primeiro caso publicado de um abcesso antebraquial odontogénico e demonstra a importância da eliminação sistemática de focos séticos orais na população geral.
- Abdominoplasty and Thoraco-Epigastric Flaps for Large Anterior Trunk Defects after Dermatofibrosarcoma Protuberans Wide Resection: Two Illustrative CasesPublication . Casal, D; Fradinho, N; Ramos, L; Ferreira, J; Varanda, A; Diogo, C; Baltazar, J; Fernandes, M; Correia, C; Almeida, MAINTRODUCTION: Excision of large dermatofibrosarcoma protuberans in the anterior aspect of the trunk often results in large surgical defects that frequently dictate the need for microsurgical reconstruction. However, this option is not always available. PRESENTATION OF CASE: The authors describe two patients with very large anterior trunk dermatofibrosarcoma protuberans: one in the epigastric region and the other in the hypogastric region. In the patient with the hypogastric tumor, a classical abdominoplasty flap associated with umbilical transposition was used to cover the skin defect after muscle and fascial plication, and placement of a polypropylene mesh. In the patient with the epigastric tumor, a synthetic mesh was also placed, and the skin and subcutaneous defect was reconstructed with a reverse abdominoplasty flap and two thoraco-epigastric flaps. In both cases, complete closure was possible without immediate or late complications. DISCUSSION: The local options described in this paper present several potential advantages compared to microsurgical reconstruction, namely they are easier and faster to perform and teach; they provide a good skin color and texture match; they are not associated with distant donor site morbidity; follow-up is usually less cumbersome; the post-operative hospital stay tends to be shorter; they are less costly; they are less prone to complete failure. CONCLUSION: The authors believe that these two patients clearly show that local flaps, although frequently neglected, continue to be valid options for reconstructing large anterior trunk defects, even in the current era of microsurgery enthusiasm.
- Agenésia da VaginaPublication . Melo, F; Godinho, CNos últimos dez anos foram tratados dez doentes com o diagnóstico de agenésia da vagina. A reconstrução foi realizada com recurso à técnica de McIndoe. Faz-se a análise retrospectiva dos doentes tratados, com referência ao diagnóstico (S. Mayer-Rokitansky-7, S. Androgenital-1, S. Testículo feminizante-2), sua classificação, tratamento, complicações e resultados. Na opinião dos autores a técnica de McIndoe é a mais indicada para o tratamento destas malformações, pela sua simplicidade, baixa morbilidade e ausência de mortalidade.
- An Unexpected Infection in a Venous Leg Ulcer: a Case ReportPublication . Veríssimo, M; Guimarães, D; Casal, D; Carvalho, S; Matias, M; Barbosa, R; Bexiga, JProvidencia rettgeri is a Gram-negative bacillus that is most usually seen in urinary tract infections. Skin and soft tissue infections with P rettgeri are rare and there few case reports described in the literature. A 43-yearold woman presented to the emergency department with a leg ulcer of 3 months duration. Microbiological results isolated P rettgeri and Pseudomonas aeruginosa. She underwent surgical debridement and split-thickness skin grafting alongside IV antibiotic therapy and dressing care, with complete resolution of the wound 3 months after admission. The differential diagnosis of leg ulcers is wide and must include infection and venous insufficiency. Although venous ulcers are common, typically they are not responsible for rapid evolving ulcers that destroy tissues below the muscular fascia. In this case report, the authors hypothesise that P rettgeri had a major role in the severity of the ulcer in an otherwise healthy woman. A greater understanding of how P rettgeri affects wound pathophysiology is needed in order to distinguish between colonisation versus infection and discriminate bacterial synergy, as well as having better treatment guidelines.
- Associated Malformations in Children with Orofacial Clefts in Portugal: A 31-Year StudyPublication . Pereira, A; Fradinho, N; Carmo, S; de Sousa, J; Rasteiro, D; Duarte, R; Leal, MJBACKGROUND: Orofacial clefts are among the most common congenital craniofacial malformations and may be associated with other birth defects. However, the proportion and type of additional anomalies vary greatly between studies. This study assessed the prevalence and type of associated congenital malformations in children with orofacial clefts, who attended the largest cleft lip and palate tertiary referral center in Portugal. METHODS: Consecutive children with orofacial clefts who attended at least 1 consultation in our Clefts Unit between 1981 and 2012 were studied. Demographic and clinical data regarding the number and type of associated malformations were retrospectively collected and analyzed. RESULTS: Of the 701 patients studied, 219 (31.2%) had associated congenital malformations. These malformations were more frequent in children with cleft palate (43.4%) than in children with cleft lip and palate (27.5%) or with cleft lip only (19.4%). Within the group with associated anomalies, 73 cases (33.3%) had conditions related with known chromosomal defects, monogenic syndromes or sequences, and 146 cases (66.7%) had multiple congenital anomalies of unknown origin. From those, head and neck malformations were the most common (60.3%), followed by malformations in the cardiovascular (28.3%) and musculoskeletal systems (26%). CONCLUSIONS: The overall prevalence of associated malformations of nearly 1 in 3 children with orofacial clefts stressed the need for a comprehensive evaluation of these patients by a multidisciplinary cleft team. Moreover, one-third of the children had multiple congenital anomalies of known origins. Thus, early routine screening for other malformations and genetic counseling might be valuable for orofacial clefts management.
- Associated Malformations in Children with Orofacial Clefts in Portugal: A 31-Year StudyPublication . Pereira, AV; Fradinho, N; Carmo, S; de Sousa, JM; Rasteiro, D; Duarte, R; Leal, MJOrofacial clefts are among the most common congenital craniofacial malformations and may be associated with other birth defects. However, the proportion and type of additional anomalies vary greatly between studies. This study assessed the prevalence and type of associated congenital malformations in children with orofacial clefts, who attended the largest cleft lip and palate tertiary referral center in Portugal.
- Avaliação do Tratamento Cirúrgico da Artrose TrapeziometacárpicaPublication . Azeda, C; Mendes, MM; Mouzinho, MMThe authors describe their experience, from July 1995 to December 1996, in the management of patients suffering from trapeziometacarpal degenerative arthrosis. Dell's classification is used for disease staging. The results of two different techniques are compared: 1) distal tenotomy of supranumerary insertions of abductor pollicis longus; and 2) trapeziectomy with tendon interposition arthroplasty and ligamentoplasty using the flexor carpi radialis.
- BD-2 and BD-3 Increase Skin Flap Survival in a Model of Ischemia and Pseudomonas Aeruginosa InfectionPublication . Casal, D; Iria, I; Ramalho, JS; Alves, S; Mota-Silva, E; Mascarenhas-Lemos, L; Pontinha, C; Guadalupe-Cabral, M; Ferreira-Silva, J; Ferraz-Oliveira, M; Vassilenko, V; Goyri-O'Neill, J; Pais, D; Videira, PThe main aim of this work was to study the usefulness of human β-defensins 2 (BD-2) and 3 (BD-3), which are part of the innate immune system, in the treatment of infected ischemic skin flaps. We investigated the effect of transducing rat ischemic skin flaps with lentiviral vectors encoding human BD-2, BD-3, or both BD-2 and BD-3, to increase flap survival in the context of a P. aeruginosa infection associated with a foreign body. The secondary endpoints assessed were: bacterial counts, and biofilm formation on the surface of the foreign body. A local ischemic environment was created by producing arterialized venous flaps in the left epigastric region of rats. Flaps were intentionally infected by placing underneath them two catheters with 105 CFU of P. aeruginosa before the surgical wounds were hermetically closed. Flap biopsies were performed 3 and 7 days post-operatively, and the specimens submitted to immunohistochemical analysis for BD-2 and BD-3, as well as to bacterial quantification. Subsequently, the catheter segments were analyzed with scanning electron microscopy (SEM). Flaps transduced with BD-2 and BD-3 showed expression of these defensins and presented increased flap survival. Rats transduced with BD-3 presented a net reduction in the number of P. aeruginosa on the surface of the foreign body and lesser biofilm formation.
- Blood Supply to the Integument of the Abdomen of the Rat: A Surgical PerspectivePublication . Casal, D; Pais, D; Iria, I; Videira, P; Mota-Silva, E; Alves, S; Mascarenhas-Lemos, L; Pen, C; Vassilenko, V; Goyri-O'Neill, JBACKGROUND: Many fundamental questions regarding the blood supply to the integument of the rat remain to be clarified, namely the degree of homology between rat and humans. The aim of this work was to characterize in detail the macro and microvascular blood supply to the integument covering the ventrolateral aspect of the abdominal wall of the rat. METHODS: Two hundred five Wistar male rats weighing 250-350 g were used. They were submitted to gross anatomical dissection after intravascular colored latex injection (n = 30); conversion in modified Spalteholz cleared specimens (n=10); intravascular injection of a Perspex solution, and then corroded, in order to produce vascular corrosion casts of the vessels in the region (n = 5); histological studies (n = 20); scanning electron microscopy of vascular corrosion casts (n = 10); surgical dissection of the superficial caudal epigastric vessels (n = 100); and to thermographic evaluation (n = 30). RESULTS: The ventrolateral abdominal wall presented a dominant superficial vascular system, which was composed mainly of branches from the superficial caudal epigastric artery and vein in the caudal half. The cranial half still received significant arterial contributions from the lateral thoracic artery in all cases and from large perforators coming from the intercostal arteries and from the deep cranial epigastric artery. CONCLUSIONS: These data show that rats and humans present a great deal of homology regarding the blood supply to the ventrolateral aspect of the abdominal integument. However, there are also significant differences that must be taken into consideration when performing and interpreting experimental procedures in rats.